A patient with Marfan's syndrome and neurofibromatosis type 1 with polyneuropathy.

Both Marfan's syndrome and neurofibromatosis type 1 are hereditary, autosomal dominant conditions. Here, we report the rare case of a patient fulfilling the clinical criteria for both diseases. In the absence of a family history of either of the two conditions, two independent de novo mutations are the most likely cause.

MRI-based volumetric differentiation of sporadic cerebellar ataxia.

The term idiopathic cerebellar ataxia (IDCA) designates a variety of cerebellar syndromes that may present with a purely cerebellar syndrome (IDCA-C) or with additional extracerebellar features (IDCA-P). Multiple system atrophy is also a sporadic neurodegenerative disorder of unknown origin that may cause prominent cerebellar symptoms (MSA-C). The final neuropathological answer to the question whether IDCA-P and MSA-C represent different varieties of one disease or two distinct entities is still lacking. Three-dimensional MRI-based volumetry allows morphological investigations intra vitam. Volumetric analysis of cerebellum, brainstem and basal ganglia was therefore performed in 46 patients with sporadic cerebellar ataxia and 16 age-matched healthy controls. Patients with dementia were excluded from the study since cognitive impairment is an exclusion criterion for the diagnosis of MSA. Cerebellar patients were clinically divided into two groups: 33 patients with multiple system atrophy with prominent cerebellar symptoms (MSA-C) and 13 patients with extracerebellar features not corresponding to MSA-C (IDCA-P). There was evidence for substantial cerebellar atrophy in both cerebellar groups while additional brainstem atrophy was significantly more pronounced in MSA-C patients. Absolute caudate and putamen atrophy was found to be restricted to single MSA-C individuals while group comparisons of mean volumes did not yield significant differences from controls. Based on the volumetric data, diagnosis could be correctly predicted in 94% of control, 82% of MSA-C and 100% of IDCA-P individuals. The finding of specific imaging characteristics strengthens (i) the value of MRI volumetry in separating MSA-C from other types of sporadic cerebellar ataxia, and (ii) the hypothesis of two independent neurodegenerative disorders in MSA-C and IDCA-P.

MRI features of primary, secondary and metastatic medulloblastoma.

Medulloblastoma is the most frequent paediatric malignant brain tumour. The purpose of this study was to define imaging characteristics and contrast uptake patterns of primary and recurrent medulloblastoma using MRI. The MRI examinations of 17 histologically proven cases of medulloblastoma diagnosed in our institution (13 males and 4 females; mean age 13 years, 7 months) were reviewed in retrospect. Only patients with pre-treatment and follow-up examinations including T2-weighted images (fluid-attenuated inversion recovery or turbo spin echo) and T1-weighted images after contrast injection (0.1 mmol/kg Gd-DTPA) were included in this study. Whereas 6 of 7 tumours ( n=17) were hyperintense on T2-weighted images, contrast enhancement was detected in 13 patients. Fifteen tumours occurred in the cerebellar vermis, two were located in the cerebellar hemispheres. Mean size at the time of presentation was 30.1 mm. All patients presented with some extent of an occlusive hydrocephalus. Local recurrent tumour or metastases were seen in 6 patients (3 months to 7 years, mean age 2.5 years). Whereas the T2 signal intensity of recurrent tumour or subarachnoidal metastases resembled the primary neoplasms, the contrast uptake tended to be less pronounced ( n=3) or was completely absent ( n=2); thus, suggestive signs of primary medulloblastoma are location in the vermis, hyperintensity on T2-weighted images and hydrocephalus. The amount of contrast enhancement is variable and nonspecific. Secondary medulloblastoma manifestation is characterized by T2 hyperintensity but not by contrast uptake.

MRI features of primary central nervous system lymphomas at presentation.

Pretreatment MRI examinations of 40 immunologically competent patients with primary CNS lymphoma (PCNSL) were evaluated (24 men, 16 women, median age 63 years). Seventy lesions were found (mean size: 19.9 mm). The number of lesions ranged from one (n = 25) to six (n = 1). The most frequent locations were the cerebral hemispheres (n = 22), the corpus callosum (n = 11), and the basal ganglia (n = 11). Cerebellar manifestations were found in 10 patients. Ocular (n = 2) and medullary cord (n = 1) manifestations were rare. Contrast enhancement was encountered in all lesions. Although 39 patients had lesions adjacent to the CSF space, leptomeningeal spread was only present in five patients. Necrosis was seen in two lesions only. Edema was extensive in 24 patients, moderate in 11 patients, and absent in five patients. Contrast-enhancing lesions in contact with the subarachnoid space and without necrosis are characteristic of PCNSL.

[Raeder- and Collet-Siccard-Syndrome. Acute pareses of cranial nerves symptomatic of a dissection of internal carotid artery]. / Raeder- und Collet-Siccard-Syndrom. Akute Hirnnervenparesen als Symptom einer A. carotis interna-Dissektion.

