Distribution of electrophysiological abnormality in Lambert-Eaton myasthenic syndrome.
OBJECTIVE
To assess the distribution of electrophysiological abnormality in Lambert-Eaton myasthenic syndrome (LEMS) to identify the most sensitive muscle to use in routine examination.


METHODS
Surface recorded compound muscle action potential (CMAP) amplitudes were made from abductor digiti minimi, abductor pollicis brevis, anconeus, biceps brachii, and trapezius in 10 patients with LEMS. The effect of 3,4-diaminopyridine (3,4-DAP) was recorded in each muscle in nine patients. CMAP amplitudes were measured at rest and immediately after 10 seconds maximal voluntary contraction in each muscle. Values were compared with results obtained from 12 healthy controls.


RESULTS
Resting CMAP amplitudes were reduced in at least one muscle in all patients compared with controls, most markedly in abductor digiti minimi and anconeus. The administration of 3,4-DAP resulted in significantly improved resting CMAP amplitudes in trapezius only. After maximal voluntary muscle contraction, characteristic increments in CMAP amplitude of over 100% above resting values were seen in abductor digiti minimi and abductor pollicis brevis in seven patients, anconeus and biceps brachii in five patients. No patient had this level of increment in trapezius.


CONCLUSIONS
Despite predominantly proximal limb weakness seen clinically in patients with LEMS, the most sensitive muscles for detecting characteristic electrophysiological abnormalities of low resting CMAP amplitude and increment of over 100% after 10 seconds maximal voluntary contraction are abductor digiti minimi, abductor pollicis brevis, and anconeus.
University Department of Clinical Neurology, Radcliffe Infirmary, Oxford, UK. paul.maddison@clinical-neurology.ox.ac.uk
P
P
Maddison
J
J
Newsom-Davis
K R
KR
Mills
D015761:4-Aminopyridine / Q000031:analogs & derivatives / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000077770:Amifampridine; D003937:Diagnosis, Differential; D004558:Electric Stimulation; D004568:Electrodiagnosis; D019054:Evoked Potentials, Motor / Q000187:drug effects / Q000502:physiology; D005260:Female; D006801:Humans; D007537:Isometric Contraction / Q000187:drug effects / Q000502:physiology; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000294:innervation*; D010525:Peripheral Nerves / Q000187:drug effects / Q000503:physiopathology
D015761:4-Aminopyridine; D000077770:Amifampridine