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

EXTRAPYRAMIDAL

DISORDERS

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.

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.

Basal ganglia Extrapyramidal disorders (=movement disorders):

Parkinsonism..

Chorea.

Dystonia.

Athetosis.

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.

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.

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.

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.

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.

Postural instability= impaired

balance.

Other features:

Dementia.

Depression.

Dysautonomia.

Sleep Disturbances.

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.

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.

II. Chorea

Involuntary

Static

Irregular

Dysrhythmic

Sudden

Jerky

Pseudopurposive

Of any part of the body

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.

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.

III. Athetosis

Involuntary

Static

Irregular

Slow

Snake-like movemets

Mainly extremities and face

hypertonia

III.Athetosis

Causes:

Congenital: e.g. hypoxic neonatal brain damage.

Acquired: Post-encephalitic.

Treatment:

Anticholinergic drugs

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.

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.

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.

Dystonia

Treatment:

]In 2ry dystonia: treatment of the cause

Treatment of dystonia:

Oral medications e.g. benzodiazepine.

Botulinum toxin injection (Botox):

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.

Spasticity

Upper limb - common clinical patterns

Adducted/internally

rotated shoulder Flexed wrist Pronotated forearm

Clenched fist Flexed elbow Thumb in palm deformity

Spasticity

Lower limb – common clinical patterns

Equinovarus Striatal toe

Stiff knee Flexed knee Adducted thighs

Thank you