Cerebral Palsy

Magnetic resonance imaging (MRI) is a safe and universally available test that has much appeal to generalists and subspecialists evaluating patients with pancreatic disease. Testoni et al. report the clinical utility of magnetic resonance pancreaticocholangiography (MRCP) and secretin-enhanced MRCP in the evaluation of patients with asymptomatic abnormalities in pancreatic enzymes. The authors report that chronic pancreatitis changes will be seen in up to a third of patients with asymptomatic elevations in pancreas enzymes when compared with age-matched controls. The changes described on MRI and secretin-enhanced MRI are not in question, but the clinical significance of these changes is unknown. The authors rightly report that some of the changes seen may be age related, nonspecific, and of unknown clinical significance. Any new imaging and diagnostic test needs to be interpreted with caution until appropriate prospective clinical trials have been performed. It appears that secretin stimulation enhances the diagnostic accuracy of MRCP for the detection of minor changes in the pancreatic duct and parenchyma. Gastroenterologists are encouraged to proceed with cautious optimism when using MRI for the evaluation of early chronic pancreatitis.Am J Gastroenterol advance online publication, 19 May 2009; doi:10.1038/ajg.2009.168.

The incidence of coronary artery anomalies is about 0.6-1.3% in angiographic studies of the adult population. Double right coronary artery is one of the rarest abnormalities reported in the literature. We report a case of a double right coronary artery originating from a single orifice in a woman who underwent coronary angiography for a pathological transprosthetic aortic gradient.

OBJECTIVE:: Neurocognitive disorders are devastating consequences of HIV infection. Although antiretroviral regimens have been efficacious in both improving life expectancy and decreasing dementia, there has not been an effect on the overall prevalence of HIV-associated neurocognitive disorders. Whether early institution of treatment, or treatment with drugs that effectively penetrate the blood-brain barrier, would help protect from such conditions is not known. Using the simian immunodeficiency virus/macaque model, we investigated the hypothesis that early introduction of antiretroviral treatment can protect the brain. DESIGN AND METHODS:: Animals were inoculated with simian immunodeficiency virus, and upon resolution of the acute infection period divided into two groups and treated, or not, with combination antiretroviral therapy. Viral, immune, and physiological parameters were measured during the course of infection, followed by assessment of viral, immune, and molecular parameters in the brain. RESULTS:: We observed that even with agents that show poor penetration into the central nervous system, early antiretroviral treatment prevented characteristic neurophysiological and locomotor alterations arising after infection and resulted in a significant decrease in brain viral load. Although the number of infiltrating immune cells in the brain did not change with treatment, their phenotype did, favoring an enrichment of effector T cells. Early treatment also significantly lowered brain levels of interferon-alpha, a cytokine that can lead to neurocognitive and behavioral alterations. CONCLUSION:: Early antiretroviral treatment prevents central nervous system dysfunction by decreasing brain viral load and interferon-alpha levels, which can have a profound impact over the course of infection.

STUDY DESIGN: Retrospective case series of magnetic resonance imaging (MRI) findings in patients with presumed infantile idiopathic scoliosis (IIS). OBJECTIVE: To identify the prevalence of intraspinal anomalies in patients with presumed IIS at a single, large volume institution and further define the role for a screening MRI. SUMMARY OF BACKGROUND DATA: Idiopathic scoliosis in patients less than 10 years of age has been shown to carry a higher prevalence of concordant intraspinal anomalies, up to 20%, in juveniles, when compared to the adolescent age group. Few studies exist assessing the prevalence of intraspinal anomalies in the IIS patient population. Dobbs et al (2002) reported a 21.7% prevalence of neural axis abnormalities in 46 patients with presumed IIS across 3 combined spinal deformity clinics and recommended a screening MRI for all IIS patients with a curve >20 degrees. METHODS: A retrospective review of the medical records of 54 patients at a single institution with a presumed diagnosis of IIS was performed. All patients satisfied the strict inclusion criteria of: curve > or =20 degrees, age <36 months at diagnosis, normal neurologic examination (i.e., normal tone, motor strength, reflexes, etc.), absence of any concomitant syndromes or congenital anomalies, and an MRI of the spine from skull to coccyx. RESULTS: MRI revealed a neural axis abnormality in 7 (13%) of 54 patients who underwent an MRI. In this subset of 7 patients, 5 (71.4%) required neurosurgical intervention. Tethered cord requiring surgical release was identified in 3 patients, Chiari malformation requiring surgical decompression was found in 2 patients, and a small nonoperative syrinx was found in 2 patients. CONCLUSION: This study represents the largest evaluation of intraspinal anomalies in IIS to date. Our patient population exhibited a smaller percentage (13%) of neural axis abnormalities than previously reported. On the basis of these findings, the close observation may be a reasonable alternative to an immediate screening MRI in patients presenting with presumed IIS and a curve >20 degrees.

