DIFFERENCES BETWEEN HEREDITARY MOTOR AND SENSORY NEUROPATHY TYPE-2 AND CHRONIC IDIOPATHIC AXONAL NEUROPATHY - A CLINICAL AND ELECTROPHYSIOLOGICAL STUDY

To evaluate whether chronic idiopathic axonal polyneuropathy (CIAP) sh ould be considered as hereditary motor and sensory neuropathy type 2 ( HMSN type 2), we compared the clinical features of 48 patients with CI AP with those of 47 patients with HMSN type 2. In addition, we studied electrophysiological data in 20 patients with CIAP and in 20 patients with HMSN type 2. We found, in patients with HMSN type 2, that the in itial symptoms were predominantly motor and that weakness and handicap were more severe and skeletal deformities more frequent, compared wit h those of CIAP patients. Electrophysiologically, the tibialis anterio r muscle showed more denervation in patients with HMSN type 2, consist ent with the predominance of motor symptoms. There was no important ef fect of age of onset on clinical features in HMSN type 2 patients. We conclude that in an individual patient with a sensory or sensorimotor idiopathic axonal polyneuropathy and no family history of polyneuropat hies, the diagnosis HMSN type 2 is unlikely. However, if motor symptom s predominate, the diagnosis of HMSN type 2 should be considered.