parkinson

1. Parkinson’s Disease Hannah H Florida,MD

2. 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. Results in disturbance of tone, abnormal posture, involuntary movement m/b caused by degeneration of dop-producing cells in the SN resulting in decrease levels of dopamine in striatumResults in disturbance of tone, abnormal posture, involuntary movement m/b caused by degeneration of dop-producing cells in the SN resulting in decrease levels of dopamine in striatum

3. Background MC movement disorder 1-2% > 65 y/o 15% between ages of 65 and 74 Cardinal signs/Classic Triad: tremor, bradykinesia, and rigidity. Dx: 2/3 Onset: insidious, unilateral progressing to B/L rising slowly with age Diagnosis based primarily on clinical assessment, no biological markers. Widely accepted that the dx shld be made only when 2 of the cardinal signs. The 3 cardinal signs of PD are resting tremor, rigidity, and bradykinesia. Of these cardinal features, 2 of 3 are required to make the clinical diagnosis. Postural instability is the fourth cardinal sign, but it emerges late in the disease, usually after 8 years or more. Brady & Rigidity tend to be more disabling than tremor.rising slowly with age Diagnosis based primarily on clinical assessment, no biological markers. Widely accepted that the dx shld be made only when 2 of the cardinal signs. The 3 cardinal signs of PD are resting tremor, rigidity, and bradykinesia. Of these cardinal features, 2 of 3 are required to make the clinical diagnosis. Postural instability is the fourth cardinal sign, but it emerges late in the disease, usually after 8 years or more. Brady & Rigidity tend to be more disabling than tremor.

4. Tremor Resting MC presenting symptom Dxtic but not required Pill-rolling motion of hand at 3-5 Hz Suppressed by activity, sleep, concentration Intensified by stress, fatigue Most begin unilaterally Onset of PD is typically asymmetric, with the most common initial finding being an asymmetric resting tremor in an upper extremity. About 20% of patients first experience clumsiness in one hand. Over time, patients notice symptoms related to progressive bradykinesia, rigidity, and gait difficulty. Tremor usually begins in one upper extremity and initially may be intermittent. As with most tremors, the amplitude increases with stress and resolves during sleep. After several months or as much as a few years, the tremor may affect the extremities on the other side, but asymmetry usually is maintained. PD tremor may also involve the legs, tongue, lips, chin, and head generally involved with more advanced disease Onset of PD is typically asymmetric, with the most common initial finding being an asymmetric resting tremor in an upper extremity. About 20% of patients first experience clumsiness in one hand. Over time, patients notice symptoms related to progressive bradykinesia, rigidity, and gait difficulty. Tremor usually begins in one upper extremity and initially may be intermittent. As with most tremors, the amplitude increases with stress and resolves during sleep. After several months or as much as a few years, the tremor may affect the extremities on the other side, but asymmetry usually is maintained. PD tremor may also involve the legs, tongue, lips, chin, and head generally involved with more advanced disease

5. Bradykinesia Required for dx Most disabling Sx Slowness/paucity 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 Gait observation in linear motion, in changes of direction; if pt uses >5 steps to complete 180 degree turn, PD shld be considered Bradykinesia refers to slowness of movement but also includes a paucity of spontaneous movements and decreased amplitude of movement. Bradykinesia also is expressed as micrographia (small handwriting), decreased blink rate, and hypophonia (soft speech). hypomimia (decreased facial expression), Gait observation in linear motion, in changes of direction; if pt uses >5 steps to complete 180 degree turn, PD shld be considered Bradykinesia refers to slowness of movement but also includes a paucity of spontaneous movements and decreased amplitude of movement. Bradykinesia also is expressed as micrographia (small handwriting), decreased blink rate, and hypophonia (soft speech). hypomimia (decreased facial expression),

6. 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. Cogwheeling may be appreciated in tremors not associated with an increase in tone (ie, essential tremor). RigidityCogwheeling may be appreciated in tremors not associated with an increase in tone (ie, essential tremor). Rigidity

7. Postural Instability 4th cardinal sign, but it emerges late in the disease, usually after 8 years or more Imbalance and loss of righting reflexes. Its emergence is an important milestone, because it is poorly amenable to treatment and a common source of disability in late disease. Assumption by patient of a stooped-forward posture Presence, usually, of a festinating gait pattern (stumbling forward); however, retropulsion also can occur Decreased arm swing during ambulatory activity Postural instability refers to imbalance and loss of righting reflexes. Its emergence is an important milestone, because it is poorly amenable to treatment and a common source of disability in late disease. Postural instability refers to imbalance and loss of righting reflexes. Its emergence is an important milestone, because it is poorly amenable to treatment and a common source of disability in late disease.

9. History 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) Sialorrhea Decreased sense of smell Symptoms and signs of PD typically begin in one extremity or side but eventually involve the other limbs and trunk. Historical features reported by patients with PD can include the following Symptoms and signs of PD typically begin in one extremity or side but eventually involve the other limbs and trunk. Historical features reported by patients with PD can include the following

10. Clinical Manifestation

11. 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) Seborrheic dermatitis, usually of the face and scalp Olfactory dysfunction (hyposmia), which may be present prior to motor symptoms and often is not recognized by the patient In addition to finding the 4 cardinal signs Poor olfactory function, which appears to be correlated with loss of dopamine receptors in the nigrostriatal regionIn addition to finding the 4 cardinal signs Poor olfactory function, which appears to be correlated with loss of dopamine receptors in the nigrostriatal region

12. 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

13. Physical Exam Dementia generally occurs late in PD and affects 15-30% of patients. Short-term memory and visuospatial function may be impaired, but aphasia is not present. Cognitive dysfunction within a year of onset of motor features suggests a diagnosis of Lewy body disease, a disease closely related to PD and marked by the presence of significant cortical Lewy bodies.

