Prof. A.V. SRINIVASAN , MD, DM, Ph.D, F.A.A F.I.A.N, EMERITUS PROFESSOR TAMILNADU DR.M.G.R MEDICAL UNIVERSITY CHENNAI FORMER PROFESSOR AND HEAD INSTITUTE OF NEUROLOGY MADRAS MEDICAL COLLEGE 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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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
RECENT ADVANCES IN THE MANGEMENT OF EXTRA PYRAMIDAL- (BASAL GANGLIA) DISORDERS
Sir William OslerSir William Osler
To study the phenomenon of disease To study the phenomenon of disease without books is to sail an uncharted without books is to sail an uncharted sea, while to study books, without sea, while to study books, without patients is not to go to sea at all.patients is not to go to sea at all.
Sir William Osler AphorismsSir William Osler Aphorisms
DEFINITIONDEFINITION
Movement disorders can be defined as Movement disorders can be defined as neurologic syndromes in which there is neurologic syndromes in which there is either an excess of movement or either an excess of movement or paucity of voluntary and automatic paucity of voluntary and automatic movements, unrelated to weakness or movements, unrelated to weakness or spasticity.spasticity.
MOVEMENT DISORERS MOVEMENT DISORERS DESCRIBEDDESCRIBED
Started on 1567 to 2008 – 111 Started on 1567 to 2008 – 111 conditionsconditions
MovementMovement No. of No. of PercentPercentDisorder Disorder PatientsPatientsParkinsonismParkinsonism 7568 7568 32.932.9
DEDICATED TO PROF DEDICATED TO PROF C.D.MARSDEN- A GENIUS IN C.D.MARSDEN- A GENIUS IN MOVEMENT DISORDERSMOVEMENT 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
CHOREACHOREARandom, quick Random, quick
unsustained unsustained purposeless purposeless movements that movements that have an have an unpredictable unpredictable flowing patternflowing pattern
Ballismus, Chorea, Athotosis Ballismus, Chorea, Athotosis and Dystoniaand Dystonia
These should NOT be thought of as These should NOT be thought of as separate entities amenable to specific separate entities amenable to specific definition but rather as a SPECTRUM definition but rather as a SPECTRUM of movements that blend into one-of movements that blend into one-anotheranother
WHY?WHY?
Because……..Because……..
• They often co-exist They often co-exist
• Even neurologists may often not Even neurologists may often not be be able to agree as to how a able to agree as to how a particular particular movement movement should be classified!should be classified!
• They often ( with some notable They often ( with some notable exceptions ) have the same exceptions ) have the same
significance in terms of aetiology. significance in terms of aetiology.
The spectrumThe spectrum
Ballismus DystoniaChorea Athetosis
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
BallismusBallismus
• Violent “flinging” movement of entireViolent “flinging” movement of entirelimblimb
• Almost always unilateral and thereforeAlmost always unilateral and therefore
use term “ HEMIBALLISMUS”use term “ HEMIBALLISMUS”
• Involves proximal musculature and is Involves proximal musculature and is sometimes thought of as a sometimes thought of as a
• ElectrolyteElectrolyte - Low Ca, Mg, - Low Ca, Mg,
- High Na- High Na
• Polycythemia rubra veraPolycythemia rubra vera
Secondary to medical Secondary to medical disordersdisorders(A SHEEP)(A SHEEP)
Chorea ( “dance” in Greek)Chorea ( “dance” in Greek)
• Rapid irregular muscle jerksRapid irregular muscle jerks• May affect limbs, head, face and tongueMay affect limbs, head, face and tongue
• In the limbs chorea refers more to distal In the limbs chorea refers more to distal movements ( as proximal movements movements ( as proximal movements usually called ballismus)usually called ballismus)
• Patients often attempt to conceal involuntary Patients often attempt to conceal involuntary movements by superimposing voluntary movements by superimposing voluntary movements onto them e.g. an involuntary movements onto them e.g. an involuntary movement of arm towards face may be movement of arm towards face may be adapted to look-like an attempt to look at adapted to look-like an attempt to look at watchwatch
Sydenham’s choreaSydenham’s chorea
• Mainly children / adolescentsMainly children / adolescents
• Complication of previous group A Complication of previous group A streptococcal infectionstreptococcal infection
• Usually no recent history of infectionUsually no recent history of infection
• Acute / subacute onsetAcute / subacute onset
• May have behavioural problems May have behavioural problems
• Usually remits spontaneouslyUsually remits spontaneously
Chorea gravidarumChorea gravidarum
• Chorea of any cause that begins in Chorea of any cause that begins in pregnancypregnancy
• May represent recurrence of May represent recurrence of Sydenham’s chorea.Sydenham’s chorea.
