56 ABPN Neurology Board Exam Practice Questions

Glasgow Coma Scale (GCS) 14, with focal neurological deficit(s) is the correct answer, as Grade 3 is described as a GCS of 13-14 in the presence of focal neurological deficits.

Glasgow Coma Scale (GCS) 15, with no focal neurological deficits is incorrect as Grade 1 is described as a GCS of 15, with absent focal neurological deficits.

Glasgow Coma Scale (GCS) 15, with focal neurological deficit(s) is incorrect as Grade 2 is described as a GCS of 13-14, with no focal neurological deficits.

Glasgow Coma Scale (GCS) 14, with no focal neurological deficits is incorrect as Grade 4 is described as a GCS of 7-12, in the presence or absence of focal neurological deficits.
Glasgow Coma Scale (GCS) 12, with no focal neurological deficit(s) is incorrect as Grade 5 is described as a GCS of < 7 in the presence or absence of focal neurological deficits.

Echocardiography is the correct answer as it is a noninvasive test with high sensitivity and specificity for the detection of proximal left marginal coronary artery and right coronary artery abnormalities.
White blood cell count is incorrect because although it will be raised, it is not a specific test for the evaluation or detection of Kawasaki disease.

Intravascular ultrasound (IVUS) is incorrect as this is used in select patients who have persistent fever, coronary abnormalities, ventricular dysfunction, pericardial effusion, or valvular regurgitation.

Transesophageal echocardiography is incorrect as this is used in select patients who have persistent fever, coronary abnormalities, ventricular dysfunction, pericardial effusion, or valvular regurgitation.

C-reactive protein is incorrect because although it will be raised, it is not a specific test for the evaluation or detection of Kawasaki disease.

Systolic BP of 180 mm Hg and diastolic BP of 105 mm Hg is correct. Intravenous thrombolysis with recombinant tissue plasminogen activator alteplase is administered in patients with acute ischemic stroke when the systolic blood pressure is less than 185 mm Hg and diastolic blood pressure is less than 110 mm Hg. Randomized controlled trials have concluded that intravenous alteplase administration in patients with systolic blood pressure more than 185 mm Hg and diastolic blood pressure more 110 mm Hg is associated with an increased risk of hemorrhage.

Systolic BP of 220 mm Hg and diastolic BP of 120 mm Hg is incorrect because intravenous thrombolysis with recombinant tissue plasminogen activator alteplase is administered in patients with acute ischemic stroke when the systolic blood pressure is less than 185 mm Hg and diastolic blood pressure is less than 110 mm Hg.

Systolic BP of 200 mm Hg and diastolic BP of 100 mm Hg is incorrect because intravenous thrombolysis with recombinant tissue plasminogen activator alteplase is administered in patients with acute ischemic stroke when the systolic blood pressure is less than 185 mm Hg and diastolic blood pressure is less than 110 mm Hg.

Systolic BP of 220 mm Hg and diastolic BP of 105 mm Hg is incorrect because intravenous thrombolysis with recombinant tissue plasminogen activator alteplase is administered in patients with acute ischemic stroke when the systolic blood pressure is less than 185 mm Hg and diastolic blood pressure is less than 110 mm Hg.

Systolic BP of 160 mm Hg and diastolic BP of 100 mm Hg is incorrect because intravenous thrombolysis with recombinant tissue plasminogen activator alteplase is administered in patients with acute ischemic stroke when the systolic blood pressure is less than 185 mm Hg and diastolic blood pressure is less than 110 mm Hg.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is correct because this patient has a positive diagnostic test of granular osmiophilic material (GOM). CADASIL is characterized by the presence of early-onset stroke and cognitive impairment, as is indicated in this patient. CADASIL is diagnosed by the detection of a genetic mutation in the NOTCH3 gene or a skin biopsy positive for GOM.

Arteriovenous malformation is incorrect because this patient has cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), and MRI has not shown any signs of arteriovenous malformation.

Subarachnoid hemorrhage is incorrect because neuroimaging studies in this patient do not show any signs of hemorrhage.

Sickle cell disease is incorrect because this patient has cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Sickle cell disease is characterized by vaso-occlusive pain crises from an early age, which are not present in this patient.

Cavernous sinus syndrome is incorrect because this patient has symptoms of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), characterized by early-onset stroke and cognitive impairment along with a NOTCH3 mutation. Cavernous sinus syndrome is characterized by the involvement of V1 and V2 divisions of the trigeminal nerve, abducens nerve, and internal carotid artery, which are not shown to be abnormal in this patient.

Intracerebral hemorrhage is correct because this patient developed sudden onset of left-sided paralysis shown as large hyperintensity on a CT scan with the presence of an arteriovenous malformation (AVM) in the right frontal region. Intracerebral hemorrhage in this patient is the most appropriate diagnosis secondary to an AVM pertaining to the patient’s location, age, and no history of trauma or existing hypertension.

Hydrocephalus is incorrect because the CT scan in this patient does not show any enlargement of ventricular regions. This patient is suffering from intracranial hemorrhage secondary to arteriovenous malformation (AVM).

Subdural hematoma is incorrect because this patient’s intraparenchymal hyperintensity is suggestive of intracerebral bleeding. A subdural hematoma presents as a crescent-shaped hemorrhage below the dura and outside of the arachnoid and brain tissue, usually followed by head injury, which is not present in this patient.

Transtentorial herniation is incorrect because this patient is suffering from intracerebral hemorrhage. Transtentorial herniation presents as a classic triad of coma, dilated pupils, and decerebrate posturing, none of which are present in this patient.

Post-traumatic epilepsy is incorrect because no history of seizures or head injury has been reported in this patient. The symptoms in this patient have been developed due to sudden intracerebral hemorrhage secondary to arteriovenous malformation (AVM).

Digital subtraction angiography (DSA) is correct because this patient is suspected of having an unruptured aneurysm from the right middle cerebral artery. DSA would be the most appropriate next step in the workup of this patient.

Transcranial Doppler ultrasonography (TCD) is incorrect because this patient is suspected of an unruptured cerebral aneurysm, and TCD has no role in diagnosing such patients. TCD is used to monitor blood flow and to evaluate for the presence of vasospasm, which is not necessary for this patient.

MR venography is incorrect because this patient is suspected of having a cerebral aneurysm. No suspicious pathology has been noted in the cerebral venous system; therefore, this option is not recommended in the workup of this patient.

Holter monitoring is incorrect because this patient is being evaluated for the presence of a possible cerebral aneurysm. Holter monitoring has no role in evaluating such patients. Holter monitoring is used to rule out atrial fibrillation, which is not needed in this patient as ECG and ECHO are within normal limits.

Transesophageal ECHO is incorrect because this patient has no significant findings in the transthoracic ECHO. He is being evaluated for a possible unruptured aneurysm. Transesophageal ECHO has no role in the workup of such patients.

As part of the evaluation of patients with suspected narcolepsy to confirm the diagnosis is correct. The following are indications for the MSLT:
(1) The MSLT is indicated as part of the evaluation of people with suspected narcolepsy to confirm the diagnosis.
(2) The MSLT may be indicated as part of the evaluation of suspected idiopathic hypersomnia to differentiate it from narcolepsy.
(3) The MSLT is not routinely indicated for the initial evaluation and diagnosis of obstructive sleep apnea syndrome or in the assessment of change following treatment with nasal continuous positive airway pressure.
(4) The MSLT is not routinely indicated for the evaluation of sleepiness in medical and neurological disorders other than narcolepsy, or in insomnia or circadian rhythm disorders.
(5) Repeat MSLT testing may be indicated when the initial test is affected by extraneous circumstances or when appropriate study conditions were not present during initial testing when ambiguous or uninterpretable findings are present, and/or when the patient is suspected to have narcolepsy but earlier MSLT evaluation(s) did not provide polygraphic confirmation.

For evaluation of circadian rhythm disorders is incorrect as a sleep log or diary is used in the evaluation of circadian rhythm disorders.

For evaluation of sleepiness in insomnia is incorrect as sleepiness in insomnia is evaluated using questionnaires such as the Consensus Sleep Diary (CSD) and the Insomnia Severity Index (ISI).

In assessment of change following treatment with nasal continuous positive airway pressure is incorrect because this is accomplished by clinical judgment and possible in-lab CPAP titration study.

In the initial evaluation and diagnosis of obstructive sleep apnea syndrome is incorrect as the initial evaluation and diagnosis of obstructive sleep apnea syndrome are made by in-lab polysomnography or home sleep apnea testing.

