Matrix metalloproteinases (MMPs) are involved in the pathogenesis of neuroinflammatory diseases (such as multiple sclerosis) as well as in the expansion of malignant gliomas because they facilitate penetration of anatomical barriers (such as the glia limitans) and migration within the neuropil. This review elucidates pathomechanisms and summarizes the current knowledge of the involvement of MMPs in neuroinflammation and glioma, invasion highlighting microglia as major sources of MMPs. The induction of expression, suppression, and multiple pathways of function of MMPs in these scenarios will also be discussed. Understanding the induction and action of MMPs might provide valuable information and reveal attractive targets for future therapeutic strategies.

A naturally short sleeper phenotype with a sleep need of less than 6 hours without negative impact on health or performance is rare. We present a case of an acquired short sleeper phenotype after third ventriculostomy. A 59-year-old patient suffering from chronic hydrocephalus reported an average of 7-8 h of nocturnal sleep. After surgical intervention, the patient noted a strikingly reduced sleep need of 4-5 h without consequent fatigue or excessive daytime sleepiness, but with good daytime performance and well-balanced mood. C A S E R E P O R T SA population-based study of sleep habits in 110,441 noninstitutionalized US adults shows an average self-reported sleep duration longer than 6 hours in more than 90%.1 Habitual sleep shorter than 6 hours per day is associated with unfavorable health and performance status.2 However, there is a small population of naturally short sleepers with a daily sleep need less than 6 hours without any subjective or objective consequences. Familial accumulation of a short sleeper phenotype led to the discovery of genes involved in the regulation of individual sleep need. 3 We present a case of a patient developing short sleeper phenotype after third ventriculostomy. REPORT OF CASEA 59-year-old man with a history of vertigo and gait unsteadiness for about 2 years was diagnosed with chronic internal hydrocephalus. Cranial MRI showed several cystic formations in the thalamic, hypothalamic, and mesencephalic region ( Figure 1A, 1B). Lumbar puncture revealed cerebrospinal fl uid pressure of 21-22 mm Hg within the upper normal range and increased protein level (680 mg/L), but no signs of infl ammation. Detailed serologic analyses including echinococcosis and other parasitic infections were negative. Eventually the MRI pattern was interpreted as congenital ectopic ependymal cysts leading to chronic hydrocephalus and increased protein in the context of a presumably reduced CSF volume turnover in the context of hydrocephalus.An endoscopic third ventriculostomy was performed due to progression of gait disturbance. Neuronavigation allowed performing a right frontal trajectory to the widened fl oor of the third ventricle posterior to the hypophysial recess and anterior to the basilar artery ( Figure 1C) signs of decreased pressure both supratentorially ( Figure 1C) and locally around the anterior third ventricle ( Figure 1D). A biopsy of the cystic formations was not performed to conserve critical structures. After intervention, vertigo and gait disturbance remitted and neurological examination was normal. However, the patient reported that he was unable to sleep more than 4-5 h per night. In the family, no short sleepers were known. Before third ventriculostomy, he had slept 7-8 h per 24 h since early adulthood without complaining of excessive daytime sleepiness. Interestingly, any negative effects of short sleep on physical or cognitive performance were denied and confi rmed by neuropsychological testing. In contrast, a remarkable postoperative improvement of a formerly depressed mood was ...

Involvement of the central nervous system (CNS) is rarely described in malignant mixed Mül-lerian tumors (MMMTs). Only four intracranial and two spinal cases have been published to date. Here we report two more cases with heterogeneous clinical, radiologic and pathologic features and summarize the available contemporary literature. One patient presented with aphasia due to an intra-axial contrastenhanced left temporal lesion with marked perifocal edema. After surgical resection, histology showed collections of small uniform tumor cells embedded in a myxoid matrix and compartmentalized by connective tissue septations, consistent with an MMMT. The other patient presented with trigeminal/tongue hypesthesia and double vision accompanied by left radiculopathy and paresis. Magnetic resonance imaging MRI revealed an extraaxial lesion at the petrous tip with mild perifocal edema and multiple small intradural contrast-enhancing lesions of the conus and cauda medullaris. Histologic examination of the intracranial lesion showed a mainly papillary architecture, also consistent with MMMTs. The spinal lesions were not excised, and both patients received adjuvant radiochemotherapy. The first patient died 3 months and the second patient 12 months after surgery. As illustrated by the heterogeneous clinicopathologic features of our two cases as well as the reviewed literature, CNS metastasis of MMMTs is diagnostically challenging, shows a variable outcome, and thus requires individualized treatment. In the present cases and CNS metastases reported to date, a higher histologic ratio of sarcomatous to epithelial components portends a worse outcome. Histological examination of the intracranial lesion showed a mainly papillary architecture, also consistent with MMMT. The spinal lesions were not excised and both patients received adjuvant radiochemotherapy. The first patient died 3 months and the second patient 12 months after surgery.As illustrated by the heterogeneous clinicopathological features of our 2 cases as well as the reviewed literature, CNS metastasis of MMMT are diagnostically challenging, show a variable outcome, and thus require individualized treatment. In the present cases and CNS metastases reported to date, a higher histological ratio of sarcomatous to epithelial components portends a worse outcome.

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