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Abstract. Paroxysmal choreoathetosis is a rare, involuntary movement disorder. Attacks occur spontaneously or may be induced by movement, startle, or anxiety. The movements are tonic, dystonic, or choreoathetotic. Sporadic and, more commonly, familial cases have been reported. Onset occurs most often during childhood, and the course is nonprogressive. Response to anticonvulsant therapy is usually excellent. Five cases of the sporadic form of paroxysmal choreoathetosis are reported. Three of the five patients had attacks after initiation of movements such as rising from a chair. Results of physical examination were normal in four patients. One child had mild hemiatrophy and unilateral hyper-reflexia. Results of laboratory studies, including determinations of serum calcium and ceruloplasmin levels, EEGs, and CAT scans of the head, were normal. The attacks ceased in all patients after treatment with either phenytoin or carbamazepine. [ABSTRACT FROM AUTHOR]

Four cases of lead neuropathy in children with hemoglobin S-S or S-C disease are reported. Neuropathy is a rare manifestation of lead poisoning in children, and only ten other cases have been well documented in the pediatric literature. The last previous case report of lead neuropathy was also in a child with hemoglobin S-S disease. The neuropathy seen in the children with sickle cell disease was clinically similar to that seen in the previously reported cases in nonsicklers, but differed in both groups from that usually seen in adult cases. It is, therefore, postulated that children with sickle cell disease have an increased risk of developing neuropathy with exposure to lead. The exact mechanism for this association remains unknown, but in children with sickle cell disease presenting with symptoms or signs of peripheral weakness, the possibility of lead poisoning must be considered. [ABSTRACT FROM AUTHOR]

Provides information on the role and the pharmacology of medications used to treat children with attention deficit disorders. Potentially useful drugs; Dose levels; Adverse effects.

Discusses the Supplemental Security Income (SSI) program for children that was implemented by Congress in 1974. Financial and resource eligibility criteria; Evaluation process; Role of pediatricians in ensuring that eligible children receive the SSI cash and associated benefits.

Presents letters to the editor on pediatric topics. Validity of prescribing medication for all children with school behavior problems; Prevention of unintentional esophageal intubation; Use of diazepam in neonatal narcotic withdrawal syndrome; Susceptibility of medical students to mumps.

Focuses on the growth of the field of infant and child development. Increase in the knowledge about special services that can improve early development of children; Debate on the factors involved in the developmental outcome of a child; Awareness of the importance of the family in the development of children.

Defines the context in which rehabilitation therapies should be prescribed, emphasizing the identification and enhancement of the child's function and abilities. Therapy prescriptions; Spectrum of motor impairments; Multiple needs of children.

Presents guidelines for screening infants and young children for developmental disabilities. Importance of early identification to effective therapy; Utilization multiple sources of information for screening; Issues in methodology; Essential components of the screening process; Required skills and procedures.

This study compares primary stereotypies (repetitive, self-stimulating, and seemingly nonsensical movements that can occur within typically developing children) and secondary stereotypies (those occurring within autistic or mentally retarded children). Utilizing a retrospective chart review from 1995 to 2010, the current study compares primary and secondary stereotypies by the application of a classification system that organizes the movement by its type (motor only, phonic only, mixed) and complexity. In addition, it investigates other parameters associated with the movements such as duration, frequency, age, functional impairment, and progression. The sample group consisted of 28 primary and 28 secondary cases. Primary stereotypies were predominantly motor, simple, of shorter duration, and of less frequency, whereas secondary stereotypies had more vocalization, complexity, longer durations, and higher frequencies. Moreover, functional impairment due to stereotypies was noted in 3 primary and 7 secondary cases, and worsening of stereotypies was noted in 70% of primary versus 44% of secondary cases. [ABSTRACT FROM PUBLISHER]

