Parkinson’s disease

PARKINSON’S DISEASE&

COMPLICATIONS OF THIS DISORDER

PARKINSON’S DISEASE

.Originally described by James Parkinson in 1817 and characterized as Shaking Palsy.

. Chronic slowly prog, neurodegenerative disease of the Basal Ganglia (BG).

. Basic path-lack of dopamine-producing cells in the BG.

BACKGROUND MOSTLY known as movement disorder 1-2% > 65 y/o 15% between ages of 65 and 74Cardinal signs;

tremor, bradykinesia, rigidity & postural instability.

Dx: 2/3 Onset: unilateral progressing to B/L

TREMOR Resting . Pill-rolling motion Suppressed by

activity, sleep, concentration

Intensified by stress, fatigue

Mostly begin unilateral.

BRADYKINESIA

Required for dx Most disabling Sx Slowness of movement/motion Affects facial muscle & masked face Inability to change direction while walking/dif

walking around obstacle Causes gait/postural abnormality Clumsy or weak limb maybe early sign

RIGIDITY refers to an increase in resistance to passive movement about

a joint; either osclillating (cogwheel) or smooth (lead pipe). Rigidity usually is tested by flexing and extending the patient's relaxed wrist.

Cogwheeling Racheting through the ROM due to subtle tremor

superimposed on the rigidity Lead pipe

Smooth resistance to passive movement that is independent of velocity (in contradistinction to spasticity, which is velocity dependent)

Lead pipe tone can be made more obvious with voluntary movement or mental task in the c/l limb.

POSTURAL INSTABILITY 4th cardinal sign, but it emerges late in the disease, usually after 8 years or more

Imbalance and loss of righting reflexes. Assumption by patient of a stooped-forward

posture Presence, usually, of a festinating gait pattern

(stumbling forward).Decreased arm swing during ambulation

CONSEQUENCES OF DISEASE Stiffness and slowed movements Tremor or shaking at rest Difficulty getting out of a chair or rolling over in

bed Frequent falls or tripping Difficulty walking Memory loss Shifting forward of posture into a stoop Speech changes (eg, whispering, rapid speech) Smaller handwriting Slowness in performing activities of daily living

(ADL)

CLINICAL MANIFESTATION

PHYSICAL EXAM Painful dystonia, usually occurring in the early morning Rapid, monotonous, low-volume speech Hypokinetic dysarthria Dysphagia Masklike facies Depression

Can affect up to 50% of patients Suicide risk

Akathisia (inability to sit still) . Olfactory dysfunction (hyposmia), which may be present

prior to motor symptoms and often is not recognized by the patient

PHYSICAL EXAM Autonomic Dysfunction Slowed enteric motility and constipation Urinary retention and incontinence Orthostatic hypotension

Patients may experience freezing when starting to walk (start-hesitation), during turning, or while crossing a threshold, such as going through a doorway

CLASSIFICATION OF PARKINSON’S

Idiopathic PD – 85% of all PS cases Drug induced Parkinsonism – 7-9% Parkinson-Plus Syndrome Vascular Parkinson syndrome -3% Toxin-induced –rare Recurrent Head trauma-rare

IDIOPATHIC PD D/O of the Basal Ganglia (BG) Loss of dopamine producing cells in the

substantia nigra (SN) and locus ceruleus (LC) Degeneration of nigrostriatal pathway Sx manifest if decreased dopamine content by

> 50%) Loss of inhibitory input to the cholinergic

system> > excess excitatory output Imbalance of cholinergic input in the striatum

EPIDEMIOLOGY/M&M

Male to female ratio = 3:2 Prevalence = 160/100,000 Incidence = 20/100,000 per year /general

population Morbidity=progressive Mortality=mean survival after onset @ 15 yrs

PD survival >MSA,PSP MC cause of death: pulmonary infection/aspiration,

UTI, PE, of falls/fractures

ACCELERATED AGINGNormal aging is associated with clinical features that may resemble PD.

Aging is associated with a decline of pigmented neurons in the substantia nigra and with decreased levels of striatal dopamine and dopa decarboxylase.

