Eosinophilic meningitis is an uncommon condition often associated with helminthic infections. We present a patient with idiopathic eosinophilic meningoencephalomyelitis occurring 9 months after an episode of Well syndrome, an idiopathic eosinophilic cellulitis. The disorder mimics Gordon phenomenon, an experimentally induced encephalitis in animals associated with eosinophil-derived neurotoxicity. Case report.A 55-year-old woman developed a rash on her legs. Skin biopsy revealed an eosinophilic cellulitis consistent with Well syndrome (figure, A and B). A peripheral eosinophilia of 1,134 cells/L was present. The cellulitis resolved with a short course of oral steroids, and the eosinophilia diminished.Nine months later, she developed vertigo and ataxia, followed by progressive dysarthria, double vision, headache, and tinnitus. A peripheral eosinophilia of 1,900 cells/L was present. An initial brain MRI was unremarkable. Two weeks later, the MRI showed mild leptomeningeal enhancement and 2 globoid temporal white matter lesions. The CSF revealed a lymphocytic pleocytosis with 140 white blood cells and protein of 104 mg/dL. Three weeks later, the eosinophilic fraction increased (351 cells; 81% lymphocytes, 8% eosinophils, 8% monocytes). Cytology was negative for malignancy. A FiP1L1 platelet-derived growth factor receptor test was negative, making eosinophilic leukemia unlikely.1 After a short course of empiric IV methylprednisolone, the patient was transferred to our hospital.On admission, 5 weeks after onset, her examination revealed cerebellar dysarthria, extremely slow saccades in all directions, profound dysmetria, and gait ataxia. Despite an empiric course of albendazole, these findings gradually worsened, and she developed downbeat nystagmus and mild left hemiparesis. Serial CSF examination revealed 76, 78, and 61 white blood cells, with an increasing eosinophilic fraction (4%, 24%, 33%). Oligoclonal bands were present. An infectious evaluation was negative for strongyloides, toxocara, Bartonella, enterovirus, Whipple, coccidiomycosis, cysticercosis, Epstein-Barr virus, cytomegalovirus, varicella zoster virus, JC virus, parvovirus, hepatitis B and C, HIV, Lyme, syphilis, toxoplasma, mycoplasma, rickettsia, and histoplasmosis. Rheumatologic serologies and paraneoplastic testing were unremarkable. Whole body CT scan and breast ultrasound revealed no abnormalities. MRI 8 weeks after presentation revealed mild cerebellar vermal enhancement, new enhancement of the right temporal lesion, and 2 nonenhancing cervical spine lesions. Biopsy of the right temporal lobe revealed a brisk leptomeningeal, perivascular, and intraparenchymal mixed infiltrate with florid gliosis (figure, C and D). High-dose IV methylprednisolone was initiated 1 week after completing albendazole, and the patient's saccades improved rapidly. She was discharged on oral prednisone. The eosinophilic cellulitis recurred after tapering the prednisone several months later, but resolved after reinitiating it. Eighteen months after presentation, she con...

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Five children with chronic progressive polyneuropathy but no familial history of it showed electrophysiological evidence of demyelination with partial conduction block, temporal dispersion, and focal slowing of nerve conduction velocities in multiple nerves. These findings are indicative of an acquired demyelinating polyneuropathy that is chronic and inflammatory and differentiate this condition from most of the inherited neuropathies. It is very important to recognize this entity because of the availability of various treatments.

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