PARKINSONS DISEASE Chapter 18 Physical Rehabilitation Prepared by

PARKINSON’S DISEASE Chapter 18 Physical Rehabilitation Prepared by: Pethuel M. Pomaloy

Characteristics • Progressive disorder of CNS • Disturbance in the dopamine system of basal nuclei • Both motor and non-motor symptoms • Cardinal Features: • Rigidity • Bradykinesia • Tremor • Postural instability

Incidence • 1 million Americans • 7 – 10 people worldwide • Average of onset: 50 – 60 years old • 4 – 10%: Early onset PD (< 40 years old) • Juvenile onset (< 21 y. o. ) • Young onset PD (21 – 40 y. o. ) • Men: 1. 2 to 1. 5 times more frequent

Etiology • Parkinsonism • Generic term used to describe a group of disorders with primary disturbances in the dopamine system of the basal ganglia (BG). • PD/ Idiopathic parkinsonism • most common form (78%) • Secondary parkinsonism • results from a number of identifiable causes including viruses, toxins, drugs, tumors etc. • Parkinson-plus syndromes • Conditions that mimic PD in some respects but are caused by other neurodegenerative diseases

Etiology 1. Parkinson’s Disease • first described as “shaking palsy” by James Parkinson in 1817 • Refers to cases where etiology is unknown or genetically determined • Clinical subgroups: • Postural Instability and Gait Disturbed (PIGD) • Tremor Predominant

Etiology 2. Secondary Parkinsonsim • A. Postencephalitic Parkinsonism • influenza epidemics of encephalitis lethargica (1917 to 1926) • onset of parkinsonian symptoms typically occurred after many years

Etiology 2. Secondary Parkinsonsim • B. Toxic Parkinsonism • Environmental toxins (pesticides) • Industrial chemicals (carbon disulfide, carbon monoxide, cyanide, methanol) • Most common: Manganese

Etiology 2. Secondary Parkinsonsim • C. Drug induced parkinsonsim (DIP) • drugs that produce extrapyramidal dysfunctions that mimics sign of PD • interfere dopaminergic mechanisms • Neuroleptic drugs: chlorpromazine, haloperidol, thirodiazine • Antidepressant drugs: amitriptyline, amoxapine, trazodone • Antihypertensive drugs: methyldopa (aldomet), reserpine

Etiology 3. Parkinson-Plus syndrome • Neurodegenerative diseases that affects substantia nigra and produce parkinsonian symptoms along with other neurological signs • Striatonigral degeneration (SND) • Shy-Drager syndrome • Progressive supranuclear palsy • Olivopontocerebellar atrophy • Cortical basal ganglionic degeneration

Etiology 3. Parkinson-Plus syndrome • Multi-infarct vascular disease • Alzheimer’s disease • Diffuse Lewy body disease • Normal pressure hydrocephalus • Creutzfeldt-Jakob disease • Wilson’s disease • Juvenile huntington’s disease • Diagnostic feature: do not show measurable improvement to anti- parkinson medication such as levodopa (apomorphine test)

Pathophysiology • Degeneration of the dopaminergic neurons in the BG in the pars compactus of the substantia nigra • As neurons degenerate, presence of cytoplasmic inclusion bodies called Lewy Bodies • Onset of motor symptoms 30 -60% degeneration

Pathophysiology

Pathophysiology • Direct Motor Loop • Ventrolateral thalamus to Cortex (Supplementary Motor Area) • Excitatory and facilitates discharge of cells in the SMA • Indirect Motor Loop • Involves the subthalamic nucleus, globus pallidus interna and substantia nigra pars reticulata to midbrain tegmentum • Inhibits/ decreases thalamocortical projections

Clinical Presentation Primary Motor Symptoms • Rigidity • Increased resistance to passive motion that is constant regardless of the task, amplitude or speed of movement • Both agonist and antagonistic muscles • Often assymmetrical during early stage • Proximal muscles first • Active movement, mental concentration, emotional stress increase rigidity • Types: • Cogwheel: jerky, ratchet like, muscles alternate tense and relax • Lead pipe: sustained resitance, (-) fluctuations

