Ten step approach to movement disorders

Diamond jubliee year(1950-2010)Diamond jubliee year(1950-2010) 1112/06/201012/06/2010

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

TEN STEP APPROACH TO MOVEMENT DISORDERS

12/06/201012/06/2010 Diamond jubliee year(1950-2010)Diamond jubliee year(1950-2010) 22

MOVEMENT DISORDERSMOVEMENT DISORDERS

• Step 1Step 1 What are the Movements ?What are the Movements ?• Step 2Step 2 Identify the overall syndromeIdentify the overall syndrome• Step 3Step 3 Decide the disease/Syndrome pattern from Decide the disease/Syndrome pattern from

differential differential diagnosisdiagnosis• Step 4Step 4 If not, is it Odd dyskinesias?If not, is it Odd dyskinesias?• Step 5Step 5 Emphasis on clinical clues and diagnostic pathwayEmphasis on clinical clues and diagnostic pathway• Step 6Step 6 If primary movement disorder – Principle If primary movement disorder – Principle

investigationsinvestigations• Step 7Step 7 General PlanGeneral Plan• Step 8Step 8 Investigations for Symptomatic Movement DisordersInvestigations for Symptomatic Movement Disorders• Step 9Step 9 Additional tests in specific clinical syndromesAdditional tests in specific clinical syndromes• Step 10Step 10Guidelines for Movement Disorders in Guidelines for Movement Disorders in

children/Young Adultschildren/Young Adults

STEP 1 – WHAT ARE THE STEP 1 – WHAT ARE THE MOVEMENTSMOVEMENTS

1. AKINETIC OR DYSKINETIC TREMOR JERKS Myclonus Chorea Tic SPASMS Dystonia Rhythmic / arhythmic

Stereo typed / in consistant Continous Action Paroxysms

STEP 2 – IDENTIFY WHAT IS THE STEP 2 – IDENTIFY WHAT IS THE OVERALL SYNDROMEOVERALL SYNDROME

• Akinetic rigid syndromeAkinetic rigid syndrome Dystonic syndromeDystonic syndrome Choreic syndromeChoreic syndrome Tic syndromeTic syndrome Myoclonic syndromeMyoclonic syndrome

STEP 3 – WHAT IS THE CAUSE ?STEP 3 – WHAT IS THE CAUSE ?

• Differential diagnosis of various Differential diagnosis of various syndromesyndrome

• See standard- text bookSee standard- text book

STEP 4 – ODD DYSKINESIASSTEP 4 – ODD DYSKINESIAS

A.A. ODDTREMORODDTREMOR

Mid brain tremorMid brain tremor Task specific tremorTask specific tremor Neuro pathic tremorNeuro pathic tremor Dystonic tremorDystonic tremor Primary orhtostatic tremorPrimary orhtostatic tremor

B. B. ODD JERKSODD JERKS11..FOCAL MYOCLONUSFOCAL MYOCLONUS• Angio endothelioma- s1- rootAngio endothelioma- s1- root Toe jerks aloneToe jerks alone22..CORTICAL MYOCLONUSCORTICAL MYOCLONUS• EncephalitisEncephalitis Jerks of postureJerks of posture Action myoclonusAction myoclonus Stimulus sensitive myoclonusStimulus sensitive myoclonus

• B. B. ODD JERKSODD JERKS3.3. GIANT SOMATO SENSORYGIANT SOMATO SENSORY

• Syrinx Syrinx • Repetitive jerks lower limbsRepetitive jerks lower limbs4.4. HYPEREXPLEXIAHYPEREXPLEXIA5.5.ODD SPASMSODD SPASMS• PLMTPLMT• HemidystoniaHemidystonia

STEP – 5STEP – 5EMPHASIS ON CLINICAL CLUES EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYAND DIAGNOSTIC PATHWAY

Encephalopathy and lowdensity lesions in MRI

No infection – Urea cycle defect mitochonrdial or pyruvate disorder, organic acid disorder

Organomegaly Wilson’s Gaucher’s Niemann Pick disease Galactosaemia

Peripheral Neuropathy Adreno myelo – leucodystrophy GM2 Gangliosidosis Krabbe’s disease

Meta Chromatic leukodystrophy Gaucher’s disease Mucolipidosis Mitochondrial disorders Myoclonus and epilepsy Lafora body disease ceroid lipo fuscinosis GM2

Gangliosidosis Gaucher’s disease Polychstic lipomembranous asteodysplasia Mitochondrial disease.

