Neuro Exam
Prognosis and Treatment
Demyelinating Disorders
Quality of Life
100

In MS, damage to the corticospinal tract often leads to which type of reflex and motor abnormality:
(A) Hyporeflexia and flaccid weakness
(B) Hyperreflexia and spastic weakness
(C) Absent reflexes and fasciculations
(D) Hyporeflexia with muscle atrophy

B. Hyperreflexia and spastic weakness
(UMN lesion pattern, seen in Janet’s R arm and leg with Babinski sign.)

100

Which of the following best describes the most common disease course of MS?
(A) Primary progressive
(B) Relapsing-remitting
(C) Secondary progressive
(D) Progressive-relapsing

B. Relapsing-remitting
(about 85% of patients begin with relapsing-remitting MS. Janet fits this with prior optic neuritis followed by new deficits years later.)

100

Which antibody is associated with neuromyelitis optica (NMO, Devic’s disease)?
(A) Anti-MOG
(B) Anti-AQP4
(C) Anti-Hu
(D) Anti-MBP

B. Anti-AQP4 (aquaporin-4)
➡ NMO is an MS mimic with severe optic neuritis + longitudinally extensive transverse myelitis.

100

Q: How does pregnancy typically affect MS relapse risk?
(A) Increases relapse risk during pregnancy
(B) Decreases relapse risk during pregnancy
(C) Has no effect on relapse risk
(D) Prevents progression to secondary progressive disease

Correct Answer: B. Decreases relapse risk during pregnancy
➡ Protective immunologic shift during pregnancy → relapses often decrease.

200

Janet’s MRI showed a lesion in the medial longitudinal fasciculus (MLF). On exam, this produced which abnormality?

(A) Loss of pupillary light reflex
(B) Loss of corneal reflex
(C) Diplopia due to impaired adduction of one eye and nystagmus of the other
(D) Ptosis with a dilated pupil

C. Diplopia due to impaired adduction of one eye and nystagmus of the other
(This is internuclear ophthalmoplegia (INO), classic for MS.)

200

Interferon-β and glatiramer acetate are first-line disease-modifying therapies (DMTs) for MS. What is their main purpose?
(A) Reverse demyelination
(B) Cure the disease
(C) Reduce relapse frequency and slow disability progression
(D) Treat acute attacks only

C. Reduce relapse frequency and slow disability progression
(DMTs do not cure but alter the disease course.)

200

A child develops multifocal neurologic symptoms 1 week after a viral illness. MRI shows diffuse, bilateral white matter involvement. Which demyelinating disorder is most likely?
(A) Acute disseminated encephalomyelitis (ADEM)
(B) Multiple sclerosis
(C) PML
(D) Leukodystrophy

A. Acute disseminated encephalomyelitis (ADEM)
➡ Typically post-infectious/post-vaccine; monophasic; more common in children.

200

What happens to relapse risk in the postpartum period?
(A) Relapse risk remains low
(B) Relapse risk increases
(C) Relapse risk disappears permanently
(D) Relapse risk is unaffected by breastfeeding

B. Relapse risk increases
➡ First 3–6 months postpartum = high relapse window.

300

Which of the following describes Lhermitte’s sign, a classic MS finding?
(A) Electric-shock sensation down the spine with neck flexion
(B) Loss of vibration and proprioception in both feet
(C) Burning neuropathic pain in one dermatome
(D) Tingling in the fingers after repetitive motion

A. Electric-shock sensation down the spine with neck flexion
(Due to demyelination of cervical spinal cord posterior columns.)

300

Natalizumab, a monoclonal antibody used in MS, carries risk of which potentially fatal complication?
(A) Pulmonary fibrosis
(B) Progressive multifocal leukoencephalopathy (PML)
(C) Guillain-Barré syndrome
(D) Acute disseminated encephalomyelitis (ADEM)

B. Progressive multifocal leukoencephalopathy (PML)
(Reactivation of JC virus → fatal demyelination. Board Exam favorite.)

