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Child Neurologist (child + neurologist)

Distribution by Scientific Domains

Medical Sciences	83%

Selected Abstracts

Clinical characteristics of language regression in children

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 8 2003
Sy Wilson MD;
The spectrum of language regression in childhood is incompletely understood. To describe the features of this disorder more fully, we reviewed the records of 196 consecutive children (143 males and 53 females) with language regression or perceived plateau evaluated between 1988 and 1994 by a child neurologist. Mean age at regression was 21.2 months and the mean interval to referral was 34.8 months. A trigger for the regression was identified in 74 of the children (38%) and was associated with a more rapid regression. Mean age at follow-up was 64 months (SD 55). Seventy per cent of the children became nonverbal, and 75% were cognitively impaired. Language regression was associated with a more global autistic regression in 93% of children. There was a history of seizures in 15% of the children. Some recovery occurred in 61% but only one child recovered fully. Improvement was more likely in the 49% who were entirely developmentally normal before the regression. We conclude that language regression in childhood is a serious disorder with significant long-term morbidity. [source]

Usefulness of the Head-Upright Tilt Test for Distinguishing Syncope and Epilepsy in Children

EPILEPSIA, Issue 6 2001
J. Eirís-Puñal
Summary: ,Purpose: Episodic loss of consciousness in children, whether or not associated with hypertonia or short-duration clonic movements, presents a diagnostic challenge to the pediatrician and child neurologist. We provide some evidence of the usefulness of the head-upright tilt test for investigating the causes of transient loss of consciousness in children, and for distinguishing between syncope, convulsive syncope, and epilepsy. Methods: We studied nine children previously diagnosed as epileptic on the basis of compatible clinical events and epileptiform findings in routine EEGs who were treated over the long term with antiepileptic drugs, but whose clinical records suggested syncope or convulsive syncope rather than epilepsy on reevaluation. All subjects underwent head-upright tilt testing. Results: The tilt-test result was positive in all nine cases, with the patients reporting the same symptoms as in the previously considered epileptic attacks. Conclusions: Inadequate histories and misuse/overinterpretation of EEG results often lead to misdiagnosis of epilepsy in children. The head-upright tilt test is a useful and reliable diagnostic technique, allowing syncopal events to be induced and evaluated under controlled conditions. In a subset of patients, it may help to distinguish epilepsy from simple or convulsive syncope. [source]

Cortical Dysplasias and Epilepsy: Multi-Institutional Survey in Japan

EPILEPSIA, Issue 2000
Morimi Shimada
Purpose: Cortical dysplasia (CD) is a major brain malformation causing intractable epilcpsy. Neurosurgery now succcssfully controls some intractable epilepsies associated with CD. In this study, thc incidence 11 epilepsy and thc frequency of seizurcs were analyzed in different types 01 CD. Methods: This study, supported by a rcse;lrch grant from the Ministry of Health and Wellare of Japan, is part of a research project on the clinical presentation and pathogcnesis of brain dysgenesis. Questionnaires regarding the type of CD, family and pact historics, clinical signs and symptoms and their severity were distributed to I200 institutions comprising child neurologists or pediatricians. CDs werc classified into following 6 types; lissencephaly (agyria-pachygyria spcctrum), cobblestone lissencephaly, polymicrogyria including schizencephaly and hilateral perisylvian syndrome, diffuse heterotopia, focal heterotopia, and hemiinegalencephaly. All patients who had been diagnoscd as CD either by MRI, CT, autopsy or histological cxamination at or after surgical treatment wcre included. Diagnosis of CD by CT or MRI was mainly made by a radiologist, child neurologist, or pediatrician. Double classification was corrected. Epilepsy was classified according to criteria of the ILEA. Seizure frcquencies wcre recorded. Results: A total or 676 cases from 328 institutions was availablc, and distributed as follows: 277 of lissencephaly, I48 of cobblestone lissencephaly (10 cases of Walkcr-Warburg syndrome and 138 Fukuyaina type congcnital muscular dystrophy), I30 of polymicrogyria, 40 of diffuse heterotopia (24 subcortical band hetcrotopia and I6 perivcntricular nodular hcterotopia), 37 of focal heterotopia, and 44 of hemimegalencephaly. In 130 cases of polymicrogyria, 13 cases of bilatcral perisylvian syndrome, and 38 cases of schizencephaly were includcd. Of 667 cases available for study, 500 (75.0%) had epilepsy in which generalized epilepsy including West and Lcnnox syndromes comprised 54.1 % and localization-related epilepsies comprised 46.7%. Thc frequency of seizures could be ascertained in 455 cases, of which 36.0% had daily seizures, and I I .4%) had more than onc seizure per week. The incidencc of epilepsy in cach type of CD was as follows: 86% inlissencephaly, 50% in cobblestone lissencephaly (patients with WalkcrWarburg syndrome had epilepsy in 90%, whercas those with Fukuyama type congenital muscular dystrophy had epilepsy in 46.7%), 71.3% in polymicrogyria, 77.5% in diffusc hetcrotopia (9 1.7% in subcortical band heterotopia and 56.2% in periventricular nodular heterotopia), 74.3% in focal heterotopia, and 93.2% in hemimegalcncephaly. Conclusion: As recent investigations have reported, this study confirmed the high incidence of intractable cpilepsy in CDs. Epilepsy was more prevalcnt in cases with subcortical heterotopia than i n cascs with periventricular nodular helcrotopia. Thc incidcnce or epilepsy was also higher in the focal hcterotopias located subcortically than those dccper in white matter or in the periventricular region. Thcse differences in incidence of epilepsy depending on the location of hcterotopia may give somc clues to the nature of epileptogenesis in CD. [source]

