PARKINSON'S DISEASE IN PAKISTAN MANAGEMENT ISSUES

PARKINSON’S DISEASE IN PARKINSON’S DISEASE IN PAKISTANPAKISTAN

MANAGEMENT ISSUESMANAGEMENT ISSUES

Prof. Shaukat AliProf. Shaukat AliHead of the Department of NeurologyHead of the Department of Neurology

Jinnah Postgraduate Medical Centre, KarachiJinnah Postgraduate Medical Centre, Karachi

Parkinson's DiseaseParkinson's Disease James Parkinson’s original 1817 describe “shaking palsy” James Parkinson’s original 1817 describe “shaking palsy” now now called parkinsons disease.called parkinsons disease.

Parkinson's disease is a progressive degenerative disorder Parkinson's disease is a progressive degenerative disorder of the of the central nervous system.central nervous system. Idiopathic Parkinson's disease is caused by the Idiopathic Parkinson's disease is caused by the progressive loss of progressive loss of dopaminergic neurons in the substantia nigra and dopaminergic neurons in the substantia nigra and nigrostriatal nigrostriatal pathway of the midbrain and the presence of lewy bodies.pathway of the midbrain and the presence of lewy bodies.

The hallmark physical signs of Parkinson's disease are tremor, rigidity and bradykinesia.

Poor postural reflexes are sometimes included as the fourth hallmark sign. When postural reflexes are inadequate, patients may fall if they are pushed even slightly forward or backward, or if they are standing in a moving vehicle such as a bus or train.

Parkinson’s disease is a disorder of the basal ganglia

Degeneration ofdopamine neuronsin substantia nigra.

These neuronsusually project tothe striatum.

Tremor, slowness of movement (bradykinesia), trouble initiating movement (akinesia), rigidity.Affects 1/250 over 40; 1/100 over 65.

EPIDEMIOLOGYEPIDEMIOLOGY

Parkinson’s disease effect over 1% of Parkinson’s disease effect over 1% of ll peoples>50years old.ll peoples>50years old.

5-10%of patients with PD present at 5-10%of patients with PD present at age <40years.age <40years.

There is a similar incidence in males There is a similar incidence in males and females.and females.

All ethnic group are equally effected.All ethnic group are equally effected.

CLINICAL MENIFESTATION OF CLINICAL MENIFESTATION OF PDPD

Cardinal menifestation:Cardinal menifestation:

Resting tremorResting tremor RigidityRigidity Akinesia/bradykinesiaAkinesia/bradykinesia Postural instabilityPostural instability

Secondary manifestations:Secondary manifestations:

Cognitive dysfunctionCognitive dysfunction Ocular dysfunctionOcular dysfunction Facial and oropharyngeal dysfunctionFacial and oropharyngeal dysfunction Musculoskeletal deformitiesMusculoskeletal deformities Pain and sensory symptomsPain and sensory symptoms Autonomic dysfunctionAutonomic dysfunction Dermatological problemsDermatological problems

Parkinson’s DiseaseParkinson’s Disease PD is a progressive neurological condition causing PD is a progressive neurological condition causing

Physical disabilityPhysical disability Mental disabilityMental disability

Rx does not alter progression of disease Rx does not alter progression of disease helps to alleviate various symptomshelps to alleviate various symptoms helping to live independent & productive liveshelping to live independent & productive lives

Ideal management Ideal management Pharmacological / SurgicalPharmacological / Surgical Psychiatric / psychologicalPsychiatric / psychological MultidisciplinaryMultidisciplinary Social Rehabilitation Social Rehabilitation Health EducationHealth Education

Lack of specialistsLack of specialists

Population Population ~160 ~160 millionmillion

UrbanUrban 35%35% RuralRural 65%65%

No. of available specialistsNo. of available specialists < 100< 100

Lack of awareness - Lack of awareness - amongst healthcare providers amongst healthcare providers General practitioners managing PD General practitioners managing PD

patientspatients Not confident in their diagnosisNot confident in their diagnosis Inadequate Rx prescribedInadequate Rx prescribed Not updated in newer available Rx modalitiesNot updated in newer available Rx modalities Unable to handle the labile course of disorder / Unable to handle the labile course of disorder /

complications / Rx SEcomplications / Rx SE Focus only on pharmacological RxFocus only on pharmacological Rx

Lack of awareness - Lack of awareness - amongst healthcare seekers amongst healthcare seekers

