[German S1 Guideline Long-/Post-COVID]. / S1-Leitlinie Long-/Post-COVID.

The German Society of Pneumology initiated 2021 the AWMF S1 guideline Long COVID/Post-COVID. In a broad interdisciplinary approach, this S1 guideline was designed based on the current state of knowledge.The clinical recommendations describe current Long COVID/Post-COVID symptoms, diagnostic approaches, and therapies.In addition to the general and consensus introduction, a subject-specific approach was taken to summarize the current state of knowledge.The guideline has an explicit practical claim and will be developed and adapted by the author team based on the current increase in knowledge.

[S2k Guideline - Recommendations for Inpatient Therapy of Patients with COVID-19]. / S2k-Leitlinie ­ Empfehlungen zur stationären Therapie von Patienten mit COVID-19.

Since December 2019, the novel coronavirus SARS-CoV-2 (Severe Acute Respiratory Syndrome - Corona Virus-2) has been spreading rapidly in the sense of a global pandemic. This poses significant challenges for clinicians and hospitals and is placing unprecedented strain on the healthcare systems of many countries. The majority of patients with Coronavirus Disease 2019 (COVID-19) present with only mild symptoms such as cough and fever. However, about 6 % require hospitalization. Early clarification of whether inpatient and, if necessary, intensive care treatment is medically appropriate and desired by the patient is of particular importance in the pandemic. Acute hypoxemic respiratory insufficiency with dyspnea and high respiratory rate (> 30/min) usually leads to admission to the intensive care unit. Often, bilateral pulmonary infiltrates/consolidations or even pulmonary emboli are already found on imaging. As the disease progresses, some of these patients develop acute respiratory distress syndrome (ARDS). Mortality reduction of available drug therapy in severe COVID-19 disease has only been demonstrated for dexamethasone in randomized controlled trials. The main goal of supportive therapy is to ensure adequate oxygenation. In this regard, invasive ventilation and repeated prone positioning are important elements in the treatment of severely hypoxemic COVID-19 patients. Strict adherence to basic hygiene, including hand hygiene, and the correct wearing of adequate personal protective equipment are essential when handling patients. Medically necessary actions on patients that could result in aerosol formation should be performed with extreme care and preparation.

Cerebrospinal fluid findings in reversible cerebral vasoconstriction syndrome: a way to differentiate from cerebral vasculitis?

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by an acute onset of severe headache and multi-focal segmental vasoconstriction of cerebral arteries resolving within 12 weeks. Diagnostic criteria include normal or near-normal findings in cerebrospinal fluid (CSF) analysis, especially leucocyte levels < 10/mm³. Distinguishing RCVS from primary angiitis of the central nervous system (PACNS) is essential to avoid unnecessary and sometimes unfavourable immunosuppressive treatment. We reviewed retrospectively the clinical and diagnostic data of 10 RCVS patients who presented in our neurological department from 1 January 2013 to February 2017. The main purpose was to verify whether CSF leucocyte counts < 10/mm³ serve to discriminate RCVS from PACNS. Five of six patients who underwent lumbar puncture presented with CSF leucocyte levels ≥ 10/mm³. Two patients had a history of misinterpretation of CSF pleocytosis as cerebral vasculitis and of immunosuppressive treatment. A complete restitution of cerebral vasoconstriction was evident in all. No patient had further cerebral strokes or bleedings without immunosuppressive treatment over more than 12 weeks. Despite the established diagnostic criteria, RCVS can manifest with CSF leucocyte levels > 10/mm³. Careful anamnesis and the response of 'vasculitis-like angiography' to nimodipine given as a test during angiography and as oral medication are key to differentiate RCVS from cerebral vasculitis.

[Neurological Disorders and Pregnancy]. / Neurologische Erkrankungen und Schwangerschaft - eine Übersicht.

