370.4 📋 章末速蚘 Summary

370.4.1 🔑 䞀句話瞜結

Movement disorders = involuntary movements (hyperkinetic) or paucity of movement (hypokinetic — Ch379 Parkinson); hyperkinetic categories — tremor (rhythmic oscillation), dystonia (sustained contraction → twisting posture), chorea (irregular flowing), athetosis (slow writhing), ballism (high-amplitude flinging), myoclonus (sudden jerks), tics (stereotyped suppressible with urge), stereotypies (repetitive purposeless), akathisia (inner restlessness), RLS; tremor types — rest (Parkinson pill-rolling 4-6 Hz, asymmetric onset), postural (essential tremor most common adult movement disorder, bilateral, alcohol-responsive, AD often, propranolol + primidone + DBS Vim + FUS thalamotomy FDA 2016), kinetic/intention (cerebellar), task-specific; dystonia focal vs segmental vs generalized + primary vs secondary vs heredodegenerative; common focal dystonia — cervical/torticollis + blepharospasm + writer’s cramp + spasmodic dysphonia → botulinum toxin first-line; dopa-responsive dystonia (DRD, Segawa) childhood + diurnal + dramatic response to small dose levodopa (always trial in early-onset!) + GCH1 mutation; DYT1 generalized AD Ashkenazi; chorea — Huntington disease (AD, CAG ≥ 40 in HTT, anticipation, onset 30-50s, chorea + cognitive + psychiatric triad, caudate atrophy “box-car ventricles”, no disease-modifying therapy, symptomatic VMAT-2 tetrabenazine/deutetrabenazine/valbenazine) + Sydenham chorea post-strep (major Jones criterion rheumatic fever, children, self-limited) + chorea gravidarum + drug-induced (levodopa, antipsychotics tardive, stimulants, phenytoin, OCPs) + lupus/APLS + stroke (basal ganglia) + Wilson; ballism (hemiballism — STN stroke) dopamine antagonists/tetrabenazine; myoclonus cortical (action-induced + epilepsy) + subcortical + spinal + negative myoclonus asterixis (hepatic encephalopathy) + post-anoxic Lance-Adams + JME + CJD + SSPE + MERRF mitochondrial → valproate + levetiracetam + clonazepam + piracetam; tics + Tourette syndrome (childhood onset 5-7 yo, motor + vocal tics > 1 year, comorbid OCD/ADHD) → CBIT (Comprehensive Behavioral Intervention for Tics) + α2 agonists clonidine/guanfacine + antipsychotics aripiprazole/haloperidol/risperidone + VMAT-2; restless legs syndrome (Willis-Ekbom) — urge to move legs + worse rest + relieved movement + worse evening; iron deficiency most common secondary (check ferritin < 75 supplement) + pregnancy + ESRD + neuropathy + medications; α2ÎŽ ligands first-line now (gabapentin, pregabalin, gabapentin enacarbil) over dopamine agonists (augmentation concern); akathisia drug-induced (antipsychotics) → propranolol + benzodiazepines + mirtazapine; tardive syndromes after chronic antipsychotic — tardive dyskinesia oro-facial-lingual + tardive dystonia + tardive akathisia → VMAT-2 inhibitors valbenazine Ingrezza FDA 2017 + deutetrabenazine Austedo FDA 2017 + switch to clozapine/quetiapine。