ETIOLOGY

Unknown Theories

Accelerated aging Genetic susceptibility Environmental Factors Oxidative stress

ETIOLOGY UNCLEAR

.Genetic susceptibility - Twin studies inconclusive Genetic factors play a

greater role with early onset PD

Increased incidence of a family history PD observed

Environmental factors use of pesticides, living in a rural

environment consumption of well

water exposure to herbicides proximity to industrial

plants or quarries

ETIOLOGY UNCLEAR Head Trauma increases the risk of developing

Parkinson . The former champion

boxer Muhammad Ali was diagnosed with Parkinson's in 1984 at the age of 42, and is one of the most high-profile people battling the condition.

OXIDATIVE STRESS

Free radical damage, resulting from dopamine's oxidative metabolism, plays a role in the development or progression of PD.

Dopamine oxidation via MAO result in formation of hydrogen peroxide.

Hydrogen peroxide normally cleared by glutathione

Hydrogen peroxide reactions with ferrous ions, resulting in formation of hydroxyl radical.

- hydroxyl radicals can cause damage to lipids, DNA, amino acids

PD associated with: increased dopamine turnover, decreased protective mechanisms (glutathione),increased iron (a pro-oxidation molecule),

evidence of increased lipid peroxidation.

CLUES SUGGESTING ATYPICAL PARKINSONISM

Early onset of, or rapidly progressing dementia

Rapidly progressive course Supranuclear gaze palsy Cerebellar signs-dysmetria, ataxia Early urinary incontinence.

PARKINSON’S SYNDROME Parkinson’s Disease

Survival approximates US population when treated

Slow progressive onset of asymmetric bradykinesia

Onset with either classic pill-rolling tremor or rigidity

Parkinson-Plus syndromesShorter survival,

more frequent complications

Early instabilityRapid disease

progressionPoor response to

LevodopaPyramidal and

cerebellar signsEarly dysarthria,

dysphasia

PARKINSON-PLUS SYNDROME

PSPSupranuclear downgaze palsy, square wave

jerksUpright posture/frequent fallsPseudobulbar emotionalityFurrowed brows/stare

Corticobasal degenerationcognitive impairmentUnilat, coarse tremor,limb apraxia/limb

dystonia-myoclonus/alien limb

PD VS ESSENTIAL TREMOR

ET should be tremor with no other signs of parkinsonism

Both can have kinetic and rest component

Cogwheel rigidity can be found in ET

TREATMENT OPTIONS Preventive = no definite one available Symptomatic

Pharmacological Surgical

Non-motor management Restorative-experimental only

Transplantation Neurotrophic factors

Nonpharmacologic approaches PT/OT/ST

DRUG CLASSES IN PD Dopaminergic agents

Levodopa (LD) Dopaminergic Agonists- Bromocriptine, Ropinirole,

Pramipexole

COMT inhibitors Tolcapone, Entacapone LD + Entacapone (Stalevo)

MAO-B inhibitors- Selegiline (Eldepryl) Anticholinergics

Trihexyphenidyl, Benztropine

Antivirals-Amantadine

SURGICAL MANAGEMENT Candidates for deep brain

stimulation disabling medication-resistant

tremor

levodopa-responsive patients with medication-resistant disabling motor fluctuations and/or levodopa-induced dyskinesia.

no significant cognitive impairment, mood or behavioral disturbances

No other factors that may increase the risk of surgery.

SURGICAL MANAGEMENT Deep drain stimulationThalamic

Dec. tremor in 90% of pt

No effect on cardinal signs

Pallidal Improves cardinal signs,

dyskinesiaSubthalamic

Improves cardinal signs, dyskinesia, motor fluctuations

FUTURE MANAGEMENT

Neural transplantation dopamine-producing

cells, ex. fetal nigral cells.

Gene therapy

MANAGING EARLY COMPLICATIONS :ALTERED MENTAL

STATES

Confusion, sedation, dizziness, hallucinations, delusions

Reduce /eliminate CNS-active drugs of lesser priority Anticholinergics - Sedatives and many other

medications.

