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Aim To expand the understanding of stereotypic movement disorder (SMD) and its differentiation from tics and autistic stereotypies. Method Forty-two children (31 males, mean age 6y 3mo, SD 2y 8mo; 11 females, mean age 6y 7mo, SD 1y 9mo) consecutively diagnosed with SMD, without-self-injurious behavior, intellectual disability, sensory impairment, or an autistic spectrum disorder (ASD), were assessed in a neuropsychiatry clinic. A list of probe questions on the nature of the stereotypy was administered to parents (and to children if developmentally ready). Questionnaires administered included the Stereotypy Severity Scale, Short Sensory Profile, Strengths and Difficulties Questionnaire, Repetitive Behavior Scale – Revised, and the Developmental Coordination Disorder Questionnaire. The stereotyped movement patterns were directly observed and in some cases further documented by video recordings made by parents. The probe questions were used again on follow-up at a mean age of 10 years 7 months (SD 4y 4mo). Results Mean age at onset was 17 months. Males exceeded females by 3:1. Family history of a pattern of SMD was reported in 13 and neuropsychiatric comorbidity in 30 (attention-deficit–hyperactivity disorder in 16, tics in 18, and developmental coordination disorder in 16). Obsessive–compulsive disorder occurred in only two. The Short Sensory Profile correlated with comorbidity (p

The aims of this descriptive study were to examine the prevalence and associations of coprophenomena (involuntary expression of socially unacceptable words or gestures) in individuals with Tourette syndrome. Participant data were obtained from the Tourette Syndrome International Database Consortium. A specialized data collection form was completed for each of a subset of 597 consecutive new patients with Tourette syndrome from 15 sites in seven countries. Coprolalia occurred at some point in the lifetime of 19.3% of males and 14.6% of females, and copropraxia in 5.9% of males and 4.9% of females. Coprolalia was three times as frequent as copropraxia, with a mean onset of each at about 11 years, 5 years after the onset of tics. In 11% of those with coprolalia and 12% of those with copropraxia these coprophenomena were one of the initial symptoms of Tourette syndrome. The onsets of tics, coprophenomena, smelling of non-food objects, and spitting were strongly intercorrelated. Early onset of coprophenomena was not associated with its longer persistence. The most robust associations of coprophenomena were with the number of non-tic repetitive behaviors, spitting, and inappropriate sexual behavior. Although coprophenomena are a frequently feared possibility in the course of Tourette syndrome, their emergence occurs in only about one in five referred patients. Because the course and actual impact of coprophenomena are variable, additional prospective research is needed to provide better counseling and prognostic information.

We examined the rates of comorbid pervasive developmental disorders in participants with Tourette syndrome. We used 7288 participants from the Tourette Syndrome International Database Consortium Registry. We found 334 (4.6%; 1 of every 22 participants) with Tourette syndrome had a comorbid pervasive developmental disorder. In participants with Tourette syndrome and comorbid pervasive developmental disorders, 98.8% had one or more comorbidities (pervasive developmental disorder was not counted) compared to 13.2% in the group of participants with Tourette syndrome only. Variables from logistic modeling that were significant predictors of Tourette syndrome and pervasive developmental disorders were: male gender; no family history of tics/Tourette syndrome; and an increased number of comorbidities (P < .001). We found rates of comorbid Tourette syndrome and pervasive developmental disorders to be increased by 13 times. Identification of differences between subgroups of patients with Tourette syndrome may increase understanding of syndromal susceptibility, severity, and outcome.

In this discussion, children are considered to have multiple disabilities and low functioning when they have a combination of severe neurodevelopmental disabilities, such as visual impairment, epilepsy, intellectual deficits, cerebral palsy, attention-deficit–hyperactivity disorder (ADHD), autism, eating difficulties, and other neuropsychiatric problems.

