34218_EXTRAPYRAMIDAL SYSTEM DISORDERS.ppt

7/18/2019 34218_EXTRAPYRAMIDAL SYSTEM DISORDERS.ppt

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EXTRAPYRAMIDAL

SYSTEM

DISORDERS



BASAL GANGLION

DYSFUNCTION



ANATOMY

Caudate nucleaus Putamen

Globus pallidus

Substantia nigra Subthalamic nuleus

Thallamus







EFFECTS OF DYSFUNCTION IN GENERAL

Involuntary movements

Altered movements• slow

• Interrupted

• Uncordinated Posture and tone altered



MOVEMENT DISORDERS

BRADYKINETIC ---TOO LITTLE

Parkinson disease

Wilsons disease

Huntingtons disease



HYPERKINETIC – TOO MUCH Athetosis Hemiballismus Dystonia Dyskinesia Chorea

Myoclonus Tremors Tics



NEUROTRANSMITTERS

Dopamine > Ach = hyperkinetic

Ach > dopamine =hypokinetic



CAUSES OF EXTRAPYRAMIDAL DISORDERS

Drugs---chlorpromazine

---butyrophenons

---metochlorpramide---reserpine



Causes cont.

Toxins — CO and manganese poisoning

Inherited and metabolic disorders :

wilsons disease

spinocerebellar ataxia Encephalitis lethargica

Diffuse small vascular disease



Causes cont.

Inherited or degenerative disease

huntingtons disease

progressive supra nuclear gaze palsy

{Steel Richardson}



PARKINSONS DISEASE

Effects dopaminergic neurons

Neurons are lost from substantia nigra

Rarely presents before 50 years

Neurodegenerative disease Equal sex distribution



CLINICAL FEATURES

Characterized by:

Tremors Rigidity

bradykinesia



TREMORS

Rest tremor

Starts in the thumb

Adduction and abduction of the thumb

Pill rolling Tremors may effect the legs, mouth or the

tongue



RIGIDITY

Leadpipe or plastic

Cogwheel

BRADYKINESIA

Slow movements Develop gradually

Impairement of fine movements



General clinical features

Slow and monotonous speech

Greasy skin

Expressionless face ---mask face

Infrequent blinking Flexed posture

Reduced arm swing



Clinical features cont.

Gait — slow in initiating

---rapid small steps tendency to run-

festination

---shuffling Impaired balance

Glabber tap



Clinical features cont.

Muscle power is normal

Reflexes – normal

Sensations – normal

Cognitive abnormality as the diseaseadvances



INVESTIGATIONS

Clinical diagnosis

Exclude other causes – pts who presentbefore 50 years

Brain CT scan or MRI



TREATMENT

Levodopa

Anticholinergic drugs Amantadine

Dopamine agonists — bromocriptine, pergolide

COMT inhibitors (catechol-o-methyltransferase) — tolcapone

MAO – inhibitors--selegine







HUNTINGTONS DISEASE

Inherited disorder

Autosomal dominant Males females equally affected

Presents during the 4th decade

Chorea which worsens with time Cognitive disorders

Dementia



Cont.

Abnormal facial movement

Mood swings

Jaw clenching

Slurred speech

Difficulty in walking

Personality changes



Cont.

Abnormal facial movement

Mood swings

Jaw clenching

Slurred speech

Difficulty in walking

Personality changes



WILSONS DISEASE

Hepato lenticular disorder

Autosomal recessive

Treatable cause of parkinsonsim

Due to deposition of copper in basalganglia

Onset during childhood rarely in adulthood



Cont.

Present with liver disease in childhood

Impaired concentration

Decling intellect

Behavioural problems

Involuntary movements

Generalized dystonia



Cont.

Ataxia

Kayser Fleicher ringDiagnosis

Serum ceruloplasmin level

24 hour urine for copper LFT

Liver biopsy



HYPERKINETIC MOVEMENTS

large variety of hyperkinetic disorders

Most are organic

All movements disappear during sleep



CHOREA

Continous unsustained rapid abrupt andrandom contractures

Small fidgety movements

Distal muscles involved



CAUSES

Huntingtons disease

Drugs — Rx of parkinsonism , oral c.pills SLE

Sydenhams chorea

Wilsons disease Polycythemia

Friedricks ataxia



HEMIBALLISMUS

Throwing of the limbs on one side of the

body

Usually due to CVA involving thesubthalamic nucleus



MYOCLONUS

Simple jerky movements that are not co-

ordinated or suppressibleCAUSES

Renal failure

Hepatic failure Creutz feldt jacob disease

Subacute sclerosing panencephalitis



DYSTONIA

Repeated patterned twisting and sustained

movements that may be either slow orrapid

Involuntary movements occur before 20

years



Dystonia Cont.

Disturbance of the affected muscle groups

depend upon age

--distally---in children

---cranial - cervical ---adults



Dystonia cont.

Primary----focal----torticollis

----writers cramps

generalized

Secondary----wilsons disease

----toxins



ATHETOSIS

Writhing movements

Mainly due to cerebral palsy

DYSKINESIA

Tardive — drugs-> 6 wks exposure todopamine agonists

Orofacial repeated movements



TREMORS

Physiological

Familial

Resting — parkinsons disease

Intention or action ---cerebellar



TICS

Brief stereotyped supressible movements

Worse with stressCause

Dopamine excess causes inhibition of limbic

systemRx

Dopamine agonist

























































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34218_EXTRAPYRAMIDAL SYSTEM DISORDERS.ppt