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Background: Although three randomised control trials have shown that selective dorsal rhizotomy (SDR) reduces spasticity in children with cerebral palsy, a meta-analysis of the results demonstrated that the procedure conferred only small functional benefit on the patient., Aim: To determine whether applying strict criteria for patient selection as practised in Oswestry leads to improved outcomes, using gait analysis as an outcome measure., Methods: Ambulant children with cerebral palsy were selected for SDR using very strict clinical criteria. Instrumented gait analysis was used as the main outcome measure., Results: Of 53 children referred for the procedure, only 19 (35%) fulfilled our strict criteria for selection. These children underwent surgery and when pre- and post-SDR data were compared, they showed improvement in cosmesis of gait, clinical examination and temporal, kinetic and kinematic parameters of gait. After SDR the children walked, on average, 0.15 m/s faster, with a step length improvement of 0.11 m. Changes were seen at hip, knee and ankle, with those at the knee being most marked. A 0.3 grade improvement in knee extensor power on clinical examination led to a 13 degrees improvement in stance phase knee extension. Knees also became less stiff, with an 82 degrees /s improvement in the rate of flexion into swing phase. A functional tool (the GMFCS) applied retrospectively also confirmed post-operative improvement, with 15 of the 19 children improving by at least one level., Conclusion: Application of strict selection criteria when considering children for SDR leads to encouraging results as demonstrated by gait analysis and other measures.

A series of oligomeric glycans can be extracted from the cell walls of developing cotton fibers with weak acid. Glycans that produce similar profiles on high pH anion chromatography with pulsed amperometric detection (HPAEC-PAD) are also found in a protein complex extracted from developing fibers and in amorphous aggregates found in association with immature fibers in developing, but not in mature cotton bolls. The quantity and composition of the glycans recovered from the carbohydrate-protein complex varies significantly with the time of day when the bolls are harvested. This diurnal variation is consistent with the hypothesis that secondary cell walls are deposited primarily at night. Incubation of re-hydrated cotton fibers in the presence of exogenous oligosaccharides, myo-inositol and glycerol substantially alters the apparent quantity of the oligomers extracted from the fibers. The same and similar glycans have also been extracted from cotton fabric, marine algae, various paper products and wood. While many of the oligomers isolated from the various cellulose sources display the same peaks by HPAEC-PAD, the specific number of oligomers and their relative quantities appear unique for each source of cellulosic material. Oligomeric glycans, as described in the preceding, are present in all cellulose sources that have been investigated. Their relative abundance changes in response to source, stage of development and other physiological variables. We hypothesize that the glycans are intermediates in the biological assembly of cellulose, and that their incorporation in cellulose is mediated by physicochemical and enzymatic mechanisms.

We report a 13 year old boy with fragile X syndrome resulting from a de novo deletion of the FMR1 and FMR2 genes extending from (and including) DXS7536 proximally to FMR2 distally. The patient has severe developmental delay, epilepsy, and behavioural difficulties, including autistic features. He has epicanthic folds, in addition to facial features typical of fragile X syndrome, and marked joint hypermobility. We compare our patient to the three other cases reported in which both FMR1 and FMR2 are deleted. This case has the smallest deletion reported to date. All four patients have epilepsy and a more severe degree of mental retardation than is usual in fragile X syndrome resulting from FMR1 triplet repeat expansion. Three of the patients have joint laxity and two have epicanthic folds. We suggest that these features, in particular severe developmental delay and epilepsy, may form part of the characteristic phenotype resulting from deletion of both FMR1 and FMR2 genes. The diagnosis in this case was delayed because routine cytogenetics showed no abnormality and standard molecular tests for FMR1 triplet repeat expansion (PCR and Southern blotting) failed. Further DNA studies should be undertaken to investigate for a deletion where clinical suspicion of fragile X syndrome is strong and routine laboratory tests fail.

