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Nerve compression syndromes are referred to as chronic irritation or pressure palsies of peripheral nerves in areas of preformed anatomical constriction. Carpal tunnel syndrome is the most common nerve compression syndrome, followed by cubital tunnel syndrome. In addition, less frequent nerve compression syndromes of the upper extremities that affect the median, ulnar or radial nerves have been described. This review provides an overview of current treatment options for nerve compression syndromes of the upper extremities.Systematic overview.Based on established national (AWMF) and international guidelines as well as the Cochrane Library, we performed a systematic literature search on PubMed (NLM), focusing on randomised controlled trials.Over the research period (2012 - 2020), there were 43 randomised trials that investigated surgical carpal tunnel release methods, 68 that compared different conservative therapies and 12 that compared surgical versus conservative treatments. Furthermore, eight studies analysed surgical techniques and four analysed conservative techniques for cubital tunnel syndrome. No randomised trials were conducted on the less common peripheral nerve compression syndromes, but case reports and observational studies were conducted.For carpal and cubital tunnel syndromes, there are evidence-based diagnostic methods as well as therapeutic recommendations based on randomised and controlled trials. When diagnosis is made after clinical examination and supported by radiological and electrophysiological evaluation, surgical treatment is considered superior to conservative treatment. In particular, excellent long-term results can be achieved by surgery for carpal and cubital tunnel syndromes. More controlled studies are needed to establish evidence-based therapeutic recommendations for the less common nerve compression syndromes, which are somewhat controversially and inconsistently defined.Als Nervenkompressionssyndrome bezeichnet man chronische Druckläsionen peripherer Nerven im Bereich präformierter anatomischer Engstellen. Hierbei bildet das Karpaltunnelsyndrom das häufigste Engpasssyndrom eines peripheren Nervs, gefolgt vom Kubitaltunnelsyndrom. Daneben gibt es an der oberen Extremität noch seltener vorkommende Engpasssyndrome des N. medianus, N. ulnaris und N. radialis. Die vorliegende Übersichtsarbeit soll den aktuellen Stand bez. der Therapiemöglichkeiten und -empfehlungen für die Behandlung von Nervenkompressionssyndromen der oberen Extremität darstellen.Systematische Übersichtsarbeit.Ausgehend von bestehenden nationalen (AWMF) und internationalen Leitlinien sowie der Cochrane Library führten wir eine systematische Literaturrecherche über PubMed (NLM) mit dem Fokus auf randomisiert kontrollierte Studien durch.Zum Zeitpunkt der Recherche (12 – 2020) existieren für das Karpaltunnelsyndrom 43 randomisierte Studien zum Vergleich der operativen Techniken, 68 zum Vergleich nicht operativer Therapien und 12 zum Vergleich der operativen mit nicht operativen Therapien. Für das Kubitaltunnelsyndrom existieren 8 randomisierte Studien zum Vergleich der operativen Techniken und 4 zum Vergleich der nicht operativen Techniken. Für die selteneren peripheren Nervenkompressionssyndrome existieren keine randomisierten Studien, es dominieren Fallberichte und Beobachtungsstudien zu den einzelnen Entitäten.In Bezug auf das Karpaltunnel- und das Kubitaltunnelsyndrom existieren sowohl evidenzbasierte Diagnoseverfahren als auch Therapieempfehlungen, die randomisiert kontrolliert belegt werden können. Bei klinisch und ggf. auch elektrophysiologisch gesicherter Diagnose stellt das operative Vorgehen meist eine im Vergleich zum nicht operativen Vorgehen überlegene Therapieoption dar. Hierbei lassen sich insbesondere für das Karpaltunnel-, aber auch für das Kubitaltunnelsyndrom gute Ergebnisse in Bezug auf das Langzeitresultat erreichen. Für die selteneren Kompressionssyndrome, die teilweise umstritten, teilweise uneinheitlich benannt sind, sind weitere kontrollierte Studien notwendig, um evidenzbasierte Therapieempfehlungen aussprechen zu können.

ZusammenfassungVon weltweit mehr als 400 Mio. Menschen mit Diabetes mellitus entwickeln bis zu 50% im Laufe ihrer Erkrankung eine Neuropathie. Trotz oder gerade wegen dieser Häufigkeit darf jedoch nicht jede Neuropathie, die in Koinzidenz mit einem Diabetes mellitus auftritt, unkritisch als diabetische Neuropathie diagnostiziert werden. Eine präzise Ausschluss- und Ausmaßdiagnostik ist entscheidend, um andere behandelbare Erkrankungen wie z. B. die Chronisch Inflammatorische Demyelinisierende Polyradikuloneuropathie oder die hereditäre Transthyretin-Amyloidose nicht zu übersehen. Einfache, nicht-invasive, preiswerte und allzeit verfügbare Screeningmethoden stellen Anamnese und klinische Untersuchung dar. Ergänzend ist in frühen Erkrankungsstadien die Quantitativ Sensorische Testung hilfreich zur Eingrenzung einer Small Fiber-Dysfunktion. Sind, typischerweise im Verlauf, große Nervenfasern geschädigt, so ist das charakteristische elektrophysiologische Bild das einer längenabhängigen, axonalen, sensibel betonten oder sensomotorischen Neuropathie. Die Nervensonografie kann zur Unterscheidung von autoimmun-demyelinisierenden Neuropathien hilfreich sein. Moderne Untersuchungsverfahren wie die MR-Neurografie können auch proximale Nervenabschnitte bis auf Faszikelebene darstellen, sind allerdings nur an wenigen Zentren verfügbar. Haut- und Nervenbiopsien sind v. a. bei untypischen Verläufen zur Abgrenzung von Differenzialdiagnosen hilfreich. Diabetische Neuropathien können zu einer erheblichen Reduktion von Lebensqualität und Lebensdauer führen. Zur frühest- und bestmöglichen ursächlichen und symptomatischen Therapieeinleitung ist eine präzise Diagnostik essentiell.