(1)(1) ? Nature of illness? Nature of illness

Consider it to be a part of natural ageing process andConsider it to be a part of natural ageing process and

do not seek medical advicedo not seek medical advice Incorporated in the integrated family systemIncorporated in the integrated family system

? Best Rx provider? Best Rx provider GPGP Medical InternistMedical Internist PsychiatristPsychiatrist NeurosurgeonNeurosurgeon NeurologistNeurologist

Lack of awareness - Lack of awareness - amongst healthcare seekers amongst healthcare seekers

(2)(2) ? Rx options? Rx options

PharmacologicalPharmacological SurgicalSurgical RehabilitationRehabilitation

Expected Rx outcome Expected Rx outcome A “cure” A “cure” Unaware that Rx alleviate symptoms which help live an Unaware that Rx alleviate symptoms which help live an

independent & independent & productive life, Overall improves the QOL productive life, Overall improves the QOL

Rx limitationsRx limitations Drug resistance Drug resistance Side effect – involuntary movements, on-off fluctuations, Side effect – involuntary movements, on-off fluctuations,

dystonic phenomenondystonic phenomenon

Lack of “Holistic Approach”Lack of “Holistic Approach”

“ “Treatment Bias”Treatment Bias” Only pharmacological Rx offeredOnly pharmacological Rx offered Surgical Rx - Limited facilities, costlySurgical Rx - Limited facilities, costly Lack of recent advanced technologiesLack of recent advanced technologies

Lack of Coordinated Multidisciplinary CareLack of Coordinated Multidisciplinary Care PhysiotherapyPhysiotherapy Occupational therapyOccupational therapy Speech therapySpeech therapy Psychiartic / psychological therapyPsychiartic / psychological therapy Social / occupational rehabilitationSocial / occupational rehabilitation Health awarenessHealth awareness

Compliance (1)Compliance (1) CostCost

Rx ExpensiveRx Expensive33% population below national poverty line33% population below national poverty line1% of national budget allotted for health1% of national budget allotted for healthHealth insurance almost non-existentHealth insurance almost non-existent

Low national health priority Low national health priority Infectious diseases of priorityInfectious diseases of priority

No health insuranceNo health insurance

Lack of awarenessLack of awareness Importance of Regularity of RxImportance of Regularity of Rx Long-term RxLong-term Rx Rx limitations – “not curative”, no reversibilityRx limitations – “not curative”, no reversibility Rx side-effectsRx side-effects Rx resistenceRx resistence

Compliance (2)Compliance (2)

Inconsistent LogisticsInconsistent Logistics 65% live in rural areas65% live in rural areas Inconsistent availabilityInconsistent availability

Socio-cultural beliefsSocio-cultural beliefs No cure No RxNo cure No Rx Alternative Rx – faith healer, hakim, homeopath, Alternative Rx – faith healer, hakim, homeopath,

masseur masseur Normal ageing process & easily incorporated in the Normal ageing process & easily incorporated in the

integrated family integrated family systemsystem

SummarySummary Not a national health priority Not a national health priority Few to non-existing facilities for management ofFew to non-existing facilities for management of

chronic diseaseschronic diseases Lack of specialistsLack of specialists Lack of availability of recent Rx advancementsLack of availability of recent Rx advancements Lack of multidisciplinary input Lack of multidisciplinary input Lack of rehabilitative facilities Lack of rehabilitative facilities Lack of sustained logistics Lack of sustained logistics Poor socioeconomic conditions Poor socioeconomic conditions Lack of public health education & awarenessLack of public health education & awareness Easy incorporation in the existing family systemEasy incorporation in the existing family system

NOCTURNAL SYMPTOM COMPLEX OF PDNOCTURNAL SYMPTOM COMPLEX OF PD

Parkinson’s Disease RelatedParkinson’s Disease Related

InsomniaInsomnia Fragmentation of sleep (sleep Fragmentation of sleep (sleep

maintenance insomnia)maintenance insomnia)

Sleep onset insomniaSleep onset insomnia

Motor Function- Motor Function- Akinesia (difficulty turning)Akinesia (difficulty turning)

Related Related Restless Legs Restless Legs

Periodic limb movements of sleep Periodic limb movements of sleep

Urinary Difficulties Urinary Difficulties NocturiaNocturia

Nocturia with secondary posturalNocturia with secondary postural

hypotensionhypotension

Neuropsychiatric/ Neuropsychiatric/ DepressionDepression

ParasomniasParasomnias Vivid dreams Vivid dreams

Altered dream contentAltered dream content

NightmaresNightmares

Night terrorsNight terrors

Sleep talkingSleep talking

Nocturnal vocalisationsNocturnal vocalisations

SomnabulismSomnabulism

HallucinationsHallucinations

Panic attacksPanic attacks

REM Behavior disorderREM Behavior disorder

Treatment-Related:Treatment-Related:Motor: Motor: Nocturnal off-period-related tremorNocturnal off-period-related tremor

