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Background and Purpose The diagnostic work-up in patients with spontaneous intracranial hypotension (SIH) and spinal longitudinal extradural CSF collection (SLEC) on magnetic resonance imaging (MRI) comprises dynamic digital subtraction myelography (dDSM) in prone position for leak detection. Dynamic computed tomography (CT) myelography (dCT-M) in prone position follows if the leak is not unequivocally located. A drawback of dCT‑M is a high radiation dose. This study evaluates the diagnostic needs of dCT-M examinations and measures to reduce radiation doses. Methods Frequency, leak sites, length and number of spiral acquisitions, DLP and effective doses of dCT‑M were retrospectively recorded in patients with ventral dural tears. Results Of 42 patients with ventral dural tears, 8 underwent 11 dCT‑M when the leak was not unequivocally shown on digital subtraction myelography. The median number of spiral acquisitions was 4 (range 3–7) and the mean effective radiation dose 30.6 mSv (range 13.1–62.16 mSv) mSv. Five of eight leaks were located in the upper thoracic spine (range C7/Th1–Th2/3). Bolus tracking of intrathecal contrast agent in dCT‑M was used to limit the number and length of spiral acquisitions. Discussion A dCT‑M in prone position to localize a ventral dural tear is needed in every fifth patient with a SLEC on MRI. It is typically needed when the leak is located in the upper thoracic spine and when patients have broad shoulders. Measures to reduce the radiation dose include bolus tracking or to repeat the DSM with adjusted positioning of patient.

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.

OBJECTIVE Spinal CSF leaks cause spontaneous intracranial hypotension (SIH). Surgical closure of spinal CSF leaks is the treatment of choice for persisting leaks. Surgical approaches vary, and there are no studies in which minimally invasive techniques were used. In this study, the authors aimed to detail the safety and feasibility of minimally invasive microsurgical sealing of spinal CSF leaks using nonexpandable tubular retractors. METHODS Consecutive patients with SIH and a confirmed spinal CSF leak treated at a single institution between April 2019 and December 2020 were included in the study. Surgery was performed via a dorsal 2.5-cm skin incision using nonexpandable tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach. The primary outcome was successful sealing of the dura, and the secondary outcome was the occurrence of complications. RESULTS Fifty-eight patients, 65.5% of whom were female (median age 46 years [IQR 36–55 years]), with 38 ventral leaks, 17 lateral leaks, and 2 CSF venous fistulas were included. In 56 (96.6%) patients, the leak could be closed, and in 2 (3.4%) patients the leak was missed because of misinterpretation of the imaging studies. One of these patients underwent successful reoperation, and the other patient decided to undergo surgery at another institution. Two other patients had to undergo reoperation because of insufficient closure and a persisting leak. The rate of permanent neurological deficit was 1.7%, the revision rate for a persisting or recurring leak was 3.4%, and the overall revision rate was 10.3%. The rate of successful sealing during the primary closure attempt was 96.6% and 3.4% patients needed a secondary attempt. Clinical short-term outcome at discharge was unchanged in 14 patients and improved in 25 patients, and 19 patients had signs of rebound intracranial hypertension. CONCLUSIONS Minimally invasive surgery with tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach is safe and effective for the treatment of spinal CSF leaks. The authors suggest performing a minimally invasive closure of spinal CSF leaks in specialized centers.

CSF-venous fistulas — initially described in 2014 — are a significant cause of spontaneous intracranial hypotension. Dynamic lateral decubitus digital subtraction and/or CT myelography typically show a hyperdense paraspinal vein. In case of a central drainage toward the internal vertebral venous plexus, it is the “hyperdense basivertebral vein” which should be searched for carefully.

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.

The aim of this study was to evaluate whether ganglioglioma (GGL), dysembryoplastic neuroepithelial tumour (DNET) and FCD (focal cortical dysplasia) are distinguishable through diffusion tensor imaging. Additionally, it was investigated whether the diffusion measures differed in the perilesional (pNAWM) and in the contralateral normal appearing white matter (cNAWM). Six GGLs, eight DNETs and seven FCDs were included in this study. Quantitative diffusion measures, that is, axial, radial and mean diffusivity and fractional anisotropy, were determined in the lesion identified on isotropic T2 or FLAIR-weighted images and in pNAWM and cNAWM, respectively. DNET differed from FCD in mean diffusivity, and GGL from FCD in radial diffusivity. Both types of glioneuronal tumours were different from pNAWM in fractional anisotropy and radial diffusivity. For identifying the tumour edges, threshold values for tumour-free tissue were investigated with receiver operating characteristic analyses: tumour could be separated from pNAWM at a threshold ≤ 0.32 (fractional anisotropy) or ≥ 0.56 (radial diffusivity) *10–3 mm2/s (area under the curve 0.995 and 0.990 respectively). While diffusion parameters of FCDs differed from cNAWM (radial diffusivity (*10–3 mm/s2): 0.74 ± 0.19 vs. 0.43 ± 0.05; corrected p-value