ZusammenfassungHintergrundDie Hälfte aller Diabetiker ist von einer diabetischen Neuropathie betroffen. Mikroangiopathie, dysfunktionale Schwann-Zell-Interaktion, Akkumulation toxischer Metabolite und inflammatorische Prozesse führen gemeinsam zur Nervenschädigung.Ziel der ArbeitAus- und Überblick zum aktuellen Kenntnisstand der Pathophysiologie mit aktuellen und zukünftigen Therapieimplikationen.MethodenLiteraturrecherche (1990–2020).ErgebnisseKlinisch führend sind sensible und autonome Symptome, Paresen können jedoch auftreten. Komplikationen wie stumme Myokardinfarkte oder das diabetische Fußsyndrom können lebensbedrohlich verlaufen und zu schwerer Behinderung führen. In ihrer Pathophysiologie unterscheiden sich Neuropathien bei Typ-1- und Typ-2-Diabetikern durch die Gegenwart zusätzlicher Risikofaktoren des metabolischen Syndroms. Die intensivierte ist der konventionellen Insulintherapie im Hinblick auf die Neuropathierisikoreduktion überlegen. Orale Antidiabetika sind nach Nebenwirkungsprofil auszuwählen. Metformin kann zu einem iatrogenen Vitamin-B12-Mangel führen. Zur Behandlung neuropathischer Schmerzen besitzt der Kalziumkanalblocker Pregabalin die höchste Empfehlungsstufe. Das Trizyklikum Amitriptylin gilt als ähnlich wirksam, ist aber bei autonomer Dysfunktion sowie kognitiven Einschränkungen kontraindiziert. Alternativ ist der Serotonin-Noradrenalin-Wiederaufnahmehemmer Duloxetin zur symptomatischen Therapie der diabetischen Neuropathie zugelassen. Umstritten sind weitere, zum Teil nicht zugelassene Medikamente wie α‑Liponsäure, Epalrestat und L‑Serin.SchlussfolgerungenDie diabetische Neuropathie ist häufig und komplikationsreich. Ein gutes Verständnis der Pathophysiologie kann zur Entwicklung neuer Therapiestrategien beitragen.

ZusammenfassungDie neuropathische Osteo(arthro-)pathie, auch Charcot-Fuß genannt, ist eine progressive, nicht-infektiöse Schwellung mit Überwärmung und Demineralisierung, gefolgt von Knochendestruktion und Deformierung, die in ca. 75% unilateral auftritt und in einer Defektheilung zum Stillstand kommt. Resultierende Fehlstellungen können zu neuropathischen Ulzera führen, die sich infizieren und Amputationen erforderlich machen können. Die häufigste, aber nicht einzige Ursache ist der Diabetes mellitus. Etwa 2% aller Diabetiker entwickeln einen Charcot-Fuß. Der pathophysiologische „Charcot-Prozess“ ist komplex, scheint aber untrennbar mit der vorausgehenden Neuropathie verbunden zu sein. Die C- und Aδ- Fasern sind im Rahmen der diabetischen Neuropathie früh und häufig geschädigt, was ein Ungleichgewicht an CGRP, VIP, Substanz P und weiteren Transmittern erklärt. Störungen der Knocheninnervation verschieben das Verhältnis von Knochenan- und -abbau, von OPG und RANKL zugunsten des Abbaus. Demnach stellt die Fehlregulation nozizeptiver Nervenfasern auf molekularer Ebene eine pathophysiologische Brücke zwischen Diabetes mellitus und neurogener Inflammation dar.

Zusammenfassung Einleitung Eine nicht-ketotische Hyperglykämie kann über eine Affektion der Basalganglien zu einer hemiballistisch-hemichoreatischen Bewegungsstörung führen. Anamnese Ein 68-jähriger Patient stellte sich mit einer subakut aufgetretenen und schmerzlosen hemiballistisch-choreatischen Bewegungsstörung vor. Untersuchungen und Diagnose Eine erweiterte laborchemische und bildgebende Diagnostik konnte bei einem HbA1c von 16,4 %, negativen Ketonkörpern im Urin sowie charakteristischem MR-grafischem Nachweis eines hyperintensen Striatums in der T1-Wichtung die Diagnose einer nicht-ketotischen Hyperglykämie-induzierten Bewegungsstörung erbringen. Therapie und Verlauf Eine antidiabetische Therapie mit Metformin, Insulin glargin und Sitagliptin sowie eine symptomatische Therapie mit 3 mg Risperidon wurde begonnen. Die Blutzuckerwerte normalisierten sich, sodass es im frühen stationären Verlauf zu einer langsamen und leichtgradigen Regredienz der Bewegungsstörung kam. Folgerung Die genauen pathophysiologischen Mechanismen dieser Bewegungsstörung sind noch nicht voll verstanden. Bei entsprechender Klinik und passenden laborchemischen wie bildmorphologischen Befunden sollte aber immer an eine mögliche nicht-ketotische Hyperglykämie-induzierte Bewegungsstörung gedacht werden.

Zusammenfassung Einleitung Ziel der vorliegenden Studie war der Vergleich möglicher Risikofaktoren für die beiden häufigsten Formen der spontanen intracerebralen Blutung (ICB): der hypertensiven ICB und der mit einer zerebralen Amyloidangiopathie (ZAA) assoziierten ICB. Methoden Retrospektiv wurden verschiedene Parameter und Faktoren bei Patienten mit einer hypertensiven ICB (n = 141) und Patienten mit einer ZAAassoziierten ICB (n = 95) verglichen. Dazu zählten Alter, INR-Wert und Blutdruck bei Aufnahme, kardiovaskuläre Risikofaktoren sowie die Vormedikation. Als Testverfahren wurden der Chi-Quadrat-Test mit der Yates‘ Kontinuitätskorrektur sowie der t-Test verwendet. Ergebnisse Patienten der Gruppe mit ZAA-assoziierter ICB waren signifikant älter als Patienten mit einer hypertensiven ICB (p = 0,001). Zudem bestanden bei den ZAA-Patienten signifikant häufiger eine ASS-Vormedikation (p = 0,042) sowie ein vorausgegangener Schlaganfall (p = 0,048). Patienten beider Gruppen hatten zu einem großen Anteil einen arteriellen Hypertonus als Vordiagnose in der Vorgeschichte, wobei dies bei Patienten mit einer hypertensiven ICB signifikant häufiger vorlag (p Schlussfolgerung Der arterielle Hypertonus ist bei beiden Blutungsformen ein relevanter Risikofaktor und sollte daher zur Blutungsprophylaxe adäquat behandelt werden. Zur genaueren Einordung weiterer Risikofaktoren sind Studien mit größeren Fallzahlen notwendig.