Neurological disorders caused by pregnancy and puerperium include the posterior reversible encephalopathy syndrome, the amniotic fluid embolism syndrome (AFES), the postpartum angiopathy due to reversible vasoconstriction syndrome, and the Sheehan syndrome. Hypertension and proteinuria are the hallmarks of preeclampsia, seizures define eclampsia. Hemolysis, elevated liver enzymes and low platelets constitute the HELLP syndrome. Vision disturbances including cortical blindness occur in the posterior reversible encephalopathy syndrome (PRES). The Sheehan syndrome presents with panhypopituitarism post partum due to apoplexia of the pituitary gland in severe peripartal blood loss leading to longstanding hypotension. Some neurological disorders occur during pregnancy and puerperium with an increased frequency. These include stroke, sinus thrombosis, the restless legs syndrome and peripheral nerve syndromes, especially the carpal tunnel syndrome. Chronic neurologic diseases need an interdisciplinary approach during pregnancy. Some anticonvulsants double the risk of birth defects. The highest risk exists for valproic acid, the lowest for lamotrigine and levetiracetam. For MS interval treatment, glatiramer acetate and interferones seem to be safe during pregnancy. All other drugs should be avoided.

[Cerebral amyloid angiopathy and dementia]. / Zerebrale Amyloidangiopathie und Demenz.

Cerebral amyloid angiopathy (CAA) is one of the most frequent causes of intracerebral hemorrhage (ICH). The deposition of beta amyloid leads to vascular fragility due to degeneration of vessel walls, formation of microaneurysms particularly in cortical blood vessels and fibrinoid vessel wall necrosis. The Congo red positive amyloid deposits are biochemically similar to the material comprising senile plaques in Alzheimer's disease. Recurrent or multiple simultaneous hemorrhages particularly in older patients should raise the suspicion of CAA. Gradient echo magnetic resonance imaging (MRI) is a sensitive, non-invasive technique for identifying even very small hemorrhages and superficial siderosis, which may cause transient symptoms in CAA. There is also a correlation between CAA, microbleeding and cognitive decline. Inflammatory variants of CAA must be suspected whenever patients present with progressive dementia, headache and multifocal symptoms in association with CAA findings in MRI. Histopathologically, a distinction is made between CAA-related inflammation (CAA-ri) with perivascular inflammatory infiltrates and amyloid beta-related angiitis (ABRA) with histological detection of transmural vasculitis. Inflammatory variants should be treated with corticosteroids and immunosuppressants.

Recurrent Bacterial Meningitis Perpetuated by an Infected Rathke's Cleft Cyst.

A 52-year-old woman with a 6-month history of prednisolone treatment for suspected diagnosis of myositis presented 3 months after withdrawal of steroids with headache, nuchal rigidity, fever, nausea, and vomiting. While routine blood work was unremarkable, CSF analysis was consistent with bacterial meningitis. MRI confirmed a non-enhancing pituitary cystic lesion that had been incidentally diagnosed 6 years earlier as a suspected Rathke's cleft cyst (RCC). Under the suspected diagnosis of RCC empyema, the patient underwent transsphenoidal surgery. Neuropathological examination revealed purulent material containing gram-positive cocci within a RCC.

Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls.

Cerebral vasculitis is a rare cause of juvenile stroke. It may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation in the setting of systemic vasculitis. Clinical hints for vasculitis are headache, stroke, seizures, encephalopathy and signs of a systemic inflammatory disorder. Diagnostic work-up includes anamnesis, whole body examination, laboratory and cerebral spinal fluid (CSF) studies, magnetic resonance imaging (MRI), angiography and brain biopsy. Due to the rarity of the disease, exclusion of more frequent differential diagnoses is a key element of diagnostic work -up. This review summarizes the steps that lead to the diagnosis of cerebral vasculitis and describes the red flags and pitfalls. Despite considering the dilemma of angiography-negative vasculitis and false-negative brain biopsy in some cases, it is important to protect patients from 'blind' immunosuppressive therapy in unrecognized non-inflammatory differential diagnosis.

[Painful leg paresis - a long way to diagnosis]. / Schmerzhafte einseitige Beinschwäche - der lange Weg zur Diagnose.

This 70-year-old female patient presented with a painful paresis of her left leg. After one year with several hospital stays, numerous radiological examinations, multimodal pain treatment and three surgeries performed, the diagnosis was made on the basis of a neurological examination.

