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Neurology · USMLE Step 1

USMLE Neurology: Demyelinating and Neuromuscular Disease

Multiple sclerosis, myasthenia gravis, and Guillain-Barre syndrome mechanisms and distinguishing features tested on USMLE Step 1.

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What type of cell does multiple sclerosis primarily destroy?
Oligodendrocytes, the myelin-producing cells of the CNS
Which type of hypersensitivity reaction underlies multiple sclerosis?
Type IV (T-cell mediated) hypersensitivity
What are the three components of Charcot's classic neurologic triad, historically associated with MS?
Nystagmus, intention tremor, and scanning (staccato) speech
What is the most common initial presenting symptom of multiple sclerosis?
Optic neuritis, unilateral vision loss with pain on eye movement
Where are demyelinating plaques classically located on MRI in multiple sclerosis?
Periventricular white matter
What CSF finding supports a diagnosis of multiple sclerosis?
Oligoclonal IgG bands present in CSF but not in a matched serum sample
What demographic is most commonly affected by multiple sclerosis?
Young women, typically between ages 20 and 40
What is internuclear ophthalmoplegia, a classic MS eye finding?
Impaired adduction of one eye with nystagmus of the abducting eye, caused by a medial longitudinal fasciculus lesion
What is Uhthoff phenomenon in multiple sclerosis?
Temporary worsening of symptoms with increased body temperature, such as a hot shower or exercise
What is Lhermitte sign?
An electric shock-like sensation down the spine or limbs triggered by neck flexion
What is the most common clinical course of multiple sclerosis at the time of diagnosis?
Relapsing-remitting
What is the first-line treatment for an acute multiple sclerosis relapse?
High-dose intravenous corticosteroids
What is the target antigen of autoantibodies in myasthenia gravis?
Postsynaptic nicotinic acetylcholine receptors at the neuromuscular junction
Which type of hypersensitivity reaction is myasthenia gravis?
Type II hypersensitivity
What is the hallmark pattern of muscle weakness in myasthenia gravis?
Fatigable weakness that worsens with repeated activity and improves with rest

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