CBT has shown efficacy for health anxiety symptoms is correct as cognitive behavioral therapy (CBT) has been proven to be effective over placebo for health anxiety in a variety of patient populations across different clinical settings and is considered the first-line treatment for IAD. The core principle of IAD treatment is care over cure, meaning that treatment focuses on helping the patient deal with the anxieties of their health. Similar to other somatoform spectrum disorders, treatment tries to minimize excessive diagnostic intervention to avoid iatrogenic harm. Treatment focuses on improving the patient’s ability to function at home, work, and social spheres.

No effective treatment exists for IAD because it is a new diagnosis is incorrect as therapy (preferably CBT) is helpful in ameliorating the symptoms of IAD and helping patients regain function.

Psychodynamic therapy is the psychotherapy of choice for IAD is incorrect. Antidepressants can help in decreasing overall anxiety, but they are viewed as a second-line treatment. Multiple randomized control studies (RCTs) compared CBT to a control group, and they each demonstrated superior results in the CBT arm.

CBT (cognitive behavioral therapy) is not the treatment of choice for IAD is incorrect as cognitive behavioral therapy (CBT) has been proven to be effective over placebo for health anxiety in a variety of patient populations across different clinical settings and is considered the first-line treatment for IAD.

Paroxetine by itself is more effective than CBT for IAD is incorrect. A large randomized control study (RCT) from 2007 in the AJB demonstrated slight superiority in CBT over paroxetine. Generally, CBT is viewed as the first-line agent, and paroxetine is viewed as a second-line treatment but can be used to augment CBT.

Advise genetic testing and further workup, explaining politely how she may not have Huntington’s disease and that her stress may be unfounded is correct. There is a 50% chance that the patient has Huntington’s disease and a 50% chance that she does not. Huntington’s disease is an autosomal dominant disorder, so 50% of the children acquire it from their parents. Simple tremors can be explained by anxiety, hyperthyroidism, and caffeine dependence. Mood disturbance and short-term memory issues can be explained by anxiety and depression secondary to the stress of potentially having a debilitating illness. The patient needs further workup to confirm that there is a neurological basis for her symptoms. Consoling her that there is a chance she might not have the disorder and how genetic testing would be helpful is the most professional advice the physician can give. It might alleviate her anxiety and give her clarity to make further decisions.

Advise her to seek psychotherapy to help her reach a decision is incorrect because the patient did not present to a neurologist just to be referred to a psychiatrist or psychologist. The patient requires counseling and insight into her symptoms and how the outcome doesn’t necessarily have to be poor. Advising psychotherapy without any medical help is very unprofessional of the physician.

Encourage her to get the partner involved, start pharmacotherapy, and instill hope as medical treatments have advanced in the last decade is incorrect because the physician should not assume that the patient has Huntington’s disease without confirmation testing. Giving the patient hope that medical treatments have advanced for Huntington’s disease would make her firmly believe that she has the disorder when, in reality, she may not have it. Encouraging her to get her partner involved is also not appropriate. There might be more complex issues to her wanting to keep her diagnosis confidential. The physician should only encourage a referral to someone reliable and supportive, such as a friend or a relative. Starting pharmacotherapy without investigating the disease is premature.

Advise genetic testing and psychotherapy is incorrect because the patient is stressed and requires an explanation instead of a referral for psychotherapy and genetic testing. The physician should consider the patient’s emotional state and try their best to counsel them while presenting all possibilities. Given that there is a 50% possibility that the patient does not have Huntington’s disease, it is the physician’s responsibility to sensitively inform the patient.

Encourage her to bring her partner on the next visit and discuss this issue together is an incorrect choice because the physician should not assume involving the partner would help resolve the patient’s issues. There may be complex interpersonal reasons as to why the patient has not yet involved the partner. The physician should not encourage her to involve the partner unless the patient develops the idea herself. In such sensitive issues, it is always paramount to discuss diagnosis, investigations, and treatment options privately with the patient so there is no pressure from a third party. Then, the patient can make independent decisions.

Assigning him low-risk tasks that have fewer chances of error is correct because low-risk tasks have a lower probability of endangering patients. The principle of fatigue-proofing is that workers should be provided low-risk tasks at the time when fatigue risk is higher (end of night shift) and high-risk tasks when fatigue risk is lower (beginning of shift) to minimize the margin of error and to keep patients safe from errors and accidents due to physician fatigue.

Allowing him 4 hours of uninterrupted sleep immediately is incorrect because it is not feasible to offer uninterrupted sleep on night shifts during residency training. The patients often require urgent consults and assistance and, thus, uninterrupted sleep can never be guaranteed when on call. The principle of optimizing sleep arises under the domain of fatigue reduction, not fatigue proofing.

Offering him coffee and tea is incorrect because stimulating agents, like caffeine, work by reducing fatigue, and not by fatigue-proofing. Caffeine is highly used by physicians during night shifts for alertness and reducing fatigue. The question asks about the principle of fatigue-proofing and not fatigue reduction.

Prescribing amphetamines is incorrect because stimulating agents reduce fatigue and are not directly related to fatigue-proofing. Amphetamines are very rarely prescribed for the sole purpose of fatigue. The main approaches to enhancing performance and reducing fatigue include optimizing sleep and taking naps during shifts.

Assigning the new resident high-risk tasks so that he stays busy is incorrect because the fatigue-proofing principle applies to low-risk tasks. Assigning high-risk tasks would endanger the patients because the resident’s fatigue wouldn’t enable him to perform to the standard required for a high-risk task.

Sickle-cell disease is correct. Studies have demonstrated the importance of modifier genes and gene-gene interactions in determining stroke risk; they have also shown the potential role of genetics in predicting stroke risk. Sickle cell disease is the most common cause of childhood stroke, with the highest incidence occurring before five years of age. By adulthood, the risk of ischemic stroke is surpassed by that of hemorrhagic stroke; as many as 25% of Hb-S homozygous patients will have a stroke by age 45 years. These patients often have large vessel thrombus formation and remodeling, smaller vessel border zones, and subcortical region disease. Stroke recurrence is fairly common, as are multiple silent infarcts evident by imaging.

Ehlers-Danlos syndrome is incorrect. Although vascular Ehlers-Danlos syndrome is associated with a higher risk of juvenile stroke, the pathology is different than that seen in sickle cell disease.

Fabry disease is incorrect. The mechanisms underlying the increased risk of stroke in patients with Fabry disease have not been clearly understood.

Homocystinuria is incorrect. It is a connective tissue disease due to defects in methionine metabolism, leading to an elevated plasma concentration of homocysteine.

Marfan syndrome is incorrect. It is an autosomal dominant connective tissue disorder in the ocular, skeletal, and cardiovascular systems.

Idebenone is correct. The patient presented in this case has Leber hereditary optic neuropathy (LHON), which is a mitochondrial disorder that leads to loss of central vision and optic nerve atrophy. Several mutations in the subunits of complex I of the respiratory chain have been identified in this condition. Approximately 80-90% of cases occur in males who first experience symptoms in their teens and 20s. Patients with LHON typically report reduced color vision, blurred vision, and painless loss of central vision in one eye. Over weeks to months, the impaired vision affects the opposite eye. Dense central or centrocecal scotomas can be identified in these individuals. The central retinal vessels demonstrate vascular tortuosity on fundoscopic examination, while the optic disc shows hyperemia and peripapillary telangiectasias. A short-chain benzoquinone known as idebenone has been identified as a successful therapeutic approach in this population. When given early in the course of the disease for at least one year, idebenone has been shown to improve visual acuity and tritan color contrast (blue-yellow color domain).

Gene therapy is incorrect. The patient in this scenario has Leber heredity optic neuropathy based on the family history, examination findings, and testing. Preliminary work in gene therapy has shown promising results for this mitochondrial disorder, but this intervention is currently deemed investigational.

Stem cell therapy is incorrect. In the Stem Cell Ophthalmology Treatment Study, patients with Leber hereditary optic neuropathy improved visual field and visual acuity upon treatment with autologous bone marrow-derived stem cells. At this time, however, additional investigation is required before this therapy can be routinely recommended.

Infrared light therapy is incorrect. The patient, in this case, has Leber hereditary optic neuropathy (LHON). After administering infrared light therapy, experimental models have shown improved cell survival and mitochondrial function. However, visual anomalies in patients with LHON were not resolved after administering infrared light therapy.

Systemic corticosteroid therapy is incorrect. Although steroids have been administered to patients with Leber hereditary optic neuropathy, they failed to restore visual acuity in this population.

Anti-mycobacterial therapy with corticosteroids is correct. Tuberculous optic neuropathy should be treated with corticosteroids and anti-mycobacterial therapy to reduce inflammation and improve visual prognosis. This patient is most likely suffering from tuberculosis (TB), as evidenced by a history of TB in family members, lymphadenopathy, and the presence of acid-fast bacilli. Tuberculous optic neuropathy is a rare manifestation of TB, which is probably the cause of visual loss in this patient.

