Superficial siderosis of the central nervous system - treatment with steroids?

Steroids have been suggested as a therapeutic option for superficial siderosis of the central nervous system (SSCNS) without identifiable bleeding source. Longitudinal observational data of a patient with idiopathic SSCNS who was repeatedly treated with methylprednisolone over a course of 2 years are reported. The case history is critically discussed on the background of the sparse literature. In conclusion, if at all, there is only a limited and temporary, mostly subjective clinical response to steroids in SSCNS. Systematic studies of this medication in SSCNS do not seem warranted. Pathophysiological considerations hopefully may lead to more helpful medications for this chronic and debilitating disorder.

Neurologic manifestations of ulcerative colitis.

Ulcerative colitis (UC) has traditionally been considered to be an inflammatory disease limited to the colonic mucosa. However, since it has been shown that UC is frequently accompanied by various extraintestinal disorders, there is increasing evidence that UC may also manifest in the nervous system. The following review focuses particularly on these possible manifestations of UC, both in the peripheral (PNS), and in the central nervous system (CNS). A systematic literature search according to the MEDLINE database was performed for this purpose. Although a reliable differentiation may clinically not always be possible, three major pathogenic entities can be differentiated: (i) cerebrovascular disease as a consequence of thrombosis and thromboembolism; (ii) systemic and cerebral vasculitis; (iii) probably immune mediated neuropathy and cerebral demyelination. With the exception of thromboembolism and sensorineural hearing loss, evidence for a causal relationship relies merely on single case reports or retrospective case series. Considering the CNS-manifestations, similarities between UC-associated disorders of the white matter and acute disseminated encephalomyelitis (ADEM) are obvious. Epileptic seizures, unspecified encephalopathies and confusional states are most likely epiphenomena that have to be regarded symptomatic rather than as own entities. A prospective study on the neurologic aspects of UC would be very welcome.

The competitive NMDA antagonist CGP40.116 enhances L-dopa response in MPTP-treated marmosets.

Experiments in MPTP-treated non-human primates testing potential antiparkinsonian action have shown both, beneficial and adverse effects of gutamate receptor antagonists. To investigate this matter further, the novel competitive NMDA antagonist CGP40.116 was administered systemically to three adult MPTP-treated marmosets. When coadministered subcutaneously with a subthreshold dose of L-DOPA, 2 mg/kg, CGP40.116 25-250 micrograms/kg, increased locomotor activity. However, when administered alone, CGP40.116 had no effect on locomotor activity.

Cloning of hepatitis A virus genome.

Hepatitis A virus (HAV) was highly purified from faeces. The genomic RNA was transcribed to cDNA and this DNA was then cloned into plasmid pBR 322 at the Pst I site, and clones were selected in presence of tetracycline. Most clones contained inserts which hybridized to HAV-specific RNA isolated from HAV-infected cell cultures derived from a human hepatocellular carcinoma. Two clones expressed low amounts of viral antigens.

Spontaneous inclusion body hepatitis in young tamarins: I. Morphological study.

Over a period of 4 years approximately 60% of the new born and juvenile animals in a breeding colony of tamarins (Saguinus fuscicollis) died a sudden death. Histological examination at necropsy revealed interstitial hepatitis in 22 of the 30 young animals of the present study. The hepatocytes contained intranuclear inclusion bodies in 12 of the 22 cases. Upon ultrastructural examination, tubulovesicular structures and amorphous material were found in the nuclei. The endoplasmic reticulum had proliferated and was closely associated with undulating curved membranes. These morphological alterations resemble those reported in chimpanzees experimentally infected with NANB hepatitis viruses.

Comparison of two methods designed to improve pediatric dental case presentations utilizing videotapes.

