Escalating immunotherapy of multiple sclerosis--new aspects and practical application.

Recent clinical studies in multiple sclerosis (MS) provide new data on the treatment of clinically isolated syndromes, on secondary progression, on direct comparison of immunomodulatory treatments and on dosing issues. All these studies have important implications for the optimized care of MS patients. The multiple sclerosis therapy consensus group (MSTCG) critically evaluated the available data and provides recommendations for the application of immunoprophylactic therapies. Initiation of treatment after the first relapse may be indicated if there is clear evidence on MRI for subclinical dissemination of disease. Recent trials show that the efficacy of interferon beta treatment is more likely if patients in the secondary progressive phase of the disease still have superimposed bouts or other indicators of inflammatory disease activity than without having them. There are now data available, which suggest a possible dose-effect relation for recombinant beta-interferons. These studies have to be interpreted with caution, as some potentially important issues in the design of these studies (e. g. maintenance of blinding in the clinical part of the study) were not adequately addressed. A meta-analysis of selected interferon trials has been published challenging the value of recombinant IFN beta in MS. The pitfalls of that report are discussed in the present review as are other issues relevant to treatment including the new definition of MS, the problem of treatment failure and the impact of cost-effectiveness analyses. The MSTCG panel recommends that the new diagnostic criteria proposed by McDonald et al. should be applied if immunoprophylactic treatment is being considered. The use of standardized clinical documentation is now generally proposed to facilitate the systematic evaluation of individual patients over time and to allow retrospective evaluations in different patient cohorts. This in turn may help in formulating recommendations for the application of innovative products to patients and to health care providers. Moreover, in long-term treated patients, secondary treatment failure should be identified by pre-planned follow-up examinations, and other treatment options should then be considered.

Three cases of hindbrain herniation in adults with comments on some diagnostic difficulties.

In discussing the clinical features and surgical treatment of adult patients with Arnold Chiari deformity type I the authors focus on the differential diagnosis from demyelinating disease and present three illustrative cases. Out of 118 patients initially diagnosed as having demyelinating disease and admitted to our hospital between December 1991 and April 1993, three were subsequently found to have the Arnold Chiari deformity on MRI. The delay until correct diagnosis amounted to 10, 16 and 32 years, respectively. Our study stresses the diagnostic importance of MRI of the craniocervical area in cases of unexplained neurological symptoms and external signs of dysplasia. Accurate examination, early diagnosis and selection of proper surgical management can alleviate severe symptoms in many instances and prevent neurological complications.

[Patient education in multiple sclerosis: experiences and wishes of affected patients]. / Patientenaufklärung bei Multipler Sklerose: Erfahrungen und Wünsche Betroffener.

110 patients suffering from multiple sclerosis (MS) were asked about their experiences and their preferences concerning patient information. 85% were informed about diagnosis by physicians. The time from the occurrence of first symptoms until information about the diagnosis was 5.6 years on average. Most of our patients require frankness, but 29% would prefer to be informed only after disabilities have occurred. Contradictory statements of some patients seem to express their ambivalent feelings. It is difficult and needs a lot of sensitivity to find the best moment and the right quantity of information a patient is able to accept.

[Thrombolysis in acute occlusion of cerebral blood vessels]. / Thrombolyse beim akuten Verschluss zerebraler Gefässe.

Thrombolysis may achieve recanalization in cases of occlusion of the cerebral vessels. If therapy is initiated in good time, development of cerebral infarction may be at least partially prevented. Thrombolytic treatment was performed in 14 patients at the Wagner-Jauregg Hospital within a period of one year. Urokinase was given locally, while rtPA was applied locally and/or systemically. 4 patients had an occlusion of the internal carotid artery, 6 an occlusion of the middle cerebral artery, and 4 an occlusion of the basilar artery. Complete recanalization was achieved in 6 patients, partial recanalization in 4, and no recanalization in 4. The neurological outcome of the cases with complete recanalization was good with the exception of one patients who died. Partial recanalization resulted in a fair outcome in 2 patients, while the other 2 died. 3 out of the 4 patients in whom no recanalization was achieved died. Our findings show that this form of therapy may considerably improve the natural history of the disease, provided recanalization is achieved in good time. They encourage us to continue this form of therapy and to work at improving the therapeutic criteria.

[Single-photon emission-computed tomography in the differential diagnosis of dementia]. / Single-Photon-Emissions-Computertomographie bei der Differentialdiagnose der Demenz.

To assess the value of single photon emission computed tomography (SPECT) in the differential diagnosis of dementia SPECT and conventional computed tomography were performed in 77 patients (50 men, 27 women, mean age 59 [28-90] years) with dementia diagnosed by a battery of psychometric tests. In 13 out of 15 patients with dementia of Alzheimer type SPECT showed a typical Alzheimer pattern (bilateral parietotemporal perfusion defects). Both patients with dementia of Pick type had definite decreases in frontal perfusion. All 21 patients with dementia due to Huntington's chorea showed a typical pattern with absent perfusion of the head of the caudate nucleus, although in three of them conventional computed tomography didn't demonstrate complete atrophy of this structure. Out of 23 patients with multi-infarct dementia there were 17 with focal uptake deficits at various sites, while six displayed an Alzheimer pattern. In Korsakoff's syndrome (n = 11), Down's syndrome (n = 1), Fahr's syndrome (n = 2), senile chorea (n = 1) and HIV encephalopathy (n = 1) no typical distribution patterns were noted. Single photon emission computed tomography can make a contribution to the diagnosis and classification of primary degenerative dementias and can be used to differentiate them from other forms of dementia.

[The problem of diagnosis and therapy of myotonic dystrophy]. / Zur Problematik der Diagnostik und Therapie der myotonen Dystrophie.

Three cases of myotonia dystrophica are presented with special problems of diagnosis and treatment. Diagnosis at an early stage can be difficult because of a wide onset of the disease and varying symptomatology. Muscle biopsies should be taken from distal muscle groups since the proximal musculature may be involved at a later stage of the disease. The diagnosis can be established with a typical electromyographic finding. Therapeutic benefits can be obtained by membrane-stabilizing substances like anticonvulsants and antiarrhythmics, the dystrophic process however cannot be halted.

[Post-traumatic meningioma (author's transl)]. / Das posttraumatische Meningeom.

Report on a post-traumatic meningioma diagnosed 37 years after the trauma. Reference to relevant cases and the value of computed tomography for medico-legal opinion in post-traumatic sequelae.