Endoscopic resection of an intraventricular cavernoma: a case report.

Cerebral cavernous malformations occur in 0.5% of the population. They consist of thin-walled vessels and can be found as congenital or sporadic lesions. Most of them are asymptomatic, however, due to their anatomical features blood leakage into the surrounding tissue can cause severe neurological symptoms. Although risk of bleeding is low, symptomatic lesions should be treated, with microsurgical resection being the therapy of choice for surgically accessible cavernomas. Intraventricular cavernous malformations are a rare subtype, and due to their anatomical localization, they are eligible for endoscopic surgery. However, there are only a few reports on endoscopic resection of intraventricular cavernomas to be found in the literature. We report the case of a 48-year-old woman who suffers from multiple cerebral cavernous malformations. Since the first diagnosis, several of these cavernomas had been removed in open microsurgical interventions. Most recently, a new lesion arose intraventricularly, adjacent to the ependymal wall of the right lateral ventricle. In follow-up, cranial MR imaging microbleeding and an increasing size were detected. Eventually, the lesion was endoscopically removed. Presurgery the patient suffered from right-sided sensibility loss and gait disturbances as a consequence of prior surgeries. Postsurgery, no new neurological symptoms could be found. We here present MR images and intraoperative pictures as well as a short video of the resection itself. In our opinion, endoscopic resection of intraventricular cavernomas should be considered in selected cases.

Decreased microglial numbers in Vav1-Cre+:dicer knock-out mice suggest a second source of microglia beyond yolk sac macrophages.

Microglia represent the resident macrophages of the central nervous system (CNS). While it is clear that microglia recruitment is established by differentiation of primitive yolk sac (YS) macrophages and consecutive invasion of the brain, starting around E8 in rodents (Ginhoux et al., 2010), more recent studies suggest that a non-YS contribution to the microglia population should not entirely be dismissed (Swinnen et al., 2013; Xu et al., 2015). Therefore, we used Vav1-Cre+:dicer knock-out mice in order to study the effect of the post-YS hematopoiesis on the definitive microglial population in late prenatal (E16.5, E18.5) and early postnatal brains (P0, P1). Since Vav1 is thereby exclusively expressed in hematopoietic cells starting at E11, the depletion of the micro RNA processing enzyme dicer in Vav1-positive cells allows interfering with post-YS microglia recruitment. Using this approach, analysis of the number of Iba-1 positive microglia revealed a reduction of microglial numbers by 40% in knock-out mice at P1 compared to their individual control littermates. Noteworthy, immunolabeling for Ki-67 and active caspase 3 confirmed that the differences in the microglial numbers are not related to differential rates of proliferation or apoptosis. Therefore, our data demonstrates that interfering with the definitive hematopoiesis highly impacts on the microglial population, implicating an important role of post-YS hematopoiesis on microglial development and recruitment.

Late-onset hydrocephalus in a child with Joubert syndrome: a case report.

INTRODUCTION: The ciliopathy "Joubert syndrome" was first described in 1969 by Dr. Marie Joubert and most subtypes follow an autosomal recessive inheritance. The complex disorder shows typical clinical features, such as hyperventilation, abnormal eye movements, and retardation. A pathognomonic midbrain-hindbrain malformation, the molar tooth sign, can be found on magnetic resonance imaging of the brainstem. There are a little more than 200 reports of Joubert syndrome in the literature. CASE PRESENTATION: We report a case of a 9-year-old boy who developed a progressive hydrocephalus starting from the age of 4. He underwent VP shunt placement at 8 years, which relieved hydrocephalus-related clinical symptoms and put development of the macrocephalus to a halt. CONCLUSION: Neonatal hydrocephalus due to the altered anatomy of the posterior fossa has been reported earlier, but to our knowledge, this is the first case of a delayed onset of hydrocephalus in a patient with Joubert syndrome.