A 16-Year-Old Boy Is Evaluated for Progressive Gait Instability, Dysmetria

question 48

Multiple Choice

A 16-year-old boy is evaluated for progressive gait instability, dysmetria, and dysarthria for the past several years.  The patient enjoys basketball but can no longer play due to his worsening symptoms.  He is worried as his brother died of a neurologic disease at age 21.  Physical examination of the lower extremities reveals bilateral motor weakness and loss of deep tendon reflexes, joint position, and vibration sense.  MRI of the brain and spine shows degeneration of the posterior columns and spinocerebellar tracts.  Which of the following conditions has clinical manifestations that closely resemble this patient's disease?

• A)Acute intermittent porphyria
• B)Lead intoxication
• C)Poliomyelitis
• D)Prion disease
• E)Thiamine deficiency
• F)Vitamin E deficiency

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Definitions:

Transformational Grammar

A linguistic theory concerning how deep structures of language are transformed into various surface structures through grammatical rules.

Language

A system of communication that uses grammar and vocabulary to convey meaning through the use of spoken, written, or gestural symbols.

Sign Language

A language that uses manual communication and body language to convey meaning, primarily used by deaf and hard-of-hearing individuals.

Speak

The act of producing sounds that form words in order to convey information or express thoughts and feelings.