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Immune trigeminal sensory neuropathy with esophageal achalasia: Improvement with long‐term immunotherapy
Author(s) -
Figueroa Juan J.,
Engelstad Janean K.,
Spinner Robert J.,
Dyck P. James B.
Publication year - 2011
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21889
Subject(s) - medicine , achalasia , anhidrosis , swallowing , sensation , surgery , esophagus , neuroscience , biology
We report a patient who developed subacute facial‐predominant numbness and anhidrosis, oral incoordination, and esophageal achalasia with resultant cachexia. Great auricular nerve biopsy showed extensive epineurial perivascular inflammatory infiltrates. Sensation, sweating, and swallowing improved with pulse intravenous methylprednisolone given over 5 years. We suggest that the patient's deficits, including achalasia, were due to an immune‐mediated sensory and autonomic neuropathy and that, in such cases, pathologic studies of the great auricular nerve may be diagnostically informative. Muscle Nerve 43: 289–293, 2011