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ITM2B amyloidosis

ORPHA:439254· ICD-10 E85.4+

Definition

A rare, neurodegenerative disease characterized by progressive dementia and ataxia, widespread cerebral amyloid angiopathy and parenchymal amyloid deposition. Two subtypes have been identified, ABri amyloidosis and ADan amyloidosis.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Adult