370.4.2 💊 治療粟芁

  • essential tremorpropranolol 60-320 mg/d + primidone 50-750 mg/d first-line; topiramate + gabapentin + clonazepam; botulinum toxin head tremor; refractory DBS Vim thalamic nucleus or focused ultrasound (FUS) thalamotomy (non-invasive, FDA 2016)
  • focal dystoniabotulinum toxin (BTX-A) every 3-4 months first-line for cervical/blepharospasm/writer’s cramp/spasmodic dysphonia; trihexyphenidyl anticholinergic; baclofen; benzodiazepines
  • dopa-responsive dystonialevodopa-carbidopa 100/25 BID-TID (dramatic response — always trial in early-onset dystonia)
  • generalized dystoniaDBS GPi (globus pallidus internus) for refractory; intrathecal baclofen; selective denervation
  • Huntington choreatetrabenazine (Xenazine) + deutetrabenazine (Austedo) + valbenazine (Ingrezza) VMAT-2 inhibitors — depression BBW + parkinsonism + akathisia + QT side effects; antipsychotics alternative
  • Sydenham choreapenicillin (treat strep) + supportive (self-limited); valproate or carbamazepine if severe
  • tardive dyskinesiavalbenazine (Ingrezza) 40-80 mg/d or deutetrabenazine (Austedo) 12-48 mg/d FDA 2017; reduce/discontinue offending antipsychotic if possible; switch to clozapine or quetiapine (lower TD risk)
  • Tourette syndromeCBIT (Comprehensive Behavioral Intervention for Tics) + α2 agonists clonidine 0.1-0.3 mg/d, guanfacine 1-4 mg/d + antipsychotics aripiprazole 5-15 mg, risperidone 0.25-2 mg, haloperidol 0.5-3 mg + VMAT-2 (deutetrabenazine, valbenazine) + DBS refractory severe
  • RLScheck ferritin (target > 75) + iron supplementation (oral or IV) + α2ÎŽ ligands first-line (gabapentin 300-1800 mg, pregabalin 75-450 mg, gabapentin enacarbil 600-1200 mg) > dopamine agonists (pramipexole, ropinirole — augmentation risk); opioids for severe refractory
  • akathisiareduce/change offending medication + propranolol 30-120 mg/d + benzodiazepines + anticholinergics + mirtazapine
  • myoclonusvalproate + levetiracetam + clonazepam + piracetam

370.4.3 🎯 盧醫垫的考前提醒

  1. Tremor types (memorize): rest (PD pill-rolling 4-6 Hz asymmetric) + postural (essential tremor most common adult MD, alcohol-responsive, AD often) + kinetic/intention (cerebellar) + task-specific
  2. Essential tremor treatment: propranolol + primidone first-line + topiramate + gabapentin; refractory DBS Vim thalamic nucleus or focused ultrasound (FUS) thalamotomy FDA 2016 non-invasive
  3. Dopa-responsive dystonia (DRD, Segawa): childhood + diurnal worsening + dramatic response to small dose levodopa (always trial in early-onset dystonia!) + GCH1 mutation
  4. Focal dystonia first-line: botulinum toxin every 3-4 months (cervical/blepharospasm/writer’s cramp/spasmodic dysphonia)
  5. Huntington disease: AD + CAG repeat ≥ 40 in HTT + anticipation (longer repeats in offspring) + onset 30-50s + triad chorea + cognitive decline + psychiatric + caudate atrophy (“box-car” ventricles) + no disease-modifying therapy + symptomatic VMAT-2 (tetrabenazine, deutetrabenazine, valbenazine) for chorea
  6. VMAT-2 inhibitors (memorize indications): tetrabenazine (Xenazine) + deutetrabenazine (Austedo) + valbenazine (Ingrezza) for HD chorea + tardive dyskinesia + Tourette tics; side effects depression (BBW) + parkinsonism + akathisia + QT prolongation
  7. Sydenham chorea: post-streptococcal + children + major Jones criterion for rheumatic fever + self-limited
  8. Tourette syndrome: childhood onset 5-7 yo + motor + vocal tics > 1 year + comorbid OCD + ADHD → CBIT (Comprehensive Behavioral Intervention for Tics) + α2 agonists clonidine/guanfacine + antipsychotics aripiprazole/risperidone + VMAT-2 + DBS refractory
  9. Restless legs syndrome (Willis-Ekbom): urge to move + worse rest + relieved by movement + worse evening; iron deficiency most common secondary (check ferritin < 75 supplement) + α2Ύ ligands (gabapentin, pregabalin, gabapentin enacarbil) first-line now > dopamine agonists (augmentation concern)
  10. Tardive syndromes after chronic antipsychotic — tardive dyskinesia oro-facial-lingual + tardive dystonia + tardive akathisia → VMAT-2 inhibitors valbenazine (Ingrezza) + deutetrabenazine (Austedo) FDA 2017 + switch to clozapine/quetiapine (lower risk); asterixis (negative myoclonus) = hepatic/uremic encephalopathy