A girl with partial lipodystrophy is described presenting with muscle weakness and developmental delay several years before lipoatrophy became apparent. The patient subsequently developed epilepsy, fatty liver, secondary amenorrhoea, hirsutism, insulin-resistant diabetes mellitus, hyperlipidaemia, and hypothyroidism. She remains weak with poor exercise tolerance. This case illustrates an atypical presentation of the Barraquer-Simon syndrome.

The authors report two cases of multiple sclerosis occurring in very young children. Age at onset in one child was 13 months; he had the primary progressive form of the condition and died at nine years. Age at onset of the other child was two years; he has the classical relapsing-remitting form and is well at 15 years. These children illustrate the broad clinical spectrum of the condition even in very-early-onset disease.

Twenty-eight patients, all of whom developed multiple sclerosis before 16 years of age and all from a well-defined geographical population (Grampian, north-eastern Scotland) were retrospectively studied. Age at presentation, presenting features and interval between first and second attacks are discussed. Follow-up ranged from three to 47 years and adult categorisation was applied to outcome. This suggested that on the whole, childhood-onset multiple sclerosis is a less aggressive disease than its adult counterpart.

This study was undertaken to examine the dependence of the regulatory enzymes of photosynthetic induction on photon flux density (PFD) exposure in soybean (Glycine max L.). The induction state varies as a function of both the magnitude and duration of the PFD levels experienced prior to an increase in PFD. The photosynthetic induction state results from the combined activity of separate processes that each in turn depend on prior PFD environment in different ways. Direct measurement of enzyme activities coupled with determination of in situ metabolite pool sizes indicated that the fast-induction component was associated with the activation state of stromal fructose-1,6-bisphosphatase (FBPase, EC 3.1.3.11) and showed rapid deactivation in the dark and at low PFD. The fast-induction component was activated at low PFD levels, around 70 [mu]mol photons m-2 s-1. Ribulose-1,5-bisphosphate carboxylase/oxygenase (Rubisco, EC 2.7.1.19) deactivated very slowly in the dark and required higher PFD for activation. Both enzymes saturated at lower PFD than did photosynthesis, around 400 [mu]mol photons m-2 s-1. Ribulose-5-phosphate kinase (EC 2.7.1.19) appeared never to be limiting to photosynthesis, and saturated at much lower PFD than either FBPase or Rubisco. Determination of photosynthetic metabolite pool sizes from leaves at different positions within a soybean canopy showed a limitation to carbon uptake at the stromal FBPase and possibly the sedoheptulose-1,7-bisphosphatase (EC 3.1.3.37) in shade leaves upon initial illumination at saturating PFD levels.

The mechanisms regulating transient photosynthesis by soybean (Glycine max) leaves were examined by comparing photosynthetic rates and carbon reduction cycle enzyme activities under flashing (saturating 1 s lightflecks separated by low photon flux density (PFD) periods of different durations) and continuous PFD. At the same mean PFD, the mean photosynthetic rates were reduced under flashing as compared to continuous light. However, as the duration of the low PFD period lengthened, the CO2 assimilation attributable to a lightfleck increased. This enhanced lightfleck CO2 assimilation was accounted for by a greater postillumination CO2 fixation occurring after the lightfleck. The induction state of photosynthesis, ribulose-1,5-bisphosphate carboxylase/oxygenase (rubisco), fructose 1,6-bisphosphatase (FBPase) and ribulose 5-phosphate kinase (Ru5P kinase) activities all responded similarly and were all lower under flashing as compared to constant PFD of the same integrated mean value. However, the fast phase of induction and FBPase and Ru5P kinase activities were reduced more than were the slow phase of induction and rubisco activity. This was consistent with the role of the former enzymes in the fast induction component that limited RuBP regeneration. Competition for reducing power between carbon metabolism and thioredoxin-mediated enzyme activation may have resulted in lower enzyme activation states and hence lower induction states under flashing than continuous PFD, especially at low lightfleck frequencies (low mean PFD).

