Neurology Module 5

A 35-year-old woman presents with recurrent episodes of transient ischemic attacks (TIAs) characterized by right hemiparesis and aphasia. CT angiography (CTA) shows narrowing of the distal internal carotid arteries and proximal middle cerebral arteries, with a characteristic “puff of smoke” appearance due to the formation of a network of small, dilated vessels.

Which of the following is the next best step in confirming the diagnosis?

Digital subtraction angiography (DSA)

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MRI with diffusion-weighted imaging

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Transcranial Doppler ultrasonography

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PET scan with oxygen-15 labeled water

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CT perfusion scan

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Which of the following symptoms is considered a negative symptom in myopathy?

Muscle Atrophy

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Cramp

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Contracture

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Muscle Hypertrophy

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Myoglobinuria

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Which of the following is a common clinical feature of Emery-Dreifuss muscular dystrophy?

Early contractures of the elbows, Achilles tendons, and neck

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Proximal muscle weakness and wasting

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Delayed motor milestones

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Hypertrophic cardiomyopathy

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Cognitive impairment

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In the context of anti-synthetase syndrome, which of the following antibodies is least associated with interstitial lung disease?

Anti-Zo

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Anti-Jo-1

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Anti-PL-7

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Anti-PL-12

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Anti-glycyl transfer RNA synthetase

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A 45-year-old female presents with a three-month history of progressive proximal muscle weakness. On examination, she has difficulty rising from a chair and climbing stairs. Laboratory investigations reveal a raised CK level of 4000 U/L. Other blood tests and serologies are still pending.

Which of the following conditions is most likely responsible for her presentation?

Immune-Mediated Necrotizing Myopathy

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Acid Maltase Deficiency

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Myasthenia Gravis

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Myotonic Dystrophy

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Inclusion Body Myositis

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Which of the following muscle disorder is correctly matched with its associated gene testing:

Myotubular Myopathy – MTM1 mutation

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Oculopharyngeal Muscular Dystrophy – EMD

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Emery-Dreifuss Muscular Dystrophy – DMPK

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Myotonic Dystrophy – FSHD-1

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Facioscapulohumeral Dystrophy – PAPBN1

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Which type of myopathy is predominantly associated with anti-NT5C1A antibodies?

Inclusion body myositis

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Anti-synthetase syndrome

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Immune mediated necrotizing myopathy

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Non-inflammatory muscle disease

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Dermatomyositis

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Which of the following drugs/substances is NOT known to cause rhabdomyolysis with myoglobinuria?

Tacrolimus

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Statins

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Alcohol

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Cocaine

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Amphetamine

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Which of the following best describes the main pathophysiology of myasthenia gravis?

An autoimmune disorder where a T cell-dependent immune response leads to the production of antibodies that attack the acetylcholine receptors on the post-synaptic membrane of the neuromuscular junction, resulting in muscle weakness.

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A genetic disorder characterized by mutations in the dystrophin gene, resulting in the degeneration of muscle fibers and progressive muscle weakness.

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An inflammatory disease where cytokines induce the proliferation of T-cells that attack myelin sheaths, causing demyelination and muscle weakness.

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A metabolic disorder that affects the production of ATP in muscle cells, leading to reduced energy availability and muscle weakness.

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An infectious disease caused by a viral infection that directly damages the neuromuscular junction, leading to muscle weakness.

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Which of the following is the most common presenting symptom of myasthenia gravis (MG)?

Binocular diplopia

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Fluctuating muscle weakness

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Bulbar weakness

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Neck flexion weakness

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Facial weakness

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A 45-year-old female presents to the neurology clinic with a three-month history of progressive muscle weakness, particularly in her facial and proximal limb muscles. She reports difficulty with activities such as combing her hair, climbing stairs, and lifting objects.

On physical examination, she demonstrates fatigable weakness upon repetitive movements, such as eyebrow elevation and sustained grip.

Which of the following tests is the most sensitive in confirming a neuromuscular transmission problem in this patient?

Single-fiber electromyography (EMG) performed in a weak muscle

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Anti-MUSK antibodies

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Anti-acetylcholine receptor antibody

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Edrophonium test

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Ice pack test

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45-year-old female patient with a confirmed diagnosis of anti-MuSK (muscle-specific kinase) myasthenia gravis (MG) has been experiencing generalized muscle weakness, particularly affecting her bulbar muscles.

