Temporal lobe epilepsy due to meningoencephaloceles into the greater sphenoid wing: a consequence of idiopathic intracranial hypertension?

PURPOSE: Antero-inferior temporal lobe meningoencephaloceles are a rare, but increasingly recognized cause of drug-resistant temporal lobe epilepsy (TLE). In order to evaluate whether these lesions are related to idiopathic intracranial hypertension (IIH), we analyzed clinical and MRI findings of a cohort of patients undergoing presurgical work-up. METHODS: Seizure onset in the anterior temporal lobe was proven by EEG electrodes in 22 patients, and in 21 patients, anterior temporal lobectomy (mostly with sparing of the hippocampus) was performed. MRI signs of IIH (in particular empty sella) and the volumes of the ventricles and external CSF spaces were determined and related to the body mass index (BMI) and clinical outcome. RESULTS: Six of seven obese (BMI > 30 kg/m2) compared to four of 15 non-obese patients had partial empty or empty sella (p = 0.007). Bilateral lesions were found in all obese and 11 patients. Seizure freedom (Engel class 1A) was achieved in 12 of 21 patients (5 obese compared to 7 non-obese patients). BMI was related to the volume of the external CSF spaces (r = 0.467), and age at seizure onset was higher in obese patients. CONCLUSION: Roughly a third of patients with temporal lobe epilepsy due to antero-inferior meningoencephaloceles is obese and has MRI signs of idiopathic intracranial hypertension.

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis.

INTRODUCTION: Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. METHODS: Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. RESULTS: SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). CONCLUSION: SWMB is a newly described MRI sign rather specific for VGKC-LE.

MRI artefacts after Bonebridge implantation.

The new transcutaneous bone conduction implant (BCI) Bonebridge (BB, MED-EL) allows the skin to remain intact and therefore overcomes some issues related to percutaneous systems, such as skin reaction around the external screw and cosmetic complaints. According to manufacturer, BB is MRI conditional up to 1,5 Tesla (T). The artefact of the neurocranium after BB implantation is extensive as shown in the present report. This has to be taken into account when patients suffering conductive, mixed or single-sided hearing loss with candidacy for a BCI are counselled. In patients with comorbid intracranial tumour or other diseases of the brain that require imaging control scans with MRI percutaneous, BCI should be the implant of choice considering the very small artefact of the percutaneous screw in MRI.

Superior neurologic recovery after 15 minutes of normothermic cardiac arrest using an extracorporeal life support system for optimized blood pressure and flow.

OBJECTIVE: Sudden cardiac arrest is one of the leading causes of death. Conventional CPR techniques after cardiac arrest provide circulation with reduced and varying blood flow and pressure. We hypothesize that using pressure- and flow-controlled reperfusion of the whole body improves neurological recovery and survival after 15 min of normothermic cardiac arrest. METHODS: Pigs were randomized in two experimental groups and exposed to 15 min of ventricular fibrillation (VF). After this period, the animals in the control group received conventional CPR with open chest compression (n=6), while circulation in the treatment group (n=6) was established with an extracorporeal life support system (ECLS) to control blood pressure and flow. Follow-up included the assessment of neurological recovery and magnetic resonance imaging (MRI) for up to 7 days. RESULTS: Five of the six animals in the control group died, one animal was resuscitated successfully. In the treatment group, 1/6 could not be separated from ECLS. Five out of the six pigs survived and were transferred to the animal facility. One animal was unable to walk and had to be sacrificed 30 hours after ECLS. The remaining 4 animals of the treatment group and the surviving pig from the control group showed complete neurological recovery. Brain MRI revealed no pathological changes. CONCLUSION: We were able to demonstrate a significant improvement in survival after 15 minutes of normothermic cardiac arrest. These results support our hypothesis that using an ECLS for pressure- and flow-controlled circulation after circulatory arrest is superior to conventional CPR.

[Expectoration of embolization coils 15 years after embolization of pulmonary arteriovenous malformations in a patient with hereditary hemorrhagic telangiectasia]. / Expektoration von Embolisationsspiralen 15 Jahre nach Embolisation pulmonaler arteriovenöser Malformationen bei hereditärer hämorrhagischer Teleangiektasie (M. Osler-Weber-Rendu).

