RECENT ADVANCES IN THEMANGEMENT OF EXTRA PYRAMIDAL-      (BASAL GANGLIA) DISORDERSProf. A.V. SRINIVASAN, MD, DM, Ph.D, F.A...
Sir William OslerTo study the phenomenon of diseasewithout books is to sail an uncharted sea,while to study books, without...
DEFINITIONMovement disorders can be defined asneurologic syndromes in which there iseither an excess of movement or paucit...
MOVEMENT DISORERSDESCRIBEDStarted on 1567 to 2008 – 111 conditions
Movement                      No. of     PercentDisorder                      PatientsParkinsonism                  7568  ...
CATEGORIES OF MOVEMENTS1.   Automatic2.   Voluntary3.   Semivoluntary4.   Involuntary
DEDICATED TO PROF      C.D.MARSDEN- A GENIUS IN      MOVEMENT DISORDERS•P-   PARKINSONISM•R- RESTLESS LEG SYNDROME•O- OROF...
CHOREARandom, quick         Huntington disease unsustained            Neurocanthocytosis purposeless movements that hav...
Ballismus, Chorea, Athotosis          and DystoniaThese should NOT be thought of asseparate entities amenable to specificd...
Because……..• They often co-exist• Even neurologists may often not be  able to agree as to how a particular  movement shoul...
The spectrumBallismus     Chorea        Athetosis         Dystonia Movements become - Less violent / explosive / jerky    ...
Ballismus• Violent “flinging” movement of entire limb• Almost always unilateral and therefore     use term “ HEMIBALLISMUS...
Causes of Chorea, Dystonia             and athetosis•   Hereditary•   Static Encephalopathy ( Cerebral Palsy )•   Drugs•  ...
Hereditary• Huntington’s disease• Wilson’s disease• Neuroacanthocytosis• Hereditary dystonias   - idiopathic torsion dysto...
Secondary to medical              disorders             (A SHEEP)•   Anoxic brain damage ( post – CPR )•   Systemic lupus ...
Chorea ( “dance” in Greek)• Rapid irregular muscle jerks• May affect limbs, head, face and tongue• In the limbs chorea ref...
Sydenham’s chorea• Mainly children / adolescents• Complication of previous group A    streptococcal infection•   Usually n...
Chorea gravidarum• Chorea of any cause that begins in  pregnancy• May represent recurrence of  Sydenham’s chorea.• Most co...
Athetosis “ changeable” in Greek•   Slow, flowing, often twisting movements•   Occurs mainly distally ( hands, fingers)•  ...
MYOCLONUS                      Physiologic myoclonus Sudden, Shocklike    Essential myoclonus  movements           Meta...
TREATMENT OF MYOCLONUS        Drug       Initial Adult DoseClonazepam      0.5 mg / dayLevetiracetam   250 mg / dayPi...
TREATMENT OF MYOCLONUS                                             Indication      Usual Effective Dose                   ...
TREMORRepetitive oscillation   Essential tremor  of a body part          Physiologic tremor                          P...
TREATMENT OF ESSENTIAL TREMOR Drug          Initial Adult         Usual Effective                 Dose                    ...
TREMOR CONTD..Class                 Drug          Usual Effective                                    Dose (mg/day)Antichli...
TICStereotyped,    Tourette syndrome automatic       Celebral palsy or purposeless      developmental delay movements a...
PRIMARY TIC DISORDERS                         DIAGNOSTIC CRITERIADISORDER                 Presence of multiple motor andTo...
Primary tic disorders                            Diagnostic CriteriaDisorder                            Chronic motor or c...
DIFFERENTIAL DIANOSIS OF TICSlassificationC                    Diffenential                     DiagnosisSimple Motor Tics...
Classification                                                           Diffenential                                     ...
Treatment of tic disordersDrug                   Usual        Potential                       EffectiveClonide            ...
Treatment of neuropsychiatric     symptoms associated with tic     disordersNeuropsychiatric        Drug             Usual...
Dopamine receptor – blockingagentsClass                     DrugPhenothiazine                          Chlorpromazine,• Al...
Dopamine receptor – blocking    agents                                Drug Clas s                         Metoclopramide, ...
General guidelines for treatingtardive syndromes•   Taper and slowly eliminate causative medications, if clinically possib...
NEUROLEPTIC INDUCED        MOVEMENT DISORDERS1.   Acute reaction     a. Acute dystonia     b. Acute (Subacute) akathisia2....
TERMINOLOGY OF THE TARDIVE SYNDROMESDescription               Equivalent                          CommonNamesTardive syndr...
