(mentions CMT) Familial, demyelinating sensory and motor polyneuropathy with co

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Muscle Nerve. 2010 Apr;41(4):558-62.

Familial, demyelinating sensory and motor polyneuropathy with conduction block.

Scelsa SN.

Neuromuscular Division, Department of Neurology, Beth Israel Medical Center,

Albert Einstein College of Medicine, Ambulatory Care Center, 10 Union

Square East, Suite 5D, New York, New York 10003, USA.

Both multifocal, demyelinating features and prednisone responsiveness are rare

in Charcot-Marie-Tooth (CMT) disease.

We report a mother and son with a prednisone-responsive, multifocal,

demyelinating, predominantly sensory polyneuropathy that was associated with an

isoleucine92valine polymorphism of lipopolysaccharide-induced TNF-alpha factor

(LITAF).

The mother had a multifocal, acquired, demyelinating sensory and motor

polyneuropathy (MADSAM)-like presentation.

The son developed left peroneal neuropathy during acute Lyme disease with a

subsequent relapsing, MADSAM-like illness, despite antibiotic treatment.

Both shared prednisone responsiveness and multifocal, demyelinating features

electrophysiologically. MADSAM may be familial (FaDSAM) and respond to

prednisone.