Background The objective of this study was to characterize the clinical features, course, and treatment of essential tremor ( ET) in children. Methods A retrospective chart review was conducted over 25 years (1984-2011). Inclusion criteria were age <21 years and satisfying the core diagnostic criteria for ET. Primary exclusion criteria included other neurological findings, tremorogenic medications, sudden onset/stepwise progression, primary orthostatic tremor, and isolated task specificity; and secondary exclusion criteria were abnormal neuroimaging or metabolic/thyroid studies. Cases were reviewed for age, sex, family history, tremor characteristics, functional impairment, treatment, and follow-up. Results In total, 211 children had ET, including 130 males and 81 females. The mean ± standard deviation age was at diagnosis was 14.09 ± 5.0 years, the age of onset was 9.71 ± 5.62 years, and the age of onset was birth in 7 children. One hundred ninety-nine children had bilateral hand tremor, 34 had asymmetry, 9 had unilateral onset but later became bilateral. Twelve children had voice tremors, 13 had leg tremors, 5 had head tremors, and 7 had trunk tremors. Tremor at rest was present in 20 children. Thirty-five percent of the children had a family history of ET, including in a father (n = 21), mother (n = 13), brother (n = 6), sister (n = 3), and other family member (n = 28). Fifty-five percent of patients had functional disabilities, including writing (n = 66), eating (n = 28) drinking from a cup (n = 13), typing (n = 4), playing instruments (n = 6), buttoning (n = 6), and playing (n = 3). For treatment, 33 patients received propranolol, 1 received atenolol, 13 received primidone, 3 received metoprolol, and 1 received nadolol. In total, 99 patients were followed for a mean ± standard deviation of 1.82 ± 2.21 years. If left untreated, tremor remained unchanged in 33 patients, and 7 had an apparent short-term improvement. On propranolol, 15 of 20 patients significantly improved. Conclusions This is the largest series to date of ET in children. The current findings indicate that onset at birth is possible, family history is less common in children than in adults, and tremor at rest is possible. Functional disability was noted in 55% of children, and 29.4% required medication. [ABSTRACT FROM AUTHOR]

Abstract: Background: The objective of this study was to determine the frequency, nature, and impact of sleep disorders in children and adolescents with Tourette syndrome and to raise awareness about their possible inclusion as a Tourette syndrome comorbidity. Methods: Using a prospective questionnaire, we interviewed 123 patients of age ≤21 years with a confirmed diagnosis of Tourette syndrome. Each completed questionnaire was then reviewed in accordance with Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, criteria for categorization to a form of sleep disorder. Results: Of the 123 patients with Tourette syndrome, 75 (61%) had comorbid attention deficit hyperactivity disorder and 48 (39%) had Tourette without attention deficit hyperactivity disorder. The sleep problems observed included problems in the nature of sleep, abnormal behaviors during sleep, and impact of sleep disturbances on quality of life. Within these cohorts, 31 (65%) of the 48 Tourette-only patients and 48 (64%) of the 75 Tourette + attention deficit hyperactivity disorder patients could fit into some form of Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, coded sleep disorders. Of the 48 Tourette + attention deficit hyperactivity disorder patients with sleep disorders, 36 (75%) had insomnia signs, which could be explained by the co-occurrence of attention deficit hyperactivity disorder and high stimulant use. However, 10 (32%) of the 31 Tourette-only patients with sleep disorders had insomnia irrespective of attention deficit hyperactivity disorder or medication use. Conclusions: Sleep problems are common in children with Tourette syndrome irrespective of comorbid attention deficit hyperactivity disorder, justifying their inclusion as a comorbidity of Tourette syndrome. [Copyright &y& Elsevier]

Background: Tourette Syndrome (TS) has a complex etiology and wide variability in phenotypic expression. Identifying underlying symptom patterns may be useful for etiological and outcome studies of TS. Methods: Lifetime tic and related symptom data were collected between 1996 and 2001 in 121 TS subjects from the Central Valley of Costa Rica and 133 TS subjects from the Ashkenazi Jewish (AS) population in the US. Subjects were grouped by tic symptoms using an agglomerative hierarchical cluster analysis. Cluster membership was tested for association with available ancillary information (age of onset, tic severity, comorbid disorders, medication treatment and family history). Results: Cluster analysis identified two distinct groups in each sample, those with predominantly simple tics (cluster 1), and those with multiple complex tics (cluster 2). Membership in cluster 2 was correlated with increased tic severity, global impairment, medication treatment, and presence of comorbid obsessive-compulsive symptoms in both samples, and with family history of tics, lower verbal IQ, earlier age of onset, and comorbid obsessive-compulsive disorder and attention-deficit/hyperactivity disorder in the AS sample. Conclusions: This study provides evidence for consistent and reproducible symptom profiles in two independent TS study samples. These findings have implications for etiological studies of TS. [Copyright &y& Elsevier]