Hereditary hemorrhagic telangiectasia can manifest itself with pulmonary arteriovenous malformations (pavm). A transcatheter coil embolization should be made to avoid complications and to close off relevant arteriovenous shunts. We report on a patient with expectoration of embolization coils 15 years after embolotherapy. In case of hemoptysis following embolotherapy with coils, even years after their placement one should consider coil migration into the pulmonary system, besides newly formed pavms, in the differential diagnosis and initiate contrast-CT of the thorax and bronchoscopy.

[Pulmonary neuroendocrine neoplasms]. / Neuroendokrine Neoplasien der Lunge.

The pulmonary neuroendocrine neoplasms originate from the enterochromaffin cells which are diffusely distributed in the body. The incidence of these tumors has increased significantly in recent decades due to the available diagnostics. They make up about 1-2% of all lung tumors and 20-30% of all neuroendocrine neoplasms. The current WHO classification from 2004 divides them into typical carcinoids (TC), atypical carcinoids (AC), large cell neuroendocrine carcinomas (LCNEC) and small cell carcinomas (SCLC). The major neuroendocrine biomarkers are chromogranin A, synaptophysin and CD56. TC have a low mitotic rate of <2 mitoses/2mm(2) (10 HPF), whereas the mitotic rate of the AC is 2-10 mitoses/2 mm(2) (10 HPF). The Ki-67 staining is helpful to distinguish typical and atypical carcinoids from the highly malignant LCNEC and SCLC. Clinically, the patient presents usually with cough, hemoptysis or bronchial obstruction. The occurrence of a carcinoid or Cushing's syndrome and a tumor-associated acromegaly are rare. Surgical resection with radical lymph node dissection is the treatment of choice for achieving long-term survival. Endoscopic resection of the endobronchial tumor growth is a good alternative for inoperable endobronchially localized tumors. Peptide receptor radionuclide therapy (PRRT) is a promising treatment option for patients with metastatic or unresectable pulmonary neuroendocrine tumors. New targeted therapies using angiogenesis inhibitors, mTOR inhibitors, and tyrosine kinase inhibitors are being tested for their effectiveness in many previous studies. Typical carcinoid tumors metastasize less frequently than AC, the 5-year survival rate of patients with TC being over 90%. Patients with AC have a 5-year survival rate between 35% and 87%. The highly malignant LCNEC and SCLC, on the other hand, have a 5-year survival rate between 15% and 57%, and <5% respectively. The increasing number of therapeutic options and diagnostic procedures requires a multidisciplinary approach and decision-making in multidisciplinary tumor conferences to ensure a personalized treatment approach. Therefore patients with a neuroendocrine neoplasm of the lung should be treated in specialized centers.

Prolonged cardiac arrest and resuscitation by extracorporeal life support: favourable outcome without preceding anticoagulation in an experimental setting.

State-of-the-art cardiopulmonary resuscitation (CPR) restores circulation with inconsistent blood-flow and pressure. Extracorporeal life support (ECLS) following CPR opens the opportunity for "controlled reperfusion". In animal experiments investigating CPR with ECLS, systemic anticoagulation before induced cardiac arrest is normal, but a major point of dispute, since preliminary heparinization in patients undergoing unwitnessed cardiac arrest is impossible. In this study, we investigated options for ECLS after an experimental 15 minutes normothermic cardiac arrest, without preceding anticoagulation, in pigs. Neurological recovery was assessed by a scoring system, electroencephalography and brain magnetic resonance imaging. Additionally, brain histology was performed on day seven after cardiac arrest. We demonstrated that preliminary heparin administration was not necessary for survival or neurological recovery in this setting. Heparin flushing of the cannulae seemed sufficient to avoid thrombus formation. These findings may ease the way to using ECLS in patients with sudden cardiac arrest.

Compound heterozygosity of the protein S-gene as a cause of severe cerebral sinovenous thrombosis in a 7-year-old child.

