Anti-GAD antibody syndrome with concomitant cerebellar ataxia, stiff person syndrome, and limbic encephalitis

Anti-GAD positive stiff-person syndrome and cerebellar ataxia: two treatable conditions that clinicians should be aware of.

The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome.

OBJECTIVE To evaluate the clinical spectrum of anti-GAD-positive patients with stiff-person syndrome (SPS) and provide reproducible means of assessing stiffness. BACKGROUND SPS can be difficult to diagnose. Delineation of the clinical spectrum in a well defined population will increase diagnostic sensitivity. METHODS In 20 anti-GAD-positive patients with SPS (six men, 14 women), screened am...

Anti-GAD antibody cerebellar ataxia mimicking Creutzfeldt-Jakob disease.

In a patient with a rapidly progressive neurological condition with ataxia and cognitive complaints, Creutzfeldt-Jakob disease (CJD) is often high in the differential, particularly when there is an elevated CSF 14-3-3 protein level. We present a case of anti-glutamic acid decarboxylase antibody (anti-GAD65) positive cerebellar ataxia associated with cognitive complaints and elevated CSF 14-3-3 ...

Disease-specific monoclonal antibodies targeting glutamate decarboxylase impair GABAergic neurotransmission and affect motor learning and behavioral functions

Autoantibodies to the smaller isoform of glutamate decarboxylase (GAD) can be found in patients with type 1 diabetes and a number of neurological disorders, including stiff-person syndrome, cerebellar ataxia and limbic encephalitis. The detection of disease-specific autoantibody epitopes led to the hypothesis that distinct GAD autoantibodies may elicit specific neurological phenotypes. We explo...

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...