Background: Though Todd’s phenomenon (TP) is a relatively rare occurrence, its correct identification is of key diagnostic and therapeutic importance as a stroke mimic. Here we describe a case of isolated gaze palsy as a manifestation of TP, discuss periictal gaze abnormalities as lateralizing sign involving the frontal eye field (FEF), and present a narrative literature review. Methods: We reviewed the main features of the case and conducted a structured literature search of TP and gaze palsy using PubMed. We restricted the search to publications in English, Spanish, French, and German. Case presentation: A 71-year-old male with a history of right frontotemporal subarachnoid hemorrhage was admitted to the Emergency Department of our institution after suffering a first unprovoked focal to bilateral tonic-clonic seizure with ictal gaze deviation to the left. Cranial imaging showed no signs of ischemia, intracerebral hemorrhage, or tumor. The patient presented the following postictal features: involuntary eye deviation to the right due to left-sided gaze palsy and disorientation in time with preserved responsiveness. Eye movements were normal three days later. We concluded that the patient suffered from new-onset epilepsy due to sequelae following the right frontotemporal subarachnoid hemorrhage, affecting the FEF with contralateral ictal gaze deviation, and postictal gaze palsy with ipsilateral eye deviation as an unusual Todd’s phenomenon. Conclusion: Unusual manifestations of TP are uncommon but clinically highly relevant, as they can mimic stroke or epileptic status and are decisive in the diagnostic and therapeutic decision-making process. Though postictal gaze palsy has been reported associated with other deficits, this constitutes, to our knowledge, the first report of isolated gaze palsy as a form of TP. Further research into the underlying causes is needed. Ictal contralateral gaze and head deviation, and probably postictal ipsilateral gaze deviation if present, are very helpful for the lateralization of the seizure-onset zone.

ObjectiveTo study the diagnostic value of volume perfusion CT (VPCT) in patients with transient focal neurologic deficits following and during epileptic seizures, that mimic symptoms of stroke.MethodsA retrospective case-control study was performed on 159 patients who presented with a seizure and received an emergency VPCT within the first 3.5 hours of admission, after being misjudged to have an acute stroke. The reference test was a clinical-based, EEG-supported diagnostic algorithm for seizure.ResultsWe included 133 patients: 94 stroke-mimicking cases with postictal focal neurologic deficits (“Todd phenomenon,” n = 67) or ongoing seizure on hospital admission (“ictal patients,” n = 27), and 39 postictal controls without focal neurologic deficits. Patients with Todd phenomenon showed normal perfusion (64%), hypoperfusion (21%), and hyperperfusion (14%) on early VPCT. Ictal patients displayed more hyperperfusion compared to postictal patients (p = 0.015). Test sensitivity of hyperperfusion for ictal patients is 38% (95% confidence interval [CI] 20.7%–57.7%), specificity 86% (95% CI 77.3%–91.7%), positive predictive value is 42% (95% CI 27.5%–58.7%), and the negative predictive value 83% (95% CI 78.6%–86.9%). A cortical distribution was seen in all hyperperfusion scans, compared to a cortico-subcortical pattern in hypoperfusion (p < 0.001). A history of complex focal seizure and age were associated with hyperperfusion (p = 0.046 and 0.038, respectively).ConclusionVPCT can differentiate ictal stroke mimics with hyperperfusion from acute ischemic stroke, but not postictal patients who display perfusion patterns overlapping with ischemic stroke.Classification of evidenceThis study provides Class IV evidence that VPCT accurately differentiates ictal stroke mimics from acute ischemic stroke.

The differentiation between epileptic and non-epileptic episodes can be challenging. Our aim was to compare lactate, anion gap (AG), bicarbonate and the Denver Seizure Score (DSS) as point-of-care test (POCT) markers for episodes of transient alterations of consciousness. The blood serum parameters were drawn at arrival in the emergency department (ED) within 2 h of the episode. After calculating AG and DSS values, the four parameters were compared retrospectively between patients with generalized tonic–clonic seizures (GTCS) (n = 165) and patients with other disorders of consciousness [syncopes (n = 43), and psychogenic non-epileptic seizures (n = 15)]. Additionally, we compared all values among men and women. In GTCS patients, all four parameters differed significantly compared to non-epileptic episode patients (p

Laborparameter haben einen hohen Stellenwert in der Differenzialdiagnostik akuter Bewusstseinsstorungen. Zur Unterscheidung zwischen generalisierten tonisch-klonischen Anfallen (GTKA) und Synkopen als Ursache einer akuten Bewusstseinsstorung konnen Serumlaktat- und Serumkreatinkinase(CK)-Konzentrationen wertvolle Hinweise liefern. Bei Patienten mit klinisch eindeutigen GTKA (n = 30) und klinisch eindeutigen Synkopen (n = 15) wurden die Serumlaktatkonzentrationen bei Aufnahme sowie die CK-Konzentrationen 10–48 h nach der zur Aufnahme fuhrenden Episode verglichen. Zusatzlich wurde deren Sensitivitat und Spezifitat als diagnostische Indikatoren fur die Unterscheidung zwischen einer Synkope und einem GTKA bestimmt. Die Serumlaktat- und Serum-CK-Konzentrationen waren bei GTKA-Patienten gegenuber Synkopen-Patienten signifikant erhoht (Serumlaktat: p

Concentrations of serum creatine kinase (CK) and serum lactate are frequently measured to help differentiate between generalized tonic–clonic seizures (GTCS) and syncope. The aim of this prospective cohort study was to systematically compare these two markers. The primary outcome is the measurement of serum lactate and CK in blood samples drawn within 2 h of the event in patients admitted with either a GTCS (n = 49) or a syncope (n = 36). Furthermore, the specificity and sensitivity of serum lactate and CK are determined as diagnostic markers in distinguishing between GTCS and syncope. GTCS patients have significantly higher serum lactate levels compared to syncope patients (p

We report on a 32-year-old male patient with acute left-hemispheric stroke caused by embolism due to infective endocarditis affected from the HACEK group. Additionally, atypical findings from the transesophageal echocardiography (TEE) which showed fluttering structures belonging to the papillary muscle could be proven as infectious agents with the help of a glucose positron emission tomography (PET) scan. TEE controls showed increasing vegetation involving the mitral valve so that surgery became necessary. The current work reflects, in detail, the emergent clinical course of this young patient, suffering from both an unusual localization and an infrequent cause of endocarditis and focuses on an actual view to the literature.

