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Background The purpose of this study was to analyse less known clinical scenarios associated with idiopathic intracranial hypertension. Methods The study involved analysis of magnetic resonance imaging signs of idiopathic intracranial hypertension in patients with spontaneous rhinoliquorrhoea ( n = 7), in patients with temporal lobe epilepsy and surgically treated antero-inferior temporal lobe meningo-encephaloceles (n = 15), and in patients who developed clinical signs of idiopathic intracranial hypertension following the treatment of spontaneous intracranial hypotension ( n = 7). Results Three of six patients with spontaneous rhinoliquorrhoea and six of 15 operated patients with temporal lobe epilepsy due to temporal lobe meningo-encephaloceles showed magnetic resonance imaging signs of idiopathic intracranial hypertension and had a body mass index >30 kg/m2. Rebound high pressure headaches and sings of idiopathic intracranial hypertension occurred in seven of 44 surgically treated spontaneous intracranial hypotension patients. Conclusions Magnetic resonance imaging findings should guide the clinician to consider (idiopathic) intracranial hypertension when patients develop spontaneous rhinoliquorrhoea, temporal lobe epilepsy secondary to temporal lobe meningoencephaloceles or high pressure headaches in spontaneous intracranial hypotension. Whether idiopathic intracranial hypertension must be regarded as a differential diagnosis or as a cause, or whether there are common pathophysiological pathways that lead to signs of idiopathic intracranial hypertension in this wider spectrum of disease is the focus of further study.

Glioblastoma multiforme might develop radiologically within a few days following unremarkable CT scan of the brain. Glioblastoma multiforme is the most frequent primary brain tumor. Initial presentations are diverse, including headache, seizures and transient or persistent neurological deficits. Cerebral imaging followed by histological examination of a tissue specimen is the mainstay of diagnosis. We report the case of a 79-year-old female patient whose computer tomography (CT) of the brain was unremarkable at first clinical presentation with a transient hemiparesis of the right side, but revealed a cerebral space-occupying lesion ultimately diagnosed as glioblastoma only 10 days later. According to our case presentation glioblastoma might develop radiologically within a few days following unremarkable CT scan of the brain. Since clinical manifestation with a transient ischemic attack (TIA)-like episode was preceding CT manifestation, this case indicates, that a magnetic resonance imaging (MRI) should be routinely performed in all patients presenting with TIA.

Background and Purpose To develop a fully automatic algorithm for the magnetic resonance imaging (MRI) identification of patients with spontaneous intracranial hypotension (SIH). Material and Methods A support vector machine (SVM) was trained with structured reports of 140 patients with clinically suspected SIH. Venous sinuses and basal cisterns were segmented on contrast-enhanced T1-weighted MPRAGE (Magnetization Prepared-Rapid Gradient Echo) sequences using a convolutional neural network (CNN). For the segmented sinuses and cisterns, 56 radiomic features were extracted, which served as input data for the SVM. The algorithm was validated with an independent cohort of 34 patients with proven cerebrospinal fluid (CSF) leaks and 27 patients who had MPRAGE scans for unrelated reasons. Results The venous sinuses and the suprasellar cistern had the best discriminative power to separate SIH and non-SIH patients. On a combined score with 2 points, mean SVM score was 1.41 (±0.60) for the SIH and 0.30 (±0.53) for the non-SIH patients (p Conclusion A fully automatic algorithm analyzing a single MRI sequence separates SIH and non-SIH patients with a high diagnostic accuracy. It may help to consider the need of invasive diagnostics and transfer to a SIH center.

Background Spontaneous intracranial hypotension (SIH) is an underdiagnosed disease. Its incidence is estimated at 5 per 100 000 persons per year. Methods This review is based on a selective literature search in PubMed covering the years 2000-2019, as well as on the authors' personal experience. Results The diagnostic and therapeutic methods discussed here are supported by level 4 evidence. SIH is caused by spinal leakage of cerebrospinal fluid (CSF) out of ventral dural tears or nerve root diverticula, or, in 2-5% of cases, through a fistula leading directly into the periradicular veins (CSF-venous fistula). In half of all patients, no CSF leak is demonstrable. A low CSF opening pressure on lumbar puncture is present in only one-third of patients; imaging studies are thus needed to confirm and localize a spinal CSF leak. Half of all patients in whom myelographic computed tomography (CT) reveals contrast medium reaching the epidural space have ventral dural tears, which tend to be located at upper thoracic spinal levels. Epidural blood patches applied under fluoroscopic or CT guidance can seal the CSF leak in 30-70% of patients, but 90% of patients with ventral dural tears will need operative closure. Some patients who have no visible epidural contrast medium on CT presumably do not have SIH, while others do, in fact, have a CSF leak from a diverticulum or a CSF-venous fistula and will need to have the site of the leak demonstrated with the aid of further studies, such as dynamic (subtraction) myelography in the lateral decubitus position. Conclusion The management of patients with SIH calls for complementary imaging studies to demonstrate the causative spinal CSF leak. Often, successful treatment requires surgical closure of the leak. In view of the sparse evidence available to date, controlled studies should be performed.