[Postpartum secondary cerebral venous sinus thrombosis with axial transtentorial and tonsillar herniation as a complication of intracranial hypotension syndrome after spinal anaesthesia]. / Postpartale sekundäre Sinusvenenthrombose mit axialer transtentorieller und tonsillärer Herniation als seltene Komplikation eines Liquorunterdrucksyndroms nach spinaler Anästhesie.

We describe the case of a 28-year-old postpartum female patient who suffered from a secondary cerebral venous sinus thrombosis due to an intracranial hypotension syndrome with axial transtentorial and tonsillar herniation and the clinical signs of incarceration after spinal anaesthesia.

[Focal seizure status caused by a cerebral hyperperfusion syndrome after carotid endarterectomy]. / Status einfach-fokaler Anfälle auf dem Boden eines zerebralen Hyperperfusionssyndroms nach Karotis-Thrombendarteriektomie.

The cerebral hyperperfusion syndrome is a rare complication after revascularisation procedures of the carotid artery. It is typically presenting hours to few days after the intervention with headache, seizures and/or neurological focal symptoms. Severe complications include brain oedema or intracranial haemorrhage. We present a 74-year-old female patient who developed a cerebral hyperperfusion syndrome presenting with a status of focal motor seizures of her left arm after carotid endarterectomy of an 80 % clinically asymptomatic stenosis with a coexisting contralateral 80 % asymptomatic stenosis of the internal carotid artery.

What is the expert's option on antiplatelet therapy in moyamoya disease? Results of a worldwide Survey.

BACKGROUND AND PURPOSE: Moyamoya disease (MMD) is a rare idiopathic vasculopathy characterized by an extensive network of fine collaterals in the setting of bilateral progressive stenosis and finally occlusion of the intracranial portion of the internal carotid artery and proximal anterior and medial arteries of Circle of Willis. Owing to the rarity of the disease and ethical concerns, double-blinded, randomized controlled trials about treatment options are completely lacking. The appropriate conservative treatment instead, before or after revascularization surgery, is nearly completely neglected in scientific literature, even in Asia. METHODS: We developed a questionnaire with response options offered in the multiple choice method. The survey was sent with the request to reply within September 2010 to January 2011 by email to experts in the treatment of MMD. As an international expert, authors were selected who had written more than one scientific article or book chapter on the subject. We took special care not to select only Asian scientists, but also considered experts on the disease in Caucasian patients. RESULTS: Among the 77 physicians surveyed, 32 (41.55%) responded. Twenty-one (65.6%) Asian experts and 11 (34.4%) non-Asian experts participated in this survey. The majority of experts thought that long-term antiplatelet therapy is not essential in the treatment of MMD. Ten of 32 (31%) participants agreed to long-term antiplatelet treatment with acetylsalicylic acid 100 mg/day. There was a significant difference in answers between Asian and non-Asian experts (P = 0.0128). CONCLUSION: It is interesting that the majority of Non-Asian respondents recommend antiplatelet drugs, while this is an unusual approach in MMD of the Asian experts. Perhaps, this significant difference is well founded by the different experiences of the experts related to the difference in disease presentation between Asians and Caucasians. The role of conservative and surgical treatment in MMD needs further evaluation with larger cohorts and a focus on long-term clinical outcome.

Inheritance of moyamoya disease in a Caucasian family.

BACKGROUND AND PURPOSE: Moyamoya disease is a very rare occlusive cerebrovascular disorder characterized by progressive stenosis or occlusion of the intracranial portion of the internal carotid artery and proximal cerebral arteries with an extensive network of fine collaterals. The aetiology and genetic susceptibility of moyamoya disease, especially in Caucasians, still remains unclear. METHODS AND RESULTS: We describe the cases of affected German father, daughter and son with juvenile stroke because of idiopathic moyamoya disease. The rare existing literature is reviewed and discussed. CONCLUSIONS: This is the first report on a father-to-child inheritance pattern in Caucasian patients with idiopathic Moyamoya disease (MMD). Our cases indicate possible genetic risk factors for the genesis of Caucasian Moyamoya disease.

[New options in the therapeutic management of acute ischaemic stroke. Good results with combined i. v. and i. a. lysis and mechanical thrombectomy]. / Neue Wege bei der Akuttherapie des ischämischen Schlaganfalls. Behandlungserfolge mit kombinierter i. v. und i. a. Lyse sowie mechanischer Thrombektomie.

