Journal of Pediatric Neurology - Volume 2, issue 2

Authors: Dyken, Paul Richard

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 53-56, 2004

Authors: Dan, Bernard | Cheron, Guy

Article Type: Research Article

Abstract: Forty years ago, a consensual definition of the cerebral palsy concept was suggested, delineating it as a disorder of movement and posture secondary to non-progressive pathological processes that affect the immature brain. Because this concept is pragmatic and based on function, it has survived unaltered many changes in pathophysiolgical knowledge, diagnostic technology and general nosology. However, its basis has appeared to be flawed. Its main justification remains management, for which the need to meticulously select …patients, define adapted objectives, design appropriate management programs and evaluate results has been increasingly recognized. Fine movement analysis using recent technologies can provide a wealth of information about neurological functioning in cerebral palsy that can serve these purposes. Specific patterns of motor organization reveal different modes of motor control in individuals with developmental motor problems. The different motor patterns reflect individual adaptation to the impairment of the central nervous system. Taken phenomenologically these patterns can contribute to the clinical approach to cerebral palsy and redefine patients groups within this framework. (J Pediatr Neurol 2004; 2(2): 57–64). Show more

Keywords: cerebral palsy, history, motor control, movement analysis

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 57-64, 2004

Authors: Hood, Bernadette M. | Harbord, Michael G.

Article Type: Research Article

Abstract: This paper provides an overview of the diagnosis and management of narcolepsy in early childhood. Focusing on the case literature for children aged eight years and under the paper reviews aspects of the pathophysiology, clinical symptomology and clinical management of the illness. In addition factors critical to the differential diagnosis of narcolepsy from a range of other pediatric illnesses are discussed. The literature examining the psychosocial implications of the disorder for children is also presented. Two …major themes emerge from this review (i) the need for a reconceptualisation of the current diagnostic criteria for narcolepsy to allow for the observed heterogeneity and developmental variability of childhood onset narcolepsy, and, (ii) the need for increased attention to the psychosocial difficulties experienced by children in adjusting to this chronic illness. (J Pediatr Neurol 2004; 2(2): 65–71). Show more

Keywords: pediatric narcolepsy, sleep, chronic illness

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 65-71, 2004

Authors: Khadilkar, Satish V. | Patil, Shekhar G. | Kulkarni, Kedar S.

Article Type: Research Article

Abstract: Thirty two patients with subacute sclerosing panencephalitis (SSPE) admitted under the care of Department of Neurology at JJ Hospital and Grant Medical College, Mumbai during the period 1998-2003 were analyzed. All patients were evaluated clinically, with relevant investigations and neuroimaging wherever possible. Particular attention was given to early clinical features. Diagnosis was confirmed by cerebrospinal fluid study for measles antibody and by electroencephalography. The mean age of our patients was 13.4 years and the …vaccinated patients tended to be older. Nine patients had received measles vaccination. Twelve percent of patients were older than the age of 20 years at the onset of symptoms. Approximately 40.6% of patients presented with symptoms of loss of vision, seizures and behavioral change. At this stage myoclonus and cognitive decline were conspicuous by their absence. Eventually typical features like myoclonus and cognitive decline evolved after a mean period of 8 months. Even in the present era, SSPE continues to remain the most important cause of progressive myoclonic epilepsy. With progressive increase in age of presentation, in patients with features like loss of vision, seizures and behavioral changes, SSPE should be carefully considered. (J Pediatr Neurol 2004; 2(2): 73–77). Show more

Keywords: early clinical features, SSPE, vision loss, seizures

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 73-77, 2004

Authors: Kułak, Wojciech | Sobaniec, Wojciech

Article Type: Research Article

Abstract: The aim of this study was to identify antenatal, intrapartum and neonatal risk factors for cerebral palsy (CP) among babies. Antenatal, intrapartum, and neonatal events were compared between 204 children with CP born between 1983 to 2000 and 206 controls, children matched by birth weight, gestational age, and sex via a retrospective case-control method. Antenatal, intrapartum and neonatal factors were expressed as odds ratios and 95% confidence intervals. Multiple logistic regression was conducted, entering only …those variables found to be significant at the bivariate level. Factors associated with an increased risk of CP identified as antenatal and intrapartum risk factors were abruptio placenta, pre-labour rupture of membranes, prematurity, preterm labour, cesarean section and low birth weight (< 2500 gram). Respiratory distress syndrome, prolonged ventilation, septicemia, meningitis, hyperbilirubinemia, neonatal seizures, and severe cranial ultrasound abnormality were associated with an increased risk of CP in the neonatal period. In the logistic regression models prematurity and Apgar scores ≤ 4 at 1st min were significantly associated with an increased risk of CP. Several antenatal, intrapartum and neonatal risk factors for CP among preterm and term babies are responsible for the etiology of CP. Our findings are in agreement with reports from Western countries. (J Pediatr Neurol 2004; 2(2): 79–84). Show more

Keywords: neonatal, risk factors, cerebral palsy

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 79-84, 2004

Authors: Csibra, Gergely | Henty, Julian | Volein, Ágnes | Elwell, Clare | Tucker, Leslie | Meek, Judith | Johnson, Mark H.

