SUMMARY Neuroacanthocytosis is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis in the peripheral blood. Clinical manifestations of this disorder resemble those of Huntington disease (HD). Neuroimaging features of neuroacanthocytosis are atrophy and signal intensity change of the striata on MR imaging, as in HD. We report herein the ca...

The term "neuroacanthocytosis" describes a heterogeneous group of molecularly-defined disorders which result in progressive neurodegeneration, predominantly of the basal ganglia, and erythrocyte acanthocytosis. The clinical presentation of neuroacanthocytosis syndromes typically involves chorea and dystonia, but a range of other movement disorders may be seen. Psychiatric and cognitive symptoms...

Neuroacanthocytosis syndromes (NA) include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. Fluor 18 -2-fluoro-2-deoxyglucose (18F-FDG-PET) positron emission tomography (PET) and technetium 99m -d, l-h...

Neuroacanthocytosis is an autosomal recessive neurodegenerative disease, characterized by chorea, dementia, seizure, acanthocytes on peripheral blood smear and caudate atrophy on brain magnetic resonance imaging (MRI).1,2 These patients have severe orofacial dyskinesia and especially eating dystonia that causes severe eating problems and tongue and cheek biting. Eating or feeding dystonia, in c...

We report on an uncommon manifestation of neuroacanthocytosis in a 31-year-old woman and the successful use of levetiracetam in the treatment of her neurological symptoms. Truncal tic is one of the major presenting features of this patient. We find that Levetiracetam, a new antiepileptic drug, was effective in eliminating this patient's truncal jerks and motor tic manifestations, such as eyelid...

Neuroacanthocytosis is a heterogeneous group of disorders which result in progressive neurodegeneration, predominantly of the basal ganglia, and erythrocyte acanthocytosis. We report a case of neuroacanthocytosis with typical phenotype of choreoacanthocytosis. A 40 year male presented with features of chorea with orofaciolingual dystonia producing eating and speech difficulties. There were feat...