This document discusses various hyperkinetic motor disorders including tremor, dystonia, chorea, tics, myoclonus, and hemiballismus. It defines each disorder and describes their typical presentations, causes, pathophysiology, and treatment approaches. Essential tremor, Parkinson's disease, Huntington's disease, Sydenham's chorea, Tourette syndrome, and other conditions are explained in further detail. Overall, the document provides a comprehensive overview of hyperkinetic movement disorders for medical professionals.

1. Hyperkinetic Motor
Disorders
Dr Leta A. (MD)

2. Tremor
• Definition: rhythmic and oscillatory movement of a
body part with a relatively constant frequency and
variable amplitude
• Due to alternating contractions of agonist and antagonist
muscles in an oscillating, rhythmic manner.
• The most common movement disorder

3. Tremor
• Rest Tremor: tremor prominent at rest
• Although when a patient totally relaxes or sleeps, this tremor
usually disappears
• Postural Tremor: tremor revealed by maintenance of a
posture, such as extending the arms parallel to the
floor
• Kinetic (Intention) Tremor: tremor revealed while
moving the body part to and from a target

4. Tremor
• Action tremor has been used for both postural and
kinetic
• Terminal tremor: applies to the component of kinetic
tremor that exaggerates when approaching the target.
• Kinetic tremor is present throughout goal-directed movement

5. Tremor
• Physiologic tremor: tremor seen in normal individuals
that typically manifests as a mild, high-frequency,
postural or action tremor that is usually of no clinical
consequence and often is only appreciated with an
accelerometer.
• An enhanced physiologic tremor (EPT) can be seen in
up to 10% of the population, often in association with
anxiety, fatigue, underlying metabolic disturbance,
drugs, or toxins

6. Essential Tremor

7. Essential Tremor
• ET is the commonest movement disorder
• Affects ~5–10 million persons in the United States.
• It can present in childhood, but dramatically increases
in prevalence over the age of 70 years.
• High frequency tremor
• Most often manifest as a postural or kinetic tremor.
• Typically bilateral and symmetric, but may begin on one
side and remain asymmetric.
• Patients with severe ET can have an intention tremor
with overshoot and slowness of movement.

8. Essential Tremor
• Tremor involves the head in ~30% of cases, voice in
~20%, tongue in ~20%, face/jaw in ~10%, and lower
limbs in ~10%.
• Improved by alcohol and worsened by stress.

9. Essential Tremor

10. Essential Tremor
• The etiology and pathophysiology of ET are not known.
• ~50% of cases have a positive family history with an
autosomal dominant pattern of inheritance

11. Essential Tremor- Rx
• Many cases are mild - require no treatment
• Reassurance.
• Occasionally, tremor can be severe and interfere with
eating, writing, and activities of daily living.
• This is more likely to occur as the patient ages and is
often associated with a reduction in tremor frequency.

12. Essential Tremor- Rx
• β-Blockers
• Primidone

13. Dystonia

14. Dystonia
• Involuntary, twisting, sustained movement of the limb
or head resulting in an abnormal posture.

15. Dystonia
• Can range from minor contractions in an individual
muscle group to severe and disabling involvement of
multiple muscle groups.
• Dystonia is often brought out by voluntary
movements (action dystonia) and can become
sustained and extend to involve other body regions.

16. Dystonia
• It can be aggravated by stress and fatigue, and
attenuated by relaxation and sensory tricks such as
touching the affected body part
• Classification
• Based on age: Childhood vs adult
• Based on distribution: Focal, multifocal, segmental or
generalized
• Based on etiology: Primary vs. Secondary

17. Dystonia- Medical Rx
• Botulinum toxin treatment continues to be the first
choice treatment for most types of focal dystonia.
• Pharmacological and neurosurgical treatments have
also a role in the treatment algorithm

18. Chorea

19. Chorea
• Rapid, semi purposeful, graceful, dance like, non
patterned involuntary movements involving distal or
proximal muscle groups

20. Huntington’s Diseases
• George Huntington in 1872
• HD is a fatal, highly penetrant autosomal dominant
disease characterized by a progressive motor,
behavioral and cognitive dysfunction
• Onset 25-45 yrs

21. HD- Etiology
• Caused by an increase in the number of polyglutamate
(CAG) repeats in the coding sequence of the
Huntington gene located on the short arm of
chromosome 4
• The larger the repeat the earlier the disease appears
• The gene codes for the protein called huntingtin the
function of which is not known

22. HD-Treatment
• Multidisciplinary approach
• Supportive
• Chorea: dopamine blocking agents but associated with
high risk of S/Es
• Depression and anxiety:
• Psychosis: atypical neuroleptics
• Neuroprotective therapy not yet available

23. Sydenham’s Chorea
• Aka St Vitus’ dance
• Disease of children(5-15 yrs)
• More common in females
• Chorea, behavioral disturbances and
occasionally other motor disorders
• 10% of pts with RF
Thomas Sydenham
‘The English Hippocrates

24. Sydenham’s treatment
• Dopamine blocking agents
• Carbamazepine
• Valproic acid
• Steroid
• Prophylactic B.Penicillin
• Risk of recurrence especially with pregnancy (chorea
gravidarum)

