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.