Bei Kindern treten Spiegelbewegungen physiologisch auf und sind wahrscheinlich der entwicklungsbedingten Unreife des Balkens geschuldet. Pathologische, angeborene Spiegelbewegungen konnen klinisch entweder isoliert oder als Teil komplexer genetischer Syndrome wie dem Kallmann-Syndrom und dem Klippel-Feil-Syndrom auftreten, kommen aber auch im Rahmen der infantilen Zerebralparese (Morbus Little) vor. Kongenitale isolierte Spiegelbewegungen folgen meist einem autosomal-dominanten Erbgang.

BACKGROUND AND PURPOSE: The impact of various radiologic and clinical features on the long-term outcome in spinal dural arteriovenous fistulas is still unclear; thus, they are the purpose of this study. MATERIALS AND METHODS: We retrospectively analyzed our medical data base for all patients treated for spinal dural arteriovenous fistula in our institution between 2006 and 2016. Patient age, neurologic status at the time of diagnosis, the duration of symptoms from onset to diagnosis, and follow-up information were evaluated. The extent of medullary T2WI hyperintensity, intramedullary contrast enhancement, and elongation of perimedullary veins on MR imaging at the time of diagnosis were additionally analyzed. RESULTS: Data for long-term outcome analysis were available in 40 patients with a mean follow-up of 52 months (median, 50.5 months; range, 3–159 months). The mean age at the time of diagnosis was 69.27 ± 9 years (median, 71 years; range, 53–84 years) with a male predominance (n = 32; 80%). The mean duration of symptoms was 20.2 months (median, 10 months; range, 1–120 months). Shorter duration of symptoms at the time of diagnosis was significantly correlated with better outcome of symptoms (P CONCLUSIONS: Spinal dural arteriovenous fistulas are characterized by interindividually variable clinical presentations, which make a determination of specific predictors for long-term outcome more difficult. Fast and sufficient diagnosis might result in a better outcome after treatment. The diagnosis of spinal dural arteriovenous fistula remains markedly delayed, reflecting an ongoing lack of knowledge and awareness among treating physicians of this rare-but-serious disease.

Aim Knowledge about cardiac stress related to seizures in electroconvulsive therapy (ECT) and spontaneously occurring generalized convulsive seizures (GCS) is limited. The aim of the present study was to analyze cardiac function and circulating markers of cardiac stress in the early postictal period after ECT and GCS. Methods Patients undergoing ECT in the Department of Psychiatry, Psychotherapy and Psychosomatics and patients undergoing diagnostic video-EEG monitoring (VEM) in the Department of Neurology were prospectively enrolled between November 2017 and November 2018. Cardiac function was examined twice using transthoracic echocardiography within 60 min and > 4 h after ECT or GCS. Established blood markers (troponin T high-sensitive, N-terminal pro brain natriuretic peptide) of cardiac stress or injury were collected within 30 min, 4 to 6 h, and 24 h after ECT or GCS. In the ECT group, the troponin T values were also correlated with periprocedural heart rate and blood pressure values. Because of organizational or technical reasons, the measurement was not performed in all patients. Results Twenty patients undergoing ECT and 6 patients with epilepsy with a GCS during VEM were included. Postictal echocardiography showed no wall motion disorders and no change in left ventricular and right ventricular functions. Four of 17 patients displayed a transient increase in high-sensitive cardiac troponin T 4–6 h after the seizure (3 patients with ECT-induced seizure). None of these 4 patients had signs of an acute cardiac event, and periprocedural blood pressure or heart rate peaks during ECT did not significantly differ in patients with and without troponin T elevation. Conclusions Signs of mild cardiac stress can occur in some patients following ECT or GCS without clinical complications, probably related to excessive catecholamine release during the seizure.

Etwa 5% aller in Spezialambulanzen gesehenen Bewegungsstorungen haben einen psychogenen Ursprung. Tremor und Dystonie stellen unter diesen die haufigsten psychogenen Bewegungsstorungen dar. In der Regel ist die Diagnosestellung zeitaufwendig und kostenintensiv, obwohl es eine Vielzahl von positiven klinischen Befunden fur das Vorliegen einer psychogenen Genese der Bewegungsstorung gibt. Insofern kommt der Kenntnis dieser positiven Befunde eine grose Bedeutung zu. Ein prompter Beginn der Erkrankung, die variable Auspragung der Symptomatik, Ablenkbarkeit und spezielle klinische Provokationsmanover konnen bei der Diagnosestellung helfen. Die Zusatzdiagnostik mittels Elektromyografie sowie kinematischer Aufzeichnungsverfahren konnen die Diagnose stutzen als auch, als Verlaufsparameter eingesetzt werden. Im Gegensatz dazu dienen die bildgebenden Verfahren eher der Ausschlussdiagnostik. Die vorliegende Ubersichtsarbeit widmet sich der Anamnese und Klinik, der Differenzialdiagnose und der Zusatzdiagnostik psychogener Bewegungsstorungen.