Autism is a neurodevelopmental disorder of unknown etiology characterized by social and communication deficits and the presence of restricted interests/repetitive behaviors. Higher rates of epilepsy have long been reported, but prevalence estimates vary from as little as 5% to as much as 46%. This variation is probably the result of sample characteristics that increase epilepsy risk such as sample ascertainment, lower intelligence quotient (IQ), the inclusion of patients with nonidiopathic autism, age, and gender. However, critical review of the literature reveals that the rate in idiopathic cases with normal IQ is still significantly above the population risk suggesting that autism itself is associated with an increased risk of epilepsy. Recently, there has been interest in the occurrence of epileptiform electroencephalograms (EEGs) even in the absence of epilepsy. Rates as high as 60% have been reported and some investigators propose that these abnormalities may play a causal role in the autism phenotype. Although this phenomenon is still not well understood and risk factors have yet to be determined, the treatment implications are increasingly important. We review the recent literature to elucidate possible risk factors for both epilepsy and epileptiform EEGs. We then review existing data and discuss controversies surrounding treatment of EEG abnormalities.

Orbital lipoma is a rare entity with only a small number of cases previously described. The authors describe a case of orbital lipoma in a 56-year-old man, which was treated with surgical excision. Cytogenetic analysis of the lesion demonstrated abnormalities of chromosome 12, consistent with chromosomal abnormalities in lipomas found elsewhere in the body. Therefore, cytogenetic analysis may be useful to differentiate lipomatous tumors from normal orbital fat.

STUDY DESIGN.: Prospective study. OBJECTIVE.: To determine the effectiveness and correction with posterior multilevel vertebral osteotomy in severe and rigid curves without anterior release. SUMMARY OF BACKGROUND DATA.: For the correction of severe and rigid scoliotic curve, anterior-posterior combined or posterior vertebral column resection (PVCR) procedures are used. Anterior procedure might compromise pulmonary functions, and PVCR might carry risk of neurologic injuries. Therefore, authors developed a new technique, which reduces both. METHODS.: Thirteen neuromuscular patients (7 cerebral palsy, 2 Duchenne muscular dystrophy, and 4 spinal muscular atrophy) who had rigid curve >100 degrees were prospectively selected. All were operated with posterior-only approach using pedicle screw construct. To achieve desired correction, posterior multilevel vertebral osteotomies were performed at 3 to 5 levels (apex, and 1-2 levels above and below apex) through partial laminotomy sites connecting from concave to convex side, just above the pedicle; and repeated cantilever manipulation was applied over temporary short-segment fixation, above and below the apex, on convex side. On concave side, rod was assembled with screws and rod-derotation maneuver was performed. Finally, short-segment fixation on convex side was replaced with full-length construct. Intraoperative MEP monitoring was applied in all. RESULTS.: Mean age was 21 years and average follow-up was 25 months. Average preoperative flexibility was 20.3% (24.1 degrees ). Average Cobb's angle, pelvic obliquity, and apical rotation were 118.2 degrees , 16.7 degrees , and 57 degrees preoperatively, respectively, and 48.8 degrees , 8 degrees , and 43 degrees after surgery showing significant correction of 59.4%, 46.1%, and 24.5%. Average number of osteotomy level was 4.2 and average blood loss was 3356 +/- 884 mL. Mean operation time was 330 +/- 46 minutes. None of the patient required postoperative ventilator support or displayed any signs of neurologic or vascular injuries during or after the operation. CONCLUSION.: This technique should be recommended because (1) it provides release of anterior column without anterior approach and (2) our results supports its superiority as a technique.

