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Biomedical subjects

R Kurlan

Publications and source records attributed to R Kurlan.

At least 37 records · Page 2Linked to original sources

Features resembling Tourette's syndrome in developmental stutterers.

Developmental stuttering (DS) may be related to the extrapyramidal motor system and shares many clinical similarities with Tourette's syndrome (TS), which is widely believed to be associated with extrapyramidal dysfunction. Twenty-two stutterers were examined for neuropsychiatric features commonly seen in TS, including tics, obsessive-compulsive behaviors (OCB), and attention deficit disorders. Eleven stutterers displayed motor tics, and symptoms of OCB were observed at rates similar to those seen in persons with TS. Few stutterers demonstrated significant attentional deficits. Findings are consistent with models suggesting extrapyramidal involvement in DS and raise the possibility that DS and TS are pathogenetically related.

Adolescent↗

Tourette's syndrome and 'PANDAS': will the relation bear out? Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection.

Despite strong evidence of the importance of hereditary factors in the etiology of Tourette's syndrome (TS), research findings have consistently pointed to a role of environmental influences. A recent line of research has suggested that tic disorders and associated behavioral disturbances, such as obsessive-compulsive disorder, might develop following streptococcal infection by the process of molecular mimicry, whereby antibodies directed against bacterial antigens cross-react with brain targets. Such investigations have given rise to the notion that there is a spectrum of childhood neurobehavioral disorders (termed pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection [PANDAS]) that arise by postinfectious autoimmune mechanisms. This article reviews research results supporting the concept of PANDAS and discusses their limitations. Well-designed and adequately controlled studies are needed to determine whether there is a true etiologic relation between streptococcal infection and the onset or exacerbation of childhood neuropsychiatric disorders and whether the use of immune-modifying therapies for these conditions is rational.

Autoimmune Diseases↗

Hypothesis III. Tourette syndrome is only one of several causes of a developmental basal ganglia syndrome.

Tourette syndrome (TS) is a hereditary condition characterized by the presence of chronic, multiple motor tics and is commonly associated with obessive-compulsive behavior, attentional and executive dysfunction, and aggressive behavior. A wide range of other genetic and environmental conditions that interfere with normal basal ganglia developmental processes can produce a clinical syndrome that we call the developmental basal ganglia syndrome (DBGS), and which closely resembles TS. Failure to distinguish TS from other causes of DBGS has probably hampered research efforts aimed at clarifying the neurobiological mechanisms, localizing the genetic defect, assessing therapy, and establishing accurate prevalence rates for TS.

Aggression↗

Gilles de la Tourette's syndrome in special education schools: a United Kingdom study.

In order to determine the prevalence of tic disorders in children with severe school problems requiring a residential facility and comparison groups of children in regular day schools, we performed direct clinical examinations for the presence of tics and Gilles de la Tourette's syndrome (GTS) in 20 children from a residential school for emotional and behavioral difficulties (EBD); 25 children from a residential school for learning disabilities; 17 "problem" children (PC) (identified by teachers as having academic or behaviour problems) and 19 normal children (NC) selected at random (using random numbers) from a regular school. Of the EBD students, 65% were judged to have definite tics as compared with 24% of students with learning difficulties (P < 0.05), 6% of PC (P < 0.003) and none of the NC (P < 0.0006) group. Most of the affected students met diagnostic criteria for GTS. Our findings suggest that GTS is commonly associated with the need for special education and that this association is particularly robust for children with severe school problems. In these children, the presence of tics may be an indicator of an underlying dysfunction of neurological development.

Adolescent↗

Tourette syndrome. Treatment of tics.

There are a number of medications that effectively relieve symptoms in patients with functionally disabling tics. Neuroleptic drugs remain the most predictably effective tic-suppressing agents, but other approaches may be beneficial. This article reviews the spectrum of available therapies to suppress tics and provides practical guidelines for their selection and use.

Antipsychotic Agents↗

Future direction of research in Tourette syndrome.

Recent research results have clarified many aspects of Tourette syndrome but they have also raised important questions. These questions, which regard the nature of the condition and its etiology, will guide future directions of research in Tourette syndrome. This article discusses critical, basic, and unanswered questions that require scientific exploration including what Tourette syndrome is and what causes it.

Forecasting↗

Serotonin syndrome and the combined use of deprenyl and an antidepressant in Parkinson's disease. Parkinson Study Group.

The manufacturer of deprenyl (selegeline; Eldepryl) (Somerset Pharmaceuticals, Tampa, FL) recently advised physicians to avoid prescribing the drug in combination with an antidepressant because of potentially serious CNS toxicity that may represent the serotonin syndrome. Manifestations of the serotonin syndrome vary but may include changes in mental status and motor and autonomic function. To better estimate the frequency of the serotonin syndrome in patients with Parkinson's disease (PD) treated with deprenyl and an antidepressant, we surveyed all investigators in the Parkinson Study Group. Based on estimates provided by the 47 investigators (75%) who responded, 4,568 patients were treated with the combination of deprenyl and an antidepressant medication. Eleven patients (0.24%) were reported to have experienced symptoms possibly consistent with the serotonin syndrome. Only two patients (0.04%) experienced symptoms considered to be serious. No deaths were reported. We also reviewed all published case reports and adverse experiences reported to the U.S. Food and Drug Administration and the manufacturer of Eldepryl. Available information indicates that serious adverse experiences resulting from the combined use of deprenyl and an antidepressant medication in patients with PD are quite rare and that the frequency of the true "serotonin syndrome" is even rarer.