DystoniaDystonia

DyskinesiasDyskinesias

Off-period-related pain/ paresthesia/Off-period-related pain/ paresthesia/

muscle crampsmuscle cramps

Off-period-related incontinence of urineOff-period-related incontinence of urine

HAllucinationsHAllucinations

Vivid dreamingVivid dreaming

? Off-Related panic attacks? Off-Related panic attacks

? REM Behavior disorder? REM Behavior disorder

AkathisiaAkathisia

InsomniaInsomnia

Sleep-Altering MedicationsSleep-Altering Medications

Sleep and Parkinson's Sleep and Parkinson's DiseaseDisease

1.1. Sleep disorders secondary to motor Sleep disorders secondary to motor

dysfunction.dysfunction.

2. Sleep disorders secondary to 2. Sleep disorders secondary to behavioral behavioral

dysfunction.dysfunction.

3.3. Sleep disorders associated with Sleep disorders associated with

respiratory dysfunction. respiratory dysfunction.

Autonomic and Vegetative Autonomic and Vegetative Functions in Parkinson’s DiseaseFunctions in Parkinson’s Disease

Bladder SymptomsBladder Symptoms Frequency Frequency

dysfunctiondysfunction

Irritative Irritative Frequency, urgencyFrequency, urgency 57-57-83%83%

ObstructiveObstructive Hesitancy, post-viod dribbilingHesitancy, post-viod dribbiling

17-23%17-23%

Transient and new Transient and new Urinary tract infection Urinary tract infection

Onset incontinenceOnset incontinence MedicationsMedications

Faecal impaction.Faecal impaction.

Chronic incontinenceChronic incontinence ParkinsonismParkinsonism

Lack of mobility Lack of mobility

Anatomic stress incontinenceAnatomic stress incontinence

(women)(women)

Bladder-neck obstruction Bladder-neck obstruction

(prostate in men)(prostate in men)

Other peripheral or centralOther peripheral or central

neurological disordersneurological disorders

Dementia or apathy Dementia or apathy

ParkinsonianParkinsonian Idiopathic parkinsonism with central Idiopathic parkinsonism with central

syndromessyndromes autonomic involvement autonomic involvement

Multiple system atrophyMultiple system atrophy

DrugsDrugs LevodopaLevodopa

Dopamine agonistsDopamine agonists

Amantadine Amantadine

Selegiline (especially combined Selegiline (especially combined with with lovodopa)lovodopa)

AntidepressentsAntidepressents

Sedative hypnoticsSedative hypnotics

AntipsychoticsAntipsychotics

BenzodiazepinesBenzodiazepines

AnalgesicsAnalgesics

Antihypertensive Antihypertensive

VasodilatorsVasodilators

DiureticsDiuretics

Coexistent diseasesCoexistent diseases Autonomic neuropathiesAutonomic neuropathies

(diabetes, alcohol)(diabetes, alcohol)

Brainstem and spinal Brainstem and spinal cord lesionscord lesions

Dehydration, Dehydration, intercurrent illness intercurrent illness

Decreased oral intake Decreased oral intake from dysphagia from dysphagia

Decreased salt intakeDecreased salt intake

Immobility.Immobility.

A.A. Elimination or reduction of hypertensive medications Elimination or reduction of hypertensive medications B.B. Pharmacortisone management Pharmacortisone management FludrocortisoneFludrocortisone PropranololPropranolol ClonidineClonidine

YohimbineYohimbine EphedrineEphedrine

caffeinecaffeine IndomethacinIndomethacin

DomperidoneDomperidoneC.C. Non-pharmacological managementNon-pharmacological management

Sodium chloride tabletsSodium chloride tabletsElevation of the head of the bed 5-20 degreesElevation of the head of the bed 5-20 degreesChanging position slowlyChanging position slowlyPressure stockings, pantyhosePressure stockings, pantyhoseliberalizing salt and fluid intakeliberalizing salt and fluid intakeAvoidance of hot weather, hot tubs or baths, alcohol, Avoidance of hot weather, hot tubs or baths, alcohol, large meals.large meals.Patient and caregiver education.Patient and caregiver education.

