Cerebral Palsy

Therapeutic hypothermia in acute resuscitation medicine has a long history, but its currently recommended use dates back to work in the mid-1960s by the late Dr. Peter Safar and colleagues. Compared with normothermia, mild therapeutic hypothermia, induced right after restoration of spontaneous circulation in comatose survivors of cardiac arrest, leads to 1 additional patient with intact neurological outcome for every 6 patients treated. Demonstrating benefit from therapeutic hypothermia in other acute neurological insults, such as traumatic brain injury, has been more difficult. Current research to optimize the benefits of mild therapeutic hypothermia in cardiac arrest is focused on hypothermia’s profound effects on drug metabolism, determining the best anesthetics and sedatives to use with cooling, and identifying compounds that may promote induction of hypothermia or create a poikilothermic state. Future applications of therapeutic hypothermia may include induction of emergency preservation and resuscitation to buy time for damage-control surgery in patients with exsanguination cardiac arrest.

PURPOSE: To evaluate the late effects of radiation-induced damages in the rat brain by means of in vivo multiparametric MRI. MATERIALS AND METHODS: The right hemibrains of seven Sprague-Dawley rats were irradiated with a highly collimated 6 MV photon beam at a single dose of approximately 28 Gy. Diffusion tensor imaging (DTI), proton MR spectroscopy ((1)H-MRS), T2-weighted imaging, and T1-weighted imaging were performed to the same animals 12 months after radiation treatment. RESULTS: Compared with the contralateral side, a significantly higher percentage decrease in fractional anisotropy was observed in the ipsilateral fimbria of hippocampus (29%) than the external capsule (8%) in DTI, indicating the selective vulnerability of fimbria to radiation treatment. Furthermore, in (1)H-MRS, significantly higher choline, glutamate, lactate, and taurine peaks by 24%, 25%, 87%, and 58%, respectively, were observed relative to creatine in the ipsilateral brain. Postmortem histology confirmed these white matter degradations as well as glial fibrillary acidic protein and glutamine synthetase immunoreactivity increase in the ipsilateral brain. CONCLUSION: The microstructural and metabolic changes in late radiation-induced brain injuries were documented in vivo. These multiparametric MRI measurements may help understand the white matter changes and neurotoxicity upon radiation treatment in a single setting. J. Magn. Reson. Imaging 2009;29:1013-1020. (c) 2009 Wiley-Liss, Inc.

Functional impairment after focal CNS lesion is highly dependent on damage that occurs in regions that are remote but functionally connected to the primary lesion site. This pattern is particularly evident in the cerebellar system, in which functional interactions between the cerebellar cortex, deep cerebellar nuclei, and precerebellar stations are of paramount importance. Diffuse degeneration after development of a focal CNS lesion has been associated with poor outcomes in several pathologies, such as stroke, multiple sclerosis, and brain trauma. A greater understanding of the mechanisms that underlie the spread of death signals from focal lesions, however, can aid in identifying a neuroprotective approach for CNS pathologies. To this end, studies on degenerative mechanisms in the inferior olive and pontine nuclei after focal cerebellar damage have been a valuable asset in which pharmacological approaches have been tested. In this review, we focus on mechanisms of remote cell death in cerebellar circuits, analyzing the neuroprotective effects of inflammation-modulating drugs in particular.

