[Long QT syndrome causing grand mal epilepsy: case report, pedigree, therapeutic options, and review of the literature]. / Long-QT-Syndrom als Differenzialdiagnose einer Grand-Mal-Epilepsie: Fallbericht, Stammbaum, Therapieoptionen und Literaturübersicht.

A 24-year-old female with a history of epileptic seizures was admitted after prolonged cardiac resuscitation. The clinical course together with additional examinations led to the diagnosis of severe hypoxic cerebral damage, with poor prognosis for neurological outcome. In her initial ECG, as in the ECGs of several family members, QT prolongation was diagnosed. Meticulous history taking and ensuing genetic analysis led to the diagnosis of familial long QT syndrome (LQTS) with a mutation in the LQT-2 gene (HERG). In retrospect, the previous seizure episodes have to be considered cardiac syncopes. Two family members had previously died suddenly, and ECG and genetic analysis revealed that a total of eight family members were affected. These relatives were prophylactically treated with beta blockers or supplied with automated implantable cardioverter defibrillating devices. The literature concerning LQTS, diagnosis and prognosis of cerebral hypoxic damage, and differentiation between seizures and cardiac syncopes is discussed.

High resolution susceptibility weighted MR-imaging of brain tumors during the application of a gaseous agent.

PURPOSE: To employ a high resolution blood oxygenation level dependent (BOLD) method called susceptibility weighted imaging (SWI) together with the breathing of carbogen to investigate the response of cerebral tumors to this breathing gas and to assess tumor anatomy at high resolution. METHODS: Five patients with cerebral tumors (four glioblastoma multiforme, one astrocytoma [WHO grade II]) were studied using a susceptibility weighted 3D gradient echo, first order velocity compensated sequence (TE = 45 ms, TR = 67 ms, alpha = 25 degrees , FOV = 256 x 192 x 64 mm(3), typical matrix = 512 x 192 x 64), on a 1.5 T MR scanner while they were breathing air and carbogen. Signal changes between the two breathing conditions were investigated. RESULTS: The glioblastomas showed strong but heterogeneous signal changes between carbogen and air breathing, with changes between + 22.4 +/- 4.9 % at the perimeter of the tumors and - 5.0 +/- 0.4 % in peritumoral areas that appeared hyperintense on T (2)-weighted images. The astrocytoma displayed a signal decrease during carbogen breathing (- 4.1 +/- 0.1 % to - 6.8 +/- 0.3 % in peritumoral areas that correspond to hyperintense regions on T (2)-weighted images, and - 3.1 +/- 0.1 % in the tumor-center). CONCLUSIONS: SWI provides high resolution images of cerebral anatomy and venous vascularization. Combined with hypercapnia it allows for regional assessment of tumor activity.

[Differential diagnosis of solitary neurosarcoidosis]. / Differenzialdiagnose der isolierten Neurosarkoidose.

Sarcoidosis is an inflammatory multisystemic disease characterised by noncaseating epithelioid granulomas. The lung is affected in over 90% of patients. According to clinical criteria, the nervous system is involved in 5-9%. However, in autopsy series this number increases to 25%. Solitary involvement of the nervous system without signs of systemic disease is rare and diagnostically cryptic. Due to the wide variety of neurologic symptoms, definite diagnosis of sarcoidosis is possible only by histopathological proof of noncaseating epithelioid granulomas. We report a 51-year-old woman who presented with chronic basal meningitis and involvement of the peripheral and central nervous system due to solitary neurosarcoidosis. Diagnostic procedures and differential diagnosis are discussed.

Echoplanar diffusion-weighted MRI with intravenous gadolinium-DTPA.

It is occasionally necessary to repeat diffusion weighted imaging (DWI) after giving intravenous contrast medium (CM). However, the effects of CM on DWI and apparent diffusion coefficients (ADC) have not been fully examined. The aim of this prospective study was to investigate whether there are any diagnostically significant differences between echo-planar imaging (EPI)-DWI before and after intravenous CM. EPI-DWI was acquired twice in 203 consecutive patients before and after i.v. CM. Three blinded readers rated the diagnostic image quality. Quantitative ADC calculations were performed before and after CM in all 72 patients with lesions sufficiently large for quantification, and in 72 normal brain regions. Of the 203 patients, 127 had abnormalities on MRI, including ischaemic stroke (52), bleeding (nine), brain tumour with disturbed blood-brain barrier (BBB) (18) and other lesions (48). There were no significant signal differences on isotropic DWI before and after CM, even in lesions with definite disturbance of the BBB. No statistically significant difference between ADC of lesions and contralateral normal brain was observed.

High-resolution blood oxygen-level dependent MR venography (HRBV): a new technique.

Our purpose of this study was to demonstrate the clinical potential and spatial resolution of a new MRI technique: high-resolution blood oxygen-level dependent venography (HRBV), in well-known intracranial vascular lesions, such as cavernous and venous angiomas, and venous sinus thrombosis. HRBV provides unique high-resolution information on veins without administration of contrast medium. The data are independent of conventional findings on MRI and potentially useful in characterising and demonstrating the architecture of vascular lesions of the brain.

[Epidemiologic and clinical aspects of HIV infection in homosexual patients and intravenous drug addicts in a comparative study]. / Epidemiologische und klinische Aspekte der HIV-Infektion bei homosexuellen Patienten und intravenös Drogenabhängigen im Vergleich.

The clinical course of HIV-infection was analysed in a group of homosexual patients (n = 76, 72%) compared to intravenous drug abusers (IVDA, n = 30, 28%) in a retrospective cross-sectional study. The mean age of homosexual patients was 37.5 years compared to 28 years for IVDA. The following diseases are found significantly more frequently in homosexual patients compared to IVDA: Pneumocystis-carinii pneumonia (PCP) 17.1% vs. 0% (p < 0.05); Kaposi' sarcoma 16% vs. 0% (p < 0.05); diarrhoea 47.4% vs. 23.3% (p < 0.05); oral candidiasis 51.3% vs. 23.3% (p < 0.01); non-specific pneumonia of bacterial aetiology or due to unknown organisms 30% vs. 0% (p < 0.001) und seborrhoeic dermatitis 13.2% vs. 0% (p < 0.05). In contrast, viral hepatitis, non-specific abscesses and gonorrhoea were seen significantly more often in IVDA. The data show clearly that the spectra of HIV-associated diseases and HIV-unconnected diseases are significantly different in the two main groups. A risk-oriented preventive prophylaxis of HIV-related diseases and other infections is therefore required for each of these groups.