Introduction I-123-IBZM-SPECT is often used to differentiate between idiopathic Parkinson's syndrome and atypical parkinsonian syndromes. The aim of this study was to compare three different procedures to quantify the receptor availability of striatal dopamine D2 receptors. (a) Manual quantification performed using individually adjusted volume of interests sets (mVoi). (b) Automatic quantification applying the commercially available Hermes BRASS software (BRASS). (c) Automatic quantification applying the open-source software IBZM Toolbox (TBX).Materials and methods Using the three methods, we analyzed 100 scans. For the mVOI methods, three different investigators (two experienced, one inexperienced) carried out the analysis. We compared the different methods with each other and with the reference standard established by clinical follow-up. The diagnostic performance was assessed by calculating receiver-operating characteristic (ROC) curves.Results Correlation analyses resulted in the following: mVOI versus BRASS (r(2)=0.694) (P

Objective Deep lumbosacral dural arteriovenous fistulas (lsDAVF) are rare and present serious diagnostic and treatment difficulties. In our current analysis we present our treatment strategy and the long-term clinical outcome of nineteen patients with lsDAVF. Patients and methods We retrospectively analyzed our radiological and medical records for patients presenting with SDAVF between 1990 and 2018 at the University Hospital Aachen. We identified twenty patients with a lsDAVF. All patients were treated surgically. One patient died of pulmonary embolism three months after treatment and was excluded from our outcome analysis. Clinical data at time of admission, discharge, one year after discharge and at the last follow-up were evaluated according to modified Aminoff-Logue disability score (AL-score) for this analysis. Results Mean age was 65 ± 7 years (median, 67; range, 53–78), sixteen patients (84 %) were male. After surgery, four patients developed a recurrent fistula in the same shunt zone and were re-treated microsurgically. Follow-up data one year after treatment was available in 15 patients. No relevant changes in AL-score were observed within this period. For the long-term follow-up analysis, data of 13 patients were available; 38.5 % of patients developed late functional deterioration. Conclusion In our cohort, patients with deep lumbosacral dural arteriovenous fistula had a higher risk of early recurrence compared to patients with thoracolumbar SDAVF, with a considerable percentage of late functional deterioration. Thus strict clinical and radiologic long-term follow-up examinations are recommended in those patients.

Background Seasickness and travel sickness are classic types of motion illness. Modern simulation systems and virtual reality representations can also induce comparable symptoms. Such manifestations can be alleviated or prevented by various measures. Methods This review is based on pertinent publications retrieved by a PubMed search, with special attention to clinical trials and review articles. Results Individuals vary in their susceptibility to autonomic symptoms, ranging from fatigue to massive vomiting, induced by passive movement at relatively low frequencies (0.2 to 0.4 Hz) in situations without any visual reference to the horizontal plane. Younger persons and women are considered more susceptible, and twin studies have revealed a genetic component as well. The various types of motion sickness are adequately explained by the intersensory conflict model, incorporating the vestibular, visual, and proprioceptive systems and extended to include consideration of postural instability and asymmetry of the otolith organs. Scopolamine and H1-antihistamines, such as dimenhydrinate and cinnarizine, can be used as pharmacotherapy. The symptoms can also be alleviated by habituation through long exposure or by the diminution of vestibular stimuli. Conclusion The various types of motion sickness can be treated with general measures to lessen the intersensory conflict, behavioral changes, and drugs.

BACKGROUND AND PURPOSE: The purpose of this study was to discuss the clinical and radiologic characteristics of spinal epidural arteriovenous fistulas (SEAVF) and demonstrate their specific angiomorphology in a single-center series. MATERIALS AND METHODS: Thirteen consecutive patients were diagnosed with SEAVF at RWTH Aachen University Hospital between 2006 and 2018 and were included in this study. All patients had MR imaging and DSA before treatment; 10 of these 13 patients received contrast-enhanced MRA (CE-MRA). RESULTS: The mean patient age was 72 ± 8 years. Paraparesis was present in 12 (92%) patients. Sphincter dysfunction and sensory symptoms were observed in 7 (54%) and 6 (46%) patients, respectively. The mean duration of symptoms was 6 ± 8 months. Congestive myelopathy on MR imaging was present in all patients. Prominent arterialized perimedullary veins were demonstrated in only 3 cases. CE-MRA revealed arterialized perimedullary veins and an arterialized epidural pouch in 9/10 (90%) patients, mostly located ventrolaterally. DSA demonstrated a multisegmental extension of the arterialized ventrolateral epidural pouch in 6 (46%) cases. An intradural radicular drainage vein was localized distant from the original fistula point in 3 (23%) patients. CONCLUSIONS: Congestive myelopathy with an acute/subacute clinical course was the dominant finding in spinal epidural arteriovenous fistulas. CE-MRA is a powerful diagnostic tool for identifying arterialized perimedullary veins as well as an arterialized epidural pouch. While arterialized perimedullary veins frequently present with only mild enlargement and elongation in spinal epidural arteriovenous fistulas, the arterialized epidural pouch is frequently located ventrolaterally and may extend over several vertebral levels. DSA remains the criterion standard to precisely visualize a spinal epidural arteriovenous fistula and its intradural radicular drainage vein, which may be located distant from the fistulous point.

BACKGROUND AND PURPOSE: Spinal dural arteriovenous fistulas located in the deep lumbosacral region are rare and the most difficult to diagnose among spinal dural arteriovenous fistulas located elsewhere in the spinal dura. Specific clinical and radiologic features of these fistulas are still inadequately reported and are the subject of this study. MATERIALS AND METHODS: We retrospectively evaluated all data of patients with spinal dural arteriovenous fistulas treated and/or diagnosed in our institution between 1990 and 2017. Twenty patients with deep lumbosacral spinal dural arteriovenous fistulas were included in this study. RESULTS: The most common neurologic findings at the time of admission were paraparesis (85%), sphincter dysfunction (70%), and sensory disturbances (20%). Medullary T2 hyperintensity and contrast enhancement were present in most cases. The filum vein and/or lumbar veins were dilated in 19/20 (95%) patients. Time-resolved contrast-enhanced dynamic MRA indicated a spinal dural arteriovenous fistula at or below the L5 vertebral level in 7/8 (88%) patients who received time-resolved contrast-enhanced dynamic MRA before DSA. A bilateral arterial supply of the fistula was detected via DSA in 5 (25%) patients. CONCLUSIONS: Clinical symptoms caused by deep lumbosacral spinal dural arteriovenous fistulas are comparable with those of spinal dural arteriovenous fistulas at other locations. Medullary congestion in association with an enlargement of the filum vein or other lumbar radicular veins is a characteristic finding in these patients. Spinal time-resolved contrast-enhanced dynamic MRA facilitates the detection of the drainage vein and helps to localize deep lumbosacral-located fistulas with a high sensitivity before DSA. Definite detection of these fistulas remains challenging and requires sufficient visualization of the fistula-supplying arteries and draining veins by conventional spinal angiography.

