HNPP in childhood: report of a case and brief review

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(Note: HNPP is the genetic opposite of CMT 1A)

Brain Dev. 2005 Mar;27(2):152-4.

Hereditary neuropathy with liability to pressure palsies in childhood: report of

a case and a brief review.

Ichikawa K, Nezu A.

Department of Pediatrics, Yokohama City University Medical Center, 4-57

Urafune-cho, Minami-ku, Yokohama 232-0024, Japan.

We present a 10-year-old female diagnosed having hereditary neuropathy with

liability to pressure palsies (HNPP). She had suffered from acute, recurrent

monoplegic episodes affecting both the sciatic nerves and the left brachial

plexus since the age of 7 years. The paresis seemed to be triggered by hiking

and athletic training. Electrophysiological studies showed a conduction block in

the proximal portions of affected nerves. The FISH method disclosed a deletion

of the peripheral myelin protein 22 gene. This school child having HNPP is

considered to be susceptible to the influence of abundant physical training,

rather than minor trauma or compression at sites of entrapment of peripheral

nerves.

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