OBJECTIVE: The optimal surgical approach for a trigone meningioma is still controversial. Here, we report two patients with trigone meningioma treated successfully via an occipital inter-hemispheric and trans-cortical approach in the lateral semi-prone position. CLINICAL PRESENTATION: A 53-year-old woman was admitted to a local hospital with sudden transient dizziness and vomiting. The CT brain scan demonstrated a right intra-ventricular tumour. She was therefore transferred to our hospital for surgical treatment. The other patient was a 67-year-old woman who was admitted to a local hospital after a traffic accident and a CT brain scan revealed an incidental right intra-ventricular tumour. After referral to our hospital, periodic MRI examinations revealed gradual tumour enlargement within a one-year period. Neither of the patients had any neurological deficits, including in the visual fields. INTERVENTION: The head of each patient was positioned so that the tumour-containing right ventricle was oriented downwards and laterally. An occipital inter-hemispheric approach was performed and using a navigation system, the tumour was identified about 1 cm in depth from the cortical surface. After the medial part of the tumour was debulked, the posterior and then the anterior choroidal blood supplies to the tumour were identified. Occlusion of these vessels achieved tumour haemostasis. The tumours were totally removed via a 1.5-cm cortical incision. Brain retraction was minimal because the right hemisphere was pulled down by gravity. Therefore, the para-splenial cisterns were easily accessed, resulting in early release of cerebrospinal fluid. Post-operative MRI showed complete removal of the tumour and the patients had no neurological deficits. Anti-epileptic medication was withdrawn one week after the operation. CONCLUSIONS: The occipital inter-hemispheric fissure lacks important bridging veins. The approach used and patient positioning minimized damage to the lateral aspect of the optic radiation and the corpus callosum. Except in patients with very large trigone meningiomas, this approach is useful for decreasing the risk of post-operative hemianopsia or epilepsy, and possibly speech disturbance, even in patients with a tumour in the dominant hemisphere.

Hypersalivation associated with cerebral palsy may be treated with injection of botulinum toxin A (BTX-A) into the submandibular gland, and the use of ultrasound permits its accurate administration. In our series four patients with cerebral palsy and hypersalivation had bilateral ultrasound-guided injection of BTX-A into the submandibular gland. At 4 weeks there was objective improvement in all patients and subjective improvement in three. The only reported side effect was the temporary inability to retain prosthetic orbital globes in one patient. Ultrasound-guided injection of BTX-A for hypersalivation is effective, and side effects are rare, but they have yet to be fully described.

This review presents a brief account of the most significant biological effects and clinical applications of botulinum neurotoxins, in a way comprehensive even for casual readers who are not familiar with the subject. The most toxic known substances in botulinum neurotoxins are polypeptides naturally synthesized by bacteria of the genus Clostridium. These polypeptides inhibit acetylcholine release at neuromuscular junctions, thus causing muscle paralysis involving both somatic and autonomic innervation. There is substantial evidence that this muscle-paralyzing feature of botulinum neurotoxins is useful for their beneficial influence on more than 50 pathological conditions such as spastic paralysis, cerebral palsy, focal dystonia, essential tremor, headache, incontinence and a variety of cosmetic interventions. Injection of adequate quantities of botulinum toxins in spastic muscles is considered as a highly hopeful procedure for the treatment of people who suffer from dystonia, cerebral palsy or have experienced a stroke. So far, numerous and reliable studies have established the safety and efficacy of botulinum neurotoxins and advocate wider clinical therapeutic and cosmetic applications. Copyright © 2009 S. Karger AG, Basel.

A 45-year-old female developed mild dysesthesia and swelling, followed by ptosisand trigeminal pain, in the right side of the face. Her past medical history was unremarkable, and she had not been aware of any infectious sign. A local otolaryngologist administered glucocorticoid therapy that resolved the face pain, but the ptosis persisted. Neurological examination found complete right oculomotor nerve paresis and mild sensory loss in the first and second segments of the right trigeminal nerve. Blood examination found no abnormalities. Neuroimaging revealed a saccular aneurysm at the branching site of the posterior communicating artery, projecting posteriorly and adjacent to the dorsum sellae, without other intracranial abnormalities. Cerebral angiography demonstrated poor opacification of the superior ophthalmic vein and cavernous sinus on the right side. The patient underwent coil embolization under a diagnosis of symptomatic aneurysm, but her oculomotor neuropathy was only partially improved. We thought that the impairment of the oculomotor function by inflammatory reaction in the cavernous sinus and mechanical compression by the aneurysm had already persisted for too long for post-treatment recovery. We think that the simultaneous occurrence of Tolosa-Hunt syndrome and oculomotor nerve palsy may have resulted because trigeminal neuralgia had increased the blood pressure to induce rapid growth of the preexisting aneurysm, or the inflammatory reaction in the cavernous sinus had promoted the growth of the aneurysm, or that the association was by chance.

