Immunocompetent patients with primary central nervous system lymphoma (PCNSL) present with a median age of 55 years, immunosuppressed patients with a median age of 40 years. They show a broad range of signs and symptoms. Symptoms of increased intracranial pressure and personality change are most frequent, followed in frequency by ataxia and hemiparesis. The median time from onset of symptoms to diagnosis is 3–5 months in immunocompetent patients and 2 months in immunodeficient patients. The time to diagnosis can be considerably longer in patients with slowly developing personality change or fluctuating symptoms due to spontaneous or steroid-induced remission of so-called sentinel lesions. Native CT scans show iso- or hyperdense lesions with homogenous contrast enhancement. T1-weighted MRI scans show hypointense and T2-weighted scans hyperintense lesions. The definitive diagnosis of PCNSL requires biopsy. In some cases, however, the definitive diagnosis may exclusively be made by the demonstration of malignant B-lymphocytes in the cerebrospinal fluid.

We report the case of a 35-year-old woman who developed headache and psychosis and gradually became comatose within 3 weeks after a flu-like infection. MRI revealed bifrontal demyelination consistent with acute disseminating encephalomyelitis (ADEM). Two different cerebrospinal fluid samples were positively tested for Legionella cincinnatiensis by direct sequencing of a PCR-amplified Legionella-specific fragment. This result made it possible to interpret the initial symptoms as Pontiac fever. We think it most likely that this is a case of ADEM following the very rare situation of a systemic infection with L. cincinnatiensis. A review of the literature on Legionella-associated encephalopathy suggests that some of these cases may also have had ADEM.

This retrospective study analyzes clinical features and therapeutic outcome in 26 immunocompetent patients with primary central nervous system lymphoma (PCNSL). Most patients presented with personality changes. PCNSL lesions were mainly iso-or hyperdense, enhancing lesions on CT scan, hypointensive on T-1-, and hyperintensive on T-2-weighted MRI. Multiple lesions were found in about 60% of patients. Nine of 11 patients receiving radiotherapy alone showed complete remission (CR). Median survival time after diagnosis (MST) was 13 months. Seven patients received intravenous and intrathecal methotrexate, radiotherapy, and postirradiation intravenous cytarabine. Six of these patients had CR and 5 patients are alive in CR after a median follow-up of 12 months. Five patients received various other radiochemotherapy regimens (MST 6 months), and 3 patients died before receiving any radio-or chemotherapy. Our preliminary treatment results show a tendency to improved survival with radiochemotherapy. This is consistent with pertinent data from the literature which favors radiochemotherapy for patients with PCNSL.

In order to validate an association between pituitary size and severity of growth hormone deficiency (GHD) we evaluated the magnetic resonance images (MRI) of 107 children with different causes of short stature. Ninety-one MRIs were evaluable (64 male, 27 female; age: 9.1 ± 3.9 years). The levels of insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3), and tests of GH stimulation and spontaneous secretion, led to the following sub-groups: severe isolated GHD (SIGHD) (GH < 7 ng/ml) (n = 21); partial, isolated GHD (GH 7–10 ng/ml) (n = 22); multiple pituitary hormone deficiency (MPHD) (n = 13); neurosecretory dysfunction (n = 10); non-classifiable diagnosis (NC) (n = 13); idiopathic short stature (n = 9); and intra-uterine growth retardation (n = 3). Pituitary height (PHT) was measured and hypoplasia was assumed when PHT was <−2 SDS. An ectopic posterior pituitary with missing stalk and a hypoplastic anterior pituitary was present in 12 (57%) SIGHD cases, 12 (92%) MPHD cases and 1 patient from the NC group. An isolated hypoplastic anterior pituitary was observed in 15%−33% of the other groups. PHT (mm; mean, SD) in MPHD (1.7 ± 0.5) was lower than in SIGHD (2.7 ± 1.0, P < 0.05), with PHT of both groups being lower than in all the other groups (3.8 ± 0.9, P < 0.0001). PHT SDS correlates with IGF-I SDS (r = 0.48, P < 0.0001), IGFBP-3 SDS (r = 0.46, P < 0.0001) and the highest peaks in tests of GH stimulation and GH spontaneous secretion (r = 0.36, P < 0.0001). In contrast to all the other groups, no correlation with age was observed in MPHD and SIGHD. Breech delivery was recorded in up to 26% of patients in all seven groups. Surprisingly, only 1 out of 23 patients with an ectopic posterior pituitary was born by breech delivery, suggesting that ectopia of the posterior lobe is not necessarily related to breech delivery.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy 1 Chabriat H Vahedi K Iba-Zizen MT et al. Clinical spectrum of CADASIL: a study of 7 families. Lancet. 1995; 346: 934-939 Summary PubMed Google Scholar (CADASIL) is an autosomal dominant-inherited microangiopathy leading to stroke and other symptoms, including subcortical dementia, migraine-like headache, and psychiatric disturbance. Weller et al 2 Weller M Petersen D Dichgans J Klockgether T Cerebral angiography complications link CADASIL to familial hemiplegic migraine. Neurology. 1996; 46: 844 PubMed Google Scholar described two CADASIL patients who experienced neurological complications induced by cerebral angiography. We have evidence that there is a high rate of angiographic complications in this syndrome.

