Fetta Anna, Pruccoli Jacopo, Biasucci Giacomo, Parisi Roberto, Toni Francesco, Melchionda Fraia, Cordelli Duccio M
IRCCS Istitito delle Scienze Neurologiche di Bologna, UOC di Neuropsichiatria dell'età Pediatrica.
Pediatrics and Neonatology Unit, Mother and Child Health Department, Guglielmo da Saliceto Hospital, AUSL, Piacenza, Italy.
J Pediatr Hematol Oncol. 2022 Apr 1;44(3):e765-e769. doi: 10.1097/MPH.0000000000002287.
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.
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.
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.
弥漫性软脑膜神经胶质瘤(DLGNT)是一种低度肿瘤,其特征为软脑膜弥漫性浸润。症状通常继发于脑积水。偏瘫性偏头痛(HM)样发作从未与DLGNT相关联,但曾有报道称其与累及软脑膜的不同炎症和肿瘤性病变有关。
我们报告了一名10岁男孩的病例,该男孩反复出现右侧肌无力、失语和头痛,持续数小时至数天,随后完全缓解。发作期间的脑电图显示左半球弥漫性慢波活动,其随着症状改善而好转。在后续的磁共振成像检查中发现了DLGNT,并经活检证实。
这是DLGNT中出现HM样发作的首例报告。我们讨论了本病例以及先前报道的伴有软脑膜受累的“症状性”HM病例的发病机制假说。