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Parkinsonism

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Page 1: Parkinsonism
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Parkinson’s disease is a progressiveParkinson’s disease is a progressive

neurodegenerative disorder that neurodegenerative disorder that results inresults in

deterioration of neurons in basal deterioration of neurons in basal ganglia.ganglia.

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EPIDEMIOLOGYEPIDEMIOLOGY

Incidence = 0.2 / 1000Incidence = 0.2 / 1000Prevalence = 1.5 / 1000Prevalence = 1.5 / 1000The incidence and prevalence both The incidence and prevalence both

increase with age.increase with age.Sex incidence is almost equal.Sex incidence is almost equal. It is less common in smokers.It is less common in smokers.

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Typical Age of OnsetTypical Age of Onset

Average age of onset is 60 yrs.Average age of onset is 60 yrs.

5-10% of cases occur under the age 5-10% of cases occur under the age of 40. Referred to as of 40. Referred to as Young-Onset Young-Onset Parkinson Disease.Parkinson Disease.

Rarely seen under age 30.Rarely seen under age 30.

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Famous Faces of ParkinsonFamous Faces of Parkinson

Michael J. Fox

Muhammad Ali

Katharine Hepburn

Pope John Paul IIJohnny Cash

Mao Tse Tung

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EtiologyEtiology

Parkinson’s disease is referred to as Parkinson’s disease is referred to as idiopathic idiopathic (unknown cause).(unknown cause).

Genetic link is seen in a small Genetic link is seen in a small number of Parkinsons cases. number of Parkinsons cases.

Larger genetic link is found in young-Larger genetic link is found in young- onset Parkinsons disease.onset Parkinsons disease.MPTPMPTP cause severe parkinsonism in cause severe parkinsonism in

young drug usersyoung drug users

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Risk factorsRisk factors

Positive family history Positive family history Male genderMale genderHead injuryHead injuryExposure to pesticideExposure to pesticideConsumption of well waterConsumption of well waterRural livingRural living

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Factors which decrease Factors which decrease incidence of PDincidence of PD

SmokingSmokingCoffee drinkingCoffee drinkingUse of NSAIDSUse of NSAIDSEstrogen replacement in Estrogen replacement in

postmenopausal women postmenopausal women

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Repeated head traumaRepeated head trauma Infectious & post infectious diseaseInfectious & post infectious diseaseDrugs (neuroleptics, antipsychotics , Drugs (neuroleptics, antipsychotics ,

alpha methyldopa, lithium carbonate , alpha methyldopa, lithium carbonate , fluoxetine )fluoxetine )

ToxinsToxinsMPTP MPTP CyanidesCyanidesMethanol Methanol

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Unilateral ParkinsonismUnilateral Parkinsonism

Vascular Vascular TraumaticTraumaticNeoplasm Neoplasm

Lower body ParkinsonismLower body Parkinsonism

It may represent a form of vascular It may represent a form of vascular parkinsonismparkinsonism

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Parkinsonism plus syndromeParkinsonism plus syndrome

Features of parkinsonism associated with Features of parkinsonism associated with complex clinical presentation complex clinical presentation

Failure to respond to treatmentFailure to respond to treatment Worse prognosisWorse prognosis

Examples include : Examples include :

1. Shy Dragger syndrome1. Shy Dragger syndrome

2. Steel Richardson syndrome2. Steel Richardson syndrome

3. Parkinsonism-dementia 3. Parkinsonism-dementia plusplus

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PATHOLOGYPATHOLOGY

There is depletion of pigmented There is depletion of pigmented dopaminergic neurons in the dopaminergic neurons in the substantia nigra, atrophic changes in substantia nigra, atrophic changes in substantia nigra and depletion of substantia nigra and depletion of neurons in locus coeruleus.neurons in locus coeruleus.

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PATHOLOGYPATHOLOGY

Reduced dopaminergic output from Reduced dopaminergic output from the substantia nigra to globus the substantia nigra to globus pallidus leads to reduced inhibitory pallidus leads to reduced inhibitory effect on subthalamic nucleus, effect on subthalamic nucleus, neurons of which become more neurons of which become more active in inhibiting activation of active in inhibiting activation of cortex resulting in bradykinesia.cortex resulting in bradykinesia.

