PARKINSON’S DISEASE IN PAKISTAN MANAGEMENT ISSUES

1. PARKINSON’S DISEASE IN PAKISTANMANAGEMENT ISSUES Prof. Shaukat Ali Head of the Department of Neurology Jinnah Postgraduate Medical Centre, Karachi

2. Parkinson's Disease James Parkinson’s original 1817 describe “shaking palsy” now called parkinsons disease. Parkinson's disease is a progressive degenerative disorder of the central nervous system. Idiopathic Parkinson's disease is caused by the progressive loss of dopaminergic neurons in the substantia nigra and nigrostriatal 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.

3. Parkinson’s disease is a disorder of the basal ganglia Degeneration of dopamine neurons in substantia nigra. These neurons usually project to the striatum. Tremor, slowness of movement (bradykinesia), trouble initiating movement (akinesia), rigidity. Affects 1/250 over 40; 1/100 over 65.

4. EPIDEMIOLOGY • Parkinson’s disease effect over 1% of ll peoples>50years old. • 5-10%of patients with PD present at age <40years. • There is a similar incidence in males and females. • All ethnic group are equally effected.

5. CLINICAL MENIFESTATION OF PD Cardinal menifestation: • Resting tremor • Rigidity • Akinesia/bradykinesia • Postural instability Secondary manifestations: • Cognitive dysfunction • Ocular dysfunction • Facial and oropharyngeal dysfunction • Musculoskeletal deformities • Pain and sensory symptoms • Autonomic dysfunction • Dermatological problems

6. Parkinson’s Disease • PD is a progressive neurological condition causing • Physical disability • Mental disability • Rx does not alter progression of disease • helps to alleviate various symptoms • helping to live independent & productive lives • Ideal management • Pharmacological / Surgical • Psychiatric / psychological • Multidisciplinary • Social Rehabilitation • Health Education

7. Lack of specialists • Population ~160 million • Urban 35% • Rural 65% • No. of available specialists < 100

8. Lack of awareness - amongst healthcare providers • General practitioners managing PD patients • Not confident in their diagnosis • Inadequate Rx prescribed • Not updated in newer available Rx modalities • Unable to handle the labile course of disorder / complications / Rx SE • Focus only on pharmacological Rx

9. Lack of awareness - amongst healthcare seekers (1) • ? Nature of illness • Consider it to be a part of natural ageing process and do not seek medical advice • Incorporated in the integrated family system • ? Best Rx provider • GP • Medical Internist • Psychiatrist • Neurosurgeon • Neurologist

10. Lack of awareness - amongst healthcare seekers (2) • ? Rx options • Pharmacological • Surgical • Rehabilitation • Expected Rx outcome • A “cure” • Unaware that Rx alleviate symptoms which help live an independent & productive life, Overall improves the QOL • Rx limitations • Drug resistance • Side effect – involuntary movements, on-off fluctuations, dystonic phenomenon

11. Lack of “Holistic Approach” • “Treatment Bias” • Only pharmacological Rx offered • Surgical Rx - Limited facilities, costly • Lack of recent advanced technologies • Lack of Coordinated Multidisciplinary Care • Physiotherapy • Occupational therapy • Speech therapy • Psychiartic / psychological therapy • Social / occupational rehabilitation • Health awareness

12. Compliance (1) • Cost • Rx Expensive • 33% population below national poverty line • 1% of national budget allotted for health • Health insurance almost non-existent • Low national health priority • Infectious diseases of priority • No health insurance • Lack of awareness • Importance of Regularity of Rx • Long-term Rx • Rx limitations – “not curative”, no reversibility • Rx side-effects • Rx resistence

13. Compliance (2) • Inconsistent Logistics • 65% live in rural areas • Inconsistent availability • Socio-cultural beliefs • No cure No Rx • Alternative Rx – faith healer, hakim, homeopath, masseur • Normal ageing process & easily incorporated in the integrated family system

14. Summary • Not a national health priority • Few to non-existing facilities for management of chronic diseases • Lack of specialists • Lack of availability of recent Rx advancements • Lack of multidisciplinary input • Lack of rehabilitative facilities • Lack of sustained logistics • Poor socioeconomic conditions • Lack of public health education & awareness • Easy incorporation in the existing family system

15. Thank you

16. NOCTURNAL SYMPTOM COMPLEX OF PD Parkinson’s Disease Related Insomnia Fragmentation of sleep (sleep maintenance insomnia) Sleep onset insomnia Motor Function- Akinesia (difficulty turning) Related Restless Legs Periodic limb movements of sleep Urinary Difficulties Nocturia Nocturia with secondary postural hypotension Neuropsychiatric/ Depression Parasomnias Vivid dreams Altered dream content Nightmares Night terrors Sleep talking Nocturnal vocalisations Somnabulism Hallucinations Panic attacks REM Behavior disorder

