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EXTRAPYRAMIDAL SYSTEM DISORDERS - PowerPoint PPT Presentation


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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

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Presentation Transcript
slide4
ANATOMY
  • Caudate nucleaus
  • Putamen
  • Globus pallidus
  • Substantia nigra
  • Subthalamic nuleus
  • Thallamus
slide7
EFFECTS OF DYSFUNCTION INGENERAL
  • Involuntary movements
  • Altered movements
  • slow
  • Interrupted
  • Uncordinated
  • Posture and tone altered
slide8
MOVEMENT DISORDERS

BRADYKINETIC ---TOO LITTLE

  • Parkinson disease
  • Wilsons disease
  • Huntingtons disease
slide9
HYPERKINETIC –TOO MUCH
  • Athetosis
  • Hemiballismus
  • Dystonia
  • Dyskinesia
  • Chorea
  • Myoclonus
  • Tremors
  • Tics
slide10
NEUROTRANSMITTERS
  • Dopamine > Ach = hyperkinetic
  • Ach > dopamine =hypokinetic
slide11
CAUSES OF EXTRAPYRAMIDALDISORDERS
  • Drugs---chlorpromazine

---butyrophenons

---metochlorpramide

---reserpine

slide12
Causes cont.
  • Toxins—CO and manganese poisoning
  • Inherited and metabolic disorders :
  • wilsons disease
  • spinocerebellar ataxia
  • Encephalitis lethargica
  • Diffuse small vascular disease
slide13
Causes cont.
  • Inherited or degenerative disease
  • huntingtons disease
  • progressive supra nuclear gaze palsy

{Steel Richardson}

slide14
PARKINSONS DISEASE
  • Effects dopaminergic neurons
  • Neurons are lost from substantia nigra
  • Rarely presents before 50 years
  • Neurodegenerative disease
  • Equal sex distribution
slide15
CLINICAL FEATURES

Characterized by:

  • Tremors
  • Rigidity
  • bradykinesia
slide16
TREMORS
  • Rest tremor
  • Starts in the thumb
  • Adduction and abduction of the thumb
  • Pill rolling
  • Tremors may effect the legs, mouth or the tongue
slide17
RIGIDITY
  • Leadpipe or plastic
  • Cogwheel

BRADYKINESIA

  • Slow movements
  • Develop gradually
  • Impairement of fine movements
slide18
General clinical features
  • Slow and monotonous speech
  • Greasy skin
  • Expressionless face ---mask face
  • Infrequent blinking
  • Flexed posture
  • Reduced arm swing
slide19
Clinical features cont.
  • Gait—slow in initiating

---rapid small steps tendency to run-

festination

---shuffling

  • Impaired balance
  • Glabber tap
slide20
Clinical features cont.
  • Muscle power is normal
  • Reflexes –normal
  • Sensations –normal
  • Cognitive abnormality as the disease advances
slide21
INVESTIGATIONS
  • Clinical diagnosis
  • Exclude other causes –pts who present before 50 years
  • Brain CT scan or MRI
slide22
TREATMENT
  • Levodopa
  • Anticholinergic drugs
  • Amantadine
  • Dopamine agonists—bromocriptine, pergolide
  • COMT inhibitors (catechol-o-methyl transferase)—tolcapone
  • MAO –inhibitors--selegine
slide25
HUNTINGTONS DISEASE
  • Inherited disorder
  • Autosomal dominant
  • Males females equally affected
  • Presents during the 4th decade
  • Chorea which worsens with time
  • Cognitive disorders
  • Dementia
slide26
Cont.
  • Abnormal facial movement
  • Mood swings
  • Jaw clenching
  • Slurred speech
  • Difficulty in walking
  • Personality changes
slide27
Cont.
  • Abnormal facial movement
  • Mood swings
  • Jaw clenching
  • Slurred speech
  • Difficulty in walking
  • Personality changes
slide28
WILSONS DISEASE
  • Hepato lenticular disorder
  • Autosomal recessive
  • Treatable cause of parkinsonsim
  • Due to deposition of copper in basal ganglia
  • Onset during childhood rarely in adulthood
slide29
Cont.
  • Present with liver disease in childhood
  • Impaired concentration
  • Decling intellect
  • Behavioural problems
  • Involuntary movements
  • Generalized dystonia
slide30
Cont.
  • Ataxia
  • Kayser Fleicher ring

Diagnosis

  • Serum ceruloplasmin level
  • 24 hour urine for copper
  • LFT
  • Liver biopsy
slide31
HYPERKINETIC MOVEMENTS
  • large variety of hyperkinetic disorders
  • Most are organic
  • All movements disappear during sleep
slide32
CHOREA
  • Continous unsustained rapid abrupt and random contractures
  • Small fidgety movements
  • Distal muscles involved
slide33
CAUSES
  • Huntingtons disease
  • Drugs—Rx of parkinsonism , oral c.pills
  • SLE
  • Sydenhams chorea
  • Wilsons disease
  • Polycythemia
  • Friedricks ataxia
slide34
HEMIBALLISMUS
  • Throwing of the limbs on one side of the body
  • Usually due to CVA involving the subthalamic nucleus
slide35
MYOCLONUS
  • Simple jerky movements that are not co-ordinated or suppressible

CAUSES

  • Renal failure
  • Hepatic failure
  • Creutz feldt jacob disease
  • Subacute sclerosing panencephalitis
slide36
DYSTONIA
  • Repeated patterned twisting and sustained movements that may be either slow or rapid
  • Involuntary movements occur before 20 years
slide37
Dystonia Cont.
  • Disturbance of the affected muscle groups

depend upon age

--distally---in children

---cranial - cervical ---adults

slide38
Dystonia cont.
  • Primary----focal----torticollis

----writers cramps

generalized

  • Secondary----wilsons disease

----toxins

slide39
ATHETOSIS
  • Writhing movements
  • Mainly due to cerebral palsy

DYSKINESIA

  • Tardive—drugs-> 6 wks exposure to dopamine agonists
  • Orofacial repeated movements
slide40
TREMORS
  • Physiological
  • Familial
  • Resting—parkinsons disease
  • Intention or action ---cerebellar
slide41
TICS
  • Brief stereotyped supressible movements
  • Worse with stress

Cause

Dopamine excess causes inhibition of limbic system

Rx

Dopamine agonist