Purpose: To differentiate PCA segments and cortical branches by means of transcranial colorcoded duplex sonography (TCCD) and to measure flow parameters at rest and during visual stimulation.Materials and Methods: 60 healthy subjects with a good acoustic temporal bone window were examined. The main stem of the PCA (P1, P2 and P3) and 4 main cortical branches - the anterior temporal artery (ATA), the occipital temporal artery (OTA), the parietooccipital artery (POA) and the calcarine artery (CA) - were assessed using an axial transtemporal approach. Systolic and diastolic blood flow velocities (BFVs) were recorded at rest and during visual stimulation.Results: Identification of the P1 segment of the PCA was successful in 97.5 % (117/120) of cases. The P2 and P3 segments were visualized in all cases. The 4 main cortical branches could be identified to varying degrees: ATA in 88 %, OTA in 96 %, POA in 69 % and CA in 62 %. There was an evoked flow response in the P2 main stem and in all cortical branches. The most pronounced increase in diastolic/systolic BFV after visual stimulation test was seen in the CA (42 %/35 %), followed by P2 (30 %/24 %), the POA (27 %/27 %), the OTA (16 %/13 %) and the ATA (9 %/8 %).Conclusion: Insonation through the temporal bone window with TCCD confidently allows the assessment of the P1 to P3 segments of the PCA as well as the 2 proximal branches, the ATA and the OTA. An ultrasound- based classification of PCA anatomy and its cortical branches may be used as a noninvasive method for the evaluation of posterior circulation pathology.

In 2006, Zamboni re-introduced the concept that chronic impaired venous outflow of the CNS is associated with multiple sclerosis (MS), coining the term of chronic cerebrospinal venous insufficiency ("CCSVI"). The diagnosis of "CCSVI" is based on sonographic criteria, which he found exclusively fulfilled in MS. The concept proposes that chronic venous outflow failure is associated with venous reflux and congestion and leads to iron deposition, thereby inducing neuroinflammation and degeneration. The revival of this concept has generated major interest in media and patient groups, mainly driven by the hope that endovascular treatment of "CCSVI" could alleviate MS. Many investigators tried to replicate Zamboni's results with duplex sonography, MRI, and catheter angiography. The data obtained here do generally not support the "CCSVI" concept. Moreover, there are no methodologically adequate studies to prove or disprove beneficial effects of endovascular treatment in MS. This review gives a comprehensive overview of the methodological flaws of the ultrasound based "CCSVI" concept. In our view, there is currently no basis to diagnose or treat "CCSVI" in the care of MS patients.

Recently, chronic cerebrospinal venous insufficiency ("CCSVI") was suggested as a new pathogenetic hypothesis in multiple sclerosis (MS) by Zamboni and colleagues: impairment of the cerebrocervical venous outflow by venous stenoses or other vessel obstructions should lead to stasis in cerebral veins with transendothelial extravasation of erythrocytes and subsequent inflammatory reactions in brain tissue. Based on this hypothesis and their own sonographic and angiographic data, therapeutic interventions by means of angioplasty were proposed as treatment for MS by Zamboni et al.. The "CCSVI" hypothesis, termed "the big idea" by Zamboni and colleagues, has caused substantial interest in the scientific community as well as amongst patients, caregivers, and the mass media. However, an independent confirmation of the data published by Zamboni et al. is lacking, while the number of negative studies with various techniques that question the "CCSVI" hypothesis is steadily increasing. The aim of this review is to delineate the "CCSVI" hypothesis and to summarise the data published by Zamboni et al. and subsequent investigators which mostly refute this hypothesis. According to the current evidence, there is no scientific plausibility of the "CCSVI" hypothesis and no further data that support this pathogenetic concept. Thus, there is no justification for any angioplastic therapies in MS patients.

Kürzlich wurde die sog. chronische zerebrospinale venöse Insuffizienz („CCSVI“) als neue Hypothese zur Pathogenese der Multiplen Sklerose (MS) von Zamboni et al. postuliert: Hiernach führt eine Behinderung des zerebrozervikalen venösen Abstroms zu einer Stase in zerebralen Venen und zu einer transendothelialen Extravasation von Erythrozyten mit nachfolgender Entzündungsreaktion im Gewebe. Basierend auf dieser Hypothese und eigenen sonografischen sowie angiografischen Daten wurden therapeutische Interventionen mittels Angioplastie zur Behandlung der MS von Zamboni et al. propagiert. Die „CCSVI“-Hypothese, von ihren Erfindern als „die große Idee“ bezeichnet, hat beträchtliches Interesse nicht nur unter Wissenschaftlern und Ärzten, aber auch bei Patienten, Angehörigen und in den Massenmedien erzeugt. Bislang fehlt jedoch eine Reproduktion der Ergebnisse durch unabhängige Arbeitsgruppen, und die Zahl der negativen Publikationen, die mit verschiedenen Verfahren die „CCSVI“-Hypothese in Frage stellen, steigt kontinuierlich. Diese Übersichtsarbeit fasst die „CCSVI“-Theorie und die Zamboni-Daten zusammen und stellt diesen die neueren, überwiegend kritischen Arbeiten gegenüber. Nach dem gegenwärtigen Stand des Wissens gibt es keine wissenschaftliche Plausibilität für die „CCSVI“-Hypothese und keine weiteren Daten, die diese stützen würden. Daher gibt es keine Indikation und Rechtfertigung für angioplastische Interventionen bei Patienten mit MS.

Background: Chronic cerebrospinal venous insufficiency (CCSVI) was proposed as the causal trigger for developing multiple sclerosis (MS). However, current data are contradictory and a gold standard for venous flow assessment is missing. Objective: To compare structural magnetic resonance venography (MRV) and dynamic extracranial color-coded duplex sonography (ECCS) in a cohort of patients with MS. Methods: We enrolled 40 patients (44 ± 10 years). All underwent contrast-enhanced MRV for assessment of internal jugular vein (IJV) and azygos vein (AV) narrowing, graded into 3 groups: 0%–50%, 51%–80%, and >80%. ECCS analysis of blood flow direction, cross-sectional area (CSA), and blood volume flow (BVF) in both IJV and vertebral veins (VV) occurred in the supine and upright body position. Results: MRV identified 1 AV narrowing. IJV analysis yielded 12 patients for group 1 (30%), 19 patients for group 2 (48%), and 9 patients for group 3 (22%). By ECCS criteria, 4 patients (10%) presented with venous drainage abnormalities. Jugular BVF was different only between groups 1 and 3 (616 ± 133 vs 381 ± 213 mL/min, p = 0.02). No other parameters in supine position and none of the parameters in the upright body position, apart from the IJV-BVF decrease in groups 1 and 3 (479 ± 172 vs 231 ± 144 mL/min, p = 0.01), were different. Conclusions: Our ECCS data contradict the postulated 100% prevalence of CCSVI criteria in MS. MRV seems more sensitive to detect IJV narrowing compared to ECCS. A measurable hemodynamic effect only exists in vessel narrowings >80%. Our combined data argue against a causal relationship of venous narrowing and MS, favoring the rejection of the CCSVI hypothesis.

Fixed dystonic postures are rare manifestations in idiopathic or symptomatic dystonias. So far, fixed dystonia has only been reported in a few patients with advanced idiopathic dystonia. It has, however, more often been observed in patients with symptomatic dystonia who have lesions in the basal ganglia or as a symptom in neurodegenerative diseases. Conversely, fixed dystonia represents the key symptom in a group of movement disorders whose nosological and pathogenetic allocations has been subject to a controversial debate over the past 20 years. These movement disorders encompass post-traumatic fixed dystonia, the complex regional pain syndrome with fixed dystonia and the causalgia-dystonia syndrome. The core of the controversy relates to the question whether fixed dystonia - with or without preceding trauma - is to be classified as a primarily psychogenic disease or whether fixed dystonia has a primarily organic aetiology. In the largest series of patients with fixed dystonia so far In the authors observed that a very high percentage of patients had a psychiatric co-morbidity - a finding which, however, does not prove a purely psychogenic aetiology. Here we present the case of a 39-year-old woman with a generalised fixed dystonia whose history highlights the most important aspects of the controversy in an exemplary fashion.

