Stiff-Person Syndrome 68 articles
Inhibition of gamma-aminobutyric acid synthesis by glutamic acid decarboxylase autoantibodies in stiff-man syndrome.
We assessed the effect of GAD autoantibodies (GAD-A) on GAD enzymatic activity in vitro; 83% of GAD-A-positive SMS sera reduced GABA production in crude rat cerebellar extracts, whereas GAD-A- sera from SMS patients or healthy blood donors did not alter the enzyme activity. Inhibition of GABA synthe...
Rigidity and spasms from autoimmune encephalomyelopathies: stiff-person syndrome.
Stiff-person syndrome (SPS) is a disorder characterized by progressive muscle rigidity with superimposed painful muscle spasms and gait impairment due to continuous motor activity. Evidence has accumulated in favor of SPS representing an autoimmune, predominantly encephalomyelopathic disorder result...
Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus.
We report on eight patients with stiff-man syndrome (SMS) or its "plus" variant, progressive encephalomyelopathy with rigidity and myoclonus (PERM) receiving intrathecal baclofen via pump. In six of the patients, follow-ups continued for approximately 2.5 to 6.5 years after pump implantation. Intrat...
Stiff-man syndrome in a woman with breast cancer: an uncommon central nervous system paraneoplastic syndrome.
We report a patient who developed stiff-man syndrome, including disabling shoulder subluxation and wrist ankylosis, in association with breast cancer. Immunologic investigations disclosed autoimmunity directed against not only glutamic acid decarboxylase but also amphiphysin, a 128-kd protein locate...
[Autoantibodies against GAD and GAD65].
Nippon rinsho. Japanese journal of clinical med... 56 Suppl 3:190 (1998) PMID 9513413
[Stiff limb syndrome associated with Hashimoto's encephalopathy: improvement after corticotherapy].
We report a case of a 70-year old man who presented with a stiff limb syndrome associated with symptoms highly suggestive of Hashimoto's encephalopathy. The signs of encephalopathy dramatically resolved after corticosteroid treatment, and remissions was complete after 12 months. The stiff limb syndr...
Paraneoplastic neuromuscular syndromes.
Although paraneoplastic syndromes are rare, a number of well- defined, neuromuscular paraneoplastic syndromes have been described and their pathophysiology listed. Many different malignancies have been associated with these syndromes, but small-cell lung cancer is the most common. Features shared by...
[Unusual causes of gait disorders].
We believe that a better knowledge of these unusual forms of gait disorder may allow general practitioners to identify them more accurately, to assess them and to improve therapeutic strategies....
Stiff person syndrome.
This communication considers a patient with episodic muscle spasms. Pharmacological intervention and electrophysiological studies provided adequate evidence confirming the diagnosis of stiff person syndrome. Electrophysiological studies in present communication similar to earlier reports, confirmed...
Stiff-person syndromes: motor cortex hyperexcitability correlates with anti-GAD autoimmunity.
The motor cortex is hyperexcitable in SPS and PER patients. However, hyperexcitability is partly masked by GABAmimetic treatment. Correlation of elevated GAD antibody levels with enhanced ICF suggests that motor cortex hyperexcitability in SPS and PER is related to anti-GAD autoimmunity....