A 3-week-old infant with meningococcal meningitis is described whose clinical course was complicated by paraparesis and urinary retention. This rare complication of meningococcal meningitis has not previously been reported in the neonatal age group.

The temporally variable light environment of natural plant canopies presents distinct limitations to carbon assimilation, partially as a result of the photosynthetic induction requirement that develops when leaves are shaded. This study was undertaken with soybean (Glycine max L.) leaves to further identify factors contributing to the activation state of the fast component of induction during low photosynthetic photon flux density (PPFD) periods. Determination of pool sizes of carbon reduction cycle intermediates at low light and upon return to saturating light indicated that different limitations to photosynthetic activity arise over the time course of a 10-minute low PPFD period. Photosynthetic activity upon reillumination was limited by the regeneration of ribulose 1,5-P(2). There was an increase in the levels of fructose 1,6-P(2), sedoheptulose 1,7-P(2), triose-P, ribose 5-P, and ribulose 5-P pools, indicating inactivation of stromal enzymes, most notably fructose 1,6-bisphosphatase, sedoheptulose 1,7-bisphosphatase, and ribulose 5-P kinase. The fast-induction component was the most important factor limiting assimilation during rapid, brief light transients, during which the decay of the slow component was minimal. This may be particularly significant for upper leaves in soybean canopies that generally experience very rapid light transients.

Citrobacter diversus brain abscess occurred in two infants in Aberdeen, 5 months apart. These are the first reported cases of this condition in the UK since 1976. Restriction endonuclease analysis with SacI enzyme showed blood and CSF isolates from both patients to be identical and different from 10 other clinical isolates of C. diversus and one C. amalonaticus strain. Furthermore, isolates of C. diversus from patients belonged to biotype "d" whereas control isolates were of biotypes "a" or "e". Because both infants attended the same central and peripheral maternity units, this raised suspicions of long term contamination of the hospital environment by this organism. Sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) patterns of whole-cell proteins and immunoblotting with normal human serum were remarkably homogeneous for all 13 C. diversus strains and thus were not useful for typing. However, the only C. amalonaticus strain was clearly differentiated from C. diversus strains by SDS-PAGE. Management of the infants included multiple intravenous antibiotic therapy for 4-6 weeks and repeated computerised tomography (CT) scanning and drainage of the abscess cavity. Both children survived albeit with some minor degree of brain damage.

Plasma ammonia was measured in 59 children requiring anticonvulsant drugs: 37 children (group 1) on sodium valproate alone or in combination with other drugs and 22 children (group 2) on drugs other than sodium valproate. Plasma ammonia was higher in group 1 children. Total and free carnitine was measured in plasma and erythrocytes of all children and in the urine of 16 children from group 1 and eight from group 2. Plasma and erythrocyte free carnitine was significantly lower in the children on sodium valproate, along with a significant increase in the ratio of acyl (bound) carnitine to free carnitine. No significant correlation was found between plasma ammonia and carnitine concentrations for either group of children. Plasma and erythrocyte concentrations were not related. Urinary free carnitine was reduced in children treated with valproate, with a significant increase in the ratio of bound to free carnitine. Carnitine supplementation is discussed.

In 1964, one of us (WHG) undertook a retrospective study of bacterial meningitis in childhood in the north east of Scotland during the period 1946-61. We have recently carried out a similar review of cases occurring during 1971-86, to compare the incidence, mortality, and bacteriological patterns. During the earlier period 285 cases occurred, a total incidence of 16.9/100,000 children per year. In the later period 274 children were affected, an annual incidence of 17.8/100,000. The overall mortality rate fell dramatically from 11.9% to 1.8%, the latter figure comparing favourably with recent published studies from Scandinavia and the United States. There was a change in the bacteriological profile in the second period with a significant rise in cases due to Haemophilus influenzae at all ages. A fall in cases of meningococcal meningitis was significant in infants under 1 year of age only. Possible reasons for the change in the bacteriological pattern are discussed.