• Most commonly associated with Most commonly associated with anti-phospholipid syndrome +/- SLEanti-phospholipid syndrome +/- SLE
• Usually resolves spontaneouslyUsually resolves spontaneously
Athetosis “ changeable” in Athetosis “ changeable” in GreekGreek• Slow, flowing, often twisting Slow, flowing, often twisting
fingers)fingers)• Can also affect face and tongueCan also affect face and tongue• Often use term “ choreoathetosis ” Often use term “ choreoathetosis ”
due to overlap between syndromes due to overlap between syndromes ( chorea referring to less smooth , ( chorea referring to less smooth , more jerky movements)more jerky movements)
DIAGNOSTIC CRITERIADIAGNOSTIC CRITERIAPresence of multiple motor Presence of multiple motor
and vocal ticsand vocal ticsAge at onset <21 yAge at onset <21 yTics must occur many times Tics must occur many times
daily, nearly every day, over daily, nearly every day, over a period of >1y a period of >1y
Disturbance causes marked Disturbance causes marked distress or significant distress or significant impairment in daily impairment in daily functioning functioning
Condition cannot be ascribed Condition cannot be ascribed to known neurological to known neurological disorder (symptomatic or disorder (symptomatic or secondary tic disorder)secondary tic disorder)
Duration of tic disorder <1 yDuration of tic disorder <1 y
Primary tic disordersPrimary tic disorders
DisorderDisorder
Chronic tic disorderChronic tic disorder
Chronic single tic Chronic single tic disorderdisorder
Diagnostic CriteriaDiagnostic CriteriaChronic motor or chronic vocal Chronic motor or chronic vocal
tics (but not both of >1ytics (but not both of >1y
Chronic single motor or chronic Chronic single motor or chronic single vocal ticsingle vocal tic
Tic order that begins > age 21Tic order that begins > age 21Two temporal patterns:Two temporal patterns:- De novo adult-onset ticDe novo adult-onset tic- Recurrent childhood tic – a tic Recurrent childhood tic – a tic
disorder than goes into disorder than goes into remission and recurs during remission and recurs during adulthoodadulthood
DIFFERENTIAL DIANOSIS OF TICSDIFFERENTIAL DIANOSIS OF TICSCClassificationlassification Diffenential Diffenential DiagnosisDiagnosisSimple Motor TicsSimple Motor TicsClonicClonic MyoclonusMyoclonus ChoreaChorea SeizuresSeizuresDystonia Dystonia DystoniaDystonia AthetasisAthetasisTonicTonic Muscle spasms and Muscle spasms and crampscrampsComplex Motor TcsComplex Motor Tcs MannerismsMannerisms StereotypiesStereotypies Restless legs syndromeRestless legs syndrome SeizureSeizurePhenomenologyPhenomenologyAbruptAbrupt MyoclonusMyoclonus ChoreaChorea HyperekplexiaHyperekplexia Paraxysmal dyskinesiaParaxysmal dyskinesia
SeizuresSeizuresSensory phenomenon Sensory phenomenon Akathisia-stereotypyAkathisia-stereotypy(urge relief) (urge relief) Restless legs syndromeRestless legs syndrome DystoniaDystonia
Classification Classification
Diffenential Diffenential DiagnosisDiagnosis
Perceived as voluntaryPerceived as voluntary Akasthisia AkasthisiaSuppressibility Suppressibility All hyperkinesias but less than All hyperkinesias but less than
ticsticsDecrease with distractionDecrease with distraction Akasthisia Akasthisia Psychogenic movements Psychogenic movements Increase with stressIncrease with stress Most hyperkinesias Most hyperkinesiasIncrease with relaxationIncrease with relaxation Parkinsonian tremor Parkinsonian tremor (after a period of stressA)(after a period of stressA)Multifocal migrateMultifocal migrate Chorea Chorea MyoclonusMyoclonusFluctuate spontaneouslyFluctuate spontaneously Paraxysmal dykinesias Paraxysmal dykinesias SeizuresSeizuresPresent during sleepPresent during sleep Myoclonus (segmental) Myoclonus (segmental) Periodic movementsPeriodic movements Painfullegs / moving toesPainfullegs / moving toes Other hyperkinesiasOther hyperkinesias SEizuresSEizures
