A comparison of clinically atypical with typical chronic inflammatory demyelinating polyradiculoneuropathy.
BACKGROUND
There is a paucity of prospective studies evaluating the atypical features of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).


OBJECTIVE
To compare clinical and electrodiagnostic features of clinically typical and atypical CIDP patients.


METHODS
The patients with typical and atypical CIDP diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society were included. The disability was graded on a 0-10 scale. Neurophysiological study included motor and sensory nerve conduction and F wave studies of both upper and lower limbs. The patients were treated with prednisolone with or without azathioprine. The outcome was evaluated at 6 months and improvement was defined as at least 2 grades improvement.


RESULTS
Eight out of 37 CIDP patients had atypical features, which included asymmetry in 2, dysautonomia in 3 and pure motor weakness, amyotrophic-lateral-sclerosis-like syndrome and distal weakness in 1 patient each. Tendon reflexes were retained in 3 patients. The mean duration of symptoms was 43 weeks in the typical and 30 weeks in the atypical group. The age, sex, disability, therapeutic response and neurophysiological features were similar in the 2 groups.


CONCLUSIONS
About one fifth of CIDP patients may have atypical clinical features; however, their electrodiagnostic features and response to treatment are no different from typical CIDP.
Misra
U K
UK
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. ukmisra@sgpgi.ac.in
Kalita
J
J
Yadav
R K
RK
D000200:Action Potentials / Q000502:physiology / Q000528:radiation effects; D000293:Adolescent; D000328:Adult; D000368:Aged; D004558:Electric Stimulation / Q000379:methods; D004568:Electrodiagnosis / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D010525:Peripheral Nerves / Q000473:pathology / Q000503:physiopathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology; D011446:Prospective Studies; D011930:Reaction Time / Q000502:physiology / Q000528:radiation effects; D012189:Retrospective Studies; D012720:Severity of Illness Index