Three cases of congenital cranial hemihypertrophy are described. CT or ultrasound scans showed unilateral cerebral enlargement with dilatation of the ipsilateral ventricle. Seizures occurred in two patients and the neurodevelopmental outlook appears poor. These patients represent a poor prognosis subgroup of the congenital hemihypertrophies.

Transketolase and pyruvate changes were studied in rats infected with Trypanosoma lewisi and fed complete, thiamine-deficient and pair-fed control diets. Regardless of the dietary group, marked increases in pyruvate levels were observed in the infected animals. There were no significant differences in erythrocyte transketolase activity of rats given a full complement diet. Significant decreases, however, were observed in the transketolase activity of pair-fed and thiamine deficient rats. The greater decreases occurred in the infected animals.

Malaria, the number one disease in the world, is caused by intracellular protozoans belonging to the Subphylum, Sporozoa; Suborder, Haemosphoridia; and Family, Plasmodiidae. The four classical organisms producing disease in man are Plasmodium vivax, P. falciparum, P. malariae, and P. ovale. Although malaria has been known to man for centuries, attempts are still being made to control and eliminate its devastating effects in tropical and subtropical areas of the world. Current active interest in malarial immunology and immunopathology derives from two main facts: (1) that human malaria is still one of the chief health problems in a broad tropical and subtropical zone in which lie most of the developing countries; and (2) most of the seminal leads in basic immunology are being applied to malarial immunology, either directly in human patients, or using laboratory animals as test objects.This paper addresses the nature of malarial immunity and target organs in malarial pathology.

Twelve albino children with IQs in the normal range were psychometrically assessed and compared to a control group of equally visually handicapped children who were non-albino. A striking and consistent pattern of cognitive functioning emerged in the albino group (a large verbal/performance discrepancy in favour of verbal skills) which could not be attributed to their poor visual acuity. This unusual pattern of intellectual functioning may be related to the unique neuroanatomy and neurophysiology recently demonstrated in people with albinism.

Ten experiments were performed to compare mitotic activity in livers of rats infected with Trypanosoma rhodesiense and Trypanosoma lewisi. The host dietary groups were: (1) complete of full complement; (2) folic acid deficient, and (3) pair-fed or calorically restricted. Liver mitotic counts of T. lewisi-infected rats fed complete and pair-fed control diets showed a high degree of significance over that of control rats. From days 35 to 55, liver mitotic counts of T. lewisi-infected rats deficient in folic acid showed an average of 16 +/- 2 mitotic phases as compared to 9 +/- 2 for livers of uninfected animals. Liver mitotic counts of T. rhodesiense-infected and non-infected rats fed complete and pair-fed control diets showed that there were essentially no differences in the mitotic counts from that seen in liver from control, uninfected animals. On day 25 (day 5 of infection), folic acid-deficient infected rats showed a significantly higher number of dividing cells than the controls. Folate was measured in isolated, washed T. lewisi and T. rhodesiense cells. T. lewisi and T. rhodesiense contained 9.6 ng folate per 10(8) cells and 6.2 ng of folate per 10(8) cells respectively. Metabolic products of T. lewisi contained an average of 0.51 ng of folate per 10(8) trypanosomes. T. rhodesiense metabolic products contained 0.3 ng of folate per 10(8) trypanosomes.

Malnutrition combined with susceptibility to infection threatens the health and survival of a large portion of the world's population. Of the many components of the host-parasite relation, nutrition has not received the attention it deserves. There has been increasing activity in recent years on the elucidation of the role nutritional factors play in the behavior of both host and parasite.This paper reviews the effects vitamins exert on the course of parasitic infections.