STEP – 5STEP – 5EMPHASIS ON CLINICAL CLUES AND EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYDIAGNOSTIC PATHWAY

Macrocephaly Alexander’s disease metachromaticleukodystrophy

Muscle weakness and wasting Neuronal Intranuclear inclusiondisease

Vertical supra Nuclear Palsy Niemann pick diseaseGaucher’s Disease

Cherry Red spotin Macula Sialidosis GM & GM2 gangliosidosisMemann Pick’s disease

Dysmorphic features Mucopolysacridoses MucolipodiosisInvestigations for primary movementdisorder

STEP – 6STEP – 6INVESTIGATIONS FOR PRIMARY INVESTIGATIONS FOR PRIMARY MOVEMENT DISORDERSMOVEMENT DISORDERS

Imaging (MRI)Imaging (MRI) Exclusion of Wilson <50)Exclusion of Wilson <50) Genentic testingGenentic testing Routine blood wing BiochemistryRoutine blood wing Biochemistry SyphilisSyphilis

STEP – 7 GENERAL PLANSTEP – 7 GENERAL PLAN

Extent of nervous system Extent of nervous system involvementinvolvement

Psychometric evaluationPsychometric evaluation EEG (epilepti form discharges)EEG (epilepti form discharges) ENMG (peripheral neruropathy)ENMG (peripheral neruropathy) EMG and VEPEMG and VEP

STEP – 8STEP – 8INVESTIGATIONS IN SYMPTOMATICINVESTIGATIONS IN SYMPTOMATICMOVEMENTMOVEMENTDISORDERS METABOLIC AND STORAGE DISORDERS METABOLIC AND STORAGE DISORDERSDISORDERS

• Metabolic encephalopathies categories Metabolic encephalopathies categories and investigationand investigation

• Metabolic Storage Disorders: Categories Metabolic Storage Disorders: Categories And InvestigationAnd Investigation

• Degenerative And Systemic DisordersDegenerative And Systemic Disorders

STEP 9 : ADDITIONAL TEST TO STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL SYNDROMSSPECIFIC CLINICAL SYNDROMS

Smptomatic parkinsonismSmptomatic parkinsonism MSA (Anal or uretheral EMG)MSA (Anal or uretheral EMG) MRI – Low density in GB/Putamen MSA / PSPMRI – Low density in GB/Putamen MSA / PSP

SYMPTOMAIC TREMORSSYMPTOMAIC TREMORS• T3T4 – ThyrotoxicosisT3T4 – Thyrotoxicosis• Peripheral Neuropathy ParaprotenemiasPeripheral Neuropathy Paraprotenemias• Hg. PoisoningHg. Poisoning• Unilateral tremors – opp. Basal ganglia, Unilateral tremors – opp. Basal ganglia,

Thalamus, Thalamus, Sub Thalamic body of Luys.Sub Thalamic body of Luys.

SYMPTO, CHOREASYMPTO, CHOREA• Neuroacanthocystosis – peripheral smear /CKNeuroacanthocystosis – peripheral smear /CK TT33,T,T44 – Thyrotoxicosis – Thyrotoxicosis Polycythemia rubraviraPolycythemia rubravira Calcium and magnesium metabolismCalcium and magnesium metabolism HyponatremiaHyponatremia Auto immune disordersAuto immune disorders Syden ham’s choreaSyden ham’s chorea

SLESLE APLSAPLS Struct, lesion of Sub Thalamic Body of luy.Struct, lesion of Sub Thalamic Body of luy.

SYMPTOMATIC TICSYMPTOMATIC TIC– NeurocanthocytosisNeurocanthocytosis

SYMPOTOMATICSYMPOTOMATIC MYOCLONUS MYOCLONUS

• Establish the site of origin n the nervous system Establish the site of origin n the nervous system by electrophysiologyby electrophysiology

Lafora body diseaseLafora body disease Neuronal ceroid lipofuscinosisNeuronal ceroid lipofuscinosis SialidosisSialidosis Mitochondrial disordersMitochondrial disorders• Unverricht Lundborg DiseaseUnverricht Lundborg Disease

SYMPT. DYSTONIA (RARESYMPT. DYSTONIA (RARE)) Niemann Pick type C – Bone marow Sea blue histiocytesNiemann Pick type C – Bone marow Sea blue histiocytes DRDDRD Sandifer syndromeSandifer syndrome Atalanto axial subluxation (fixed painful torticollis)Atalanto axial subluxation (fixed painful torticollis)SYNDROME WITH CONTINOUS MUSCLE FIBRE ACTIVITYSYNDROME WITH CONTINOUS MUSCLE FIBRE ACTIVITY• Detailed ENMG studyDetailed ENMG study Episodic or paroxysmal movement disordersEpisodic or paroxysmal movement disorders Video telemetry EEG / distinquish from epilepsyVideo telemetry EEG / distinquish from epilepsy Paroxysmal spasm – M.S.Paroxysmal spasm – M.S. Intermitant ataxias – Amino acid disordersIntermitant ataxias – Amino acid disorders

