Henriette J. Tschampa

Radiological assessment of Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The “pulvinar sign”, defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD.

Endovascular Treatment of Epistaxis: Indications, Management, and Outcome

ObjectiveEpistaxis is a common clinical problem, and the majority of bleedings can be managed conservatively. However, due to extensive and sometimes life-threatening bleeding, further treatment, such as superselective embolization, may be required. We report our experience with endovascular treatment of life-threatening epistaxis.MethodsAll patients presenting with excessive epistaxis, which received endovascular treatment at a German tertiary care facility between January 2001 and December 2009, were retrospectively identified. Demographic data, etiology, origin and clinical relevance of bleeding, interventional approach, therapy-associated complications, and outcome were assessed.ResultsA total of 48 patients required 53 embolizations. Depending on the etiology of bleeding, patients were assigned to three groups: 1) idiopathic epistaxis (31/48), 2) traumatic or iatrogenic epistaxis (12/48), and 3) hereditary hemorrhagic telangiectasia (HHT) (5/48). Eleven of 48 patients required blood transfusions, and 9 of these 11 patients (82%) were termed clinically unstable. The sphenopalatine artery was embolized unilaterally in 10 of 53 (18.9%) and bilaterally in 41 of 53 (77.4%) procedures. During the same procedure, additional vessels were embolized in three patients (3/53; 5.7%). In 2 of 53(3.8%) cases, the internal carotid artery (ICA) was occluded. Long-term success rates of embolization were 29 of 31 (93.5%) for group 1 and 11 of 12 (91.7%) for group 2 patients. Embolization of patients with HHT offered at least a temporary relief in three of five (60%) cases. Two major complications (necrosis of nasal tip and transient hemiparesis) occurred after embolization.ConclusionsEndovascular treatment proves to be effective for prolonged and life-threatening epistaxis. It is easily repeatable if the first procedure is not successful and offers a good risk–benefit profile.

Noninvasive Evaluation of Cerebral Arteriovenous Malformations by 4D-MRA for Preoperative Planning and Postoperative Follow-Up in 56 Patients: Comparison with DSA and Intraoperative Findings

BACKGROUND AND PURPOSE: 4D-MRA is a promising technique in the diagnosis and follow-up of cAVMs. The purpose of this study was to compare 4D-MRA in the pre- and postoperative evaluation of cAVMs with DSA or intraoperative findings as the standards of reference regarding qualitative and quantitative parameters. MATERIALS AND METHODS: Fifty-six consecutive patients with cAVMs (30 women) underwent both 4D-MRA and DSA. Preoperative 4D-MRA was excluded from analysis in 1 patient (movement artifacts). Twenty-five patients underwent surgery on cAVMs and underwent both imaging modalities pre- and postoperatively. 4D-MRA was performed with either 0.5-mol/L gadolinium-diethylene-triamine pentaacetic acid (group 1: voxel size, 1.1 × 1.1 × 1.4 mm3; 608 ms/dynamic frame; 19 patients) or 1.0-mol/L gadobutrol (group 2: voxel size, 1.1 × 1.1 × 1.1 mm3; 572 ms/dynamic frame; additional alternating view sharing; 37 patients). Two readers independently reviewed 4D-MRA and DSA regarding the Spetzler-Martin classification, arterial feeders, and postoperative residual filling. Vessel sharpness, vessel diameter, and VBC of 4D-MRA were quantified. RESULTS: Preoperative Spetzler-Martin classification 4D-MRA and DSA ratings matched in 55/55 patients (Spetzler-Martin grades: I, 12; II, 22; III, 15; IV, 5; V, 1), and 93/100 arterial feeders were correctly identified by preoperative 4D-MRA (7 additional arterial feeders identified by DSA only: group 1, 3/19; group 2, 4/36). Postoperative 4D-MRA and DSA matched in 25/25 patients (residual filling, 1/25). Vessel sharpness and diameters did not differ substantially between the 2 groups. VBC was significantly higher in group 2 (P < .005). CONCLUSIONS: 4D-MRA is a reliable tool that allows predicting Spetzler-Martin classification and postoperative residual filling; it hence allows substituting DSA in the pre- and postoperative evaluation of patients with cerebral AVMs.