14. Classification of Parkinson’s Syndrome(PS)/Parkisonism Idiopathic PD – 85% of all PS cases Drug induced Parkinsonism – 7-9% Parkinson-Plus Syndrome MSA (SDS,SND,OPCD) – 2.5% PSP – 1.5% Vascular Parkinson syndrome -3% Toxin-induced –rare Recurrent Head trauma-rare Parkinsonism (also known as Parkinson's syndrome, atypical Parkinson's, or secondary Parkinson's) is a neurological syndrome characterized by tremor, hypokinesia, rigidity, and postural instability.[1] The underlying causes of parkinsonism are numerous, and diagnosis can be complex.[2] While the neurodegenerative condition Parkinson's disease (PD) is the most common cause of parkinsonism, a wide-range of other etiologies can lead to a similar set of symptoms, including some toxins, a few metabolic diseases, and a handful Patients with Parkinson-plus syndromes typically have a worse prognosis than those with Parkinson disease (PD), and Parkinson-plus syndrome responds poorly to the standard anti-Parkinson treatments. An adequate response to treatment in a patient with parkinsonian symptoms may indicate that a Parkinson-plus syndrome is developing, and searching for the signs and symptoms of degeneration in other neuronal systems is important. Parkinsonism (also known as Parkinson's syndrome, atypical Parkinson's, or secondary Parkinson's) is a neurological syndrome characterized by tremor, hypokinesia, rigidity, and postural instability.[1] The underlying causes of parkinsonism are numerous, and diagnosis can be complex.[2] While the neurodegenerative condition Parkinson's disease (PD) is the most common cause of parkinsonism, a wide-range of other etiologies can lead to a similar set of symptoms, including some toxins, a few metabolic diseases, and a handful Patients with Parkinson-plus syndromes typically have a worse prognosis than those with Parkinson disease (PD), and Parkinson-plus syndrome responds poorly to the standard anti-Parkinson treatments. An adequate response to treatment in a patient with parkinsonian symptoms may indicate that a Parkinson-plus syndrome is developing, and searching for the signs and symptoms of degeneration in other neuronal systems is important.

15. 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 (SN to corpus striatum) 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 Lewy bodies- microscopically , IC eosinophillic inclusions are found in damaged cellsLewy bodies- microscopically , IC eosinophillic inclusions are found in damaged cells

16. Basal Ganglia The basal ganglia are a group of nuclei in the brain associated with motor functions. Nuclei: caudate, putamen substantia nigra, subthalamic, globus pallidum

17. Basal Ganglia motor circuit modulates cortical output Signals from the cerebral cortex are processed through the basal ganglia-thalamocortical motor circuit and return to the same area via a feedback pathway. Output from the motor circuit is directed through the internal globus pallidus (GPi) and the substantia nigra pars reticulata (SNr). inhibitory output is directed to the thalamocortical pathway and suppresses movement.

18. Pathophysiology Loss of pigmented dopaminergic neurons in the substantia nigra Approximately 60-80% of dopaminergic neurons are lost before the motor signs of PD emerge. The presence of Lewy bodies.

19. Lewy Bodies Lewy bodies are concentric, eosinophilic, cytoplasmic inclusions with peripheral halos and dense cores. Present within pigmented neurons of substantia nigra. Characteristic of PD but not pathognomonic

20. Epidemiology/ M&M Male to female ratio = 3:2 Prevalence = 160/100,000 Incidence = 20/100,000 per year /general population Morbidity=progressive more rapid in MSA and PSP Mortality=mean survival after onset @ 15 yrs PD survival >MSA,PSP MC cause of death:pulmonary infection/aspiration,UTI,PE,Cx of falls/fractures

21. Etiology Unknown Theories Accelerated aging Genetic susceptibility Environmental Factors Oxidative stress The exact cause of PD remains unclear. A combination of factors probably is responsible for the condition's development. Various theories include the following: Many authors = combination Risks: definite old age,prob,family hx,possible,pesticides/herbecides,heavy metals,proximity to industry,rural residence/well H2O,repeated head trauma,High BMI Possible protective effect: smoking, small coffee drinkingThe exact cause of PD remains unclear. A combination of factors probably is responsible for the condition's development. Various theories include the following: Many authors = combination Risks: definite old age,prob,family hx,possible,pesticides/herbecides,heavy metals,proximity to industry,rural residence/well H2O,repeated head trauma,High BMI Possible protective effect: smoking, small coffee drinking

22. Accelerated Aging Normal 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. Some authorities believe that PD may result from the effects of aging superimposed on an insult to the nigrostriatal system earlier in life.

23. 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 (16% vs 4% of control) Accounts to <5% of PD cases. Environmental factors use of pesticides, living in a rural environment consumption of well water exposure to herbicides proximity to industrial plants or quarries Twin studies often have proven inconclusive. Genetic factors seem to play a greater role in PD that has an earlier onset. An increased incidence of a family history of PD is observed in affected individuals (16% vs 4% of control population). Twin studies often have proven inconclusive. Genetic factors seem to play a greater role in PD that has an earlier onset. An increased incidence of a family history of PD is observed in affected individuals (16% vs 4% of control population).

24. 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. This theory receives much support and attention in the literature. PD patients may suffer the combined effects of multiple factors, culminating in damage from free radicals. Dopamine oxidation can result in the formation of hydrogen peroxide, as well as the superoxide anion radical. Hydrogen peroxide can undergo reactions with ferrous ions, resulting in formation of the highly toxic hydroxyl radical. These hydroxyl radicals can cause cell membrane damage. This theory receives much support and attention in the literature. PD patients may suffer the combined effects of multiple factors, culminating in damage from free radicals. Dopamine oxidation can result in the formation of hydrogen peroxide, as well as the superoxide anion radical. Hydrogen peroxide can undergo reactions with ferrous ions, resulting in formation of the highly toxic hydroxyl radical. These hydroxyl radicals can cause cell membrane damage.

25. Etiology Unclear Head Trauma the risk of developing Parkinsonism increases eightfold for patients who have had head trauma requiring hospitalization. 11-fold for patients who have experienced severe head injury. Dementia Pugilistica

26. Clues Suggesting Atypical Parkinsonism Early onset of, or rapidly progressing dementia Rapidly progressive course Supranuclear gaze palsy UMN signs Cerebellar signs-dysmetria, ataxia Early urinary incontinence Early symptomatic postural hypotension Factors useful in separating aatypical form from idiopathic.early onset /rapid progressive dementia more likely have diffuse lewy body disease or Alz dementia. Progressive course look for other parkinsonian synd. Supranuc plasy esp downgaze palsy=PSP, a parkinsonism-plus syndrome. UMN involvement=babinski,mild hyperreflexia suggets pyramidal pathway Cerebellar signs such as dysmetrai,ataxia,nystagmus,titubation,or gait ataxia=eval for cerebellar disease, early onset u incon eval for NPH Early symptomatic postural hypoten is rare in PD= suggest MSA. Factors useful in separating aatypical form from idiopathic.early onset /rapid progressive dementia more likely have diffuse lewy body disease or Alz dementia. Progressive course look for other parkinsonian synd. Supranuc plasy esp downgaze palsy=PSP, a parkinsonism-plus syndrome. UMN involvement=babinski,mild hyperreflexia suggets pyramidal pathway Cerebellar signs such as dysmetrai,ataxia,nystagmus,titubation,or gait ataxia=eval for cerebellar disease, early onset u incon eval for NPH Early symptomatic postural hypoten is rare in PD= suggest MSA.

