1. 2016 MOVEMENT DISORDERS Hal S. Pineless, D.O., FACN
NeuroCare Institute of Central Florida
Winter Park, FL 32792
Clinical Assistant Professor of Medicine (Neurology)
Florida State University College of Medicine

2. Cardinal Features of Parkinson’s Disease
Bradykinesia
Postural instability
Truncal rigidity
Resting Tremor

3. Parkinson’s Disease Treatments
Carbidopa/levodopa Dopamine Agonists COMT inhibitors; MAO-B inhibitors Amantadine and anticholinergics Deep Brain Stimulator (DBS)

4. Anticholinergics
Not used much today due to side effects (poor cognition, dementia)
Great for treating Parkinson resting tremors
Trihexyphenidyl (Artane) 2 mg TID is usual dose
Used in psychiatry for tardive dyskinesias

5. Carbidopa/levodopa
Still the gold standard in treating PD, even after all these years
Available in regular, ER/CR, enteral formulations
Hallucinations, orthostatic hypotension, GI, dyskinesias are SE
Carbidopa/levodopa ER (Sinemet CR) 25/100, 50/200
Carbidopa/levodopa (Sinemet) 10/100, 25/100, 25/250
Rytary –ER formulation given TID—dose varies

6. Dopamine Agonists
Pramipexole and ropinirole are the most common ones used today
Pramipexole (Mirapex): Available in regular (TID) and ER form
Ropinirole (Requip): Available in regular (TID) and XL form
Maximum dose of pramipexole is 4.5 mg daily
Maximum dose of ropinirole is 24 mg daily
Rotigotine patch (Neupro)—maximum dose is 8 mg/24 hours
Rotigotine patch dose=ropinirole dose=1/4 for pramipexole dose
Compulsive behaviors and hypersexuality notable side effects

7. COMT Inhibitors Entacapone (Comtan) 200mg is the most common one used
Used to enhance effectiveness of carbidopa/levodopa
Can’t be used without carbidopa/levodopa
Give at same time as carbidopa/levodopa
Orange colored urine is side effect
Similar side effects as carbidopa/levodopa
Ticlopidine 200 mg TID can be used, but aplastic anemia is risk

8. MAO-B Inhibitors
Rasagaline (Azilect) and Selegiline (Eldepryl, Zelpar)
Rasagaline 0.5 mg initially, then 1 mg q AM thereafter
Rasagaline doesn’t raise BP; selegiline might raise BP due to amphetamine metabolities
Neuroprotective?
Can be used initially or adjuctive in treatment of PD
Interaction with SSRIs causing serotonin syndrome is theoretical!

9. Amantadine Amantadine 100 mg BID-TID
Useful for postural instability (imbalance) in PD
Helpful with PD tremors
Has antiviral properties (flu prevention)
Can be used for MS related fatigue
Biggest side effect is visual hallucinations

10. Deep Brain Stimulation (DBS)
Used in medically refractory cases of PD. Should be used sooner?
Great with treating tremors (Parkinson and BET)
Useful with rigidity, bradykinesia, dyskinesias
Avoid in Parkinson-Plus syndromes, psychotic patients, alcoholics
Levodopa Effect=DBS effect
Use with caution in dementia patients

11. How I Treat Parkinson’s Disease
Exercise 30 minutes daily at least 5 days a week
Over age 75: Carbidopa/levodopa ER 25/100 TID to start. Add entacapone later if needed before increasing carbidopa/levodopa. May use rasagaline before instituting carbidopa/levodopa or later in therapy. Dopamine agonist? Amantadine if imbalance or bad tremors.
Under age 75: Rasagaline? Carbidopa/levodopa ER 25/100 TID to start. Add dopamine agonist. Push dopamine agonist to toxicity. Entacapone. Amantadine if imbalance or bad tremors. DBS?
Movement disorder tertiary center?

12. Parkinson-Plus Syndromes
Lewy Body Disease/Dementia=Dementia with Lewy Bodies (LBD)
Progressive Supranuclear Palsy (PSP)
Multisystem Atrophy (MSA)
Corticobasal degeneration (CBD)
Not responsive to anti-PD drugs

13. Lewy Body Dementia (LBD)
Sometimes classified as a dementia (ICD-10)
Progressive cognitive decline with EPS
Visual hallucinations early in disease are pathognomonic

14. Progressive Supranuclear Palsy (PSP)
Also known as Steele-Richardson-Olszewski syndrome
Frequent falls
Impaired vertical gaze, especially downward gaze
Emotional and personality changes, like PBA

15. Multisystem Atrophy (MSA)
Formerly known as Shy-Drager Syndrome
Characterized by symptoms of ANS failure
Lightheadedness, syncope, constipation, ED, urinary retention
EPS, dysarthria
Loss of muscle coordination
Check for orthostatic hypotension in the office

16. Corticobasal Degeneration (CBD)
Main symptom is apraxia (inability to perform coordinated movements or use familiar objects)
Pronounced asymmetry
Stiffness that is worse than PD
Myoclonus (twitching or jerking), usually in the hand

17. Benign Essential Tremors
Also known as benign familial tremors
Primidone and propranolol/propranolol ER effective treatments
Primidone mg daily; push to toxicity
Propranolol mg/d: watch for BP < 90/50 or HR < 50
Alcohol improves tremors, but alcoholism worsens tremors
Weighted kitchen utensils, pens help decrease tremors
Bronchodilators, stress, CNS stimulants increase tremors
Alprazolam, topiramate, gabapentin, clonazepam, DBS probably work

18. Cervical Dystonia (Spasmodic Torticollis)
Involuntary contractions of the shoulders and neck causing abnormal postures or movements of the neck, shoulders and head
Treatment of choice: onabotulinumtoxinA (Botox); rimabotulinumtoxinB (Myobloc); abobotulinumtoxinA (Dysport)
Antipsychotic and antinausea meds might trigger cervical dystonia
Prototype: female; aged 40-70; family history positive for it
Other treatments: anti-PD drugs, pain meds, muscle relaxants, PT
Selective denervation surgery; DBS in very rare cases

19. Wilson’s Disease
Rare, inherited disorder causing high copper in organs (liver, brain)
Symptoms start in ages 12-23
Low serum ceruloplasmin, high copper levels
Signs & symptoms: fatigue, decreased appetite, abdominal pain, jaundice, edema, dysarthria, dysphagia, EPS, muscle stiffness, depression, personality changes, psychosis
Kayser-Fleischer rings (golden brown discoloration) on eyes
Hereditary: ATP7B gene
Treat with penicillamine, trientine, zinc acetate, liver transplant

20. Kayser-Fleischer Ring

21. Tourette’s Syndrome
Developmental disorder that begins in childhood and teenage years
Chartacterized by motor and vocal tics
ADHD and OCD often accompany Tourette’s
No cure, but there are treatments
Treatments: haloperidol, pimozide, clonidine, behavioral therapy

22. Huntington’s Disease (HD)
Genetic disorder causing breakdown of neurons in the brain, causing death years after diagnosis
Symptoms appear in ages 30-50, but can be earlier or later
Chorea, abnormal body postures, behavioral changes, dementia, personality changes, dysarthria, dysphagia
Caused by mutation in huntingtin gene that cause cytosine, adenine, and guanine (CAG) building blocks of DNA to repeat more times than normal. Each child of a parent with HD has a 50% chance of inheriting the HD gene.
Treatment: tetrabenazine, deutetrabenazine for HD chorea