Teaching Video NeuroImages: Electromyographic variation in stiff-person syndrome

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Teaching Video NeuroImages: Electromyographic variation in stiff-person syndrome.

A 58-year-old man diagnosed with stiff-person syndrome presented with increased axial muscle tone (video 1, links.lww.com/WNL/A42). A successional electromyographic video in the right rectus abdominis demonstrated a large quantity of continuous motor unit potentials at rest. One minute after administration of diazepam, motor unit potentials were extraordinarily attenuated. However, 10 minutes a...

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Teaching NeuroImages: PET-CT hypermetabolism paralleling muscle hyperactivity in stiff-person syndrome.

A 72-year-old woman with autoimmune hypothyroidism and type 1 diabetes mellitus presented with 1 year of progressively worsening neck, back, and right thigh spasms and leg stiffness. Sudden loud noises would startle her. She ambulated with a walking frame. Serum glutamic acid decarboxylase 65 antibody was markedly elevated (1,118 nmol/L; normal value #0.02 nmol/L). EMG demonstrated poor relaxat...

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Stiff eyes in stiff-person syndrome.

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Teaching video neuroimages: involuntary muscle contractions in Hoffman syndrome.

Caitlin Loomis, MD Shawn J. Bird, MD Joshua M. Levine, MD Hoffman syndrome is a rare constellation of features that occurs in severely hypothyroid adults. It is characterized by muscle hypertrophy, weakness, stiffness, and slowness of movement with or without muscle cramps.1 Elevation of creatine kinase is common, but does not correlate with disease severity.2 A 50-year-old man from Ghana prese...

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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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ژورنال

عنوان ژورنال: Neurology

سال: 2018

ISSN: 0028-3878,1526-632X

DOI: 10.1212/wnl.0000000000004831