Anti-fungal therapy is incorrect. Antifungals have no role in treating TB.

Anti-viral therapy is incorrect. This patient has severe vision loss in her left eye due to tuberculous optic neuropathy and needs urgent anti-mycobacterial therapy. Antivirals have no role in treating TB.

Observation only is incorrect. This patient is most likely suffering from tuberculous optic neuropathy, as evidenced by systemic signs of TB and clear ocular media with optic nerve swelling.

Anti-mycobacterial therapy is incorrect. This patient has severe vision loss, and corticosteroids should be added to reduce the inflammatory reaction.

Symptomatic treatment is correct. Acute vestibular neuronitis usually resolves spontaneously and should be managed conservatively with vestibular suppressants, anti-emetics, and intravenous hydration. Therefore, this patient should be managed medically until his symptoms resolve. This patient probably suffers from acute vestibular neuronitis secondary to an upper respiratory tract infection.

Vestibular neurectomy is incorrect. It is usually reserved for patients with severe symptoms that do not respond to medical management and vestibular rehabilitation. It is also an intradural procedure with an increased risk of complications. Therefore, symptomatic treatment and vestibular rehabilitation should be tried first in this patient.

Labyrinthectomy is incorrect. It is also a surgical procedure for intractable vertigo with failed medical management. This procedure is also associated with a high rate of hearing loss when hearing preservation is required. Therefore, it is not appropriate for this patient.

Antiviral therapy is incorrect. Current evidence has not shown any influence of antiviral therapy on disease outcomes in patients with vestibular neuronitis. Therefore, treating this patient with antiviral agents is not recommended.

Oral corticosteroids is incorrect. Corticosteroids have not shown any significant impact on clinical outcome and disease course. However, few studies have shown the benefit of corticosteroid treatment when initiated within 72 hours of the onset of symptoms. This patient has been experiencing symptoms of vertigo for the last 10 days, therefore, steroid therapy is not appropriate for this patient.

Best supportive care is the correct response because this patient is not a candidate for radiotherapy, chemotherapy, or re-resection. When treating recurrent high-grade gliomas, three things should be considered to decide the best course of action. The first is the patient’s age. A patient with age less than 60 has a chance to benefit from aggressive interventions, given that other parameters are also favorable. The second is performance status. A patient with good performance scores, like KPS > 60 points and ECOG less than 2 points, is likelier to respond well to chemotherapy and re-resection. The third is the nature and site of the tumor. A high-grade glioma has a poor prognosis.

Radiotherapy followed by the best supportive care is incorrect because the role of irradiation in recurrent high-grade gliomas is unclear. Considering the poor functional status of the patient (KPS <60 and ECOG more than 2), the irradiation risk is more than the indeterminate benefit.

Radiotherapy combined with temozolomide (TMZ) chemotherapy is incorrect because even though chemotherapy has an established role in recurrent high-grade gliomas, considering the poor performance status of the patient, associated multiple comorbid diseases and advanced age irradiation, chemotherapy would be more dangerous in this patient with no beneficial yield on overall survival (OS).

Re-resection is incorrect because re-resection of high-grade gliomas is performed when there is an acute neurological emergency, such as hydrocephalus, an impending herniation, or acutely raised intracranial pressure. However, a good performance score is the most significant indicator of beneficial re-resection. Given the low-performance score of this patient and his multiple comorbidities, a re-resection would not be suitable given the poor prognosis despite high-risk surgical resection.

Systemic palliative therapy with bevacizumab is incorrect because studies report a vague benefit of bevacizumab monotherapy in recurrent high-grade gliomas. This patient has ischemic heart disease and chronic kidney disease, and bevacizumab is known to cause cardiovascular adverse effects, thromboembolism, and kidney injury. A trial of a reduced dose can be tried, but given the low-performance scores and advanced age, no benefit can be expected.

Inflammatory polyradiculopathy is correct. The history of subacute to chronic, asymmetrical distal more than proximal sensory loss weakness with reduced deep tendon reflexes should raise the possibility of an inflammatory polyradiculopathy.

Inflammatory myopathy is incorrect. In general, inflammatory myopathies produce symmetrical weakness and are not associated with a loss of muscle stretch reflexes.

Paraneoplastic myasthenic syndrome is incorrect. Myasthenic syndrome also produces symmetrical weakness and is not associated with low back pain.

Spinal cord tumor is incorrect. Unlike in this patient, spinal cord neoplasms frequently affect the pyramidal tract, leading to areflexia and extensor plantar responses.

Spinal stenosis is incorrect. Although spinal stenosis may affect multiple nerve roots, its course is chronic and rarely produces fixed weakness.

Bones is correct. Bone is a target tissue of lead toxicity, as well as a reservoir of systemic lead. Skeletal development and the regulation of skeletal mass are ultimately determined by the four types of cells: osteoblasts, lining cells, osteoclasts, and osteocytes. These cells are responsible for matrix formation, mineralization, and bone resorption under the control of both systemic and local factors. As lead is stored long-term in bones, lead intoxication, directly and indirectly, alters many aspects of bone cell function.

Blood is incorrect. It is not a lead storage organ.

Liver is incorrect. It is not a lead storage organ.

Muscle is incorrect. It is not a lead storage organ.

Pancreas is incorrect. It is not a lead storage organ.

Complete blood count with peripheral smear is the correct option. This patient has developed a vitamin B12 deficiency as a result of her gastrectomy. A complete blood count will confirm the anemia with a raised mean corpuscular volume (MCV); peripheral smear will show macro-ovalocytes, anisocytosis, poikilocytosis, and hypersegmented neutrophils.

Anti-signal recognition particle (SRP) antibody is incorrect because these patients usually present with muscle weakness of the shoulders and hips, along with muscle necrosis.

Upper gastrointestinal endoscopy is incorrect because it will not yield any additional information about the etiology of this patient’s neuropathy.

PET scan is incorrect because this patient doesn’t have a recurrence of her malignancy. Her symptoms are due to a deficiency of vitamin B12.

CA 72-4 level is incorrect because it is a tumor marker used to diagnose gastric cancer. This patient’s symptoms are due to vitamin B12 deficiency.

Amifampridine is the correct option. This patient has Lambert-Eaton myasthenic syndrome. Progressive muscular weakness, hyporeflexia, autonomic features, and mild ptosis all point to the underlying condition. Electrophysiological studies and antibodies to voltage-gated calcium channels can confirm the diagnosis. This patient needs an extensive workup to look for a primary malignancy, especially with his history of dry cough. Usually, treatment of the underlying cause is sufficient to produce substantial clinical improvement. If patients still require therapy, amifampridine is the drug of choice. It is a potassium channel blocker and is the only FDA-approved drug for this condition.

Discontinue atorvastatin is incorrect because this patient does not have statin-induced myopathy. The absence of generalized muscle pain and the presence of autonomic features make statin-induced myopathy unlikely.

Pyridostigmine is incorrect because it is used in the management of myasthenia gravis. Although patients may have bilateral ptosis with proximal myopathy, reflexes are usually present and disappear only on repeated exercise.

Corticosteroids is incorrect because they are used to treat polymyositis and overlap myositis.

Physical therapy is incorrect because, although it will benefit this patient, he needs definitive therapy for his underlying condition.

Postsynaptic tyrosine kinase receptor is the correct option. This patient has myasthenia gravis, as evidenced by her fatigability, as well as her ocular and bulbar symptoms. Muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG) is rare, refractory to conventional treatment, and a more severe disease with frequent myasthenia crisis. MuSK antibodies bind to postsynaptic, muscle-specific tyrosine kinase receptors. These patients are younger, have bulbar involvement, and benefit from steroids and immunosuppressants.

Nicotinic acetylcholine receptors is incorrect because anti-acetylcholine antibodies bind to nicotinic acetylcholine receptors in myasthenia gravis.

Muscarinic 1 acetylcholine receptors is incorrect because the M1 receptor is primarily found in the cerebral cortex, gastric glands, and salivary glands.

Ryanodine receptors is incorrect because they are present in the sarcoplasmic reticulum, and their disorder is associated with malignant hyperthermia. Moreover, antibodies to these receptors are found in about 50% of patients with myasthenia gravis who have a thymoma.

Presynaptic voltage-gated calcium channels is incorrect because this is the binding site of antibodies in Lambert-Eaton myasthenic syndrome. These patients present with muscle fatigue that improves with exercise.

Polymyositis is the correct option because this patient’s muscle soreness and weakness in her shoulder and pelvic girdle muscles without any associated skin findings are evident for a diagnosis of polymyositis (PM). Dysphagia is seen in this patient, which is also a characteristic symptom of PM.

