Eosinophilic myelitis associated with atopic diathesis: a combined neuroimaging and histopathological study.
Histologically proven eosinophilic myelitis has rarely been reported except in connection with parasitism. To clarify its clinicopathological features, we conducted a nationwide survey of biopsy-proven eosinophilic myelitis of unknown cause throughout Japan. Six such cases were collected and studied immunologically and pathologically. All were young to middle-aged men. All showed a protracted and fluctuating course with mild disability for 3-25 (mean 12.5) months before biopsy. Magnetic resonance imaging revealed localized lesions of T2-high and T1-iso signal intensity with a partial gadolinium enhancement in all cases. Cerebrospinal fluid (CSF) examinations were completely normal except for modest pleocytosis in two cases. Eosinophilia was present in the peripheral blood in two cases but was absent from the CSF of all cases. In spite of the chronic nature of the disease, spinal cord pathology revealed very active lesions with marked cell infiltration consisting mainly of CD8(+) T cells and varying numbers of eosinophils in the perivascular areas and the parenchyma. Both the myelin and axons were severely disrupted in all cases. Moreover, eosinophil cationic protein (ECP), an activated eosinophil product, was heavily deposited in the tissues. All but one case had hyperIgEemia and mite antigen-specific IgE in the sera, and two had accompanying atopic disorders. The present study thus revealed idiopathic eosinophilic myelitis to be a localized and persistent inflammation of the spinal cord, with distinct clinicopathological features, that has a possible link to atopic diathesis.
Osoegawa
Manabu
M
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.
Ochi
Hirofumi
H
Kikuchi
Hitoshi
H
Shirabe
Susumu
S
Nagashima
Tatsuya
T
Tsumoto
Tomoyuki
T
Tamura
Yoji
Y
Yamabe
Kazutoshi
K
Takahashi
Hiroshi
H
Iwaki
Toru
T
Kira
Jun-Ichi
J
D000328:Adult; D001798:Blood Proteins / Q000378:metabolism; D004198:Disease Susceptibility; D047091:Eosinophil Granule Proteins; D004802:Eosinophilia / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology*; D004804:Eosinophils / Q000378:metabolism; D006801:Humans; D006969:Hypersensitivity, Immediate / Q000150:complications; D007073:Immunoglobulin E / Q000097:blood; D008297:Male; D008875:Middle Aged; D009187:Myelitis / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology*; D011859:Radiography; D012260:Ribonucleases; D013116:Spinal Cord / Q000000981:diagnostic imaging / Q000473:pathology*
D001798:Blood Proteins; D047091:Eosinophil Granule Proteins; D007073:Immunoglobulin E; D012260:Ribonucleases