Vorbereitungskurs Neurologische Intensivmedizin:

Akute Verwirrtheit ist als Fragestellung in Praxen, Notfallzentren oder im Konsildienst ein häufiges diagnostisches Problem. So kann es nach rein klinischen Kriterien schwierig oder unmöglich sein, eine akute Verwirrtheit sicher von einer chronischen kognitiven Beeinträchtigung zu unterscheiden. Die häufigste Form der akuten Verwirrtheit ist das Delir und bleibt gerade in seiner hypoaktiven Form sicher unterdiagnostiziert.

Der akute, starke Kopfschmerz kann Indikator einer bedrohlichen intrakraniellen Pathologie sein, die rasches Handeln erfordert. Doch auch Patienten mit starken primären Kopfschmerzen bedürfen einer gezielten Behandlung, um eine zügige Schmerzlinderung herbeizuführen. Eine Stratifizierung und Differenzierung gefährlicher und harmloserer Kopfschmerzsyndrome im Notfallbereich kann durch die Beachtung von Warnsymptomen (Red Flags) erleichtert werden.

ZusammenfassungDie therapeutische Dringlichkeit der hypertensiven Entgleisung wird vorrangig vom Vorliegen von akuten neurologischen, okulären, kardiopulmonalen oder renalen Endorganschäden bestimmt. Ohne akute Endorganschäden (hypertensive urgency) ist in der Regel eine orale Behandlung mit dem Ziel der RR-Normalisierung über 24–48 h ausreichend. Bei gravierenden hypertensiven Organsymptomen (hypertensive emergency, hypertensiver Notfall) ist eine zügige intravenöse Initialtherapie erforderlich, bei der die Präparatewahl und das Blutdruckziel von der Organmanifestation und Begleiterkrankungen abhängig sind. Empfehlungen zur optimalen Blutdrucksteuerung bei akuten neurovaskulären Erkrankungen werden durch divergierende Studienergebnisse und die Vielfalt potentiell relevanter Parameter erschwert. Das breite Spektrum des posterioren reversiblen Enzephalopathie-Syndroms kann in atypischen Fällen die Diagnose erschweren. Clevidipin könnte in Zukunft eine willkommene Erweiterung der intravenösen Therapieoptionen auch für neurologische Patienten darstellen.

Third nerve palsy is bilateral in only about 10% of cases, of which one in five cases is due to brainstem stroke. Bilateral oculomotor nerve palsy as an isolated clinical finding after brainstem stroke is extremely rare. We present a case of severe bilateral fascicular oculomotor nerve palsy due to distal basilar occlusion and subsequent midbrain infarction of cardioembolic origin. The patient required mechanical aids and subsequent ptosis surgery to relieve complete ptosis at least unilaterally.

The urgency and intensity of therapeutic response to a hypertensive crisis are governed by the presence or absence of acute end-organ damage, which define hypertensive emergency and hypertensive urgency, respectively. In case of hypertensive urgency a slow and moderate lowering of blood pressure by oral antihypertensive agents seems adequate, while the approach to hypertensive emergency has to be tailored to the specific type of organ failure. Optimal blood pressure management in the context of neurovascular emergencies is made difficult by contradictory data from observational and interventional studies. It might prove advantageous to individualize treatment according to characteristics such as the location of persistent vessel occlusion or the presence of collaterals. Reversible posterior encephalopathy may present with atypical features that might make diagnosis difficult. Clevidipine might be a welcome supplement to current intravenous antihypertensive agents in neurological disease.

Considering the causative or contributory effects of diabetes mellitus on common neurological diseases such as polyneuropathy, stroke and dementia, modern antidiabetic drugs may be expected to reduce incidence or progression of these conditions. Nevertheless, most observed benefits have been small, except in the context of therapy for diabetes mellitus type I and new-onset polyneuropathy. Recently, semaglutide, a GLP-1 analog, has been shown to significantly reduce stroke incidence in a randomized controlled trial. Beneficial effects of antidiabetic drugs on stroke severity or outcome have been controversial, though. The level of risk conferred by diabetes mellitus, the complex pathophysiology of neurological diseases, issues of trial design, side-effects of antidiabetic drugs as well as co-medication might be interacting factors that determine the performance of antidiabetic therapy with respect to neurological outcomes. It might be speculated that early treatment of prediabetes might prevent cerebral arteriosclerosis, cognitive decline or polyneuropathy more effectively, but this remains to be demonstrated.

Few movement disorders seem to make a straightforward approach to diagnosis and treatment more difficult and frustrating than myoclonus, due to its plethora of causes and its variable classifications. Nevertheless, in recent years, exciting advances have been made in the elucidation of the pathophysiology and genetic basis of many disorders presenting with myoclonus. Here, we provide a review of all of the important types of myoclonus encountered in pediatric and adult neurology, with an emphasis on the recent developments that have led to a deeper understanding of this intriguing phenomenon. An up-to-date list of the genetic basis of all major myoclonic disorders is presented. Randomized studies are scarce in myoclonus therapy, but helpful pragmatic approaches at diagnosis as well as treatment have been recently suggested.

Aufgrund ihrer variablen Phänomenologie und des vielfältigen ätiologischen Hintergrundes stellen Myoklonien und das Stiff-Person-Syndrom mit seinen Varianten oft eine Herausforderung in der Differenzialdiagnostik und Therapieplanung dar.

Findings from both cross-sectional and longitudinal studies have indicated a long preclinical period characterizing most neurodegenerative diseases. The study of presymptomatic movement disorders has implications for our understanding of their pathophysiology, early functional compensation and subclinical disease progression. This might lead to earlier diagnosis, might shift current disease staging and might help define at-risk populations amenable to preventive or disease-modifying therapeutic intervention. Here, we provide an up-to-date overview of the current knowledge about the preclinical stages of common movement disorders.