Adverse        Haloperidal   Clozapine Quetiapin Lanzopine   RisperidonEffect                                 e           ...
NATURE HISTORY OFTOURETTE SYNDROME•   Excerbation                   Remission ?•   Obsessive-compulsive behavior•   Vocal ...
Summary• Movement disorders are often difficult to define precisely, but  have similar differential diagnoses.• They are o...
Dedicated to my family formaking everything worthwhile
READ not to contradict or confute   Nor to Believe and Take for Granted   but TO WEIGH AND CONSIDER  THANK YOU  My sincere...
Recent advances in the mangement of extra pyramidal basal ganglia disorders
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Recent advances in the mangement of extra pyramidal basal ganglia disorders

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Recent advances in the mangement of extra pyramidal basal ganglia disorders

  1. 1. RECENT ADVANCES IN THEMANGEMENT OF EXTRA PYRAMIDAL- (BASAL GANGLIA) DISORDERSProf. 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. 2. Sir William OslerTo study the phenomenon of diseasewithout books is to sail an uncharted sea,while to study books, without patients isnot to go to sea at all. Sir William Osler Aphorisms
  3. 3. DEFINITIONMovement disorders can be defined asneurologic syndromes in which there iseither an excess of movement or paucity ofvoluntary and automatic movements,unrelated to weakness or spasticity.
  4. 4. MOVEMENT DISORERSDESCRIBEDStarted on 1567 to 2008 – 111 conditions
  5. 5. Movement No. of PercentDisorder PatientsParkinsonism 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. 6. CATEGORIES OF MOVEMENTS1. Automatic2. Voluntary3. Semivoluntary4. Involuntary
  7. 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. 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. 9. Ballismus, Chorea, Athotosis and DystoniaThese should NOT be thought of asseparate entities amenable to specificdefinition but rather as a SPECTRUM ofmovements that blend into one-anotherWHY?
  10. 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. 11. The spectrumBallismus 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. 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. 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. 14. Hereditary• Huntington’s disease• Wilson’s disease• Neuroacanthocytosis• Hereditary dystonias - idiopathic torsion dystonia - dopa - responsive dystonia
  15. 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. 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. 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. 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. 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. 20. MYOCLONUS Physiologic myoclonus Sudden, Shocklike Essential myoclonus movements Metabolic encephalopathy Postanoxic myoclonus Progressive myoclonic epilepsy
  21. 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. 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. 23. TREMORRepetitive oscillation Essential tremor of a body part Physiologic tremor Parkinson tremor Crebellar tremor
  24. 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. 25. TREMOR CONTD..Class Drug Usual Effective Dose (mg/day)Antichlinergic Trihexyphenidyl 6 – 80agent Benztropine 4–8 Ethopropazine 100-400Benzodiazepine Clonazepam 1–4 Diazepam 10 – 60 Lorazepam 1–6Dopamine-deple- Tetrabenzine 50 – 200ting agent Reser[ome 1–3GABA against Baclofen 30 – 80
  26. 26. TICStereotyped, Tourette syndrome automatic Celebral palsy or purposeless developmental delay movements and syndromes vocalizations Autism Huntington disease
  27. 27. PRIMARY TIC DISORDERS DIAGNOSTIC CRITERIADISORDER Presence of multiple motor andTourette 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 yTransient tic disorder
  28. 28. Primary tic disorders Diagnostic CriteriaDisorder Chronic motor or chronic vocalChronic tic disorder tics (but not both of >1y Chronic single motor or chronic single vocal ticChronic single tic Tic order that begins > age 21 disorder Two temporal patterns: - De novo adult-onset tic - Recurrent childhood tic – a ticAdult-onset tic discorder disorder than goes into remission and recurs during adulthood
