In Huntington’s chorea, neurons are affected in which part of brain?
Huntington's disease is an autosomal dominant condition caused by a CAG trinucleotide repeat expansion in the HTT gene, which codes for huntingtin protein. The main areas affected are the basal ganglia. Specifically, the caudate nucleus and the putamen. These are parts of the striatum, which is the primary input nucleus of the basal ganglia. The degeneration here leads to the motor symptoms like chorea, which is the involuntary, jerky movements.
Now, looking at the options. The correct answer should be the caudate nucleus and putamen. Let's see the other options. If the options were, say, the substantia nigra, that's more involved in Parkinson's disease. The cerebellum is involved in ataxia. The globus pallidus might be part of the basal ganglia but is more involved in Parkinson's. The thalamus is part of the relay center but not the primary site in Huntington's.
Wait, the question might be expecting the answer as the caudate nucleus specifically. But in reality, both caudate and putamen are affected. So the answer is the striatum, which includes both. But the options might list them separately. Let me check standard references. Yes, the caudate nucleus and putamen (striatum) are the primary sites. So if the correct answer is listed as caudate nucleus, that's the main one. The putamen is also affected but might not be an option. So the key is the striatum, specifically the caudate nucleus.
Clinical pearl: Huntington's disease is associated with chorea, cognitive decline, and psychiatric symptoms. The caudate atrophy is a key feature. The correct answer is the caudate nucleus.
**Core Concept**
Huntington’s chorea is a neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the *HTT* gene. The **caudate nucleus** and **putamen** (collectively termed the *striatum*) are the primary brain regions affected, leading to motor dysfunction via disruption of basal ganglia circuits.
**Why the Correct Answer is Right**
The striatum (caudate + putamen) serves as the primary input nucleus of the basal ganglia. In Huntington’s disease, mutant huntingtin protein causes excitotoxicity, mitochondrial dysfunction, and apoptosis in these neurons. This degeneration disrupts the indirect pathway of the basal ganglia, resulting in chorea (involuntary movements) and cognitive decline.
**Why Each Wrong Option is Incorrect**
**Option A:** *Substantia nigra* is implicated in Parkinson’s disease, not Huntington’s.
**Option B:** *Cerebellum* degeneration is seen in ataxias (e.g., spinocerebellar ataxias).
**Option C:** *Globus pallidus* is part of the basal ganglia but is not the primary site of degeneration in Huntington’s.
**Option D:** *Thalam