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CLINICAL FEATURESCLINICAL FEATURES

Tremors TTremors TRigidity RRigidity RAkinesia / Bradykinesia Akinesia / Bradykinesia

AAPostural instability PPostural instability P

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Head bent forward

Tremors of head

Maslike facial expression

Drooling

Rigidity

Stooped posture

Weight loss

Akinesia

tremors

Loss of postural reflexes

Propulsive gait

CLINICAL FEATURES

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General FeaturesGeneral Features

Expressionless face (mask like)Expressionless face (mask like)Greasy skinGreasy skinSoft rapid indistinct speech, Soft rapid indistinct speech,

monotonousmonotonousGlabellar tap sign / Myersons signGlabellar tap sign / Myersons signFlexed postureFlexed posture Impaired postural reflexesImpaired postural reflexes

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GaitGait

Slow to start walkingSlow to start walkingShortened strideShortened strideRapid small steps tendency to turn Rapid small steps tendency to turn

en blocen blocReduced arm swingReduced arm swing Impaired balance on turningImpaired balance on turning

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TremorsTremors

Resting (4 - 6 Hz)Resting (4 - 6 Hz)

Postural (8-10 Hz)Postural (8-10 Hz)

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RigidityRigidity

Cogwheel :Cogwheel :

mostly in upper limbsmostly in upper limbs

Plastic / lead-pipe:Plastic / lead-pipe:

mostly in lower limbsmostly in lower limbs

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BradykinesiaBradykinesia

Slowness in initiation or repeating Slowness in initiation or repeating movementsmovements

Impaired fine movementsImpaired fine movements

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Hoehn & Yahr ScaleHoehn & Yahr Scale

Stage IStage I: Unilateral involvement only, usually : Unilateral involvement only, usually with minimal or no function impairment.with minimal or no function impairment.

Stage IIStage II: Bilateral or midline involvement : Bilateral or midline involvement w/o impairment of balance.w/o impairment of balance.

Stage IIIStage III: 1: 1stst signs of righting reflex signs of righting reflex impairment; functionally restricted in impairment; functionally restricted in his/her activities but can lead independent his/her activities but can lead independent life. Disability mild/moderate.life. Disability mild/moderate.

Stage IVStage IV: Severely disabled. Able to walk & : Severely disabled. Able to walk & stand unaided but is markedly handicapped.stand unaided but is markedly handicapped.

Stage VStage V: Confined to bed.: Confined to bed.

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InvestigationsInvestigationsThere is no specific medical test used There is no specific medical test used

to diagnose Parkinson disease. to diagnose Parkinson disease.

However, However, MRIsMRIs and and blood testsblood tests are are used to rule out other possible used to rule out other possible conditions that have similar conditions that have similar symptoms to Parkinson disease.symptoms to Parkinson disease.

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TREATMENTTREATMENT

Drug therapyDrug therapySurgerical Treatment Surgerical Treatment Physiotherapy Physiotherapy Speech therapySpeech therapy

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LEVODOPALEVODOPA

It is the oldest and most effective treatment It is the oldest and most effective treatment ofof

PD. PD. Brain enzymes modify the drug to create Brain enzymes modify the drug to create

dopamine.dopamine. It reduces slowness and stiffness of muscles.It reduces slowness and stiffness of muscles. Given in combination with peripheral Given in combination with peripheral

decarboxylase inhibitors, carbidopa & decarboxylase inhibitors, carbidopa & benserazide.benserazide.

Combinations are called Sinemet & Madopar Combinations are called Sinemet & Madopar respectively.respectively.

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Adverse effectsAdverse effects:: Nausea, vomiting, Nausea, vomiting,

hypotension,orofacial dyskinesias, hypotension,orofacial dyskinesias, dystonias, hallucinations.dystonias, hallucinations.

Two important phenomenon Two important phenomenon associated with levodopa use are:associated with levodopa use are:

1) end- of- dose deterioration1) end- of- dose deterioration 2) on-off phenomenon2) on-off phenomenon

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Useful effects on tremors & rigidity butUseful effects on tremors & rigidity but do not help bradykinesia.do not help bradykinesia.

Adverse effects :Adverse effects : Dry mouth , blurred vision , Dry mouth , blurred vision ,

difficult difficult urination , constipation, urination , constipation, confusion, confusion,

hallucinations hallucinations

Anticholinergic agentsAnticholinergic agents

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AmantadineAmantadine

Useful in controlling dyskinesia Useful in controlling dyskinesia produced by dopaminergic treatment produced by dopaminergic treatment later in the disease.later in the disease.