Clinical Presentation Primary Motor Symptoms • Bradykinesia • Slowness of movement • Insufficient recruitment of muscle force during initiation of movement • Akinesia – poverty of spontaneous movements; ex. Hypomimia • Freezing episodes • Hypokinesia – slowed and reduced movements (micrographia)

Clinical Presentation Primary Motor Symptoms • Tremor • involuntary shaking or oscillating movement of a part or parts of the body resulting from contractions of opposite muscles • Resting tremor: suppressed by rest and disappears with sleep • Postural tremor • Action tremor – seen in advanced PD

Clinical Presentation Primary Motor Symptoms • Postural Instability • Rare in first 5 years • Abnormal and inflexible postural responses • Difficulty in regulating feed forward anticipatory judgements • Weakness of antigravity muscles: adoption of a flexed, stooped posture with increased flexion of trunk, neck, hips and knees • LE: hip and knee flexors, hip rotators, adductors, PFors • Spine: dorsal spine and neck flexors • UE: shoulder adductors and IR, elbow flexors • Frequent falls and fall injuries

Clinical Presentation Secondary Motor Symptoms • Muscle performance • Reduction in strength that may be dopamine related • EMG studies: motor unit recruitment delayed with under -recruitment of muscles • Disuse weakness • Fatigue: difficulty in sustaining activity and experiences weakness and lethargy as the day progresses

Clinical Presentation Secondary Motor Symptoms • Motor Function • Motor planning deficits: loss of voluntary and automatic movement responses • Speed – Accuracy Trade-off • Dual-task control problems • Start Hesitation • Motor learning deficits may be seen but not universal

Clinical Presentation Secondary Motor Symptoms • Gait • 13 -33% of patients presents with postural instability and gait disturbance as their initial motor symptom • Reduction in arm swing with assymetry • Festinating gait • progressive increase in speed with shortening of stride • anteropulsive / retropulsive • Problems in turning and changing direction

Clinical Presentation Nonmotor Symptoms • Sensory Symptoms • Do not suffer primary sensory loss • 50% experience paresthesias and pain, sensation of numbness, tingling, • • • cold, aching pain and burning Postural stress syndromes Proprioceptive and Visuospatial deficits Olfactory dysfunction: Anosmia Visual disturbance caused by conventional drugs (anticholinergics): blurred vision, sensitivity to light (photophobia), eye pursuit may be jerky Decreased blinking

Clinical Presentation Nonmotor Symptoms • Dysphagia • present in 95% of patients with PD • Result of rigidity, reduced mobility and restricted range of movement • Excessive drooling (sialorrhea): increased saliva production, decreased spontaneous swallowing • Can be present in all stages

Clinical Presentation Nonmotor Symptoms • Speech Disorders • Hypokinetic dysarthria • Decreased voice volume, monotone, imprecise articulation, uncontrolled speech rate • Mutism

Clinical Presentation Nonmotor Symptoms • Cognitive Dysfunctions • mild (mildly impaired memory) or severe (psychosis) • PD dementia occurs in approximately 20% to 40% of the patients • Bradyphrenia

Clinical Presentation • Depression and anxiety • Feelings of guilt, hopelessness, worthlessness, loss of energy, poor concentration, deficits in short term memory, loss of ambition and enthusiasm, suicidal thoughts • Hypomimia • Dysthmic d/o: chronic depression, poor appetite or overeating, insomnia or hypersomnia, low energy, low self-esteem • Panic attacks • Social phobia • Agoraphobia • OCD, panic d/o

Clinical Presentation • Autonomic dysfunction • direct manifestation of disease • thermoregulatory dysfunctions • seborrhea and seborrheic dermatitis • slow pupillary responses • GI disorders: constipation, urinary incontinence • erectile dysfunction • diminished heart function • orthostatic Hypotension

