Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'.
We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area.
Clinica Neurologica, Istituto di Scienze Biomediche S. Gerardo, Universita di Milano, v. Donizetti 106, I-20052 Monza, Italia.
G
G
Cavaletti
P
P
Santoro
E
E
Agostoni
A
A
Zincone
C
C
Gori
L
L
Frattola
G
G
Tredici
D001342:Autonomic Nervous System Diseases / Q000188:drug therapy / Q000473:pathology*; D001369:Axons / Q000187:drug effects / Q000473:pathology*; D001706:Biopsy; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007249:Inflammation / Q000188:drug therapy; D008875:Middle Aged; D010525:Peripheral Nerves / Q000187:drug effects / Q000473:pathology* / Q000503:physiopathology; D011115:Polyneuropathies / Q000188:drug therapy / Q000473:pathology*; D011241:Prednisone / Q000008:administration & dosage; D012678:Sensation Disorders / Q000188:drug therapy / Q000473:pathology*
D011241:Prednisone