Treatment of tic disordersTreatment of tic disordersDrugDrug Usual Usual PotentialPotential EffectiveEffective
General guidelines for treating General guidelines for treating tardive syndromestardive syndromes• Taper and slowly eliminate causative medications, if clinically Taper and slowly eliminate causative medications, if clinically
possible. Avid sudden cessation of these drugs, which can exacerbate possible. Avid sudden cessation of these drugs, which can exacerbate symptomssymptoms
• If treatment of tardive movements is necessary, the drugs of first If treatment of tardive movements is necessary, the drugs of first choice are the dopamine-depleting drugs reserpine, tetrabenazine, choice are the dopamine-depleting drugs reserpine, tetrabenazine, and a-methylparatyrosine. Monitor for the development of and a-methylparatyrosine. Monitor for the development of depression, hypotension sedation, and parkinsonism.depression, hypotension sedation, and parkinsonism.
• If dopamine-depleting agents are ineffective, consider a trial of If dopamine-depleting agents are ineffective, consider a trial of clozpine or quetiapine.clozpine or quetiapine.
• Dopamine receptor-blocking agents can be used as medications of Dopamine receptor-blocking agents can be used as medications of last resort for patients with tardive syndromes despite the risk of last resort for patients with tardive syndromes despite the risk of worsening the syndrome over the long term.worsening the syndrome over the long term.
• Consider globus pallidus stimulation if pharmacotherapy is Consider globus pallidus stimulation if pharmacotherapy is ineffective.ineffective.
NEUROLEPTIC INDUCED NEUROLEPTIC INDUCED MOVEMENT DISORDERSMOVEMENT DISORDERS
1.1. Acute reactionAcute reaction a. Acute dystoniaa. Acute dystonia b. Acute (Subacute) akathisiab. Acute (Subacute) akathisia2. 2. Toxicity state (over dosage)Toxicity state (over dosage) a. Drug-induced parkinsonisma. Drug-induced parkinsonism3.3. Neuroleptic malignant syndrome (NMS)Neuroleptic malignant syndrome (NMS)4.4. Tardive stbdrinesTardive stbdrines a. Withdrawal emergent syndromea. Withdrawal emergent syndrome b.Classic fardive dyskinesiab.Classic fardive dyskinesia c. Tardive dystoniac. Tardive dystonia d. Tardive akathisiad. Tardive akathisia e. Tardive myoclonuse. Tardive myoclonus f. Tardive tremorf. Tardive tremor g. Tardive ticsg. Tardive tics h. Tardive Choreah. Tardive Chorea i. (?) Tardive parkinsonismi. (?) Tardive parkinsonism
TERMINOLOGY OF THE TARDIVE SYNDROMESTERMINOLOGY OF THE TARDIVE SYNDROMES
• Motor tics (rostrocaudalprogession)Motor tics (rostrocaudalprogession)
• Attention deficit with hyperactivityAttention deficit with hyperactivity
SummarySummary
• Movement disorders are often difficult to define Movement disorders are often difficult to define precisely, but have similar differential diagnoses.precisely, but have similar differential diagnoses.
• They are often a manifestation of a more widespread They are often a manifestation of a more widespread neurological or internal medical problem.neurological or internal medical problem.
• Other than the specific treatments mentioned, most Other than the specific treatments mentioned, most details of therapy are beyond the scope of this course.details of therapy are beyond the scope of this course.
• In some cases treatment includes treatment of In some cases treatment includes treatment of underlying cause e.g. Wilson’s disease underlying cause e.g. Wilson’s disease
Dedicated to my family for Dedicated to my family for making everything worthwhile making everything worthwhile
My sincere thanks to Faculty of Madras Institute of Neurology and Madras Medical
College for giving this opportunity to speak in the CME Program
My sincere thanks to Faculty of Madras Institute of Neurology and Madras Medical
College for giving this opportunity to speak in the CME Program