The genotype-phenotype relationship of compound heterozygous protein S-deficiency in a 7-year-old girl with reduced protein S-levels and a severe cerebral sinovenous thrombosis is illustrated. In this patient we identified a novel deletion in the protein S-gene causing a compound heterozygous state and subsequently a symptomatic protein S-deficiency. In case of thrombosis analysis of protein S is recommended. Low levels of protein S should be further investigated by molecular diagnostics.

Epilepsy surgery in patients with multiple cerebral cavernous malformations.

PURPOSE: Cerebral cavernous malformations (CCMs) are frequently associated with intractable epilepsy. Whereas surgery indication in single CCMs is clear, data regarding the efficacy of epilepsy surgery in patients with multiple CCMs are scarce. We sought to clarify diagnostic requirements and postoperative outcome in patients with multiple CCMs and refractory epilepsy. METHODS: Retrospective analysis of clinical records of hospitalized patients who underwent comprehensive diagnostic work-up including long-term video-EEG monitoring. RESULTS: From a total of 63 consecutive patients with CCMs and medically refractory epilepsy, 11 (17%) had multiple CCMs and underwent epilepsy surgery. There were three females and eight males. Mean age at epilepsy onset was 28.3 years (S.D. 12.3), and at epilepsy surgery, 40.7 years (S.D. 10.3). On average, each patient had 3.7 (S.D. 2.2) supratentorial CCMs. In all cases we identified only one epileptogenic zone. The epileptogenicity was higher for the CCMs located within the temporal lobe. At 2 years follow-up, the outcome according to the Engel classification was Ia (seizure-free) in nine patients (81.8%) and IIb (rare seizures) and IVc (worsening) in two patients, respectively. In one patient, a dual pathology was present and, in another case, de novo appearance of CCMs was demonstrated. CONCLUSIONS: Our results show that postoperative outcome in patients with multiple CCMs can be as good as in those with single malformations if proper presurgical identification of the epileptogenic CCMs is done. The possibility of the novo appearance of CCMs or dual pathology may occur and may affect long-term outcome negatively.

Connecting and merging fibres: pathway extraction by combining probability maps.

Probability mapping of connectivity is a powerful tool to determine the fibre structure of white matter in the brain. Probability maps are related to the degree of connectivity to a chosen seed area. In many applications, however, it is necessary to isolate a fibre bundle that connects two areas. A frequently suggested solution is to select curves, which pass only through two or more areas. This is very inefficient, especially for long-distance pathways and small areas. In this paper, a novel probability-based method is presented that is capable of extracting neuronal pathways defined by two seed points. A Monte Carlo simulation based tracking method, similar to the Probabilistic Index of Connectivity (PICo) approach, was extended to preserve the directional information of the main fibre bundles passing a voxel. By combining two of these extended visiting maps arising from different seed points, two independent parameters are determined for each voxel: the first quantifies the uncertainty that a voxel is connected to both seed points; the second represents the directional information and estimates the proportion of fibres running in the direction of the other seed point (connecting fibre) or face a third area (merging fibre). Both parameters are used to calculate the probability that a voxel is part of the bundle connecting both seed points. The performance and limitations of this DTI-based method are demonstrated using simulations as well as in vivo measurements.

Gibbs tracking: a novel approach for the reconstruction of neuronal pathways.

Reconstruction of neuronal fibers using diffusion-weighted (DW) MRI is an emerging method in biomedical research. Existing fiber-tracking algorithms are commonly based on the "walker principle." Fibers are reconstructed as trajectories of "walkers," which are guided according to local diffusion properties. In this study, a new method of fiber tracking is proposed that does not engage any "walking" algorithm. It resolves a number of inherent problems of the "walking" approach, in particular the reconstruction of crossing and spreading fibers. In the proposed method, the fibers are built with small line elements. Each line element contributes an anisotropic term to the simulated DW signal, which is adjusted to the measured signal. This method demonstrates good results for simulated fibers. A single in vivo result demonstrates the successful reconstruction of the dominant neuronal pathways. A comparison with the diffusion tensor imaging (DTI)-based fiber assignment with continuous tracking (FACT) method and the probabilistic index of connectivity (PICo) method based on a multitensor model is performed for the callosal fibers. The result shows a strong increase in the number of reconstructed fibers. These almost fill the total white matter (WM) volume and connect a large area of the cortex. The method is very computationally expensive. Possible ways to address this problem are discussed.

