Immune trigeminal sensory neuropathy with esophageal achalasia: improvement with long-term immunotherapy.
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.
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Juan J
JJ
Figueroa
Janean K
JK
Engelstad
Robert J
RJ
Spinner
P James B
PJ
Dyck
D000368:Aged; D001833:Body Temperature Regulation / Q000502:physiology; D004931:Esophageal Achalasia / Q000150:complications / Q000276:immunology / Q000628:therapy*; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D007167:Immunotherapy; D008297:Male; D020433:Trigeminal Nerve Diseases / Q000150:complications / Q000276:immunology / Q000628:therapy*