ZusammenfassungDieser Übersichtsartikel ist speziell auf den praktisch arbeitenden Ophthalmologen zugeschnitten. Im Fokus soll der Umgang mit der Kernspintomografie im Allgemeinen und speziell in der Anwendung/Abklärung von Erkrankungen stehen, welche die Sehbahn beeinträchtigen. Übersichtlich geben mehrere Tabellen Auskunft über die Anwendung von verschiedenen MRT-Protokollen im Bereich der Sehbahn, Eigenschaften häufig angewendeter MRT-Sequenzen sowie häufige Krankheitsbilder in den jeweiligen Abschnitten der Sehbahn. Anhand einzelner ausgewählter Erkrankungen soll die Anwendung und Interpretation der MRT-Bildgebung exemplarisch dargestellt werden. Aufgrund der enormen Vielfalt an Erkrankungen, die Einfluss auf die Sehbahn haben können, kann und will dieser Artikel keinen Anspruch auf Vollständigkeit erheben, sondern vielmehr dem Leser die MRT im Bereich der Sehbahn näherbringen und praktische „Werkzeuge“ für den Alltag (in Form von Tabellen) an die Hand geben. Zudem wird aus aktuellem Anlass auf die laufende Diskussion (Gadolinium-Retention im Gehirn) über den Umgang mit Gadolinium eingegangen.

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.

This overview is especially intended for practitioners in ophthalmology. It focuses on how to handle diseases affecting the optic pathway with MRI in general and in particular. Several tables accompany this article, in order to illustrate the different MRI scanning protocols, properties of frequently used MRI sequences, as well as common pathologies to be found in the optic pathway. With the help of exemplary cases, the application and interpretation of MRI imaging will be shown. There is a tremendous variety of diseases influencing the optic pathway - which is why this article does not intend to give a complete representation of this medical issue, but rather focuses on giving hands-on guidance (through multiple tables) for daily routine. Moreover, the article also addresses the issue of retained gadolinium in the brain.Dieser Übersichtsartikel ist speziell auf den praktisch arbeitenden Ophthalmologen zugeschnitten. Im Fokus soll der Umgang mit der Kernspintomografie im Allgemeinen und speziell in der Anwendung/Abklärung von Erkrankungen stehen, welche die Sehbahn beeinträchtigen. Übersichtlich geben mehrere Tabellen Auskunft über die Anwendung von verschiedenen MRT-Protokollen im Bereich der Sehbahn, Eigenschaften häufig angewendeter MRT-Sequenzen sowie häufige Krankheitsbilder in den jeweiligen Abschnitten der Sehbahn. Anhand einzelner ausgewählter Erkrankungen soll die Anwendung und Interpretation der MRT-Bildgebung exemplarisch dargestellt werden. Aufgrund der enormen Vielfalt an Erkrankungen, die Einfluss auf die Sehbahn haben können, kann und will dieser Artikel keinen Anspruch auf Vollständigkeit erheben, sondern vielmehr dem Leser die MRT im Bereich der Sehbahn näherbringen und praktische „Werkzeuge“ für den Alltag (in Form von Tabellen) an die Hand geben. Zudem wird aus aktuellem Anlass auf die laufende Diskussion (Gadolinium-Retention im Gehirn) über den Umgang mit Gadolinium eingegangen.

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.

Background Propionibacterium acnes was found in lungs and lymph nodes of patients with sarcoidosis and may induce hypersensitivity type granuloma formation. Data regarding the immune response to P. acnes of European sarcoid patients are scarce. Methods We assessed the total IgG and IgA amount and specific antibodies to P. acnes and to Staphylococcus aureus, serving as a control, in BAL fluid of 64 patients with sarcoidosis and of 21 healthy volunteers. In a subcohort of sarcoid patients and controls, TNF-α and GM-CSF production of BAL cells stimulated with heat-killed P. acnes were measured. Results In sarcoid patients, the total IgG and IgA levels in BAL fluid were significantly elevated compared to healthy volunteers. IgG and IgA titres against P. acnes and S. aureus were increased in sarcoid patients, yet based on the total amount of antibodies, only antibodies directed against P. acnes were relatively and significantly increased. Furthermore, BAL cells of sarcoid patients produced significantly more TNF-α and GM-CSF upon stimulation with heat-killed P. acnes compared to controls. Conclusions Patients with sarcoidosis had elevated levels of specific antibodies against P. acnes which suggest contact with this bacterium in the past. Furthermore, BAL cells of sarcoid patients produced inflammatory cytokines (TNF-α and GM-CSF) upon stimulation with P. acnes indicating potential involvement of this pathogen in the pathogenesis of sarcoidosis in some patients.

Background Autoimmune and inflammatory mechanisms in psychotic disorders have attracted increasing scientific attention in recent years. In this regard, we performed routine cerebrospinal fluid (CSF) basic diagnostics and CSF/serum analyses for antibodies directed against neuronal intracellular and surface antigens in psychotic patients. In this context, the patient presented in this paper was diagnosed. Case presentation We present the case of a 20-year-old female patient with a first episode of a drug-induced psychotic syndrome but without neurological deficits. Further investigations showed a reproducible low-titre positive anti-Yo reactivity in the CSF and serum with two independent immunoblot assays. Magnetic resonance imaging showed frontoparietal and cerebellar atrophy. On [18F]fluorodeoxyglucose positron emission tomography, a mild cerebellar hypometabolism was found. No underlying tumor was detected. Conclusion Despite the presence of anti-Yo reactivity, the diagnostic criteria for a paraneoplastic neurological syndrome were not fulfilled. Previously published data indicate the possible association between low-titer antibodies against intracellular localized, onconeural antigens, and psychotic disorders. Large prospective studies that investigate the prevalence and clinical significance of antibodies against intracellular onconeural antigens in psychiatry are needed.

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.