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EXTRAPYRAMIDAL SYSTEM DISORDERS
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BASAL GANGLION DYSFUNCTION
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ANATOMY Caudate nucleaus Putamen Globus pallidus Substantia nigra
Subthalamic nuleus Thallamus
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EFFECTS OF DYSFUNCTION IN GENERAL
Involuntary movements Altered movements slow Interrupted Uncordinated Posture and tone altered
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MOVEMENT DISORDERS BRADYKINETIC ---TOO LITTLE Parkinson disease Wilsons disease Huntingtons disease
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HYPERKINETIC –TOO MUCH
Athetosis Hemiballismus Dystonia Dyskinesia Chorea Myoclonus Tremors Tics
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NEUROTRANSMITTERS Dopamine > Ach = hyperkinetic Ach > dopamine =hypokinetic
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CAUSES OF EXTRAPYRAMIDAL DISORDERS
Drugs---chlorpromazine ---butyrophenons ---metochlorpramide ---reserpine
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Causes cont. Toxins—CO and manganese poisoning Inherited and metabolic disorders : wilsons disease spinocerebellar ataxia Encephalitis lethargica Diffuse small vascular disease
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Causes cont. Inherited or degenerative disease huntingtons disease progressive supra nuclear gaze palsy {Steel Richardson}
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PARKINSONS DISEASE Effects dopaminergic neurons Neurons are lost from substantia nigra Rarely presents before 50 years Neurodegenerative disease Equal sex distribution
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CLINICAL FEATURES Characterized by: Tremors Rigidity bradykinesia
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TREMORS Rest tremor Starts in the thumb Adduction and abduction of the thumb Pill rolling Tremors may effect the legs, mouth or the tongue
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RIGIDITY Leadpipe or plastic Cogwheel BRADYKINESIA Slow movements Develop gradually Impairement of fine movements
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General clinical features
Slow and monotonous speech Greasy skin Expressionless face ---mask face Infrequent blinking Flexed posture Reduced arm swing
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Clinical features cont.
Gait—slow in initiating ---rapid small steps tendency to run- festination ---shuffling Impaired balance Glabber tap
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Clinical features cont.
Muscle power is normal Reflexes –normal Sensations –normal Cognitive abnormality as the disease advances
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INVESTIGATIONS Clinical diagnosis Exclude other causes –pts who present before 50 years Brain CT scan or MRI
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TREATMENT Levodopa Anticholinergic drugs Amantadine Dopamine agonists—bromocriptine, pergolide COMT inhibitors (catechol-o-methyl transferase)—tolcapone MAO –inhibitors--selegine
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HUNTINGTONS DISEASE Inherited disorder Autosomal dominant Males females equally affected Presents during the 4th decade Chorea which worsens with time Cognitive disorders Dementia
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Cont. Abnormal facial movement Mood swings Jaw clenching Slurred speech Difficulty in walking Personality changes
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Cont. Abnormal facial movement Mood swings Jaw clenching Slurred speech Difficulty in walking Personality changes
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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
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Cont. Present with liver disease in childhood Impaired concentration Decling intellect Behavioural problems Involuntary movements Generalized dystonia
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Cont. Ataxia Kayser Fleicher ring Diagnosis Serum ceruloplasmin level 24 hour urine for copper LFT Liver biopsy
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HYPERKINETIC MOVEMENTS
large variety of hyperkinetic disorders Most are organic All movements disappear during sleep
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CHOREA Continous unsustained rapid abrupt and random contractures Small fidgety movements Distal muscles involved
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CAUSES Huntingtons disease Drugs—Rx of parkinsonism , oral c.pills SLE Sydenhams chorea Wilsons disease Polycythemia Friedricks ataxia
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HEMIBALLISMUS Throwing of the limbs on one side of the body Usually due to CVA involving the subthalamic nucleus
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MYOCLONUS Simple jerky movements that are not co-ordinated or suppressible CAUSES Renal failure Hepatic failure Creutz feldt jacob disease Subacute sclerosing panencephalitis
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DYSTONIA Repeated patterned twisting and sustained movements that may be either slow or rapid Involuntary movements occur before 20 years
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Dystonia Cont. Disturbance of the affected muscle groups depend upon age --distally---in children ---cranial - cervical ---adults
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Dystonia cont. Primary----focal----torticollis ----writers cramps generalized Secondary----wilsons disease ----toxins
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ATHETOSIS Writhing movements Mainly due to cerebral palsy DYSKINESIA Tardive—drugs-> 6 wks exposure to dopamine agonists Orofacial repeated movements
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TREMORS Physiological Familial Resting—parkinsons disease Intention or action ---cerebellar
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TICS Brief stereotyped supressible movements Worse with stress Cause Dopamine excess causes inhibition of limbic system Rx Dopamine agonist
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