27. Parkinson’s Syndrome Parkinson’s Disease Survival approximates US population when treated Slow progressive onset of asymmetric bradykinesia Excellent sustained Levodopa response Onset with either classic pill-rolling tremor or rigidity Parkinson-Plus syndromes Shorter survival, more frequent complications Early instability Rapid disease progression Poor response to Levodopa Pyramidal and cerebellar signs Early dysarthria, dysphasia

28. Parkinson-Plus Syndrome PSP Supranuclear downgaze palsy, square wave jerks Upright posture/frequent falls Pseudobulbar emotionality Furrowed brows/stare Corticobasal degeneration cognitive impairment Unilat, coarse tremor,limb apraxia/limb dystonia-myoclonus/alien limb Idio PD specifically results from degeneration of the NS pathway,however, other neurodege d/o can present with parkinsoian-type features. After Idio PD,PSP MC parkinsonian neurodegeraTIVE D/O.HALLMARK Signs of dg palsy &sw jerks often late stage sx, but always should be looked for in pts with parkinsonism.unlike stooped posture of PD,PSP pts often quite upright.furthermore,when these pts sit in a chr,rather than carefully sittingto protect themselves like PD pts,PSP will almost fall into the seat, sometimes striking their heads unto wall with their feet leaving the ground. Laughing/crying somewhat inapprop/often uncontrollably. Finally classic PSP type facial expression is one of furrowed brows and a significant stare with lack of eye blinks/lack of abiity to move eyes. Again this is often later sign of PSP. Early course of PSP, pts respond fairly well to dopaminergic meds but response usually wanes. Cb degeneration, also known as corticobalsal ganglionic degeneration, is a neurodegenerative parkinosonian d/o often asso w/ a very coarse unilat tremor.pathognomonic clinical sign is limb apraxia,usually 1 of upper lilmb.arm often held in dystonic posture,&the arm may move seemingly on its own and under poor control by pt, the alien limb sign.this condition dos not respond to anti PD meds but the dystonic posture may benefit fro botulinum toxin injectionIdio PD specifically results from degeneration of the NS pathway,however, other neurodege d/o can present with parkinsoian-type features. After Idio PD,PSP MC parkinsonian neurodegeraTIVE D/O.HALLMARK Signs of dg palsy &sw jerks often late stage sx, but always should be looked for in pts with parkinsonism.unlike stooped posture of PD,PSP pts often quite upright.furthermore,when these pts sit in a chr,rather than carefully sittingto protect themselves like PD pts,PSP will almost fall into the seat, sometimes striking their heads unto wall with their feet leaving the ground. Laughing/crying somewhat inapprop/often uncontrollably. Finally classic PSP type facial expression is one of furrowed brows and a significant stare with lack of eye blinks/lack of abiity to move eyes. Again this is often later sign of PSP. Early course of PSP, pts respond fairly well to dopaminergic meds but response usually wanes. Cb degeneration, also known as corticobalsal ganglionic degeneration, is a neurodegenerative parkinosonian d/o often asso w/ a very coarse unilat tremor.pathognomonic clinical sign is limb apraxia,usually 1 of upper lilmb.arm often held in dystonic posture,&the arm may move seemingly on its own and under poor control by pt, the alien limb sign.this condition dos not respond to anti PD meds but the dystonic posture may benefit fro botulinum toxin injection

29. Parkinson-Plus Syndrome MSA Cerebellar ataxia, pyramidal weakness,autonomic failure, nocturnal stridor Shy-Drager syndrome Autonomic insufficiency- orthostasis, impotence Striatonigral degeneration Tremor less prominent Olivopontocerebellar atrophy Cerebellar ataxia, dysarthria Other nd d/o that present with parkinsonism are the various types of MSA. As the name implies,des r d/o n w/c mutiple neuronal systems have degenerated. N pts w/ sdy,main chac tic dif it fr PD is autonomic disturbance such as OH, impotence,bowel/blad incontinence.although parkinsonian features respond poorly to antiPD meds, low BP respond to midodrine and fludrocortisone. Striatonigral dege s clini similar to idio PD but treor is not a prominent feature & has a limited response to antidopaminergic meds OPCA have cerebellar s/sx that dif it from idio PD. Der r currently no tx options for these pts, although 1 recent study reports some benefit from amantadine.Other nd d/o that present with parkinsonism are the various types of MSA. As the name implies,des r d/o n w/c mutiple neuronal systems have degenerated. N pts w/ sdy,main chac tic dif it fr PD is autonomic disturbance such as OH, impotence,bowel/blad incontinence.although parkinsonian features respond poorly to antiPD meds, low BP respond to midodrine and fludrocortisone. Striatonigral dege s clini similar to idio PD but treor is not a prominent feature & has a limited response to antidopaminergic meds OPCA have cerebellar s/sx that dif it from idio PD. Der r currently no tx options for these pts, although 1 recent study reports some benefit from amantadine.

30. Differential Diagnosis Diffuse Lewy body disease Early onset dementia Delusions/hallucinations Agitation Alzheimer’s disease Dementia primary clinical sx Rest tremor rare Finally pts w/parkinsonism dat hav erly onset dementia or wheredementia is primary clin finding likely hav diffuse lewy body disease or AD.all pts with idio PD have lewy bodies in the SN & many if not all will have them in the cortex & other areas of the brain.dif lb dis have significant cortical lewy bodies believed to produce the sx of dementia,delusions,hallucinations,agitation&delerium. AD s almost always chac by dementia & thiss d primary clin abnormality, but parkinsonian features such as bradykinesia, rigidity and postural instability may occur. Resting tremor is usually rare in AD population N general, all these neurodegenerative d/o respond quite poorly to dopaminergic med, although early in the course of the disease any ot these can have motor benefits from dopaminergic agents.Finally pts w/parkinsonism dat hav erly onset dementia or wheredementia is primary clin finding likely hav diffuse lewy body disease or AD.all pts with idio PD have lewy bodies in the SN & many if not all will have them in the cortex & other areas of the brain.dif lb dis have significant cortical lewy bodies believed to produce the sx of dementia,delusions,hallucinations,agitation&delerium. AD s almost always chac by dementia & thiss d primary clin abnormality, but parkinsonian features such as bradykinesia, rigidity and postural instability may occur. Resting tremor is usually rare in AD population N general, all these neurodegenerative d/o respond quite poorly to dopaminergic med, although early in the course of the disease any ot these can have motor benefits from dopaminergic agents.

