Severe myoclonic epilepsy associated with mitochondrial cytopathy

We describe a case in which severe myoclonic epilepsy of infancy is associated with a disturbance in mitochondrial function. EEG traces showed diffuse spike-wave patterns inducible by intermittent photic stimulation. Laboratory analyses revealed high lactic acid levels in cerebrospinal fluid and urine, without metabolic acidosis or high lacticacidaemia. Muscle biopsy showed a slight increase in the number of mitochondria, which had a tendency towards subsarcolemmal locations, and clefts in the myofibrillar membrane that contained granular material staining positive for oxidative enzymes and red with modified Gomori stain. Quantification of the enzymatic activities of homogenized muscle showed partial deficiency of the mitochondrial respiratory chain complexes III and IV. Severe myoclonic epilepsy associated with mitochondrial cytopathy was diagnosed, but the possibility cannot be ruled out that the myoclonic epilepsy (or perhaps simply nonspecific epileptic encephalopathy) was secondary to the mitochondrial cytopathy. Thorough diagnostic analysis in severe myoclonic epilepsy cases is called for with a view to elucidation of a possible metabolic aetiology.     

Hypertrophic neuropathy — neurosurgical and neuropathological aspects

Summary Three cases of hypertrophic peripheral neuropathy are presented and their different aetiology is discussed. Hypertrophic peripheral neuropathy involving spinal nerve roots may act as a space occupying lesion mimicking tumors especially neurinomas. Therefore the neurosurgical approach may have a therapeutic as well as a diagnostic value.

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Zusammenfassung Drei Fälle von hypertrophischer peripherer Neuropathie werden beschrieben und ihre unterschiedliche Aetiologie wird diskutiert. Bei Beteiligung spinaler Nervenwurzeln an der hypertrophischen peripheren Neuropathie kann eine Raumforderung entstehen, die einen Tumor, insbesondere ein Neurinom, vortäuschen kann. Ein neurochirurgisches Vorgehen kann in diesem Falle neben der diagnostischen Bedeutung auch von therapeutischem Nutzen sein.

     

Einfluß einer adjuvanten Immunglobulintherapie auf Infektionen bei Patienten einer operativen Intensiv-Therapie-Station

Summary A randomized controlled clinical trial was conducted on the effects of immunoglobulin in therapy for infections in 104 intensive care patients. At the first sign of infection, one group of 50 patients received an i.v. preparation of immunoglobulin (4×100 ml) combined with antibiotics. The other 54 control patients received antibiotics alone. The most common infections in these patients were pneumonia, septicemia, peritonitis and wound sepsis. Infections were significantly seldom the cause of death, especially in patients with high-risk surgery who had been treated with immunoglobulin (p0.05). Likewise ventilation time in the high-risk surgery group averaged only 5.5 days for those receiving immunoglobulin as opposed to 12.7 days in controls (p0.01). Whereas the control group, in particular patients with pneumonia, remained in intensive care an average of 21.5 days, those receiving immunoglobulin stayed only 14.8 days (p0.01). In general, patients treated with immunoglobulin recovered more rapidly from infections than did controls (p0.01).

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Abkürzungsverzeichnis BKS Blutkörperchensenkungsgeschwindigkeit - Diff-BB Differentialblutbild - EDV Elektronische Datenverarbeitung - Hb Hämoglobin - Hkt Hämatokrit - IgA, M, G Immunglobulin der Klasse A, M, G - IPS Intensivpflegestation - Koag. pos./neg. Koagulase positiv/negativ Patientengruppen G Gesamtgruppe - Ü Überlebende - V Verstorbene - R0–R3 Risikogruppen 0–3 - S Sepsispatienten - P Pneumoniepatienten - PTT partial thromboplastin time - S.D. Standardabweichung - SGPT Serum-Glutamat-pyruvat-transaminase - SGOT Serum-Glutamat-oxalazetat-transaminase - SPSS Statistical package for the social science - ZVD Zentraler Venendruck     

Thermoregulation in the rat: deficits following 6-OHDA injections in the hypothalamus