INVASIVE INVESTIGATIONSINVASIVE INVESTIGATIONS

• Skin biopsy (Axilla)Skin biopsy (Axilla) Muscle biopsyMuscle biopsy Peripheral nerve biopsyPeripheral nerve biopsy

• Brain biopsyBrain biopsy

STEP – 10 :STEP – 10 :GUIDE LINES FRO MOVEMNET DISORDERS IN GUIDE LINES FRO MOVEMNET DISORDERS IN CHILDREN /YOUNG ADULTSCHILDREN /YOUNG ADULTS

• CHILDHOOD NEURODEGENERATIVE CHILDHOOD NEURODEGENERATIVE DISEASES THAT MAY PRESENT IN YOUNG DISEASES THAT MAY PRESENT IN YOUNG ADULT LIFE WITH A MOVEMENT DISORDERADULT LIFE WITH A MOVEMENT DISORDER

• SPECIAL STUDIES TO BE CONSIDERED IN SPECIAL STUDIES TO BE CONSIDERED IN CHILDREN OR YOUN ADULTS WITH A CHILDREN OR YOUN ADULTS WITH A SYMPTOMATIC MOVEMENT DISORDERSYMPTOMATIC MOVEMENT DISORDER

MYOCLONUS IN THE SETTING OF MYOCLONUS IN THE SETTING OF EPILEPSYEPILEPSY

• Generalized Idiopathic EpilepsyGeneralized Idiopathic Epilepsy

• Progressive Myoclonic EpilepsyProgressive Myoclonic Epilepsy

• Neuronal Ceroid LipofuscinosisNeuronal Ceroid Lipofuscinosis

• Mitochondrial EncepalomyopathyMitochondrial Encepalomyopathy

• SialidosisSialidosis

• Lafora Body DiseaseLafora Body Disease

MYOCLONUS IN THE SETTING OF MYOCLONUS IN THE SETTING OF EPILEPSYEPILEPSY

• Unverricht-Lundborg Disease:”Baltic Unverricht-Lundborg Disease:”Baltic Myoclonus”Myoclonus”

• Miscellaneous Causes of PMEMiscellaneous Causes of PME

• Action MyoclonusAction Myoclonus

• Postthpoxic MyoclonusPostthpoxic Myoclonus

• Focal MyoclonusFocal Myoclonus

• Palatal MyoclonusPalatal Myoclonus

• Epilepsy Partialis ContinuaEpilepsy Partialis Continua

• Segmental Myoclonus.Segmental Myoclonus.

STARTLE EPILEPSYSTARTLE EPILEPSY

• UNILATERAL STARTLE UNILATERAL STARTLE

- HEMITONIC SPASM- HEMITONIC SPASM

- HEMI ATONIC ATTACK- HEMI ATONIC ATTACK

• BILATERAL STARTLEBILATERAL STARTLE

- GENERALISED TONIC SPASM- GENERALISED TONIC SPASM

• GLOBAL ATONIC ATTACKGLOBAL ATONIC ATTACK

TREATMENT OF STARTLE EPILEPSYTREATMENT OF STARTLE EPILEPSY

• ClobazamClobazam

• ClonazepamClonazepam

• CarbamazepineCarbamazepine

• ChlordiazepoxideChlordiazepoxide

ACQUIRED PAROXYSMAL ACQUIRED PAROXYSMAL DYSKINESIASDYSKINESIAS

• Multiple sclerosisMultiple sclerosis• MyelopathyMyelopathy• Vascular or developmental anomaliesVascular or developmental anomalies• Drug-induced paroxymal dyskinesiasDrug-induced paroxymal dyskinesias• Cerebral ischemiaCerebral ischemia• Head traumaHead trauma• Cerebral palsyCerebral palsy• HemiparasisHemiparasis• Focal seizuresFocal seizures

ACQUIRED PAROXYSMAL ACQUIRED PAROXYSMAL DYSKINESIASDYSKINESIAS

• EncephalitisEncephalitis

• RadiculapathyRadiculapathy

• HypoparathyroidismHypoparathyroidism

• ThyrotoxicosisThyrotoxicosis

• HypoglycemiaHypoglycemia

• PsychogenicPsychogenic

• Reflex sympathetic dystrophy.Reflex sympathetic dystrophy.