31. Differential Diagnosis Hereditary disorders associated with parkinsonism Wilson’s disease Huntington’s disease Dentatorubro-pallidoluysian atrophy(DRPLA) Machado-Joseph diseaseor spinal cebellar ataxia (SCA-3) WD s a d/o of copper metabolism n w/c cop deposits in the BG can produce parkinsonian type features.often des pts develop an action tremor, a more severe “wing-beating tremor”& psychiatric cx.any pt w/ signs of parkinsonism under age 50 should have serum ceruloplasmin test, a 24 hr urine copper, & an opthalmologic eval using slit lamp to assess kayser-fleischer rings. F des tests r normal & wilson’s dis is being strongly considered, liver bx m/b necessary. Important to eval feo wd since tx w/ chelating agents or zinc markedly improve all of the sx esp in young-onset hd pts,o can present akinetic and rigid syndrome much like PD.autosomal dominant d/o eval by genetic testing DRPLA m/b confirmed by genetic testing SCAtype III(spinal cerebellar ataxia) parkinsonian-cerebellar d/o m/b confirmed by genetic testingWD s a d/o of copper metabolism n w/c cop deposits in the BG can produce parkinsonian type features.often des pts develop an action tremor, a more severe “wing-beating tremor”& psychiatric cx.any pt w/ signs of parkinsonism under age 50 should have serum ceruloplasmin test, a 24 hr urine copper, & an opthalmologic eval using slit lamp to assess kayser-fleischer rings. F des tests r normal & wilson’s dis is being strongly considered, liver bx m/b necessary. Important to eval feo wd since tx w/ chelating agents or zinc markedly improve all of the sx esp in young-onset hd pts,o can present akinetic and rigid syndrome much like PD.autosomal dominant d/o eval by genetic testing DRPLA m/b confirmed by genetic testing SCAtype III(spinal cerebellar ataxia) parkinsonian-cerebellar d/o m/b confirmed by genetic testing

32. Differential Diagnosis Secondary Parkinsonism Drug – induced Toxin – induced Metabolic Structural lesions (vascular parkisonism, etc) Hydrocephalus Infections Careful hx and appropriate labs useful confirming the dx Careful hx and appropriate labs useful confirming the dx

33. Drug-Induced Parkinsonism Antipsychotics/neuroleptics Haldol, chlorpromazine, thioridazine, resperidone, olanzapine Antiemetics- MC drugs causing PS Metoclopramide,prochlorperazine Dopamine depletors methyldopa, reserpine containing anti-HTN Combination drugs- know components Triavil (amitriptyline + perphenazine) Crucial to R/O, most cases are reversible Careful med hx-list drug names Common offending drugs Haldol,chlorpromazine(thorazine),thioridazine(mellaril), perphenazine(trilafon),resperidone,olanzapine (zyprexa metoclopramide(reglan),prochlorperazine(compazine) Tx- stop offending meds Crucial to R/O, most cases are reversible Careful med hx-list drug names Common offending drugs Haldol,chlorpromazine(thorazine),thioridazine(mellaril), perphenazine(trilafon),resperidone,olanzapine (zyprexa metoclopramide(reglan),prochlorperazine(compazine) Tx- stop offending meds

34. Metabolic/Infectious Causes Metabolic Often reversible Hypo or hyperthyroidism Hypo or hyperparathyroidism Liver failure Central pontine myelinolysis - rapid correction of hyponatremia Infectious Post-encephalitis Creutzfeld-Jakob disease- Infectious masses compressing SN/BG HIV CJD rapidly progressive disease w/ dementia,parkisonism,myoclonus,seizures …EEG HIV asso parkisonism m/b seen with viral destruction in the extrapyramidal pathway or from progressive muultifocal leukoencephalopathy CJD rapidly progressive disease w/ dementia,parkisonism,myoclonus,seizures …EEG HIV asso parkisonism m/b seen with viral destruction in the extrapyramidal pathway or from progressive muultifocal leukoencephalopathy

35. Toxin-Induced Parkinsonism MPTP Cyanide Iron Manganese Carbon disulfide/monoxide Pesticides/organic solvents Lead methanol MPTP derivative of meperidine is a contaminant in the manufacture of illicit drugs, crosses BBB & is converted to MPPP+, a neurotoxin specifically taken up by dopaminergic nerve terminals leading to degeneration of dopamine neurons thus parkinsonism CO, if not fatal may result in parkinsonism due to BG destruction as well cyanide exposure. A high intake of supplemental iron, especially in combination with high manganese intake, may be related to risk for PD. Powers et al, University of Washington School of Medicine, Seattle. Dept of Environmental Health2006 MPTP derivative of meperidine is a contaminant in the manufacture of illicit drugs, crosses BBB & is converted to MPPP+, a neurotoxin specifically taken up by dopaminergic nerve terminals leading to degeneration of dopamine neurons thus parkinsonism CO, if not fatal may result in parkinsonism due to BG destruction as well cyanide exposure. A high intake of supplemental iron, especially in combination with high manganese intake, may be related to risk for PD. Powers et al, University of Washington School of Medicine, Seattle. Dept of Environmental Health2006

36. Vascular Parkinsonism Abrupt onset, usually unilateral Step-wise or no progression Other signs-hemiparesis,aphasia,hyperreflexia Infarcts on neuroimaging helpful in confirming dx Multi cerebral infarctions affecting BG,SN,caudate,putamen,GP,brain stem or their pathways develop parkinsonian features.Vascular parkisonism usuallly has abrupt onset, usually unilat &progresion if any, usually stepwise fashion.other signs include hemiparesis,aphasia,hyperreflex.although seeing abn on imaging studies m/b helpul, small vessel ischemic changes, or lacunar or cortical infarction may occur in other atypical forms of parkinsonism as well as in pts with idio pD Multi cerebral infarctions affecting BG,SN,caudate,putamen,GP,brain stem or their pathways develop parkinsonian features.Vascular parkisonism usuallly has abrupt onset, usually unilat &progresion if any, usually stepwise fashion.other signs include hemiparesis,aphasia,hyperreflex.although seeing abn on imaging studies m/b helpul, small vessel ischemic changes, or lacunar or cortical infarction may occur in other atypical forms of parkinsonism as well as in pts with idio pD