Article Type: Research Article

Abstract: We used near infrared spectroscopy to measure changes in cerebral oxygenation in both human infants and adults as they viewed images of faces or control "visual noise" stimuli. At an occipital site, adults showed a significant increase in oxyhaemoglobin and a contrasting pattern of results was observed in infants. While the same general difference between the processing of the two stimuli was observed, a larger decrease in oxyhemoglobin concentration in response to faces than to visual …noise was found in infants. These results demonstrate that near infrared spectroscopy can detect differences in stimulus processing induced by a complex visual stimulus in both infants and adults. (J Pediatr Neurol 2004; 2(2): 85–89). Show more

Keywords: near infrared spectroscopy, face perception, infant brain development

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 85-89, 2004

Authors: Awaad, Yasser | Tayem, Hassan | Munoz, Sharon | Thomas, Munoz | Soliman, Sherif | Michon, Anne Marie | Minarik, Sarah

Article Type: Research Article

Abstract: Cerebral palsy (CP) is a severely debilitating condition. The underlying insult results in irreversible damage to the central nervous system, treatment is essentially palliative. Botulinum toxin type-A (BTX/A) Botox?Allergan, Inc. injections have been found to be effective palliation for dynamic spasticity in CP. There is a paucity of data regarding the maximum dose tolerated in the pediatric population. The present study examines the safety and efficacy of BTX/A therapy at up to 40 U/kg. Twenty-two patients …were treated from 1995 to 1999. The population included four adults and 18 pediatric patients. Patients were in three groups: diplegia (n=12), hemiplegia (n=5), and quadriplegia (n=5). They were evaluated by the Modified Ashworth Scale. The incidence and severity of complications is also reported. The significance of these data is also commented upon. This study suggests that BTX/A therapy is safe, efficacious, and cost effective at higher doses, and should be considered as part of a comprehensive therapeutic regimen in appropriate candidates. (J Pediatr Neurol 2004; 2(2): 91–96). Show more

Keywords: botulinum toxin, cerebral palsy, spasticity, safety and efficacy

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 91-96, 2004

Authors: Lücke, Thomas | Illsinger, Sabine | Hartmann, Hans | Sasse, Michael | Donnerstag, Frank | Schneppenheim, Reinhard | Das, Anibh M.

Article Type: Research Article

Abstract: Congenital ornithine transcarbamylase deficiency (OTCD, OMIM 311250, Xp21.1) in humans results in hyperammonaemia with subsequent neurological symptoms including hypotonia, seizures and mental retardation. At the age of 3 years a hyperkinetic-hyperactive behaviour disorder of unknown origin was diagnosed in our female patient. The girl, ultimately diagnosed as having OTCD presented at the age of 6 years with an episode of fever-associated metabolic stroke including coma, epilepsy and further neurological symptoms due to …hyperammonaemia. The pattern of plasma amino acids, the elevated level of orotic acid in urine and mutation analysis confirmed the diagnosis of OTCD. The possibility of early diagnosis and therapy of a disease which otherwise leads to severe neurological and psychiatric sequelae emphasizes the importance of precise evaluation of a possible organic cause for hyperkinetic-hyperactive behaviour disorders. This case of late-onset OTCD demonstrates that total recovery is possible even after a long coma-episode with slow reconvalescence. (J Pediatr Neurol 2004; 2(2): 97–100). Show more

Keywords: ornithine transcarbamylase deficiency, hyperammonaemia, encephalopathy, glutamine, MR-spectroscopy

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 97-100, 2004

Authors: Kumar, Raj | Singhi, Vinita

Article Type: Research Article

Abstract: Tuberculous brain stem abscess is a highly uncommon entity. Three children with tuberculous brain stem abscesses of 1 month, 7 and 12 years of age were treated by microsurgical evacuation and biopsy of capsule, utilizing safe incisions over dorsal brain stem, through the floor of IV^{th} ventricle. There was no added deficit postoperatively in these children. Microsurgical evacuation and biopsy helped in differentiating these lesions from pyogenic abscesses in two cases for …appropriate treatment. The third operated case was a child, who while being on antituberculosis treatment for proven tuberculous meningitis for 1.5 year developed medullary and pontine abscess and tuberculoma exemplifying a paradoxical response to chemotherapy. Brain stem abscess especially tuberculous need surgical drainage, as it provides an accurate proof of offending organism and also takes care of a paradoxical response to antituberculosis chemotherapy in diagnosed cases. The safe incisions on brain stem are helpful to avoid the morbidity. (J Pediatr Neurol 2004; 2(2): 101–106). Show more

Keywords: brain stem abscess, tuberculosis

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 101-106, 2004

Authors: Tubbs, R. Shane | Oakes, W. Jerry | Wellons, John C.

Article Type: Research Article

Abstract: Midline cysts of the anterior brain are common in infants e.g. cavum septum pellucidum et vergae. We report twin brothers with midline intracranial cysts, mildly increased ventricular size, and macrocephaly. One brother had a cavum septum pellucidum and vergae and the other a cavum septum pellucidum. With the obliteration of each brother's midline cyst the head circumference was noted to return to a normal head circumference curve and the ventricles decreased in size. We hypothesize that …in infancy some cases of infantile macrocephaly are induced by a midline cyst and that as the cyst obliterates that physiologic cerebrospinal fluid pathways are established so that the head circumference returns within normal parameters. This knowledge should be kept in mind by the clinician who may wish to surgically intervene with a midline cyst prior to it demonstrating its natural history. Moreover, these data should especially be entertained in the premature infant. (J Pediatr Neurol 2004; 2(2): 107–110). Show more

Keywords: siblings, midline cysts, brain, cerebrospinal fluid, hydrocephalus

Citation: Journal of Pediatric Neurology, vol. 2, no. 2, pp. 107-110, 2004