NEUROLOGICAL MULTISYSTEM MANIFESTATION IN MULTIPLE SYMMETRICAL LIPOMATOSIS - A CLINICAL AND ELECTROPHYSIOLOGICAL STUDY

Multiple symmetric lipomatosis (MSL) is characterized by a typical nec k and shoulder distribution of subcutaneous lipomata and is often asso ciated with polyneuropathy. Occasionally, the central nervous system ( CNS) can be involved. Twelve of 14 patients in this retrospective stud y had clinical or electrophysiological evidence of a predominantly axo nal polyneuropathy. Among those were 10 with alcohol abuse, but 2 pati ents without alcohol abuse also showed clinical or electrophysiologica l polyneuropathy. Clinical CNS involvement was present in 4 patients. CNS dysfunction was documented by evoked potentials in 8 subjects [pro longed latency or low amplitude of the motor response following cortic al magnetic stimulation (4 patients), abnormal visually evoked potenti als (4 patients) or somatosensory evoked potentials (SEP) (4 patients) l. These findings were compared to 10 chronic alcoholics without clini cal signs of MSL. Five of these showed mild sensory neuropathy. Additi onally, 2 also had delayed SEP latencies. Motor evoked potentials were normal in ail controls. We propose that the multisystem involvement i n MSL demonstrated here cannot be attributed to alcohol abuse alone. B iochemical studies have suggested mitochondrial dysfunction as the bas is of the widespread neurological pathology in MSL. (C) 1995 John Wile y and Sons, Inc.