Primary diffuse leptomeningeal gliomatosis

Abstract

Glioma arising primarily from the leptomeninges is extremely rare and often diagnosed only in postmortem examination. It is thought to derive from heterotopic nests of neuroglial tissue within the leptomeninges, which are present in 1% of necropsies. Primary leptomeningeal glioma can be in the form of a solitary tumour or a diffuse tumour involving intracranial or spinal cord leptomeninges 1 . We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) established by meningeal biopsy. CASEA 13-year-old boy initiated symptoms at age of 7 years, with a partial complex seizure. He remained stable until the age of 8, when he presented another seizure. At this time he was admitted in coma into the critical care unit, and underwent treatment for viral encephalitis with acyclovir for 14 days. He was discharged with clinical improvement. Afterwards he presented recurrent headaches.Two years later he was admitted with headache, neck stiffness, drowsiness and vomiting. At this time an intracranial hypertension syndrome was identified. The cranial MRI showed a communicating hypertensive hydrocephalus that required a ventriculoperitoneal shunt. He was stable up to the age of 11, when a dysfunction of the ventriculoperitoneal shunt was identified and the shunt was changed. At this time etiologic investigation for chronic meningitis was initiated. Histoplasma capsulatum was considered the probable agent, because of his positive epidemiology and he underwent treatment with fluconazole. He was treated for two years and referred to our service.On admission at our hospital, at age of 13, he complained of sporadic headache, which was being treated with sodium valproate and fluconazole, with no seizures in the last year. During the investigation, different lumbar punctures were performed with elevated protein levels (up to 5.4 g/L), but glucose levels were normal. CSF white blood cells were up to 80/mm³. No neoplastic cells were detected. Acid fast bacilli smear, culture and tuberculosis DNA polymerase chain reaction (PCR) technique were all negative. Mycological analysis for histoplasmosis, paracoccidioidomycosis, aspergillosis and candidiasis were all negative. Immunological reactions to syphilis, toxoplasmosis, cytomegalovirus, cysticercosis and brucellosis were negative. Electroencephalograms revealed a generalized asynchronous slowing of background waves.Cranial CT showed diffuse calcifications in the basal cisterns suggesting some consequence of inflammatory process (Fig 1A).

Gliomatosis cerebri: Diagnostic considerations in three cases

“…[5] Since then, just over 300 cases have been reported in the literature. [2,[6][7][8][9][10][11][12][13][14][15][16][17][18][19] The largest case series was by Taillibert et al [3] who analyzed 90 cases from the French neurooncology network between 1993 and 2004 and more than 206 cases described in the literature between 1938 and 2004.…”

Primary diffuse leptomeningeal gliomatosis: a rare disease in pediatric patients

“…Todos esses casos tiveram taxas de sobrevida mais altas do que a mediana do grupo (variação de 15-96 meses). É interessante notar que o caso relatado por Gonçalves et al 5 teve uma sobrevida impressionante (somente equiparada a um outro caso, ver referências) 3,4 . Essa criança usou valproato, uma droga que teve seus efeitos antitumorais relatados recentemente e que poderia potencializar os efeitos da RT e da QT em pacientes com tumores cerebrais 6 .…”

“…Com isso, por falta de sustentação científica, não compactuamos. Vale a pena ressaltar que estudos [4][5][6] mostram que o risco atribuído pela síndrome metabólica não é maior que a soma de seus componentes, isto é, a síndrome não é maior que a soma das partes. Desse modo, faz-se necessário que todos os fatores de risco sejam individualmente combatidos, não sendo necessários os percentuais da síndrome por este ou aquele critério, para que se tratem, prescrevam e estimulem os hábitos saudáveis.…”