Choreoacanthocytosis

Chorea-acanthocytosis (ChAc) is a form of neuroacanthocytosis (see this term) and is characterized clinically by a Huntington disease-like phenotype with progressive neurological symptoms including movement disorders, psychiatric manifestations and cognitive disturbances.

Dementia

A loss of global cognitive ability of sufficient amount to interfere with normal social or occupational function. Dementia represents a loss of previously present cognitive abilities, generally in adults, and can affect memory, thinking, language, judgment, and behavior.

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PMID (PMCID)
20544815	OTHER	Adult
	Characteristic head drops and axial extension in advanced chorea-acanthocytosis.
	Schneider SA, Lang AE, Moro E, Bader B, Danek A, Bhatia KP. Mov Disord. 2010;25(10):1487-91.
	Chorea-acanthocytosis is a rare autosomal recessive neurodegenerative disorder with a complex clinical presentation comprising of a mixed movement disorder (mostly chorea and dystonia), seizures, neuropathy and myopathy, autonomic features as well as dementia and psychiatric features.
1419352	FEMALE	Adult
	[Studies on the erythrocyte membrane skeleton in a patient with chorea-acanthocytosis--theoretical speculation on the mechanism of neurological involvement].
	Hosokawa T, Omoto K, Kanaseki T, Sugi Y, Wakamatsu H, Hamaguchi K. No To Shinkei. 1992;44(8):739-44.
	Patients with chorea-acanthocytosis exhibit symptoms of self-biting, choreic movement, and acanthocytosis, but not dementia.
2960785	MIXED_SAMPLE	Adult
	Positron emission tomography in cases of chorea with different underlying diseases.
	Hosokawa S, Ichiya Y, Kuwabara Y, Ayabe Z, Mitsuo K, Goto I, Kato M. J Neurol Neurosurg Psychiatry. 1987;50(10):1284-7.
	Hypometabolism was observed in the striatum bilaterally in patients with Huntington's disease, choreoacanthocytosis, sporadic progressive chorea and dementia, and pseudo-Huntington form of dentato-rubro-pallido-luysian atrophy (DRPLA).