25. Other causes of chorea
• Hypo and hyperglycemia
• Hyperthyroidism
• SLE
• HIV
• Polycythemia
• Drugs (cocaine, lithium, CNS stimulants, estrogen,
anticonvulsants)

26. Athetosis
• Slow, distal, writhing, involuntary movements with a
propensity to affect the arms and hands

27. Hemiballismus
• Hemiballismus is a violent form of chorea composed of
wild, flinging, large-amplitude movements on one side
of the body. Proximal limb muscles tend to be
predominantly affected.
• Greek: ballismos= “a jumping about or dancing”
• 100 times rarer than Parkinson’s Disease

28. Hemiballismus
• Both the proximal and distal muscle groups involved
• Hemiballismus often evolves into hemichorea
• The movements may be so severe as to cause
exhaustion, dehydration, local injury, and in extreme
cases, death.

29. Hemiballismus- DDx
• Trauma
• Traumatic Brain
Hemorrhage
• Neoplastic Disorders
• Metastatic brain disease
• Anaplastic astrocytoma
• Other Brain tumor
• Glioblastoma multiforme
• Arteriosclerotic,
vascular, venous
• Cerebrovascular accident
• Cerebral hemorrhage
• Drugs
• Dopamine agonist
• Phenytoin
• Poisoning
• Mercury chronic toxicity
• Infectious
• Tuberculoma
• Toxoplasmosis

30. Hemiballismus
• Pharmacologic treatment is the same as that
prescribed for other choreatic disorders
• The most widely used agents- Neuroleptics
• Mechanism of action: Dopamine receptor blockage
• Neuroleptics: Typical & Atypical

31. Hemiballismus
• Neuroleptics ctd
• Typical Neuroleptics
• Haloperidol and fluphenazine
• Atypical neuroleptics
• Risperidone, Olanzapine, Clozapine, and Quetipine

32. Hemiballismus
• Dopamine depleting agents such as Reserpine and
Tetrabenazine represent another option in the
treatment of chorea
• GABAergic drugs, such as clonazepam, gabapentin and
valproate, can be used as adjunctive therapy.

33. Tics
• Brief ,rapid ,recurrent and seemingly purposeless
stereotyped motor contraction
• Can be suppressed
• Can be motor, vocal, sensory
• Can be simple or complex

34. Tics
• Motor tics- associated muscle movments
• Simple Motor tics: movement only affecting an individual muscle
group
• E.g., blinking, twitching of the nose, jerking of the neck)
• Complex Motor tics: with coordinated involvement of multiple
muscle groups
• E.g., jumping, sniffing, head banging, and echopraxia (mimicking
movements)

35. Tics
• Vocal tics- can also be simple or complex
• Simple- e.g., grunting
• Complex- e.g
• Echolalia- repeating other people's words
• Palilalia- repeating one's own words, and
• Coprolalia- expression of obscene words
• Sensory tics: composed of unpleasant focal sensations
in the face, head, or neck

36. Tics
• Tics may present in adulthood and can be seen in
association with a variety of other disorders, including
PD, HD, trauma, dystonia, drugs (e.g., levodopa,
neuroleptics), and toxins.

37. Tourette syndrome
• Neurobehavioral disorder named after the French
neurologist Georges Gilles de la Tourette
• Predominantly affects males
• Prevalence 0.03-1.6%
• It is thought to be genetic but no specific gene has
been identified
• Alterations in dopamine neurotransmission, opioids, and
second messenger systems are proposed
mechanisms

38. TS- Etiology & Pathophysiology
• TS is thought to be a genetic disorder, but no specific
gene mutation has been identified.
• Current evidence supports a complex inheritance
pattern, with one or more major genes, multiple loci,
low penetrance, and environmental influences.
• The risk of a family with one affected child having a
second is about 25%.

39. TS- Treatment
• Mild cases: education and counseling
• Alpha agonists clonidine, Guanfacine
• Antipsychotics atypical and classical neuroleptics
• Botulinum toxin for focal tics
• DBS targeting the anterior portion of the internal
capsule is under study

40. Myoclonus
• Brief, rapid(<100ms) , shock like, jerky movement
consisting of single or repetitive muscle discharges
• Can be focal ,multifocal, segmental, or generalized
• Can occur spontaneously, with voluntary movements
(action myoclonus), or in response to an external
stimulus(reflex or startle myoclonus)
• Are not suppressible
• Metabolic disturbances (hypocalcaemia, renal failure)
• Negative myoclonus consists of a twitch due to brief
loss of muscle activity as in asterixis

41. Myoclonus
• Essential myoclonus is a relatively benign familial
condition characterized by multifocal lightning-like
movements.
• Myoclonic jerks can be disabling when they interfere
with normal movement.
• They can also be innocent and are commonly observed
in normal people when waking up or falling asleep
(hypnogogic jerks)

42. Myoclonus-Treatment
• Treating the underlying cause or removing the
offending agent
• Pharmacologic therapy: GABAergic agents such as
valproic acid, clonazepam, primidone, levetiracetam

43. Thank You!