Journal of Neuro-Ophthalmology:
Gliomatosis Cerebri Presenting as Idiopathic Intracranial Hypertension in a Child
Zunz, Eran MD; Sira, Liat Ben MD; Constantini, Shlomi MD, MSc; Fattal-Valevski, Aviva MD; Yalon, Michal MD; Roth, Jonathan MD; Cagnano, Emanuela MD; Kesler, Anat MD
Section Editor(s): McCulley, Timothy J. MD
Neuro-Opthalmology Unit, Department of Ophthalmology (EZ, AK); Neuroradiology Unit, Department of Imaging (LBS); Department of Pediatric Neurosurgery (SC) and Pediatric Neurology Unit (AF-V), Dana Children's Hospital; Pediatric Oncology Unit, Tel-Hashomer Sheba Medical Center (MY); Institute of Pathology (EC), Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
The authors report no conflicts of interest.
Address correspondence to Eran Zunz, MD, Department of Ophthalmology, Neuro-Opthalmology Unit, Tel-Aviv Sourasky Medical Center, 6 Weisman Street, Tel Aviv 64239, Israel; E-mail: firstname.lastname@example.org
Abstract: We present a rare case of a diffuse anaplastic astrocytoma (gliomatosis configuration) in a child, which was misdiagnosed as pseudotumor cerebri following initially normal CT of the brain and elevated opening pressure on lumbar puncture with normal cerebrospinal composition.
An 11-year-old previously healthy boy was admitted to hospital with headaches and diplopia. Physical and neurological examinations were normal except for bilateral papilledema and right sixth nerve palsy. Brain CT (Fig. 1) and CT venography were normal. The patient underwent lumbar puncture with an opening pressure of 500 mmH2O, with normal protein and glucose levels, and no cells. Felt to have idiopathic intracranial hypertension (IIH), he was treated with acetazolamide.
Although the patient reported some improvement in headache, papilledema persisted, and 1 month later, MRI of the brain was performed. This study revealed subtle nonspecific signal changes in the right temporal and parietal lobes (Fig. 2). Magnetic resonance venography showed no evidence of venous sinus thrombosis. Another lumbar puncture revealed an opening pressure of 350 mm Hg, with normal cerebrospinal fluid (CSF) content.
Three months later, a second MRI demonstrated diffuse multifocal areas of T2 signal abnormality in the right parietal subcortical white matter and in the right temporal and occipital lobes involving the corpus callosum. These lesions did not enhance following contrast administration.
Five months after initial presentation, a third MRI (Fig. 3) showed progression of the subcortical infiltrative lesions in addition to involvement of the brainstem and superior cervical cord.
The patient underwent an open brain biopsy (Fig. 4) revealing anaplastic cells, which were positive for glial fibrillary acidic protein, and the proliferation marker MIB1 was present in 15%–20% of the cells (World Health Organization grade 3). Necrosis and vascular proliferation were absent. These findings were compatible with anaplastic astrocytoma, and the treatment with whole brain irradiation was initiated.
A number of neoplastic disorders of the central nervous system can mimic IIH. One such example is nonsolid tumor infiltration of the leptomeninges. While this disorder may cause only subtle changes on neuroimaging (1–3), there is often a CSF pleocytosis with elevation in protein. Spinal cord tumors may also mimic IIH but are likely to be evident in imaging (1). The most challenging masquerading lesion is an infiltrative brain tumor that does not cause mass effect, nor influence CSF composition, and may not be detected on initial neuroimaging (1,4). As our case illustrates, this may occur in gliomatosis cerebri (5).
Armstrong et al (6) reported a cohort of 13 pediatric patients with gliomatosis cerebri. The median age at time of diagnosis was 12 years, and 77% of patients were male. The most common presentation was seizure followed by hemiparesis. Only 1 patient reported headache. Frequency of papilledema was not stated, although on neuroimaging 8 patients demonstrated mass effect and 2 had hydrocephalus. Vates et al (7) described 22 patients with gliomatosis cerebri with only 1 in the pediatric age-group (6 years). Papilledema was present in 27% of cases, and in all these patients, there were varying degrees of mass effects on MRI. In contrast, our patient had papilledema without evidence of mass effect on initial neuroimaging. There are few reports of gliomas mimicking IIH. Weston and Lear (5) reported the case of a 44-year-old woman with signs of increased intracranial pressure, including papilledema. Initial brain CT and CSF analysis were normal. Despite undergoing a CSF shunt procedure, the patient continued to deteriorate, and 7 months later, brain MRI showed an infiltrating tumor of the cerebral hemisphere (5) and brain stem. Stereotactic biopsy confirmed the diagnosis of gliomatosis cerebri. Aroichane et al (8) documented the case of a 16-year-old girl on minocycline who presented with headaches for 12 months and diplopia and papilledema for 5 weeks. Brain MRI and CSF were normal, although the authors state that some neuroradiologists felt that there was subtle nonenhancing enlargement of the thalami, optic chiasm, and infundibulum. Eight weeks later, vision declined in the left eye, there was an incomplete bitemporal hemianopia on perimetry, and MRI now revealed a large mass involving the optic chiasm and diencephalon. This proved to be a glioblastoma multiforme.
Similarly, our case serves to emphasize that IIH is a diagnosis of exclusion, and patients must be monitored carefully. After initiating treatment, if neuro-ophthalmic signs and symptoms persist or progress, repeat neuroimaging is mandatory. Even subtle and seemingly nonspecific findings on imaging studies must be closely monitored.
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