A CLINICAL APPROACH TOMOVEMENT DISORDERS-NEUROLOGIST              By          PERSPECTIVES                Dr. A.V. SRINIVA...
GLUTAMATE                                            CORTEX                          GLUTAMATE                            ...
PREVALENCE OF MOVEMENT DISORDERSEssential tremor       415 (Haerer et al., 1982)Parkinson’s disease    187 (kurland, 195...
THE HIERARCHICAL LEVELS FOR RECOGNIZING THEVARIOUS DYSKINESIASLevel A.Rhythmical Vs. Arrhythmical.Sustained Vs. Non-sust...
THE HIERARCHICAL LEVELS FOR RECOGNIZING THEVARIOUS DYSKINESIAS Level C  Patterned Vs. non-patterned  Speed : Slow vs. fa...
Table –2: DIFFERENTIAL DIAGNOSIS OF RHYTHMICALAND ARRHYTHMICAL HYPERKINESIASRHYTHMICAL VS.       ARRHYTHMICALTremor       ...
Tabel –2: DIFFERENTIAL DIAGNOSIS OF RHYTHMICALAND ARRHYTHMICAL HYPERKINESIASRHYTHMICAL VS.                  ARRHYTHMICALMy...
TABLE – 3:  DIFFERENTIAL DIAGNOSIS OF SUSTANED HYPERKINESIASSUSTAINED CONTRACTIONS   VS.      NON-SUSTAINEDOR POSTURES    ...
TABLE – 4  DIFFERENTIAL DIAGNOSIS OF PAROXYSMALAND NON-PAROXYSMAL HYPERKINESISPAROXYSMAL VS . CONTINUAL          VS. CONTI...
TABLE – 5:   DIFFERENTIAL DIAGNOSIS OF HYPERKINESIS THAT ARE PRESENT WHILE ASLEEP OR AWAKE      APPEARS DURING SLEEP VERSI...
TABLE – 6 :  DIFFERENTIAL DIAGNOSIS OF HYPERKINESIASTHAT ARE PRESENT AT REST OR WITH ACTION  At rest only (disappears with...
TABLE – 6 :  DIFFERENTIAL DIAGNOSIS OF HYPERKINESIASTHAT ARE PRESENT AT REST OR WITH ACTION  At rest and continues with ac...
TABLE 7:    THE LOWEST HIERARCHICAL LEVELS INDIFFERENTIATING THE HYPERKINESIS         PATTERNED                   NON-PATT...
SPEED : FAST VS SLOW FASTEST            INTERMEDIATE           SLOWEST Myoclonus          Chorea                 Athetosis...
Step 1      What are the Movements ?Step 2      Identify the overall syndromeStep 3      Decide the disease/Syndrome pa...
STEP 1 – WHAT ARE THE MOVEMENTS1. AKINETIC OR DYSKINETIC• TREMOR• JERKS             Myclonus                    Chorea    ...
STEP 2 – IDENTIFY WHAT IS THE OVERALL           SYNDROMEAkinetic rigid syndrome• Dystonic syndrome• Choreic syndrome• Tic...
STEP 3 – WHAT IS THE CAUSE ?Differential diagnosis of various syndromeSee standard text book
STEP 4 – ODD DYSKINESIASA.    ODDTREMOR• Mid brain tremor• Task specific tremor• Neuro pathic tremor• Dystonic tremor• Pri...
STEP 4 – ODD DYSKINESIASB.    ODD JERKS1.FOCAL MYOCLONUSAngio endothelion a s 1 root• Toe jerks alone2.CORTICAL MYOCLONUS...
STEP 4 – ODD DYSKINESIASB. ODD JERKS3. GIANT SOMATO SENSORYSyrinxRepetitive jerks lower limbs4.HYPEREXPLEXIA5.ODD SPASM...
STEP – 5    EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYEncephalopathy and lowdensity lesions   No infection – Urea c...
STEP – 5EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYMacrocephaly                   Alexander’s disease metachromatic ...
STEP – 6INVESTIGATIONS FOR PRIMARY MOVEMENTDISORDERS    •   Imaging (MRI)    •   Exclusion of Wilson <50)    •   Genentic ...
STEP – 7 GENERAL PLAN• Extent of nervous system involvement• Psychometric evaluation• EEG (epilepti form discharges)• ENMG...
STEP – 8INVESTIGATIONS IN SYMPTOMATICMOVEMENTDISORDERS METABOLIC AND STORAGEDISORDERSMetabolic encephalopathies categorie...
STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMS • Smptomatic parkinsonism • MSA (Anal or uretheral EMG) • MRI – Low ...
STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMSSYMPTO, CHOREANeuroacanthocystosis – peripheral smear /CK• T3,T4 – T...
STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMSSYMPTOMATIC TIC   NeurocanthocytosisSYMPOTOMATIC MYOCLONUSEstablish...
STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL SYNDROMSSYMPT. DYSTONIA (RARE)• Niemann Pick type C – Bone marow Sea blue hi...
STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMSINVASIVE INVESTIGATIONSSkin biopsy (Axilla)• Muscle biopsy• Peripher...
STEP – 10 :GUIDE LINES FRO MOVEMNET DISORDERS IN CHILDREN /YOUNGADULTSCHILDHOOD NEURODEGENERATIVE DISEASES THAT MAY PRESE...
CONCLUSIONThe composition in this talk has been for the author a long struggle of escape, and so must the reading of it b...
Dedicated to my family formaking everything worthwhile
READ not to contradict or confuteNor to Believe and Take for Grantedbut TO WEIGH AND CONSIDERTHANK YOU
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
A clinical approach to movement disorders neurologist perspectives
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A clinical approach to movement disorders neurologist perspectives

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A clinical approach to movement disorders neurologist perspectives

  1. 1. A CLINICAL APPROACH TOMOVEMENT DISORDERS-NEUROLOGIST By PERSPECTIVES Dr. A.V. SRINIVASAN, M.D,D.M,PhD,DSc,FRCP(London),F.I.A.N,F.A.A.N. EMERITUS PROFESSOR THE TAMIL NADU DR MGR MEDICAL UNIVERSITY CHENNAI Former Professor and HEAD, INSTITUTE OF NEUROLOGY MADRAS MEDICAL COLLEGE
  2. 2. GLUTAMATE CORTEX GLUTAMATE GLUTAMATE + + PUTAMENSUBSTANTIA NIGRA DOPA PARS COMPACTA D2E D1 nk Sub P DOPA GABA - D I GA THALAMUS GLOBUS PALLIDUS IN RE BA EXTERNA DI CT RE PA SU GABA G TH B. CT WA A PA Y P B TH SUB THALAMIC A W NUCLEUS AY G GLUTAMATE L + U T GABA AMA GLOBUS PALLIDUS INTERNA T G E A NIGRA RETICULATA B A BRAIN STEM PEDUNCULO PONTINE NUCLEUS
  3. 3. PREVALENCE OF MOVEMENT DISORDERSEssential tremor 415 (Haerer et al., 1982)Parkinson’s disease 187 (kurland, 1958)Tourette’s syndrome 29-1052 (Caine et al., 1988)Dystonia 33 (Nutt et al., 1988)Hereditary ataxia 6 (Schoenberg, 1978)Huntington’s disease 2-12 (Harper, 1992)Wilson’s disease 3 (Reilly and Hutchinson, 1993)Progressive supra- nuclear palsy 2 (Golbe, 1994)  Rate are given per 100,000 population. For Parkinson’s disease, the rate is 347 per 100,000 for ages over 40 years (Schoemberg et al, 1985).
  4. 4. THE HIERARCHICAL LEVELS FOR RECOGNIZING THEVARIOUS DYSKINESIASLevel A.Rhythmical Vs. Arrhythmical.Sustained Vs. Non-sustaine.Paroxysmal Vs. Continual Vs. Continuous.Sleep Vs. AwakeLevel BAt Rest Vs. with Action
  5. 5. THE HIERARCHICAL LEVELS FOR RECOGNIZING THEVARIOUS DYSKINESIAS Level C  Patterned Vs. non-patterned  Speed : Slow vs. fast  Amplitude : Ballistic vs. not ballistic  Force : Powerful (painful) vs. easy-to-overcome  Suppressibility  Vocalizations  Self mutilation  Complex movements  Combinations of varieties of movements  Sensory component  Continual means over and over again: continuous means unbroken.