In response to recent publicity regarding the potential use of deep brain stimulation (DBS) for reducing tic severity in Tourette's syndrome (TS), the Tourette Syndrome Association convened a group of TS and DBS experts to develop recommendations to guide the early use and potential clinical trials of DBS for TS and other tic disorders. The goals of these recommendations are to ensure that all surgical candidates are (1) fully informed about the risks, benefits, and alternative treatments available; (2) receive a comprehensive evaluation before surgery to ensure that DBS is clearly the appropriate clinical treatment choice; and (3) that early clinical experience will be documented publicly to facilitate rational decision-making for both clinical care and future clinical trials. © 2006 Movement Disorder Society [ABSTRACT FROM AUTHOR]

Objective: Substantial evidence suggests that both environmental and genetic factors contribute to the development and clinical expression of Tourette's syndrome. Although genetic studies of Tourette's syndrome are common, studies of environmental factors are relatively few and have not identified consistent risk factors across studies. This study examines in a large cohort of subjects (N= 180) the relationship between prenatal/perinatal adverse events with Tourette's syndrome severity as determined by tic severity and rates of commonly comorbid disorders such as obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), and self-injurious behavior. Method: Tic severity, OCD, ADHD, self-injurious behavior, and exposure to a variety of prenatal!perinatal events were systematically assessed in all subjects enrolled in three genetic studies of burette's syndrome. Using linear and logistic regression, a best-fit model was determined for each outcome of interest. Results: Prenatal maternal smoking was strongly correlated with increased tic severity and with the presence of comorbid OCD in these Tourette's syndrome subjects. Other variables, such as paternal age and subject's birth weight, were significantly but less strongly associated with increased symptom severity. The authors found no association between symptom severity and hypoxia, forceps delivery, or hyperemesis during pregnancy, which have been previously identified as risk factors. Conclusions: This study identifies prenatal maternal smoking as a strong risk factor for increased symptom severity in Tourette's syndrome. [ABSTRACT FROM AUTHOR]

Abstract. Psychogenic seizures in 21 nonepileptic children and adolescents, aged 8 to 18 (mean 14.5) years, were recorded by means of video recording and electroencephalography. The episodes included thrashing movements, limb jerking, or staring, with unresponsiveness. Ictal and interictal electroencephalograms showed no abnormalities after antiepileptic medication was discontinued. Fifteen patients had psychogenic seizures spontaneously during recording, and six had seizures in response to suggestion and intravenous saline injection. After the video-electroencephalographic evaluation, patients and families were told that the episodes were emotional in origin. All patients but I agreed to remain without antiepileptic medication, and 16 patients (76%) agreed to begin psychiatric treatment. At 6 to 66 (mean 30) months' follow-up, 14 of 18 (78%) were free of episodes. These data indicate that the majority of young patients with psychogenic seizures have a good outcome. A firm diagnosis is critical so that the episodes can be classified and emphasized as medically not worrisome, permitting a shift away from antiepileptic medication and toward psychiatric treatment. [ABSTRACT FROM AUTHOR]

Provides information on fetal alcohol syndrome and fetal alcohol effects. Physical and mental manifestations in children; Review of studies on the risk of alcohol use during pregnancy; Recommendations of the American Academy of Pediatrics.

Discusses the instability of atlantoaxial joints in persons with Down syndrome. Incidence of atlantoaxial instability among persons with Down syndrome; Sports that require medical examination for persons with Down syndrome during Special Olympics; Causes of the atlantoaxial instability.