LATE COMPLICATIONS

Motor fluctuations, dyskinesias,dystonia,freezing,falls

Behavioral/neuropsychological Depression,sleep d/o, psychosis

Autonomic OH, hyperhidrosis ,constipation, impotence, urinary

incontinence or retention

FREEZING AND FALLS

Freezing motoric block; at initiation of gait, turning, narrow

spaces use auditory(marching steps to the beat of a

metronome), visual, proprioceptive cues ( mental rehearsal and imaging)

Falls Physical therapy evaluation Cane, scooter, wheelchair may be necessary

COGNITIVE ASSESSMENT

Memory difficulties: 11-29% of PD patients Benign forgetfulness Delirium Alzheimer’s disease Other dementias

Evaluation Brain imaging Lumbar puncture EEG Blood work for thyroid profile, vitamin B12, serology,

chemistry panel

PSYCHOSIS Features

Vivid dreams/nightmares, disorientation, hallucinations, delusional thought

Simplify medical regimen Stop unnecessary non-PD meds Stop: anticholinergic drugs, amantadine, selegiline,

dopamine agonists, COMT inhibitors Change from CR to standard carbidopa/levodopa Try atypical antipsychotic agents Try low-potency traditional antipsychotic agents

ANXIETY/RESTLESSNESS Primary anxiety disorder: treat with benzodiazepines Associated with

“off-periods” or low-levopoda levels: adjust levopoda dosing

Restless Leg Syndrome:

SLEEP DISORDERS Insomnia

careful history difficulty with sleep initiation treat depression REM-behavioral disorder: clonazepam

Excessive daytime sleepiness correct poor sleep at night discontinue anticholinergics, amantadine reduce dopamine agonist, levopoda dosages if possible

ORTHOSTATIC HYPOTENSION

Tilt table training for severe cases Taper anti-hypertensive agents Taper non-PD drugs Increase salt intake Elevate HOB, arising slowly, isometric exercises Compression stockings, abdl binders

URINARY INCONTINENCE/FREQUENCY

Rule out urinary tract infection Bladder evaluation Urinary frequency

IMPAIRED GI MOTILITY

Constipation Vomiting Impaired absorption Treatment Options

small frequent meals increased fiber/bulking agents stool softeners and suppositories

NAUSEA

Levodopa-related: take with meals, add carbidopa, add domperidone

Other anti-PD medications: same. If no improvement: withdraw newest agent, re-initiate

at minimal doses, slowly increase

EXCESSIVE SWEATING

Usually levodopa related, and may be seen at peak or trough dose drug levels

PROBLEMS THAT MAY RESPOND TO NONPHARMACOLOGICAL APPROACHES

Motor, mobility,balance, posture, gait ADL difficulties Speech : hypophonia, sialorrhea,dysphagia Inadequate nutrition Sleep disturbance Autonomic dysfunction:

OH, delayed gastric emptying, constipation,bladder dysfunction

Sexual dysfunction Depression, Anxiety

REHABILITATION IMPAIRMENTS

¨ Gait disturbance Decreased stride

length, cadence, velocity. Festination

Stooped flexed posture Cautious gait(fear of

falling) Impaired balance

REHABILITATION MANAGEMENTRehabilitation &interventions are directed at the main causes of impairments.

Multidisciplinaryapproach:PT,OT,ST,RT,Neuropsych

RATIONALE FOR REHABILITATION

While rehabilitation services are often given to the patient with Parkinson disease, this occurrence is more based on common practice rather than clear research design. There is a paucity of well-designed research studies looking at specific rehabilitation techniques. The existing literature is both sparse and fraught with confounding variables such as changes in medication regimens. A recent review examined 11 studies involving various physical therapy techniques in Parkinson disease. The authors found insufficient evidence to support or refute the efficacy of any form of physical therapy over another form. Furthermore, there was insufficient evidence found to support the efficacy of any therapy compared with no therapy. Perhaps the best designed study was a prospective randomized crossover investigation of 4 weeks of outpatient physical therapy, in which medication changes were not allowed.

CARDIOPULMONARY IMPAIRMENT

The patient's flexed posture can lead to kyphosis, cause a reduction in pulmonary capacity, and produce a restrictive lung disease pattern.

Breathing exercises, postural reeducation, and trunk exercises may be helpful.

Institution of a general conditioning program can increase the patient's endurance.

If pulmonary function progressively worsens, assisted coughing techniques, incentive spirometry, and respiratory therapy intervention may be required.

REHABILITATION AND RATING SCALE

Stages 0-II are mild disease;

Stage III is moderate disease;

Stages IV and V are marked or advanced disease. There are gray areas between the successive stages.