We have established a multisite, international database of 3,500 individuals diagnosed with Tourette syndrome (TS). The male:female ratio is 4.3:1 for the total sample, with wide variation among sites; the male excess occurs at every site. Anger control problems, sleep difficulties, coprolalia, and self-injurious behavior only reach impressive levels in individuals with comorbidity. Anger control problems are strongly correlated with comorbidity, regardless of site, region, or whether assessed by neurologists or psychiatrists. The mean age at onset of tics is 6.4 years. At all ages, about 12% of individuals with TS have no reported comorbidity. The most common reported comorbidity is attention-deficit-hyperactivity disorder. Males are more likely to have comorbid disorders than females. The earlier the age at onset, the greater the likelihood of a positive family history of tics. An understanding of the factors producing these and other variations might assist in better subtyping of TS. Because behavioral problems are associated with comorbidity, their presence should dictate a high index of suspicion of the latter, whose treatment may be at least as important as tic reduction. The established database can be used as the entry point for further research when large samples are studied and generalizability of results is important.

Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non-epileptic, and one had epileptic and non-epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.

Objective: Tourette syndrome (TS) is a common developmental disorder with childhood onset. This study utilizes the TIC consortium data to develop models of variables associated with increased severity of tics in people with TS. The TIC database comprised 5,500 people with TS. Method: This study included the 5,450 participants with clinician reported severity of tics (mild, moderate, or severe). We developed 3 models of tic severity. Results: Model 1 comprised age of diagnosis and total number of comorbidities and correctly classified 71% of mild–moderate cases, 46% of severe, and 68% of the total group. Model 2 comprised 7 of 21 comorbid conditions and classified 78% of mild–moderate cases, 53% of severe cases, and 74% of the total population. Model 3 comprised 6 comorbid conditions and correctly classified 75% of mild–moderate cases, 57% of severe cases, and 72% of the total cohort. We found the attention-deficit hyperactivity disorder was not associated with tic severity but obsessive compulsive disorder was an important variable associated with tic severity. Conclusion: This large dataset provides 3 models of variables associated with increased tic severity at the time of diagnosis. Further research is needed to identify factors associated with longitudinal changes in tic severity. This should include factors that are associated with both increased and decreased severity.

This is the first study to examine effective doses of controlled-release (CR) melatonin in children with chronic sleep wake cycle disorders. All 42 subjects had severe neurodevelopmental difficulties. Initially, a randomized double-blinded cross-over design was used in 16 children, comparing the effectiveness of fast-release (FR) and CR melatonin. In the remainder of the patients, the CR melatonin was studied on a clinical basis. The effectiveness of treatment was assessed by sleep charts and clinical follow-up. Emphasis was placed on the judgement of the parents, who had guidance from the physicians. The average final CR melatonin dose in the 42 patients was 5.7 mg (2-12 mg). The studies showed that the FR melatonin was most effective when there was only delayed sleep onset, but CR formulations were more useful for sleep maintenance. Children appeared to require higher doses than adults.

Lerner, a dermatologist who isolated melatonin (MLT) in 1959, was the first to report on its hypnotic properties, and was also the first to self-administer this hormone. Later, in 1971, Anton-Tay and coworkers injected MLT into volunteers who quickly fell asleep. In adults, MLT treatment appears to have beneficial effects in those who have sleep disorders due to delayed sleep onset, shift work, and jet lag. MLT can enhance adaptation to forced phase shift in simulated shift-work and jet-lag conditions. However, better controlled, long-term studies are still required. MLT may also benefit certain types of adult insomnia.

Until the late 1700s, the eyes were thought to be the organs of sight while the visual function of the brain remained unclear. Just over a century ago a German professor of physiology, Hermann Munk, argued against scientific community scepticism that because blindness could be caused by damage to the occipital lobes, these anatomical areas must have an important function in vision1. During the Russo–Japanese war of 1904–5, Tatsuji Inouye, a Japanese physician and pioneer vision researcher, began to correlate the visual difficulties of soldiers with their head wounds, thereby increasing our understanding of the role of the brain in vision.