Infekt-assoziierte Erkrankungen des ZNS stellen seltene, aber in der Regel schwer verlaufende Erkrankungen im klinischen Alltag dar. Wir berichten uber den Fall eines 22-jahrigen Patienten, der nach einem respiratorischen Infekt bei bekanntem Asthma bronchiale ausgepragte neurologische Defizite entwickelte, die unter einer empirischen Therapie mit Vancomycin, Ampicillin und Ceftriaxon progredient waren. Im Liquor und in der kranialen Magnetresonanztomografie fanden sich Hinweise auf eine Meningoenzephalitis. Mikrobiologische Untersuchungen konnten im Verlauf eine akute Infektion mit Mykoplasma pneumoniae nachweisen, so dass eine Therapie mit Minozyklin und Prednisolon erfolgte, die zu einer nahezu kompletten Remission der Erkrankung fuhrte. Die Pathomechanismen und Behandlungsstrategien der Erkrankung werden im Folgenden dargestellt.

Background Cerebral amyloid angiopathy (CAA) is a degenerative vasculopathy that is classically associated with lobar intracerebral or sulcal hemorrhage. Its prevalence is estimated at 30% in the seventh decade and 50% in the eighth and ninth decades. In this review, we summarize the risks linked to CAA with respect to the treatment and prevention of stroke. Methods This review is based on pertinent publications retrieved by a selective search employing the terms "amyloid cerebral angiopathy," "stroke," "intra - cerebral bleeding," and "acute stroke therapy." Results Among patients given systemic lytic treatment for stroke, those who have microhemorrhages tend to have a higher risk of treatment-associated brain hemorrhage. In a meta-analysis, 70% of patients who sustained a hemorrhage after thrombolytic therapy were found to have CAA, compared to only 22% in a control population. Patients with cerebral hemorrhages have microhemorrhages more commonly than patients with transient ischemic attacks (TIA) or infarcts. This was observed among persons under treatment with vitamin K antagonists (odds ratio, 2.7) or platelet aggregation inhibitors (odds ratio, 1.7). Moreover, the apolipoprotein E2 allele is associated with a higher incidence of intracerebral hemorrhage (ICH) under oral anticoagulation. Strict treatment of arterial hypertension can lower the risk of ICH in persons with probable CAA by 77%. On the other hand, the use of statins after a lobar ICH increases the risk for a clinically manifest recurrent hemorrhage from 14% to 22%. Conclusion In patients with CAA, arterial hypertension should be tightly controlled. On the other hand, caution should be exercised in prescribing oral anticoagulants or platelet aggregation inhibitors for patients with CAA, or statins for patients who have already sustained a lobar ICH.

Hintergrund: Die periphere Fazialisparese stellt die haufigste Mononeuropathie im Kopfbereich dar. In uber 80% der Falle handelt es sich dabei um eine idiopathische Fazialisparese (Bell’s ­palsy). Die Abgrenzung zu den symptomatischen Formen allein auf Basis der klinischen Prasentation ist haufig schwierig, insbesondere wenn typische Befunde wie z. B. ein Erythema chronicum migrans in der Vorgeschichte fehlen. Ziel dieser Arbeit war die Uberprufung der Wertigkeit der kranialen Computertomografie in der differen­zialdiagnostischen Abklarung einer akut aufgetretenen peripheren Fazialisparese. Methodik: Es wurden 550 konsekutive Patienten mit Fazialisparese, die am Universitatsklinikum Aachen in den Jahren 2000–2010 behandelt wurden, retrospektiv ausgewertet. Neben den klinischen Befunden erfolgten die Erfassung der Bildgebungsergebnisse und deren Auswertung im Hinblick auf ihren Vorhersagewert. Ergebnisse: Bei 80% aller Patienten wurde eine kraniale Computertomografie inklusive Darstellung der Felsenbeine angefertigt. Bei 3 Patienten (0,7%) konnte durch die Bildgebung ein die Parese erklarender Befund erhoben werden. In diesen 3 Fallen war die Diagnose einer nicht-idiopathischen Fazialisparese allerdings auch anhand der Anamneseerhebung und klinisch-neurologischen Untersuchung zu stellen. Schlussfolgerung: Entsprechend den Leitlinien der Deutschen Gesellschaft fur Neurologie ist die kraniale Computertomografie bei der Differen­zialdiagnostik einer akut aufgetretenen peripheren Fazialisparese ohne zusatzliche bestehende neurologische Ausfalle und fehlenden Hinweisen auf ein Trauma oder eine Tumorerkrankung nicht indiziert und sollte als Routineuntersuchung nicht mehr durchgefuhrt werden.

Medikamenteninduzierter Schwindel tritt in der Praxis nicht selten auf. Schwindel verursachende Substanzen lassen sich in solche mit vorwiegend toxischer Wirkung an den labyrintharen Strukturen und solche mit vorwiegend zentraler Wirkung einteilen. Substanzen, die Sehstorungen hervorrufen, schadigen das Pigmentepithel oder den Sehnerv oder interagieren mit Transmittern der Retina.

Drug-induced seizures are in view of a constantly ageing population and increasingly frequent polypharmacotherapy an increasing problem in daily routine praxis. Identification of potentially seizure-inducing drugs may help generating risk profiles for individual patients. Drug-induced seizures have often been seen as a complication of psychopharmacological therapy, but its occurrence has also been described in response to a great diversity of compounds such as antibiotics, sympathomimetics and anaesthetics. The present article outlines a synopsis of the most prevalent seizure-inducing drugs as well as strategies how to deal with a patient suffering from a drug-induced seizure.