Adverse effects:Adverse effects:

livedo reticularis , peripheral livedo reticularis , peripheral edema, edema,

confusion , seizures confusion , seizures

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Dopamine receptor agonistsDopamine receptor agonists

Bromocriptine , lisuride , Bromocriptine , lisuride , pergolide ,cabergoline , ropinirole & pergolide ,cabergoline , ropinirole & pramipexolepramipexole

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OTHER DRUGSOTHER DRUGS

COMT inhibitors:COMT inhibitors:

Catechol-O-methyl transferase Catechol-O-methyl transferase inhibitorsinhibitors

SelegelineSelegeline

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Surgical procedures are now Surgical procedures are now available for specific patients who no available for specific patients who no longer respond to drug treatments.longer respond to drug treatments.

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PALLIDOTOMYPALLIDOTOMY

It is a neurosurgical procedure that can It is a neurosurgical procedure that can reduce many of the symptoms of reduce many of the symptoms of Parkinson DiseaseParkinson Disease

Performed by a neurosurgeon, who Performed by a neurosurgeon, who inserts a hallow probe into the globus inserts a hallow probe into the globus pallidus and then liquid nitrogen is pallidus and then liquid nitrogen is circulated in the probe.circulated in the probe.

The probe destroys part of the globus The probe destroys part of the globus pallidus by creating a scar .pallidus by creating a scar .

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The patient is awake during the The patient is awake during the procedure which takes about 6 hours.procedure which takes about 6 hours.It can permanently eliminate It can permanently eliminate

dyskinesiasdyskinesiasReduces tremor, rigidity, Reduces tremor, rigidity,

bradykinesia and shuffling gait.bradykinesia and shuffling gait.It is not a cure.It is not a cure.

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Stereotatic thalamotomyStereotatic thalamotomy

Used to treat tremors Used to treat tremors

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Deep Brain StimulationDeep Brain Stimulation

An electrode is placed in one of several An electrode is placed in one of several parts of the brain (thalamus, globus parts of the brain (thalamus, globus palladus, or subthalamic nucleus).palladus, or subthalamic nucleus).

The electrode is attached to a The electrode is attached to a computerized pulse-generator which is computerized pulse-generator which is put under this skin in the chest.put under this skin in the chest.

Symptoms are relieved through the Symptoms are relieved through the regulation of electrical impulses from regulation of electrical impulses from those three areas of the brain.those three areas of the brain.

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Deep Brain StimulationDeep Brain Stimulation

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Current ResearchCurrent ResearchNeural graftingNeural grafting, or transplantation of , or transplantation of

nerve cells, is an experimental nerve cells, is an experimental technique proposed for treating the technique proposed for treating the disease. Investigators have shown in disease. Investigators have shown in animal models that implanting fetal animal models that implanting fetal brain tissue from the substantia nigra brain tissue from the substantia nigra into a parkinsonian brain causes into a parkinsonian brain causes damaged nerve cells to regenerate. damaged nerve cells to regenerate.

Gene TherapyGene Therapy

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General Treatment StrategiesGeneral Treatment Strategies

Develop routine for self careDevelop routine for self care Help family/care partner, along with Help family/care partner, along with

patient, create ideas for adapting home patient, create ideas for adapting home environment to meet the patient’s needs. environment to meet the patient’s needs. Modified kitchenModified kitchenModified bathroom Modified bathroom Full bathroom and bedroom on first Full bathroom and bedroom on first

floorfloorLift chairLift chairRemove rugs/carpet/furniture that Remove rugs/carpet/furniture that

could be easily tripped overcould be easily tripped over

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Social ParticipationSocial Participation

Support groupsSupport groups Important to encourage continued Important to encourage continued

involvement in activities important to involvement in activities important to their life. their life.

Participation in activities which are Participation in activities which are not physically taxing.not physically taxing.

Introduce techniques for enhancing Introduce techniques for enhancing voice volume.voice volume.

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Educate & inform co-workersEducate & inform co-workersExplore work-at-home optionsExplore work-at-home optionsAdapt job requirements as Adapt job requirements as

disease progressesdisease progresses

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PrognosisPrognosis

Depends upon the age of onsetDepends upon the age of onset If symptom starting in middle life ,the If symptom starting in middle life ,the

disease is usually progressive & disease is usually progressive & likely to shorten lifespan.likely to shorten lifespan.

After 70 is unlikely to shorten life or After 70 is unlikely to shorten life or become severe.become severe.

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