OBJECTIVE: To test the hypothesis that pain would be reduced after botulinum toxin type A (Btx/A) treatment for children with cerebral palsy (CP). METHOD: Thirty-four pediatric patients with CP (mean age 9 y; 56% male) and their parents were recruited through a regional specialty healthcare center medical clinic and pain research program. A 1-group pretest, posttest treatment design was used on the basis of a standardized parent-proxy report of their child’s pain. RESULTS: Overall parent ratings of their child’s pain were significantly reduced after Btx/A injection with 62% of parents reporting the absence of pain 1 month after injection (proportion=0.38; 95% confidence interval=0.23-0.55). There were no significant differences for males or females. CONCLUSIONS: Although there is considerable evidence that Btx/A is efficacious for the treatment of spasticity associated with CP, there is little direct evidence specific to associated analgesic effects after Btx/A treatment. These preliminary findings indicate that Btx/A treatment for spasticity resulted in significant pain reduction for this patient sample. This was the first study to directly ask parents about their child’s pain pre-Btx/A and post-Btx/A treatment. These findings have implications for the management of pain associated with spasticity and CP and suggest further research is warranted.

Sonies BC, Cintas HL, Parks R, Miller J, Caggiano C, Gottshall SG, Gerber L: Brief assessment of motor function: Content validity and reliability of the oral motor scales. OBJECTIVE:: The Brief Assessment of Motor Function consists of five 0- to 10-point hierarchical scales designed for rapid assessment of gross, fine, and oral motor skills. We describe the development and evaluation of the two Brief Assessment of Motor Function Oral Motor Scales: Oral Motor Articulation and Oral Motor Deglutition. DESIGN:: This validation study employed an expert panel of 28 speech-language pathologists, who rated the Brief Assessment of Motor Function Oral Motor Scales items on a scale from 1 to 4 (disagree to agree) to establish content validity. For reliability, oral motor performances of 18 participants (6 mos-20 yrs) were videotaped to represent a wide range of articulation and deglutition capabilities. Four speech-language pathologists, and 1 undergraduate and 10 graduate speech-language pathology students rated the participants’ taped samples using the Brief Assessment of Motor Function Oral Motor Scales. RESULTS:: All items on the content validity questionnaire had average agreement scores that exceeded criteria, except two, which were not clearly worded; these were clarified. Interrater and intrarater reliability values were 0.997 and 0.986 for the Oral Motor Articulation Scale and 0.977 and 0.997 for the Oral Motor Deglutition Scale. CONCLUSIONS:: Expert feedback and reliability procedures suggest that the Brief Assessment of Motor Function Oral Motor Articulation and Deglutition Scales represent the content that they are designed to assess and are reliable for rapid assessment of oral motor skills.

BACKGROUND The majority of cases of cerebral palsy (CP) have their pathogenesis during fetal development and are a form of congenital anomaly, the aetiology of which is uncertain. Anomalous development of other organs evident at birth is also a congenital anomaly. A small proportion of these are known to be caused by chromosomal or gene abnormalities, environmental tetratogens and dietary deficiencies. The majority are of unknown aetiology. METHODS A review of monochorionic (MC) monozygotic (MZ) placentation in the pathogenesis of congenital anomalies and CP was conducted using the PubMed, MEDLINE, EMBASE and Cochrane databases. RESULTS Zygote division and MC placentation have serious implications for the development of both conceptuses. Most reports observe predominantly cerebral abnormalities in one or both conceptuses. These cerebral abnormalities often present as CP or other disabilities attributable to central nervous system impairment. In addition to the anomalies in central nervous system development, anomalies in the fetal development of a wide variety of other organs have been reported with MC MZ twinning. CONCLUSIONS CP and congenital anomalies share a common pathogenic mechanism attributable to MZ twinning. These abnormalities in singletons are coincident with very early loss of one conceptus. The quantitative contribution of monozygosity and monochorionicity to the genesis of CP and congenital anomalies needs to be made.