Her symptoms initially improved after several weeks of intravenous immunoglobulin but relapsed after a 6-month remission period, despite being on optimal doses of prednisone (80mg/day) and azathioprine (200mg/day).

Which of the following treatment options would be the most appropriate next step for managing this patients refractory anti-MuSK myasthenia gravis?

Switch azathioprine to mycophenonate.

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Start rituximab therapy.

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Switch azathioprine to cyclosporine.

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Quadruple her dose of prednisone.

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Start plasma exchange.

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Neurological autoimmunity is increasingly recognized as a complication of immune checkpoint inhibitor (ICI) cancer immunotherapy.

Which of the following is NOT a commonly reported neurological autoimmune complication associated with ICI therapy?

Multiple Sclerosis

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Myasthenia Gravis

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Guillain-Barré Syndrome

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Aseptic Meningitis

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Encephalitis

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Which of the following is an indication for thymectomy in patients with Myasthenia Gravis (MG)?

Presence of a thymoma

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Pure ocular MG with no generalized symptoms

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MG seronegative for acetylcholine receptor antibodies

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MG solely controlled by pyridostigmine

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Elderly patients with longstanding, stable MG

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A 35-year-old male presents to the emergency department with progressive muscle weakness in his lower limbs over the past week. He reports no recent infections or illnesses. Upon examination, the patient has diminished deep tendon reflexes and significant muscle weakness in both upper and lower extremities. Sensory examination reveals normal sensation to light touch, pinprick, and vibration.

Nerve conduction studies show reduced compound muscle action potentials (CMAPs) without significant sensory nerve action potentials (SNAPs) abnormalities.

Cerebrospinal fluid analysis reveals elevated protein with normal cell count (albuminocytologic dissociation).

Based on the clinical presentation and diagnostic findings, what is the most likely diagnosis?

AMAN (Acute Motor Axonal Neuropathy)

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AMSAN (Acute Sensory Motor Axonal Neuropathy)

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AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy)

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CIDP (Chronic Inflammatory Demyelinating Polyradiculoneuropathy)

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Multifocal Motor Neuropathy (MMN)

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A 45-year-old woman presents to the emergency department with a 5-day history of progressive double vision, difficulty swallowing, and unsteady gait. She also reports feeling excessively sleepy during the day. On examination, she has ophthalmoplegia, facial weakness, ataxia, and diminished deep tendon reflexes. Her mental status is impaired, with episodes of confusion and drowsiness.

MRI of the brain shows hyperintense lesions in the brainstem on T2-weighted images. Cerebrospinal fluid (CSF) analysis reveals elevated protein levels with normal cell counts (albuminocytologic dissociation).

Anti-GQ1b antibodies are positive.

Based on the clinical presentation and diagnostic findings, what is the most likely diagnosis?

Bickerstaff Brainstem Encephalitis (BBE)

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Miller Fisher Syndrome (MFS)

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Wernicke's Encephalopathy

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Myasthenia Gravis

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Multiple Sclerosis (MS)

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A 58-year-old male presents with progressive weakness in his arms and legs, along with muscle twitching and difficulty speaking. On examination, you note both upper and lower motor neuron signs in multiple body regions. His sensory function is intact, and electromyography (EMG) shows signs of denervation and reinnervation.

Given these findings, which of the following criteria supports a possible diagnosis of amyotrophic lateral sclerosis (ALS)?

Involvement of both upper and lower motor neurons in at least 1 body region

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Absence of muscle weakness in the lower limbs.

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Presence of sensory abnormalities in the affected areas.

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Positive family history of Parkinson's disease.

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Normal findings on electromyography (EMG).

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In the context of motor neuron disease, the ‘split hand syndrome’ refers to:

Preferential wasting of adductor policis brevis (APB) and first dorsal interosseous muscle (FDI) with relative preservation of the lateral abductor digiti minimi (ADM)

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Preferential atrophy of flexor policis brevis (FPB).

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Preferential asymmetrical atrophy of the thenar muscles in one hand.

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Sensory abnormalities observed in the hand, leading to altered sensation.

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Preferential wasting of lateral abductor digiti minimi (ADM) with relative preservation of the adductor policis brevis (APB) and first dorsal interosseous muscle (FDI)

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Which of the following spectrum of motor neuron diseases (MND) is typically associated with the worst prognosis in terms of survival and disease progression?

Amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD)

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Flail arm syndrome

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Flail leg syndrome

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Progressive muscle atrophy

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Pure primary lateral sclerosis

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Neurology Module 5

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