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Recent Advances in the Management of Extrapyramidal (Basal Ganglia) Disorders
- 1. RECENT ADVANCES IN THE MANGEMENT OF EXTRA PYRAMIDAL- (BASAL GANGLIA) DISORDERS Prof. A.V. SRINIVASAN, MD, DM, Ph.D, F.A.A.N, F.I.A.N, EMERITUS PROFESSOR TAMILNADU DR.M.G.R MEDICAL UNIVERSITY CHENNAI FORMER PROFESSOR AND HEAD INSTITUTE OF NEUROLOGY MADRAS MEDICAL COLLEGE 17th MAY 2009
- 2. Sir William Osler To study the phenomenon of disease without books is to sail an uncharted sea, while to study books, without patients is not to go to sea at all. Sir William Osler Aphorisms
- 3. DEFINITION Movement disorders can be defined as neurologic syndromes in which there is either an excess of movement or paucity of voluntary and automatic movements, unrelated to weakness or spasticity.
- 4. MOVEMENT DISORERS DESCRIBED Started on 1567 to 2008 – 111 conditions
- 5. Movement No. of Percent Disorder Patients Parkinsonism 7568 32.9 Dystonia 6798 31.3 Tremor 3013 13.9 Tics 1022 4.7 (Tourette Syndrome) Chorea 658 3.1 Tardive Syndrome 583 2.7 Myoclonus 547 2.5 Hemifacial Spasm 359 1.7 Ataxia 316 1.5 Paraxysmal dyskniesias 169 0.8 Stereotypies 163 0.7 Restless legs syndrome 108 0.5 Stiff-person syndrome 32 0.1 Psychogenic movement disorder 43.4 2.0 Grand Total 21,766 100
- 6. CATEGORIES OF MOVEMENTS 1. Automatic 2. Voluntary 3. Semivoluntary 4. Involuntary
- 7. DEDICATED TO PROF C.D.MARSDEN- A GENIUS IN MOVEMENT DISORDERS •P- PARKINSONISM •R- RESTLESS LEG SYNDROME •O- OROFACIAL DYSKINESIA •F- FIBRILLATION AND FASCICULATIONS • C –CHOREA INCLUDING HEMIBALISM • D- DYSTONIA • M- MYOCLONUS,MYOKYMIA,MYORHYTHMIA,MTAF • A-ATAXIA,AKATHESIA,ATHETOSIS,ABD.DYS • R-RETT SYNDROME, • S-STEREOTYPY,SPASM(HEMIFACIAL),JUMPY STUMPS • D-DYSKINESIA(PAROXYSMAL) • E-ESSENTIAL TREMOR, EKPLEXIA(HYPER) • N-NEUROLEPTIC INDUCED -TARDIVE DYSKINESIA
- 8. CHOREA Random, quick Huntington disease unsustained Neurocanthocytosis purposeless movements that have Postinfectious an unpredictable chorea flowing pattern Drug-induced chorea Vascular chorea Autoimmune chorea Chorea gravidarum
- 9. Ballismus, Chorea, Athotosis and Dystonia These should NOT be thought of as separate entities amenable to specific definition but rather as a SPECTRUM of movements that blend into one-another WHY?
- 10. Because…….. • They often co-exist • Even neurologists may often not be able to agree as to how a particular movement should be classified! • They often ( with some notable exceptions ) have the same significance in terms of aetiology.