Statin-induced myopathy is incorrect because it typically does not take years to develop. Moreover, the erythrocyte sedimentation rate is usually normal in these patients. In addition, fluvastatin has the lowest risk of myopathy as compared to the other statin drugs. This patient’s presentation is not consistent with a diagnosis of statin-induced myopathy.

Guillan-Barre syndrome (GBS) is incorrect because it typically occurs due to Campylobacter jejuni infection or the Zika virus. GBS is associated with respiratory and gastrointestinal dysfunction and presents as asymmetric, ascending paralysis of muscles with diminished or absent deep tendon reflexes (DTRs). This patient’s findings are inconsistent with GBS.

Dermatomyositis is incorrect because this patient does not present with dermatological features of dermatomyositis, including a shawl-like sign or a heliotrope rash, suggesting another diagnosis.

Myasthenia gravis (MG) is incorrect because patients with MG typically have muscle weakness with use and improves with rest. Moreover, diplopia or drooping eyelids are a typical MG presentation, which is not present in this patient. In addition, creatine kinase levels are usually normal in such patients.

Cefepime, vancomycin, and ampicillin is the correct management approach in this patient. The patient is suspected of having acute bacterial meningitis. The results of blood cultures and lumbar punctures are still not available, so it is best to treat the patient with empiric antibiotics until the results of the tests are available. After the results are available, the treatment regimen can be tailored according to the organism detected and its susceptibility.

Ceftriaxone alone is not the correct option, as the patient’s blood cultures and lumbar puncture reports are still pending. In such cases, empiric antibiotics should be given to the patient. Ceftriaxone would have been the treatment of choice if the patient had meningitis due to Neisseria meningitidis or Hemophilus influenzae.

Vancomycin is not the correct treatment approach in this patient, as the patient has clinical manifestations of acute bacterial meningitis, which would need empiric antibiotics. Vancomycin would only cover cephalosporin-resistant pneumococci.

Cefepime and ampicillin is an incorrect management option because the patient is suspected of having acute bacterial meningitis, which would require empiric antibiotics until test results are available. Cefepime and ampicillin should be given along with vancomycin, as cefepime would cover most of the organism responsible for bacterial meningitis, and ampicillin would cover listeria; however, vancomycin should also be added to the regimen to cover cephalosporin-resistant pneumococci.

Ceftazidime and vancomycin would be an incorrect antibiotic regimen in this patient, as ampicillin would also be required as empiric therapy to cover listeria. Ceftazidime and vancomycin cover gram-negative rods, coagulase-negative staphylococci, and methicillin-resistant staphylococcal aureus. They are indicated for patients who have had penetrating trauma, shunt, or neurosurgery.

Median nerve compression is correct. Compression in the carpal tunnel is the most common and early presenting feature of rheumatoid arthritis. These entrapment neuropathies occur due to hypertrophied synovium or due to joint subluxation.

Ulnar nerve compression is incorrect. Compression at the elbow or wrist is a peripheral entrapment neuropathy associated with rheumatoid arthritis.

Lateral popliteal nerve compression is incorrect. This compression is a peripheral entrapment neuropathy that is associated with rheumatoid arthritis.

Mononeuritis multiplex is incorrect. Mononeuritis multiplex occurs in patients with rheumatoid vasculitis.

Diffuse symmetrical peripheral neuropathy is incorrect. It occurs in patients with rheumatoid vasculitis and is less common.

Penicillamine is correct. The scenario describes a patient with Wilson’s disease. Treatment of this condition aims to arrest the accumulation of copper in the tissues and to remove excess copper. Penicillamine is a copper chelating agent used to remove excess copper, which is deposited at various sites, including the brain and liver.

Zinc salts is incorrect. Zinc can decrease copper excretion as maintenance therapy in patients whose symptoms have regressed following chelation therapy. However, the first-line therapy for this patient with Wilson’s disease is Penicillamine.

Tetrathiomolybdate is incorrect. It can be used as a copper chelating agent, but its safety and use are still under clinical trial.

Liver transplantation is incorrect. It is indicated when there is liver failure secondary to Wilson’s disease. The patient in the scenario mentioned above has neurological symptoms only. He does not have any non-neurological manifestations of Wilson’s disease; therefore, liver transplantation would not be a suitable treatment at the moment.

Dietary restriction of copper is incorrect. It is insufficient as a sole treatment for Wilson’s disease.

Rivastigmine, a medication more commonly used to treat Alzheimer’s disease, is approved by the FDA for the treatment of pervasive developmental disorders (PDD).

Quetiapine is incorrect. Atypical antipsychotics with minimal effects on dopamine receptors, such as quetiapine and clozapine, are sometimes used off-label for the treatment of psychotic symptoms associated with pervasive developmental disorders (PDD), but are not mainstays of it’s treatment.

Clozapine is incorrect. Atypical antipsychotics with minimal effects on dopamine receptors, such as quetiapine and clozapine, are sometimes used off-label for the treatment of psychotic symptoms associated with pervasive developmental disorders (PDD), but are not mainstays of it’s treatment.

Haloperidol is contraindicated in patients with Parkinson’s disease.

Amantadine is effective for the treatment of dyskinesias associated with Parkinson’s disease, it is not effective for the treatment of pervasive developmental disorders (PDD).

Pramipexole is correct because bradykinesia and an unilateral resting tremor (“pill-rolling tremor”), which worsens with stress and improves with voluntary movements, are consistent with Parkinson’s disease. A non-ergot dopamine agonist, such as pramipexole or ropinirole, is the typical first-line treatment for Parkinson’s disease in young patients (< 65 years) without cognitive impairment.

Clonazepam is incorrect because it can be used to treat orthostatic tremor. The average age of onset of orthostatic tremor is 60 years of age, and this patient is 55. However, the typical clinical features of orthostatic tremor are triggered by longer periods of standing; they include tremor of the legs and a subjective sensation of instability. As this condition only affects the legs, it cannot be the cause of this patient’s hand tremor.

Methimazole is incorrect because it is used in the treatment of hyperthyroidism, which can manifest with a tremor that increases with stress. However, the tremor is usually symmetric and occurs while holding positions against gravity (e.g., extending the arms in front of the body).

Propranolol is incorrect because it is a drug of choice in the treatment of benign essential tremor. Essential tremor can be exacerbated by stress, as reported by this patient. However, unlike the asymmetric resting tremor seen here, essential tremor is symmetrical, worsens with voluntary movements, and resolves when the patient is completely at rest.

Trihexyphenidyl is incorrect because it is an antimuscarinic drug that can be used to treat tremors and rigidity in patients with Parkinson’s disease. However, this type of drug would not improve bradykinesia, which is seen in this patient.

Dementia with Lewy bodies is the correct option, as it presents with the development of dementia before or within one year of developing spontaneous parkinsonism. It is characterized by fluctuating cognition (attention deficits, short-term memory loss, and decreased visuospatial skill) and visual hallucinations that impair daily functioning. In addition, alertness and arousal are also affected.

Multiple system atrophy is not the correct option, as it presents with early onset of autonomic dysfunction, like erectile dysfunction or urinary dysfunction, which is not indicated in the patient’s presentation. Although parkinsonian features like slow movement and muscle rigidity can develop, classic parkinsonian symptoms, like pill-rolling tremors, are a rare presentation. Additionally, personality changes and mood disorders can also be found. Respiratory complaints, like nocturnal stridor and sleep apnea, are also common. Memory loss and attention deficits are not found in multiple system atrophy.

Idiopathic Parkinson’s disease is not the correct option, as it does not present with cognitive function deficits. Parkinson’s presents with a tetrad of hypokinesia/bradykinesia, resting tremor, postural instability, and rigidity. This tetrad presents as an expressionless face, slowed voluntary movements, stooped posture, shuffling gait, and axillary instability.

Cortical-basal ganglionic degeneration is not the correct option, as it does not present with hallucinations and pill-rolling tremors. It is characterized by progressive asymmetrical extrapyramidal rigidity, combined with signs of cortico-spinal disease. This presents as asymmetrical clumsiness, rigidity, tremors, and an inability to direct voluntary movements. Attempts to move their limbs can result in inappropriate action. Dementia, and impaired eyelid and oculomotor movements can also be found.

Progressive supranuclear palsy is not the correct option, as it presents in the sixth decade and is not associated with hallucinations and pill-rolling tremors. It presents with balance difficulties and abrupt falls that can be like parkinsonism. However, difficulty with voluntary, vertical movements of the eye and impairment of voluntary saccades are characteristic of this disease.

