Chronic inflammatory demyelinating polyneuropathy-like disorder associated with amyotrophic lateral sclerosis

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.