Neurological multisystem manifestation in multiple symmetric lipomatosis: a clinical and electrophysiological study.
Multiple symmetric lipomatosis (MSL) is characterized by a typical neck and shoulder distribution of subcutaneous lipomata and is often associated with polyneuropathy. Occasionally, the central nervous system (CNS) can be involved. Twelve of 14 patients in this retrospective study had clinical or electrophysiological evidence of a predominantly axonal polyneuropathy. Among those were 10 with alcohol abuse, but 2 patients without alcohol abuse also showed clinical or electrophysiological polyneuropathy. Clinical CNS involvement was present in 4 patients. CNS dysfunction was documented by evoked potentials in 8 subjects [prolonged latency or low amplitude of the motor response following cortical magnetic stimulation (4 patients), abnormal visually evoked potentials (4 patients) or somatosensory evoked potentials (SEP) (4 patients)]. These findings were compared to 10 chronic alcoholics without clinical signs of MSL. Five of these showed mild sensory neuropathy. Additionally, 2 also had delayed SEP latencies. Motor evoked potentials were normal in all controls. We propose that the multisystem involvement in MSL demonstrated here cannot be attributed to alcohol abuse alone. Biochemical studies have suggested mitochondrial dysfunction as the basis of the widespread neurological pathology in MSL.
Naumann
M
M
Department of Neurology, University of Würzburg, Germany.
Schalke
B
B
Klopstock
T
T
Reichmann
H
H
Lange
K W
KW
Wiesbeck
G
G
Toyka
K V
KV
Reiners
K
K
D000328:Adult; D001369:Axons / Q000502:physiology*; D002490:Central Nervous System / Q000502:physiology / Q000503:physiopathology; D004594:Electrophysiology / Q000379:methods; D005071:Evoked Potentials; D005073:Evoked Potentials, Somatosensory; D005074:Evoked Potentials, Visual; D006801:Humans; D008069:Lipomatosis, Multiple Symmetrical / Q000503:physiopathology*; D008280:Magnetics; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000502:physiology / Q000503:physiopathology*; D010808:Physical Examination; D012016:Reference Values; D012189:Retrospective Studies