Tourette s Syndrome 妥 瑞 氏 症 長 庚 兒 童 醫 院 兒 童 心 智 科

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2 Tourette s Syndrome 妥 瑞 氏 症 長 庚 兒 童 醫 院 兒 童 心 智 科

3 歷 史 發 展 1885: 法 國 醫 師 Georges Gilles de la Tourette 首 先 提 出 相 關 病 例 1893: Freud 提 出 Frau Emmy von N 之 病 例 : 情 緒 障 礙 伴 隨 有 身 體 不 自 主 運 動

4 生 物 與 心 理 交 互 作 用 之 產 物 The pioneering work of Drs. Arthur and Elaine Shapiro in 1960s The introduction of Haloperidol in 1970s The development of developmental psychopathology, developmental neuroscience, developmental psychology and child psychiatry Tourette s syndrome becomes our hysteria the model disorder for exploring mind- brain relationships in health and disease

5 甚 麼 是 妥 瑞 氏 症?

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7 甚 麼 是 Tics? 短 暫 的 無 法 控 制 的 正 常 的 動 作 或 聲 音 突 然 的 重 複 的 刻 板 的 動 作 或 聲 音 發 作 時 常 使 用 一 整 批 肌 肉 正 常 行 為 中 的 一 部 份 Tics 因 出 現 的 地 點 數 目 頻 率 長 度 強 度 及 複 雜 性 而 區 分 為 不 同 型 態

8 Motor tics ( 動 作 tics)

9 Simple motor tics( 單 純 動 作 tics) 突 然 的 短 促 的 通 常 少 於 1 秒 年 幼 的 個 案 常 完 全 不 自 知 Face Head Shoulder body Eye blinking, eye movement,grimacing, nose twitching, mouth movement, lip pouting, frowning Head jerks, jaw snap, tooth clicking Shoulder shrug Arm jerk, abdominal tensing, kicks, finger movement, rapid jerking of any part of the body

10 Fractal character of the temperal occurance of tics IIIIIII IIII IIIIII IIIIIII seconds III IIIIII--IIIIII---III IIIIII hours III--III III---III---IIIII days III III--IIIIII---IIIII III weeks III III----IIIII IIIIIIIII IIII----- months

11 Complex motor tics ( 複 雜 動 作 tics)

12 複 雜 動 作 tics 的 例 子 Face Body Sustained look, facial gestures, biting the mouth or lip,kissing, sticking out the tongue, rolling eye upwards or side to side Bending, gyrating, hopping, clapping,dystonic posture, touching objects or self, throwing, banging, thrusting arms, self abusive acts, gestures with hands, writing movements, copropraxia, picking scabs,tearing papers,pulling pencil while writing.

13 Degree of impairment Frequency 頻 率 Forcefulness 用 力 程 度 Complexity 複 雜 性 Duration 時 間 長 度 Impact on self esteem, family life 對 自 身 響 Social acceptance 社 會 接 受 度 School or job functioning physical well being 對 生 活 功 能 影 響

14 聲 音 tics (Phonic tics) 大 多 在 motor tics 發 生 後 才 出 現 好 發 於 8 至 15 歲 之 間 少 於 5 % 的 個 案 只 有 phonic tics 的 症 狀 發 展 方 式 一 般 由 短 暫 的 情 形 衍 生 為 長 時 間 的 單 純 simple phonic tics: sudden,meaningless sounds or noise 複 雜 complex phonic tics: sudden, more meaningful utterance

15 phonic tics 的 例 子 Simple phonic tics Throat clearing, coughing, sniffling, spitting, screeching, barking, grunting, gurgling, clacking, hissing, sucking Complex phonic tics Syllables, words, phrases, statements, speech atypicalities (rhythms, tones, accents, intensity of speech), echo phenomenon,coprolali a

16 Vocal tics 的 各 種 理 論 Failure of the gating of cortico-thalamo-striatocortical circuits? Stimulation of Cingulate cortex, supplementory motor area may also produce vocalization A bias toward sexual contents Determine by phonologic content or semantics? Hearing people use obscenities because of its lingual consonants and phonation? The meaning of the obscenities is the prime determinant?