Certification in neuromuscular medicine: A new neurologic subspecialty,,

MUSCLE AND NERVE, Issue 4 2007
Michael J. Aminoff MD
Information is provided concerning the new subspecialty certificate in neuromuscular medicine of the American Board of Psychiatry and Neurology and the eligibility requirements for such certification of practicing neurologists and child neurologists. The Accreditation Council for Graduate Medical Education has approved fellowship training in the subspecialty, and it is likely that residents who wish to pursue a career in neuromuscular medicine will select this training option. Muscle Nerve 35: 409,410, 2007 [source]

Dyskinesias and associated psychiatric disorders following streptococcal infections

CHILD: CARE, HEALTH AND DEVELOPMENT, Issue 6 2004
Richard Reading
Dyskinesias and associated psychiatric disorders following streptococcal infections . DaleRC, HeymanI, SurteesRAH, ChurchAJ, GiovannoniG, GoodmanR & NevilleBGR . ( 2004 ) Archives of Disease in Childhood , 89 , 604 , 610 . Background The classical extrapyramidal movement disorder following , haemolytic streptococcus (BHS) infection is Sydenham's chorea (SC). Recently, other post-streptococcal movement disorders have been described, including motor tics and dystonia. Associated emotional and behavioural alteration is characteristic. Aims To describe experience of post-streptococcal dyskinesias and associated comorbid psychiatric features presenting to a tertiary referral centre 1999,2002. Methods In all patients, dyskinetic movement disorders followed BHS pharyngeal infection. BHS infection was defined by pharyngeal culture of the organism, or paired streptococcal serology. Movement disorders were classified according to international criteria, and validated by experienced child neurologists. Psychiatric complications were defined using ICD-10 criteria using a validated psychiatric interview. Results In the 40 patients, the following dyskinetic movement disorders were present: chorea (n = 20), motor tics (n = 16), dystonia (n = 5), tremor (n = 3), stereotypies (n = 2), opsoclonus (n = 2) and myoclonus (n = 1). Sixty-five per cent of, the, chorea, patients, were, female,, whereas, 69% of the tic patients were male. ICD-10 psychiatric diagnoses were made in 62.5%. Using the same psychiatric instrument, only 8.9% of UK children would be expected to have an ICD-10 psychiatric diagnosis. Emotional disorders occurred in 47.5%, including obsessive-compulsive disorder (27.5%), generalized anxiety (25%) and depressive episode (17.5%). Additional psychiatric morbidity included conduct disorders (27.5%) and hyperkinetic disorders (15%). Psychiatric, movement and post-streptococcal autoimmune disorders were commonly observed in family members. At a mean follow-up of 2.7 years, 72.5% had continuing movement and psychiatric disorders. Conclusion Post-streptococcal dyskinesias occur with significant and disabling psychiatric comorbidity and are potential autoimmune models of common ,idiopathic' movement and psychiatric disorders in children. Multiple factors may be involved in disease expression including genetic predisposition, developmental status and the patient's sex. [source]