Bilateral microinjections of 6-hydroxydopamine (6-OHDA) were made in a volume of 0.5--0.75 microliter through chronically implanted cannulae into anterior hypothalamic, preoptic loci. Sites were selected at which 1.0 to 12.5 microgram of norepinephrine (NE) had previously elicited a fall in the rat's body temperature. After 2.0 to 6.0 microgram of 6-OHDA were injected in the same volume at the same loci, a comparable hypothermia ensued. When the rats were exposed repeatedly for one-hour intervals to an environmental temperature of either 35.0 degrees C or 8.0 degrees C, they were unable to thermoregulate against the heat and their colonic temperature rose. In some experiments, the rats also failed to defend adequately against the cold ambient temperature, but mainly following the microinjection of the higher doses of 6-OHDA . The intakes of food and water were generally suppressed; this was accompanied by a transient decline in body weight. Overall, the severity, duration and direction of the thermoregulatory impairment depended upon the anatomical site of injection and the dose regimen of the neurotoxin employed. These results offer further evidence that an intact catecholaminergic pathway within the anterior hypothalamus is required for the rat's physiological control of heat loss in a warm environmental temperature.     

Mammary ductoscopy by helical CT: -Initial experience-

BACKGROUND: An attempt was made to visualize minute intraductal lesions using helical CT in patients with abnormal nipple discharge. METHODS: Galactography was performed, immediately followed by CT (ductal CT examination). Based on the image data obtained, ductal images were constructed on a workstation using a Pegasus viewer (ductal CT imaging). Since no criteria for diagnosis by this method are available, ductal CT images were diagnosed by reference to the known ductal fiberscopic findings. RESULTS: Ductal CT examination was performed in 10 cases, in 9 of whom ductal CT images were successfully constructed. Pathological examination was performed in 8 cases. It was possible to observe the structure of the luminal surface on the constructed ductoscopic images from all directions, but the color tone or the presence or absence of hemorrhage could not be observed. CONCLUSIONS: In the examination for abnormal nipple discharge, ductal CT examination was useful for intraductal observation. Currently, it is a method that allows for observation of the most minute intraductal lesions. However, some issues still remain unresolved. The results of this study suggest that further studies with more cases hold the promise of making ductal CT imaging a useful examination method.     

Functional magnetic resonance imaging reveals similar brain activity changes in two different animal models of schizophrenia

Rationale and objectives

In schizophrenia research, most of the functional imaging studies have been performed in psychotic patients, but little is known about brain areas involved in the expression of psychotic-like symptoms in animal models. The objective of this study was to visualize and compare brain activity abnormalities in a neurodevelopmental and a pharmacological animal model of schizophrenia.

Methods

Blood perfusion of specific brain areas, taken as indirect measure of brain activity, was investigated in adult rats following either neonatal ventral hippocampal lesion or acute administration of phencyclidine. Quantitative perfusion magnetic resonance imaging was performed on five frontal brain slices using the continuous arterial spin labeling technique. The mean perfusion was calculated in several brain structures, which were identified on anatomical images.

Results

Lesioned animals exhibiting deficits in prepulse inhibition of the startle reflex showed a significant blood perfusion increase in the nucleus accumbens, basolateral amygdala, ventral pallidum, entorhinal–piriform cortex, orbital prefrontal cortex, and in the bed nucleus of the stria terminalis, and a decrease of perfusion in the temporal cortex. Similar effects were seen following acute phencyclidine administration in naïve animals.

Conclusion

Our data point out specific cortical and subcortical brain areas involved in the development of psychotic-like symptoms in two different animal models of schizophrenia. The observed brain activity abnormalities are reminiscent of classical neuroimaging findings described in schizophrenic patients.

     

Curdlan sulphate in human severe/cerebral Plasmodium falciparum malaria

Preclinical studies have shown that curdlan sulphate (CRDS), a sulphated 1-->3-beta-D glucan, inhibits Plasmodium falciparum in vitro and down-modulates the immune response. A direct, non-specific effect on cytoadherence and rosetting may be predicted, as has been described with other sulphated polysaccharides, e.g. heparin. The anticoagulant effect of CRDS is 10-fold lower than heparin. Curdlan sulphate has, therefore, emerged as a candidate for adjunct medication in the treatment of severe/cerebral malaria. Two clinical studies were conducted using CRDS as adjunct medication to conventional therapy (artesunate) in patients with severe and severe/cerebral malaria. Both studies were double-blind and placebo-controlled to evaluate the efficacy and safety of the combination. Curdlan sulphate appeared to reduce the severity of the disease process, e.g. fever clearance time was shortened. Due to the small number of patients, there was no difference in mortality. The two treatment arms in both studies showed similar results for all laboratory parameters. The only adverse event recorded during CRDS treatment was an increase in activated partial thromboplastin time. This can be monitored easily. It seems that the patients who may benefit most are severe/cerebral cases with no organ damage on admission.