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Morvan’s syndrome

Clinical Presentation and Symptoms: – Muscle weakness, fatigue, twitching, sweating, salivation – Joint pain, itching, weight loss – Confusional episodes, hallucinations, insomnia – Constipation, urinary […]

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Clinical Presentation and Symptoms:
– Muscle weakness, fatigue, twitching, sweating, salivation
– Joint pain, itching, weight loss
– Confusional episodes, hallucinations, insomnia
– Constipation, urinary incontinence, excessive lacrimation
– Neurological examination findings: muscle twitching, myoclonus, muscle atrophy, skin lesions

Specific Symptoms and Findings:
– Insomnia: severe reduction in sleep need, atypical REM sleep, absence of sleep rhythms
– Neuromyotonia: muscle twitching, cramping at rest, exacerbation with exercise
– Other symptoms: breathing difficulties, metabolic changes in brain regions
– Imaging findings: temporal lobe involvement
– CSF analysis: oligoclonal bands, increased IgG

Pathophysiology and Antibody Basis:
– VGKC antibodies detectable in acquired neuromyotonia patients
– Antibodies binding to hippocampus similar to known VGKCs
– Anti-VGKC antibodies cause nerve hyperexcitability
– Additional antibodies against neuromuscular junction channels described

Comorbid Conditions and Differential Diagnosis:
– Association with pulmonary hyalinizing granulomas
– Presence of thymoma, prostate adenoma in some cases
– Connection with in situ carcinoma of the sigmoid colon
– Similarity with limbic encephalitis, differentiation based on symptoms
– VGKC antibodies found in patients with limbic encephalitis

Treatment and Epidemiology:
– Plasmapheresis with steroids commonly used
– Intravenous immunoglobulin effective in some cases
– High-dose corticosteroids show improvement in symptoms
– Limited reported cases of Morvan’s syndrome in English literature
– Highly variable natural history, some cases require long-term immunosuppression

Morvan's syndrome (Wikipedia)

Morvan's syndrome is a rare, life-threatening autoimmune disease named after the nineteenth century French physician Augustin Marie Morvan. "La chorée fibrillaire" was first coined by Morvan in 1890 when describing patients with multiple, irregular contractions of the long muscles, cramping, weakness, pruritus, hyperhidrosis, insomnia and delirium. It normally presents with a slow insidious onset over months to years. Approximately 90% of cases spontaneously go into remission, while the other 10% of cases lead to death.

Morvan's syndrome
SpecialtyNeurology Edit this on Wikidata
SymptomsMorvan's fibrillary chorea

In 1890, Morvan described a patient with myokymia (muscle twitching) associated with muscle pain, excessive sweating, and disordered sleep. This rare disorder is characterized by severe insomnia, amounting to no less than complete lack of sleep (agrypnia) for weeks or months in a row, and associated with autonomic alterations consisting of profuse perspiration with characteristic skin miliaria (also known as sweat rash), tachycardia, increased body temperature, and hypertension. Patients display a remarkable hallucinatory behavior, and peculiar motor disturbances, which Morvan reported under the term “fibrillary chorea” but which are best described in modern terms as neuromyotonic discharges.

The association of the disease with thymoma, tumour, autoimmune diseases, and autoantibodies suggests an autoimmune or paraneoplastic aetiology. Besides an immune-mediated etiology, it is also believed to occur in gold, mercury, or manganese poisoning.

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