Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: A long-term follow-up study |
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Authors: | Gert W van Dijk Nicolette C Notermans Hessel Franssen John H J Wokke |
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Institution: | (1) Department of Neurology, University Hospital Utrecht, 3508 GA Utrecht, The Netherlands e-mail: G.W.Dijk@neuro.azu.nl, Tel.: +31-302507975, Fax: +31-302542100, NL;(2) Department of Clinical Neurophysiology, University Hospital Utrecht, 3508 GA Utrecht, The Netherlands, NL |
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Abstract: | This long-term follow-up study examined patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and only sensory
symptoms at first presentation, with emphasis on the development of motor symptoms and long-term disability. From all CIDP
patients referred to our Department between 1987 and 1995, seven had only sensory symptoms at first clinical presentation.
These were investigated according to a standard protocol, including a quantified clinical neurological examination and nerve
conduction studies. The mean duration of the disease before weakness developed was 3.1 years, but varied considerably (0.8–6.3
years). At follow-up, weakness developed in five patients and persisted in three of them. Five patients were not seriously
incapacitated by their disease (Rankin 1 or 2), four of them being in remission now and one showing a very slow progression
of disease. Two patients were moderately disabled (Rankin 3); one had severe persistent sensory ataxia and only weakness during
relapses and one had stepwise progression and moderate weakness. Motor nerve conduction studies revealed that the most notable
worsening in the entire group of patients was a decrease in distal compound muscle action potential amplitudes, indicating
the development of distal conduction block or axonal degeneration. These findings show that CIDP with only sensory symptoms
is a transient clinical stage that precedes the appearance of weakness in about 70% of patients. The long-term prognosis does
not differ from that of patients with CIDP who have weakness at the beginning of the disease.
Received: 3 December 1998 Received in revised form: 17 May 1999 Accepted: 2 July 1999 |
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Keywords: | Sensory neuropathy Chronic inflammatory demyelinating polyneuropathy Follow-up |
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