Hydrocephalus due to a Cavernoma-Like Lesion of an Obliterated Cerebral Arteriovenous Malformation Treated by Embolization and Radiosurgery.

BACKGROUND: Gamma-Knife radiosurgery can be the treatment of choice for small cerebral arteriovenous malformations (AVMs) in eloquent brain areas or, in association with endovascular treatment, for large and complex AVMs. Among the possible delayed complications occurring after radiosurgery of AVMs, de novo formation of a cavernoma has only recently been described. The authors report a unique case of communicating hydrocephalus with highly proteinaceous cerebrospinal fluid due to a cavernoma-like lesion of an obliterated cerebral AVM treated by embolization and radiosurgery. CASE DESCRIPTION: A 43-year-old female patient with a left parieto-occipital AVM (Spetzler-Martin grade III) underwent a multimodality treatment comprising several endovascular procedures and Gamma-Knife radiosurgery. At the follow-up angiograms, the AVM was completely obliterated. Twelve years later, she came back to our attention for headache and vomit with the radiological evidence of brain edema, severe hydrocephalus, and a chronic encapsulated intracerebral hematoma. At the beginning, the drainage of hydrocephalus proved ineffective to improve symptoms and edema. Therefore, the surgical resection of both the obliterated AVM and the cavernoma-like lesion-histologically confirmed-was necessary. The hydrocephalus resolved as well as the cerebral edema after 3 weeks, without necessity of a ventriculoperitoneal shunt. CONCLUSIONS: In previous neurosurgical literature, the association of a caveroma-like lesion in an obliterated AVM and communicating hydrocephalus with highly proteinaceous cerebrospinal fluid has not yet been discussed. We believe that only the surgical resection of both the obliterated AVM and the cavernoma-like lesion can lead to complete clinical recovery of the hydrocephalus.

Long-duration epilepsy affects cell morphology and glutamatergic synapses in type IIB focal cortical dysplasia.

To investigate hypothesized effects of severe epilepsy on malformed cortex, we analyzed surgical samples from eight patients with type IIB focal cortical dysplasia (FCD) in comparison with samples from nine non-dysplastic controls. We investigated, using stereological quantification methods, where appropriate, dysplastic neurons, neuronal density, balloon cells, glia, glutamatergic synaptic input, and the expression of N-methyl-D-aspartate (NMDA) receptor subunits and associated membrane-associated guanylate kinase (MAGUK). In all FCD patients, the dysplastic areas giving rise to epileptic discharges were characterized by larger dysmorphic neurons, reduced neuronal density, and increased glutamatergic inputs, compared to adjacent areas with normal cytology. The duration of epilepsy was found to correlate directly (a) with dysmorphic neuron size, (b) reduced neuronal cell density, and (c) extent of reactive gliosis in epileptogenic/dysplastic areas. Consistent with increased glutamatergic input, western blot revealed that NMDA regulatory subunits and related MAGUK proteins were up-regulated in epileptogenic/dysplastic areas of all FCD patients examined. Taken together, these results support the hypothesis that epilepsy itself alters morphology-and probably also function-in the malformed epileptic brain. They also suggest that glutamate/NMDA/MAGUK dysregulation might be the intracellular trigger that modifies brain morphology and induces cell death.