Abstract
The association between demyelinating neuropathy and amyotrophic lateral sclerosis (ALS) has been reported rarely. We report four patients who presented with clinical features and investigations suggestive of a neuropathy but who later progressed and received a final diagnosis of ALS according to the original El Escorial criteria. Three patients met the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2006 definition for "definite" chronic inflammatory demyelinating polyneuropathy (CIDP), as well as the American Academy of Neurology (AAN) 1991 and Nicolas et al. [2002] electrodiagnostic criteria for CIDP. Two of them showed segmental demyelination on teased-fiber preparations and one had a raised cerebrospinal fluid protein level. Another patient met the Nicolas et al. CIDP criteria and presented with brachial plexus hyperintensity on T2-weighted magnetic resonance imaging, indicative of an inflammatory process. No significant objective response to immunomodulatory treatment was observed in any of these patients who all subsequently progressed unfavorably.
Original language | English |
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Pages (from-to) | 855-60 |
Number of pages | 6 |
Journal | Muscle & Nerve |
Volume | 38 |
Issue number | 1 |
DOIs | |
Publication status | Published - Jul 2008 |
Bibliographical note
(c) 2008 Wiley Periodicals, Inc.Keywords
- Action Potentials/physiology
- Aged
- Amyotrophic Lateral Sclerosis/complications
- Disease Progression
- Electrodiagnosis
- Female
- Humans
- Immunologic Factors/therapeutic use
- Magnetic Resonance Imaging
- Male
- Middle Aged
- Muscle Weakness/etiology
- Neural Conduction/physiology
- Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications