[Intensive care therapy of space-occupying large hemispheric infarction. Summary of the NCS/DGNI guidelines]. / Intensivtherapie des raumfordernden ischämischen Hemisphäreninfarkts. Zusammenfassung der NCS/DGNI-Leitlinie.

Large hemispheric infarction (LHI), synonymously called malignant middle cerebral artery (MCA) infarction, is a severe neurological disease with a high mortality and morbidity. Treating physicians as well as relatives are often faced with few and low quality data when attempting to apply optimal treatment to these patients and make decisions. While current stroke treatment guidelines focus on risk factors, prevention and acute management, they include only limited recommendations concerning intensive care management of LHI. The Neurocritical Care Society (NCS) and the German Society for Neurocritical and Emergency Medicine (DGNI) organized an interdisciplinary consensus conference on intensive care management of LHI to meet this demand. European and American experts in neurology, neurocritical care, neurosurgery, neuroradiology and neuroanesthesiology were selected based on their expertise and research focus. Subgroups for several main topics elaborated a number of central clinical questions concerning this topic and evaluated the quality of the currently available data according to the grading of recommendation assessment, development and evaluation (GRADE) guideline system. Subsequently, evidence-based recommendations were compiled after weighing the advantages against the disadvantages of certain management options. This is a commented abridged version of the results of the consensus conference.

[Case of atypical polypoid adenomyoma of the uterine cevix].

Atypical polypoid adenomyoma (APA) is a rare benign polypoid tumor occuring in young reproductive age women. There are no specific clinical features of APA. Grossly the tumor presents as a small polypoid lession in the low uterin cavum segment and cervical canal, mimicring infiltative endocervical or endometrial carcinoma. Microscopicaly APA consists of irregular glands, as endometrial with mild cellular atypia and diffuse smooth-mussle stroma. We present a case of APA of the uterine cervix in a young woman in regard to the diagnostical and treatment obstacles usually accompaning this rare pathology.

Sex differences in perihemorrhagic edema evolution after spontaneous intracerebral hemorrhage.

BACKGROUND AND PURPOSE: Clinical data on sex differences in perihemorrhagic edema (PHE) after intracerebral hemorrhage (ICH) are lacking. METHODS: Patients with spontaneous supratentorial ICH who had received standard conservative treatment were identified retrospectively. PHE development was assessed over a 14-day period on follow-up CT scans (days 1, 2-4, 5-7, 8-11, and 12-14). ICH and PHE volumes were assessed semiautomatically and compared between men and women. RESULTS: A total of 387 patients (233 men) were included. Starting at days 2-4, women showed lower PHE values (P < 0.05; days 2-4, 8-11) or a trend toward significance (P ≤ 0.1; days 5-7, 12-14). CONCLUSIONS: Female sex may predict lower PHE volumes.

Radiopacity of intracerebral hemorrhage correlates with perihemorrhagic edema.

BACKGROUND: Experimental evidence indicates that iron plays a key role in edema formation after intracerebral hemorrhage (ICH). We investigated the relationship between ICH radiopacity on CT as a marker of hematoma iron content and perihemorrhagic edema (PHE) after ICH. METHODS: We retrospectively investigated patients with spontaneous lobar and ganglionic supratentorial ICH who received follow-up CT scans during the first 7days after symptom onset (d1, d2-4, d5-7). Measurements of ICH and edema volumes were taken using a semiautomatic threshold-based volumetric algorithm. Radiopacity of the blood clot was determined using the mean Hounsfield unit (HU) count of the ICH. RESULTS: A total of 117 patients aged 71.92±11.55years with spontaneous ICH (34.63±32.44ml) were included in the analysis. Mean ICH radiopacity was 59.7±3.4HU. We found significantly larger relative PHE at d2-4 (1.7±0.9 vs. 1.3±0.8; P=0.032) and d5-7 (2.0±1.3 vs. 1.3±0.9; P=0.007) and larger peak relative PHE (2.3±1.6 vs. 1.6±1.1; P=0.006) in patients with ICH radiopacity >60HU (n=59), as compared to patients with ICH radiopacity <60HU (n=58). CONCLUSIONS: Higher ICH radiopacity, reflecting higher in vivo hematoma iron content, is associated with more PHE after ICH.

[Posterior reversible encephalopathy syndrome]. / Posteriores reversibles Enzephalopathiesyndrom.

Posterior reversible encephalopathy syndrome (PRES) is a clinically and radiologically defined syndrome characterized by seizures, headaches, visual disturbances, and altered mental status or focal neurological deficits. Imaging frequently shows abnormalities in the posterior brain regions, especially the occipital and the parietal lobes. PRES has been described in association with a wide spectrum of underlying causes. The most common conditions include hypertension, renal disease, preeclampsia/eclampsia, or status post transplantation. The pathophysiology of PRES has not been sufficiently elucidated as yet; however, endothelial damage caused by different mechanisms possibly plays a central role. The prognosis of PRES is usually benign when intensive care and withdrawal of the causative agent is instituted early. Delay in diagnosis and treatment may lead to complications and permanent neurological deficits. The clinical and radiological landmarks of this syndrome as well as causes, pathophysiological hypotheses, and the basic therapeutic principles of PRES are the subject of the present review.

[Management of intracerebral hemorrhage: can we still learn something?]. / Management intrazerebraler Blutungen : Lernen wir dazu?

Intracerebral hemorrhage (ICH) is the most devastating form of stroke. It affects approximately 2 million people worldwide every year and is a major cause of mortality and morbidity. Despite the focus of intensive scientific research on ICH for decades there is still no proven treatment strategy for this disease. Advances in knowledge on the underlying pathomechanisms of ICH and the clinical impact have contributed to the development of novel treatment approaches. Currently, surgical treatment, aggressive blood pressure management and intraventricular fibrinolysis in patients with additional severe intraventricular hemorrhage are being investigated in large scale phase III clinical trials.

Single versus bilateral external ventricular drainage for intraventricular fibrinolysis in severe ventricular haemorrhage.

BACKGROUND: Intraventricular fibrinolysis (IVF) through bilateral external ventricular drains (EVD) may provide better access of the thrombolytic agent to the intraventricular clot, potentially influencing clot clearance and outcome. METHODS: Patients with spontaneous ganglionic intracerebral haemorrhage (ICH)<40 cm(3) and intraventricular haemorrhage (IVH) with acute hydrocephalus have been treated with IVF. The decision for placement of one or two EVDs has been left to the discretion of the treating physician. CT volumetry, the effects on cerebrospinal fluid (CSF) circulation and outcome at 3 months have been analysed for patients with one (group I, n = 13) or two EVDs (group II, n = 14). RESULTS: No difference was found in clot resolution between the two groups (clot half life 2.1 (SD 1.2) vs 2.4 (1.3) days). A separate analysis of the third and fourth ventricle clearance was similar (1.6 (0.6) versus 1.8 (0.8) days), indicating no difference in reconstitution of CSF circulation. A trend towards a longer EVD duration and higher infection rate was found in the bilateral EVD group. No difference was found in outcome at 3 months. CONCLUSIONS: Our results do not support the use of bilateral EVDs for IVF in patients with severe IVH.

In vivo detection of hepatitis C virus (HCV) RNA in the brain in a case of encephalitis: evidence for HCV neuroinvasion.

We report here a 27-year-old woman who presented with encephalitis of unknown origin. Magnetic resonance imaging (MRI) of the brain revealed leukoencephalopathy, cerebrospinal fluid showed signs of inflammation. Serum and brain biopsy tissue was tested positive for hepatitis C virus (HCV). Neuropathological investigation supported the hypothesis of viral encephalitis. C3, C4 and cryoglobulins as well as cerebral MR-angiography were normal. Neurological complications of HCV infection other than hepatic encephalopathy are generally attributed to parainfectious phenomena. This is the first case of HCV-RNA detection in vivo in human brain in literature and it raises the possibility that HCV is able to induce encephalitis caused by neurotrophism. This is supported by the fact that there is a growing body of literature on HCV-induced cerebral dysfunction and laboratory findings indicating HCV neuroinvasion.

Acute onset of fatal vegetative symptoms: unusual presentation of adult Alexander disease.

Since genetic analysis of the GFAP gene for the diagnosis of adult Alexander disease (AD) has been established in 2001, several cases of both sporadic and familial cases of AD have been described. Except for one patient, all subjects revealed glial fibrillary acidic protein (GFAP) mutations, and clinical progression of symptoms, mainly bulbar and pseudobulbar, were moderate. Here we report on a patient with acute onset of vegetative symptoms, rapid progression, and death within 2 months. Although histology and final magnetic resonance imaging (MRI) were characteristic of AD, sequencing of the encoding GFAP gene revealed no mutation. We believe that this case report expands the so far known clinical spectrum and MRI dynamics of adult AD, and suggest that analysis of the coding part of GFAP may be inconclusive in rare cases. In such patients, only histology may lead to definitive diagnosis.

[New treatment strategies for intraventricular hemorrhage]. / Neue Therapiestrategien bei zerebraler intraventrikulärer Blutung.

The presence of additional intraventricular hemorrhage (IVH) in patients with intracerebral hemorrhage (ICH) is associated with a much higher mortality and worse functional outcome. Although evidence-based specific treatment options for this entity are still lacking, knowledge about the pathophysiology of IVH has grown in recent decades, leading to the development of promising treatment strategies. Intraventricular fibrinolysis (IVF) accelerates IVH resolution and removal from the ventricular system. The additional usage of lumbar drains probably reduces the incidence of permanent posthemorrhagic hydrocephalus. The influence of these treatment modalities on functional outcome is currently being investigated in ongoing studies. The present article gives an overview of pathophysiological and clinical aspects of IVH, emphasizing novel treatment options.

Therapeutic hypothermia for space-occupying Herpes simplex virus encephalitis.

Development of space-occupying edema in patients with severe Herpes simplex virus encephalitis is a major factor for high morbidity and mortality. Conventional intracranial pressure-lowering modalities are limited and more aggressive treatment options for such patients have rarely been described. This article reports on a HIV-positive patient with Herpes simplex type 2 encephalitis and elevated intracranial pressure refractory to medical therapy. Induced moderate hypothermia of 33 °C resulted in fast and sustained control of intracranial pressure. After three months, the patient had a fairly good functional outcome with a Glasgow Outcome Scale score of 4.

[Treatment of intraventricular hemorrhage and hydrocephalus]. / Behandlung von intraventrikulären Blutungen und Hydrozephalus.

Most cases of intraventricular hemorrhage (IVH) occur secondary to spontaneous intracerebral or subarachnoid hemorrhage. The main concern is development of hydrocephalus, which is related to a poor prognosis. Over the last years, several treatment options for IVH have been introduced, but prospective data regarding the efficacy of those therapies (external ventricular drainage, intraventricular fibrinolysis, lumbar drainage, endoscopic hematoma evacuation) do not yet exist. This review focuses on combined therapy using an external ventricular drain and intraventricular fibrinolysis with r-TPA for IVH-associated initial occlusive hydrocephalus. Moreover, a continuing treatment strategy for persistent malresorptive communicating hydrocephalus using lumbar drainage is described.

Optic nerve compression analyzed by using plastination.

Plastination is an excellent tool for studying different anatomical and clinical questions. The E12 technique provides transparent slices which show the anatomical structures in their initial position, especially those of the muscular, vascular and interstitial tissue. Here we demonstrate on a plastinated specimen optic nerve compression due to hypertrophy and degeneration of the extraocular muscles near the apex of the orbit. The paper aims to demonstrate the possibilities offered by the E12 plastination technique in analyzing anatomical structures. The plastinated slices obtained were scanned and then analyzed. In a further step the plastinated slices were cut into thin slices (150 microm), stained and finally examined histologically. We measured the intraorbital length (A-B), which had an average value of 25,93+/-0,03 mm. Dividing the optic nerve into uncompressed (UP) and compressed (CP) parts, we obtained the following values for optic nerve width: UP=4192+/-0,455 mm, CP=3215+/- 0,411 mm. Using the E12 plastination method we were able to take morphometric measurements on plastinated orbital slices with coincidentally detected optic nerve compression. This allowed the determination of different parameters of the optic nerve, as well as histological examination by hematoxylin and eosin staining up to a magnification of x40.