The document discusses extrapyramidal disorders associated with the basal ganglia, detailing their anatomy, functions, and associated movement disorders such as parkinsonism, chorea, dystonia, and athetosis. It describes the clinical features and treatment options for parkinsonism, emphasizing the balance between acetylcholine and dopamine, and outlines the symptoms and treatments for other movement disorders. Additionally, it covers the classification of dystonia and various therapeutic approaches, including medications and surgical interventions.

1. Amr Hassan, M.D.
Associate professor of Neurology - Cairo University
EXTRAPYRAMIDAL
DISORDERS

2. Basal ganglia
Anatomy:
 A group of brain nuclei are known collectively as the basal ganglia.
 The basal ganglia include caudate nucleus,the putamen ,the
globus pallidus ,the subthalamic nucleus ,and the substantia
nigra.
 The motor components of the basal ganglia make up the
extrapyramidal motor system that comprise fibers that influence
the motor end plate activity and do not pass in the pyramidal
tract.
 The caudate nucleus and putamen are collectively known as the
corpus striatum (i.e. striated body) because of their appearance.
 Similarly, the shape of the putamen and globus pallidus
resembles a lens, and they are collectively called the lentiform
nucleus.

6. Basal ganglia
Functions:
 Regulation and integration of voluntary motor activity.
 Regulation and maintenance of the muscle tone.
 Regulation and maintenance of emotional and associative
movements.

7. Basal ganglia
Extrapyramidal disorders (=movement disorders):
 Parkinsonism..
 Chorea.
 Dystonia.
 Athetosis.

8. Parkinsonism
Definition:
 The term parkinsonism is used for a motor syndrome whose main
symptoms are rest tremor, rigidity, bradykinesia and postural
instability.
Types:
 Parkinsonian syndromes can be divided into four subtypes
according to their origin:
 Primary or idiopathic (Parkinson’s disease).
 Secondary or acquired.
 Hereditary parkinsonism.
 Parkinson plus syndromes.

9. Parkinson’s disease
 It is a "primary" parkinsonism, meaning parkinsonism with
no external identifiable cause.
Pathogenesis:
 There is degeneration of the pigmented cells of the
substantia nigra, which becomes pale deficiency of
dopamine in the brain imbalance between the levels of
acetylcholine and dopamine in the basal ganglia and
substantia nigra.
Clinical picture:
 The age of onset is above 50 years. Both sexes are equally
affected.

10. Rest (static) tremors:
•Due to disturbance in the integration
and regulation of voluntary motor
activity.
•Rhythmic and regular, occur at the rate
of 4-8/second.
•↑ With emotional stress, anxiety and
fatigue.
•Disappear during sleep and during
active voluntary movements.
•Begin unilaterally in the U.L. and spread
to all 4 limbs.
•They give the hand the pill-rolling
posture with the thumb moving
rhythmically back and forward on the
palm.

11. Rigidity:
Due to disturbance in the regulation and
maintenance of normal muscle tone resulting in
hypertonia.
Affecting the proximal more than the distal
muscles.
Affecting more the flexors of the neck, trunk
limbs resulting in the gorilla-like attitude.
It may be present throughout the act to the
same degree & is then described as lead pipe
rigidity; it may be interrupted by the tremors
& is then described as cog wheel rigidity.
Causing difficulty in starting the act of
walking leading to a slow, shuffling
(festinanting or short steppage) gait with
propulsion.

12. Bradykinesia:
Due to disturbance in the regulation
and maintenance of emotional
and associated movements.
Mask face= expressionless face
with
infrequent blinking.
Loss of swinging of arms during
walking.
Monotonus speech.

13. Postural instability= impaired
balance.
Other features:
Dementia.
Depression.
Dysautonomia.
Sleep Disturbances.

14. The UPDRS (Unified Parkinson’s Disease Rating Scale)has
long been the major rating scale that is used to assess severity
of symptoms of Parkinson's disease. It can assess:
 Daily activities.
 Motor skills.
 Mental capacity (including behavior and mood).
 Complication of therapy.