Summary Background The European Friedreich's Ataxia Consortium for Translational Studies (EFACTS) is a prospective international registry investigating the natural history of Friedreich's ataxia. We used data from EFACTS to assess disease progression and the predictive value of disease-related factors on progression, and estimated sample sizes for interventional randomised clinical trials. Methods We enrolled patients with genetically confirmed Friedreich's ataxia from 11 European study sites in Austria, Belgium, France, Germany, Italy, Spain, and the UK. Patients were seen at three visits—baseline, 1 year, and 2 years. Our primary endpoint was the Scale for the Assessment and Rating of Ataxia (SARA). Secondary outcomes were the Inventory of Non-Ataxia Signs (INAS), the Spinocerebellar Ataxia Functional Index (SCAFI), phonemic verbal fluency (PVF), and the quality of life measures activities of daily living (ADL) and EQ-5D-3L index. We estimated the yearly progression for each outcome with linear mixed-effect modelling. This study is registered with ClinicalTrials.gov, number NCT02069509, and follow-up assessments and recruitment of new patients are ongoing. Findings Between Sept 15, 2010, and Nov 21, 2013, we enrolled 605 patients with Friedreich's ataxia. 546 patients (90%) contributed data with at least one follow-up visit. The progression rate on SARA was 0·77 points per year (SE 0·06) in the overall cohort. Deterioration in SARA was associated with younger age of onset (–0·02 points per year [0·01] per year of age) and lower SARA baseline scores (–0·07 points per year [0·01] per baseline point). Patients with more than 353 GAA repeats on the shorter allele of the FXN locus had a higher SARA progression rate (0·09 points per year [0·02] per additional 100 repeats) than did patients with fewer than 353 repeats. Annual worsening was 0·10 points per year (0·03) for INAS, −0·04 points per year (0·01) for SCAFI, 0·93 points per year (0·06) for ADL, and −0·02 points per year (0·004) for EQ-5D-3L. PVF performance improved by 0·99 words per year (0·14). To detect a 50% reduction in SARA progression at 80% power, 548 patients would be needed in a 1 year clinical trial and 184 would be needed for a 2 year trial. Interpretation Our results show that SARA is a suitable clinical rating scale to detect deterioration of ataxia symptoms over time; ADL is an appropriate measure to monitor changes in daily self-care activities; and younger age at disease onset is a major predictor for faster disease progression. The results of the EFACTS longitudinal analysis provide suitable outcome measures and sample size calculations for the design of upcoming clinical trials of Friedreich's ataxia. Funding European Commission.

Wir berichten uber den Fall eines 63-jahrigen Patienten mit einer Meningoenzephalitis, die sich durch Kopfschmerzen und eine allgemeine psychomotorische Verlangsamung manifestierte. Nebenbefundlich fand sich eine schmerzhafte Rotung und Schwellung beider Ohrmuscheln. MR-tomografisch zeigten sich basal betonte konfluierende, kontrastmittelaufnehmende Herde und im Liquor eine lymphozytare Pleozytose ohne spezifischen Erregernachweis. Eine Biopsie der Ohrmuschel ergab eine chronische lympho-plasmazellulare Entzundungsreaktion. Wir stellten die Diagnose einer Polychondritis atrophicans, einer entzundlichen Erkrankung des Knorpels, die in seltenen Fallen auch das zentrale Nervensystem miteinbeziehen kann. Regelhaft klagen die Patienten uber rote, geschwollene Ohren, das „red puffy ear sign“. Unter Einleitung einer Steroid- und Azathioprin-Medikation kam es im Verlauf zu einer restitutio ad integrum.

Die 63-jährige Patientin wurde nach einem prolongierten Weaning (insgesamt 3 Monate) nach kardiogenem Schock im Rahmen eines Vorderwandinfarktes und anschließendem schwerem Lungenversagen (ARDS) nach diffuser pulmonaler Blutung mit thrombotischer Totalatelektase links und Teilatelektase rechts und mehrfachen Pneumonien sowie spontanem Pneumothorax links dem neurologischen Konsiliarius aufgrund einer Minderbewegung der Beine rechts >links mit der Frage nach einer CIP/CIM vorgestellt.

Despite of precipitous blood pressure falls in Parkinson's Disease (PD) patients, they may not experience syncope or postural complaints. Can cerebral blood flow regulation explain why orthostatic hypotension (OH) has often no accompanying symptoms? In patients with PD and OH (18 asymptomatic; 8 symptomatic), arterial blood pressure (ABP) as well as Doppler-detected cerebral blood flow velocity (CBFV) in middle and posterior cerebral arteries (MCA and PCA) were monitored during head-up tilt and compared with 25 controls and eight non-PD-OH patients. Analysis included the transfer function between slow spontaneous pressure and flow-oscillations. ABP and CBFV were maintained at significantly higher levels in asymptomatic than symptomatic PD-OH (ABP: 85.7 ± 10.5 vs. 66.9 ± 12.5%; MCA-FV: 83.3 ± 9.3 vs. 66.1 ± 6.8%; PCA-FV: 84.4 ± 12.2 vs. 65.9 ± 9.3% of supine). When orthostatic complaints occurred, CBFV depended directly on ABP changes (MCA r(2) = 0.64; PCA r(2) = 0.62; both p < 0.05). Despite of a tilt-induced blood pressure instability in PD-OH, the transfer function parameters did not differ from normal [phase: MCA: 46.6 ± 20.5°; PCA 39.2 ± 28.8°, gain: MCA 2.0 ± 0.7; PCA 2.9 ± 1.6)]. Results showed a normal autoregulatory response to downward blood pressure shifts in PD. Moreover, orthostatic blood pressure instability is compensated equally sufficient in anterior and posterior parts of cerebral circulation. Whether in PD patients, OH becomes symptomatic rather seems to depend on blood pressure falling below the autoregulated range.

The concept of predictive coding supposes the brain to build predictions of forthcoming events in order to decrease the computational load, thereby facilitating efficient reactions. In contrast, increasing uncertainty, i.e., lower predictability, should increase reaction time and neural activity due to reactive processing and believe updating. We used functional magnetic resonance imaging (fMRI) to scan subjects reacting to briefly presented arrows pointing to either side by pressing a button with the corresponding index finger. Predictability of these stimuli was manipulated along the independently varied factors "response type" (known hand or random, i.e., unknown order) and "timing" (fixed or variable intervals between stimuli). Behavioural data showed a significant reaction-time advantage when either factor was predictable, confirming the hypothesised reduction in computational load. On the neural level, only the right temporo-parietal junction showed enhanced activation upon both increased task and timing uncertainty. Moreover, activity in this region also positively correlated with reaction time. There was, however, a dissociation between both factors in the frontal lobe, as increased timing uncertainty recruited right BA 44, whereas increased response uncertainty activated the right ventral premotor cortex, the pre-SMA and the DLPFC. In line with the theoretical framework of predictive coding as a load-saving mechanism no brain region showed significantly increased activity in the lower uncertainty conditions or correlated negatively with reaction times. This study hence provided behavioural and neuroimaging evidence for predictive motor coding and points to a key role of the right temporo-parietal junction in its implementation.

