Gliomatosis cerebri presenting as intractable epilepsy during early childhood

Jennings, Mark T.; Frenchman, M.; Shehab, Thomas M.; Johnson, Melissa D.; Creasy, Jeffrey L.; LaPorte, K.; Dettbarn, Wolf D.

doi:10.1016/0887-8994(94)90406-5

Cited by 17 publications

(33 citation statements)

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“…Only one 6-year-old child is reported in another series of 22 patients (Vates et al, 2003) while Kim et al (1998) reported only adults in a series of 16 patients with CG. Presentation in early life as in our Case 1 is exceptional (Barth et al, 1988;Jennings et al, 1995).…”

Section: Discussionsupporting

confidence: 52%

Section: Discussionmentioning

confidence: 99%

“…The diagnosis of GC may be challenging and patients are often misdiagnosed initially as leukoencephalopathy, CNS vasculitis, encephalitis, multiple sclerosis, or a developmental abnormality such as hemimegalencephaly Jennings et al, 1995;Taillibert et al, 2006). Before the introduction of MRI, GC was largely a postmortem diagnosis at autopsy (Jennings et al, 1995). While the MRI findings of GC are not specific, they may suggest the diagnosis by demonstrating asymmetrical confluent areas of increased signal intensity on T2WI accompanied by absent or minimal contrast enhancement or mass effect relative to the magnitude of signal abnormality (Peretti et al, 2002, Taillibert et al, 2006.…”

Section: Discussionmentioning

confidence: 99%

“…The presentation of GC is diverse. Epilepsy is reportedly present in 30-40% of the adult series (Jennings et al, 1995;Kim et al, 1998;Vates et al, 2003;Taillibert et al, 2006) but is the most frequent presenting symptom in the pediatric population (Barth et al, 1988;Jennings et al, 1995;Shahar, 2002).…”

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confidence: 99%

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Epilepsy Surgery in Children with Gliomatosis Cerebri

et al. 2007

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Summary:Purpose: Gliomatosis cerebri (GC) is a rare neoplastic disorder that may present as intractable epilepsy during early life. We report our experience regarding the evaluation and the surgical treatment of epilepsy in this population.Methods: All children evaluated between 1990 and 2006 for surgery of epilepsy (n = 741) with pathologically proven GC were selected.Results: We identified four male children with age at seizure onset ranging from 4 months to 11 years. Two had hemiparesis and one child with infantile spasms was developmentally delayed. Seizures occurred daily (n = 3) or monthly (n = 1). Ictal semiology was consistent with psychomotor seizures (n = 1), partial motor seizures (n = 2), and asymmetric epileptic spasms (n = 1). Surgery was symptomatic and aimed at debulking and controlling the epilepsy. Procedure was individually tailored based on the presurgical evaluation. Brain MRI revealed widespread hemispheric involvement (n = 3) or infiltration of the temporal lobe and basal ganglia (n = 1). Two patients were initially misdiagnosed as hemispheric cortical dysplasia and hemimegalencephaly. Scalp EEG was nonlocalizing in two cases, showed a right temporal focus in one case, and was not performed in one case. Interictal SPECT in one patient revealed widespread hemispheric hypoperfusion. Three cases were resected under ECoG guidance after a mean delay of 11 months after seizure onset. Following functional hemispherectomy (n = 1) or focal cortical resection (n = 2), all children were alive and seizure free with a mean follow-up of 48 months (2-5 years). No unexpected complication was reported. One nonoperated case was alive but still seizing after 15 months follow-up. Chemotherapy was associated in three cases.Conclusions: GC is a rare cause of medically resistant epilepsy that may present in early life. The lack of a discrete lesion may lead to diagnostic uncertainty, especially in infancy. Epilepsy surgery is an effective therapy that can improve quality of life.

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Section: Discussionsupporting

confidence: 52%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Epilepsy Surgery in Children with Gliomatosis Cerebri

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“…Children with gliomatosis cerebri have been described in literature throughout the paediatric age group who present with intractable epilepsy (e.g. Jennings et al 1995). Although this child did not have seizures on presentation, she deteriorated with tonic seizures progressing to status epilepticus requiring ventilation.…”

Section: Discussionmentioning

confidence: 96%

Gliomatosis cerebri in a 10-year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumour

Jayawant¹,

Neale²,

Stoodley³

et al. 2001

Dev Med Child Neurol

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Gliomatosis cerebri is the unifying term used when diffuse glial infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiological and pathological findings. Adult cases may simulate an acute diffuse encephalomyelitis and show postmortem evidence of a marked swelling of the spinal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10-year-old girl who presented with a history and physical signs suggestive of benign intracranial hypertension. A diffuse encephalomyelopathy occurred, which was complicated by spinal cord swelling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination.

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Gliomatosis cerebri: Molecular pathology and clinical course

Herrlinger

Felsberg

Küker

et al. 2002

Annals of Neurology

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Gliomatosis cerebri is a rare, diffusely growing neuroepithelial tumor characterized by extensive brain infiltration involving more than two cerebral lobes. Among 13 patients with gliomatosis cerebri (median age, 46 years), biopsies showed features of diffuse astrocytoma (n = 4), oligoastrocytoma (n = 1), anaplastic astrocytoma (n = 5), anaplastic oligoastrocytoma (n = 1), or glioblastoma (n = 2). Molecular genetic investigation showed TP53 mutations in three of seven tumors and both PTEN mutation and epidermal growth factor receptor overexpression in one tumor. Amplification of CDK4 or MDM2 or homozygous deletion of CDKN2A was not detected. Three of 10 patients receiving radiotherapy showed a partial response (one patient) or had stable disease (two patients) lasting for more than 1 year. Four of six patients treated with procarbazine, carmustine, vincristine chemotherapy demonstrated partial remission (one patient), minor response (two patients), or stable disease (one patient). Median survival time from diagnosis was 14 months (range, 4-91+ months). Infratentorial involvement was associated with shorter survival. We conclude that (1) the molecular genetic alterations in gliomatosis cerebri resemble those in diffuse astrocytomas; (2) the prognosis of gliomatosis cerebri is variable but for at least 50% of patients as poor as for glioblastoma; and (3) some patients respond to radiotherapy and/or procarbazine, carmustine, vincristine chemotherapy.

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Gliomatosis cerebri presenting as intractable epilepsy during early childhood

Cited by 17 publications

References 0 publications

Epilepsy Surgery in Children with Gliomatosis Cerebri

Epilepsy Surgery in Children with Gliomatosis Cerebri

Gliomatosis cerebri in a 10-year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumour

Gliomatosis cerebri: Molecular pathology and clinical course

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