4.0 Article

Hemiplegic-Migraine-like Attacks as First Manifestation of Diffuse Leptomeningeal Glioneuronal Tumor: A Case Report

Journal

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
Volume 44, Issue 3, Pages E765-E769

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPH.0000000000002287

Keywords

hemiplegic migraine; symptomatic hemiplegic migraine; cortical spreading depression; DLGNT; leptomeninges; meningeal enhancement

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We report a case of HM-like attacks in a DLGNT patient and discuss the pathogenesis of this case, as well as previously reported cases with similar symptoms involving the leptomeninges.
Background: Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a low-grade tumor characterized by diffuse leptomeningeal infiltrates. Symptoms are usually secondary to hydrocephalus. Hemiplegic migraine (HM)-like episodes have never been associated with DLGNT, but they have been reported with different inflammatory and tumoral entities involving leptomeninges. Observations: We report the case of a 10-year-old boy with recurrent episodes of right hyposthenia, aphasia, and headache lasting hours to days with complete remission. The electroencephalogram during the attack showed diffuse slower activity on the left hemisphere, which improved together with the symptoms. DLGNT was discovered during a follow-up magnetic resonance imaging and confirmed by biopsy. Conclusions: This is the first report of HM-like attacks in DLGNT. We discuss the pathogenetic hypotheses of our case and previously reported cases of symptomatic HM with leptomeningeal involvement.

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