Thursday, December 24, 2009

Morvan syndrome aka "Choree Fibrillaire"

Four cardinal features of Morvan syndrome are

1. Neuromyotonia or myokymia

2. Dysautonomia (esp hyperhidrosis, hypersalivation, labile hypertension). Weight loss is common.

3. Severe insomnia

4. Fluctuating encephalopathy with vivid hallucinations

Other notes-- MRI and random eeg is often normal. Patients are usually young males, EMG and PSG are not normal, and VGKC's are often present. Differential includes FFI, CJD, rabies virus, and Lewy body disease. The key clinical finding that differentiates is the dysautonomia and neuromyotonia. Often is fatal, but Ligouri et al. reversed one case with plasma exchange.

Ligouri R, Vincent A, Clover L, et al. Morvan's syndrome. Peripheral and central nervous system and cardiac involvement with antibodies to voltage gated potassium channels. Brain 124: 2417, 2001.

Note-- there is a second "Morvan's disease" that refers to atrophic changes in bone, skin, muscles of hand in syringomyelia.

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