17 手 語 tics (Sign language tics) A 29 y/o prelingually deaf man with chronic motor tics palm back finger spelled obscene words combined with grabbing of genitals A woman with preexisting TS Learn sing language and develop sign language tics The idea underlying coprolalia is more important than the phonology of the utterance!

18 Premonitory sensory urges 約 在 Tics 開 始 後 三 年 會 產 生 此 種 感 覺 75-90% of TS patients had this urge, 92 % reported that the tics are partly or wholly voluntary in character! 此 種 感 覺 有 時 比 tics 更 亦 使 人 不 舒 服 及 分 心 The existence of premonitory urges is considered an important sign of the involvement of a cortico-striao-thalamocortical circuit on the production and maintenance of tic smptoms

19 premonitory urges 出 現 的 地 點 Shoulder girdle, throat, hands, midline of the stomach, front of the thighs and feet are hot spots Scalp, neck, upper arms abdomens, have the second highest density

20 TS 的 其 他 特 質 依 賴 刺 激 源

21 許 多 行 為 問 題 More behavioral problems beside ADHD and OCD 45% 社 會 及 學 習 問 題 social difficulties and problematic academic achievement 易 罹 患 憂 鬱 症 High rates of depressive concerns TS in child psychiatry settings Wodrich et al 1997, J Am Acad Child Adolesc Psychiatry

22 Cross cultural review

23 中 國 人 病 患 之 臨 床 表 徵 Retrospective study Interview Age of onset: Age of onset of coprolalia: Duration of illness: % of coprolalia : 44% Retrospective study Interview, SCL-90R Age of onset: M:F= 7.6:1 Eye-blinking: most common Age of onset of coprolalia: Duration of treatment: % of coprolalia : 44.1% older patients reported more emotional distress, and younger patients showed more somatization and obsessivecompulsive symptoms

24 診 斷 及 分 類 的 困 難 沒 有 敏 感 及 專 門 的 診 斷 工 具 診 斷 完 全 仰 賴 病 史 及 臨 床 病 徵 診 斷 標 準 完 全 仰 賴 智 慧 人 士 的 準 則 屋

25 TS 過 動 症

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27 SIB in TS Investigations have reported SIB in 33-53% of TS patients 23 types of SIB: head banging(47%, most common), body punching/slapping, head or face punching/slapping, banging or poking sharp objects into body, scratching of parts of body, inflicting severe eye injuries. SIB is related to hostility and obsessionality. Psychosurgery may be indicated Mild TS cases may also have SIB

28 病 因 Aetiological aspects Mixed model Walkup et al, 1996 家 族 研 究 : French Canadian family linkage at 11q23 手 足 研 究 : Sib- pair study: chromosome 4q and 8p Stress diathesis model --Leckman The clinical expression of TS is a product of an inherited vulnerability with environmental factors

29 危 險 因 子 Genetic vulnerability Gestational and perinatal risk factor (25% birth complication in TS) 1. Severe nausea and vomiting during the first trimester 2. Severe psychosocial stress of the mother during pregnancy 3. Maternal use during pregnancy of coffee (more than 2 ups per day), cigarettes(more than 10 a day), or alcohol(fewer than 2 drinks a day) 4. Identical twin with low birth weight 5. Low birth weight children with evidence of parenchymal lesions, ventricular enlargement or both 6. Transient hypoxia or ischemia during birth(labor> 24 h), use of forceps, evidence of fetal distress 7. Low 5min Apgar scores

30 Recurrent streptococcal infections with post-infectious autoimmune response PANDAS(pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection) A clinical spectrum between TS and Sydenham s chorea( a variant of rheumatic fever neurological involvement,ocd symptoms and vocal tics Increase antineuronal antibodies in the sera of both patients

31 Severe psychosocial trauma, recurrent daily stresses or high level of emotional excitement (heightened reactivity of the hypothalamic-pituitary-adrenal axis) Drug abuse 1. Exposure to androgenic drug 2. Chronic intermittent use of cocaine and other psychostimulants Co-existing medical or psychiatric disorders 1. ADHD 2. LD 3. Depression

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