Medication side-effect is correct because this patient’s presentation is most consistent with drug-induced Parkinson’s disease (PD). Patients taking anti-dopaminergic drugs (i.e., risperidone) can experience a number of extrapyramidal side-effects, such as secondary PD, dystonias, akathisia, or tardive dyskinesia. Discontinuation of the causative medication usually reverses the symptoms.

Loss of dopaminergic neurons in the substantia nigra is incorrect because this patient demonstrates all of the classic motor findings of Parkinson?s disease. The temporality and rapid progression of her symptoms after starting risperidone, however, suggests her condition is drug-induced rather than primary/idiopathic.

Major depression is incorrect because patients with Parkinson’s disease often experience a comorbid psychiatric illness–depression being the most common. While this patient does have a known history of psychiatric illness, this does not explain her tremor or shuffling gait.

Paraneoplastic syndrome from malignancy is incorrect because, although rare, several malignancies can present with neurological-dominant paraneoplastic syndrome, particularly those malignancies associated with Hu, Yo, or Ri antibodies. Although this patient has a family history of small cell lung cancer, her new use of an anti-dopaminergic agent, lack of smoking, and presence of bradykinesia, shuffling gait, and tremors are more suggestive of secondary Parkinson’s disease.

Excessive alcohol use is incorrect because delirium tremens is a complication of alcohol withdrawal, usually presenting within three days of alcohol abstinence in a heavy drinker. While this patient does demonstrate tremor, her lack of alcohol use, shuffling gait, bradykinesia, and recent initiation of risperidone make drug-induced PD more likely.

Echocardiogram and chest X-ray is the correct option. The tests must be performed before cabergoline is initiated. Ergot-derived dopamine agonists, such as cabergoline, can often cause retroperitoneal, pulmonary, and cardiac fibrosis. For this reason, a baseline serum creatinine, chest X-ray, and echocardiogram should be obtained before treatment is initiated.

Liver function tests and chest X-ray is incorrect as cabergoline is not known to cause a sufficient rise in liver transaminases. Cabergoline does, however, cause pulmonary fibrosis, and hence, a chest X-ray is warranted.

Liver function tests and creatinine is incorrect as cabergoline is not known to cause a sufficient rise in liver transaminases. Cabergoline does, however, cause retroperitoneal fibrosis, leading to uropathy for which baseline creatinine levels should be obtained.

Liver function tests and echocardiogram is incorrect as cabergoline is not known to cause a sufficient rise in liver transaminases. Cardiac fibrosis and valvular damage are known complications of cabergoline, so an echocardiogram should be obtained before initiation of therapy.

Creatinine and thyroid function tests is incorrect because although therapy with cabergoline can lead to uropathy secondary to retroperitoneal fibrosis, this drug is not known to significantly affect thyroid hormone levels.

Pramipexole is correct. Since this 45-year-old patient presents with bradykinesia and a unilateral resting tremor that improves with movement, a diagnosis of young-onset Parkinson’s disease is most likely. The average age at which individuals are diagnosed with Parkinson’s disease is 60; when a person younger than 50 presents with this disease, he or she is considered to have young-onset Parkinson’s. A non-ergot dopamine agonist, such as pramipexole, is a treatment option for this situation. Pramipexole appears to be effective as monotherapy in the early stages of Parkinson’s disease and may be used as an adjunct as the disease progresses. Since motor complications can arise with long-term use of levodopa, pramipexole is a good choice. Not only can this medication improve resting tremor, rigidity, and akinesia, but it also provides a beneficial effect on non-motor symptoms, such as depression.

Clonazepam is incorrect. This particular benzodiazepine is used in the management of orthostatic tremor, a disorder characterized by a rapid (13-18 Hz) tremor in the lower extremities that is triggered upon standing. Patients may report a sense of imbalance or unsteadiness. The tremor may be reduced or eliminated when the individual is walking or sitting.

Methimazole is incorrect. This particular thionamide is indicated in the management of hyperthyroidism. The patient in this case demonstrates an asymmetric tremor. Hyperthyroidism is associated with a symmetric tremor that is most prominent when holding a position against gravity, such as holding one’s hands outstretched in front of the body. The high-frequency and low-amplitude tremor of hyperthyroidism resembles an enhanced physiologic tremor. Proper treatment of hyperthyroidism with an agent, such as methimazole, tends to alleviates the tremor.

Propranolol is incorrect. This medication is indicated for essential tremor, which is an involuntary oscillation movement disorder that tends to be mostly symmetric, rhythmic, and progressive. Essential tremor is typically observed during postural and intentional movements and is usually absent at rest.

Trihexyphenidyl is incorrect. This centrally-acting, anticholinergic agent is used in the management of Parkinson’s disease to address tremors and rigidity. However, this agent has no effect on bradykinesia and is, therefore, not the best choice for the patient presented in this case.

Octreotide is the correct option and an appropriate add-on therapy in hypoglycemia caused by sulfonylureas. In this condition, administration of dextrose may stimulate the pancreatic B-cells to secrete insulin, resulting in persistent hypoglycemia. Administration of octreotide can block the secretion of insulin by inhibiting the B-cells.

0.9% saline bolus is an incorrect option as this would be an appropriate therapy for hyperglycemic states such as diabetic ketoacidosis and hyperosmolar nonketotic hyperglycemia. It is not, however, an approved therapy for hypoglycemia.

Potassium is an incorrect option as this is an appropriate adjunctive therapy for hyperglycemic states where the potassium level falls below 5 mmol/L.

Bicarbonate is an incorrect option as this is an appropriate adjunctive therapy for hyperglycemic states where the PH falls to 6.9.

Glucagon is an incorrect option because, although this drug is efficient at increasing blood glucose by increasing the production and release of glucose from hepatocytes, it does not effectively blunt the concurrent insulin release from the pancreas. Glucagon will not be the first-line therapy for sulfonylurea-induced hypoglycemia

Parsonage-turner syndrome (neuralgic amyotrophy) is correct. Neuralgic amyotrophy (Parsonage-Turner syndrome) is characterized by acute, severe pain in the shoulder and radiating into the arm, neck, and back. To prevent pain, movement of the arm is avoided, and the arm is held in a position of flexion at the elbow and adduction at the shoulder (the flexion-adduction sign). The pain is followed within several hours to days by paresis of the shoulder and predominantly proximal arm musculature. Sensory loss can occur but is generally not marked. The muscles innervated by the axillary, suprascapular, radial, musculocutaneous, and long thoracic nerves are most commonly affected. Unilateral or bilateral phrenic nerve paralysis may occur. The pain usually resolves within several days. The process is thought to be a brachial plexitis or multiple mononeuritis and is usually idiopathic but may follow a viral illness, immunizations, surgery, or childbirth.

Acute cervical disc protrusion is incorrect. While acute cervical disc protrusion could cause a similar clinical presentation (although the rapid and permanent onset of weakness would be very atypical) a radiographic abnormality would be expected on the cervical spine MRI.

Amyotrophic lateral sclerosis (ALS) is incorrect. It is not associated with significant pain, and symptoms do not improve.

Carcinomatous meningitis is incorrect. It does not present with such localized symptoms.

Metastatic disease to the brachial plexus is incorrect. Symptoms of metastatic disease do not improve.

100% oxygen supplementation via a face mask is the correct answer because the above case describes a patient with the most likely diagnosis of cluster headache. To abort an acute attack, 100% oxygen inhalation via a face mask for 10 to 15 minutes has been found to be helpful.

CT of the brain is incorrect because the patient is experiencing cluster headache in which there is no indication for emergency imaging. The age, clinical presentation, and examination findings do not suggest any acute neurological emergency; therefore, a CT is not needed.

Intravenous methylprednisone is incorrect because steroids are used for the prevention of cluster headaches rather than for aborting acute episodes, especially in patients with chronic cluster headaches not responding to ergotamine and sumatriptan.

Oral verapamil is incorrect because verapamil is recommended to terminate clusters of ongoing headaches rather than use in terminating acute attacks.

Oral amitriptyline is incorrect because amitriptyline is used for the treatment of tension-type headaches associated with depression.

Diffuse high amplitude nonsynchronous paroxysms of theta and delta activity with superimposed multifocal sharp waves is correct. The characteristics of this seizure are consistent with infantile spasms. The characteristic interictal EEG finding is hypsarrhythmia, characterized by a high amplitude, nonsynchronous, disorganized pattern.

3 Hertz spike and wave pattern is incorrect. An EEG finding of 3 Hz spike and wave pattern is characteristic of absence seizures.

Burst suppression pattern is incorrect. Burst suppression is characterized by periods of high-voltage electrical activity alternating with periods of no activity. The pattern is found in patients with general anesthesia, coma, or hypothermia.

