Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Eosinophilic granulomatosis with polyangiitis presentin with Guillain-Barré syndrome-like acute course. A case report

Ryota Ueda, M.D.1), Keisuke Imai, M.D.1), Atsushi Yamamoto, M.D.1), Tetsuya Ioku, M.D.1), Masatoshi Kadoya, M.D., Ph.D.2) and Masashi Hamanaka, M.D.3)

1) Department of Neurology and Stroke Treatment, Kyoto Daiichi Red Cross Hospital
2) Department of Rheumatology, Kyoto Daiichi Red Cross Hospital
3) Department of Neurology, Kyoto Daini Red Cross Hospital

A 57-years-old man with a history of bronchial asthma and pansinusitis developed acute progressive muscle weakness and sensory disturbance of the distal limbs after upper respiratory infection. On day 15 after onset of sensory disturbance and muscle weakness, the patient admitted to our hospital. A neurological examination revealed asymmetry weakness of both proximal and distal muscles, "glove and stocking type" hypoesthesia, and paresthesia without obvious pain. Blood tests and a nerve conduction study demonstrated eosinophilia and elevation of MPO-ANCA, axonal multiple mononeuropathy, respectively. The cerebrospinal fluid was normal. Eosinophilic granulomatosis with polyangiitis (EGPA) or Guillain-Barré syndrome (GBS) were suspected. So intravenous immunoglobulin therapy (IVIg) and high dose methylprednisolone pulse therapy (HDMP) followed by oral prednisolone were started. However, neurological symptoms did not improve. Sural nerve biopsy on day 31 revealed varying myelinating fiber loss at every nerve bundle and perivascular lymphocytic infiltration. The results did not fulfill the pathologic criteria for EGPA, but supported the changes of vasculitis. Cyclophosphamide (CPA) pulse therapy was administered for the additional therapy. Neurological symptoms did not improve and worsened again after decreasing oral prednisolone; therefore, combined therapy with IVIg, HDMP, and CPA was administered. Neurological symptoms then diminished gradually and the MPO-ANCA level and number of eosinophils normalized. This case suggests the importance of early nerve biopsy to obtain pathological findings supportive of EGPA diagnosis to allow introduction of aggressive immunosuppressive therapy such as CPA in a case with acute progressive motor-sensory neuropathy due to EGPA mimicking GBS.
Full Text of this Article in Japanese PDF (1969K)

(CLINICA NEUROL, 61: 624−629, 2021)
key words: eosinophilic granulomatosis with polyangiitis, Guillain-Barré syndrome, nerve biopsy, intravenous immunoglobulin therapy, cyclophosphamide pulse therapy

(Received: 9-Feb-21)