Our data show that neural tube defects to this day can remain undetected despite medical care during pregnancy. The most common associated diseases with MMC are Chiari II malformations and hydrocephalus. In the seven cases of simultaneous repair of MMC with shunt implantation, no additional complications were encountered. An interdisciplinary approach was allowed in a high percentage independence and social continence.

Background:Primary intraosseous cavernous hemangiomas (PICH) of the skull represent an infrequent bone tumor. Although some rare cases of PICHs located in the skull base have been published, to our concern only three cases have been reported in the English literature of PICHs arising within the clivus.Case Description:We present the case of a patient presenting an isolated abducens paresis due to a rare PICH of the clivus showing also an unusual destruction of the inner table as well as infiltration of the dura mater. Due to this uncommon infiltrative pattern of an otherwise expected intraosseous tumor, a cerebrospinal fluid (CSF)-fistula occurred while performing a transnasal biopsy. The patient recovered successfully without need of lumbar drainage or re-surgery. Additionally, intratumoral decompression was sufficient to relief the abducens paresis.Conclusions:Our case provides new and meaningful information about clinical features as well as growth pattern of these rare clival tumors. We also discuss the importance of knowing these peculiarities before surgery in order to plan the optimal operative management as well as to avoid complications while approaching PICHs localized in such a delicate cranial region.

Due to its low incidence in Western countries, physician awareness of juvenile Moyamoya disease should be improved. The benefits of revascularisation surgery have only been proven in the juvenile version of the disease. Therefore, early revascularisation may prevent irreversible ischaemic deficits and rapidly progressive mental retardation in young patients.From 1984 to 2009, a total of 19 children (mean age 8 years, range 1-18 years, female predominance 2:1, 17/19 European white patients, 2/19 Asian origin of at least one parent) were treated for juvenile Moyamoya disease by surgical revascularisation. The leading symptoms were epilepsy (17/19), followed by transient ischaemic attacks (TIA) or prolonged reversible ischaemic neurologic deficits (PRIND) (15/19) and mental retardation (11/19). Angiography showed a clear neovascularisation in the majority of patients after indirect bypasses after 6 months. The mean follow-up was 17 years and 3 months (maximum 25 years, minimum 2 years). Two patients were lost to follow-up. In accordance with the literature, ischaemic symptoms were eliminated by the revascularisation operation in 94% of our patients with a very low rate of complications, and no lasting morbidity and mortality in any of the patients.Early diagnosis and surgical treatment seem to potentiate the benefits independently of the type of revascularisation procedure.

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Intracranial glioblastoma multiforme (GBM) constitutes the most frequent and unfortunately aggressive primary central nervous system malignancy. Despite the high tendency of these tumors to show local relapse within the brain after primary therapy, dissemination into the spinal axis is an infrequent event. If spinal metastases occur they are leptomeningeal in the vast majority of cases and always in the context of intracranial progressive disease. Spinal intramedullary metastases of intracranial GBM have rarely been described to date. We report the unique case of a young woman with subacute progressive paraparesis due to spinal intramedullary metastases of a temporal lobe GBM despite the remarkable absence of intracranial tumor relapse. The patient had undergone gross total resection of a left temporal GBM in contact with the ventricles and cisternal space followed by radio- and chemotherapy 13 months before. At the moment of diagnosis of spinal intramedullary metastases, there were no signs of intracranial tumor recurrence as revealed by MRI scans. Since a high level of suspicion may be needed to detect this rare evolution of intracranial GBM and other differential diagnoses must be ruled out at presentation, we discuss the important features of this case regarding clinical manifestation, diagnosis, surgery, and management. Furthermore, we mention possible factors that may have contributed to the development of these metastases in the context of intracranial remission.