1. A CLINICAL APPROACH TO
MOVEMENT 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. GLUTAMATE
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TH SUB THALAMIC A
W NUCLEUS
AY G GLUTAMATE
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T GABA
AMA GLOBUS PALLIDUS INTERNA
T G
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NIGRA RETICULATA B
A
BRAIN STEM
PEDUNCULO
PONTINE NUCLEUS

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. THE HIERARCHICAL LEVELS FOR RECOGNIZING THE
VARIOUS DYSKINESIAS
Level A
.Rhythmical Vs. Arrhythmical
.Sustained Vs. Non-sustaine
.Paroxysmal Vs. Continual Vs. Continuous
.Sleep Vs. Awake
Level B
At Rest Vs. with Action

5. THE HIERARCHICAL LEVELS FOR RECOGNIZING THE
VARIOUS 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. Table –2: DIFFERENTIAL DIAGNOSIS OF RHYTHMICAL
AND ARRHYTHMICAL HYPERKINESIAS
RHYTHMICAL VS. ARRHYTHMICAL
Tremor Akathitic movemnts
Resting Athetosis
Postural Ballism
Action Chorea
Intention Dystonia
Dystonia Tremor Hemifacial spasm
Dystonia myorhythmia Hermifacial spasm

7. Tabel –2: DIFFERENTIAL DIAGNOSIS OF RHYTHMICAL
AND ARRHYTHMICAL HYPERKINESIAS
RHYTHMICAL VS. ARRHYTHMICAL
Myoclonus, segmental Arrhythmic myoclonus
Epilepsia partialis continua Stereotypy
Myoclonus, Oscillatory Tics
Moving toes/fingers
Myorhythmia
Periodic movements in sleep
Tardive dyskinesia (tardive stereotypy)

8. TABLE – 3: DIFFERENTIAL DIAGNOSIS OF SUSTANED
HYPERKINESIAS
SUSTAINED CONTRACTIONS VS. NON-SUSTAINED
OR POSTURES CONTRACTION
Rigidity All others
Dystonia
Cculogyric crisis
Paroxysmal dystonia
Dystonic tics
Sandifer’s syndrom
Stiff – person
Neuromyotonia
Congenital torticollis
Orthopedic torticollis

9. TABLE – 4 DIFFERENTIAL DIAGNOSIS OF PAROXYSMAL
AND NON-PAROXYSMAL HYPERKINESIS
PAROXYSMAL 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. TABLE – 5: DIFFERENTIAL DIAGNOSIS OF HYPERKINESIS
THAT ARE PRESENT WHILE ASLEEP OR AWAKE
APPEARS DURING SLEEP VERSISTS DURING SLEEP
DIMINISHERS DURING SLEEP
Hypnogenic Dyskinesias Palatal myoclonus All others
Periodic mvts in sleep Ocular myoclonus
Oculofacinomasticatory
Myorhythmia
Myokymia

11. TABLE – 6 : DIFFERENTIAL DIAGNOSIS OF HYPERKINESIAS
THAT 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. TABLE – 6 : DIFFERENTIAL DIAGNOSIS OF HYPERKINESIAS
THAT 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. TABLE 7: THE LOWEST HIERARCHICAL LEVELS IN
DIFFERENTIATING 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. SPEED : FAST VS SLOW
FASTEST INTERMEDIATE SLOWEST
Myoclonus Chorea Athetosis
Hyperekplexia Ballism moving toes/fingers
Hemifacial spasm Jump stumps Myorhythmia
Tremors Alkathitic
movements
Tradive Streotypy
AMPLITUDE: BALLISTIC VS NOT BALLISTIC
Ballism Chorea and all others
Jumpy stumps would be ballistic, but short
Stump keeps the amptitude relatively small

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. STEP 1 – WHAT ARE THE MOVEMENTS
1. AKINETIC OR DYSKINETIC
• TREMOR
• JERKS Myclonus
Chorea
Tic
• SPASMS Dystonia
Rhythmic / arhythmic
Stereo typed / in consistant
Continous
Action
Paroxysms

17. STEP 2 – IDENTIFY WHAT IS THE OVERALL
SYNDROME
Akinetic rigid syndrome
• Dystonic syndrome
• Choreic syndrome
• Tic syndrome
• Myoclonic syndrome

18. STEP 3 – WHAT IS THE CAUSE ?
Differential diagnosis of various syndrome
See standard text book

19. STEP 4 – ODD DYSKINESIAS
A. ODDTREMOR
• Mid brain tremor
• Task specific tremor
• Neuro pathic tremor
• Dystonic tremor
• Primary orhtostatic tremor

20. STEP 4 – ODD DYSKINESIAS
B. ODD JERKS
1.FOCAL MYOCLONUS
Angio endothelion a s 1 root
• Toe jerks alone
2.CORTICAL MYOCLONUS
Encephalitis
• Jerks of posture
• Action myoclonus
• Stimulus sensitive myoclonus

21. STEP 4 – ODD DYSKINESIAS
B. ODD JERKS
3. GIANT SOMATO SENSORY
Syrinx
Repetitive jerks lower limbs
4.HYPEREXPLEXIA
5.ODD SPASMS
PLMT
Hemidystonia

22. STEP – 5
EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAY
Encephalopathy and lowdensity lesions No infection – Urea cycle defect mitochonrdial o
in MRI 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.

23. STEP – 5
EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAY
Macrocephaly Alexander’s disease metachromatic
leukodystrophy
Muscle weakness and wasting Neuronal Intranuclear inclusion
disease
Vertical supra Nuclear Palsy Niemann pick disease
Gaucher’s Disease
Cherry Red spotin Macula Sialidosis GM & GM2
gangliosidosis
Memann Pick’s disease
Dysmorphic features Mucopolysacridoses
Mucolipodiosis
Investigations for primary
movement disorder

24. STEP – 6
INVESTIGATIONS FOR PRIMARY MOVEMENT
DISORDERS
• Imaging (MRI)
• Exclusion of Wilson <50)
• Genentic testing
• Routine blood wing Biochemistry
• Syphilis

25. STEP – 7 GENERAL PLAN
• Extent of nervous system involvement
• Psychometric evaluation
• EEG (epilepti form discharges)
• ENMG (peripheral neruropathy)
• EMG and VEP

26. STEP – 8
INVESTIGATIONS IN SYMPTOMATIC
MOVEMENT
DISORDERS METABOLIC AND STORAGE
DISORDERS
Metabolic encephalopathies categories and
investigation
Metabolic Storage Disorders: Categories And
Investigation
Degenerative And Systemic Disorders

27. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL
SYNDROMS
• 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. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL
SYNDROMS
SYMPTO, 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. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL
SYNDROMS
SYMPTOMATIC TIC
Neurocanthocytosis
SYMPOTOMATIC 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. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL
SYNDROMS
SYMPT. 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. STEP 9 : ADDITIONAL TEST TO SPECIFIC CLINICAL
SYNDROMS
INVASIVE INVESTIGATIONS
Skin biopsy (Axilla)
• Muscle biopsy
• Peripheral nerve biopsy
Brain biopsy

32. STEP – 10 :
GUIDE LINES FRO MOVEMNET DISORDERS IN CHILDREN /YOUNG
ADULTS
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

40. 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

43. Dedicated to my family for
making everything worthwhile

44. READ not to contradict or confute
Nor to Believe and Take for Granted
but TO WEIGH AND CONSIDER
THANK YOU