Diffuse generalized slowing is incorrect. It is a nonspecific finding that can be seen in encephalopathies of various kinds.

Periodic high amplitude repetitive discharges in bilateral temporal hemispheres is incorrect. Also known as lateralized periodic discharges (LPDs), they are an epileptogenic pattern most commonly associated with herpes simplex virus encephalitis.

Zellweger syndrome is the correct option. It is a rare autosomal recessive disorder characterized by the absence of peroxisomes. Mutations in PEX genes cause this syndrome. Patients have characteristic facies, stridor, poor feeding, hypotonia, and mental impairment. There is no cure, and the prognosis is dismal.

McArdle disease is incorrect because it is caused by myophosphorylase deficiency. Patients get severe cramps during exercise and get a “second wind” after resting.

Alexander’s disease is incorrect. It is caused by glial fibrillary acidic protein (GFAP) mutation. It is an autosomal recessive disorder. that is rare and progressive. It is characterized by developmental delay, failure to thrive, psychomotor impairment, and seizures. Physical examination reveals macrocephaly, spasticity, ataxia, bulbar, and pseudobulbar impairment.

Chediak-Higashi syndrome is an incorrect option. It is a rare disorder characterized by the absence of melanin resulting in oculocutaneous albinism. These patients have an impaired immune system and blood and coagulation abnormalities.

DiGeorge syndrome is incorrect. Although these patients have hypertelorism, they also have hypercalcemia, low-set ears, and a groove in their upper lips.

KCNQ2 is correct. Benign familial neonatal convulsions (BFNC) is an epileptic syndrome of neonates that is associated with mutations in the voltage-gated potassium channels (KCNQ2 and KCNQ3). Family history is positive, with an autosomal dominant mode of inheritance. Seizures may be generalized or focal and present between 3 days and 6 months. Neurological examination is unremarkable, and no abnormalities are noted on interictal EEG. This syndrome has a benign course, with most patients becoming seizure-free by 6 months of age. No neurocognitive deficits are seen later in life.

SCN1A is incorrect. Mutations of SCN1A gene are associated with several epilepsy disorders, including generalized epilepsy with febrile seizures plus (GEFS+) and severe myoclonic epilepsy of infancy (SMEI).

CHNRA4 is incorrect. Mutations of the CHNRA4 gene are associated with autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE). ADNFLE occurs in young adults and adolescents.

LG11 is incorrect. Mutations of the LG11 gene are associated with autosomal dominant epilepsy with auditory features.

EFHC1 is incorrect. Mutations in the EFHC1 gene area are associated with juvenile myoclonic epilepsy type 1 (JME1).

Alpers-Huttenlocher syndrome is correct. It is a rare type of hepatocerebral mitochondrial-DNA depletion syndrome (MDS). It classically presents with episodic psychomotor retardation, intractable seizures, cortical blindness, and hepatic dysfunction. The most specific indicator of this diagnosis is a valproate-induced fulminant hepatic failure. Valproate is strictly contraindicated in hepatocerebral MDS.

Kearns-Sayre syndrome is incorrect because it presents with typical eye impairments (ptosis, external ophthalmoplegia, and pigmentary retinopathy). Neurological symptoms include cerebellar ataxia, intellectual disability, and episodic coma. Seizures and hepatic derangements are not seen in Kearns-Sayre syndrome. However, hypoparathyroidism, a common endocrine abnormality, can precipitate seizures. None of these abnormalities are described in the vignette.

Valproate-induced liver failure is incorrect because the patient had an ongoing neurological disorder, with subsequent hepatic dysfunction, long before valproate administration. Although valproate precipitated fulminant hepatic failure in this patient, the primary diagnosis is not merely valproate-induced liver failure. It encompasses a complex constellation of hepatocerebral disorders.

Myoclonic epilepsy and ragged-red fibers (MERRF) is incorrect because it presents with myoclonus, generalized tonic-clonic seizures, hearing loss, and cerebellar impairment. The patient has hepatic derangement with valproate-induced fulminant failure, a phenomenon that doesn’t occur in MERRF.

Wilson’s disease is incorrect because the provided 24-hour urinary copper and serum ceruloplasmin levels are within normal ranges. Wilson’s disease presents with neuropsychiatric and hepatic impairments, but cortical blindness is very unlikely in Wilson’s. The prevalence of intractable seizures in Wilson’s disease is also very low.

Prevents additional seizures while the patient is febrile is correct. Seizures occurring during fever in children younger than 18 months or children with a family history of febrile seizures are associated with an increased risk for recurrence, with 90% of recurrences occurring within 2 years of the initial episode. When the benign febrile seizure lasts for more than 15 minutes, diazepam is given to control the acute episode and to prevent the recurrence of febrile seizures.

Prevents subsequent afebrile seizures is incorrect. To prevent subsequent afebrile seizures, which are rarely observed as a sequel of febrile seizures, long-term anti-epileptic therapy is given. Diazepam has no role in the prevention of afebrile seizures.

Limits long-term use of anti-epileptics is incorrect. Anti-epileptics like phenobarbital and valproic acid are used to treat any subsequent episode of afebrile seizures (if any). As diazepam cannot prevent such seizures, it also doesn?t have any role in the limitation of long-term use of anti-epileptics.

Prevents permanent neurological damage is incorrect. Permanent neurological damage is not associated with the scenario mentioned above. If there is any reason for neurological impairment, management with benzodiazepines will have no role in prevention or treatment.

Reduces morbidity and hospital stay is incorrect. Benzodiazepines as an initial treatment would not directly reduce morbidity and hospital stay as there must be some underlying cause for the high-grade fever, which will decide the prognosis and needs to be addressed urgently.

Absence seizures is correct because they are characterized by brief periods of unresponsiveness and unawareness that may be associated with eyelid fluttering, subtle head nods, or oral automatisms. Onset and termination of attacks are abrupt, and they may occur several times a day. Childhood absence seizures have a peak age of onset of 6 years. Classic cases demonstrate 3-Hz generalized spike-and-wave complexes on electroencephalogram (EEG). Ethosuximide is the first-line treatment, but valproate is an alternative.

Atypical absence seizures is incorrect, because they are characterized by a more gradual onset and termination, more pronounced changes in tone, and a longer recovery compared to typical absence seizures. Patients may be able to continue with their activity during this type of seizure; however, they may make mistakes or work more slowly while the episode is ongoing. Subtle myoclonic jerks or a gradual slump (due to loss of muscle tone in the limbs, head, or trunk) may also occur in this disorder. Atypical absence seizures may occur with Doose syndrome and Lennox-Gastaut syndrome.

Postictal phenomena is incorrect because this refers to behavior occurring at the termination of a seizure. Examples include headache, mania, psychosis, spitting, hypersalivation, coughing, nose-wiping, and motor weakness (Todd’s paresis). Recovery from these symptoms is variable, lasting hours to days.

Epileptic drop attacks is incorrect because these events are characterized by a sudden loss of muscle tone that may be limited to only the head or involve the entire body. These atonic seizures may be focal or generalized. They are typically very brief, lasting less than 15 seconds.

Generalized tonic seizures is incorrect. These seizures are characterized by stiffening of all limbs, which is not the primary feature observed in this patient.

Pregabalin is the drug of choice, as it will help manage the patient’s neuropathic pain and her seizures. Neuropathic pain arises directly from a lesion or disease that affects the somatosensory system, such as herpes zoster, which affects the nerve roots.

Amitriptyline is one of the most commonly prescribed drugs for neuropathic pain. It is a tricyclic antidepressant that has been used to treat post-herpetic neuralgia and painful diabetic neuropathy. However, it will not help control the patient’s seizures.

Valproic acid has not been shown to be effective in bringing relief for neuropathic pain.

Lamotrigine has not been shown to be effective in bringing relief for neuropathic pain.

Oxycodone will not be useful for the pain or for the seizures.

Self-limited epilepsy with centrotemporal spikes (SeLECTS), previously known as benign rolandic epilepsy, is correct. This autosomal dominant disorder has a variable penetrance and peak age of onset between 5 and 8 years old. It primarily occurs at night, usually within 2 hours of sleep onset, but it may also present during a daytime nap. SeLECTS is characterized by focal motor seizures in the face, arm, and leg. Salivation and speech arrest are common. Secondary generalization may occasionally occur. Children with this condition develop normally and have no focal findings on neurologic examination. The electroencephalogram (EEG) demonstrates unilateral or bilateral independent centrotemporal spikes. Given its benign course, children may not necessarily need pharmacologic intervention; carbamazepine is an option if treatment is warranted. The prognosis is quite good with remission expected in the early teen years.