[The respiratory therapist]. / Der Atmungstherapeut.

Because of the expected significant growth in the elderly population and respiratory diseases, the topic of "delegation of physician's duties" is of increasing importance to the German health-care system. In 2004 the German Respiratory Society (Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP)) established the new profession: respiratory therapist. A curriculum was defined which offers training for certified nurses and physiotherapists. Respiratory therapists evaluate, treat, document and care for patients with pulmonary disorders. Under appropriate supervision a licensed respiratory therapist performs some of the work previously done by physicians at the same quality of care. The first respiratory therapists have finished their professional training in Germany. Most of these respiratory therapists are now employed in hospital-based positions requiring their specific skills. Generally, the increased medical responsibility and the increased degree of decision-making possibilities associated with the new profession contribute to a better job satisfaction. However, this is not yet true for all the newly employed respiratory therapists. Only few of the new graduate respiratory therapists were awarded higher salaries. It is a strongly recommendation to the heads of medical departments and the human resources managers of hospitals that they should recognise the increased qualifications of nurses and physiotherapists who become respiratory therapists by appropriate remuneration.

Progressive multifocal leukoencephalopathy: value of diffusion-weighted and contrast-enhanced magnetic resonance imaging for diagnosis and treatment control.

Progressive multifocal leukoencephalopathy (PML) is caused by the replication of JC virus in oligodendrocytes of immunocompromised patients. Diagnosis usually relies on the polymerase chain reaction (PCR)-based demonstration of JC virus DNA in the cerebrospinal fluid. As previous reports have suggested that some patients may benefit from antiviral therapy, non-invasive early diagnosis is highly desirable. Repetitive magnetic resonance imaging (MRI) examinations (two to nine) were obtained in seven patients (aged 40-67 years, six males, one female) with classical clinical and imaging findings of PML. Five patients had underlying hematological disorders and two acquired immune deficiency syndrome. PCR of the cerebrospinal fluid (CSF) specimen was positive for JC virus DNA in six patients. MRI sequences included T2-, T1- and diffusion-weighted (DW) images in all patients and diffusion-tensor imaging (DTI) in four cases. DTI was once performed at 3T, in the remaining patients at 1.5T. All patients received antiviral treatment with cidofovir in addition to the treatment of the underlying disorder. MRI showed areas of T2 hyperintensity with involvement of the subcortical U-fibers and restricted diffusion in all patients. Areas of diffusion abnormality correlated with disease progress. Contrast enhancement was encountered once after successful treatment and heralded clinical remission with virus elimination from the CSF. Hence, MRI including DW and contrast-enhanced images may be used to evaluate disease activity. Contrast enhancement may indicate an inflammatory response and thus herald immunologic virus elimination.

Dynamic 3D MR angiography of intra- and extracranial vascular malformations at 3T: a technical note.

Time-resolved, contrast-enhanced 3D MR angiography combined with parallel imaging at 3T was applied to an intracranial arteriovenous malformation, a dural arteriovenous fistula, and an extracranial facial arteriovenous malformation. The temporal resolution was one image every 1.5 seconds. Arterial feeders were depicted in all three cases. Early venous drainage was observed in the intracerebral arteriovenous malformation and the dural arteriovenous fistula, but not in the facial arteriovenous malformation. All findings were concordant with conventional angiography.

Presurgical MR Imaging in Epilepsy.

Primary goal of magnetic resonance imaging in epilepsy patients is to detect epileptogenic lesions with small lesions best detectable on a 3D FLAIR SPACE sequence with 1 mm(3) voxels. Morphometric analysis of 3D T1-weighted data sets helps to find subtle lesions and may reveal the true extent of a lesion. In further presurgical work-up, language lateralization and spatial relationship of epileptogenic lesions to eloquent cortex and white matter tracts must be evaluated. With clear left lateralization language, fMRI is sufficient; in atypical lateralizations, Wada test and electrical stimulation mapping may be added. Primary motor cortex and corticospinal tract on one and visual cortex and optic radiation on the other side are displayed with fMRI and diffusion tensor tractography. For the corticospinal tract a "global" tracking algorithm, for the optic radiation including Meyer' loop, which may be damaged in anterior temporal lobe resections, a probabilistic algorithm is best suited.

