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Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 47
Author(s) -
Ragno M,
Capasso M,
Caporale CM,
De Angelis MV,
Lupo S,
Di Muzio A,
Uncini A
Publication year - 2003
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2003.00047.x
Subject(s) - medicine , chronic inflammatory demyelinating polyneuropathy , weakness , polyradiculoneuropathy , nerve conduction velocity , neurological examination , peripheral neuropathy , guillain barre syndrome , sensory nerve , nerve biopsy , compound muscle action potential , pathology , polyneuropathy , sensory system , electrophysiology , surgery , antibody , immunology , neuroscience , endocrinology , psychology , diabetes mellitus
A 38‐year‐old man begun to have paresthesias in the last three fingers of the left hand followed in few months by paresthesias in the right hand, weakness of distal muscles in upper limbs and paresthesias of lower limbs. HCV positivity was discovered by routine blood test. Neurological examination, 18 months after onset, showed asymmetric weakness and sensory loss with a nerve distribution more on the right side. Electrophysiological examination showed a sensory‐motor demyelinating neuropathy with motor conduction blocks and or excessive temporal dispersion of proximal compound muscle action potential in five nerves. Coexistence of focal motor and sensory conduction blocks at the same sites was demonstrated in three nerves. CSF examination showed increased protein content (66 mg/dl) and no cells. Cryoglobulins were absent. Active HCV‐RNA replication was detected in the serum by RT‐PCR. Liver biopsy revealed chronic hepatitis. Sural nerve biopsy showed fiber loss, prevalently of large diameter fibers, asymmetrical between fascicles and few clusters of regeneration. There were no demyelinating features, onion bulbs or inflammatory infiltrates. Weakness and electrophysiological findings worsened after high doses intravenous immunoglobulins but improved after intravenous methylprednisolone and interferon alpha treatment with resolution of conduction blocks in two months. Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), as in the patient we describe, is a rare disorder considered an asymmetric variant of chronic inflammatory demyelinating neuropathy (CIDP). HCV infection is usually associated with a multineuropathy due to vasculitis or a symmetric sensory‐motor axonal polyneuropathy. CIDP has been described but never MADSAM. It is difficult to be sure of a pathogenetic link between a dysimmune demyelinating neuropathy and HCV infection, nonetheless the coexistence of hepatitis makes the choice of treatment problematic in these patients.