Heredofamilial Brachial Plexopathy

Synonyms:

a.k.a. hereditary brachial plexus neuropathy a.k.a. hereditary neuralgic amyotrophy HNA, rare:

Clinical features:

Usually Painless
Attacks of weakness with recovery over weeks +some cranial involvement

Genetics:

Mapped to chr. 17q24-25
Autosomal dominant

Findings on investigations:

NCS/EMG: may be normal between attacks

Pathology, nerve biopsy:

Semithin plastic sections: Tomacula (sausage like excess myelin)
Teased fibres: Tomacula (sausage like excess myelin)

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