We describe a patient who developed significant cognitive decline with profound amnesia following non-dominant temporal lobectomy for refractory seizures, in whom the original suspicion of structural pathology was revised following the discovery of clinical and neuropathological markers of inflammation, neuropsychological evidence of bilateral involvement, and high titres of antibodies directed...

The development of excitatory and inhibitory synaptic connections has been studied in postnatal neurons from the caudate and putamen maintained in tissue culture. Excitatory postsynaptic potentials which were sensitive to the glutamate antagonist CNQX (6-cyano-7-nitroquinoxaline-2,3-dione) appeared between 4 and 8 d in vitro. This is the first indication that glutamatergic excitatory neurons ma...

Glutamic acid decarboxylase (GAD), the enzyme that catalyzes the conversion of glutamate to -aminobutyric acid (GABA), is expressed in GABA-secreting neurons and pancreatic cells.1 Anti-GAD antibodies (GAD Abs) have been described in patients with type 1 diabetes mellitus and patients with two CNS disorders, stiff-person syndrome and cerebellar ataxia associated with polyendocrine autoimmunity....

Stiff person syndrome (SPS) is a highly-disabling neurological disorder of the CNS characterized by progressive muscular rigidity and spasms. In approximately 60-80% of patients there are autoantibodies to glutamic acid decarboxylase (GAD), the enzyme that synthesizes gamma-amino butyric acid (GABA), the predominant inhibitory neurotransmitter of the CNS. Although GAD is intracellular, it is th...

In immunohistochemical reactions against glutamic acid decarboxylase (GAD), gamma-aminobutyric acid (GABA) and glycine (Gly), neurons in the mouse cerebellum showed the following reactivities: (1) the dendrites and cell bodies of the Purkinje cells were only GAD-positive, but their axonal terminals were GABA- and GAD-positive; (2) in both stellate and basket cells, the cell bodies and terminals...

1. Stiff-person syndrome (SPS) is an autoimmune disease characterized by muscle rigidity, episodic muscle spasms, as well as continuous cocontraction of agonist and antagonist muscles. 2. Although a criterion for diagnosis of SPS, anti– glutamic acid decarboxylase (anti-GAD) antibodies can be negative in up to 40% of patients. In absence of antibodies, characteristic EMG findings of continuous ...