Tourette s diso sorder de and oth ot er tic disorders

Transcription

1 Tourette s disorder dsode and other tic disorders

2 Tic disorders The disorder described by Itard, in France first in 1825, with the case of the Marquise de Dompierre George Gilles de la Tourette, in France, described the full disorder in 9 cases, in 1885: a disorder of the nervous system characterized by motor incoordination accompanied by echolalia and coprolalia.

3 Georges Gilles de la Tourette description Emphasis on: Coprolalia Echolalia Multiple tics Noted the hereditary nature of the condition and its association with obsessive symptoms

4 Current understanding of Tourette s disorder Motor tics plus Vocal tics Frequent, changing in nature, changing over time

5 Motor Tics Movements or fragments of actions Carried out automatically Some control over them if conscious Return when not paying attention Individual movements brief duration Can be supressed temporarily

6 Motor tics Blinking Mouth twitching Smacking lips Movements with tongue Mov. Shoulder Abdomen, breathing Twitching trunk Changing postures Touching the head Touching objects Sucking cheecks or tongue Hitting oneself or others Biting self Copropraxia (obscene gestures)

7 Groups of symptoms (Alsobrook and Pauls, 2002) Purely motor and phonic tics (more common in males) Compulsive behaviors. Touching others, touching objects Aggressive behaviors. Kicking, temper tantrums Tapping behaviors, absence of grunting

8 Tic states Baseline state.. t Tics of intermediate t duration and frequency Bursting state. Short duration and high frequency ticks Quiescence state. Longer duration and Quiescence state. Longer duration and milder tics

9 Vocal (phonic) tics Making noises, Vocalizations, humming, coughing, Clearing throat, barking Obscene words, expletives, coprolalia etc. Repeating the last two or three words heard or just said echoing

10 tics There may be premonitory tension or urge before the movement or noise The child may feel tension to suppress them, or to produce them, which is relieved by the tic The child may say the action is voluntary when it is not Can only be suppressed temporarily Tics may disappear during sleep, but they may continue or be partial

11 Other movements. Differential Stereotyped movement disorders (rocking, thumb sucking, head banging, eat.) other dyskinesias, e.g. chorea and choreiform movements Tremor disorders Compulsive movements due to obsessive compulsive disorder (ritualistic)

12 Other entities Transient Motor Tics Tics are only motor Last a few months and disappear Normal persons may have some tics Simple motor tics usually in orbital region, eye blinking

13 Epidemiology Some tics, motor and vocal, fairly common: 1-13% of boys Male to female prevalence 2:1 Highest prevalence between ages 7 and 11 years old Isle of Wight study: tics prevalence of 5.9% in boy sand 2.9 % in girls Of Tourette s disorder per se, about 5 in people or up to 0.6% More frequent in Caucasians and Orientals?

14 Genetic vulnerability Coocurrence in families of Tourette disorders, attention deficit disorder and obsessive compulsive disorderd Concordance in identical twins, about 50 to 90% Higher frequency in first degree relatives (10 to15%)

15 Mode of inheritance? Proposed semidominant i pattern of inheritance, greater penetrance in homozygotes Or multifactorial polygenic inheritance If matrilineal, more complex motor tics and compulsions If patrilineal, more vocal tics

16 Several candidate genes have been excluded Some chromosomal translocations detected in individuals, such as a translocation between chromosomes 7 and 18 and between 3 and 8 a genome scan of a sample of South African Afrikaners with TS found evidence for linkage on chromosomes 2, 8, and 11, other studies with chromosome 17

17 Environmental factors Role of sexual hormones? E.g. testosterone and puberty? Autoimmune condition in some cases? i.e. PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus)

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19 Pathophysiology Disorder of neurochemical regulation Dysfunction of the basal ganglia? E.g. smaller caudate nucleus? Decreased metabolism and blood flow in the putamen and globus pallidus Often deficit in visuomotor skills and in executive functions Importance of central dopaminergic pathways possible role of DRD4*7 allele of the DRD4 dopamine receptor

20 comorbidity Obsessive compulsive disorder. About 40% of adults diagnosed with Tourette have OCD Hyperactivity. 40 to 50% of children with Tourette have ADHD Attention deficit

21 Sleep disturbance Sleep disturbances, including sleep walking, sleep talking, night terrors, nightmares, difficulties falling asleep, and difficulties staying asleep, have been reported frequently Associating with severity of tics?

22 Other difficulties impairments i in visuomotor skills fine motor skills more difficulty with executive functioning (EF) F t t i t l i it th ht t Frontostriatal circuits are thought to subserve performance on EF tasks

23 Clinical course Typically start at around age 5 or 6, one or several simple tics Peak of tics at around age 10 or 11, higher in frequency, severity Tend to decline through adolescence Tics proceed in cephalocaudal direction Usually vocal tics take several years to appear

24 course May interfere with social development Obsessive and compulsive symptoms develop during course After adolescence symptoms tend to improve Later substantial reduction in umber and severity of tics

25 Effects Effects of tics in terms of sical relationships Acceptability Degree of disruption Self esteem issues Learning and other problems associated with complicating symptoms

26 Interventions Assistance to educate about the anture of the condition To the child and the family, school staff etc Treatment depending on severity of tics Behavioral strategies, relaxation techniques, cognitive strategies

27 Pharmacological If necessary Alpha adrenergic medications may be tried first Neuroleptic medications,usually in smaller doses, such as risperdal, sulpiride, haloperidol, aripiprazole pp