Postsurgical Intracranial Hypotension: Diagnostic and Prognostic Imaging Findings

BACKGROUND AND PURPOSE: CSF loss with consecutive intracranial hypotension has been discussed as a possible pathogenetic mechanism in poor clinical outcome after uneventful neurosurgery and appears to be correlated to specific imaging findings. The purpose of this study was to describe the clinical and imaging findings of symptomatic intracranial hypotension likely induced by wound suction drainage. MATERIALS AND METHODS: This is a review of previously published cases of patients in whom this condition developed after uneventful intracranial surgery. We performed an analysis of 3 more cases, of which 2 occurred after spinal surgery with accidental dural opening. RESULTS: Sixteen patients who remained unconscious or did not become fully responsive after surgery showed symmetric bilateral thalamic/basal ganglia signal intensity changes on CT and MR imaging studies. Of these 16 patients, 4 died and 2 also had brain stem signal intensity changes. All patients had rapid and distinct intraoperative and postoperative CSF loss documented on CT and/or MR imaging studies by a transient increase of the sag ratio, defined as maximal anteroposterior midbrain diameter by maximal bipeduncular diameter. CONCLUSIONS: The clinical course and typical MR imaging findings characterize the disease entity postsurgical intracranial hypotension. These findings also underline the potential danger of wound suction drainage in the case of possible CSF loss.

Liposomal cytarabine given concomitantly with radiotherapy in a patient with leptomeningeal metastasis from breast cancer

JO N 3 01 4 therapy (WBRT) and MTX lead to severe neurotoxic side effects [5]. Liposomal cytarabin (DepoCyte) may be an alternative to MTX with a longer application interval of 14 days. Patients treated with DepoCyte showed a longer time to neurological progression as compared to patients treated with MTX [6]. However, the combination of DepoCyte given concomitantly with WBRT has never been reported so far. Thus, it appears to be interesting to evaluate toxicity and efficacy of this combined treatment modality.

Outcome after epilepsy surgery in patients with MRI features of bilateral ammon's horn sclerosis.

In refractory temporal lobe epilepsy with unilateral ammons horn sclerosis (uAHS) resective epilepsy surgery is an established treatment option whereas little evidence exists about the consequences of unilateral hippocampal resection in patients with bilateral ammons horn sclerosis (bAHS). The aim of this study was to evaluate the post-surgical outcome of patients with bAHS after selective amygdalo-hippocampectomy (SAH) in comparison to uAHS patients. For this purpose, all bAHS and uAHS patients, identified at our center between 2003 and 2009 were analyzed retrospectively. Thirty-one bAHS patients and 291 uAHS patients were identified. Only 55% of the bAHS, but 80% of the uAHS patients were referred for formal pre-surgical diagnostics (p=0.001). Eleven bAHS and 127 uAHS patients underwent SAH. There was no difference in seizure-free outcome (Engel IA) at 12 months (bAHS 82% vs. 69%, p=0.40) and 24 months follow up (bAHS 57% vs. 60%, p=0.80). None of the bAHS patients became globally amnesic post-surgically. The favorable outcome in these highly selected bAHS patients suggests that bAHS patients may have a fair chance of becoming seizure-free after SAH. However, due to the large number of bAHS patients not undergoing formal presurgical evaluation, the results cannot be extrapolated to bAHS patients in general.