Aged↗

A survey of antidepressant drug use in Parkinson's disease. Parkinson Study Group.

Selective serotonin reuptake inhibitors (SSRIs) are a newer class of antidepressants that may have particular efficacy in Parkinson's disease (PD) given the known serotonergic alterations in this disease. These agents are also thought to have a favorable side-effect profile, particularly in the elderly. Several recent case reports, however, have raised concern that SSRIs may worsen parkinsonian motor function. We surveyed 71 Parkinson Study Group (PSG) investigators using a standardized questionnaire about their usage of antidepressants in PD. Based on estimates provided by 49 investigators (70%) (caring for approximately 23,410 PD patients) who responded, 26% of patients with PD are on pharmacotherapy for depression. These physicians use SSRIs as first line therapy 51% of the time, tricyclic antidepressants 41% of the time and other agents 8% of the time. The most common reasons for selecting SSRIs were their better side-effect profile and perceived greater efficacy. The most common reasons for selecting tricyclic antidepressants were their potential to help with sleep and the physician's experience with this class. Forty-three percent of investigators were concerned that SSRIs might worsen motor function, and 37% of them have had at least one patient in whom they believe this had occurred. Our survey confirms that for treating physicians there remain uncertainties regarding the relative efficacy and tolerability of available antidepressant medications for patients with PD. A controlled clinical trial of antidepressant therapy in PD would be valuable for settling these concerns.

Antidepressive Agents↗

Asymmetry of basal ganglia perfusion in Tourette's syndrome shown by technetium-99m-HMPAO SPECT.

UNLABELLED: Our study involved performing brain perfusion SPECT scans on Tourette's subjects to observe any common perfusion abnormalities involving the cerebral cortex or subcortical structures. METHOD: Six patients with Tourette's syndrome and nine normal control subjects underwent a brain SPECT study with 99mTc-HMPAO. Regions of interest were generated over the cerebral cortex, basal ganglia, thalamus and cerebellum to evaluate any relative perfusion abnormalities or asymmetry in the Tourette's subjects. RESULTS: Five of the six Tourette's subjects demonstrated a significant decrease in right basal ganglia activity which was not present in any of the normal control subjects. CONCLUSION: Our study suggests an etiology for Tourette's syndrome involving the right basal ganglia. Furthermore, brain SPECT may be useful in the evaluation of these patients if it proves to be sufficiently sensitive and specific in larger study populations.

Adult↗

School problems in Tourette's syndrome.

BACKGROUND: A retrospective study of 138 children with Tourette's syndrome for associated school problems revealed that at the time of initial evaluation, 64 subjects (46%) experienced a school-related problem. OBJECTIVE: To survey a childhood population with Tourette's syndrome to explore the contributions of neurobehavioral concomitants to academic difficulties. RESULTS: A diagnosis of a specific learning disorder had previously been made in 30 (22%) of 138 children. Among the 108 without a diagnosis of learning disorder, 36 (33%) experienced school difficulties defined as grade retention (16 [15%]) and/or special education placement (41 [38%]). Regression analysis of subjects without a diagnosis of learning disability revealed that the presence of attention-deficit hyperactivity disorder served as a significant predictor of school problems. CONCLUSIONS: Tics represented the primary reason for referral, but did not emerge as a significant predictor of academic problems. Rather, school-related difficulties appeared to be strongly associated with comorbid attention-deficit hyperactivity disorder.

Adolescent↗

Clinical research in neurology. From observation to experimentation.

Clinical research in neurology may be based on observation or intervention. Basic science and observational clinical studies often address questions of cause and pathogenesis, whereas interventional clinical experiments normally are required to establish the safety, tolerability, and efficacy of therapies. This article outlines various study paradigms and discusses the concepts of intervention, randomization, blinding, and control in the context of clinical trial design. Accurate interpretation of trial results is predicated on the care with which experimental therapeutic studies are designed and conducted.

Bias↗

Non-obscene complex socially inappropriate behavior in Tourette's syndrome.

The authors surveyed 87 adolescent or adult patients with Tourette's syndrome (TS) regarding the presence, characteristics, and functional impact of non-obscene socially inappropriate (SI) behavior. Reported behaviors included insulting others (22%), other SI comments (5%), and SI actions (14%). More often, subjects described having an urge to carry out these behaviors (30%, 26%, 22%, respectively), which they often attempted to suppress. Non-obscene SI behavior was usually directed at a family member or familiar person, at home or in a familiar setting. Social difficulties commonly resulted. Non-obscene SI behavior is a common and potentially disabling feature of TS. It is closely associated with conduct disorder and attention deficit disorder and may represent part of a more general dysfunction of impulse control in TS.

Adolescent↗