Fixierte dystone Körperhaltungen zählen zu den seltenen Manifestationen bei idiopathischen und symptomatischen Dystonien. Bislang wurde eine fixierte Dystonie bei wenigen Patienten mit einer fortgeschrittenen idiopathischen Dystonie beschrieben, etwas häufiger dagegen bei symptomatischen Dystonien mit Läsionen in den Basalganglien oder im Rahmen neurodegenerativer Erkrankungen. Demgegenüber stellt eine fixierte dystone Körperhaltung das Kernsymptom einer Gruppe von Bewegungsstörungen ohne nachweisbare zerebrale Schädigung dar, deren nosologische und pathogenetische Zuordnung in den letzten 20 Jahren kontrovers debattiert wird. Hierzu zählen die posttraumatische fixierte Dystonie, das komplexe regionale Schmerzsyndrom mit fixierter Dystonie und das Kausalgie-Dystonie-Syndrom. Der Kern der Kontroverse berührt die Frage, ob es sich bei der fixierten Dystonie – mit oder ohne vorausgegangenem Trauma – um eine primär psychogene oder um eine primär organische Erkrankung handelt. In der bislang größten publizierten Fallserie mit 103 Patienten [3] kamen die Autoren zu dem Schluss, dass ein hoher Prozentsatz der Patienten mit isolierter fixierter Dystonie eine psychiatrische Komorbidität aufweist, ohne dass diese Beobachtung jedoch die psychogene Ätiologie allein bewiese. Wir präsentieren hier den Fall einer 39-jährigen Frau mit einer generalisierten fixierten Dystonie, der verschiedene Aspekte der Kontroverse exemplarisch beleuchtet.

Hintergrund: Die Pathogenese der Migräne ist bis heute nicht verstanden. Derzeit existieren unterschiedliche Theorien zu diesem Thema. Unbestritten liegt zumindest eine durale Mitbeteiligung vor. Unter der Annahme, dass den Dura versorgenden Gefäßen eine wichtige Rolle in der pathophysiologischen Kaskade der Migräne zukommt, untersuchten wir mithilfe der extra- und transkraniellen Duplexsonografie (ECCS und TCCS) systematisch die A. meningea media (MMA) und die A. maxillaris (MA), wofür bislang keine Daten publiziert sind.

PURPOSE:The effect of transcranial duplex ultrasound (US) on the intraventricular temperature in patients was analyzed. Temperature increases during examination have been identified as a potential risk factor but only data from model studies is currently available.MATERIALS AND METHODS:Patients who had an intracranial pressure/temperature transducer implanted and underwent US assessment were included. In an examination series (B-mode, combined B- and color mode, combined B- and color mode plus Doppler, 3 min for each mode), the intracranial thermodilution thermistor was focused while intraventricular temperature and body temperature (bladder catheter or rectal probe) were recorded continuously and temperature changes were analyzed.RESULTS:Thirty-one US examinations were performed in 14 patients. Twenty-six examinations in 9 patients in which the intracranial temperature probe was depicted were included. Initial patient temperatures ranged from 35.1dgC to 38.7dgC. No significant increase or decrease in intracranial temperature was seen after the first (B-mode), second (B- and color mode) and third (B- and color mode plus Doppler) duplex US examination. T-test for paired samples showed a constant temperature throughout US examination (two-sided significance: 1.000, 1.000, 0.731).CONCLUSION:Routine transcranial duplex ultrasound does not increase the intracranial temperature in patients.

Hintergrund: Die Möglichkeit, mit Ultraschall (US) Veränderungen der Blut-Hirn-Schranke (BHS) zu induzieren, ist bekannt. In letzter Zeit wurden im Rahmen von Studien zur Sonothrombolyse und zu US-induzierten Veränderungen der zerebralen Hämodynamik Blutungskomplikationen und Störungen der BHS berichtet.

The pathophysiology of primary exertional headache (EH) is unknown. Physical exertion is associated with Valsalva-like manoeuvres (VM). VM leads to increased intrathoracic pressure and reduces cerebral venous drainage. Internal jugular vein valve incompetence (IJVVI) leads to retrograde venous flow during VM with transient increase of intracranial pressure. We analysed the prevalence of IJVVI in EH patients using duplex ultrasound. Bilateral measurements were performed at rest and during VM in 20 patients and 40 controls. Incompetence was concluded if retrograde venous flow could be seen in the jugular Doppler spectrum during repeated VM. Seventy percent of EH patients and 20% of controls demonstrated IJVVI, yielding a significant difference (P = 0.0004). IJVVI was always observed on the dominant venous drainage side. Our study suggests that intracranial venous congestion caused by retrograde jugular venous flow might play a role in the pathophysiology of EH with IJVVI as a risk factor.

