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Steroid-responsive multifocal demyelinating neuropathy with central involvement

✍ Scribed by Rocco Liguori; Romana Rizzi; Roberto Vetrugno; Fabrizio Salvi; Alessandra Lugaresi; Sabina Cevoli; Pasquale Montagna


Publisher
John Wiley and Sons
Year
1999
Tongue
English
Weight
108 KB
Volume
22
Category
Article
ISSN
0148-639X

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✦ Synopsis


We describe 2 patients with associated central and peripheral demyelination. Electrophysiological studies revealed a demyelinating polyneuropathy with sensory and motor conduction blocks. Visual evoked potentials were abnormal. Motor evoked potentials showed abnormal central conduction time in 1 patient. Magnetic resonance imaging revealed regions of abnormal high signal in the spinal cord and brain; sural nerve biopsy disclosed a demyelinating neuropathy. Both patients showed clinical and electrophysiological improvement after steroid therapy.


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Hereditary neuropathy with liability to
✍ Anthony A. Amato; Richard J. Barohn πŸ“‚ Article πŸ“… 1996 πŸ› John Wiley and Sons 🌐 English βš– 402 KB

Hereditary neuropathy with liability to pressure palsies (HNPP) is usually caused by a 1.5-Mb deletion in chromosome 17~11.2, the inverse mutation to the duplication seen in the majority of Charcot-Marie-Tooth type 1A (CMT IA) patients. Although most patients with HNPP present with pressure palsies