Clinical Presentation • Autonomic dysfunction • airway obstruction: (air trapping, lung inflation) most frequently reported pulmonary problem • restricted lung disease • low FVC, low FEV 1 and higher RV • moderate edema d/t immobility

Clinical Presentation • Sleep disorder • excessive daytime sleepiness • insomnia • dream enacting behaviors such as agitation, physical activity during sleep

Medical Diagnosis • Made on the basis of history and clinical examination • Diagnosis is made if at least two of the four cardinal features are present • Apomorphine testing

Clinical Course • Progressive, long sublicinical period • Patients at young age/ tremor predominant: more benign progression • cardiovascular disease and pneumonia: MC of death

Hoehn-Yahr Classification of Disability Scale • Most widely used severity staging scale in clinical practice I – minimal or absent, unilateral if present II – minimal bilateral or midline involvement, balance not impaired III – Impaired righting reflex, unsteadiness when turning or rising from chair Some activities are restricted but patient can live independently IV – all symptoms present and severe, standing and walking only possible with assistance V – confined to bed or wheelchair

Unified Parkinson’s Disease Rating Scale • “Gold Standard” for measuring progression of PD • Part I: Mentation, Behavior, Mood • Part II: ADL • Part III: Motor Examination • Part IV: Complications of therapy

Medical Management • Management is directed at slowing disease progression • Increasingly more challenging over time as disease progresses

Pharmacological Management • Starting medication early has been shown to be beneficial in slowing progression of disease • 1. Levodopa/ Carbidopa (Sinemet) • Levodopa: Gold standard drug therapy for PD • Levodopa (L-dopa): precursor of dopamine, more than 99% of levodopa is metabolized before reaching the brain • Carbidopa: Decarboxylase inhibitor • Available in Immediate release (IR) and Controlled release (CR)

Pharmacological Management 1. Levodopa/ Carbidopa (Sinemet) • Primary benefits: controlling PD motor symptoms of bradykinesia • • • and rigidity, initial burst of motor activity, increased strength Honeymoon period: initial dramatic improvement in functional status, presence of clear cut drug effectiveness 4 -6 years therapeutic window, then wearing off state Dyskinesias – involuntary movements that appear as facial grimacing, twitching, puckering of lips and may progress to choreathetoic movements of shoulders, arms and hands Dystonia On-off phenomenon

Pharmacological Management 1. Levodopa/ Carbidopa (Sinemet) • Dyskinesias – involuntary movements that appear as facial grimacing, twitching, puckering of lips and may progress to choreathetoic movements of shoulders, arms and hands • Dystonia • On-off phenomenon

Pharmacological Management 1. Levodopa/ Carbidopa (Sinemet) • unsupervised reduction or sudden discontinuation is contraindicated • dose related changes: • Disabling psychiatric toxicity • Depression • GI changes • CV changes • Genitorurinary changes • Sleep disturbance

Pharmacological Management 2. Dopamine Agonists • designed to directly stimulate postsynaptic dopamine receptors • pt. ’s with declining response to levidopa and carbidopa may benefit • adverse effects similar with levodopa • increased risk for impulse control disorders: (pathological gambling, compulsive shopping, hypersexuality, overeating) • Bromocriptine

Pharmacological Management 3. Anticholinergics • Most benefit moderating tremor and dystonia • have little or no effect on other PD symptoms • Anticholinergic adverse effects include blurred vision, dry mouth, dizziness and urinary retention • trihexyphenidyl (Artane) and benztropine mesylate (Congentin) • adverse effects: blurred vision, dry mouth, dizziness and urinary retention

Pharmacological Management 4. Monoamine Oxidase B Inhibitors (MAO-B) • MAO-B is the major enzyme that acts to degrade dopamine in the brain • Selegiline (deprenyl) and Rasagiline (Azilect) • Permits lower dose of levodopa • Adverse effects: mild nausea, dry mouth, dizziness, orthostatic hypotension, confusion, hallucinations, and insomnia

