Extrapyramidal disorders

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Presentation Transcript

“EXTRAPYRAMIDAL” MOVEMENT DISORDERS : 

“EXTRAPYRAMIDAL” MOVEMENT DISORDERS Prof. AR AlTahan Division of Neurology

EXTRAPYRAMIDAL SYSTEM : 

EXTRAPYRAMIDAL SYSTEM ANATOMY: Caudate nucleus Putamen Globus pallidus Subthalamic nucleus Substantia nigra These structures are functionally connected with the Thalamus and Pre-motor Cortex

EXTRAPYRAMIDAL SYSTEM : 

EXTRAPYRAMIDAL SYSTEM PHYSIOLOGY Influence the details of a movement plan Modifies the order-plan “Pyramidal plan” Affects motor function by either: FACILITATION SUPPRESSION

EXTRAPYRAMIDAL SYSTEM : 

EXTRAPYRAMIDAL SYSTEM PATHO-PHYSIOLOGY: Failure to facilitate Failure to suppress

EXTRAPYRAMYDAL SYSTEM DYSFUNCTION : 

EXTRAPYRAMYDAL SYSTEM DYSFUNCTION Failure to facilitate - brady-hypokinesia - diminished postural responses - bradyphrenia - no weakness

EXTRAPYRAMIDAL SYSTEM DYSFUNCTION : 

EXTRAPYRAMIDAL SYSTEM DYSFUNCTION Failure to suppress ? Dyskinesia “Involuntary movements” - tremor - chorea - athetosis - dystonia

TREMORRhythmic, sinusoidal movement : 

TREMORRhythmic, sinusoidal movement Postural / Action Physiologic: - fine, exacerbated by: Thyrotoxicosis Beta agonists Essential: - head and hands (Titubation) spares legs Rubral: - Batwing tremor

TREMORRhythmic, sinusoidal movement : 

Intention: Intention tremor = Cerebellar ( brainstem ) TREMORRhythmic, sinusoidal movement

TREMORRhythmic, sinusoidal movement : 

TREMORRhythmic, sinusoidal movement Rest : -Rest or ‘Parkinsonian’ tremor -Coarse & slow 4-5/s. -Mainly distal. -Associated with rigidity & bradykinesia

Slide 10: 

TREMORRhythmic, sinusoidal movement Differential Diagnosis: - Myoclonus - Asterixes - Tics (Guilles de la Tourette syndrome)

CHOREA : 

CHOREA “dance” in Greek Rapid, forceful and semi-purposeful movement. Interferes with, and deforms voluntary movements . Differential Diagnosis: Tremor Myoclonus

CHOREA Causes : 

CHOREA Causes Drugs : - phenothiazines (Tardive dyskinesia) - methoclopramide & levodopa Sydenham chorea: Rheumatic fever Chorea gravidarum / contraceptives

CHOREA Causes : 

CHOREA Causes Huntington chorea Inherited AD, adulthood chorea & dementia. Focal or diffuse cerebral lesions: Cerebral palsy, Stroke

Slide 14: 

Hemiballismus: lesion to subthalamic nucleos of lewis Commonly ischemic

ATHETOSIS : 

ATHETOSIS Means “Changeable in Greek” Slow, writhing involuntary movement, Mainly distally. Causes Cerebral palsy, kernicterus Drugs (choreo-athetosis) Tardive dyskinesia

DYSTONIA : 

DYSTONIA Involuntary, intermittent -.- persistent abnormal posturing: Focal : spasmodic torticollis, blepharospasm. Generalized : primary torsion dystonia Secondary : drugs, vascular.

PARKINSON’s DISEASE : 

PARKINSON’s DISEASE A common idiopathic neurological disease of the elderly characterized by : Brady-hypokinesia, Rigidity Rest tremor

PARKINSON’s DISEASE Etiology??? Multifactorial : 

PARKINSON’s DISEASE Etiology??? Multifactorial Environmental: - Neurotoxins MPTP ? -Parkinsonism epidemic in young addicts. -Toxic to substantia nigra - Viral infections: Enceph. Lethargica

PARKINSON DISEASE Etiology : 

PARKINSON DISEASE Etiology Genetic: Mutations in essential proteins ? Accumulation of protein aggregate inside the cell “Lewy bodies” ? cell death - Alpha synuclein ? Synucleinopathy

Parkinson’s DiseasePathology : 

Parkinson’s DiseasePathology Substantia Nigra, Locus ceruleus, cingulat gyrus, etc… Proteinacious inclusion bodies: Lewy bodies (a synuclein)

PARKINSON DISEASE Prevalence1.5 : 1000 1% in over 50 years old. : 

PARKINSON DISEASE Prevalence1.5 : 1000 1% in over 50 years old. Natural history: Invariably progressive 25% dead or severely disabled ? 5 yrs 80% dead or severely disabled ? 15 yrs Increase Morbidity and Mortality “tripled”

PARKINSON DISEASE Clinical Features : 

PARKINSON DISEASE Clinical Features Often unilateral ( early ). Exacerbated by anxiety. Ameliorated by relaxation. Disappear during sleep.