Case Reports| February 13 2008 Prolonged Corticosteroid-lnduced Remission in Primary Central Nervous System Lymphoma: Report of a Case and Review of the Literature Subject Area: Neurology and Neuroscience U. Herrlinger; U. Herrlinger Departments of Neurology, Search for other works by this author on: This Site PubMed Google Scholar M. Schabet; M. Schabet Departments of Neurology, Search for other works by this author on: This Site PubMed Google Scholar M. Eichhorn; M. Eichhorn Departments of Neurology, Search for other works by this author on: This Site PubMed Google Scholar D. Petersen; D. Petersen Neuroradiology, Search for other works by this author on: This Site PubMed Google Scholar E.H. Grote; E.H. Grote Neurosurgery Search for other works by this author on: This Site PubMed Google Scholar R. Meyermann; R. Meyermann Neuropathology University of Tübingen Germany Search for other works by this author on: This Site PubMed Google Scholar J. Dichgans J. Dichgans Departments of Neurology, Search for other works by this author on: This Site PubMed Google Scholar Eur Neurol (1996) 36 (4): 241–243. https://doi.org/10.1159/000117261 Article history Published Online: February 13 2008 Content Tools Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn Email Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation U. Herrlinger, M. Schabet, M. Eichhorn, D. Petersen, E.H. Grote, R. Meyermann, J. Dichgans; Prolonged Corticosteroid-lnduced Remission in Primary Central Nervous System Lymphoma: Report of a Case and Review of the Literature. Eur Neurol 1 April 1996; 36 (4): 241–243. https://doi.org/10.1159/000117261 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu toolbar search search input Search input auto suggest filter your search All ContentAll JournalsEuropean Neurology Search Advanced Search This content is only available via PDF. 1996Copyright / Drug Dosage / DisclaimerCopyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Article PDF first page preview Close Modal You do not currently have access to this content.

This study addressed the use of 3D MR angiography with flip angles (FAs) linearly varying across the excitation volume in order to diminish spin saturation. The shape of the ramp profile was varied to optimize the method for different regions and pathological alterations. Radio frequency pulses with ramp-shaped excitation profiles were generated flow-compensated 3D-FISP sequence. With the use of ramp-shaped excitation profiles good results were obtained for intracranial arteries as well as for neck arteries (i.e. carotid and vertebral arteries) which were demonstrated in 6 healthy volunteers and in 5 patients with various stenoses and anomalies of the carotid and vertebral arteries. With this technique it was possible to use increased thicknesses of the excitation volume (slab) up to 256 mm. Ramp-shaped excitation pulses with linearly increasing FAs in main flow direction can provide improved contrast of the vessel parts located distally to the entry side of the slab. Although this method has no advantage concerning complex flow or other dephasing effects; its particular effectiveness lies in the reduction of spin saturation.

Maximum intensity projection (MIP), the commonly used technique for calculating MR-angiograms from three-dimensional (3D) datasets, often fails to visualize small vessels due to superposition of background signal. An improved vascular depiction can be reached by application of a connectivity algorithm, but a very good delimitation of vessel signals from signals of stationary tissue is required for this technique. Results of intracranial vessel tracking could be improved by a 3D interpolation and a preceding highpass filtering of the 3D image dataset.