One cannot read the burgeoning literature on TS and other neuropsychiatric disorders without being aware that comorbidity as a concept has been receiving increasing interest, 6,47,61,62 and rightly so. Nevertheless, although there is new respect for this concept, there is also considerable confusion over its theoretical and practical implications, among both professionals and the general public. Perhaps nowhere else is this more obvious than with the combination of TS and attention deficit hyperactivity disorder (ADHD). This is exemplified by the well-known observation that in less than 20 years we have gone from a fear of causing or permanently exacerbating TS by the use of stimulant drugs, to their increasing usage. Because the literature is so extensive, this article focuses on only two major issues: First, does the undoubtedly common co-occurrence of the two conditions in our clinics and practices really indicate comorbidity? If it does, then there are important practical consequences for counseling patients and families and for teaching our students, as well as for the study of categorical boundaries. If it does not, then there are quite different consequences. This author chooses to refer to "co-occurrence" rather than "comorbidity" in most instances, because it is my opinion that the question is still open. Second to be considered is the present state of knowledge of how to manage the co-occurrence of TS and ADHD (regardless of what may presently be concluded about the first question), using a selective review of the literature.

OBJECTIVE Attention-deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD) are the 2 most frequent psychiatric disorders co-occurring with Tourette syndrome (TS). Both usually cause greater impairment in psychosocial functioning than the tics themselves. In a previous study, we examined the relationship of attention-deficit/hyperactivity disorder to other comorbid conditions in TS. The relationship of OCD to other comorbidities in TS still remains unclear and is the focus of the present study. METHODS Pearson's χ tests and logistic regressions (year-wise) were used to examine a cross-sectional sample of children and adolescents (n = 5060) diagnosed with TS, taken from the Tourette Syndrome International Database Consortium. We explored the relationship of OCD to other comorbid conditions in TS across different age groups. RESULTS In children (ages 5-10 y) with TS, higher rates of comorbidities were found in the presence compared with the absence of OCD. Adolescents (ages 11-17 y) with TS + OCD showed higher rates of internalizing (i.e., anxiety and mood) disorders when compared with those with TS - OCD. A year-wise increase of coexisting mood disorders was found for subjects with TS with and without OCD. CONCLUSIONS Overall, children and adolescents with TS + OCD showed higher rates of comorbid disorders compared with those with TS - OCD. This underlines the necessity for a comprehensive assessment of additional comorbidities even if the 2 disorders (TS + OCD) have already been diagnosed.

Attitudes of children toward a nonspeaking child using three different augmentative and alternative communication (AAC) techniques were explored. Fourth grade children viewed videotapes showing a child communicating using either an aided electronic technique, an aided nonelectronic technique, or an unaided technique. Attitudes were measured using the Chedoke-McMaster Attitudes toward Children with Handicaps (CATCH) scale, and information was collected on the children's experience and exposure to disabled individuals and their reading comprehension level. Results indicated no difference in attitudes between the groups viewing the three different AAC techniques. More positive attitudes were exhibited by females, better readers, and children who reported experience with disabled children. Results are interpreted in light of information on development of attitudes.

To expand the understanding of stereotypic movement disorder (SMD) and its differentiation from tics and autistic stereotypies.Forty-two children (31 males, mean age 6y 3mo, SD 2y 8mo; 11 females, mean age 6y 7mo, SD 1y 9mo) consecutively diagnosed with SMD, without-self-injurious behavior, intellectual disability, sensory impairment, or an autistic spectrum disorder (ASD), were assessed in a neuropsychiatry clinic. A list of probe questions on the nature of the stereotypy was administered to parents (and to children if developmentally ready). Questionnaires administered included the Stereotypy Severity Scale, Short Sensory Profile, Strengths and Difficulties Questionnaire, Repetitive Behavior Scale--Revised, and the Developmental Coordination Disorder Questionnaire. The stereotyped movement patterns were directly observed and in some cases further documented by video recordings made by parents. The probe questions were used again on follow-up at a mean age of 10 years 7 months (SD 4y 4mo).Mean age at onset was 17 months. Males exceeded females by 3:1. Family history of a pattern of SMD was reported in 13 and neuropsychiatric comorbidity in 30 (attention-deficit-hyperactivity disorder in 16, tics in 18, and developmental coordination disorder in 16). Obsessive-compulsive disorder occurred in only two. The Short Sensory Profile correlated with comorbidity (p0.001), the Stereotypy Severity Scale (p=0.009), and the Repetitive Behavior Scale (p0.001); the last correlated with the Stereotypy Severity Scale (p=0.001). Children (but not their parents) liked their movements, which were usually associated with excitement or imaginative play. Mean length of follow-up was 4 years 8 months (SD 2y 10mo). Of the 39 children followed for longer than 6 months, the behavior stopped or was gradually shaped so as to occur primarily privately in 25. Misdiagnosis was common: 26 were initially referred as tics, 10 as ASD, five as compulsions, and one as epilepsy. Co-occurring facial grimacing in 15 children and vocalization in 22 contributed to diagnostic confusion.SMD occurs in children without ASD or intellectual disability. The generally favorable clinical course is largely due to a gradual increase in private expression of the movements. Severity of the stereotypy is associated with sensory differences and psychopathology. Differentiation of SMD from tics and ASD is important to avoid misdiagnosis and unnecessary treatment.

