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Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 45
Author(s) -
Quaranta L,
Sabatelli M,
Madia F,
Lippi G,
Conte A,
Mereu ML,
Tonali P
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.1529-8027.2003.00045.x
Subject(s) - medicine , nerve biopsy , etiology , weakness , pathological , atrophy , context (archaeology) , pathology , polyneuropathy , peripheral neuropathy , surgery , paleontology , endocrinology , biology , diabetes mellitus
Unclassified neuropathies still represent a challenge for neurologists, since in about 20–30% of patients no cause can be identified after extensive investigations. In this context, some patients sharing peculiar features have been reported as to be grouped in a single entity termed Chronic Idiopathic Axonal Polyneuropathy (CIAP). The onset occurs in middle to late adulthood, there is a predominant sensory involvement and progression is slow, usually not disabling. Electrophysiologic and pathological studies are consistent with a primary axonal injury. We describe five patients affected by adult‐onset chronic “idiopathic” axonal neuropathy with an unusual course characterized by severe disability. Onset occurred in the fifth‐seventh decades with sensorimotor symptoms and signs involving both lower and upper limbs with a distal distribution. Over a 2–14 year follow‐up, the disease showed a progressive course with severe muscle weakness and atrophy of semi‐distal and distal muscles in all patients, and involvement of proximal muscles in four of them. Two patients died because of respiratory and heart failure. All five patients were sporadic. Extensive laboratory examination excluded identifiable causes of neuropathy. Nerve biopsy was carried out in all patients and showed an axonal neuropathy. Amyloid deposits were not observed in nerve sections nor in abdominal fat aspirate. In four patients, treatment with steroids and IVIg was unsuccessful. The nosology of acquired adult‐onset axonal polyneuropathies is difficult to be drawn, since in most cases the etiology remains obscure. Our findings suggest that, in this not yet well defined group of neuropathies, different subtypes may exist and represent distinct entities.