Diagnosis and Management of Parkinson s Disease. Background and Definitions. When case reports were still in vogue.

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1 Diagnosis and Management of Parkinson s Disease Raghav Govindarajan, MD, FISQua, FACSc Assistant Professor Department of Neurology University of Missouri, Columbia Background and Definitions Parkinson s disease was first described by Dr. James Parkinson in his paper An Essay on the Shaking Palsy in Slowly progressive neurodegenerative disorder with no identifiable cause. The fourth most common neurodegenerative disease of the elderly Affects about 1% of the population over 55 years of age. When case reports were still in vogue. 1

2 Pathology Massive loss of the pigmented neurons and gliosis, most prominently in the substantia nigra with presence of Lewy bodies. Loss of approximately 80% of these neurons results in the presence of clinical symptoms. Pathology Normal PD Courtesy of Kapil D. Sethi, MD Courtesy of Kapil D. Sethi, MD Vagotomy and subsequent risk of Parkinson's disease Parkinson's disease (PD) may be caused by an enteric neurotropic pathogen entering the brain through the vagal nerve, a process that may take over 20 years. Results Risk of PD was decreased after truncal vagotomy when compared to the general population cohort by half as compared to general population. Interpretation Full truncal vagotomy is associated with a decreased risk for subsequent PD, suggesting that the vagal nerve may be critically involved in the pathogenesis of PD. Ann Neurol 2015;78:

3 Histology of PD Showing Lewy Body Disease Onset Average age of onset years Approximately 5% of cases occur before age 40 (young onset) Slowly progressive over years Motor Symptoms of PD Resting Tremor Rigidity (Cogwheel) Bradykinesia (slow movement) or Akinesia (absence of movement) Postural Instability (balance and coordination) 3

4 Tremor First Sign in 75% of patients Occurs at rest Does not need to be present to make a diagnosis Typically on ones side of body and involves a distal extremity (hand, leg) Rigidity Stiffness caused by an involuntary increase in muscle tone Can affect all muscle groups Often presents as back, neck or shoulder discomfort Often dismissed as arthritis; referrals to orthopedists initially 4

5 Akinesia/Bradykinesia Absence of Movement Describes the difficulty Parkinson s patients have in initiating and executing a motor plan. Early signs include microphagia (small writing) and loss of dexterity. Facial: Drooling, hypomimia (masked face). Vocal: hypophonia (soft voice). Postural Instability Usually the last motor sign to appear. Often the most disabling and least treatable problem. No single factor alone is responsible. Freezing is a form of akinesia which is most problematic during ambulation and often leads to falls. Non-Motor Symptoms Dysautonomias (problems in functioning of the autonomic nervous system) *constipation *impotence *urinary problems *orthostatic hypotension *regulation of heat *sensory disturbances *problems swallowing *pain 5

6 Non-Motor Symptoms, con t Speech problems Behavioral problems, including: depression anxiety panic attacks agitation Sleep Disorders Non-Motor Symptoms, cont. Loss of smell Constipation Cognitive (thinking) problems, including dementia Fatigue PARKINSON S SYMPTOMS VARIABLE from person to person VARIABLE from day to day VARIABLE response to treatment 6

7 Parkinsonism A clinical syndrome characterized by specific motor deficits. An underlying cause is usually identified: chemicals (drugs), structural NPH, or possibly a neurodegenerative disorder (PSP, MSA). Clinical Features That May Suggest a Diagnosis Other Than PD Early onset of postural instability Axial more than appendicular rigidity Poor response to adequate dosages of levodopa Early dementia Supranuclear gaze palsy DAT Scan 7

8 Clinical Pearl Parkinson disease is a clinical diagnosis. No laboratory biomarkers exist for the condition, and findings on routine magnetic resonance imaging (MRI) and computed tomography (CT) scan are unremarkable. Clues for Parkinsonism Early Features Early Falls especially when coming down the stairs Early Dementia with fluctuating cognition and hallucination Diagnosis Progressive Supranuclear Palsy Dementia of Lewy Body disease Significant autonomic symptoms Multisystem atrophy Dyspraxic, dystonic limb Corticobasal degeneration How is P.D. Treated? *rest and relaxation *exercise *stress management *nutrition *rehab therapy PT/OT, speech *mental health counseling *education *support (e.g. support groups) 8

9 Treatment and Intervention Pharmacologic Intervention Considerations: *Degree of functional impairment *cognitive impairment *Age (potential side effects) *Cost Medication Complex: Know action, dosage, side effects. Used to treat symptoms, not cure. No two people respond the same. Own responses vary. Need to monitor and change medication regime over time. Medication, con t Newly diagnosed: may hold off until symptoms interfere May start with low levels and work upwards. May use multiple medications. PD meds may interact with others. 9

10 Types of Medications Levodopa/Carbidopa (Sinemet, Sinemet CR) Dopamine Receptor Agonists (Pramipexole, Ropinirole) MAO Inhibitors (Rasagaline) Catechol-O-Methyl Transferase inhibitors (Entacapone) Anticholinergics (trihexiphenidyl) Amantadine 10

11 Frequent Side Effects of Meds Orthostatic hypotension Memory loss or confusion Agitation Depression Hallucinations and psychosis Sleep disturbances/daytime sleepiness Nausea Motor Fluctuations 11

12 Challenges of Medications Timing Monitor and adjust Side effects Complications Drug interactions Cost Frustration Incorporating med regimen into setting American Academy of Neurology Evidence Based Recommendations Entacapone and rasagiline should be offered to reduce off time (Level A) Pergolide, pramipexole, ropinirole and tolcapone should be considered to reduce off time (Level B) Sustained release carbidopa/levodopa and bromocriptine may be disregarded to reduce off time (Level C) Amantadine may be considered to reduce dyskinesia (Level C) 12

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14 Surgery for PD Deep Brain Stimulation Good for pts with Severe tremor Dyskinesias On/off fluctuations Medical failures Not for?> 70 yrs Sig. cognitive impairment/mood disorder Dopamine dysregulation syndrome Realistic expectations Does not improve non motor symptoms Not neuroprotective (ie disease continues to progress) Does not help axial symptoms (postural instability, freezing, falling) 14

15 Management contd Physical Therapy Occupational Therapy Speech and Language Therapy Mental Health Counseling Evidence Based PT in Parkinson PD patients with more than 1 fall in previous year are likely to fall again within next 3 months Most falls occur during transfers and freezing of gait Therefore, PT should focus on: Promoting active lifestyle Active exercises to improve balance, muscle power, joint mobility, and aerobic capacity Cueing strategies Postural adjustments in bed or W/C Assisted active exercises Safety Management: Ambulation Ambulation Avoid rubber or crepe soled shoes Visual, auditory cues Identify problem areas, e.g. narrow hallways, doors Remove hazards, e.g. area rugs Concentrate on one task at a time Ambulatory aids Avoid pivot turns 15

16 THANK YOU 16