Juvenile myoclonic epilepsy is incorrect. This disorder is characterized by myoclonus, generalized tonic-clonic seizures (GTCS), and absence seizures. Seizures characteristically occur upon awakening or in association with sleep deprivation. Photosensitivity is common.

Childhood absence epilepsy is incorrect. Absence seizures are characterized by brief periods of unresponsiveness and unawareness that may be associated with eyelid fluttering, subtle head nods, or oral automatisms. Onset and termination of attacks are abrupt. These seizures may occur several times a day. Childhood absence seizures have a peak age of onset of 6 years. Classic cases demonstrate 3-Hz generalized spike-and-wave complexes on EEG. Ethosuximide is the first-line treatment, but valproate is an alternative.

West syndrome is incorrect. This disorder is characterized by a triad of infantile spasms, (interictal) hypsarrhythmia on EEG, and mental retardation. Developmental decline is present. Onset occurs between 3 months to 2 years with a peak between 3 and 7 months. Steroids or vigabatrin have been used to treat this disorder.

Lennox-Gastaut syndrome is incorrect. This disorder has a childhood-onset between the ages of 1 and 8 years old (with a peak incidence between 3 and 5 years old). It is characterized by a variety of intractable seizures, including myoclonic, atypical absence, tonic, generalized tonic-clonic, and atonic. EEG may show 2-2.5 Hz slow-wave spike complexes or Gibbs patterns (paroxysmal fast activity 10-20 HZ) with a slow background. Topiramate and valproate are possible pharmacologic options. Carbamazepine should be avoided, as it tends to increase the number of drop attacks in these individuals. Vagal nerve stimulation is an option in refractory cases. To reduce the frequency of drop attacks, corpus callosotomy may be a consideration.

Valproate is correct. Valproic acid is the most effective medication for controlling myoclonic jerks. Valproic acid is avoided in women of childbearing age but would be adequate treatment for this patient.

Lamotrigine is incorrect. It can be used in the treatment of juvenile myoclonic epilepsy (JME), but is not well studied and is not considered a first-line treatments.

Carbamazepine is ineffective, and may even worsen the symptoms in patients with juvenile myoclonic epilepsy (JME).

Levetiracetam is incorrect. It can be used in the treatment of juvenile myoclonic epilepsy (JME), but is not well studied and is not considered a first-line treatments.

Topiramate is incorrect. It can be used in the treatment of juvenile myoclonic epilepsy (JME), but is not well studied and is not considered a first-line treatments.

Proceed to surgery for epilepsy is the correct answer because the patient here has the most likely diagnosis of mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis that is drug-resistant. Epilepsy surgery has an excellent response rate in MTLE after temporal lobectomy or selective amygdalohippocampectomy. Post-surgery, 60% to 80% of the patients are seizure-free.

Increase the dose of levetiracetam is incorrect because the patient is already maximized on levetiracetam.

Offer a trial of carbamazepine is incorrect because the control in seizure activity decreases if the first-line and second-line drugs fail. Therefore, a trial of carbamazepine will not be beneficial for this patient.

Offer a trial of lacosamide is incorrect because the patient has already failed to respond to the first- and second-line antiepileptic drugs. It is unlikely that the patient is going to respond to a third-line drug.

Repetitive transcranial magnetic stimulation (rTMS) is incorrect because it is predominantly used as a palliative procedure in patients with drug-resistant epilepsy who are not candidates for surgical therapy.

MRI and magnetic resonance venogram (MRV) is correct. While the patient has a history of catamenial migraines, the characteristics of this new headache are consistent with a headache due to increased intracranial pressure. While she is at risk for pseudotumor cerebri, her use of oral contraceptives and concurrent smoking puts her at risk for a hypercoagulable state and venous sinus thrombosis. The diagnostic studies of choice would be an MRI and MRV. Venous sinus thromboses can lead to ischemic and hemorrhagic strokes.

Head CT is incorrect. A head CT is inadequate to assess for venous sinus thrombosis.

Lumbar puncture is incorrect. It is contraindicated in patients with elevated intracranial pressure due to the risk of herniation.

MRI and magnetic resonance arteriogram is incorrect. An MRA would not show a venous sinus thrombosis.

Send her home with a prescription for a triptan and reassurance is incorrect. Sending the patient home is not advised, given the risks of potential stroke with venous sinus thrombosis.

Obtain an MRI with gadolinium contrast is the correct answer because a new relapse in the patient is suspected. However, other etiologies must also be excluded. If the MRI confirms the presence of one or more gadolinium-enhancing symptomatic lesions, treatment options may be reviewed with the patient.

Plasma exchange is incorrect because the diagnosis requires confirmation first. If a relapse is confirmed, plasma exchange is generally considered second-line therapy.

Natalizumab is incorrect, because this is a treatment option for aggressive multiple sclerosis. This particular diagnosis has not yet been established in the patient described here.

Interferon β-1a is incorrect as the diagnosis must first be established. Interferon β-1a is a disease-modifying agent used to decrease the chances of relapse and disease progression in patients with relapsing-remitting multiple sclerosis. This treatment option can be recommended after the MRI is obtained if it shows active or new extending lesions when compared to the previous MRI.

Arrange follow up in 6 months is incorrect as the patient is currently symptomatic and needs a diagnostic workup followed by a discussion of treatment options. The patient should then be advised that regular follow-up is strongly recommended.

Intravenous methylprednisolone, 1 g daily for 5 days, and plasmapheresis is correct as this is a case of neuromyelitis optica, which is a relapsing-remitting condition. It is characterized by optic neuritis and acute myelitis, associated with MRI changes involving 3 or more segments of the spinal cord. This condition is associated with a specific antibody, NMO-IgG, which acts on the aquaporin-4 water channel.

Oral prednisolone, 1 mg/kg/day for 1 week is incorrect as the initial management option because, for the first 5 days, the patient should be maintained on intravenous steroids. This regimen is then followed by oral prednisone at the aforementioned dose.

Urgent plasmapheresis is incorrect; it is only used when the response to intravenous and oral steroids is poor.

Intravenous immunoglobulins is incorrect because it is generally unhelpful and is not recommended.

Azathioprine, 2 mg/kg orally for 10 days is incorrect because it is part of long-term immunosuppressive therapy and is not indicated in an acute attack. Moreover, it is given for an indefinite period, as treatment suspension or discontinuation usually results in relapse.

Intravenous immunoglobulin is correct as this is a case of multifocal motor neuropathy that is characterized by progressive, asymmetric weakness due to demyelination of the motor nerves. This causes wasting, cramps, and fasciculations of the muscles. It preferentially involves the arms and hands. No sensory impairment or upper motor neuron involvement is present. Electrophysiological studies show evidence of multifocal motor demyelination, with partial motor conduction block, and normal sensory nerves. Human immune globulin is given intravenously, 2g/kg over 3 to 5 days, every 4 to 6 weeks.

Plasmapheresis is incorrect because it is proven to be ineffective in this condition.

Plasma exchange is incorrect, as it is not recommended in the treatment of this condition.

Corticosteroids is incorrect because it is proven to be ineffective in this condition.

Cyclophosphamide is incorrect because it is only utilized when a trial with intravenous immunoglobulin fails.

Multiple sclerosis (MS) is correct. MS is a demyelinating disease characterized primarily by relapsing and remitting neurological signs and symptoms involving various regions of the white matter. It manifests with a wide range of sensory and motor deficits including fatigue, weakness and spasticity, visual, cerebellar, and bladder dysfunction. Advanced disease may even involve the autonomic system causing constipation, impotence or cerebral dysfunction leading to memory impairment and personality changes. MRI brain shows classic plaques in multiple areas of the white matter.

Amyotrophic lateral sclerosis is incorrect as it involves both the upper and lower motor neurons and is characterized by progressive muscle weakness, which is absent in this case. Bowel movements, bladder control, sensation, cognitive function, and extraocular muscles are unaffected even in advanced disease.

Guillain barre syndrome is incorrect; it is an inflammatory demyelinating polyneuropathy causing progressive ascending paralysis, autonomic dysfunction, and areflexia. It is usually preceded by a viral infection.

Neuromyelitis optica is incorrect; it is a relapsing disorder of the spinal tract causing optic neuritis and acute myelitis and usually affects at least 3 segments of the spinal cord. MRI brain does not typically show white matter involvement.

Acute disseminated encephalomyelitis is incorrect; it is characterized by headache, fever, and signs of meningeal irritation along with multifocal neurological deficits including flaccid weakness and sensory disturbances in the leg, extensor plantar responses, and urinary retention. It may also affect consciousness to various degrees. However, if the condition relapses, multiple sclerosis must be ruled out.