The Enterprise Stent for the Treatment of Intracranial Aneurysms

AbstractBackground:Self-expanding microstents are typically placed before a wide-necked aneurysm is filled with coils. Alternatively, the stent may be placed at the end of the procedure, when the coil mass blocks or slows down the flow in the parent artery or a branching vessel.Patients and Methods:Between March 2007 and 2009, 20 aneurysms in 20 patients were treated with a total of 21 Enterprise stents. Nine aneurysms had reopened after coiling, eleven aneurysms were primarily treated, seven after acute subarachnoid hemorrhage. Eight aneurysms were primarily filled with coils and the stent was placed when the flow in the parent or a branching artery was blocked or slowed down. In four of these cases in which secondary stenting was considered an option in advance, the “artery at risk” was initially catheterized with a microcatheter.Results:Stent placement was successful in 19 aneurysms. In a reopened basilar tip aneurysm, Y-like stent placement through a Neuroform stent failed. With secondary stent placement (n = 8), all affected vessels could be safely reopened. None of these patients suffered from transient or permanent neurologic deficits. Using the jailing technique, it was possible to place two parallel stents in the internal carotid artery and posterior cerebral artery (PCA) in a reopened wide-necked PCA aneurysm.Conclusion:Apart from the primary use of the Enterprise stent in wide-necked aneurysm secondary stenting after coiling is a feasible technique to reopen occluded parent or branching arteries. Initial microcatheter placement in the artery which is at risk to get occluded when filling the aneurysm is a valuable option.ZusammenfassungHintergrund:Selbstexpandierende Mikrostents werden typischerweise platziert, bevor weithalsige Aneurysmen mit Platinspiralen gefüllt werden. Alternativ kann der Stent am Ende der Untersuchung platziert werden, wenn der Fluss im Trägergefäß oder in einem größeren abgehenden Gefäß blockiert wird oder abnimmt. Patienten und Methodik:Zwischen März 2007 und 2009 wurden 20 Aneurysmen bei 20 Patienten mit insgesamt 21 Enterprise- Stents versorgt. Neun Aneurysmen waren wiedereröffnet, elf Aneurysmen wurden primär behandelt, sieben davon nach frischer Subarachnoidalblutung. Acht Aneurysmen wurden primär mit Platinspiralen gefüllt, und der Stent wurde platziert, nachdem der Fluss im Trägergefäß oder in einem größeren Ast blockiert wurde oder abnahm. Bei vier dieser Patienten wurde die verschlussgefährdete Arterie primär mit einem Mikrokatheter markiert.Ergebnisse:Der Stent wurde bei 19 Patienten an der gewünschten Position freigesetzt. Bei einem Patienten misslang eine Y-förmige Stentimplantation über einen Neuroform-Stent mit Jailing des Basilariskopf-Aneurysmarests. Nach sekundärer Stentimplantation wurden alle Gefäße wiedereröffnet, kein Patient erlitt ein transientes oder permanentes neurologisches Defizit. Mit Hilfe der Jailing-Technik war es in einem Fall eines weithalsigen Aneurysmarezidivs der A. communicans posterior möglich, Stents parallel von der A. carotis interna in die A. cerebri media und die A. cerebri posterior zu platzieren.Schlussfolgerung:Neben der primären Stentimplantation vor Coiling ist die sekundäre Stentimplantation nach Coiling eine alternative Technik, um verschlossene Gefäße wiederzueröffnen. Zudem können verschlussgefährdete Arterien primär mit einem Mikrokatheter markiert werden.

Magnetic resonance imaging of focal cortical dysplasia: Comparison of 3D and 2D fluid attenuated inversion recovery sequences at 3T.

PURPOSE Focal cortical dysplasia (FCD) is a frequent finding in drug resistant epilepsy. The aim of our study was to evaluate an isotropic high-resolution 3-dimensional Fluid-attenuated inversion recovery sequence (3D FLAIR) at 3T in comparison to standard 2D FLAIR in the diagnosis of FCD. MATERIALS AND METHODS In a prospective study, 19 epilepsy patients with the MR diagnosis of FCD were examined with a sagittal 3D FLAIR sequence with modulated refocusing flip angle (slice thickness 1.10mm) and a 2D FLAIR in the coronal (thk. 3mm) and axial planes (thk. 2mm). Manually placed regions of interest were used for quantitative analysis. Qualitative image analysis was performed by two neuroradiologists in consensus. RESULTS Contrast between gray and white matter (p ≤ 0.02), the lesion (p ≤ 0.031) or hyperintense extension to the ventricle (p ≤ 0.021) and white matter was significantly higher in 2D than in 3D FLAIR sequences. In the visual analysis there was no difference between 2D and 3D sequences. CONCLUSION Conventional 2D FLAIR sequences yield a higher image contrast compared to the employed 3D FLAIR sequence in patients with FCDs. Potential advantages of 3D imaging using surface rendering or automated techniques for lesion detection have to be further elucidated.

