Bilateral hyperplasia of choroid plexus with severe CSF production: a case report and review of the glymphatic system.

BACKGROUND: An important feature of hydrocephalus is the alteration of the cerebral spinal fluid (CSF) homeostasis. New insights in the understanding of production, secretion, and absorption of CSF, along with the discovery of the glymphatic system (GS), can be useful for a better understanding and treatment of hydrocephalus in disorders with CSF overproduction. CASE DESCRIPTION: A 1-year-old patient was diagnosed with communicating hydrocephalus; ventricle peritoneal shunt (VPS) is installed and ascites developed. VPS is exposed, yielding volumes of 1000-1200ml/day CSF per day. MRI is performed showing generalized choroidal plexus hyperplasia. Bilateral endoscopic coagulation of thechoroid plexus was performed in 2 stages (CPC) however the high rate of CSF production persisted, needing a bilateral plexectomy through septostomy, which finally decreased the CSF outflow. DISCUSSION: New knowledge about the CSF physiology will help to propose better treatment depending on the cause of the hydrocephalus. The GS is becoming an additional reason to better study and develop new therapies focused of the modulation of alternative CSF reabsorption. CONCLUSION: Despite the current knowledge about hydrocephalus, we remain without a complete understanding of the pathophysiology of this condition. GS could be more important than conventional concept of reabsorption of CSF in the arachnoid villi, therefore GS could be a new key point, which will guide future investigations.

Primary intracranial smooth muscle tumor associated with Epstein-Barr virus in immunosuppressed children: two cases report and review of literature.

Primary intracranial smooth muscle tumors are rare. Most cases are related to Epstein-Barr virus proliferation in immunocompromised patients such as organ solid recipients. Only a few cases have been reported in pediatric patients. The clinical features are very variable depending mainly on the location and size of the smooth muscle tumor (SMT) and the pathogenesis is poorly understood. We describe two cases of intracranial SMT localized in the temporal lobe and associated with EBV in immunosuppressed children. A review of the literature associated with intracranial leiomyomas was also done.