Abstract

Here we report a rare case of diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 62-year-old male patient misdiagnosed as having tuberculous meningitis. Due to its rarity and radiologic findings of leptomeningeal enhancement in the basal cisterns on magnetic resonance imaging (MRI) similar to tuberculous meningitis, DLGNT in this patient was initially diagnosed as communicating hydrocephalus from tuberculous meningitis despite absence of laboratory findings of tuberculosis. The patient's symptoms and signs promptly improved after a ventriculoperitoneal shunting surgery followed by empirical treatment against tuberculosis. Five years later, mental confusion and ataxic gait developed in this patient again despite well-functioning ventriculoperitoneal shunt. Aggravation of leptomeningeal enhancement in the basal cisterns was noted in MRI. An additional course of antituberculosis medication with steroid was started without biopsy of the brain. Laboratory examinations for tuberculosis were negative again. After four months of improvement, his mental confusion, memory impairment, dysphasia, and ataxia gradually worsened. A repeated MRI of the brain showed further aggravation of leptomeningeal enhancement in the basal cisterns. Biopsy of the brain surface and leptomeninges revealed a very rare occurrence of DLGNT. His delayed diagnosis of DLGNT might be due to prevalence of tuberculosis in our country, similarity in MRI finding of prominent leptomeningeal enhancement in the basal cisterns, and extreme rarity of DLGNT in the elderly. DLGLT should be considered in differential diagnosis of medical conditions presenting as communicating hydrocephalus with prominent leptomeningeal enhancement. A timely histologic diagnosis through a leptomeningeal biopsy of the brain and spinal cord in case of unusual leptomeningeal enhancement with uncertain laboratory findings is essential because cytologic examination of the cerebrospinal fluid in DLGNT is known to be negative.

Highlights

  • Glioneuronal tumors are a group of primary brain neoplasms of relatively recent acquisition in the World Health Organization (WHO) classification of central nervous system (CNS) tumors [1]

  • There have been several case series and case reports published that might have the same entity. They were variably described as diffuse leptomeningeal glioneuronal tumor [1], superficially disseminated glioma in children [12], or diffuse leptomeningeal oligodendrogliomatosis [13,14,15]

  • His diagnosis was delayed and he was misdiagnosed as having a communicating hydrocephalus caused by tuberculous meningitis

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Summary

Introduction

Glioneuronal tumors are a group of primary brain neoplasms of relatively recent acquisition in the World Health Organization (WHO) classification of central nervous system (CNS) tumors [1]. In the literature, they have been described in a variety of similar terms, e.g., DLGNT or disseminated oligodendroglial-like leptomeningeal tumor of childhood [2, 3]. They have been described in a variety of similar terms, e.g., DLGNT or disseminated oligodendroglial-like leptomeningeal tumor of childhood [2, 3] They mostly present as diffuse leptomeningeal diseases in children and adolescents. The current case highlights the importance of histologic confirmation through brain biopsy for cases presenting leptomeningeal enhancement in the basal cistern in MRI with equivocal laboratory examinations to explain the etiology

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