Short-term sleep loss is known to cause temporary difficulties in cognition, behaviour and health but the effects of persistent sleep deprivation on brain development have received little or no attention. Yet, severe sleep disorders that last for years are common in children especially when they have neurodevelopmental disabilities. There is increasing evidence that chronic sleep loss can lead to neuronal and cognitive loss in children although this is generally unrecognized by the medical profession and the public. Without the restorative functions of sleep due to total sleep deprivation, death is inevitable within a few weeks. Chronic sleep disturbances at any age deprive children of healthy environmental exposure which is a prerequisite for cognitive growth more so during critical developmental periods. Sleep loss adversely effects pineal melatonin production which causes disturbance of circadian physiology of cells, organs, neurochemicals, neuroprotective and other metabolic functions. Through various mechanisms sleep loss causes widespread deterioration of neuronal functions, memory and learning, gene expression, neurogenesis and numerous other changes which cause decline in cognition, behaviour and health. When these changes are long-standing, excessive cellular stress develops which may result in widespread neuronal loss. In this review, for the first time, recent research advances obtained from various fields of sleep medicine are integrated in order to show that untreated chronic sleep disorders may lead to impaired brain development, neuronal damage and permanent loss of developmental potentials. Further research is urgently needed because these findings have major implications for the treatment of sleep disorders.

Sleep disturbances in children with neurodevelopmental disabilities are common and have a profound effect on the quality of life of the child, as well as the entire family. Although interventions for sleep problems in these children often involve a combination of behavioral and pharmacologic strategies, the first line of treatment is the promotion of improved sleep habits or “hygiene.” Despite the importance of sleep-hygiene principles, defined as basic optimal environmental, scheduling, sleep-practice, and physiologic sleep-promoting factors, clinicians often lack appropriate knowledge and skills to implement them. In addition, sleep-hygiene practices may need to be modified and adapted for this population of children and are often more challenging to implement compared with their healthy counterparts. This first comprehensive, multidisciplinary review of sleep hygiene for children with disabilities presents the rationale for incorporating these measures in their treatment, outlines both general and specific sleep-promotion practices, and discusses problem-solving strategies for implementing them in a variety of clinical practice settings.

The purpose of this study was to determine the efficacy of controlled-release (CR) melatonin in the treatment of delayed sleep phase syndrome and impaired sleep maintenance of children with neurodevelopmental disabilities including autistic spectrum disorders. A randomized double-blind, placebo-controlled crossover trial of CR melatonin (5 mg) followed by a 3-month open-label study was conducted during which the dose was gradually increased until the therapy showed optimal beneficial effects. Sleep characteristics were measured by caregiver who completed somnologs and wrist actigraphs. Clinician rating of severity of the sleep disorder and improvement from baseline, along with caregiver ratings of global functioning and family stress were also obtained. Fifty-one children (age range 2-18 years) who did not respond to sleep hygiene intervention were enrolled. Fifty patients completed the crossover trial and 47 completed the open-label phase. Recordings of total night-time sleep and sleep latency showed significant improvement of approximately 30 min. Similarly, significant improvement was observed in clinician and parent ratings. There was additional improvement in the open-label somnolog measures of sleep efficiency and the longest sleep episode in the open-label phase. Overall, the therapy improved the sleep of 47 children and was effective in reducing family stress. Children with neurodevelopmental disabilities, who had treatment resistant chronic delayed sleep phase syndrome and impaired sleep maintenance, showed improvement in melatonin therapy.