- 11. The spectrum Ballismus Chorea Athetosis Dystonia Movements become - Less violent / explosive / jerky - Smoother and more flowing - More sustained They differ from tics in that they cannot be suppressed by voluntary control
- 12. Ballismus • Violent “flinging” movement of entire limb • Almost always unilateral and therefore use term “ HEMIBALLISMUS” • Involves proximal musculature and is sometimes thought of as a “ proximal unilateral chorea ” • Usually due to a CVA in contralateral subthalamic nucleus
- 13. Causes of Chorea, Dystonia and athetosis • Hereditary • Static Encephalopathy ( Cerebral Palsy ) • Drugs • Cerebrovascular ( ischaemia, haemorrhage ) • Structural lesions ( subthalamic nucleus) • Secondary to medical disorders • Miscellaneous - Sydenham’s chorea - Chorea Gravidarum - Sporadic idiopathic torsion dystonia - Focal dystonias
- 14. Hereditary • Huntington’s disease • Wilson’s disease • Neuroacanthocytosis • Hereditary dystonias - idiopathic torsion dystonia - dopa - responsive dystonia
- 15. Secondary to medical disorders (A SHEEP) • Anoxic brain damage ( post – CPR ) • Systemic lupus erythematosis • Hepatic failure • Endocrine - Thyrotoxicosis - Addisons • Electrolyte - Low Ca, Mg, - High Na • Polycythemia rubra vera
- 16. Chorea ( “dance” in Greek) • Rapid irregular muscle jerks • May affect limbs, head, face and tongue • In the limbs chorea refers more to distal movements ( as proximal movements usually called ballismus) • Patients often attempt to conceal involuntary movements by superimposing voluntary movements onto them e.g. an involuntary movement of arm towards face may be adapted to look-like an attempt to look at watch
- 17. Sydenham’s chorea • Mainly children / adolescents • Complication of previous group A streptococcal infection • Usually no recent history of infection • Acute / subacute onset • May have behavioural problems • Usually remits spontaneously
- 18. Chorea gravidarum • Chorea of any cause that begins in pregnancy • May represent recurrence of Sydenham’s chorea. • Most commonly associated with anti- phospholipid syndrome +/- SLE • Usually resolves spontaneously
- 19. Athetosis “ changeable” in Greek • Slow, flowing, often twisting movements • Occurs mainly distally ( hands, fingers) • Can also affect face and tongue • Often use term “ choreoathetosis ” due to overlap between syndromes ( chorea referring to less smooth , more jerky movements)
- 20. MYOCLONUS Physiologic myoclonus Sudden, Shocklike Essential myoclonus movements Metabolic encephalopathy Postanoxic myoclonus Progressive myoclonic epilepsy
- 21. TREATMENT OF MYOCLONUS Drug Initial Adult Dose Clonazepam 0.5 mg / day Levetiracetam 250 mg / day Piracetam 400 mg 3 times a day Primidone 25 mg / day Valporate 125 mg 2 times a day
- 22. TREATMENT OF MYOCLONUS Indication Usual Effective Dose Posthypoxic myoclonus Spinal 2 mg/day divided myoclonus Progressive myoclonic 3 times a day epilepsy Essential myoclonus 1000-1500 mg/day Posthypoxic myoclous Cortical myoclonus Spinal myoclous 1200-16,000 mg/day divided 3 Posthypoxic myoclonus Cortical times a day myoclonus Progressive myoclonic epilepsy Essential myoclonus 500-750 mg/day Cortical myoclonus 750-1000 mg/day divided 2 Most forms of myoclonus times a day
- 23. TREMOR Repetitive oscillation Essential tremor of a body part Physiologic tremor Parkinson tremor Crebellar tremor
- 24. TREATMENT OF ESSENTIAL TREMOR Drug Initial Adult Usual Effective Dose Dose Propranolol 20 mg/day 80-240 mg/day Primidone 12.5-25mg 50-300 mg/day at bed time divided Bio or at bedtime Topiramate 12.5=25mg/day 400 mg/day maximum dose divided Bio