Hintergrund: Der Pathomechanismus des benignen Anstrengungskopfschmerz (BAK) ist weitgehend ungeklärt. Physische Anstrengung – der Auslöser des BAK – ist oft mit einem Valsalva-Manöver (VM) verbunden. Durch das VM kommt es zu einem kurzzeitigen Anstieg des intrathorakalen Druckes und in der Folge zu einem Abfall des venösen Rückstroms aus dem Gehirn. Im Falle einer Klappeninkompetenz der Vena jugularis interna (KI-VJI) kommt es unter VM zu einem retrograden venösen Blutfluss mit verstärktem Anstieg des intrakraniellen Druckes. Ziel unserer Studie war es, die KI-VJI-Prävalenz bei Patienten mit BAK duplexsonographisch zu untersuchen.

The purpose of this study was to identify the ophthalmic artery (OA) by means of transcranial color-coded sonography using a new approach via the transtemporal bone window and to compare blood flow measurements with values obtained from the transorbital approach. A total of 105 subjects were included. Two individuals had an insufficient unilateral bone window, according to our inclusion criteria. Of the remaining 208 OAs, 206 (99%) could successfully be insonated via the transorbital approach and 190 (91%) via the transtemporal approach. Transorbital systolic and diastolic flow velocity (FVs) ranged between 15 and 67 cm/s (mean +/- SD: 35 +/- 10) and 4 to 36 cm/s (14 +/- 5), pulsatility index ranged between 0.4 to 2.8 (1.2 +/- 0.4). Transtemporal systolic and diastolic FVs ranged between 15 and 58 cm/s (33 +/- 9) and 4 to 25 cm/s (11 +/- 4). Transorbital and transtemporal FVs correlated significantly (p = 0.01). Our study demonstrates that transtemporal insonation of the proximal intracranial OA is feasible. Whenever OA assessment for analysis of collateral function is required, this simple approach, avoiding direct eye bulb insonation, should be considered.

Hintergrund: Die Sichelzellanämie (SCA) ist bei Kindern und Jugendlichen durch die Entwicklung intrakranieller Stenosen mit einem erhöhten Schlaganfallrisiko assoziiert. Das Monitoring der Blutflussgeschwindigkeiten (BFG) mittels transkranieller Doppler- oder Duplexsonographie (TCCS) gilt bei diesen Patienten als Methode der Wahl, um Stenosen frühzeitig zu entdecken. Ein Anstieg der BFG kann jedoch nicht nur auf Stenosen, sondern auch auf eine Hyperperfusion im Rahmen der Anämie zurückzuführen sein. Deshalb wurden in dieser Studie mittels TCCS die gemessenen intrakraniellen BFG dem globalen zerebralen Blutflussvolumen (CBF) gegenübergestellt.

Hintergrund: Die klinische Differentialdiagnose zwischen einem idiopathischen Parkinsonsyndrom (IPS) und einem essentiellen Tremor (ET) kann in der Frühphase der Erkrankung Schwierigkeiten bereiten. In diesen Fällen lässt sich mittels FP-CIT-SECT eine etwaige nigrostriatale Dysfunktion sicher beurteilen. Die Transkranielle Sonographie (TCS) zeigt in etwa 90% der Patienten mit IPS eine charakteristische Hyperechogenität der Substantia nigra (SN), die bei Patienten mit ET bisher nicht beschrieben wurde. In der vorliegenden Studie sollte der Einsatz der TCS bei unklaren Tremorsyndromen direkt mit dem FP-CIT verglichen werden.

We report a case of multiple brain abscesses (BAs) in a 67-year-old man with symptoms of progredient disorientation and amnestic aphasia. Onset of symptoms occurred one week after surgical treatment of a perianal abscess. No other source of infection was identified and the abscesses were limited to the brain. The immune status was normal but a patent foramen ovale (pFO) was found. The patient was treated with high-dose antibiotics, leading to a complete radiological disappearance of the BAs. Hematogenous spread of infectious emboli from a perianal focus exclusively to the brain is very rare. In our patient, the mechanisms of infectious spread into the brain might have occurred via a cardiac right-to-left shunt or alternatively via the non-valvular vertebral venous system. In this manuscript, both pathways are critically reviewed.