15. Treatment of Parkinsonism
A. Medical
 Aims to restore the balance between acetylcholine & dopamine by
decreasing acetylcholine and/or elevating dopamine levels.
 Anticholinergic drugs.
 Dopaminergic drugs: i.e. Levo-Dopa + Carbi-Dopa (Sinemet) and Dopamine
agonists.
 Amantadine hvdrochloride.
Physical therapy
Supportive treatment:
 Good nutrition.
 Regular rest periods and avoiding stress.
 Speech therapy.
 Occupational therapy.
Interventional therapy
 Deep brain stimulation involves placing electrical stimulators in specific
areas of the brain that control movement.
 Stem cell transplantation.
 Surgery e.g. pallidotomy.

16. II. Chorea
 Involuntary
 Static
 Irregular
 Dysrhythmic
 Sudden
 Jerky
 Pseudopurposive
 Of any part of the body

17. II. Chorea
Causes:
 I. Herido-familial: Huntington's chorea.
 II. Acquired :
 Autoimmune: Rheumatic chorea.
 Infective: Post encephalitic chorea.
 Vascular: Hemiballismus.
 Toxic: Chorea gravidarum.
 Idiopathic: Senile chorea.

18. II. Chorea
Clinical picture:
1. Choreic movements: Affecting the tongue, facial, trunk
and extremities muscles, being more proximal than distal.
 Grimacing, jerking of the shoulders, shaking of the hands and
feet.
 ↑With emotional stress and anxiety.
 Disappear during sleep.
2. Hypotonia.
Treatment:
 Dopamin blocking agent.
 Treatment of the cause.

19. III. Athetosis
 Involuntary
 Static
 Irregular
 Slow
 Snake-like movemets
 Mainly extremities and face
 hypertonia

20. III.Athetosis
Causes:
 Congenital: e.g. hypoxic neonatal brain damage.
 Acquired: Post-encephalitic.
Treatment:
 Anticholinergic drugs

21. Dystonia
 Definition:
It is abnormal involuntary, static, very slow, torsion or twisting
like movements commonly involve the neck, trunk &.the
proximal muscles of the extremities.They are associated with
hypertonia during the movement & normal tone in between.

22. Dystonia
Causes:
 Primary or idiopathic dystonia: dystonia occurs as a solitary
symptom and is not associated with an underlying disorder
e.g. Early-onset primary dystonia are due to a mutation in the
dyt-1 gene.
 Secondary or symptomatic dystonia: dystonia occurs because
of another underlying disease process e.g.Wilson disease,
stroke, brain trauma or medications.

23. Dystonia
Classification: Dystonia may also be classified as follows, according
to the bodily distribution of symptoms:
 Focal dystonia: limited to one region of the body e.g. neck (see
fig. 57) or an arm or a leg.
 Segmental dystonia: affecting two adjacent areas of the body e.g.
the head and neck.
 Multifocal dystonia: affecting two areas of the body that are not
next to each other, such as the two arms, or an arm and a leg.
 Generalized dystonia: symptoms begin in an arm or a leg and
advance, becoming more widespread. Eventually, the trunk and
the rest of the body are involved.

25. Dystonia
Treatment:
]In 2ry dystonia: treatment of the cause
Treatment of dystonia:
 Oral medications e.g. benzodiazepine.
 Botulinum toxin injection (Botox):

26. Dystonia
Botulinum toxin injection (Botox):
 Botulinum toxin is a toxic protein that is produced by the bacterium
Clostridium botulinum. This toxin is known to cause botulism, a
deadly form of food poisoning that is contracted through the
ingestion of contaminated food products.
 However, when a minute amount of commercially prepared
Botulinum toxin (Botox) is injected directly into an overactive muscle
 block the release of acetylcholine  relaxes that muscle 
decreases inappropriate or excessive muscle contractions allowing
the affected area (e.g., arm, neck, leg, eyelid, etc.) to assume a more
normal position or posture.
 Surgery.
 Deep brain stimulation.

27. Spasticity
Upper limb - common clinical patterns
Adducted/internally
rotated shoulder Flexed wrist Pronotated forearm
Clenched fist Flexed elbow Thumb in palm deformity

28. Spasticity
Lower limb – common clinical patterns
Equinovarus Striatal toe
Stiff knee Flexed knee Adducted thighs

32. Thank you