PDE10A-associated childhood-onset chorea with bilateral striatal lesions
Neurodegenerative diseases
General description
PDE10A-associated childhood-onset chorea with bilateral striatal lesions is typically characterized by its onset in the teenage years, presenting as slowly progressive chorea. As the disease progresses, abnormal movements become apparent, with dysarthria observed in the majority of cases. Although the typical onset is in childhood, this disease can also appear in the elderly.
Striatal lesion (Adult)
Non-SOL
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Caudate nucleus
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Putamen
On MRI, T2WI and FLAIR demonstrate bilateral hyperintensity in the striatum. In adults, imaging studies reveal bilateral striatal atrophy without diffusion restriction.
Striatal lesion (Pediatric)
Non-SOL
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Caudate nucleus
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Putamen
On MRI, T2WI and FLAIR demonstrate bilateral hyperintensity in the striatum. In pediatric cases with a short onset course, there may be diffusion restriction and swelling in the striatum.