Chronic motor neuropathies: response to interferon-beta1a after failure of conventional therapies

OBJECTIVES: The effect of interferon-beta1a (INF-beta1a; Rebif) was studied in patients with chronic motor neuropathies not improving after conventional treatments such as immunoglobulins, steroids, cyclophosphamide or plasma exchange. METHODS: A prospective open study was performed with a duration of 6-12 months. Three patients with a multifocal motor neuropathy and one patient with a pure motor form of chronic inflammatory demyelinating polyneuropathy were enrolled. Three patients had anti-GM1 antibodies. Treatment consisted of subcutaneous injections of IBF-beta1a (6 MIU), three times a week. Primary outcome was assessed at the level of disability using the nine hole peg test, the 10 metres walking test, and the modified Rankin scale. Secondary outcome was measured at the impairment level using a slightly modified MRC sumscore. RESULTS: All patients showed a significant improvement on the modified MRC sumscore. The time required to walk 10 metres and to fulfil the nine hole peg test was also significantly reduced in the first 3 months in most patients. However, the translation of these results to functional improvement on the modified Rankin was only seen in two patients. There were no severe adverse events. Motor conduction blocks were partially restored in one patient only. Anti-GM1 antibody titres did not change. CONCLUSION: These findings indicate that severely affected patients with chronic motor neuropathies not responding to conventional therapies may improve when treated with INF-beta1a. From this study it is suggested that INF-beta1a should be administered in patients with chronic motor neuropathies for a period of up to 3 months before deciding to cease treatment. A controlled trial is necessary to confirm these findings.     

Depression and schizophrenia in epilepsy: social and biological risk factors

BACKGROUND: In a retrospective study we investigated the role of social and biological risk factors for the development of major depression and schizophreniform psychoses in epilepsy. We tested the hypotheses that social risk factors are associated with depression and biological risk factors are associated with schizophreniform psychoses. METHOD: We studied 25 patients with epilepsy and paranoid-hallucinatory psychosis, 25 patients with epilepsy and major depression, and 50 non-psychiatric epilepsy patients (controls) with respect to biological and psychosocial variables. RESULTS: Schizophrenic patients had an earlier age of onset of epilepsy and a more severe epilepsy as characterised by history of status epilepticus, multiple seizure types and severity of seizures compared to non-psychiatric controls. Simple seizure symptoms were often vegetative and EEGs showed various abnormalities including temporal lobe discharges but no lateralisation to either side. With respect to antiepileptic drugs (AED) there were only few significant differences between groups: Polytherapy as well as treatment with phenytoin (DPH) was more frequent in psychotic patients as compared to non-psychiatric patients. Patients with psychoses were also characterized by a disturbed familial background, lack of interpersonal relationships, social dependency and professional failure. Depressive patients were significantly older than non-psychiatric controls and they suffered more frequently from focal epilepsies arising from the temporal lobe. They did not differ from controls with respect to severity of epilepsy. Treatment with valproate (VPA) was inversely linked with depression, suggesting that VPA may have prophylactic antidepressive properties in epilepsy patients. There were no psychosocial variables significantly linked with depression. CONCLUSIONS: In this study, patients with different forms of psychiatric complications in epilepsy could clearly be distinguished from controls. However, we could not confirm the simple hypothesis that there are biological predictors for schizophreniform psychoses and psychosocial predictors for major depression. Neurological and sociological variables seem linked with both, suggesting a multifactorial etiology.     

Quality Determining Parameters of Balloon Angioplasty Catheters

The investigations carried out on balloon angioplasty catheters using a parameter test unit allow an objective comparison and a qualitative assessment of catheter brands with respect to their stenosis passability. The design of the test unit allows the measure of other parameters important for clinical practice, such as pushability and trackability. Altogether 8 over-the-wire and 10 monorail-catheters from 7 different manufacturers were investigated by our study. Between modern over-the-wire and monorail systems there exists no fundamental difference in passage capability. In some brands of catheter the stenosis model shows no increase in the amount of thrust required, even after repeated inflation; this is accountable to the good folding properties of the balloon. Angioplasty balloons made of less overstretchable or thinner material yielded the best results. The fact that changes in balloon quality may result from pre-inflation should carry weight in discussions concerning the reuse of catheters. The measurement results are useful for the correct selection of a catheter appropriate to the therapy task at hand.     

The 1990 United States-Japan seminar on bioorganic marine chemistry, meeting report.

The second U.S.-Japan Seminar on "Bioorganic Marine Chemistry" was held in Honolulu, Hawaii, 3-7 December, 1990. Twenty-one invited lecturers and fourteen observers from the two countries attended. Recent results in the areas of (a) new bioactive natural products, (b) biological and pharmacological activity of marine natural products, and (c) biosynthesis of marine natural products were presented and discussed. Summaries of the presentations and relevant chemical structures are presented in this report.     

Switch from a BIVAD to a LVAD in a boy with Kawasaki disease

A 9-year-old boy with Kawasaki disease survived after two severe myocardial infarctions. Thereafter pharmacologically untreatable ventricular arrhythmia and rapidly deteriorating heart failure developed in the patient. After 19 days of biventricular and a further 27 days of left univentricular mechanical circulatory support with the Berlin Heart (Cardiotechnica, Berlin, Germany) assist device the boy successfully underwent heart transplantation. At a follow-up of 54 months, the boy is leading an active and unrestricted life.     

Comment décider avec justesse,justice et équité ? Réflexion sur la décision en situation d'extrême prématurité: Deuxième partie : comment tendre vers une décision optimale ? Définition des conditions d'exercice de la décision

Extreme premature child's long-term prognostic is getting better and better known, and if a resuscitation procedure is possible at birth, it won't guarantee survival or a survival free of disability. Incertitude toward individual prognosis and outcome for those children remains considerable. In this field, we are at the frontier of medical knowledge and the answer to the question, "how to decide the ante and postnatal care" is crucial. This work is focused on this problematic of decision-making in the context of extreme prematurity. It attempts to deconstruct this concept and to explicit its stakes. Thus, with the support of the medical sources and of philosophical debates, we tried to build a decision-making procedure that complies with the ethical requirements of medical care, accuracy, justice and equity. This decision-making procedure is primarily concerned with the singularity of each decision situation and it intends to link it closely to the notions of rationality and responsibility.     

Comment décider avec justesse,justice et équité ? Réflexion sur la décision en situation d'extrême prématurité: Première partie : problématique de la décision dans le contexte de la prématurité extrême

Extreme premature child's long-term prognostic is getting better and better known, and if a resuscitation procedure is possible at birth, it won't guarantee survival or a survival free of disability. Incertitude toward individual prognosis and outcome for those childs remains considerable. In this field, we are at the frontier of medical knowledge and the answer to the question, “how to decide the ante and postnatal care?” is crucial. This work is focused on this problematic of decision making in the context of extreme prematurity. It attempts to deconstruct this concept and to explicit its stakes. Thus, with the support of the medical sources and of philosophical debates, we tried to build a decision-making procedure that complies with the ethical requirements of medical care, accuracy, justice and equity. This decision-making procedure is primarily concerned with the singularity of each decision situation and it intends to link it closely to the notions of rationality and responsibility.     

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