This study compared the effectiveness of two methods of teaching the skills required for dentist-parent pediatric case presentations. Seventy-two senior dental students were videotaped presenting a child's case presentation to a parent. The students were then randomly assigned to one of two groups. Students in Group 1 viewed and evaluated a model videotape of the principal investigator presenting a case to a parent. Students in Group 2 viewed their previously videotaped presentation. Both groups evaluated performance on the tapes using a list of 25 performance-specific criteria identified as important for a case presentation. Following this evaluation, 36 students were able to be taped while presenting a second case to another parent. One investigator blindly evaluated each taped presentation using the 25 criteria. Students who viewed the model videotape demonstrated a significant improvement in performance from their first presentation (p = 0.0044). Students who evaluated their own tape showed no significant improvement (p = 0.8830). Students perceived both methods to be helpful, minimally stressful, and requiring little time.

Effect of feedback on student attitudes.

We sought to determine the change in student attitude toward a testing program when the students used a self-assessment test and were given computer-generated feedback. Ninety-four junior dental students at one school were placed in two groups and surveyed about their attitudes toward the testing program of the Department of Operative Dentistry and themselves as operative dentists. A semantic differential instrument and Likert-scale questions were used. One week later, the students in the experimental group participated in a self-assessment and three weeks later received individual, computer-generated feedback. Both groups were surveyed again at this time. Students in the experimental group exhibited a significant improvement in attitude toward the department's testing program but not toward themselves as operative dentists. The control group showed no significant change in attitude toward either concept.

Clinical insights into paraneoplastic cerebellar degeneration.

Neuroimaging is usually unremarkable in paraneoplastic cerebellar degeneration (PCD), at least in the early stages of the disease. A patient with proven PCD is reported in whom it could be shown that cerebellar atrophy evolved very rapidly and was present in early imaging studies. Even with the use of the whole spectrum of modern diagnostic tools, the underlying malignancy can be difficult to diagnose. In addition to mammography, MRI is recommended in these cases and repeat FDG-PET may be necessary.

Correlation of cognitive status, MRI- and SPECT-imaging in CADASIL patients.

Although there is evidence for correlations between disability and magnetic resonance imaging (MRI) total lesion volume in autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), the significance of structural MRI abnormalities for cognitive dysfunction remains controversial. We performed detailed neuropsychological testing, high resolution MRI, and Tc-99m-ethyl cysteinate-dimer SPECT in three CADASIL patients. MR-images were rated independently by two investigators for the presence of white matter lesions, lacunar infarcts, microbleeds, and ventricular enlargement. Cortical atrophy was quantified by the use of automatic morphometric assessment of the cortical thickness. In addition, laboratory and patients' history data were collected in order to assess the individual vascular risk factor profile. The differences in cognitive performance between the three patients are neither explained by structural-, or functional neuroimaging, nor by the patient-specific vascular risk factor profiles. The neuroradiologically least affected patient met criteria for dementia, whereas the most severely affected patient was in the best clinical and cognitive state. Conventional structural and functional neuroimaging is important for the diagnosis of CADASIL, but it is no sufficient surrogate marker for the associated cognitive decline. Detailed neuropsychological assessment seems to be more useful, particularly with respect to the implementation of reliable outcome parameters in possible therapeutic trials.

[Arterial hypertension and dementia]. / Arterielle Hypertonie und Demenz.

Arterial hypertension (AH) is considered to be an important risk factor for vascular cognitive impairment and probably for Alzheimer's disease, too. In the current review we provide an overview of the major prospective clinical trials on this issue. With respect to the inconsistent findings of these studies one must state that the interrelations between AH and dementia are still incompletely understood. Regarding therapy there thus is a simple rule: AH should be treated until normal blood pressure values are reached. As long as the hypothesis of a J-curve between blood pressure and cognitive dysfunction has not been disproved, overaggressive blood pressure lowering cannot be recommended. Regarding the aim of preventing dementia, there is no evidence for the superiority of a specific antihypertensive treatment regimen.

Evaluation of a pelleted diet in a colony of marmosets and tamarins.

By offering various pelleted diet modifications to five species of Callitrichidae, a palatable 24% protein diet was developed. The animals received limited fruit supplement, and families received an additional meal of commercial baby food. The pellets supplied approximately 75% of dietary energy and 95% of total protein. In 15 months on this regimen, mortality dropped drastically and fertility improved. No signs of a nutritional deficiency were observed. It was concluded that the nutrient levels of this diet were adequate or above actual requirements.