Ten step approach to movement disorders

TEN STEP APPROACH TO
MOVEMENT DISORDERS
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

12/06/2010 Diamond jubliee year(1950-2010) 1

MOVEMENT DISORDERS
• 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


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


STEP 2 – IDENTIFY WHAT IS THE
OVERALL SYNDROME

• Akinetic rigid syndrome
Dystonic syndrome
Choreic syndrome
Tic syndrome
Myoclonic syndrome


STEP 3 – WHAT IS THE CAUSE ?

• Differential diagnosis of various syndrome

• See standard- text book


STEP 4 – ODD DYSKINESIAS

A. ODDTREMOR

Mid brain tremor
Task specific tremor
Neuro pathic tremor
Dystonic tremor
Primary orhtostatic tremor



B. ODD JERKS
1.FOCAL MYOCLONUS
• Angio endothelioma- s1- root
Toe jerks alone
2.CORTICAL MYOCLONUS
• Encephalitis
Jerks of posture
Action myoclonus
Stimulus sensitive myoclonus



• B. ODD JERKS
3. GIANT SOMATO SENSORY
• Syrinx
• Repetitive jerks lower limbs
4. HYPEREXPLEXIA
5. ODD SPASMS
• PLMT
• Hemidystonia


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


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


STEP – 6
INVESTIGATIONS FOR PRIMARY
MOVEMENT DISORDERS

Imaging (MRI)
Exclusion of Wilson <50)
Genentic testing
Routine blood wing Biochemistry
Syphilis


STEP – 7 GENERAL PLAN

Extent of nervous system involvement
Psychometric evaluation
EEG (epilepti form discharges)
ENMG (peripheral neruropathy)
EMG and VEP


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


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.



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.



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



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



INVASIVE INVESTIGATIONS
• Skin biopsy (Axilla)
Muscle biopsy
Peripheral nerve biopsy
• Brain biopsy


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


MYOCLONUS IN THE SETTING OF
EPILEPSY

• Generalized Idiopathic Epilepsy
• Progressive Myoclonic Epilepsy
• Neuronal Ceroid Lipofuscinosis
• Mitochondrial Encepalomyopathy
• Sialidosis
• Lafora Body Disease

MYOCLONUS IN THE SETTING OF
EPILEPSY

• Unverricht-Lundborg Disease:”Baltic Myoclonus”
• Miscellaneous Causes of PME
• Action Myoclonus
• Postthpoxic Myoclonus
• Focal Myoclonus
• Palatal Myoclonus
• Epilepsy Partialis Continua
• Segmental Myoclonus.


STARTLE EPILEPSY

• UNILATERAL STARTLE
- HEMITONIC SPASM
- HEMI ATONIC ATTACK
• BILATERAL STARTLE
- GENERALISED TONIC SPASM
• GLOBAL ATONIC ATTACK


TREATMENT OF STARTLE EPILEPSY

• Clobazam
• Clonazepam
• Carbamazepine
• Chlordiazepoxide


ACQUIRED PAROXYSMAL
DYSKINESIAS

• Multiple sclerosis
• Myelopathy
• Vascular or developmental anomalies
• Drug-induced paroxymal dyskinesias
• Cerebral ischemia
• Head trauma
• Cerebral palsy
• Hemiparasis
• Focal seizures


ACQUIRED PAROXYSMAL
DYSKINESIAS

• Encephalitis
• Radiculapathy
• Hypoparathyroidism
• Thyrotoxicosis
• Hypoglycemia
• Psychogenic
• Reflex sympathetic dystrophy.


Seizures & paraxymal dyskinesias

• CPS – Temporal lobe
• Two cases- movement themselves to be
epileptic
• In another two cases- paraxymal dyskinesias
blended with GTCS


KOTAGAL- TEMPORAL LOBE

• Unilateral dystonic posturing with super imposed
choreo-athetosis
• Other anotomical slides
• 1. mesial frontal,
• 2. frontal sagittal,
• 3. fronto temperal.


EPILEPSY & MOVEMENT DISORDERS –
OTHER CONDITIONS

1. REM Sleep behavior disorder
2. PLMS
3. RETT syndrome
4. Drug induced movement disorder
5. Frontal lobe automatism


REM SLEEP BEHAVIOUR
DISORDER

• Idiopathic
• Secondary


PLMS


RETT SYNDROME

• Infantile spasm
• Myocolonic seizures
• Abnormal early development


DRUG-INDUCED MOVENT
DISORDER
• Valproic acid –
parkinsonism, chorea, oculogyris chyris
• Phenytoin – chorea achetosis
• Anti-psychotic drugs – dystonia


FRONTAL LOBE AUTOMATISM
1. Oroalimentary(lip
smacking, chewing, swallowing, licking)
2. facial (distorted facial
expressions, frequently giving the
appearance of fear)
3. Upper extremity
(fumbling, clasping, grabbing movements
of the hands: repetitive
touching, sxratching, or rubbing of
objects)

FRONTAL LOBE AUTOMATISM

4. Lower extremity-ambulatory
(walking, runnig, bicycling movements of
the legs, kicking).
5. Sexual (pelvic
thrusting, masturbation).
6. Vocalization (sounds, words, or
phrases).


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


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


Ten step approach to movement disorders

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