To date, there have been no prospective long-term studies of melatonin therapy in children. We report here data from a prospective follow-up study of 44 children with neurodevelopmental disabilities and treatment-resistant circadian rhythm sleep disorders (CRSD) who had participated in a placebo controlled, double blind cross-over trial of sustained-release melatonin. The follow-up study involved a structured telephone interview of caregivers every 3 months for upto 3.8 yr. The caregivers provided ratings of satisfaction, adverse effects, benefits, persistence with treatment and additional medications. Changes in melatonin dose were recorded. Open ended questions were included to capture caregivers' impressions and comments concerning melatonin therapy. Adverse reaction to melatonin therapy and development of tolerance were not evident. Better sleep was associated with reported improvement in health, behavior and learning. At the end of the study, the parental comments regarding the effectiveness of long-term melatonin therapy were highly positive. Parents whose children had sleep maintenance difficulties expressed a wish to have a commercially available controlled-release melatonin product which would promote sleep for 8-10 hr. Hypnotics for children with CRSD should be considered a second line of treatment for those who fail to respond to sleep hygiene and/or melatonin.

Pineal melatonin regulates circadian rhythms and influences sleep. Melatonin also has protective actions against tissue damage from free-radicals and other toxins. Evidence is presented that this indoleamine is involved in pre- and postnatal brain (and ocular) development and intrauterine growth. In the absence of maternal melatonin, short gestation infants have a prolonged period of melatonin deficiency. Melatonin supplementation, which has a benign safety profile, may help reduce complications in the neonatal period that are associated with short gestation. We believe that this treatment might result in a wide range of health benefits, improved quality of life and reduced healthcare costs.

To evaluate the efficacy of sleep hygiene and melatonin treatment for initial insomnia in children with attention-deficit/hyperactivity disorder (ADHD).Twenty-seven stimulant-treated children (6-14 years of age) with ADHD and initial insomnia (60 minutes) received sleep hygiene intervention. Nonresponders were randomized to a 30-day double-blind, placebo-controlled, crossover trial of 5-mg pharmaceutical-grade melatonin provided by the study's sponsor.Sleep hygiene reduced initial insomnia to60 minutes in 5 cases, with an overall effect size in the group as a whole of 0.67. Analysis of the trial data able to be evaluated showed a significant reduction in initial insomnia of 16 minutes with melatonin relative to placebo, with an effect size of 0.6. Adverse events were generally mild and not different from those recorded with placebo treatment. The effect size of the combined sleep hygiene and melatonin intervention from baseline to 90 days' posttrial was 1.7, with a mean decrease in initial insomnia of 60 minutes. Improved sleep had no demonstrable effect on ADHD symptoms.Combined sleep hygiene and melatonin was a safe and effective treatment for initial insomnia in children with ADHD taking stimulant medication.

Background Tourette Syndrome (TS) is a neurodevelopmental disorder of childhood. Learning disabilities are frequently comorbid with TS. Using the largest sample of TS patients ever reported, we sought to identify differences between subjects with TS only and subjects with TS and a comorbid learning disability. Methods We used the Tourette Syndrome International Consortium database (TIC) to compare subjects with comorbid Tourette Syndrome and learning disabilities (TS + LD) to subjects who did not have a comorbid learning disability (TS - LD). The TIC database contained 5,500 subjects. We had usable data on 5,450 subjects. Results We found 1,235 subjects with TS + LD. Significant differences between the TS + LD group and the TS - LD group were found for gender (.001), age onset (.030), age first seen (.001), age at diagnosis (.001), prenatal problems (.001), sibling or other family member with tics (.024), two or more affected family members (.009), and severe tics (.046). We used logistic modeling to identify the optimal prediction model of group membership. This resulted in a five variable model with the epidemiologic performance characteristics of accuracy 65.2% (model correctly classified 4,406 of 5,450 subjects), sensitivity 66.1%, and specificity 62.2%. Conclusion Subjects with TS have high prevalence rates of comorbid learning disabilities. We identified phenotype differences between the TS - LD group compared to TS + LD group. In the evaluation of subjects with TS, the presence of a learning disability should always be a consideration. ADHD may be an important comorbid condition in the diagnosis of LD or may also be a potential confounder. Further research on etiology, course and response to intervention for subjects with TS only and TS with learning disabilities is needed.

