extrapyramidal system disorders
TRANSCRIPT
““EXTRAPYRAMIDAL” EXTRAPYRAMIDAL” MOVEMENT DISORDERSMOVEMENT DISORDERS
Prof. AR AlTahanDivision of Neurology
EXTRAPYRAMIDAL SYSTEMEXTRAPYRAMIDAL SYSTEM
ANATOMY:– Caudate nucleus– Putamen– Globus pallidus– Subthalamic nucleus– Substantia nigra
These structures are functionally connected with the Thalamus and Pre-motor Cortex
EXTRAPYRAMIDAL SYSTEMEXTRAPYRAMIDAL SYSTEM
PHYSIOLOGY Influence the details of a movement planModifies the order-plan “Pyramidal plan” Affects motor function by either: FACILITATION
SUPPRESSION
EXTRAPYRAMIDAL SYSTEMEXTRAPYRAMIDAL SYSTEM
PATHO-PHYSIOLOGY:
Failure to facilitate
Failure to suppress
EXTRAPYRAMYDAL SYSTEM EXTRAPYRAMYDAL SYSTEM DYSFUNCTIONDYSFUNCTION
(1) Failure to facilitate- brady-hypokinesia- diminished postural responses- bradyphrenia- no weakness
EXTRAPYRAMIDAL SYSTEM EXTRAPYRAMIDAL SYSTEM DYSFUNCTIONDYSFUNCTION
(2) Failure to suppress Dyskinesia “Involuntary movements”- tremor- chorea- athetosis- dystonia
TREMORTREMORRhythmic, sinusoidal movementRhythmic, sinusoidal movement
1. Postural / Action Physiologic: - fine, exacerbated by:
Thyrotoxicosis Beta agonists
Essential: - head and hands (Titubation)
spares legsRubral: - Batwing tremor
2.Intention:Intention tremor = Cerebellar
( brainstem )
TREMORTREMORRhythmic, sinusoidal Rhythmic, sinusoidal
movementmovement
TREMORTREMORRhythmic, sinusoidal movementRhythmic, sinusoidal movement
3. Rest :-Rest or ‘Parkinsonian’ tremor -Coarse & slow 4-5/s.-Mainly distal.-Associated with rigidity & bradykinesia
TREMORRhythmic, sinusoidal movement
Differential Diagnosis:
- Myoclonus
- Asterixes - Tics (Guilles de la Tourette syndrome)
CHOREACHOREA
“dance” in Greek – Rapid, forceful and semi-
purposeful movement.– Interferes with, and deforms
voluntary movements .Differential Diagnosis:
– Tremor– Myoclonus
CHOREACHOREA CausesCauses
1. Drugs : - phenothiazines (Tardive dyskinesia)
- methoclopramide & levodopa2. Sydenham chorea: Rheumatic
fever3. Chorea gravidarum /
contraceptives
CHOREACHOREA CausesCauses
4. Huntington chorea Inherited AD, adulthood chorea & dementia.
5. Focal or diffuse cerebral lesions: Cerebral palsy, Stroke
Hemiballismus:– lesion to subthalamic
nucleos of lewis– Commonly ischemic
ATHETOSISATHETOSIS
Means “Changeable in Greek” Slow, writhing involuntary
movement, Mainly distally.Causes
Cerebral palsy, kernicterusDrugs (choreo-athetosis)
Tardive dyskinesia
DYSTONIADYSTONIA
Involuntary, intermittent -.- persistent abnormal posturing:– Focal : spasmodic torticollis,
blepharospasm.– Generalized : primary torsion dystonia– Secondary : drugs, vascular.
PARKINSON’s DISEASEPARKINSON’s DISEASE
A common idiopathic neurological disease of the elderly characterized by :
Brady-hypokinesia, Rigidity Rest tremor
PARKINSON’s DISEASEPARKINSON’s DISEASE Etiology??? Etiology???
