There are a large number of well recognised syndromes comprising cerebellar ataxia in association with other neurological features. We report three family members who presented with a relapsing, early onset cerebellar ataxia, associated with progressive optic atrophy and sensorineural deafness. All three patients have areflexia (in the absence of a peripheral neuropathy), a pes cavus deformity,...

BACKGROUND Nonprogressive cerebellar ataxias are characterized by a persistent, nonprogressive ataxia associated with cognitive impairment. Cerebellar hypoplasia on imaging is variable but is not predictive of the degree of ataxia or cognitive impairment. OBJECTIVE To describe a family with a nonprogressive cerebellar ataxia associated with cognitive and motor impairments that improve with ag...

PURPOSE Common etiologies of acute acquired cerebellar ataxia include cerebrovascular diseases, toxin or drugs, infections/para-infections, and autoimmune diseases. It is a rare manifestation of Sjögren's syndrome, which is a common autoimmune disease but is often missed as a differential diagnosis. CASE REPORT This is a report of a patient with acute onset cerebellar ataxia for one month. Sh...

introduction: purkinje cell (pc) output displays a complex firing pattern consisting of high frequency sodium spikes and low frequency calcium spikes, and disruption in this firing behavior may contribute to cerebellar ataxia. riluzole, neuroprotective agent, has been demonstrated to have neuroprotective effects in cerebellar ataxia. here, the spectral analysis of pcs firing in control, 3-acety...

ataxia oculomotor apraxia1(aoa1) is the most frequent cause of autosomal-recessive cerebellar ataxia in japan,but it is reported from all of the world. the presentation is nearly identical to that of at without the non-neurological features,and accounts for up to 10% of autosomal-recessive cerebellar ataxias.gait imbalance and dysarthria are typical presenting features,oculomotor apraxia typica...