37. Hydrocephalus –induced Parkinsonism Can be communicating or obstructive NPH-idiopathic Clinical triad Parkisonism/gait disorder Urinary/fecal incontinence dementia Dx usually made by CT/MRI showing hydrocephalus. Some instances,nuclear medicine cisternography,looking for abnormLITIES IN csf FLOW is helpful.NPH pts may benefit from ventriculoperitoneal shunting. Often lumbar puncture with removal of 15-25 cc of CSF on 3 consecutive days may produce a self limitng improvement in gait and is a useful dxtic test prior to VP shunting.however, some cases in w/c no improvement is seen after serial lp may improve after VP shunting.Dx usually made by CT/MRI showing hydrocephalus. Some instances,nuclear medicine cisternography,looking for abnormLITIES IN csf FLOW is helpful.NPH pts may benefit from ventriculoperitoneal shunting. Often lumbar puncture with removal of 15-25 cc of CSF on 3 consecutive days may produce a self limitng improvement in gait and is a useful dxtic test prior to VP shunting.however, some cases in w/c no improvement is seen after serial lp may improve after VP shunting.

38. 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 There s sometimes confusion differentiating a parkinsonian tremor from ET.ET shld have tremor alone, no other signs of parkinsonism nor any other neurologic signs such hyperreflexia,weakness,or sensory loss. Both d/o may have kinetic and rest component although traditionally pt w/ ET have more postural & kinetic tremor w/ dampening upon rest,where as PD more often have rest tremor dampens with action. In addition,ET pts may exhibit mild signs of bradykinesia & cogwheeling when examined when examined for rigidity.in the early tremor pt,the historical findings of fam hx suggesting AD inheritance, a long hx of tremor w/o progression of motor difficulties & tremor response to alcohol r helpful n the dx of ET.lastly ET does not respond to antiPD drugs but may improve w/ propanolol,primidone,and benzos There s sometimes confusion differentiating a parkinsonian tremor from ET.ET shld have tremor alone, no other signs of parkinsonism nor any other neurologic signs such hyperreflexia,weakness,or sensory loss. Both d/o may have kinetic and rest component although traditionally pt w/ ET have more postural & kinetic tremor w/ dampening upon rest,where as PD more often have rest tremor dampens with action. In addition,ET pts may exhibit mild signs of bradykinesia & cogwheeling when examined when examined for rigidity.in the early tremor pt,the historical findings of fam hx suggesting AD inheritance, a long hx of tremor w/o progression of motor difficulties & tremor response to alcohol r helpful n the dx of ET.lastly ET does not respond to antiPD drugs but may improve w/ propanolol,primidone,and benzos

39. Treatment Options Preventive = no definite one available Symtomatic Pharmacological Surgical Non-motor management Restorative-experimental only Transplantation Neurotrophic factors Nonpharmacologic approaches PT/OT/ST No definite preventative tx available there is however wide range of sxmatic tx including pharmacologic and surgical methods. The non motr mgt include tx of depression,OH,excessive drowsiness,& psychosis. Finally, restorative therapies such as fetal or porcine cell transplantation are n expe use & neurotrophic factors susch as GDNF & small molecules such as neuroimmunophillins are being explored Rehabilitaion part is where I’m going to focus more since neurologist are by practice the ones managing their medications. Besides it will takeperhaps another hour to discuss the pharmacologic, side effects of these meds, we certainly discuss them some other time if you like.No definite preventative tx available there is however wide range of sxmatic tx including pharmacologic and surgical methods. The non motr mgt include tx of depression,OH,excessive drowsiness,& psychosis. Finally, restorative therapies such as fetal or porcine cell transplantation are n expe use & neurotrophic factors susch as GDNF & small molecules such as neuroimmunophillins are being explored Rehabilitaion part is where I’m going to focus more since neurologist are by practice the ones managing their medications. Besides it will takeperhaps another hour to discuss the pharmacologic, side effects of these meds, we certainly discuss them some other time if you like.

40. 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 Briefly on medical management; Dopaminergic therapy is d cornerstonr of sxmatic mngt of PD LD replaces dopamine presynaptically while dopamine agonists act directly on receptors post synaptically. LD is administered w/ peripheral decarboxylase inhibitor either benserazide or carbidopa. The newest class r the COMT inhibitors, also increase bioavailability of LD by inhibiting peripheral or central catechol o-methyl transferase. Other agents r anticholinergics, the MAO-B inhibitors selegiline and the antiviral amantadine. Bromocriptine=parlodel, pergolide=Permax,ropinirole=requip,pramipexole=mirapex COMT inhibitors-Tolcapone(Tasmar),Entacapone (Comtan) L-Dopa + Entacapone (Stalevo) MAO-B inhibitors- Selegiline (Eldepryl) Anticholinergics-Trihexyphenidyl(Artane),Benztropine (Cogentin) Antivirals-Amantadine L-Dopa(Sinemet), Dopamine Agonists- Bromocriptine (Parlodel), Ropinirole(Requip),Pramipexole(Mirapex) Briefly on medical management; Dopaminergic therapy is d cornerstonr of sxmatic mngt of PD LD replaces dopamine presynaptically while dopamine agonists act directly on receptors post synaptically. LD is administered w/ peripheral decarboxylase inhibitor either benserazide or carbidopa. The newest class r the COMT inhibitors, also increase bioavailability of LD by inhibiting peripheral or central catechol o-methyl transferase. Other agents r anticholinergics, the MAO-B inhibitors selegiline and the antiviral amantadine. Bromocriptine=parlodel, pergolide=Permax,ropinirole=requip,pramipexole=mirapex COMT inhibitors-Tolcapone(Tasmar),Entacapone (Comtan) L-Dopa + Entacapone (Stalevo) MAO-B inhibitors- Selegiline (Eldepryl) Anticholinergics-Trihexyphenidyl(Artane),Benztropine (Cogentin) Antivirals-Amantadine L-Dopa(Sinemet), Dopamine Agonists- Bromocriptine (Parlodel), Ropinirole(Requip),Pramipexole(Mirapex)

41. Medical Management Algorithm Medical management of PD can be quite complicated, as there are several classes of medications available, significant side effects to some of the medications, and no real consensus on which class of drug should be started at diagnosis. Different classes of medication often are combined to optimize symptom control. Medication management provides the most effective treatment of PD for the first 4-6 years. Thereafter, this disabling disease advances despite continuing medication management. Medical management of PD can be quite complicated, as there are several classes of medications available, significant side effects to some of the medications, and no real consensus on which class of drug should be started at diagnosis. Different classes of medication often are combined to optimize symptom control. Medication management provides the most effective treatment of PD for the first 4-6 years. Thereafter, this disabling disease advances despite continuing medication management.

