Cerebral Palsy

Trochlear nerve palsy associated with spontaneous subarachnoid hemorrhage (SAH) is known to be a rare malady. We report here on a patient who suffered with left trochlear nerve palsy following rupture of a right posterior communicating artery aneurysm. A 56-year-woman visited our emergency department with stuporous mental change. Her Hunt-and-Hess grade was 3 and the Fisher grade was 4. Cerebral angiography revealed a ruptured aneurysm of the right posterior communicating artery. The aneurysm was clipped via a right pterional approach on the day of admission. The patient complained of diplopia when she gazed to the left side, and the ophthalmologist found limited left inferolateral side gazing due to left superior oblique muscle palsy on day 3. Elevated intracranial pressure, intraventricular hemorrhage or a dense clot in the basal cisterns might have caused this trochlear nerve palsy.

This report is a case of slipped capital femoral epiphysis in a nonambulatory patient with spastic quadriplegic type of cerebral palsy. Such a case is unusual as no weight-bearing forces were acting on the hip; however, spasticity may have played a role. To the best of our knowledge no earlier cases have been reported in the literature.

Whipple’s disease is a rare multisystemic infection caused by the intracellular bacteria Thropheryma whippelii. Central nervous system (CNS) involvement is not rare. The most frequent CNS manifestations are cognitive and behavioural changes, sopranuclear ophtalmoplegia, myoclonus, epilepsy, ataxia, meningitis and focal cerebral palsy. We report one case of cerebral localization of Whipple’s disease with a clinical presentation of recurrent endocranic hypertension and hydrocephalus, and uncommon neurological symptoms, successfully treated by endoscopic third ventriculostomy and antibiotic therapy with ceftriaxone and Trimethoprim-Sulfamethoxazole. Copyright © 2010 Elsevier B.V. All rights reserved.

BACKGROUND: Children with cerebral palsy (CP) expend more energy when walking than do their typically developing peers. The effect this has on physical activity levels (PALs) and on total energy expenditure (TEE) will have important implications when determining energy requirements. OBJECTIVES: This study aimed to investigate the components of TEE in children with CP in comparison with typically developing children and to determine what effect the higher energy expenditure during walking has in ambulatory children with CP on PAL and on TEE. DESIGN: Sixteen children with mild CP and 16 typically developing children, aged 5-12 y, were recruited for the study. Resting energy expenditure (REE) and the energy expenditure during walking were measured by using indirect calorimetry. TEE was determined by using the doubly labeled water technique. PAL was calculated as the ratio of TEE to REE. Body composition was estimated by using oxygen-18. RESULTS: TEE was lower in children with CP (7012 +/- 1268 kJ/d) than in typically developing children (8309 +/- 2088 kJ/d) because of a lower PAL (1.57 +/- 0.23 compared with 1.79 +/- 0.26). The children with CP expended significantly more energy when walking than did the typically developing children (13.8 +/- 4.9 compared with 10.3 +/- 2.3 kJ/min) while walking at a lower velocity (61 +/- 10 compared with 72 +/- 8 m/min). Correlations between energy expenditure during walking and PAL were not statistically significant for either group. CONCLUSIONS: Children with CP expend more energy during walking and have a lower PAL and lower energy requirements than do typically developing children. This has important implications when estimating the energy requirements of children with CP.

Time trends for cerebral palsy (CP) prevalence in children born >/=2,500 g vary across studies and scarce data exist on trends by subtype of CP. The objective of this study was to describe changes in prevalence of CP in infants born >/=2,500 g between 1980 and 1998 in Europe. Data were collated from the SCPE (Surveillance of Cerebral Palsy in Europe collaboration) common database. Poisson regression was used to test for change in prevalence over time. Birth year and register effects were explored and trends in prevalence were estimated by CP subtype and severity. Four thousand and two children with CP and birthweight >/=2,500 g were recorded in 15 population based-registers. The overall prevalence of CP was 1.16 per 1,000 live births (99% CI, 0.88-1.48) in 1980 and 0.99 (CI, 0.80-1.20) in 1998. The trend was not significant (P = .14), except in two registers. However, there were significant changes in the prevalence of spastic CP subtypes, with a decrease in the bilateral spastic form (P < .001), and an increase in the unilateral spastic form (P = .004). There was a concurrent reduction in neonatal mortality of children with birthweight >/=2,500 g: from 1.7 (CI, 1.4-2.1) to 0.9 (CI, 0.7-1.1) per 1,000 live births. In conclusion, for children born with birthweight >/=2,500 g, the prevalence of CP in Europe was stable in spite of changes by subtype and a significant decrease in neonatal mortality.

Achievement of urinary continence is an important developmental step that most children attain with the assistance of their parents and caregivers. Debate continues as to the best time to toilet train; in some Asian and African cultures children are trained as infants, while training at age 2-3 years is more typical in Western cultures. Infant voiding is not merely a spinal reflex, as the sensation of bladder filling is relayed to the brain. However, the ability of the brain to inhibit bladder contractions, and to achieve coordinated bladder contraction with sphincter relaxation, matures over time. While there is a concern that later toilet training may be responsible for an increase in urinary incontinence in children, no controlled studies on early versus late toilet training exist to evaluate this hypothesis. A number of medical conditions such as spina bifida, posterior urethral valves, cerebral palsy and autism can cause incontinence and difficulties in toilet training. The decision to start toilet training a child should take into account both the parents’ expectation of how independent the child will be in terms of toileting, and the child’s developmental readiness, so that a realistic time course for toilet training can be implemented.