BACKGROUND: Standard therapy for acute ischaemic stroke is the intravenous thrombolysis with rtPA. A combined therapy with intravenous bridging and consecutive intraarterial thrombolysis and mechanical thrombectomy is a relatively new option in patients with proximal vessel occlusion. PATIENTS AND METHODS: 10 Patients with a CTA proven proximal vessel occlusion in the anterior circulation (ACI, carotis bifurcation, MCA) in CTA were treated with a combined therapy with i. v. and i. a. thrombolysis and thrombectomy with a Solitaire FR stent device. RESULTS: All Patients were recanalized, the NIHSS changed from 15.6 to 3.3. 8 out of 10 patients had nearly no symptoms when dismissed. There were no direct therapeutic complications. CONCLUSION: Combined therapy with i. v. and i. a. thrombolysis and thombectomy with the Solitaire FR stent device is a promising option in patients with acute proximal vessel occlusion in the anterior circulation.

Historical review: a short history of German neurology - from its origins to the 1940s.

This paper aims at reconstructing the development and role of German neurology between 1840 and 1940. Therefore a couple of original sources as well as selected material form the scattered secondary literature were assessed and reviewed. Since the middle of the nineteenth century, an intricate process of separation from internal medicine and psychiatry gradually led to forming a self-conscious community of German neurologists. While Moritz Heinrich Romberg had constructed a cognitive basis for neurology, scientific founders such as Wilhelm Erb, Carl Wernicke, Alois Alzheimer, Hermann Oppenheim, Max Nonne, and many others established the new discipline within modern medicine. In 1891, the first generation of "pure" neurologists succeeded in founding the German Journal for Neurology (Deutsche Zeitschrift für Nervenheilkunde) followed by an autonomous professional organisation, the Society of German Neurologists (Gesellschaft Deutscher Nervenärzte) in 1907. A variety of external factors, however, hampered the institutional evolution and thus the implementation of chairs and departments remained quite modest. In 1935, only 2 years after the National Socialists had seized power, the regulatory merger with the psychiatrists' society caused the cautious attempts of German neurologists for autonomy to end in complete failure. The imprisonment, murder and expulsion of neuroscientists declared as Jewish or non-Aryan caused profound changes in neurology, medicine, academic life, and health care in general. Further historical research is needed to reconstruct in detail the involvement of German neurologists in racial-hygienic and eugenic research as well as the institutional and scientific development of German neurology after World War II.

Visualization and Classification of Deeply Seated Collateral Networks in Moyamoya Angiopathy with 7T MRI.

BACKGROUND AND PURPOSE: Collateral networks in Moyamoya angiopathy have a complex angioarchitecture difficult to comprehend on conventional examinations. This study aimed to evaluate morphologic patterns and the delineation of deeply seated collateral networks using ultra-high-field MRA in comparison with conventional DSA. MATERIALS AND METHODS: Fifteen white patients with Moyamoya angiopathy were investigated in this prospective trial. Sequences acquired at 7T were TOF-MRA with 0.22 × 0.22 × 0.41 mm3 resolution and MPRAGE with 0.7 × 0.7 × 0.7 mm3 resolution. Four raters evaluated the presence of deeply seated collateral networks and image quality in a consensus reading of DSA, TOF-MRA, and MPRAGE using a 5-point scale in axial source images and maximum intensity projections. Delineation of deeply seated collateral networks by different imaging modalities was compared by means of the McNemar test, whereas image quality was compared using the Wilcoxon signed-rank test. RESULTS: The relevant deeply seated collateral networks were classified into 2 categories and 6 pathways. A total of 100 collateral networks were detected on DSA; 106, on TOF-MRA; and 73, on MPRAGE. Delineation of deeply seated collateral networks was comparable between TOF-MRA and DSA (P = .25); however, both were better than MPRAGE (P < .001). CONCLUSIONS: This study demonstrates excellent delineation of 6 distinct deeply seated collateral network pathways in Moyamoya angiopathy in white adults using 7T TOF-MRA, comparable to DSA.