Chronic inflammatory demyelinating polyneuropathy-like disorder associated with amyotrophic lateral sclerosis |
| |
Authors: | Rajabally Yusuf A Jacob Saiju |
| |
Institution: | Neuromuscular Clinic, Department of Neurology, University Hospitals of Leicester, Leicester General Hospital, Leicester, UK. yusuf.rajabally@uhl-tr.nhs.uk |
| |
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. |
| |
Keywords: | amyotrophic lateral sclerosis chronic inflammatory demyelinating polyneuropathy demyelinating neuropathy electrophysiology El Escorial |
本文献已被 PubMed 等数据库收录! |