Dispersion of compound muscle action potential in hereditary neuropathies and chronic inflammatory demyelinating polyneuropathy.
Distal compound muscle action potential (DCMAP) dispersion, defined as a DCMAP duration > or = 9 ms, and proximal-distal (P-D) CMAP dispersion are considered useful in the electrodiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Distal and P-D CMAP dispersion have not been fully studied in hereditary neuropathies, and it is not known whether these measures distinguish hereditary from acquired demyelination. We compared DCMAP duration and P-D CMAP dispersion in 91 genetically characterized hereditary neuropathies and 33 subjects with CIDP. DCMAP dispersion was more frequent in nerves affected by CIDP (41.5%) than in Charcot-Marie-Tooth disease (CMT)1A (24.4%), CMT1B (7.4%), hereditary neuropathy with liability to pressure palsies (HNPP) (10.5%), or CMTX (9.8%). P-D CMAP dispersion was more frequent in CIDP (27.7% of nerves) than in hereditary neuropathies (16.3%) when applying American Academy of Neurology (AAN) criteria; however, its frequency was similar in CIDP and the hereditary neuropathies using the more restrictive criteria of the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM). Although dispersion is more common in CIDP than in the hereditary neuropathies, DCMAP and P-D dispersion occur in at least one motor nerve in a significant proportion of hereditary neuropathies, and cannot be used in isolation to distinguish acquired from hereditary demyelination.
Stanton
Michael
M
Department of Neurology, University of Rochester Medical Center, Rochester, New York 14642, USA.
Pannoni
Valerie
V
Lewis
Richard A
RA
Logigian
Eric L
EL
Naguib
Demian
D
Shy
Michael E
ME
Cleland
James
J
Herrmann
David N
DN
D000200:Action Potentials / Q000502:physiology*; D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000473:pathology / Q000503:physiopathology; D004594:Electrophysiology; D015417:Hereditary Sensory and Motor Neuropathy / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D009046:Motor Neurons / Q000473:pathology / Q000502:physiology; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology / Q000503:physiopathology*; D009431:Neural Conduction; D011115:Polyneuropathies / Q000473:pathology / Q000503:physiopathology*