300

Which demyelinating disorder is caused by reactivation of JC virus in immunocompromised patients?
(A) Progressive multifocal leukoencephalopathy (PML)
(B) Acute disseminated encephalomyelitis (ADEM)
(C) Neuromyelitis optica
(D) B12 deficiency

A. PML
➡ Linked with natalizumab, rituximab, HIV; rapid, fatal progression.

300

Which of the following disease-modifying therapies is generally considered safest in pregnancy?
(A) Interferon-β
(B) Glatiramer acetate
(C) Natalizumab
(D) Ocrelizumab

B. Glatiramer acetate
➡ Interferon-β has emerging safety data but glatiramer has longest safety record. Monoclonals are generally avoided.

400

A patient with MS develops dysarthria, scanning speech, and intention tremor. Which CNS structure is most likely affected?
(A) Basal ganglia
(B) Cerebellum
(C) Parietal lobe
(D) Hippocampus

B. Cerebellum
(MS plaques in the cerebellum or cerebellar pathways cause the “Charcot triad”: scanning speech, intention tremor, nystagmus.)

400

Janet was prescribed tizanidine (Zanaflex). What symptom does this drug target?
(A) Fatigue
(B) Cognitive slowing
(C) Spasticity
(D) Depression

C. Spasticity
(MS symptomatic therapy: Baclofen (GABA-B agonist) or tizanidine (α2-agonist) help manage spasticity.)

400

A 56-year-old man presents with paresthesias, ataxia, and cognitive changes. Labs show macrocytic anemia. MRI reveals symmetric dorsal column demyelination. Which deficiency is most likely?
(A) Vitamin D
(B) Vitamin E
(C) Vitamin B12
(D) Folate

C. Vitamin B12 deficiency
➡ Subacute combined degeneration: dorsal columns + lateral corticospinal tracts + spinocerebellar tracts.

400

Which factor is most predictive of long-term disability in MS?
(A) Early relapsing-remitting course with full recovery between attacks
(B) Frequent relapses with incomplete recovery and progression
(C) Pregnancy in early disease course
(D) Starting DMTs early

B. Frequent relapses with incomplete recovery and progression
➡ Early high disease activity and incomplete remission = worse prognosis. Early DMT improves outcomes.

500

Janet’s neuro exam showed subtle cognitive impairment (digit span, fund of knowledge), spasticity with Babinski, sensory loss, and urinary frequency. Which principle best explains how these findings confirm MS?
(A) Dissemination in a single CNS location
(B) Dissemination in space and time
(C) Crossed findings due to brainstem stroke
(D) Peripheral demyelination affecting multiple nerves

B. Dissemination in space and time
(MS diagnosis requires evidence of multiple CNS lesions (space) occurring at different times (time). Janet’s optic neuritis years earlier + current MLF and cord lesions fulfill this.)

500

Which statement best reflects long-term MS prognosis?
(A) Patients with optic neuritis always recover fully and never relapse.
(B) Most patients progress to significant disability within 5 years.
(C) DMTs can slow progression, but many patients eventually transition from relapsing-remitting to secondary progressive disease.
(D) Mortality within 10 years is expected in most untreated patients.

C. DMTs can slow progression, but many patients eventually transition from relapsing-remitting to secondary progressive disease.
(Natural history: 85% start with relapsing-remitting; majority transition to secondary progressive after ~10–20 years if untreated. DMTs significantly delay this.)

500

Which inherited disorder causes progressive white matter degeneration due to abnormal myelin metabolism, often presenting in childhood with motor regression, spasticity, and cognitive decline?
(A) Tay-Sachs disease
(B) Adrenoleukodystrophy
(C) Metachromatic leukodystrophy
(D) Acute disseminated encephalomyelitis

C. Metachromatic leukodystrophy
➡ AR defect in arylsulfatase A → accumulation of sulfatides → impaired myelin production.
(Bonus Board exam pearl: Adrenoleukodystrophy is X-linked, with VLCFA buildup → adrenal failure + demyelination.)

500

What is the approximate lifetime risk of MS in a child if one parent is affected?
(A) Same as general population (~0.1%)
(B) ~1–2%
(C) ~10%
(D) >50%

B. ~1–2%
➡ Risk increases modestly compared to population baseline, but not strongly inherited. Counseling point: MS is not directly passed down in most cases.

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