Tractography of Association Fibers Associated with Language Processing.

INTRODUCTION: Several major association fiber tracts are known to be part of the language processing system. There is evidence that high angular diffusion-based MRI is able to separate these fascicles in a constant way. In this study, we wanted to proof this thesis using a novel whole brain "global tracking" approach and to test for possible lateralization. METHODS: Global tracking was performed in six healthy right-handed volunteers for the arcuate fascicle (AF), the medial longitudinal fascicle (MdLF), the inferior fronto-occipital fascicle (IFOF), and the inferior longitudinal fascicle (ILF). These fiber tracts were characterized quantitatively using the number of streamlines (SL) and the mean fractional anisotropy (FA). RESULTS: We were able to characterize the AF, the MdLF, the IFOF, and the ILF consistently in six healthy volunteers using global tracking. A left-sided dominance (LI > 0.2) for the AF was found in all participants. The MdLF showed a left-sided dominance in four participants (one female, three male). Regarding the FA, no lateralization (LI > 0.2) could be shown in any of the fascicles. CONCLUSION: Using a novel global tracking algorithm we confirmed that the courses of the primary language processing associated fascicles can consistently be differentiated. Additionally we were able to show a streamline-based left-sided lateralization in the AF of all right-handed healthy subjects.

Adult onset glutaric aciduria type I presenting with a leukoencephalopathy.

Glutaric aciduria type I usually presents with an acute metabolic crisis during infancy. The authors report a previously healthy 19-year-old woman who presented with recurrent headaches, oculomotor symptoms, and a severe leukoencephalopathy on MRI. The diagnosis was made by urinary organic acid analysis and confirmed by enzyme studies. Genetic analysis revealed compound heterozygosity with a deletion c.219delC in exon 3 and a novel missense mutation R132G in exon 5 of the glutaryl CoA dehydrogenase (GCDH) gene.

Acute disseminated encephalomyelitis: MR and CT features.

We describe three cases of acute disseminated encephalomyelitis (an immune-mediated inflammatory demyelinating disease of the central nervous system) and their histology, showing different radiological features. One appearance is a few ring-shaped enhancing lesions, which are found predominantly in the supratentorial white matter, the other is solid disseminated lesions.

Serial proton MR spectroscopy of contrast-enhancing multiple sclerosis plaques: absolute metabolic values over 2 years during a clinical pharmacological study.

BACKGROUND AND PURPOSE: The time courses of total creatine (Cr), N-acetylaspartate (NAA), choline (Cho), and myo-inositol have not previously been investigated in the follow-up of contrast-enhancing multiple sclerosis (MS) plaques. Therefore, over a period of 2 years, we compared the absolute concentrations of these metabolites between patients treated with a placebo or 15 +/- deoxyspergualin (DSG) and between clinical groups with relapsing-remitting or secondary-progressive MS. METHODS: Sixteen patients, recruited from a pharmacological study of DSG, and 11 healthy control subjects were investigated by a stimulated-echo acquisition mode sequence (TR/TE = 3000/20). The selected volume initially contained a contrast-enhancing plaque, which was followed up for a period of 2 years. RESULTS: In contrast-enhancing plaques, Cho was significantly elevated and showed a significant reduction after both 3 and 12 months. The initially normal Cr significantly increased between 3 and 12 months, and was negatively correlated with plaque volume on T1-weighted MR images. NAA initially showed normal values, a significant decrease at 1 month, and a slow recovery over 2 years. Myo-inositol did not show a clear tendency. The placebo group did not differ from the treated group, nor did the relapsing-remitting group differ from the secondary-progressive group. CONCLUSION: The contradictory time courses of Cr and NAA show that an absolute quantification in proton MR spectroscopy in MS is necessary to avoid a false interpretation of reduced NAA/Cr ratios. The increase in Cr is probably due to remyelination. The initial dip and later recovery of NAA seem to be related to diminishing edema and remyelination.