This document discusses recent advances in the management of extrapyramidal (basal ganglia) disorders. It defines movement disorders and lists various conditions such as Parkinsonism, dystonia, tremor, tics, chorea, tardive syndrome, and myoclonus. It provides details on classification, causes, treatment of these disorders and differential diagnosis of tics. Guidelines for treating tardive syndromes are also mentioned.
Recent Advances in the Management of Extrapyramidal (Basal Ganglia) Disorders
1. RECENT ADVANCES IN THE
MANGEMENT OF EXTRA PYRAMIDAL-
(BASAL GANGLIA) 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
17th MAY 2009
2. Sir William Osler
To study the phenomenon of disease
without books is to sail an uncharted sea,
while to study books, without patients is
not to go to sea at all.
Sir William Osler Aphorisms
3. DEFINITION
Movement disorders can be defined as
neurologic syndromes in which there is
either an excess of movement or paucity of
voluntary and automatic movements,
unrelated to weakness or spasticity.
7. DEDICATED TO PROF
C.D.MARSDEN- A GENIUS IN
MOVEMENT DISORDERS
â˘P- PARKINSONISM
â˘R- RESTLESS LEG SYNDROME
â˘O- OROFACIAL DYSKINESIA
â˘F- FIBRILLATION AND FASCICULATIONS
⢠C âCHOREA 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
8. CHOREA
ďśRandom, quick ďśHuntington disease
unsustained ďś Neurocanthocytosis
purposeless
movements that have
ďś Postinfectious
an unpredictable chorea
flowing pattern ďś Drug-induced chorea
ďśVascular chorea
ďśAutoimmune chorea
ďśChorea gravidarum
9. Ballismus, Chorea, Athotosis
and Dystonia
These should NOT be thought of as
separate entities amenable to specific
definition but rather as a SPECTRUM of
movements that blend into one-another
WHY?
10. BecauseâŚâŚ..
⢠They often co-exist
⢠Even neurologists may often not be
able to agree as to how a particular
movement should be classified!
⢠They often ( with some notable
exceptions ) have the same
significance in terms of aetiology.
11. The spectrum
Ballismus Chorea Athetosis Dystonia
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
12. Ballismus
⢠Violent âflingingâ movement of entire limb
⢠Almost always unilateral and therefore
use term â HEMIBALLISMUSâ
⢠Involves proximal musculature and is
sometimes thought of as a
â proximal unilateral chorea â
⢠Usually due to a CVA in contralateral
subthalamic nucleus
15. Secondary to medical
disorders
(A SHEEP)
⢠Anoxic brain damage ( post â CPR )
⢠Systemic lupus erythematosis
⢠Hepatic failure
⢠Endocrine - Thyrotoxicosis
- Addisons
⢠Electrolyte - Low Ca, Mg,
- High Na
⢠Polycythemia rubra vera
16. Chorea ( âdanceâ in Greek)
⢠Rapid irregular muscle jerks
⢠May affect limbs, head, face and tongue
⢠In the limbs chorea refers more to distal
movements ( as proximal movements usually
called ballismus)
⢠Patients often attempt to conceal involuntary
movements by superimposing voluntary
movements onto them e.g. an involuntary
movement of arm towards face may be adapted
to look-like an attempt to look at watch
17. Sydenhamâs chorea
⢠Mainly children / adolescents
⢠Complication of previous group A
streptococcal infection
⢠Usually no recent history of infection
⢠Acute / subacute onset
⢠May have behavioural problems
⢠Usually remits spontaneously
18. Chorea gravidarum
⢠Chorea of any cause that begins in
pregnancy
⢠May represent recurrence of
Sydenhamâs chorea.
⢠Most commonly associated with anti-
phospholipid syndrome +/- SLE
⢠Usually resolves spontaneously
19. Athetosis â changeableâ in Greek
⢠Slow, flowing, often twisting movements
⢠Occurs mainly distally ( hands, fingers)
⢠Can also affect face and tongue
⢠Often use term â choreoathetosis â due
to overlap between syndromes ( chorea
referring to less smooth , more jerky
movements)
21. TREATMENT OF MYOCLONUS
Drug Initial Adult Dose
ďśClonazepam ďś0.5 mg / day
ďśLevetiracetam ďś250 mg / day
ďśPiracetam ďś400 mg 3 times a day
ďśPrimidone ďś25 mg / day
ďśValporate ďś125 mg 2 times a day
22. TREATMENT OF MYOCLONUS
Indication
Usual Effective Dose
ďś Posthypoxic myoclonus Spinal
ďś 2 mg/day divided myoclonus Progressive myoclonic
3 times a day
epilepsy Essential myoclonus
ďś 1000-1500 mg/day ďś Posthypoxic myoclous Cortical
myoclonus Spinal myoclous
ďś 1200-16,000 mg/day divided 3 ďś Posthypoxic myoclonus Cortical
times a day myoclonus Progressive myoclonic
epilepsy Essential myoclonus
ďś 500-750 mg/day ďś Cortical myoclonus
ďś 750-1000 mg/day divided 2 ďś Most forms of myoclonus
times a day
23. TREMOR
ďśRepetitive oscillation ďśEssential tremor
of a body part ďśPhysiologic tremor
ďśParkinson tremor
ďśCrebellar tremor
24. TREATMENT OF ESSENTIAL TREMOR
Drug Initial Adult Usual Effective
Dose Dose
Propranolol 20 mg/day 80-240 mg/day
Primidone 12.5-25mg 50-300 mg/day