Pharmacological Management Implications for Physical Therapy • Fully aware of the medications and potential adverse effects • It is important to remember that patients on dopamine replacement will develop motor complications at some point • Timing of PT examination and intervention

Nutritional Management • High protein diet can block effectiveness of L-Dopa • High calorie – low protein diet (no more than 15% of calories)

Deep Brain Stimulation • implantation of electrodes in brain blocking signals • effective in treatment of advanced PD • Possible adverse effects: Confusion, Headache, Speech problem, gait disturbance

Physical Therapy Examination and Evaluation • 1. Cognitive Function • Mini Mental State Examination • 2. Psychosocial Function • Geriatric Depression scale, Beck depression Inventory • Hospital Anxiety and Depression Scale • 3. Sensory Function • 4. Musculoskeletal Function • Joint Flexibility and posture • Spinal ROM • Muscle Performance

Physical Therapy Examination and Evaluation • 4. Musculoskeletal Function • Muscle performance • Strength and Endurance • MMT, handheld and isokinetic dynamometry • 5. Motor Function • Rigidity • Bradykinesia • Movement time • Reaction time • Rapid alternating movements • Dexterity

Physical Therapy Examination and Evaluation • 5. Motor Function • Tremor • Location, persistence, severity (amplitude) • Postural control and balance (Berg balance Scale, Timed up and • • Go test) Gait Fall Risk (Fall Risk diary) Fatigue (Multidimensional Fatigue Inventory, Fatigue Severity Scale) Dyskinesia (Rush dyskinesia Scale)

Physical Therapy Examination and Evaluation • 5. Motor Function • Tremor • Location, persistence, severity (amplitude) • Postural control and balance (Berg balance Scale, Timed up and • • • Go test) Gait Fall Risk (Fall Risk diary) Fatigue (Multidimensional Fatigue Inventory, Fatigue Severity Scale) Dyskinesia (Rush dyskinesia Scale) Swallowing and speech

Physical Therapy Examination and Evaluation • 6. Autonomic Function • Cardiorespiratory Function • 6 Minute/ 12 minute walk test • Orthostatic Hypotension • drop in systolic BP of 20 mm. Hg and 10 mm. Hg in diastolic BP and 10 - 20% increase in pulse rate • Integumentary Integrity • Seborrhea and Seborrheic Dermatitis • 7. Functional status • Functional Independence Measure

Physical Therapy Examination and Evaluation • Disease Specific Measures • Parkinson’s Disease Questionnaire (PDQ-39) • Focuses on subjective report of the impact of PD on daily-life • Parkinson’s Disease Summary Index • 0 -100 • provides useful indication of global impact of PD on health status

Physical Therapy Intervention • Motor Learning Strategies • blocked practice rather than random practice • structured instructional sets • external cues • Visual • Rhytmic Auditory Stimulation (metronome) • Multisensory cueing

Physical Therapy Intervention • Exercise Training • “Training big” program (High amplitude movements) • Relaxation exercises • Slow rocking • Rhythmic Rotation • Rhythmic Initiation (counters effects of rigidity) • Bilat. Symmetrical PNF D 2 flexion pattern with diaphragmatic breathing • Flexibility Exercises • Resistance Training

Physical Therapy Intervention • Exercise Training • Functional Training • Bed Mobility Skills: Emphasize segmental rotation patterns than a log- rolling pattern • Sitting • STS • Standing • Balance Training • Should emphasize practice of dynamic stability tasks • Locomotor Training • Walk Tall, Walk fast, Take Large steps • Braiding

Physical Therapy Intervention • Spinal Orthotics • Spinal bracing for postural deformities • Pulmonary Rehabilitation • breathing exercises • exercise that recruit neck, shoulder and trunk muscles • Manual techniques such as vibration and shaking • Speech Therapy • Aerobic Exercise • Minimum: 3 sessions per week • Daily walking with short multiple bouts (20 -30) minutes throughout the day for Pt. ’s with lower functional capacity

Physical Therapy Intervention • Group and Home Exercises • Taichi classes

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