Der Begriff Multisystematrophie (MSA) bezieht sich auf eine sporadisch auftretende, jenseits des 30. Lebensjahres beginnende neurodegenerative Erkrankung (Graham und Oppenheimer 1969). Die definitive Diagnose einer MSA kann nur pathologisch gestellt werden. Die MSA ist neuropathologisch gekennzeichnet durch das Auftreten von olivopontocerebellärer Atrophie (OPCA), striatonigraler Degeneration (SND) und der Degeneration der intermediolateralen Zellsäulen des Rückenmarks. Die Diagnose einer MSA ist klinisch wahrscheinlich, wenn ein Parkinsonsyndrom und/oder eine cerebelläre Ataxie in Kombination mit autonomemVersagen jenseits des 30. Lebensjahres auftreten und die Familienanamnese leer ist (Quinn 1989).

We examined 32 patients with intracranial tumors (17 meningiomas, 8 neuromas, 7 pituitary adenomas) by conventional and dynamic contrast-enhanced MRI. Our aim was to clarify whether the pathological dural contrast enhancement adjacent to meningiomas (the "dural tail") is specific to meningiomas and, more important, whether it represents neoplastic dural infiltration or hypervascularization as a tumor accompanying reaction. A "dural tail" was found in 9 of 17 meningiomas. None of the other extra-axial tumours (neuromas, pituitary adenomas) showed comparable dural enhancement. Dynamic examinations with an ultrafast single slice imaging technique (snapshot-FLASH) after a bolus injection of contrast medium showed a "dural tail" in seven out of these nine meningiomas, while in two cases the "dural tail" turned out to be a cortical vein with a characteristic dynamic contrast enhancement pattern. In the dynamic study all seven "dural tails" were found to have earlier, steeper contrast enhancement than the corresponding tumours. All the tumours and part of the adjacent dura mater in four of the seven meningiomas with dural enhancement were examined histopathologically. In none of these four cases was neoplastic tissue found more than 2 mm away from the main tumour. The results strongly support the suggestion that the "dural tail" adjacent to meningiomas represents a hypervascular, non-neoplastic dural reaction.

Sixteen patients with a clinical diagnosis of probable multiple system atrophy (MSA) were examined clinically by MRI and by 123I-iodobenzamide single photon emission computed tomography (IBZM-SPECT). The clinical records of another 16 patients were also analysed retrospectively. On the basis of their clinical presentation, patients were subdivided into those with prominent parkinsonism (MSA-P, n = 11) and those with prominent cerebellar ataxia (MSA-C, n = 21). Autonomic symptoms were present in all patients and preceded the onset of motor symptoms in 63% of patients. Calculated median lifetime and the median time to become wheelchair bound after onset of disease were significantly shorter for MSA-P than for MSA-C (lifetime: 4.0 v 9.1 years; wheelchair: 3.1 vs 5.0 years) suggesting a better prognosis for cerebellar patients. A significant loss of striatal dopamine receptors (below 2 SD threshold) was detected by IBZM-SPECT in 63% of the patients (56% below 2.5 SD threshold). There was no difference between patients with MSA-C and those with MSA-P in the proportion with significant receptor loss and the extent of dopamine receptor loss. Planimetric MRI evaluation showed cerebellar and brainstem atrophy in both groups. Atrophy was more pronounced in patients with MSA-C than in those with MSA-P. Pontocerebellar hyperintensities and putaminal hypointensities on T2 weighted MRI were found in both groups. Pontocerebellar signal abnormalities were more pronounced in MSA-C than in MSA-P, whereas the rating scores for area but not for intensity of putaminal abnormalities were higher in MSA-P. MRI and IBZM-SPECT provide in vivo evidence for combined basal ganglia and pontocerebellar involvement in almost all patients in this series.