Multifactorial Multifactorial 1. Environmental:
- Neurotoxins MPTP -Parkinsonism epidemic
in young addicts. -Toxic to substantia nigra
- Viral infections: Enceph. Lethargica
PARKINSON DISEASEPARKINSON DISEASE Etiology Etiology
2. Genetic:Mutations in essential proteins Accumulation of protein aggregate inside the cell “Lewy bodies” cell death - Alpha synuclein Synucleinopathy
ParkinsonParkinson’’s Diseases DiseasePathologyPathology
Substantia Nigra, Locus ceruleus, cingulat gyrus, etc…
Proteinacious inclusion bodies:Lewy bodies(a synuclein)
PARKINSON DISEASEPARKINSON DISEASE PrevalencePrevalence1.5 : 10001.5 : 1000
1% in over 50 years old. 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 DISEASEPARKINSON DISEASE Clinical FeaturesClinical Features
Often unilateral ( early ). Exacerbated by anxiety. Ameliorated by relaxation. Disappear during sleep.
PARKINSON DISEASEPARKINSON DISEASE Clinical FeaturesClinical Features
Rest Tremor: presention in 65%.
Distal parts: hands, feet, jaw, tongue Head tremor unusual
PARKINSON DISEASEPARKINSON DISEASE Clinical FeaturesClinical Features
Rigidity: Increased tone throughout
movement. Lead pipe (plastic) or cogwheel. More in flexors (flexed posture).
ParkinsonParkinson’’s Diseases DiseaseClinical featuresClinical features
Hypo-bradykinesia: Main disabling feature Poverty of movements Slowness in initiation
and executionFACE
EYES
SPEECH WALKING
POSTURAL REFLEX.
PARKINSON DISEASEPARKINSON DISEASE Clinical FeaturesClinical Features
GI features: Drooling of saliva. Dysphagia. Heart burn. Constipation, Megacolon. Weight loss.
PARKINSON’s DISEASEPARKINSON’s DISEASE Clinical FeaturesClinical Features
Mental Function :Depression.Dementia.Bradyphrenia.Drugs side-effects.
PARKINSON’s DISEASEPARKINSON’s DISEASE DiagnosisDiagnosis
Typical Clinical featuresElderly L-dopa. Good response
When to investigate ? (1) Atypical clinical picture, age.(2) Atypical response to
treatment.
Differential DiagnosisDifferential 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 DiagnosisDifferential Diagnosis (Parkinsonism or P. Syndrome)(Parkinsonism or P. Syndrome)
Drug induced :–Phenothiazines–Butyrphenones–Reserpine–Tetrabenazine
Structural lesion:–Commonest multiple-infarcts
Vascular ParkinsonismsVascular Parkinsonisms
A manifestation of strokes, affecting basal ganglia
Features include: pyramidal, cerebellar and mental changes
WILSON’S DISEASEWILSON’S DISEASE““Hepato-Lenticular Degeneration”Hepato-Lenticular Degeneration”
Autosomal recessiveAbnormality of copper metabolismDeposit in all organs:
–Brain: Cortex & Basal Ganglia –Cornea –Kidneys–Liver
WILSON’S DISEASEWILSON’S DISEASE““Hepato-Lenticular Degeneration”Hepato-Lenticular Degeneration”
Clinical Features :– Adolescence.– Kayser-Fleischer ring.– Parkinsonism.– Psychiatric – mental changes.– Liver cirrhosis.– Renal tubular acidosis.
WILSON’S DISEASEWILSON’S DISEASE““Hepato-Lenticular Degeneration”Hepato-Lenticular Degeneration”
INVESTIGATIONS :– Liver-Renal functions– Copper studies– Slit-lamp test– Liver biopsy
TREATMENT :– Penicillamine – Trientin– Zinc compounds
PARKINSON’s DISEASEPARKINSON’s DISEASE ManagementManagement
General measuresPharmaceuticalSurgical treatmentRestorative «experimental»
– Transplantation
– Neurotrophic factors
General measuresGeneral measures
Education of patient and family
Support psychological and emotional needs
Regular exercise Proper nutrition
Symptomatic TherapySymptomatic Therapy
L-dopaDopamine agonistsAnticholinergic agentsAmantadine
Restors neurotransmitter balance or deficiency
L-dopaL-dopa
Most effective drug for symptomatic treatment– Initiate when akinetic symptoms
disabling– Use lowest dose that produces
response300 to 600 mg daily
Dopamine AgonistsDopamine 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 DrugsAnticholinergic 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 Surgery, Implantations and InfusionsInfusions
Thalamotomy Pallidotomy Deep brain stimulation
(DBS) Intra-striatal human
retinal cells implant (Spheramine)
Growth factor infusion Fetal tissue implant !