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Chronic progressive steroid responsive axonal polyneuropathy: A CIDP variant or a primary axonal disorder?
Author(s) -
Uncini Antonino,
Sabatelli Mario,
Mignogna Teresa,
Lugaresi Alessandra,
Liguori Rocco,
Montagna Pasquale
Publication year - 1996
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199603)19:3<365::aid-mus14>3.0.co;2-r
Subject(s) - polyradiculoneuropathy , sural nerve , chronic inflammatory demyelinating polyneuropathy , medicine , polyneuropathy , axonal degeneration , guillain barre syndrome , nerve biopsy , acute motor axonal neuropathy , nerve conduction velocity , pathology , peripheral neuropathy , endocrinology , antibody , immunology , diabetes mellitus
Five patients are presented with chronic, progressive, predominantly motor polyneuropathy. CSF protein content was increased in four patients. Motor conduction velocities and EMG were consistent with axonal involvement. Sural nerve conductions were normal in all cases and sural nerve biopsy performed in one patient was normal. Serum antibodies to GM 1 , GD 1a , GD 1b , and GM2 were negative. All patients improved after steroid treatment and 3 completely recovered. Because of therapeutic implications it is important to differentiate these patients from those with chronic idiopathic axonal neuropathies. It is unclear whether this is a primary axonal, probably immune‐mediated, polyneuropathy, or whether it represents one extreme of the chronic inflammatory demyelinating polyradiculoneuropathy spectrum characterized by severe axonal loss. We suggest that the term “chronic inflammatory polyneuropathy,” encompassing cases from pure demyelinating to pure axonal neuropathies responsive to steroids, should be reinstated and that, like in Guillain—Barré syndrome, different subtypes should be individuated. © 1996 John Wiley & Sons, Inc.