Acute cerebral nerve paresis can be caused in many different ways. One of the more rare causes of paresis of one or more neural pathways is dissection of the internal carotid artery. Early diagnosis is important, even with atypical symptoms, since prompt anticoagulative therapy can hinder stroke from embolism due to the dissection. We report on two patients with Raeder's syndrome and Collet-Sicard syndrome resulting from dissection of the internal carotid artery. Besides the cranial and caudal nerves involved in our study, loss of function has also been reported with the sixth to eighth cranial nerves, so that any painful, sudden cranial nerve deficiency can indicate possible carotid dissection.

[Not Available]. / Magnetenzephalographie in der Neurochirurgie Ein Routineprotokoll zur präoperativen Planung und intraoperativen Navigation : Ein Routineprotokoll zur präoperativen Planung und intraoperativen Navigation.

The combination of magnetoencephalography (MEG) and magnetic resonance imaging (MRI) allows the localization and functional characterization of the somatosensory and motor cortex. However, this method is technically pretentious and time consuming. Therefore, functional imaging is still not established as routine procedure in neurosurgery. The aim of the presented study was the development of a protocol, which allows the integration of functional MEG data into presurgical planning and intraoperative navigation, as a routine method.Forty-five patients with intracranial mass lesions within the central region were examined by MEG. For the somatosensory localization, the median and tibial nerves were electrically stimulated. The motor cortex was localized by tapping of the index finger. The coordinates of the functional dipole source were projected onto a 3D MRI data-set. Finally, the marked MR images were transferred to the neurosurgical navigation system. Following this strategy, after an average time of 3 hours, all data for presurgical planning and intraoperative navigation were available for the neurosurgeon.Our results show that MEG/MRI based functional neuronavigation can be used as a routine tool during daily neurosurgical practice.

[Not Available]. / Medulloblastom des Kleinhirnwurms mit Liquorraumaussaat Ungewöhnliche klinische und MRT-Präsentation : Ungewöhnliche klinische und MRT-Präsentation.

Medulloblastoma, the most frequent pediatric brain tumor, has known propensity for subarachnoid dissemination. Spinal metastases indicate a poor prognosis. Intraventricular spread is much less common.We report a case of primary intraventricular dissemination with precocious puberty as the first clinical symptom due to a metastasis at the infundibulary stalk. This has not been reported so far as initial symptom of a medulloblastoma. Furthermore, we encountered an unusually mild contrast enhancement of the spinal and intraventricular metastases. Delayed scanning after contrast administration and FLAIR sequences proved to be particularly valuable in this case.

[Not Available]. / Pneumokokkenmeningitis nach Zahnextraktion: Wertigkeit der MRT zur Früherkennung von Komplikationen.

In spite of modern antibiotic treatment, bacterial meningitis still has a dubious prognosis. Secondary complications are responsible for death or permanent neurologic deficits. The indication for imaging is 2-fold. Beside the search for a source of infection, an early detection of secondary complications is attempted.We report about a patient with hematogenous pneumococcal meningitis, who developed a subdural empyema and vascular stenoses of the basal cerebral arteries. These changes as well as the resulting infarctions were detected with MRI in an early state. Thus, an adequate therapy could be initiated. Especially FLAIR images, diffusion-weighted sequences and a time of flight MRA proved to be valuable in this setting.

[Histological classification of malignant non-Hodgkin's lymphomas and patient survival: the Kiel classification versus the working formulation]. / Histologische Einteilung maligner Non-Hodgkin-Lymphome und Patientenüberleben: Kiel-Klassifikation versus Working Formulation.

In 163 out of 251 histologically classified malignant Non-Hodgkin's lymphomas, a comparison was made of the patient survival with respect to both histological type and malignant grade according to the Kiel Classification (KC) and the three prognostic groups of the Working Formulation (WF). As expected, the survival rate of 94 low-grade malignant lymphoma cases (KC) was significantly better than that of 69 high-grade cases (p < 0.0001). Similar results were found both for 46 low grade cases (WF) in comparison to 50 high grade cases (p < 0.001) and for 67 intermediate grade cases (WF) compared with the 50 high grade cases (p < 0.05). The survival rates of low and intermediate grade cases (WF), however, showed no statistically significant difference. The low- and high-grade malignant lymphoma entities of KC, which together constitute the intermediate grade of WF, differed significantly with respect to patient survival (p < 0.05). Therefore, it appears that an intermediate grade is not essential in the KC.

The influence of folate antagonists on the metabolism of folic acid and its reduced derivatives in rat liver and kidney.

Uptake and conversion of [3H]folic acid to polyglutamate derivatives by rat liver and kidney were inhibited by methotrexate or aminopterin (15 mg/kg body weight) and DL-tetrahydromethotrexate (30 mg/kg body weight). In contrast, these antagonists did not influence the conversion of L-5-formyl-[3H]tetrahydrofolic acid or L-5-methyl[3H]tetrahydrofolic acid to polyglutamine derivatives and had little effect on the uptake of reduced folate derivatives. When [3H]methotrexate, [3H]aminopterin, and DL-[3H]tetrahydromethotrexate were administered in small amounts (15 mug/kg body wieght), no metabolites of these compounds were observed. However, at higher doses of [3H]methotrexate (300 mug/kg body weight), more than 30% of the radioactivity remaining in the tissue 24 hr after administration could be attributed to a metabolite of methotrexate. This metabolite was tentatively identified as methotrexate diglutamate.