TREATMENT PLANMaintain or increase ROM in all joints

Efforts to improve postural control and standing balance

Prevent disuse /atrophy and muscle weakness

Improve motor function and mobility

TREATMENT PLAN Improve gait pattern Improve speech,

breathing patterns chest expansion, mobility

Maintain functional independence in adl’s

Assist in psychological adjustment to new lifestyle

Upper extremity fine motor skills

Functional transfers

Swallowing evaluation

Cognitive evaluation

Recreational therapy

Pt/Family training-education

PHYSICAL THERAPY: GOAL

Maintain or increase activity level Decrease rigidity and bradykinesia Facilitate movement and flexibility; optimize gait Maximize gross motor coordination and balance Maximize independence, safety, function

PHYSICAL THERAPY Relaxation techniques Gentle ROM and

stretching techniques Exaggerated or patterned

movements High stepping,wt

shifting,repitetion, visual &verbal cues

Back extension exercises and pelvic tilt

PHYSICAL THERAPY Static and dynamic postural controls emphazing whole

body movements sitting and standing Stationary bike training to help reciprocal movements Exercise: walking(1+mile/day),swimming,golf,dancing Use of assistive devices, mobility aids, orthotics Family training and home program

Proper and energy conservation techniques After 6 mths benefit of therapy if not coninued will be gone

OCCUPATIONAL THERAPY: GOALS

Maximize independence, safety, function Improve endurance, reduce energy expenditure Training in use Adaptive Equipments Improve body image, self-esteem, psychosocial adjustment Facilitate active movement Maximize fine motor coordination Increase trunk flexibility and upright posture

OCCUPATIONAL THERAPY Patient and caregiver education goals of program transfers, task

simplification, positioning, etc.

Home exercise program Home and workplace

modifications

SPEECH THERAPY Swallowing evaluation including modified barium swallow

Articulatory speech training for dysarthria

Early therapy is effective Teaching compensatory

strategies for safer swallow

DYSPHAGIA If swallowing difficulties do not respond to conservative interventions by the speech therapist, more aggressive treatment may be required.

Such aggressive management can include invasive procedures, such as nasogastric or gastrostomy feeding tube placement.

Discussion should be initiated early on in the disease course to ascertain the patient's wishes about a feeding tube, in case dementia develops and the patient lacks the capacity for decision making when a feeding tube becomes medically indicated.

TECHNIQUES TO IMPROVE SPEECH

Increase loudness Face the listener directly Emphasize key words Use short sentences Range-of-motion exercises for muscle of speech Breathing exercises, breath control Phonatory-respiratory effort model /Lee Silverman Voice

Tx=“think loud, think shout approach”

MANAGEMENT OF SWALLOWING DIFFICULTY

Do not rush Eat soft foods, small bites of food Swallow only well-chewed food Empty mouth before next bite Chin down positioning Family should learn Heimlich maneuver Be aware of saliva accumulation and swallow often Verbal prompting Clinicians might also choose to administer antiparkinsonian

medications prior to meals, so that maximal benefit of drugs occurs during mastication.

RECREATIONAL THERAPY

identifying previous recreational interests new interests can be identified and explored social and recreational pursuits

social and recreational pursuits

COMMUNITY RESOURCES

Social worker intervention: Social Security office Medicare, Medicaid

In-home programs Meals on Wheels, home visiting, etc.

NUTRITIONAL RISK FACTORS

Inactivity Food preparation problems Dyskinesia and feeding problems Chewing and swallowing problems Increased metabolic needs Medication-related dietary restrictions Drug side effects: anorexia, nausea, vomiting,

constipation Depression and dementia

DIETARY RECOMMENDATIONS Eat a balance diet, including all food groups

Consume sufficient calories to maintain weight

Consume adequate fiber and fluids to avoid constipation

Take vitamin D and calcium to prevent osteoporosis

Reduce protein to minimum daily allowance.

PROGNOSIS/COMPLICATIONS Poor Prognostic indicatorsOld age of onset

Early cognitive deficits

Lack of tremor

Complications Underlying medical

illness ( sepsis, pneumonia, fecal impaction, urinary tract infection) should be suspected in a PD patient

REFERENCES

Neuro Rehab Book ( PTA Program) http://www.emedicine.com/pmr/topic99.htm http://www.emedicine.com/NEURO/

topic304.htm

ANY QUESTION?