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Seizure in Patient with Neurofibromatosis and Amygdala Low-Grade Glioma
Joseph A Carnevale1, Jacob L Goldberg1, Theodore Schwartz1
1Department of Neurological Surgery, New York Presbyterian Hospital-Weill Cornell Medical Center, New York, New York, USA.
Abstract:
A 31-year-old male with history of bipolar disorder, suicidal attempts requiring inpatient hospitalization, and seizures on antiepileptic medications presented with increasing seizure frequency. He was neurologically intact yet had multiple facial, axillary, and inguinal flat pigmented macules (cafe au lait spots) and ophthalmologic examination with iris hamartomas (Lisch nodules) consistent with neurofibromatosis type 1. Electroencephalogram was notable for multiple right temporal lobe seizures with anterior temporal interictal epileptiform discharges. Magnetic resonance imaging revealed a T2 hyperintense mass centered in the right amygdala, contiguous with the cystic area. Preoperative intracarotid sodium amobarbital testing showed left-sided language and memory dominance. The patient was operated on for right amygdalohippocampectomy, and initial pathology was consistent with a low-grade neuroepithelial neoplastic process. Further pathologic examination found hypercellular proliferation of predominantly bland, spindled cells with scattered, embedded neurocytic elements with dysplastic appearance, consistent with low-grade glioma. The patient was clinically diagnosed with neurofibromatosis type 1.
Insights
This case highlights a rare instance of low-grade glioma in a patient with neurofibromatosis type 1, presenting with drug-resistant epilepsy. Surgical intervention for the temporal lobe tumor improved seizure control.
Area of Science:
- Neurology
- Oncology
- Genetics
Background:
- Neurofibromatosis type 1 (NF1) is a genetic disorder associated with an increased risk of various tumors, including gliomas.
- Epilepsy is a common neurological complication in patients with NF1, often linked to brain abnormalities.
Observation:
- A 31-year-old male with NF1 and a history of epilepsy presented with worsening seizure frequency.
- Clinical examination revealed cafe au lait spots and Lisch nodules, characteristic of NF1.
- EEG and MRI identified a right amygdala mass, suggestive of a neoplastic process.
Findings:
- The patient underwent right amygdalohippocampectomy for the temporal lobe mass.
- Pathological examination confirmed a low-grade glioma with specific cellular characteristics.
- Preoperative Wada testing indicated left-hemisphere dominance for language and memory.
Implications:
- This case underscores the importance of considering low-grade gliomas in NF1 patients with refractory epilepsy.
- Early diagnosis and appropriate management, including surgical resection, are crucial for seizure control and patient outcomes.
- Further research into the specific oncogenic pathways in NF1-associated gliomas may lead to targeted therapies.
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