  6. 6. Table –2: DIFFERENTIAL DIAGNOSIS OF RHYTHMICALAND ARRHYTHMICAL HYPERKINESIASRHYTHMICAL VS. ARRHYTHMICALTremor Akathitic movemntsResting AthetosisPostural BallismAction ChoreaIntention DystoniaDystonia Tremor Hemifacial spasmDystonia myorhythmia Hermifacial spasm
  7. 7. Tabel –2: DIFFERENTIAL DIAGNOSIS OF RHYTHMICALAND ARRHYTHMICAL HYPERKINESIASRHYTHMICAL VS. ARRHYTHMICALMyoclonus, segmental Arrhythmic myoclonusEpilepsia partialis continua StereotypyMyoclonus, Oscillatory TicsMoving toes/fingersMyorhythmiaPeriodic movements in sleepTardive dyskinesia (tardive stereotypy)
  8. 8. TABLE – 3: DIFFERENTIAL DIAGNOSIS OF SUSTANED HYPERKINESIASSUSTAINED CONTRACTIONS VS. NON-SUSTAINEDOR POSTURES CONTRACTIONRigidity All othersDystoniaCculogyric crisisParoxysmal dystoniaDystonic ticsSandifer’s syndromStiff – personNeuromyotoniaCongenital torticollisOrthopedic torticollis
  9. 9. TABLE – 4 DIFFERENTIAL DIAGNOSIS OF PAROXYSMALAND NON-PAROXYSMAL HYPERKINESISPAROXYSMAL VS . CONTINUAL VS. CONTINUOUS Tics Ballism Athetosis PKC Chorea Tremors PDC Chorea Tremors PDC Dystonic Dystonic postures movemnts Paroxysmal ataxia Myoclonus, Myoclonous, arrhythmic rhythimic Paroxysmal Stereotypy Tardive tremor Stereotypy Hypnogenic Myokymia dystonia Tic status Jumpy stums
  10. 10. TABLE – 5: DIFFERENTIAL DIAGNOSIS OF HYPERKINESIS THAT ARE PRESENT WHILE ASLEEP OR AWAKE APPEARS DURING SLEEP VERSISTS DURING SLEEP DIMINISHERS DURING SLEEPHypnogenic Dyskinesias Palatal myoclonus All othersPeriodic mvts in sleep Ocular myoclonus Oculofacinomasticatory Myorhythmia Myokymia
  11. 11. TABLE – 6 : DIFFERENTIAL DIAGNOSIS OF HYPERKINESIASTHAT ARE PRESENT AT REST OR WITH ACTION At rest only (disappears with action)  Akathitic movemnts  Paradoxical dystonia*  Resting tremor  Restless legs  Orthostaic tremor* With action only  Tremor, postural, action, intention  Action dystonia  Action myoclonus
  12. 12. TABLE – 6 : DIFFERENTIAL DIAGNOSIS OF HYPERKINESIASTHAT ARE PRESENT AT REST OR WITH ACTION At rest and continues with action  Athetosis  Ballism  Chorea  Dystonia at rest*  Jumpy stumps  Moving toes/fingers  Myoclonus at rest*  Myokymia  Pseudodystonias*  Tics
  13. 13. TABLE 7: THE LOWEST HIERARCHICAL LEVELS INDIFFERENTIATING THE HYPERKINESIS PATTERNED NON-PATTERNED (I.E. SAME MUSCLE GROUPS) Dystonia All others Hemifacial spasm Moving toes/fingers Segmental myoclonus Myorhythmia Myokymia Tardive stereotypy Temor
  14. 14. SPEED : FAST VS SLOW FASTEST INTERMEDIATE SLOWEST Myoclonus Chorea Athetosis Hyperekplexia Ballism moving toes/fingers Hemifacial spasm Jump stumps Myorhythmia Tremors Alkathitic movements Tradive StreotypyAMPLITUDE: BALLISTIC VS NOT BALLISTIC Ballism Chorea and all others Jumpy stumps would be ballistic, but short Stump keeps the amptitude relatively small
  15. 15. Step 1 What are the Movements ?Step 2 Identify the overall syndromeStep 3 Decide the disease/Syndrome pattern from differential diagnosisStep 4 If not, is it Odd dyskinesias?Step 5 Emphasis on clinical clues and diagnostic pathwayStep 6 If primary movement disorder – Principle investigationsStep 7 General PlanStep 8 Investigations for Symptomatic Movement DisordersStep 9 Additional tests in specific clinical syndromesStep 10 Guidelines for Movement Disorders in children/Young Adults
  16. 16. STEP 1 – WHAT ARE THE MOVEMENTS1. AKINETIC OR DYSKINETIC• TREMOR• JERKS Myclonus Chorea Tic• SPASMS Dystonia Rhythmic / arhythmic Stereo typed / in consistant Continous Action Paroxysms
  17. 17. STEP 2 – IDENTIFY WHAT IS THE OVERALL SYNDROMEAkinetic rigid syndrome• Dystonic syndrome• Choreic syndrome• Tic syndrome• Myoclonic syndrome
  18. 18. STEP 3 – WHAT IS THE CAUSE ?Differential diagnosis of various syndromeSee standard text book
  19. 19. STEP 4 – ODD DYSKINESIASA. ODDTREMOR• Mid brain tremor• Task specific tremor• Neuro pathic tremor• Dystonic tremor• Primary orhtostatic tremor
  20. 20. STEP 4 – ODD DYSKINESIASB. ODD JERKS1.FOCAL MYOCLONUSAngio endothelion a s 1 root• Toe jerks alone2.CORTICAL MYOCLONUSEncephalitis• Jerks of posture• Action myoclonus• Stimulus sensitive myoclonus
  21. 21. STEP 4 – ODD DYSKINESIASB. ODD JERKS3. GIANT SOMATO SENSORYSyrinxRepetitive jerks lower limbs4.HYPEREXPLEXIA5.ODD SPASMSPLMTHemidystonia
  22. 22. STEP – 5 EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYEncephalopathy and lowdensity lesions No infection – Urea cycle defect mitochonrdial oin MRI pyruvate disorder, organic acid disorderOrganomegaly Wilson’s Gaucher’s Niemann Pick disease GalactosaemiaPeripheral Neuropathy Adreno myelo – leucodystrophy GM2 Gangliosidosis Krabbe’s disease Meta Chromatic leukodystrophy Gaucher’s disease Mucolipidosis Mitochondrial disordersMyoclonus and epilepsy Lafora body disease ceroid lipo fuscinosis GM2 Gangliosidosis Gaucher’s disease Polychstic lipomembranous asteodysplasia Mitochondrial disease.
