The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention.

OBJECTIVE: To determine how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. METHODS: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. RESULTS: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. CONCLUSION: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.     

Catamenial pneumothorax: case report and literature review

Since the initial case report in 1958, 69 cases of catamenial pneumothorax have appeared in English-language literature. Despite the extensive case material, the pathogenesis has not been fully elucidated. Herein an additional case is reported utilizing computed tomography (CT) to search for intrathoracic endometrial implants. The four major pathogenetic theories include passage of air from the genital tract via diaphragmatic defects, spontaneous rupture of subpleural blebs, intrathoracic endometriosis, and prostaglandin-induced bronchiolar constriction resulting in alveolar rupture. Evidence for and against each theory is presented.     

Combined medical-dental treatment of an epidermolysis bullosa patient

A patient with a debilitating disease, such as epidermolysis bullosa, is a challenge to the dentist wishing to provide optimal and comprehensive dental treatment. While tissue sloughing and bullae formation during treatment cannot be eliminated, the previously outlined protocol can limit the severity of it.
Medical assessment of these patients is mandatory to determine their overall health status. If indicated, a combination medical and dental procedure can be performed under general anesthesia, thereby limiting the number of stressful operations a patient may have to undergo.
Triannual dental examinations and continual re-evaluation are necessary to promote continued dental health and recognition of early pathosis. Aggressive preventive measures are necessary to ensure the patient's future dental needs are minimal.     

Characterization of a protein from normal human polymorphonuclear leukocytes with bactericidal activity against Pseudomonas aeruginosa.

Purification of a bactericidal protein (BP) from the cytoplasmic granules of normal human polymorphonuclear leukocytes (PMN) with activity against Pseudomonas aeruginosa is described. Bactericidal activity from acid extracts of a mixed granule population was purified 175-fold by a two-step chromatographic procedure. The first step, dye-ligand affinity chromatography with Matrex-Gel Orange A, was followed by cation-exchange chromatography with Bio-Rex 70 resin, and this combination routinely gave a yield near 80%. Only one peak of bactericidal activity against P. aeruginosa type I was found after each chromatographic step. Characterization of BP showed a single band with an apparent molecular weight of 55,000 by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Purified BP was most active against the six strains of P. aeruginosa tested and against Escherichia coli B (a deep rough mutant). Purified BP killed 5 X 10(6) CFU of P. aeruginosa type I per ml at a concentration of 60 to 80 ng/ml. Proteus mirabilis and Staphylococcus aureus were both resistant to the bactericidal activity of BP. BP was shown to be glycosylated by periodic acid staining after gel electrophoresis and to have an isoelectric point near 7.5 by chromatofocusing. The amino acid composition of BP is presented.     

Multicompartment analysis of the effects of plasmapheresis. Application to lipid kinetics in humans

The quantitative effect of plasmapheresis on concentrations of intravascular solutes is described by a minimal two-compartment model, which allows exit to and entry from a nonplasma body pool of the solute. This novel method is simple and capable of determining endogenous metabolic turnover of physiologically important plasma constituents in both therapeutic and experimental settings. The model was suggested by the therapeutic use of plasmapheresis in hyperlipidemia, and the turnover rates of triglyceride and cholesterol were calculated for one patient treated with chronic plasmapheresis. It is concluded that at least a two-compartment model is necessary to quantitatively describe the effect of plasmapheresis on any substance that undergoes appreciable endogenous turnover (metabolic clearance rate greater than 1.1 times plasmapheresis rate when both rates are in units of volume per time). Calculation of endogenous turnover rate with the use of measurements of the concentration of a solute in plasma before and after plasmapheresis and in the total plasmapheresate and measurement of the volume of plasma removed, volume of plasma substitute infused, and total plasmapheresis time is detailed. This method avoids the use of isotopic labeling experiments. In turn, the endogenous turnover rate may predict the efficacy of therapeutic plasmapheresis.     

Extensive form of progressive multifocal leucoencephalopathy associated with laryngeal carcinoma

Summary A case is reported of the rare association between progressive multifocal leucoencephalopathy and carcinoma. A 66-year-old man underwent a laryngectomy for carcinoma. Four years later he developed a local recurrence. Simultaneously there were behavioural disturbances and a left motor neglect followed by dense hemiplegia and coma. The patient died a further 5 months later. Anergy was demonstrated by skin tests. CT scan showed asymmetrical non-enhancing low-density areas in the hemispheric white matter, brain-stem and cerebellum and neuropathological examination confirmed extensive myelin loss with typical papovavirus inclusions in oligodendrocytes identified by electron microscopy.Presented at the Irish Neurological Association and Danish Neurological Society joint meeting, Cork, 16 May 1985