We sought to determine whether Tourette syndrome (TS) was related to attentional disturbance independent of Attention Deficit Hyperactivity disorder (ADHD) and obsessive-compulsive symptoms (OCS), two comorbid conditions associated with attentional problems in other populations. Participants were 74 children [21 with uncomplicated TS (TS-Only), 14 with TS and ADHD (TS-ADHD), 21 with ADHD, and 18 controls]. Impaired sustained attention and impulsivity on a Continuous Performance Task were uncommon in the TS-Only group, but common in TS-ADHD and ADHD. The TS-ADHD group was less impulsive than the ADHD group, but had a higher rate of whole-body tics than the TS-Only group, raising questions about the diagnosis of ADHD in TS. OCS was not an independent risk factor for attention problems. However, the association of tic severity and impulsivity was consistent with the theory that TS involves dysfunction in cortical-subcortical circuits mediating behavioral inhibition.

Dear Sir, During the last 16 years of research of melatonin (MT) therapy for pediatric sleep disorders, we have observed that the therapeutic dose is highly variable. The experiences of many other ...

A teenage male is described, in whom Tourette Syndrome was suspected, which was later replaced by attacks of paroxysmal kinesigenic choreo-athetosis. He also exhibited bizarre, episodic perceptual distortions of his visual environment and manifestations of an obsessive-compulsive disorder. Carbamazepine treatment not only completely eliminated the recurring attacks of his choreo-athetosis but also, contrary to expectations, the visual disturbances and even the symptoms of his obsessive-compulsive disorder. Obsessive compulsive disorder should be searched for by direct questioning in all patients with basal ganglia disorders.

A debilitating, regularly recurring, biphasic disorder is described in 6 severely multidisabled children. It was characterized by several days of lethargy, withdrawal, loss of abilities, irritability, and hypersomnolence followed or preceded by a high-energy state for several days during which the children slept very little, at times were euphoric, had improved mental ability, and were hyperactive. These cyclic episodes had been present for years but unexpectedly disappeared in one child. The etiology is unknown, in spite of detailed neurologic, metabolic, and endocrine investigations. All patients had family histories positive for affective disorder. Melatonin treatment helped to regulate the coexisting chronic sleep disorders of 3 children but failed to eliminate the cycles. Antiepileptic drug treatment, lithium, sedatives, stimulants, tranquilizers, and light therapy were largely ineffective. The children's symptoms and signs fit the diagnostic criteria of a bipolar affective illness, as it was modified for patients with associated neurologic disability; therefore, the patients appeared to have a unique disorder that closely resembles or is a variant of rapid cycling affective disorder.

Psychosocial problems in adolescents with epilepsy have been of concern for many years, but have been difficult to assess. This article presents the multicenter development of the Adolescent Psychosocial Seizure Inventory (APSI), an empirically based self-report test patterned after the Washington Psychosocial Seizure Inventory, which is used to evaluate psychosocial problems in adults. After pilot work, 120 adolescents with epilepsy from five centers in North America took the APSI and were interviewed by professionals with respect to adequacy of adjustment in eight psychosocial areas. At least one parent or guardian was also interviewed. Interrater reliability of professional ratings in each area was established. Using an item-by-item, empirically based technique, eight psychosocial scales were developed as well as three validity scales. Reliability of the scales was established by both internal consistency and test-retest procedures. Results for each adolescent are presented in profile form. These results give a visual display of the types and extent of problems that likely would be identified in a detailed professional assessment. It is anticipated that the APSI will be of value in a variety of treatment and research contexts.

The rationale underlying megavitamin therapy for remediating children's learning disorders is discussed. It is concluded that the theoretical arguments put forward for such treatment are unconvincing, based on questionable logic, and wholly unsupported by experimental findings. The need is underscored for an internally valid theoretical foundation and for controlled field studies of such therapies prior to their large-scale clinical use.