Which disease is characterized by autosomal dominant inheritance, behavioral and motor symptoms preceding diagnosis, and striatal degeneration?

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Multiple Choice

Which disease is characterized by autosomal dominant inheritance, behavioral and motor symptoms preceding diagnosis, and striatal degeneration?

Huntington disease is characterized by an autosomal dominant inheritance pattern, with a mutation in the HTT gene (CAG repeat expansion) passed in a dominant fashion. The clinical course typically starts with behavioral and personality changes, followed by motor symptoms such as chorea, and cognitive decline that precedes a formal diagnosis. The disease shows selective degeneration of the striatum, specifically the caudate nucleus and putamen, leading to the characteristic movement disorder and, on imaging, caudate atrophy with enlargement of the frontal horns of the lateral ventricles. This combination—dominant inheritance, early behavioral and motor manifestations, and targeted striatal degeneration—matches Huntington disease best. HIV-associated neurocognitive disorder isn’t inherited in a simple autosomal pattern and isn’t defined by primary striatal degeneration. Normal pressure hydrocephalus presents with a triad of gait disturbance, cognitive impairment, and urinary incontinence due to ventriculomegaly rather than striatal-focused pathology. Multiple system atrophy features autonomic failure and a mix of parkinsonian and cerebellar signs without a straightforward autosomal dominant pattern.

Huntington disease is characterized by an autosomal dominant inheritance pattern, with a mutation in the HTT gene (CAG repeat expansion) passed in a dominant fashion. The clinical course typically starts with behavioral and personality changes, followed by motor symptoms such as chorea, and cognitive decline that precedes a formal diagnosis. The disease shows selective degeneration of the striatum, specifically the caudate nucleus and putamen, leading to the characteristic movement disorder and, on imaging, caudate atrophy with enlargement of the frontal horns of the lateral ventricles.

This combination—dominant inheritance, early behavioral and motor manifestations, and targeted striatal degeneration—matches Huntington disease best. HIV-associated neurocognitive disorder isn’t inherited in a simple autosomal pattern and isn’t defined by primary striatal degeneration. Normal pressure hydrocephalus presents with a triad of gait disturbance, cognitive impairment, and urinary incontinence due to ventriculomegaly rather than striatal-focused pathology. Multiple system atrophy features autonomic failure and a mix of parkinsonian and cerebellar signs without a straightforward autosomal dominant pattern.

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