OBJECTIVE: To quantify the level of everyday physical activity in adults with bilateral spastic cerebral palsy, and to study associations with personal and cerebral palsy-related characteristics. Participants and methods: Fifty-six adults with bilateral spastic cerebral palsy (mean age 36.4 (standard deviation (SD) 5.8) years, 62% male) participated in the study. Approximately 75% had high gross motor functioning. Level of everyday physical activity was measured with an accelerometry-based Activity Monitor and was characterized by: (i) duration of dynamic activities (composite measure, percentage of 24 h); (ii) intensity of activity (motility, in gravitational acceleration (g)); and (iii) number of periods of continuous dynamic activity. Outcomes in adults with cerebral palsy were compared with those for able-bodied age-mates. RESULTS: Duration of dynamic activities was 8.1 (SD 3.7) % (116 min per day), and intensity of activity was 0.020 (SD 0.007) g; both outcomes were significantly lower compared with able-bodied age-mates. Of adults with cerebral palsy, 39% had at least one period of continuous dynamic activities lasting longer than 10 min per day. Gross motor functioning was significantly associated with level of everyday physical activity (Rs -0.34 to -0.48; p </= 0.01). CONCLUSION: Adults with bilateral spastic cerebral palsy, especially those with low-level gross motor functioning, are at risk for an inactive lifestyle.

OBJECTIVE: To determine the prevalence of motor impairments and activity limitations and their inter-relationships in Dutch children with spastic cerebral palsy. PATIENTS AND METHODS: In a population-based survey 119 children, age range 6-19 years, with spastic cerebral palsy were examined. Anthropometry, muscle tone, abnormal posture, joint range of motion, major orthopaedic impairments and gross motor functioning and manual ability were assessed or classified, in addition to limitations in mobility and self-care activities. Spearman’s correlation coefficients, bivariate post hoc analyses and univariate and multivariate logistic regression analyses were used. RESULTS: Children with spastic cerebral palsy had a lower body height and weight compared with typically developing peers. Forty percent had no range of motion deficits. Hip dislocations were rarely encountered. Motor impairments were associated with gross motor functioning and manual ability levels. Close to sixty-five percent walked independently. Children with diplegia and tetraplegia differed in activity limitations. Motor impairments and limitations in mobility and self-care activities were only modestly related in multivariate analyses. CONCLUSION: Distribution of cerebral palsy-related characteristics is consistent with that found in representative studies of other countries. The distinction between diplegia and tetraplegia is relevant from an activity point of view. The child’s activity limitations are not a mirror of the motor impairments, which suggests multifactorial influences. An activity-oriented rehabilitation approach goes beyond treating specific impairments.

here is developmental refinement of the proprioreceptive muscle afferent input to the rat ventral horn. This study explored the extent to which this occurs in the medulla. Muscle afferents were transganglionically labelled from the extensor digitorum communis forelimb muscle with cholera toxin B sub unit. Tracer amounts and transport times were adjusted for animal size. Immunohistochemistry revealed tracer localisation in the medulla and dorsal root ganglia. Labelled muscle afferent boutons were counted in the cuneate nucleus between postnatal days 7and 42, during which time a large decrease in the density of labelled boutons was observed qualitatively. Localisation of input to dorsolateral parts of the nucleus remained broadly the same at different ages, although disappearance of a marked innervation of ventromedial regions in more caudal sections was observed. Bouton counts were corrected for growth of the medulla with age, and any spread of tracer to adjacent muscles indicated by counts of labelled dorsal root ganglion neurons. There was a statistically significant, approximately 40% reduction in the number of muscle afferent boutons in the cuneate nucleus during this developmental period. Previous studies suggest that perturbations to the corticospinal input during a developmental critical period influence the eventual size of the muscle afferent input to the ventral horn. Corticocuneate fibres invade the nucleus during the same period and may influence reorganisation of its muscle afferent input, making it another potential site for aberrant reflex development in cerebral palsy.