  29. 29. DIFFERENTIAL DIANOSIS OF TICSlassificationC Diffenential DiagnosisSimple Motor TicsClonic Myoclonus Chorea SeizuresDystonia Dystonia AthetasisTonic Muscle spasms and crampsComplex Motor Tcs Mannerisms Stereotypies Restless legs syndrome SeizurePhenomenologyAbrupt Myoclonus Chorea Hyperekplexia Paraxysmal dyskinesia SeizuresSensory phenomenon Akathisia-stereotypy(urge relief) Restless legs syndrome
  30. 30. Classification Diffenential DiagnosisPerceived as voluntary AkasthisiaSuppressibility All hyperkinesias but less than ticsDecrease with distraction Akasthisia Psychogenic movementsIncrease with stress Most hyperkinesiasIncrease with relaxation Parkinsonian tremor (after a period of stressA)Multifocal migrate Chorea MyoclonusFluctuate spontaneously Paraxysmal dykinesias SeizuresPresent during sleep Myoclonus (segmental) Periodic movements Painfullegs / moving toes Other hyperkinesias SEizures
  31. 31. Treatment of tic disordersDrug Usual Potential EffectiveClonide 0.05-0.5 Drowsiness, hypotensionGuanfacine 0.5 – 4 Drowsiness, hypotensionClonazepam 0.25 – 2 Drowsiness, irritabilityTetrabenazine 12.5 – 100 Drowsiness, hypotension, depression, parkinsonismReserpine 0.25 – 3 Drowsiness, hypotension, depression parkinsonismRisperidone 0.5 – 12 Parkinsonism, risk of tardive dyskinesiasOlanzapine 2.5 – 15 Parkinsonism, riks of tardive dyskinesiasPimozide 0.5 – 10 Parkinsonism, risk of tardive dyskinesias, retinopathyHaloperidol 05 – 20 Parkinsonism, risk of tardive dyskinesias
  32. 32. Treatment of neuropsychiatric symptoms associated with tic disordersNeuropsychiatric Drug Usual EffectiveObessive Clomipramine 25 – 250Compulsive Fluoxetine 10 – 60Disorder (OCD) Sertraline 25 – 200 Fluvoxamine 25 – 300 Venlafaxine 75 – 225 Citalopram 10 – 40 Buspirone 5 – 30 Clonazepam 1.5 – 60Attention-deficit Methylphenidate 2.5 – 60Disorder (ADD) or Dextroamphetamine 2.5 - 40Attention-deficit/ Pemoline 18.75 – 112.5Hyperactivity Modafinil 100 – 400Disorder (ADHD)
  33. 33. Dopamine receptor – blockingagentsClass DrugPhenothiazine Chlorpromazine,• Alphatic triflupromazine Thioridazine,mesoridazine Trifluoperazine, prochlor perazine, perphenazine• Piperidine fluphenazine• Piperidine Chlorprothixene ThiothixeneThioxanthene Haloperidol, droperidol• Aliphatic Pimozide Loxapine• Piperazine Clozapine, quetiapineButyrophenone OlanzapineDiphenylbutylpiperidineDibenzazepineDibenzodiazepineThienobenzodiazepine
  34. 34. Dopamine receptor – blocking agents Drug Clas s Metoclopramide, tiapride,Indalone Sulpiride, Clebopride,Pyrimidinone remoxipride, veraliprideBenzothiazole MolindoneBenzisoxazole RisperidoneTricyclic antidepressant ZiprasidoeCalcum channel blocker Iloperiode Amoxapie FlunarizineCinnarizine
  35. 35. General guidelines for treatingtardive 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. 36. NEUROLEPTIC INDUCED MOVEMENT DISORDERS1. Acute reaction a. Acute dystonia b. Acute (Subacute) akathisia2. Toxicity state (over dosage) a. Drug-induced parkinsonism3. 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. 37. TERMINOLOGY OF THE TARDIVE SYNDROMESDescription Equivalent CommonNamesTardive syndromes Tardive syndromeas a group Tardive dykinesiaRepetitive, rhythmic Classic tardiveMovements, usually in dyskinesiaThe oral-buccal-lingual Tardive setreotypyRegion Rhythemic choreaDystonic movementsand posturesrestlessness and the Tardive akasthisiamovement that occuras resultMyoclonus Tardive myoclonusTremor Tardive tremorTucs Tardive tics Tardive tourettismChorea Withdrawal emergent syndrome Tardive choreaOculogyria Tardive oculogyric crisisParkinsonism Tardive Parkinsonism (if it exists)
  38. 38. Adverse Haloperidal Clozapine Quetiapin Lanzopine RisperidonEffect e eParkinsonism Yes Yes Not yet Yes YesAcute Yes Yes Yes Yes YesakastisiaAcute Yes Yes Yes Yes YesdystoniaNeuroleptic Yes Yes Not yet Yes YesmalignantsyndromeTardive Yes Yes Yes Yes Yessyndrome
  39. 39. NATURE HISTORY OFTOURETTE SYNDROME• Excerbation Remission ?• Obsessive-compulsive behavior• Vocal tics (simple complex)• Motor tics (rostrocaudalprogession)• Attention deficit with hyperactivity
  40. 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
  41. 41. Dedicated to my family formaking everything worthwhile
  42. 42. 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 MadrasInstitute of Neurology and Madras MedicalCollege for giving this opportunity to speak in the CME Program
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