- 25. TREMOR CONTD.. Class Drug Usual Effective Dose (mg/day) Antichlinergic Trihexyphenidyl 6 – 80 agent Benztropine 4–8 Ethopropazine 100-400 Benzodiazepine Clonazepam 1–4 Diazepam 10 – 60 Lorazepam 1–6 Dopamine-deple- Tetrabenzine 50 – 200 ting agent Reser[ome 1–3 GABA against Baclofen 30 – 80
- 26. TIC Stereotyped, Tourette syndrome automatic Celebral palsy or purposeless developmental delay movements and syndromes vocalizations Autism Huntington disease
- 27. PRIMARY TIC DISORDERS DIAGNOSTIC CRITERIA DISORDER Presence of multiple motor and Tourette syndrome vocal tics Age at onset <21 y Tics must occur many times daily, nearly every day, over a period of >1y Disturbance causes marked distress or significant impairment in daily functioning Condition cannot be ascribed to known neurological disorder (symptomatic or secondary tic disorder) Duration of tic disorder <1 y Transient tic disorder
- 28. Primary tic disorders Diagnostic Criteria Disorder Chronic motor or chronic vocal Chronic tic disorder tics (but not both of >1y Chronic single motor or chronic single vocal tic Chronic single tic Tic order that begins > age 21 disorder Two temporal patterns: - De novo adult-onset tic - Recurrent childhood tic – a tic Adult-onset tic discorder disorder than goes into remission and recurs during adulthood
- 29. DIFFERENTIAL DIANOSIS OF TICS lassification C Diffenential Diagnosis Simple Motor Tics Clonic Myoclonus Chorea Seizures Dystonia Dystonia Athetasis Tonic Muscle spasms and cramps Complex Motor Tcs Mannerisms Stereotypies Restless legs syndrome Seizure Phenomenology Abrupt Myoclonus Chorea Hyperekplexia Paraxysmal dyskinesia Seizures Sensory phenomenon Akathisia-stereotypy (urge relief) Restless legs syndrome
- 30. Classification Diffenential Diagnosis Perceived as voluntary Akasthisia Suppressibility All hyperkinesias but less than tics Decrease with distraction Akasthisia Psychogenic movements Increase with stress Most hyperkinesias Increase with relaxation Parkinsonian tremor (after a period of stressA) Multifocal migrate Chorea Myoclonus Fluctuate spontaneously Paraxysmal dykinesias Seizures Present during sleep Myoclonus (segmental) Periodic movements Painfullegs / moving toes Other hyperkinesias SEizures
- 31. Treatment of tic disorders Drug Usual Potential Effective Clonide 0.05-0.5 Drowsiness, hypotension Guanfacine 0.5 – 4 Drowsiness, hypotension Clonazepam 0.25 – 2 Drowsiness, irritability Tetrabenazine 12.5 – 100 Drowsiness, hypotension, depression, parkinsonism Reserpine 0.25 – 3 Drowsiness, hypotension, depression parkinsonism Risperidone 0.5 – 12 Parkinsonism, risk of tardive dyskinesias Olanzapine 2.5 – 15 Parkinsonism, riks of tardive dyskinesias Pimozide 0.5 – 10 Parkinsonism, risk of tardive dyskinesias, retinopathy Haloperidol 05 – 20 Parkinsonism, risk of tardive dyskinesias
- 32. Treatment of neuropsychiatric symptoms associated with tic disorders Neuropsychiatric Drug Usual Effective Obessive Clomipramine 25 – 250 Compulsive Fluoxetine 10 – 60 Disorder (OCD) Sertraline 25 – 200 Fluvoxamine 25 – 300 Venlafaxine 75 – 225 Citalopram 10 – 40 Buspirone 5 – 30 Clonazepam 1.5 – 60 Attention-deficit Methylphenidate 2.5 – 60 Disorder (ADD) or Dextroamphetamine 2.5 - 40 Attention-deficit/ Pemoline 18.75 – 112.5 Hyperactivity Modafinil 100 – 400 Disorder (ADHD)