Intradural extramedullary cystic mass lesions of the craniocervical junction with an anterior location are very rare. These pathologies are typically accessed via wide, open approaches, which can result in access-related sequelae such as local pain or instability. We present a dedicated posterior minimally invasive approach via a non-expandable 20 mm diameter tubular retractor for complete removal of intradural lesions anterior to the myelon. A 71-year-old female patient with increasing gait disturbances and local pain yielded a retrodental cystic lesion with severe compression of the myelon from anteriorly. Minimally invasive surgical resection was performed from posterior via a non-expandable tubular retractor. The cystic mass lesion turned out to be located completely intradural extramedullary. The patient fully recovered from all symptoms and reported no local pain while maintaining full head and neck mobility. Postoperative imaging confirmed complete removal of the benign cystic lesion and decompression of the myelon. Anteriorly located extramedullary mass lesions can be resected with appropriate expertise using a minimally invasive posterior approach with a non-expandable tubular retractor, especially if they are predominantly unilaterally localized. The presented benign cystic lesion was successfully resected, resulting in a resolution of the patient's symptoms while maintaining full mobility of the head and neck.

The identification of pathological atrophy in MRI scans requires specialized training, which is scarce outside dedicated centers. We sought to investigate the clinical usefulness of computer-generated representations of local grey matter (GM) loss or increased volume of cerebral fluids (CSF) as normalized deviations (z-scores) from healthy aging to either aid human visual readings or directly detect pathological atrophy. Two experienced neuroradiologists rated atrophy in 30 patients with Alzheimer's disease (AD), 30 patients with frontotemporal dementia (FTD), 30 with dementia due to Lewy-body disease (LBD) and 30 healthy controls (HC) on a three-point scale in 10 anatomical regions as reference gold standard. Seven raters, varying in their experience with MRI diagnostics rated all cases on the same scale once with and once without computer-generated volume deviation maps that were overlaid on anatomical slices. In addition, we investigated the predictive value of the computer generated deviation maps on their own for the detection of atrophy as identified by the gold standard raters. Inter and intra-rater agreements of the two gold standard raters were substantial (Cohen's kappa κ > 0.62). The intra-rater agreement of the other raters ranged from fair (κ = 0.37) to substantial (κ = 0.72) and improved on average by 0.13 (0.57, Highlights • The visual identification of atrophy is most accurate in the temporal lobe. • Voxel-wise deviations of tissue volume from normal aging is easy to implement. • Displaying voxel-wise deviations subjectively but not objectively aids readers. • Voxel-wise deviations themselves show high agreement with expert readings. • Information on tissue deviations should be provided with cerebral MRI scans.

Rationale: Previous studies suggest an important immunoregulatory role of vasoactive intestinal peptide (VIP) in experimental models of chronic noninfectious inflammation. Sarcoidosis is characterized by noncaseating epitheloid cell granulomas, where excessive tumor necrosis factor-α production by pulmonary macrophages plays a critical role in granuloma formation and disease progression, which may lead to fatal organ dysfunction. Objectives: To test whether inhaled VIP has an immunoregulatory role. Sarcoid alveolitis was used as a prototype of immune-mediated chronic lung inflammation. Methods: In an open clinical phase II study, we treated 20 patients with histologically proved sarcoidosis and active disease with nebulized VIP for 4 weeks. Measurements and Main Results: VIP inhalation was safe, well-tolerated, and significantly reduced the production of tumor necrosis factor-α by cells isolated from bronchoalveolar lavage fluids of these patients. VIP treatment significantly increased the numbers of bronchoalveolar lavage CD4 +CD127-CD25+ T cells, which showed regulatory activities on conventional effector T cells. In vitro experiments demonstrated the capacity of VIP to convert naive CD4+CD25- T cells into CD4+CD25+FoxP3+ regulatory T cells, suggesting the generation of peripheral regulatory T cells by VIP treatment. Conclusions: This study is the first to show the immunoregulatory effect of VIP in humans, and supports the notion of inhaled VIP as an attractive future therapy to dampen exaggerated immune responses in lung disorders. Thus, the inhalation of neuropeptides may be developed into a new therapeutic principle for chronic inflammatory lung disorders in humans.

Sporadic and hereditary forms of renal cell carcinoma (RCC), von Hippel-Lindau (VHL) disease and the familial paraganglioma syndromes are closely related in terms of their clinical, molecular, and genetic aspects. Most RCCs occur sporadically and the heritable fraction of RCC is estimated to be just 2-4%. An understanding of the molecular genetic basis, the disease-specific and gene-specific biology and the clinical characteristics of these cancer syndromes is of utmost importance for effective genetic diagnosis and appropriate treatment. In addition, such insight will improve our understanding of sporadic RCCs. To date, 10 different heritable RCC syndromes have been described. VHL syndrome is the oldest known hereditary RCC syndrome. Similar to VHL disease, phaeochromocytoma is a major manifestation of the paraganglioma syndromes types 1, 3 and 4 in which RCCs have been reported. These syndromes are therefore regarded as VHL-related disorders and are included in this Review. Multifocal tumours, bilateral occurrence, a young age at diagnosis and/or family history are clinical red flags suggestive of hereditary disease and should trigger referral for genetic and molecular analysis. The identification of an underlying genetic alteration enables gene-specific risk assessment and opens up the possibility of a tailored follow-up strategy and specific surveillance protocols as the basis of effective preventive medicine. The important goals of preventive medicine are to increase the life expectancy of affected patients and to improve their quality of life. The study of seemingly rare hereditary syndromes and their susceptibility genes has consistently revealed clues regarding the aetiology and pathogenesis of these diseases, and can aid diagnosis and the development of therapeutics for patients affected by much more common sporadic counterparts.