42. Surgical Management Candidates for ablative surgery or deep brain stimuation 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.

43. Surgical Management Ablative Thalamotomy Relieves tremors Pallidotomy Improves cardinal signs Subthalamotomy Improves cardinal signs Motor fluctuations, dyskinesia

44. Surgical Management Ablative complications Speech impairments Cognitive deficits Dysphasia

45. Surgical Management Deep drain stimulation Thalamic Dec. tremor in 90% of pt No effect on cardinal signs Pallidal Improves cardinal signs, dyskinesia Subthalamic Improves cardinal signs, dyskinesia, motor fluctuations

47. Future Management Neural transplantation dopamine-producing cells, ex. fetal nigral cells. Gene therapy

48. Managing Early Complications :Altered Mental States Confusion,sedation,dizziness,hallucinations,delusions Reduce /eliminate CNS-active drugs of lesser priority Anticholinergics - Sedatives Amantadine - Muscle relaxant Hypnotics - Urinary antispasmodics Reduce dosage of DA,COMT inhibitor or LD When pts develop confusion,sedation,dizziness,halluci or delusions the simplest intervention is to reduce or eliminate sedating meds or antiPD meds of lesser priority.commonly used sedating meds includes hypnotics, sedatives,muscle relaxant,urinary antispasmodics. Low potency antiPD meds that may contribute significantly to confusion include anticholiner, amantadine and selegiline. When this option is not adequate, dosage reduction of LD, DA or COMT inhibitor m/b necessary. Drug related psychotic symptoms in early illness are often associated w/ atypical parkinsonian syndromesWhen pts develop confusion,sedation,dizziness,halluci or delusions the simplest intervention is to reduce or eliminate sedating meds or antiPD meds of lesser priority.commonly used sedating meds includes hypnotics, sedatives,muscle relaxant,urinary antispasmodics. Low potency antiPD meds that may contribute significantly to confusion include anticholiner, amantadine and selegiline. When this option is not adequate, dosage reduction of LD, DA or COMT inhibitor m/b necessary. Drug related psychotic symptoms in early illness are often associated w/ atypical parkinsonian syndromes

49. Late Complications Motor fluctuations, dyskinesias,dystonia,freezing,falls Behavioral/neuropsychological Depression,sleep d/o, psychosis Autonomic OH, hyperhidrosis ,constipation, impotence, urinary incontinence or retention Management of late stages of PD involves tx of motor response fluctuations, dyskinesias,dystonia,freezing and falls. In add, besides these motor fluctuations there r behavioral & neuropsychological concerns, such as depression, sleep d/o, & psychosis. Autonomic problems include OH, hyperhidrosis, constipation, impotence, u inc or retention.Management of late stages of PD involves tx of motor response fluctuations, dyskinesias,dystonia,freezing and falls. In add, besides these motor fluctuations there r behavioral & neuropsychological concerns, such as depression, sleep d/o, & psychosis. Autonomic problems include OH, hyperhidrosis, constipation, impotence, u inc or retention.

50. Stages in Decline of response to Levodopa (LD) I: Pt not aware of effect of individual dose II: Mid afternoon loss of benefit III: Loss of sleep benefit; early morning akinesia, possible foot dystonia IV: regular “wearing off” q 4 hrs at first, shortens with time V: Frequent wearing off, abrupt on-off, unpredictable dose response Development of motor fluctuations can occur as early as the first year after tx w/ LD,& d literatures suggest that 50% of pts have developed motor fluctuations after 5 yrs of LD tx. Duvoisin reviewed the stages of declining response to LD & has subdivided these into 5 categories. In stage I, pt un aware of individual dose response to LD. III loss of sleep benefits w/ early morning mobility problems awa possible foot cramping or dystonia usually on the most affected side. IV heralded by predictable wearing off approx q 4 hrs, this interval shortening with disease progression V freq wearing off, w/ sometimes abrupt & unpredictable response to LD dosing. Tx of response fluctuations is based on identifying causes of motor changes.Development of motor fluctuations can occur as early as the first year after tx w/ LD,& d literatures suggest that 50% of pts have developed motor fluctuations after 5 yrs of LD tx. Duvoisin reviewed the stages of declining response to LD & has subdivided these into 5 categories. In stage I, pt un aware of individual dose response to LD. III loss of sleep benefits w/ early morning mobility problems awa possible foot cramping or dystonia usually on the most affected side. IV heralded by predictable wearing off approx q 4 hrs, this interval shortening with disease progression V freq wearing off, w/ sometimes abrupt & unpredictable response to LD dosing. Tx of response fluctuations is based on identifying causes of motor changes.

