extrapyramidal system disorders

““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 !