Depression and Dementia in Depression and Dementia in Parkinson’s DiseaseParkinson’s Disease

Depression in Parkinson’s DiseaseDepression in Parkinson’s Disease• decreased energydecreased energy• decreased mooddecreased mood• decreased enjoyment of activitiesdecreased enjoyment of activities• decreased appetite decreased appetite • sleep disturbances sleep disturbances • psychomotor dysfunction psychomotor dysfunction • Feelings of worthlessness or guiltFeelings of worthlessness or guilt• problem in concentrationproblem in concentration• indecisivenessindecisiveness• emotional lability emotional lability • thoughts of suicide of death thoughts of suicide of death • pseudo-dementia manifested as forgetfulness.pseudo-dementia manifested as forgetfulness.

TREATMENT OF DEPRESSION IN PDTREATMENT OF DEPRESSION IN PDDEMENTIA IN PARKINSON’S DISEASEDEMENTIA IN PARKINSON’S DISEASE

Psychosis in Parkinson’s DiseasePsychosis in Parkinson’s Disease

• Newer Atypical Antipsychotic DrugsNewer Atypical Antipsychotic Drugs

• ClozapineClozapine

• OlanzapineOlanzapine

• RisperidoneRisperidone

• QuetiapineQuetiapine

• Cholinesterase InhibitorsCholinesterase Inhibitors

• Choice of Drug therapy for psychosis in PD Choice of Drug therapy for psychosis in PD

Basic Principles in the Basic Principles in the Pharmacotherapy of Parkinson’s Pharmacotherapy of Parkinson’s

DiseaseDisease• SUBCLINICAL EARLY ASYMPTOMATIC PDSUBCLINICAL EARLY ASYMPTOMATIC PD• CLINICAL MILDLY SYMPTOMATIC PDCLINICAL MILDLY SYMPTOMATIC PD

• Vitamin E (2000 iu/d)Vitamin E (2000 iu/d)• Selegiline (10 mg/d)Selegiline (10 mg/d)• Riluzole (100-200 mg/d)Riluzole (100-200 mg/d)• Coenzyme Q 10 (300-1200 mg/d)Coenzyme Q 10 (300-1200 mg/d)• Carbidopa/ levodopa (150-600 mg/d)Carbidopa/ levodopa (150-600 mg/d)• BromocriptineBromocriptine

FluctuationsFluctuations• Early morning akinesiaEarly morning akinesia• Delayed onDelayed on• End-of-dose wearing-offEnd-of-dose wearing-off• On-offOn-off• FreezingFreezingDyskinesiaDyskinesia• Off period dystoniaOff period dystonia• Peak dose dyskinesiaPeak dose dyskinesia• Diphasic dyskinesiaDiphasic dyskinesia

TREATEMENT OF ADVANCED TREATEMENT OF ADVANCED PAKINSON’S DISEASEPAKINSON’S DISEASE

• Motor Fluctuations in Advanced PDMotor Fluctuations in Advanced PD • Early Morning AkinesiaEarly Morning Akinesia

• Wearing-offWearing-off • On-offOn-off

• FreezingFreezing

• Off Period DystoniaOff Period Dystonia • Peak-dose DyskinesiaPeak-dose Dyskinesia

• Diphasic DyskinesiaDiphasic Dyskinesia

1. Side effects:1. Side effects: A. Peripheral (and /or central): A. Peripheral (and /or central): a. Nausea, vomiting, anorexia a. Nausea, vomiting, anorexia b. Orthostatic hypotension b. Orthostatic hypotension

B. Central:B. Central:

a. Chorea, stereotypya. Chorea, stereotypy

b. Dystoniab. Dystonia

c. Myoclonusc. Myoclonus

d. Akathesiad. Akathesia

e. Hallucinationse. Hallucinations

2. Motor complications:2. Motor complications:

A. Motor fluctuationsA. Motor fluctuations

a. Delayed onset of responsea. Delayed onset of response

b. Wearing off phenomenonb. Wearing off phenomenon

c. Drug resistant “Off”c. Drug resistant “Off”

d. Random oscillations “On-Off phenomenond. Random oscillations “On-Off phenomenon

e. Freezinge. Freezing

B. DyskinesiasB. Dyskinesias

a. Peak dose dyskinesia (I-D-I)a. Peak dose dyskinesia (I-D-I)

b. Diphasic dyskinesia (D-I-D) b. Diphasic dyskinesia (D-I-D)

THANK YOUTHANK YOU