We tested the ability of healthy participants to learn an association between arbitrary sensory cues and the weight of an object to be lifted using a precision grip between the index finger and thumb. Right-handed participants performed a series of grip-lift tasks with each hand. In a first experiment, participants lifted two objects of equal visual appearance which unexpectedly and randomly changed their weight. In two subsequent experiments, the change in object weight was indicated by cues, which were presented (i) visually or (ii) auditorily. When no cue about the weight of the object to be lifted was presented, participants programmed grip force according to the most recent lift, regardless of the hand used. In contrast, participants were able to rapidly establish an association between a particular sensory cue with a given weight and scaled grip force precisely to the actual weight thereafter, regardless of the hand used or the sensory modality of the cue. We discuss our data within the theoretical concept of internal models.

Stroke or transient ischemic attacks (TIA) represent an urgent clinical entity that is not limited only to elderly patients. The underlying causes of stroke and TIA are diverse, with those of cardiovascular origin being among the most prominent. This review seeks to elucidate some of the most important aspects of the disease in the context of emergency and critical care practice.

The wrist drop, also called carpoptosis or drop hand, is a common clinical presentation in case of peripheral damage to the radial nerve. But what about the picture of a bilateral finger/wrist drop?! We report the case of a 61-year-old female patient who was admitted to the hospital for myocardial infarction. Subsequently she developed a right dominant bilateral wrist drop. Further neurological examination revealed a positive Wartenberg sign pointing towards a central motoric dysfunction. The following native cerebral CT scan demonstrated bilateral hypodense lesions in both hand knobs in the precentral gyri. Subsequent MRI confirmed acute cerebral infarction in these two but also several other, clinically silent, locations. Further diagnostic work-up revealed a hypokinetic cardiac apex suggesting cardiac embolism to be the cause for cerebral thrombembolism and the clinically leading symptom of right-dominant bilateral finger/wrist drop. Besides the case presentation also the differential diagnosis and clinical test for diagnostic work-up of wrist drops are presented and discussed.

The anterior biopercular syndrome or Foix-Chavany-Marie syndrome (FCMS) originally described in 1926 is defined by diminished or absent voluntary control of facial, pharyngeal, lingual, and masticatory muscles while reflectory movements are preserved [2, 8]. In the original description, the syndrome occurred after bilateral damage of the anterior opercular area surrounding the insula caused by stroke. Other causes of the syndrome are infections of the nervous system, cortical dysplasias, tumors, trauma, and, but exceedingly rare, degenerative disease [4, 7, 8]. A 70-year-old Caucasian female presented with a progressive dysarthrophonia, swallowing problems, and hypomimia. According to her daughter, the history of speech problems started 2 years earlier and were characterized by slurred speech and hoarseness, which slowly progressed into mutism. Communication was possible by writing. Writing was without any grammatical or orthographic errors and her reading as well as her auditory comprehension were intact. The first neurological examination in an outpatient clinic 2 years earlier had revealed an alert woman without behavioral disturbances and as the patient was incidentally able to smile and laugh while she was unable to do so when she was asked to smile voluntarily, a probable psychogenic speech disorder was diagnosed and therapy with antidepressants was started. On admission to our hospital 1 year later, the patient’s face showed a dystonic-like deformation, which made us think of an ancient Greek theatre mask and she was unable to imitate or produce on request any oral–verbal expression but a growling. The patient was unable to open her mouth, to blow her cheeks, or to frown. Dysphagia and sialorrhea were present. In stark contrast, the patient was observed to laugh while listening to a joke (she started laughing when the examiner started to blow his cheeks during the clinical examination procedure) and she was able to frown when testing vertical eye-movements. The masseter muscle reflex was brisk and primitive reflexes like snout-, graspingand palmo-mental-reflex were present as was Myerson’s sign positive. Due to her dysphagia, the patient had suffered a remarkable weight loss of more than 15 kg. Neuropsychological assessment ruled out both aphasia and dementia, nor were there signs of muscle weakness, Parkinsonism, fasciculation, ataxia, or apraxia. Electrophysiological investigations including nerve conduction studies, blink reflex, motor and sensory evoked potentials were all normal. Electromyography of the M. interosseus dorsalis I, M. vastus medialis, M. tibialis anterior, and the tongue muscles did not reveal any M. Dafotakis (&) Department of Neurology, RWTH Aachen, Aachen, Germany e-mail: mdafotakis@ukaachen.de

A 62-year-old man presented with mild left hemiparesis, headache, and blurred vision of his right eye. Ten days before, he had undergone thromboendarterectomy because of subtotal stenosis of his right internal carotid artery. MRI revealed confluent white matter edema together with focal hemorrhage consistent with cerebral hyperperfusion syndrome (CHS; figure 1). Likewise, funduscopy showed small retinal hemorrhages confined to the right eye (figure 2). Under careful blood pressure control, symptoms and brain edema fully resolved within weeks. CHS occurs in around 3% of patients undergoing carotid thromboendarterectomy and can induce severe neurologic deficits.1 Rarely, hyperperfusion also involves the ipsilateral eye.2

A 37-year-old woman was admitted to our department with general fatigue, fever, and asymmetric pain in her lower legs, 2 weeks after a common cold. Dynamic skeletal scintigraphy with 780 MBq (20 mCi) Tc-99m HDP revealed increased perfusion and tracer uptake in the soft tissue of the lower legs, suggesting an inflammatory etiology. A muscle biopsy revealed a vasculitic necrotizing myopathy predominantly affecting the small vessels of the lower legs. We diagnosed it as a postinfectious immunologic reaction.