HEREDITARY THERMOSENSITIVE NEUROPATHY - AN AUTOSOMAL-DOMINANT DISORDER OF THE PERIPHERAL NERVOUS-SYSTEM

We report the clinical and electrophysiologic characteristics of eight patients (four men and four women) with a hereditary neuropathy with probable thermosensitivity (HTN) of autosomal dominant inheritance. Pa tients presented reversible episodes of ascending muscle weakness, par esthesiae, and areflexia apparently triggered by an elevation of body temperature over 38.5 degrees C. Mean age at onset was 13 +/- 12 (SD; range 6 to 43). Four patients had suffered up to five attacks. EMG and pathologic findings were compatible with a reversible demyelinating n europathy such as Guillain-Barre syndrome. We excluded loci causing ot her hereditary demyelinating neuropathies, such as Charcot-Marie-Tooth disease type I (CMT type I) and hereditary neuropathy with liability to pressure palsies (HNPP), by linkage analysis; thus, HTN is not alle lic to either CMT type I or to HNPP.