Proton magnetic resonance spectroscopy in focal cortical dysplasia at 3 T

PURPOSE Focal cortical dysplasia (FCD) type II is a frequent cause of medically intractable epilepsy. On conventional MRI diagnosis may be difficult. The purpose of our study was to assess the metabolic characteristics of MRI-typical or neuropathologically confirmed FCD II lesions at 3T. METHODS In a prospective study, 13 patients with drug-resistant epilepsy and MRI diagnosis of FCD II (seven neuropathologically confirmed) were investigated by single-volume proton magnetic resonance spectroscopy ((1)H MRS). We performed an intra-individual comparison placing spectroscopic volumes of interest in the lesion and in the apparently normal contralateral hemisphere. Spectroscopic results were correlated with clinical data. RESULTS Matched pair analysis revealed a significant increase in absolute choline (Cho) concentration in the lesion volume (+32%, p=0.015) compared to the control volume. This increase was associated with a significant decrease in N-acetyl-aspartate (NAA) concentration (-13%; p=0.008). Mean myo-inositol (Ins) levels were distinctly (+36%) but not significantly (p=0.051) elevated. Lesional creatine (Cr) concentration correlated significantly with the frequency of seizures (Spearman-Rho r=0.898; p=0.002), while concentrations of NAA, Cho and Ins did not correlate with clinical or imaging parameters. CONCLUSION MR spectroscopy revealed a characteristic metabolic pattern in FCD II lesions that helps to distinguish normal from epileptogenic tissue.

MS-like presentation of Alexander disease with multifocal lesions and oligoclonal bands

Alexander disease is a leukodystrophy characterized by Rosenthal fiber accumulation, which is caused by mutations in the gene coding for glial fibrillary acidic protein (GFAP) [1]. Depending on the age at onset, three subtypes (infantile-, juvenile-, and adult-onset) can be distinguished [1], characterized by different phenotypes. Once considered a rare condition, recent studies reveal an increasing number of cases of the adult-onset condition with pseudobulbar signs, ataxia, and spasticity and abnormalities in the medulla and upper cervical cord on neuroimaging [2, 3]. We present MR imaging of a patient with a novel mutation in the GFAP gene and CSF inflammatory signs that broadens the clinical spectrum of adult-onset Alexander disease. A 43 year old woman was admitted to the Department of Neurology at the University Hospital Bonn complaining about recurrent singultus for 1 year and left-sided tonic seizures for 1 month. Cerebrospinal fluid analysis revealed CSF-specific oligoclonal bands without pleocytosis. MRI showed hyperintense T2 and FLAIR signal in the brainstem (Fig. 1a–e) and cervical cord (Fig. 1g) and a contrast-enhancing lesion in the medulla oblongata (Fig. 1f). Taking into account the CSF results, the diagnosis of a chronic inflammatory CNS disease was discussed. However, the patient did not respond to an i.v. course of methylprednisolone. The patient’s condition deteriorated slowly over the next year. She subsequently developed dysphagia, and recurrent vomiting. Repeated brain MRIs over 11 months did not show relevant change. Based on the MRI imaging, adult-onset Alexander disease was suspected. Subsequent genetic analysis of the GFAP gene revealed a novel missense mutation in exon 6 (c.1051G [ C; p.D351H positions according to RefSeq NM_002055) in heterozygous state. This mutation results in an amino acid exchange in codon 351 from aspartate to histidine and it was not detected in 1,038 control chromosomes in a cohort of ethnically matched healthy blood donors from Germany. Analysis of this new amino acid exchange is predicted as ‘‘disease causing’’ using the web-based mutation taster analysis tool [4]. In addition, one sequence variant in intron 3 in heterozygous state (c.619-35_-21del; 1/965 control chromosomes) as well as one polymorphism in exon 2 in heterozygous state (rs59291670) were detected, for which pathogenic impact is unlikely. H. J. Tschampa (&) S. Greschus H. Urbach Departments of Radiology and Neuroradiology, University of Bonn, Sigmund-Freud-Strasse 25, 53105 Bonn, Germany e-mail: Henriette.Tschampa@ukb.uni-bonn.de