- 33. Dopamine receptor – blocking agents Class Drug Phenothiazine Chlorpromazine, • Alphatic triflupromazine Thioridazine,mesoridazine Trifluoperazine, prochlor perazine, perphenazine • Piperidine fluphenazine • Piperidine Chlorprothixene Thiothixene Thioxanthene Haloperidol, droperidol • Aliphatic Pimozide Loxapine • Piperazine Clozapine, quetiapine Butyrophenone Olanzapine Diphenylbutylpiperidine Dibenzazepine Dibenzodiazepine Thienobenzodiazepine
- 34. Dopamine receptor – blocking agents Drug Clas s Metoclopramide, tiapride, Indalone Sulpiride, Clebopride, Pyrimidinone remoxipride, veralipride Benzothiazole Molindone Benzisoxazole Risperidone Tricyclic antidepressant Ziprasidoe Calcum channel blocker Iloperiode Amoxapie FlunarizineCinnarizine
- 35. General guidelines for treating tardive syndromes • Taper and slowly eliminate causative medications, if clinically possible. Avid sudden cessation of these drugs, which can exacerbate symptoms • If treatment of tardive movements is necessary, the drugs of first choice are the dopamine-depleting drugs reserpine, tetrabenazine, and a- methylparatyrosine. Monitor for the development of depression, hypotension sedation, and parkinsonism. • If dopamine-depleting agents are ineffective, consider a trial of clozpine or quetiapine. • Dopamine receptor-blocking agents can be used as medications of last resort for patients with tardive syndromes despite the risk of worsening the syndrome over the long term. • Consider globus pallidus stimulation if pharmacotherapy is ineffective.
- 36. NEUROLEPTIC INDUCED MOVEMENT DISORDERS 1. Acute reaction a. Acute dystonia b. Acute (Subacute) akathisia 2. Toxicity state (over dosage) a. Drug-induced parkinsonism 3. Neuroleptic malignant syndrome (NMS) 4. Tardive stbdrines a. Withdrawal emergent syndrome b.Classic fardive dyskinesia c. Tardive dystonia d. Tardive akathisia e. Tardive myoclonus f. Tardive tremor g. Tardive tics h. Tardive Chorea i. (?) Tardive parkinsonism
- 37. TERMINOLOGY OF THE TARDIVE SYNDROMES Description Equivalent CommonNames Tardive syndromes Tardive syndrome as a group Tardive dykinesia Repetitive, rhythmic Classic tardive Movements, usually in dyskinesia The oral-buccal-lingual Tardive setreotypy Region Rhythemic chorea Dystonic movements and postures restlessness and the Tardive akasthisia movement that occur as result Myoclonus Tardive myoclonus Tremor Tardive tremor Tucs Tardive tics Tardive tourettism Chorea Withdrawal emergent syndrome Tardive chorea Oculogyria Tardive oculogyric crisis Parkinsonism Tardive Parkinsonism (if it exists)
- 38. Adverse Haloperidal Clozapine Quetiapin Lanzopine Risperidon Effect e e Parkinsonism Yes Yes Not yet Yes Yes Acute Yes Yes Yes Yes Yes akastisia Acute Yes Yes Yes Yes Yes dystonia Neuroleptic Yes Yes Not yet Yes Yes malignant syndrome Tardive Yes Yes Yes Yes Yes syndrome
- 39. NATURE HISTORY OF TOURETTE SYNDROME • Excerbation Remission ? • Obsessive-compulsive behavior • Vocal tics (simple complex) • Motor tics (rostrocaudalprogession) • Attention deficit with hyperactivity
- 40. Summary • Movement disorders are often difficult to define precisely, but have similar differential diagnoses. • They are often a manifestation of a more widespread neurological or internal medical problem. • Other than the specific treatments mentioned, most details of therapy are beyond the scope of this course. • In some cases treatment includes treatment of underlying cause e.g. Wilson’s disease
- 42. Dedicated to my family for making everything worthwhile
- 43. READ not to contradict or confute Nor to Believe and Take for Granted but TO WEIGH AND CONSIDER THANK YOU My sincere thanks to Faculty of Madras Institute of Neurology and Madras Medical College for giving this opportunity to speak in the CME Program