PARKINSON DISEASE Clinical Features : 

PARKINSON DISEASE Clinical Features Rest Tremor: presention in 65%. Distal parts: hands, feet, jaw, tongue Head tremor unusual

PARKINSON DISEASE Clinical Features : 

PARKINSON DISEASE Clinical Features Rigidity: Increased tone throughout movement. Lead pipe (plastic) or cogwheel. More in flexors (flexed posture).

Parkinson’s DiseaseClinical features : 

Parkinson’s DiseaseClinical features Hypo-bradykinesia: Main disabling feature Poverty of movements Slowness in initiation and execution FACE EYES SPEECH WALKING POSTURAL REFLEX.

PARKINSON DISEASE Clinical Features : 

PARKINSON DISEASE Clinical Features GI features: Drooling of saliva. Dysphagia. Heart burn. Constipation, Megacolon. Weight loss.

PARKINSON’s DISEASE Clinical Features : 

PARKINSON’s DISEASE Clinical Features Mental Function : Depression. Dementia. Bradyphrenia. Drugs side-effects.

PARKINSON’s DISEASE Diagnosis : 

PARKINSON’s DISEASE Diagnosis Typical Clinical features Elderly L-dopa. Good response When to investigate ? Atypical clinical picture, age. Atypical response to treatment.

Differential Diagnosis : 

Differential Diagnosis Drug-induced parkinsonism Wilson’s disease Essential tremor Multiple system atrophy Progressive supranuclear palsy Multiple lacunar strokes Normal pressure hydrocephalus Pugilistic (post-traumatic) parkinsonism Depression

Differential Diagnosis (Parkinsonism or P. Syndrome) : 

Differential Diagnosis (Parkinsonism or P. Syndrome) Drug induced : Phenothiazines Butyrphenones Reserpine Tetrabenazine Structural lesion: Commonest ? multiple-infarcts

Vascular Parkinsonisms : 

Vascular Parkinsonisms A manifestation of strokes, affecting basal ganglia Features include: pyramidal, cerebellar and mental changes

WILSON’S DISEASE“Hepato-Lenticular Degeneration” : 

WILSON’S DISEASE“Hepato-Lenticular Degeneration” Autosomal recessive Abnormality of copper metabolism Deposit in all organs: Brain: Cortex & Basal Ganglia Cornea Kidneys Liver

WILSON’S DISEASE“Hepato-Lenticular Degeneration” : 

WILSON’S DISEASE“Hepato-Lenticular Degeneration” Clinical Features : Adolescence. Kayser-Fleischer ring. Parkinsonism. Psychiatric – mental changes. Liver cirrhosis. Renal tubular acidosis.

WILSON’S DISEASE“Hepato-Lenticular Degeneration” : 

WILSON’S DISEASE“Hepato-Lenticular Degeneration” INVESTIGATIONS : Liver-Renal functions Copper studies Slit-lamp test Liver biopsy TREATMENT : Penicillamine Trientin Zinc compounds

PARKINSON’s DISEASE Management : 

PARKINSON’s DISEASE Management General measures Pharmaceutical Surgical treatment Restorative «experimental» Transplantation Neurotrophic factors

General measures : 

General measures Education of patient and family Support psychological and emotional needs Regular exercise Proper nutrition

Symptomatic Therapy : 

Symptomatic Therapy L-dopa Dopamine agonists Anticholinergic agents Amantadine Restors neurotransmitter balance or deficiency

L-dopa : 

L-dopa Most effective drug for symptomatic treatment Initiate when akinetic symptoms disabling Use lowest dose that produces response 300 to 600 mg daily

Dopamine Agonists : 

Dopamine Agonists Ergot dopamine agonists Bromocriptine (Parlodel) Pergolide (Permax) Non-ergot dopamine agonists Piribedil (Trivastal) Pramipexole (Mirapex) Ropinirole (Requip) Apomorphine and lisuride IV for rescue therapy in sudden akinetic episodes

Anticholinergic Drugs : 

Anticholinergic Drugs Trihexyphenidyl (Artane) Benztropine (Cogentin) Adverse effects common: Memory impairment, confusion, hallucinations Dry mouth, blurred vision, constipation, nausea, urinary retention, impaired sweating, tachycardia

Surgery, Implantations and Infusions : 

Surgery, Implantations and Infusions Thalamotomy Pallidotomy Deep brain stimulation (DBS) Intra-striatal human retinal cells implant (Spheramine) Growth factor infusion Fetal tissue implant !