Seizures & paraxymal Seizures & paraxymal dyskinesiasdyskinesias

• CPS – Temporal lobeCPS – Temporal lobe

• Two cases- movement themselves to be Two cases- movement themselves to be epilepticepileptic

• In another two cases- paraxymal In another two cases- paraxymal dyskinesias blended with GTCSdyskinesias blended with GTCS

KOTAGAL- TEMPORAL LOBEKOTAGAL- TEMPORAL LOBE

• Unilateral dystonic posturing with super Unilateral dystonic posturing with super imposed choreo-athetosisimposed choreo-athetosis

• Other anotomical slides Other anotomical slides

• 1. mesial frontal,1. mesial frontal,

• 2. frontal sagittal, 2. frontal sagittal,

• 3. fronto temperal.3. fronto temperal.

1. REM Sleep behavior disorder 1. REM Sleep behavior disorder

2. PLMS2. PLMS

3. RETT syndrome3. RETT syndrome

4. Drug induced movement disorder4. Drug induced movement disorder

5. Frontal lobe automatism5. Frontal lobe automatism

EPILEPSY & MOVEMENT EPILEPSY & MOVEMENT DISORDERS – OTHER CONDITIONSDISORDERS – OTHER CONDITIONS

REM SLEEP BEHAVIOUR REM SLEEP BEHAVIOUR DISORDERDISORDER

• IdiopathicIdiopathic

• SecondarySecondary

PLMSPLMS

RETT SYNDROMERETT SYNDROME

• Infantile spasmInfantile spasm

• Myocolonic seizuresMyocolonic seizures

• Abnormal early developmentAbnormal early development

DRUG-INDUCED MOVENT DRUG-INDUCED MOVENT DISORDERDISORDER

• Valproic acid – parkinsonism, chorea, Valproic acid – parkinsonism, chorea, oculogyris chyrisoculogyris chyris

• Phenytoin – chorea achetosisPhenytoin – chorea achetosis

• Anti-psychotic drugs – dystoniaAnti-psychotic drugs – dystonia

FRONTAL LOBE FRONTAL LOBE AUTOMATISMAUTOMATISM 1. Oroalimentary(lip smacking, 1. Oroalimentary(lip smacking,

chewing, swallowing, licking)chewing, swallowing, licking) 2. facial (distorted facial 2. facial (distorted facial

expressions, frequently giving the expressions, frequently giving the appearance of fear)appearance of fear)

3. Upper extremity (fumbling, 3. Upper extremity (fumbling, clasping, grabbing movements of the clasping, grabbing movements of the hands: repetitive touching, hands: repetitive touching, sxratching, or rubbing of objects)sxratching, or rubbing of objects)

4. Lower extremity-ambulatory 4. Lower extremity-ambulatory (walking, runnig, bicycling (walking, runnig, bicycling movements of the legs, kicking).movements of the legs, kicking).

5. Sexual (pelvic thrusting, 5. Sexual (pelvic thrusting, masturbation).masturbation).

6. Vocalization (sounds, words, or 6. Vocalization (sounds, words, or phrases).phrases).

FRONTAL LOBE FRONTAL LOBE AUTOMATISMAUTOMATISM

CONCLUSIONCONCLUSION

Epileptic seizures presenting as motor Epileptic seizures presenting as motor phenomena without concomitant conscious phenomena without concomitant conscious change may be confused with one the paroxymal change may be confused with one the paroxymal movement disorders. Conversely, the attack of movement disorders. Conversely, the attack of paroxymal movement disorders maybe thought paroxymal movement disorders maybe thought to be epileptic due to a number of factors, to be epileptic due to a number of factors, including its sudden, unpredictable, and transient including its sudden, unpredictable, and transient nature, its responsie to anti convulsants, and the nature, its responsie to anti convulsants, and the premonitary sensations preceding attacks. The premonitary sensations preceding attacks. The distinction between epilepsy and movement distinction between epilepsy and movement disorders is further confused by the reports that disorders is further confused by the reports that these two conditions frequently occur in the same these two conditions frequently occur in the same families or even in the same patients. Recent--families or even in the same patients. Recent--

(PTO)(PTO)

CONCLUSIONCONCLUSION

--studies shows that a few epilepsy and --studies shows that a few epilepsy and paroxymal movement disorders are paroxymal movement disorders are “channelopathies” , indicating that they “channelopathies” , indicating that they may share some common pathophysiology may share some common pathophysiology and a possible “over lap”. A good quality of and a possible “over lap”. A good quality of history, a trail to reproduce the motor history, a trail to reproduce the motor phnomena, the application of video-EEG, phnomena, the application of video-EEG, polysomnography, and other polysomnography, and other electrophysiological recordings, together electrophysiological recordings, together with regular follow-up are important for with regular follow-up are important for differentiating these two conditions.differentiating these two conditions.

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