Brain γ-Aminobutyric Acid Changes in Stiff-Person Syndrome

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ORIGINAL CONTRIBUTION Brain -Aminobutyric Acid Changes in Stiff-Person Syndrome

Background: Patients with stiff-person syndrome (SPS) have circulating antibodies against glutamic acid decarboxylase, the rate-limiting enzyme responsible for the synthesis of -aminobutyric acid (GABA). Although the patients’ symptoms of stiffness and unexpected spasms can be explained on the basis of reduced or impaired inhibitory neurotransmitters, such as GABA, it is unclear whether the lev...

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NIH CONFERENCE The Stiff-Person Syndrome: An Autoimmune Disorder Affecting Neurotransmission of g-Aminobutyric Acid

The stiff-person syndrome, a rare and disabling disorder, is characterized by muscle rigidity and episodic spasms that involve axial and limb musculature. Continuous contraction of agonist and antagonist muscles caused by involuntary motor-unit firing at rest are the hallmark clinical and electrophysiologic signs of the disease. Except for global muscle stiffness, results of neurologic examinat...

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since 1951, it is now a weekly with 48 issues per year.

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Stiff Person Syndrome.

Stiff-person syndrome or Moersch-Woltmann is a very rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms involving axial and limb musculature. It is an autoimmune disorder resulting in a malfunction of aminobutyric acid mediated inhibitory networks in the central nervous system. We describe a patient of stiff person syndrome.

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Stiff person syndrome.

Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture. Most patients demonstrate exaggerated lum...

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ژورنال

عنوان ژورنال: Archives of Neurology

سال: 2005

ISSN: 0003-9942

DOI: 10.1001/archneur.62.6.970