Haloperidol is correct. It is a neuroleptic medication approved by the FDA for the treatment of Tourette’s syndrome in adults. It is usually indicated in patients with severe symptoms. This patient could be treated with haloperidol as his daily living is seriously affected by motor and verbal tics.

Pramipexole is incorrect. Current evidence has not shown any beneficial effect of pramipexole for reducing motor or vocal tics. It is, therefore, not appropriate for this patient.

Metoclopramide is incorrect. It is a neuroleptic medication commonly used as an anti-emetic. It is currently not recommended for the treatment of motor or verbal tics.

Omega-3 fatty acids (O3FAs) is not correct. They are not associated with reduced frequency or severity of motor or verbal tics. It is, therefore, not a standard treatment for patients with Tourette’s syndrome (TS).

N-Acetylcysteine (NAC) is incorrect. It can be used as an add-on treatment for patients with TS. NAC is never used as a monotherapy for treating patients with motor or verbal tics. Therefore, this patient needs treatment with another agent before using NAC as an adjuvant.

Memantine is correct. Current evidence has shown a small clinical benefit with memantine in improving cognitive and functional symptoms of patients with mild to moderate Alzheimer’s disease.

Simvastatin is incorrect. Statins have been extensively investigated for the treatment of Alzheimer’s disease. However, current evidence has not shown any potential benefit of statins for improving cognitive dysfunction in patients with mild to moderate dementia.

Aspirin is incorrect. Recent randomized controlled trials have not demonstrated any beneficial effect of non-steroidal anti-inflammatory drugs, such as aspirin and diclofenac, in curbing amyloid-induced neuronal inflammation. Therefore, aspirin does not improve or arrest the cognitive decline in Alzheimer’s disease.

Ginkgo supplements is incorrect. Recent evidence has not reported any positive effect of ginkgo supplementation for preventing or treating cognitive decline.

Omega-3 fatty acids is incorrect. Recent observational studies have not demonstrated any additional benefit of dietary intake of omega-3 fatty acid supplementation in patients with mild to moderate dementia in Alzheimer’s disease.

The correct answer is sensory loss in the distal extremities. In severe cases, the hands may be involved. In addition to distal atrophy and weakness, deep tendon reflexes usually are decreased or absent. Stasis dermatitis, glossiness, and thinness of the skin of the lower legs are common findings. Hyperesthesia and hyperalgesia may be seen along with hyperpathia. Excessive sweating of the soles and dorsal aspects of the feet, palms, and fingers is a common manifestation of alcoholic neuropathy. It indicates the involvement of the peripheral (postganglionic) sympathetic nerve fibers. Charcot arthropathy, also known as neuroarthropathy, is most commonly associated with diabetes mellitus despite various etiologies. It has also been associated with chronic alcoholism in nondiabetic individuals. .

Scleroderma is incorrect. It is an autoimmune rheumatic disease that affects connective tissue.

Positive Babinksi is incorrect. There is no evidence that a positive Babinski’s sign is associated with alcoholic neuropathy

Circumduction gait is incorrect. It is seen most in stroke patients with plegia of one leg.

Hyperactive deep tendon reflexes is incorrect. Depressed deep tendon reflexes, rather than hyperactive, are seen in alcohol-related neuropathy.

Cyclothymic disorder is correct. It requires a duration of symptoms for at least 2 years, the presence of numerous periods of both hypomanic symptoms and depressive symptoms not meeting the criteria for major depressive disorder, with symptom-free periods no longer than 2 months.

Bipolar II disorder with rapid cycling is incorrect. It requires major depressive and hypomanic episodes.

Bipolar I disorder with rapid cycling is incorrect. Bipolar I disorder requires the presence of mania, which is not described here.

Borderline personality disorder is incorrect. The history is not consistent with a personality disorder such as borderline personality disorder, in which patients present with emotional lability, manipulation, and problematic interpersonal relationships.

Major depressive disorder is incorrect. The history is not consistent with major depression, as the patient describes periods of hypomania with minor depressive symptoms.

Alzheimer’s disease is correct. Neurofibrillary changes in the hippocampus are a hallmark of neurocognitive disorder due to Alzheimer’s disease. Hyperphosphorylated tau is a characteristic form of tau comprising the neurofibrillary tangles of Alzheimer’s disease.

Creutzfeldt-Jakob disease causes prions within the brain which are misfolded proteins. Common diagnostics of this disease are 14-3-3 protein or tau in cerebrospinal fluid (CSF), EEG with bi- or triphasic periodic sharp wave complexes, and Brain MRI with hyperintensities on fluid-attenuated inversion recovery (FLAIR) and T2 weighted images in the cortex, corpus striatum, and putamen.

Lewy bodiy disease are composed of aggregated alpha-synuclein.

Neurosyphillis is a spirochete bacteria (T. Pallidum.) It can be seen in cerebrospinal fluid (CSF) of neurosyphilis patients.

Vascular dementia from vascular disease leads to a stepwise reduction in cognitive ability via many infarcts. Typically the blood vessels show damage and lipid deposits.

Lorazepam is the correct option and the drug of choice for delirium tremens. The history of alcohol consumption and symptoms starting 3-5 days after hospitalization makes delirium tremens the likely diagnosis. Delirium tremens usually presents with agitation, confusion, hallucinations, hemodynamic instability, fever, and sweating.

Haloperidol is incorrect as this drug is reserved for refractory cases of delirium tremens where benzodiazepine administration does not result in significant improvement.

Atenolol is incorrect as this drug is used to treat tachycardia and hypertension associated with delirium tremens that persists after administration of benzodiazepines.

Naloxone is incorrect as this is the drug of choice for acute opiate intoxication. Patients frequently receive opioids as part of pain management following hip replacement surgery. However, intoxication is rare in the hospital setting. Moreover, normal pupillary signs make opiate intoxication an unlikely option.

Physostigmine is incorrect as this medicine is indicated for intoxication with anticholinergic drugs. Signs suggestive of delirium due to anticholinergic toxicity are dry skin and membranes, urinary retention, flushing, and fixed, dilated pupils.

Lewy body dementia is the correct option. It is the second most common cause of dementia in the elderly. This patient presents with classic symptoms of progressive loss of memory, visual hallucinations, and cognitive decline. There is also evidence of autonomic involvement as illustrated by frequent falls, dizziness, urinary complaints, and constipation. He has Parkinsonism on examination. Patients with Lewy body dementia are very sensitive to the effects of neuroleptic medications, and these should not be prescribed in these patients. Midodrine, bolus water consumption, laxatives, and probiotics may improve symptoms due to autonomic dysfunction.

Alzheimer’s disease is an incorrect option because these patients have short-term memory loss and loss of insight. Visual hallucinations and features of Parkinson’s disease make it a less likely possibility.

Vascular dementia is an incorrect option because these patients often have risk factors for the development of strokes. They present with a stepladder decline in cognitive function over a period of time. CT scan will show infarcts in different brain areas, confirming the diagnosis.

Parkinson’s-associated dementia is an incorrect option because although patients with Parkinson?s disease develop cognitive impairment after 10 years, visual hallucinations and psychosis are not present.

Binswanger’s disease is an incorrect option because it is due to widespread damage to the deep layers of white matter in the brain due to atherosclerosis. Symptoms include impairment in memory, organization, attention, and decision-making skills.

Neurally mediated syncope is the correct option. It is the commonest syncope seen in young adults and occurs due to neurocardiogenic reflex. There may be syncope due to cerebral hypoperfusion due to hypotension, low heart rate, and vasodilation. Patients often experience nausea and sweating before the episode and feel fatigued afterward. It can be precipitated by strong emotions, micturition, defecation, or even rotating the head. Patients are reassured about the benign nature of the disease.

Orthostatic syncope is incorrect. It is characterized by the abrupt onset of syncope after a change in posture. It is accompanied by lightheadedness. It is caused by many disorders, including hypovolemia, medications, Parkinson’s disease, multiple system atrophy, multiple sclerosis, diabetes, or amyloidosis. Upon standing for 3 minutes, there is a decrease in systolic blood pressure of 20 mm Hg or diastolic blood pressure of 10 mm Hg or more.

Hypoglycemia-induced syncope is incorrect because it is unlikely in the absence of diabetes. Moreover, syncope due to neuroglycopenia does not resolve without appropriate intervention (glucose intake).

Postural orthostatic tachycardia syndrome is incorrect. Although it occurs in young women, it rarely causes syncope. It is characterized by a greater than 30-bpm increase in heart rate on standing without any substantial decrease in blood pressure.

Autoimmune autonomic neuropathy is an incorrect option. Patients present with syncope but may have sensory abnormalities on examination, pain, and loss of deep tendon reflexes.