17. Treatment-Related: Motor: Nocturnal off-period-related tremor Dystonia Dyskinesias Off-period-related pain/ paresthesia/ muscle cramps Off-period-related incontinence of urine HAllucinations Vivid dreaming ? Off-Related panic attacks ? REM Behavior disorder Akathisia Insomnia Sleep-Altering Medications

18. Sleep and Parkinson's Disease • Sleep disorders secondary to motor dysfunction. 2. Sleep disorders secondary to behavioral dysfunction. • Sleep disorders associated with respiratory dysfunction.

19. Autonomic and Vegetative Functions in Parkinson’s Disease • Bladder Symptoms Frequency dysfunction • Irritative Frequency, urgency 57-83% • Obstructive Hesitancy, post-viod dribbiling17-23%

20. Transient and new Urinary tract infection Onset incontinence Medications Faecal impaction. Chronic incontinence Parkinsonism Lack of mobility Anatomic stress incontinence (women) Bladder-neck obstruction (prostate in men) Other peripheral or central neurological disorders Dementia or apathy

21. Parkinsonian Idiopathic parkinsonism with central syndromes autonomic involvement Multiple system atrophy DrugsLevodopa Dopamine agonists Amantadine Selegiline (especially combined with lovodopa) Antidepressents Sedative hypnotics Antipsychotics Benzodiazepines Analgesics Antihypertensive Vasodilators Diuretics

22. Coexistent diseases Autonomic neuropathies (diabetes, alcohol) Brainstem and spinal cord lesions Dehydration, intercurrent illness Decreased oral intake from dysphagia Decreased salt intake Immobility.

23. Elimination or reduction of hypertensive medications • Pharmacortisone management Fludrocortisone Propranolol Clonidine Yohimbine Ephedrine caffeine Indomethacin Domperidone • Non-pharmacological management Sodium chloride tablets Elevation of the head of the bed 5-20 degrees Changing position slowly Pressure stockings, pantyhose liberalizing salt and fluid intake Avoidance of hot weather, hot tubs or baths, alcohol, large meals. Patient and caregiver education.

24. Depression and Dementia in Parkinson’s Disease Depression in Parkinson’s Disease • decreased energy • decreased mood • decreased enjoyment of activities • decreased appetite • sleep disturbances • psychomotor dysfunction • Feelings of worthlessness or guilt • problem in concentration • indecisiveness • emotional lability • thoughts of suicide of death • pseudo-dementia manifested as forgetfulness. TREATMENT OF DEPRESSION IN PD DEMENTIA IN PARKINSON’S DISEASE

25. Psychosis in Parkinson’s Disease • Newer Atypical Antipsychotic Drugs • Clozapine • Olanzapine • Risperidone • Quetiapine • Cholinesterase Inhibitors • Choice of Drug therapy for psychosis in PD

26. Basic Principles in the Pharmacotherapy of Parkinson’s Disease • SUBCLINICAL EARLY ASYMPTOMATIC PD • CLINICAL MILDLY SYMPTOMATIC PD • Vitamin E (2000 iu/d) • Selegiline (10 mg/d) • Riluzole (100-200 mg/d) • Coenzyme Q 10 (300-1200 mg/d) • Carbidopa/ levodopa (150-600 mg/d) • Bromocriptine

28. Fluctuations • Early morning akinesia • Delayed on • End-of-dose wearing-off • On-off • Freezing Dyskinesia • Off period dystonia • Peak dose dyskinesia • Diphasic dyskinesia

29. TREATEMENT OF ADVANCED PAKINSON’S DISEASE • Motor Fluctuations in Advanced PD • Early Morning Akinesia • Wearing-off • On-off • Freezing • Off Period Dystonia • Peak-dose Dyskinesia • Diphasic Dyskinesia

30. 1. Side effects: A. Peripheral (and /or central): a. Nausea, vomiting, anorexia b. Orthostatic hypotension B. Central: a. Chorea, stereotypy b. Dystonia c. Myoclonus d. Akathesia e. Hallucinations 2. Motor complications: A. Motor fluctuations a. Delayed onset of response b. Wearing off phenomenon c. Drug resistant “Off” d. Random oscillations “On-Off phenomenon e. Freezing B. Dyskinesias a. Peak dose dyskinesia (I-D-I) b. Diphasic dyskinesia (D-I-D)

31. THANK YOU