We used magnetic resonance imaging (MRI) to study brain and spinal cord morphology in hereditary and idiopathic ataxia. Our interest was in whether the classical neuropathologic categories-cerebellar cortical atrophy (CCA), olivopontocerebellar atrophy (OPCA), and spinal atrophy (SA)-could be identified in vivo and which clinical phenotype corresponded to which morphologic category. To this end, we measured the size of the cerebellar vermis, cerebellar hemispheres, fourth ventricle, middle cerebellar peduncles, basis pontis, medulla oblongata, and cervical spinal cord on T1-weighted images of 61 patients and 24 healthy controls. Five patients with Friedreich's ataxia (n = 7) and all with late-onset Friedreich's ataxia (n = 3) had SA without major involvement of the brainstem or cerebellum. Morphologic findings in patients with early-onset cerebellar ataxia with retained tendon reflexes (n = 11) were heterogeneous: six patients had MRI findings compatible with CCA, and two patients had a combination of SA and CCA. The three remaining patients had an atypical pattern of atrophy. Similarly, the morphologic changes in patients with autosomal-dominant cerebellar ataxia with additional noncerebellar symptoms (ADCA-I; n = 13) were nonuniform: atrophic changes typical for CCA, OPCA, or SA were each present in one case, four patients had a combination of OPCA and SA, and the remaining patients could not be assigned to one of the morphologic categories. In autosomal-dominant cerebellar ataxia with a pure cerebellar syndrome (ADCA-III; n = 6), all patients except one had CCA. Patients with idiopathic cerebellar ataxia (IDCA) fell into two clinically and morphologicly distinct groups: the majority of patients with additional noncerebellar symptoms (IDCA-P; n = 14) had OPCA, whereas almost all patients with a pure cerebellar syndrome (IDCA-C; n = 7) had CCA.

Alcoholic brain damage is reversible when the patients are continually abstinent. An increase of brain water content was the putative explanation for this phenomenon. We tested the rehydration hypothesis using CT density measurements in 29 alcohol-dependent male inpatients. During a 5-week period of controlled abstinence, CT density measures did not decrease in any of the investigated regions of the brain as one would expect with an increase in brain water. Although the volumetry of the ventricular system and the subarachnoidal spaces revealed a significant reduction of CSF volume, we found a slight increase in CT density measures. Thus, our results are in contradiction to the rehydration hypothesis. Under discussion is whether neuronal plasticity might be the explanation of the reversibility of alcoholic brain damage in abstinent patients.

We examined 11 adult patients with cerebellar encephalitis (CE) during the acute phase of the disease and at least 12 months later. Five patients were aged between 23 and 31 years, 3 patients between 43 and 44 years and 3 patients between 60 and 64 years. Serological tests gave evidence of Epstein-Barr virus infection in 4 of the 5 young patients. Two patients had serological evidence of varicella-zoster virus reactivation, whereas the serological findings were negative in all other cases. All patients in the younger and middle age groups recovered within 3-30 weeks after onset of CE. If at all, they had only minor cerebellar deficits at the follow-up examination. Magnetic resonance imaging (MRI) examination performed at the follow-up examination was normal in all of them. In contrast, 2 of 3 patients older than 60 years had persistent cerebellar ataxia following CE. In these patients, MRI revealed infratentorial atrophy. Our data show that the clinical spectrum of CE in adults is wider than assumed so far. In addition to typical cases of CE in young male patients with good recovery, CE may also occur in older patients and give rise to persistent cerebellar ataxia.

Verschiedene Lehrmeinungen zur Hirnatrophie von Alkoholabhängigen mußten in den letzten Jahren revidiert werden. Computertomographische Vergleiche zwischen Alkoholabhängigen und gesunden Kontrollen ergaben vergrößerte Liquorräume bei den Patienten [5, 7]. Verlaufsuntersuchungen zeigten eine signifikante Abnahme der Liquorräume unter Abstinenzbedingungen, was durch eine Zunahme des Hirnvolumens erklärt wird [3]. Zur Pathopysiologie wurden 1978 zwei Hypothesen formuliert: 1. Nach der „Rehydrationshypothese“kommt es unter chronischem Einfluß von Alkohol zu einer Dehydration des Hirngewebes mit entsprechender Schrumpfung. Unter Abstinenzbedingungen sorgt ein verstärkter Wassereinstrom ins Hirngewebe für eine Volumenvermehrung [3]. 2. Die „Regenerationshypothese“postuliert dagegen einen direkten toxischen Einfluß des Alkohols auf Zellen des zentralen Nervensystems mit partiell reversiblen Schädigungen im Sinne eines Verlustes an Dendritendornen und einer Verschmächtigung des Dendritenbaumes [9].