Rats with bilateral neonatal ventral hippocampus lesions (NVHL) are commonly used for modeling developmental aspects of the pathophysiology of schizophrenia. Given that functional changes become significant only after puberty, NVHL as well as sham operated rats were analyzed at the ages of 21, 42 and 63days (i.e. as pups, adolescents and adults), using MRI to examine the damage caused by surgery over time. Morphometric evaluations were considered and lesions were classified as small, medium and large. The volume of lesions increased regularly with age, to a greater extent than increases in overall brain size. This was relatively linear, corresponding to a gradually shrinking forebrain, and these observations held true for each class of lesions considered. Following the observation that the lesion procedure elicited calcifications in the brain, the same rats were subjected to 3D X-ray scanning the day after each MRI session, allowing precise measurements of skull size to be carried out. The NVHL rats had smaller skulls, however the dimensions of the calcifications did not grow more than the skull size over time. The mechanisms underlying the progressive anatomical changes following surgery are discussed. and we propose this in vivo follow-up method to investigate therapeutic strategies aimed at countering or limiting the post-lesion consequences of a neonatal brain damage. Copyright © 2010. Published by Elsevier Inc.

Spine-associated Rap guanosine triphosphatase-activating protein (SPAR) is an important regulator of activity-dependent remodeling of synapses. It is also critically involved in both mature dendritic spine formation and the maintenance of spine maturity. Glutamate is a major neurotransmitter of the brain, and is involved in all aspects of cognitive function, as it is the primary transmitter utilized by the cortical and hippocampal pyramidal neurons. Glutamate has also been associated with neuronal dendritic spine damage. The precise molecular mechanisms underlying dendritic spine damage following glutamate-induced neurotoxicity remain unknown. In the current study, we measured mRNA and protein expression levels of SPAR and serum-inducible kinase (SNK) in primary hippocampal neurons following glutamate treatment. Expression of SPAR and SNK was altered by glutamate treatment, indicating that the SPAR and SNK signaling pathways may be involved in the damage to dendritic spines in hippocampal neurons following excitotoxicity induced by glutamate. Copyright © 2010 Elsevier Ltd. All rights reserved.

As an abnormally folded and aggregated protein, tau composed of neurofibrillary-tangles (NFTs) in Alzheimer’s-disease and other tauopathies- seems to be a candidate for immunotherapy. Yet, the encephalitogenicity of full-length tau protein, recently reported by us in immunized mice, demands to carefully and selectively target pathological tau and address both efficacy (anti-NFT effect) and safety (free of encephalitis). We immunized NFT-mice with NFT-related phosphorylated (phos)-tau peptides, using an immunization protocol aimed to predispose a proinflammatory milieu in CNS as a set up to detect biohazard, an approach we used when the neurotoxicity of full length tau was detected [use of complete Freund adjuvant (CFA) with pertussis-toxin (PT)]. A decrease of about 40% in NFT-burden in CNS was demonstrated and was accompanied with an increase in microglial burden. Anti-phos-tau antibodies were detected in serum and blood vessels in the CNS, while no encephalitogenicity (free of clinical neurological deficits, of adverse effects on brain inflammatory cells and of axonal damage) was recorded. The level of the lysosomal proteases, cathepsin D and L, were affected in the immunized mice suggesting the possible involvement of the lysosomal system in the decrease of NFTs. The robust anti-NFT effect and lack of encephalitogenicity in NFT-mice immunized with phos-tau peptides, even-though CFA with PT was included in vaccine, point to their anti-NFT therapeutic potential. Copyright © 2010. Published by Elsevier Inc.

Hypertension reduces endothelial nitric oxide synthase (eNOS) expression and leads to endothelial dysfunction. However, few studies have demonstrated the influences of hypertension on eNOS function in the cerebral cortex. The present study investigates the influences of hypertension on endothelial function in the cerebral cortex and the protective effects of antihypertensive agents against brain ischemia through the preservation of endothelial function. Five- and ten-week-old male Wistar rats and spontaneously hypertensive rats (SHR) were used for experiments. Five-week-old SHR received olmesartan, hydralazine, or vehicle for 5 weeks in drinking water. eNOS activation in the cerebral cortex was evaluated by analyzing levels of total and Ser(1177)-phosphorylated eNOS protein by Western blot. Blood pressure of 10-week-old SHR without treatment was clearly high, and the ratio of phospho-eNOS/total eNOS protein was significantly low. Five-week treatment with olmesartan or hydralazine suppressed the elevation of blood pressure and the reduction of phosphorylated eNOS-Ser(1177) in SHR, and olmesartan was more effective in maintaining phosphorylation of eNOS-Ser(1177) than hydralazine. To assess the contribution of eNOS to maintaining cerebral blood flow (CBF), we monitored CBF by laser-Doppler flowmetry after L-N(5)-(1-iminoethyl)ornithine (L-NIO) infusion. CBF response to L-NIO was preserved in olmesartan-treated SHR but not in hydralazine-treated SHR. Furthermore, infarct volume 48 hr after transient focal brain ischemia in olmesartan-treated SHR was significantly reduced compared with vehicle-treated SHR. These findings indicate that chronic prehypertensive treatment with olmesartan could attenuate brain ischemic injury through the maintenance of endothelial function in the cerebral cortex in SHR. (c) 2010 Wiley-Liss, Inc.