  23. 23. STEP – 5EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYMacrocephaly Alexander’s disease metachromatic leukodystrophyMuscle weakness and wasting Neuronal Intranuclear inclusion diseaseVertical supra Nuclear Palsy Niemann pick disease Gaucher’s DiseaseCherry Red spotin Macula Sialidosis GM & GM2 gangliosidosis Memann Pick’s diseaseDysmorphic features Mucopolysacridoses Mucolipodiosis Investigations for primary movement disorder
  24. 24. STEP – 6INVESTIGATIONS FOR PRIMARY MOVEMENTDISORDERS • Imaging (MRI) • Exclusion of Wilson <50) • Genentic testing • Routine blood wing Biochemistry • Syphilis
  25. 25. STEP – 7 GENERAL PLAN• Extent of nervous system involvement• Psychometric evaluation• EEG (epilepti form discharges)• ENMG (peripheral neruropathy)• EMG and VEP
  26. 26. STEP – 8INVESTIGATIONS IN SYMPTOMATICMOVEMENTDISORDERS METABOLIC AND STORAGEDISORDERSMetabolic encephalopathies categories and investigationMetabolic Storage Disorders: Categories And InvestigationDegenerative And Systemic Disorders
  27. 27. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMS • Smptomatic parkinsonism • MSA (Anal or uretheral EMG) • MRI – Low density in GB/Putamen MSA / PSP SYMPTOMAIC TREMORS  T3T4 – Thyrotoxicosis  Peripheral Neuropathy Paraprotenemias  Hg. Poisoning  Unilateral tremors – opp. Basal ganglia, Thalamus, Sub Thalamic body of Luys.
  28. 28. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMSSYMPTO, CHOREANeuroacanthocystosis – peripheral smear /CK• T3,T4 – Thyrotoxicosis• Polycythemia rubravira• Calcium and magnesium metabolism• Hyponatremia• Auto immune disorders• Syden ham’s chorea • SLE • APLS • Struct, lesion of Sub Thalamic Body of luy.
  29. 29. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMSSYMPTOMATIC TIC NeurocanthocytosisSYMPOTOMATIC MYOCLONUSEstablish the site of origin n the nervous system by electrophysiology• Lafora body disease• Neuronal ceroid lipofuscinosis• Sialidosis• Mitochondrial disordersUnverricht Lundborg Disease
  30. 30. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL SYNDROMSSYMPT. DYSTONIA (RARE)• Niemann Pick type C – Bone marow Sea blue histiocytes• DRD• Sandifer syndrome• Atalanto axial subluxation (fixed painful torticollis)SYNDROME WITH CONTINOUS MUSCLE FIBRE ACTIVITYDetailed ENMG study• Episodic or paroxysmal movement disorders• Video telemetry EEG / distinquish from epilepsy• Paroxysmal spasm – M.S.• Intermitant ataxias – Amino acid disorders
  31. 31. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICALSYNDROMSINVASIVE INVESTIGATIONSSkin biopsy (Axilla)• Muscle biopsy• Peripheral nerve biopsyBrain biopsy
  32. 32. STEP – 10 :GUIDE LINES FRO MOVEMNET DISORDERS IN CHILDREN /YOUNGADULTSCHILDHOOD NEURODEGENERATIVE DISEASES THAT MAY PRESENT IN YOUNG ADULT LIFE WITH A MOVEMENT DISORDERSPECIAL STUDIES TO BE CONSIDERED IN CHILDREN OR YOUN ADULTS WITH A SYMPTOMATIC MOVEMENT DISORDER
  33. 33. CONCLUSIONThe composition in this talk has been for the author a long struggle of escape, and so must the reading of it be for most readers if the author’s assault upon them is to be successful - A struggle of escape from habitual modes of thought and expression. The ideas which are here expressed so laboriously and extremely simple and should be obvious. The difficulty lies not in the new ideas, but in escaping from the old ones, which ramify, for those brought up as most of us have been, into every corner of our minds
  34. 34. Dedicated to my family formaking everything worthwhile
  35. 35. READ not to contradict or confuteNor to Believe and Take for Grantedbut TO WEIGH AND CONSIDERTHANK YOU

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