Presynaptic inhibition of cerebellar GABAergic transmission by glutamate decarboxylase autoantibodies in progressive cerebellar ataxia

Cerebellar ataxia with progressive improvement.

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...

Rapidly progressive cerebellar ataxia in West Wales.

Progressive multifocal leucoencephalopathy (PML) is a severe demyelinating disease of the central nervous system that is caused by the JC virus infection. It is often fatal or severely disabling. PML exclusively happens in the context of cell-mediated immunosuppression. Prior to the era of HIV, PML was mainly confined to patients with haematological malignancies and rheumatological diseases. Th...

Progressive cerebellar ataxia and new-onset diabetes.

In October, 2012, a 37-year-old Rwandan businessman was transferred to our hospital from Nairobi, Kenya, where he had been investigated for subacute onset of progressive cerebellar ataxia and quadriparesis of unclear cause. He presented initially to a hospital in Nairobi in August, 2012, with a 2–3 week history of imbalance and weakness, as well as back pain and stiff ness, without cognitive or...

Respective implications of glutamate decarboxylase antibodies in stiff person syndrome and cerebellar ataxia

BACKGROUND To investigate whether Stiff-person syndrome (SPS) and cerebellar ataxia (CA) are associated with distinct GAD65-Ab epitope specificities and neuronal effects. METHODS Purified GAD65-Ab from neurological patients and monoclonal GAD65-Ab with distinct epitope specificities (b78 and b96.11) were administered in vivo to rat cerebellum. Effects of intra-cerebellar administration of GAD...

Cerebellar ataxia associated with anti-glutamic acid decarboxylase (anti-GAD) autoantibodies: a rare and puzzling disease.