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A 14-year-old adolescent girl had headache and intermittent crying and screaming for 4 months and upper back pain for 10 days. On examination, she was confused and had bilateral lagophthalmos and hearing loss, neck rigidity, and decreased deep tendon reflexes in the limbs. CSF analyses showed an elevated level of protein (1628 mg/L), a decreased level of glucose (0.934 mmol/L), and a normal nucleated cell count, with increased intracranial pressure (>330 mmH2O). After brain and spine MRI (Figure 1) and brain CTA (Figure 2), she had a brain biopsy. A diagnosis of diffuse leptomeningeal glioneuronal tumor (DLGNT) was pathologically confirmed (eFigure 1, links.lww.com/WNL/C508). Her parents abandoned treatment; she died 2 weeks later.
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and editor reviewed. The handling editor was Editor-in-Chief José G. Merino, MD, MPhil, FAHA, FAAN.
↵* These authors contributed equally to this work as first authors.
- Received August 5, 2022.
- Accepted in final form October 11, 2022.
- © 2022 American Academy of Neurology
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