at bed time divided Bio or
at bedtime
Topiramate 12.5=25mg/day 400 mg/day
maximum dose
divided Bio
26. TIC
ďśStereotyped, ďśTourette syndrome
automatic ďśCelebral palsy or
purposeless developmental delay
movements and syndromes
vocalizations ďśAutism
ďśHuntington disease
27. PRIMARY TIC DISORDERS
DIAGNOSTIC CRITERIA
DISORDER Presence of multiple motor and
Tourette syndrome vocal tics
Age at onset <21 y
Tics must occur many times daily,
nearly every day, over a period
of >1y
Disturbance causes marked
distress or significant
impairment in daily functioning
Condition cannot be ascribed to
known neurological disorder
(symptomatic or secondary tic
disorder)
Duration of tic disorder <1 y
Transient tic disorder
28. Primary tic disorders
Diagnostic Criteria
Disorder
Chronic motor or chronic vocal
Chronic tic disorder tics (but not both of >1y
Chronic single motor or chronic
single vocal tic
Chronic single tic Tic order that begins > age 21
disorder Two temporal patterns:
- De novo adult-onset tic
- Recurrent childhood tic â a tic
Adult-onset tic discorder disorder than goes into
remission and recurs during
adulthood
29. DIFFERENTIAL DIANOSIS OF TICS
lassification
C Diffenential
Diagnosis
Simple Motor Tics
Clonic Myoclonus
Chorea
Seizures
Dystonia Dystonia
Athetasis
Tonic Muscle spasms and
cramps
Complex Motor Tcs
Mannerisms
Stereotypies
Restless legs syndrome
Seizure
Phenomenology
Abrupt Myoclonus
Chorea
Hyperekplexia
Paraxysmal dyskinesia
Seizures
Sensory phenomenon Akathisia-stereotypy
(urge relief) Restless legs syndrome
30. Classification
Diffenential
Diagnosis
Perceived as voluntary Akasthisia
Suppressibility All hyperkinesias but less than tics
Decrease with distraction Akasthisia
Psychogenic movements
Increase with stress Most hyperkinesias
Increase with relaxation Parkinsonian tremor
(after a period of stressA)
Multifocal migrate Chorea
Myoclonus
Fluctuate spontaneously Paraxysmal dykinesias
Seizures
Present during sleep Myoclonus (segmental)
Periodic movements
Painfullegs / moving toes
Other hyperkinesias
SEizures
35. General guidelines for treating
tardive syndromes
⢠Taper and slowly eliminate causative medications, if clinically possible.
Avid sudden cessation of these drugs, which can exacerbate symptoms
⢠If treatment of tardive movements is necessary, the drugs of first choice
are the dopamine-depleting drugs reserpine, tetrabenazine, and a-
methylparatyrosine. Monitor for the development of depression,
hypotension sedation, and parkinsonism.
⢠If dopamine-depleting agents are ineffective, consider a trial of clozpine
or quetiapine.
⢠Dopamine receptor-blocking agents can be used as medications of last
resort for patients with tardive syndromes despite the risk of worsening
the syndrome over the long term.
⢠Consider globus pallidus stimulation if pharmacotherapy is ineffective.
36. NEUROLEPTIC INDUCED
MOVEMENT DISORDERS
1. Acute reaction
a. Acute dystonia
b. Acute (Subacute) akathisia
2. Toxicity state (over dosage)
a. Drug-induced parkinsonism
3. Neuroleptic malignant syndrome (NMS)
4. Tardive stbdrines
a. Withdrawal emergent syndrome
b.Classic fardive dyskinesia
c. Tardive dystonia
d. Tardive akathisia
e. Tardive myoclonus
f. Tardive tremor
g. Tardive tics
h. Tardive Chorea
i. (?) Tardive parkinsonism
37. TERMINOLOGY OF THE TARDIVE SYNDROMES
Description Equivalent
CommonNames
Tardive syndromes Tardive syndrome
as a group Tardive dykinesia
Repetitive, rhythmic Classic tardive
Movements, usually in dyskinesia
The oral-buccal-lingual Tardive setreotypy
Region Rhythemic chorea
Dystonic movements
and postures
restlessness and the Tardive akasthisia
movement that occur
as result
Myoclonus Tardive myoclonus
Tremor Tardive tremor
Tucs Tardive tics
Tardive tourettism
Chorea Withdrawal emergent syndrome
Tardive chorea
Oculogyria Tardive oculogyric crisis
Parkinsonism Tardive Parkinsonism (if it exists)
39. NATURE HISTORY OF
TOURETTE SYNDROME
⢠Excerbation Remission ?
⢠Obsessive-compulsive behavior
⢠Vocal tics (simple complex)
⢠Motor tics (rostrocaudalprogession)
⢠Attention deficit with hyperactivity
40. Summary
⢠Movement disorders are often difficult to define precisely, but
have similar differential diagnoses.
⢠They are often a manifestation of a more widespread
neurological or internal medical problem.
⢠Other than the specific treatments mentioned, most details of
therapy are beyond the scope of this course.
⢠In some cases treatment includes treatment of underlying
cause e.g. Wilsonâs disease
43. READ not to contradict or confute
Nor to Believe and Take for Granted
but TO WEIGH AND CONSIDER
THANK YOU
My sincere thanks to Faculty of Madras
Institute of Neurology and Madras Medical
College for giving this opportunity to speak
in the CME Program