Idiopathic spinal intradural arachnoid cysts can present with neurological deficits in children. Pathologies are predominantly located in the cervical spine anteriorly and in thoracic and thoracolumbar segments posteriorly to the spinal cord. In symptomatic cases, microsurgical excision and cyst wall fenestration via laminotomy are recommended. Our radiological, intraoperative, and pathological findings support the cerebrospinal fluid obstruction and vent mechanism theory of arachnoid cysts.

Mass spectrometric analysis of glioblastoma cyst fluids has disclosed a protein peak with m/z 6424–6433. Among the proteins, potentially generating this peak are ApoC1 and LuzP6. To further elucidate protein expression of glioblastoma cells, we analyzed MALDI–TOF results of cyst fluid, performed immunohistochemistry and mRNA analysis. MALDI–TOF protein extraction from 24 glioblastoma cyst fluids was performed with a weak cation exchange. 50 glioblastoma samples were stained with two custom-made antibodies against LuzP6 and commercial antibodies against ApoC1, C12orf75 and OCC-1 and analyzed. For mRNA detection, 16 tissue samples were stored in RNAlater, extracted using the miRNeasy kit and reversely transcribed. For 12 patients, synopsis of results from all three examinations was possible. MALDI–TOF confirmed the peak at 6433 Da in 75% of samples. Immunohistochemically, LuzP6 was detected in 92% (LuzP61–29) and 96% (LuzP630–58) of samples and ApoC1 in 66%. Mean mRNA levels were highest for ApoC1, followed by LuzP6. No correlation between mRNA expression, immunohistochemical staining and intensity of the MALDI–TOF peaks was found. An unequivocal identification of one protein as the source for the 6433 peak is not possible, but our results point to ApoC1 and LuzP6 as the underlying proteins.Electronic supplementary materialThe online version of this article (10.1007/s00795-019-00223-8) contains supplementary material, which is available to authorized users.

A case of an 8-year-old-boy with shunt-dependent occlusive hydrocephalus after resection of a cerebellar medulloblastoma is presented, who experienced repeated episodes of severe neurologic deterioration with signs and symptoms of raised intracranial pressure after spinal tapping. However, intracranial pressure was recorded within low ranges, only up to the opening pressure of the implanted adjustable shunt valve. Multiple shunt revisions were performed, until the condition was recognized as acute normal pressure hydrocephalus. Either enforced recumbency and downadjustment of the valve system to 0 cm H(2)O alone or external ventricular drainage seems to be successful to resolve the critical condition, depending on severity of the symptoms. The case illustrates that acute pathologic enlargement of the ventricular system is not always associated with increased intracranial pressure, even when typical signs and symptoms are present. The very rare entity of acute normal pressure hydrocephalus by two separated compartments is postulated based on the pulsatile vector force theory of brain water circulation.

Spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) leakage causes substantial disease burden. In many patients, the course is protracted and refractory to conservative treatment, requiring targeted therapy. We propose positron emission tomography (PET) of the CSF space with 68 Ga-DOTA as a state-of-the-art approach of radionuclide cisternography (RC) and validate its diagnostic value.Methods: Retrospective analysis of patients with suspected intracranial hypotension due to spinal CSF leaks who underwent whole-body PET/CT at 1, 3 and 5 hours after intrathecal lumbar injection of 68 Ga-DOTA.Two independent raters blinded to clinical data analyzed all scans by for direct and indirect RC signs of CSF leakage. Volume of interest analysis was performed to assess the biological half-life of the tracer in CSF space (T 1/2,biol ) and the ratio of decay-corrected activity in CSF space at 5 and 3 hours (R5/3; simplified marker of tracer clearance). Comprehensive stepwise neuroradiological work-up served as reference, which was additionally validated by surgical findings and follow-up.Results: Of 40 consecutive patients, 39 patients with a working diagnosis of intracranial hypotension due to a spinal CSF leak (n=31 spontaneous and n=8 post-interventional) could be analyzed. A spinal CSF leak was verified by the neuroradiological reference method in 18 of 39 patients. As the only direct and indirect diagnostic signs, extrathecal tracer accumulation at the cervicothoracic junction and lack of activity over the cerebral convexities (5h) showed a high diagnostic value for spinal CSF leaks (sensitivity/specificity: 67%/90% and 94%/67%, respectively). Their combination provided little improvement (71%/95%).

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