Rarely, not stroke but peripheral weakness can result from cervical artery dissection. In these cases, a mural hematoma compressing the ipsilateral C5 and/or C6 root can be demonstrated.

BACKGROUND:Deep brain stimulation has become an established therapy for various movement disorders but questions regarding its long-term effectiveness remain. OBJECTIVES:This study was designed to evaluate the long-term effectiveness of deep brain stimulation for movement disorders refractory to current medical therapy based on published long-term studies. METHODS:A review was carried out of all available studies with a minimum follow-up of 5 years of patients with deep brain stimulation for Parkinson's disease, essential tremor and dystonia. RESULTS:A total of 23 studies of deep brain stimulation for Parkinson's disease, 7 studies for essential tremor and 14 studies for dystonia were included. After a follow-up of at least 5 years, improvement of current motor scores could be observed in Parkinson's disease (subthalamic stimulation) by approximately 40%, by approximately 50% for essential tremor and by 60% for dystonia (mostly generalized forms). In Parkinson's disease, motor improvements tend to diminish over time due to progression of dysarthria, axial symptoms and other motor features less responsive to deep brain stimulation. Non-dopaminergic symptoms tend to progress and lessen the positive effects on the quality of life. There appears to be a subgroup of patients with essential tremor who show decreasing effectiveness of deep brain stimulation, probably related to disease progression. Currently, no single prognostic marker has been established to identify this subgroup. Most forms of secondary dystonia seem to respond more variably than primary generalized dystonia. CONCLUSION:Deep brain stimulation remains a relatively safe and effective therapy in carefully selected patients after long-term follow-up according to published data, although disease progression and other disease-specific factors seem to modify its effectiveness over time.

Die tiefe Hirnstimulation (THS) bei Bewegungsstörungen findet zunehmende Verbreitung, doch bestehen Unsicherheiten hinsichtlich der Langzeitwirksamkeit des Verfahrens.

Zu einem beliebigen Zeitpunkt bestehen bei jedem Zehnten Schmerzen an Nacken, Schulter oder Arm. Bei der Abklärung von nicht traumatischen, (sub)akuten Schmerzen des Schulter-Arm-Bereichs muss ein breites Spektrum möglicher Ursachen berücksichtigt werden. Die häufigsten Diagnosen im Überblick.

Neurologische Ursachen von Bewusstseinsstörungen sind häufig und erfordern in der Regel eine rasche Therapie. Kritisch für den optimalen Behandlungserfolg ist daher die schnelle und dennoch möglichst zuverlässige Differenzialdiagnose. Ein strukturiertes Vorgehen bei der Untersuchung in der Notfallsituation erleichtert dies. Bewusstseinsstörungen werden häufig in quantitative und qualitative Störungen eingeteilt. Wesentliche Komponente einer quantitativen Bewusstseinsstörung ist die Vigilanzminderung. Qualitative Bewusstseinsstörungen führen z. B. zu Verwirrtheitszuständen oder einem Delir. Für die Identifikation neurologischer Ursachen ist die sorgfältige Untersuchung von Begleitsymptomen kritisch. Da die Vigilanz wesentlich vom aszendierenden retikulären Aktivierungssystem im Hirnstamm gesteuert wird, sind insbesondere neurologische Symptome, die auf eine Hirnstammläsion schließen lassen, topodiagnostisch relevant. Darüber hinaus können neben beidseitigen Thalamusläsionen auch schwerwiegende raumfordernde Läsionen im Bereich der Hemisphären indirekt zu einer Hirnstammschädigung und damit zur Bewusstseinsstörung führen. Neben strukturellen Läsionen kommen als Ursache auch funktionelle Störungen in Betracht. Verwirrtheitszustände und delirante Bilder sind häufig auf mehrfaktorielle Ursachen zurückzuführen, meist in Kombination mit metabolischen oder medikamentösen Faktoren. Sie können aber auch auf nichtkonvulsiven Anfällen beruhen.

Purpose: Two of three patients with vertebrobasilar stroke harbor a stenosis of the vertebral or basilar arteries. The best treatment for secondary prophylaxis in vertebrobasilar occlusive disease has not been defined. In patients with high-grade stenoses, and especially those refractory to medication, stenting offers the chance to restore normal flow and prevent major strokes.Methods. We provide data regarding outcome and complications on 20 consecutive patients who underwent vertebrobasilar stenting at our institution (9 V0, 2V3, 5V4, and 4 basilar artery lesions). Furthermore, we provide a comprehensive overview of the literature on > 600 cases of vertebrobasilar stenting, including all published cases up to 2005.Results. Primary interventional success was achieved in all cases, with a mean residual stenosis of 3% +/- 4% in V0, 5% +/- 4% in V3/4, and 7% +/- 3% in basilar artery lesions. No peri-interventional neurologic complications and no transient ischemic attack or stroke at follow-up were noted in patients with vertebral ostial lesions, whereas two transient and three permanent clinical deteriorations occurred in patients with V4 or basilar artery lesions, some of which had presented with acute stroke. Patency rate was 100% at the last examination. According to published data on proximal vertebral artery stenting, mortality is 0.3%, the rate of neurologic complications is 5.5%, and the risk of posterior stroke at follow-up is 0.7%. Interventions for distal vertebral or basilar artery disease carry a 3.2% mortality risk, a 17.3% risk for neurologic complications and a 2% risk for stroke at follow-up.Conclusions. Stenting of the vertebral origin can be performed safely and with a low rate of cerebral ischemic events at follow-up, although restenosis may occur. Larger comparative trials are needed. Treatment decisions in distal vertebrobasilar disease have been made on an individual basis.