51. LD Response Fluctuations Peripheral causes: delayed gastric emptying dietary protein short plasma half-life Central causes: pulsating delivery to striatal receptors impaired storage capacity alteration of DA receptor

52. Off- Period Dystonia Appears when LD dose is low esp in early AM w/ or w/o parkinsonism Dose adjustments, add ons: More frequent LD dosing to avoid low plasma levels Add DA, COMT inhibitor, MAO-B inhibitor

53. Wearing Off Regular and predictable decline in response 2-4 hrs after LD dose Most common motor fluctuation Dose adjustment, ad-ons: Change to LD-CR, or increase LD freq Reduce LD, add DA or COMT inhibitor

54. On-off Response Sudden and unpredictable off periods unrelated to dosing schedule One of the hardest features to manage Dose adjustments, add-ins: Reduce LD, add DA

55. 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

56. 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

57. 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

58. Depression Reported in 30-90% of PD patients Difficult to discern from vegetative symptoms Depression may be related to a deficit in serotonergic neurotransmission or to decreased cortical levels of norepinephrine and dopamine. Usually responds quickly to medications Selective serotonin re-uptake inhibitors Tricyclic Antidepressants If ECT needed, will transiently improve PD symptoms

59. Anxiety/Restlessness Primary anxiety disorder: treat with benzodiazepines Associated with “off-periods” or low-levopoda levels: adjust levopoda dosing Restless Leg Syndrome: benzodiazepines, narcotics, levopoda, dopamine agonists

60. Sleep Disorders Insomnia careful history difficulty with sleep initiation: tricyclic agents, benzodiazepines, diphenhydramine, chloral hydrate treat depression REM-behavioral disorder: clonazepam Excessive daytime sleepiness correct poor sleep at night discontinue anticholinergics, amantadine reduce dopamine agonist, levopoda dosages if possible selegeline, caffeine, methylphenidate 5-20 mgs/d

61. 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 Fludrocortisone (0.1-0.4mg/d) Midodrine (2.5-20mg/d)

62. Urinary Incontinence/Frequency Rule out urinary tract infection Bladder evaluation for detrusor hyperactivity *oxybutinin 5-30mg/d, propanthaline 7.5-15mg/d detrusor hypoactivity *phenoxybenzamine; prazosin Urinary frequency avoid fluid pooling in feet DDAVP inhaler, tolterodine tartrate 2mg hs to 2mg tid

63. Impaired GI Motility Constipation Vomiting Impaired absorption Treatment Options small frequent meals increased fiber/bulking agents stool softeners and suppositories

64. Sexual Dysfunction Medical screening Depression, anxiety, iatrogenic causes: S/E of SSRIs Endocrinologic evaluation Prolactin, testosterone, lutenizing hormone, thyroid screen Individual variation in effect of PD Some patients have short-lived hypersexuality with dopaminergic drugs Urologic evaluation Erectile dysfunction- SE of alpha and beta – adrenergic blockers, anxiolytics, digoxin, cimetidine Yohimbine, sildenafil

65. 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

66. Excessive Sweating Usually levodopa related, and may be seen at peak or trough dose drug levels reduce levodopa add dopamine agonist or COMT inhibitor add carbidopa add Beta-blocker

67. 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

68. Rehabilitation Impairments Gait disturbance Decreased stride length, cadence, velocity. Festination Stooped flexed posture Cautious gait(fear of falling) Impaired balance

69. Rehabilitation Management Rehabilitation interventions are directed at the main causes of impairments. Multidisciplinary approach:PT,OT,ST,D,RT,Neuropsych

70. 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. This study demonstrated significant improvement in activities of daily living and motor function but no improvement in tremor, mentation, and mood. These improvements returned to baseline 6 months after termination of the intervention. Long-term rehabilitation programs have been advocated, but the stability of the benefits gained in these programs has not been demonstrated. Because PD is a progressive CNS disorder with progressive disability over time, the merits of therapy often are debated. Some studies have shown benefits in certain areas (eg, gait, independence in ADL, fine motor movements) in patients receiving therapy and medication versus those receiving only medication; however, the trials generally are quite small and improvements modest. Because the studies vary in the type of therapy and medications used, the reliability of combining the results of several trials is very small. Because PD is a progressive CNS disorder with progressive disability over time, the merits of therapy often are debated. Some studies have shown benefits in certain areas (eg, gait, independence in ADL, fine motor movements) in patients receiving therapy and medication versus those receiving only medication; however, the trials generally are quite small and improvements modest. Because the studies vary in the type of therapy and medications used, the reliability of combining the results of several trials is very small.

71. 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.

72. Rehabilitation Hoehn and Yahr Rating scale divided into five stages Stage 0 = no visible disease; Stage I = disease that involves only one side of the body; Stage II = disease that involves both sides of the body. but does not impair balance: Stage III = disease that impairs balance or walking; Stage IV = disease that markedly impairs balance or walking: and Stage V = disease that results in complete immobility.

73. Rehabilitation Hoehn and Yahr 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.

74. Treatment Plan Maintain 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

75. 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 Psychosocial intervention Dietary/Nutrition Pt/Family training-education

76. 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

77. 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 often is directed at the main causes of impairment and includes measures to decrease rigidity and increase range of motion (ROM), as well as to improve postural control, endurance, mobility, and gait. Treatment of bradykinesia and rigidity often includes daily stretching and ROM exercises, as well as task-specific activities. Gait and ambulation can be improved through a program of stretching and strengthening of the lower extremities that uses exaggerated steps and arm swings, marching steps (paced to the beat of a metronome), and mental rehearsal and imaging. The physical therapist should assess the need for ambulation aids (eg, walkers, canes) while completing gait training with the patient.Physical therapy often is directed at the main causes of impairment and includes measures to decrease rigidity and increase range of motion (ROM), as well as to improve postural control, endurance, mobility, and gait. Treatment of bradykinesia and rigidity often includes daily stretching and ROM exercises, as well as task-specific activities. Gait and ambulation can be improved through a program of stretching and strengthening of the lower extremities that uses exaggerated steps and arm swings, marching steps (paced to the beat of a metronome), and mental rehearsal and imaging. The physical therapist should assess the need for ambulation aids (eg, walkers, canes) while completing gait training with the patient.

78. 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 Exercises for the patient with PD should emphasize trunk extension, as well as lateral and rotational mobility, weight shifting, and balance training. Addressing how to fall safely and get up from the floor is important for patients with PD and their families. The physical therapist should instruct them in proper transfer techniques and try to improve their overall safety awareness during everyday activities. A general conditioning program also should be included in physical therapy to improve the patient's endurance. In addition, the physical therapist may instruct the patient and family members in a home exercise program.Exercises for the patient with PD should emphasize trunk extension, as well as lateral and rotational mobility, weight shifting, and balance training. Addressing how to fall safely and get up from the floor is important for patients with PD and their families. The physical therapist should instruct them in proper transfer techniques and try to improve their overall safety awareness during everyday activities. A general conditioning program also should be included in physical therapy to improve the patient's endurance. In addition, the physical therapist may instruct the patient and family members in a home exercise program.