An experimental model composed of the folic acid deficient Sprague-Dawley rat and Trypanosoma rhodesiense was used to study folate levels in sera and tissues. Serum folate levels in well fed rats inoculated on day 21 averaged 21 plus or minus ng/ml; well fed normal rats averaged 18 plus or minus 4 ng/ml. In rats given the pair-fed control diet, serum folate levels averaged 17.2 plus or minus 4 ng/ml for trypanosome-free animals and 20.2 plus or minus 3 ng/ml for infected ones. In rats given the folic acid-deficient diet, serum folate levels averaged 8.6 plus or minus 2 ng/ml for noninfected control animals and 9.3 plus or minus 2 ng/ml for trypanosome-infected ones. Regardless of diet, the infected animals inoculated on day 56 had higher serum folate levels over the controls on the last day of observation (day 5 of infection). Livers from rats fed complete and pair-fed diets and inoculated on day 21 showed no significant differences in folate content when compared to control animals. However, livers of rats on a deficient diet showed significantly more folate when compared with uninfected controls, reaching a maximum of 362% of day 25. Liver folate levels of rats (regardless of the dietary regimen) inoculated on day 56 showed significantly higher values than the controls on day 60. Irrespective of the time of inoculation or diet, brain and spinal cord of T. rhodesiense-infected rats had significantly higher folate values than their controls on day 5 of infection. The folate level of the brain and spinal cord, at this time, ranged up to 58 and 107% respectively.

Accumulative evidence in the scientific literature relating malnutrition and susceptibility to infection in human beings and animals has contributed significantly to the understanding of the maladies among the malnourished of the world. In the dispensation of health care systems around the world, little or no emphasis has been placed on trace-element nutrition. This paper adds to the available evidence by using a metabolic imbalance technique to investigate the effects zinc deficiency exert on the course of a parasitic infection. A model composed of zinc-deficient mice and Trypanosoma musculi was used to study changes in serum immunoglobulins.

1. Zinc deficiency was studied in mice infected with Trypanosoma musculi. 2. In all dietary groups, the infected animals consumed more food and gained more weight than the uninfected controls. 3. In all studies, at the peak of parasitemia, the zinc-deficient animals showed three times the number of trypanosomes as that of the complete and pair-fed mice. 4. The average coefficients of variation in body lengths of T.musculi cells indicated that the formation of the reproductive-inhibiting antibody (ablastin) occurred later in zinc-deficient animals compared to animals fed complete diets. The degree and duration of parasitemia in the zinc-deficient animals indicated a delay in the synthesis of the terminal lytic antibody. 5. Irrespective of diets, severe depression in the primary and secondary antibody responses to in vivo immunization of sheep erythrocytes was observed in infected animals over non-infected controls.

Recent studies of Guillain-Barré syndrome in adults have shown that the requirement for assisted ventilation correlates closely with a poor outcome, and the need for ventilation is now being used as an indication for plasmapheresis. As comparable studies in children have not been performed we reviewed our experience of patients who had Guillain-Barré syndrome severe enough to require assisted ventilation. In a group of 11 children two died in the acute stage of the illness and nine made an excellent recovery. It would seem that the need for mechanical ventilation is not necessarily a bad prognostic factor for neurological recovery in children.

Sixteen blind babies who were considered to be showing the characteristics of delayed visual maturation were studied prospectively. The diagnosis was made on clinical grounds, and the criteria for this are discussed. All of these infants developed visual responses between 4 and 6 months of age and had normal or near normal visual acuities by 1 year of age. Long term follow up, however, has shown neurological abnormalities in some of these children.

Although malaria and hookworm disease appear to be on the decline, another dreaded parasitic disease-schistosomiasis-is on the increase. Presently, the number of infected individuals with schistosomes is estimated to be 250 million, and even though only a small proportion of them become sick and die, schistosomiasis remains a medical problem of great significance. The high incidence of infection of man with Schistosoma mansoni, Schistosoma japonicum or Schistosoma haematobium, as well as the chronic debilitating diseases produced, places these organisms among the world's most important infectious agents. This paper discusses the nature of immunity to schistosomiasis.