Teaching NeuroImages: Histopathologically Confirmed Intracranial Enchondroma/Low-Grade Chondrosarcoma and IDH1-Mutated Diffuse Glioma in Ollier Disease

Teaching NeuroImages: A diffuse infiltrating retinoblastoma.

Teaching NeuroImages: Intracranial hypotension in a patient with Marfan syndrome.

An 18-year-old man with Marfan syndrome and migraine headaches presented with acute worsening of headaches with postural changes following spinal fusion surgery for scoliosis. Lumbar spine MRI done before surgery showed diffuse dural ectasia (figure 1). Brain MRI after surgery showed distended transverse and sagittal dural venous sinuses and an enlarged pituitary gland suggesting intracranial h...

Teaching NeuroImages: Multicompartmental intracranial hemorrhage in a pediatric patient.

A previously healthy 9-year-old girl presented with sudden-onset headache followed by confusion. In the emergency department, she was hypertensive, bradycardic, and no longer responsive to any stimuli. An emergent head CT scan demonstrated a left frontal intraparenchymal hematoma with intraventricular and subarachnoid extension as well as subfalcine herniation (figure). She underwent decompress...

In silico gene expression analysis reveals glycolysis and acetate anaplerosis in IDH1 wild-type glioma and lactate and glutamate anaplerosis in IDH1-mutated glioma

Hotspot mutations in isocitrate dehydrogenase 1 (IDH1) initiate low-grade glioma and secondary glioblastoma and induce a neomorphic activity that converts α-ketoglutarate (α-KG) to the oncometabolite D-2-hydroxyglutarate (D-2-HG). It causes metabolic rewiring that is not fully understood. We investigated the effects of IDH1 mutations (IDH1MUT) on expression of genes that encode for metabolic en...

Teaching NeuroImages: adrenoleukodystrophy presenting as raised intracranial pressure.

A 7-year-old boy presented with acute onset headache, vomiting, and visual deterioration 2 weeks previously without any cognitive or behavioral changes. Optic fundi showed papilledema, and there were no pyramidal signs or gait disturbances. The remainder of the neurologic examination was normal. CSF protein was 174 mg/dL. Although MRI features (figure) were consistent with adrenoleukodystrophy ...