79. 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

80. Occupational Therapy Patient and caregiver education goals of program transfers, task simplification, positioning, etc. Home exercise program Home and workplace modifications Patient and caregiver education goals of program transfers, task simplification, positioning, etc. Home exercise program Home and workplace modifications

81. Speech Therapy Swallowing evaluation including modified barium swallow Cognitive evaluation Articulatory speech training for dysarthria Early therapy esp effective Teaching conpensatory strategies for safer swallow Speech therapy may be underemployed in patients with PD, given that speech and swallowing problems are common causes of disability in this population. Speech therapy seems to improve the quality of voice in patients with hypokinetic dysarthria. Therapy itself generally emphasizes better breath and rate control, as well as improved articulation and better volume. Beneficial effects of the therapy do not seem to persist after it has been discontinued. Dysphagia tends to occur later in the disease process and can lead to drooling, aspiration, malnutrition, and inability to ingest medications. Speech therapy interventions can include positioning the neck in flexion, teaching a double swallow technique, using smaller amounts of food, or modifying the patient's diet and incorporating thickened liquids. A modified barium swallow analysis may be helpful in guiding the therapy plan and in monitoring the patient's progress.Speech therapy may be underemployed in patients with PD, given that speech and swallowing problems are common causes of disability in this population. Speech therapy seems to improve the quality of voice in patients with hypokinetic dysarthria. Therapy itself generally emphasizes better breath and rate control, as well as improved articulation and better volume. Beneficial effects of the therapy do not seem to persist after it has been discontinued. Dysphagia tends to occur later in the disease process and can lead to drooling, aspiration, malnutrition, and inability to ingest medications. Speech therapy interventions can include positioning the neck in flexion, teaching a double swallow technique, using smaller amounts of food, or modifying the patient's diet and incorporating thickened liquids. A modified barium swallow analysis may be helpful in guiding the therapy plan and in monitoring the patient's progress.

82. Dysphagia The videofluoroscopic swallowing- gold standard for dx oral-phase:prolonged chewing, excessive postswallow residuals, poor bolus control, and repetitive tongue motions. pharyngeal phase: vallecular and piriform pooling, delayed triggering of the swallow reflex, and delayed laryngeal elevation insufficient evidence to support or refute the utility of dysphagia training

83. Techniques to Improve Speech Increase loudness Face the listener directly Emphasize key words Use short sentences imagery 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”

84. Management of Swallowing Difficulty and Sialorrhea 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.

85. 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. Consultation should be obtained from an attorney regarding flexible power of attorney and a living will prior to the onset of dementia associated with the advanced form of PD. Consultation should be obtained from an attorney regarding flexible power of attorney and a living will prior to the onset of dementia associated with the advanced form of PD.

86. Recreational Therapy identifying previous recreational interests new interests can be identified and explored social and recreational pursuits Because of the high level of impairment and disability seen in many patients with PD, it is not surprising that avocational pursuits for these individuals often become more difficult. This change certainly can have a detrimental impact on a patient's overall quality of life. A recreational therapist may be helpful in identifying previous recreational interests and in helping the patient to pursue them once more, with or without assistance. If such pursuits are no longer possible, new interests can be identified and explored. The therapeutic value of social and recreational pursuits should not be underestimated in patients with PD, because many of these individuals can feel isolated and lonely because of the effects of the disease.Because of the high level of impairment and disability seen in many patients with PD, it is not surprising that avocational pursuits for these individuals often become more difficult. This change certainly can have a detrimental impact on a patient's overall quality of life. A recreational therapist may be helpful in identifying previous recreational interests and in helping the patient to pursue them once more, with or without assistance. If such pursuits are no longer possible, new interests can be identified and explored. The therapeutic value of social and recreational pursuits should not be underestimated in patients with PD, because many of these individuals can feel isolated and lonely because of the effects of the disease.

87. Community Resources Social worker intervention: Social Security office Medicare, Medicaid In-home programs Meals on Wheels, home visiting, etc.

88. 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

89. 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 concentrate in evening meal Proper nutritional support is essential, including adequate dietary fiber to prevent the common problem of constipation. Proper nutritional support is essential, including adequate dietary fiber to prevent the common problem of constipation.

90. Consultations Consult with a neurologist for (1) initiation and management of medical therapy, (2) access to clinical medication trials if patient desires, and (3) management of side effects of L-dopa therapy. Consultation with a neurosurgeon may be indicated for a surgical opinion in patients who are resistant to standard medical therapy or who develop significant complications secondary to L-dopa therapy. Consult with a psychiatrist for management of depression in patients who do not respond to typical treatment options, such as the use of selective serotonin reuptake inhibitors (SSRIs), or who show evidence of contemplating suicide.

91. Prognosis/Complications Poor Prognostic indicators Old age of onset Early cognitive deficits Lack of tremor Complications Underlying medical illness (eg, sepsis, pneumonia, fecal impaction, urinary tract infection) should be suspected in a PD patient with a rapid deterioration or new PD symptoms Underlying medical illness (eg, sepsis, pneumonia, fecal impaction, urinary tract infection) should be suspected in a PD patient with a rapid deterioration or new PD symptoms Underlying medical illness (eg, sepsis, pneumonia, fecal impaction, urinary tract infection) should be suspected in a PD patient with a rapid deterioration or new PD symptoms

92. Miscellaneous Concerns Seborrheic dermatitis shampoos or lotions with ketoconazole, selenium, pyrithione zinc Driving assess regularly for reaction speed, judgment, mental status retake driver’s test

93. Education, Support and Counseling Patient/caregiver education: newsletters, Web resources Support groups: patient, caregivers may be appropriate to wait for disability progression early-onset patients may desire separate group Counseling both patient and caregiver/family; assess needs separately anxiety, grief, guilt, anger, isolation, depression

94. U.S. Support Groups National Parkinson Foundation, Inc. TEL: (305) 547-6666 www.parkinson.org Parkinson’s Disease Foundation TEL: (312) 733-1893 (Chicago) TEL: (800) 457-6676 (New York) www.pdf.org The American Parkinson’s Disease Assoc., Inc. TEL: (800) 223-2732 or (718) 981-8001 www.apdparkinson.com The Bachman-Strauss Dystonia & Parkinson Foundation, Inc. TEL: (212) 241-5614

95. References http://www.braddomtext.com PM&R Board Review by Sara Cuccurullo http://www.mdvu.org/library/slides/pd.asp(wemove.com) http://www.emedicine.com/pmr/topic99.htm http://www.emedicine.com/NEURO/topic304.htm