Clin Med - Movement Disorders Flashcards
Tourette’s characteristics
- definition
- exacerbation
- attenuation
- sleep
- abrupt, brief, repetitive movement of sound (tics) preceded by a premonitory sensation or “urge
- Exacerbation: stress, excitement, suggestion and suppression
- Attenuation: physical activity and distraction
- Sleep: Tics may persist
Tourette’s incidence
- MC movement disorder in children (onset median age 6)
- male > female
- 50% are tic free by age 18
Tourette’s DSM-5 criteria
- Multiple motor AND vocal tics
- Greater than 1 year in duration
- Started prior to age 18
- Not a medication side effect
- Rule out other medical conditions (seizure disorder, Huntington’s Disease, psychogenic – behavior problems)
Tourette’s motor tics
-focal
- Eye blinking: most common
- Facial grimacing
- Jaw movement
- Head bobbing
- Shoulder shrugging
Tourette’s motor tics
-complex
- Hopping
- Jumping
- Clapping or snapping fingers
Tourette’s vocal tics
- Sniffing – MC
- Throat clearing
- Grunting
- Snorting
- Echolalia: repeating others (MC of the true vocal tics)
- Coprolalia: inappropriate language (rare)
Tourette’s co-morbidities
- 85% will have some comorbidity psych disorder.
- 57% have more than 2 psychiatric disorders
-Most common: ADHD (50%) and OCD (25-50%)
Tourette’s and bi-polar disorder
MC in patients having tics lasting past age 18.
-more common in patients treated with antidepressants
Tourette’s neuroleptics
-typical antipsychotics (1st gen)
- Haloperidol (Haldol)
- pimozide (Orap)
- fluphenazine (Prolixin)
Tourette’s neuroleptics
-atypical antipsychotics
- Risperidine (Risperdol)
- Aripiprazole (Abilify)
- Olanzapine (Zyprexa)
- Quetiapine (Seroquel)
- Ziprasidone (Geodon)
Other meds for Tourette’s
- Alpha Agonist [Clonidine (Catapres), Guanfacine (Tenex)]
- Antiepileptics [Toparimate (Topamax), Levetracetam (Keppra)]
- Antidepressents: SSRI, SNRI
- Stimulants: Ritalin, Adderall etc.
- THC: Controversial
Botox for Tourette’s
- effective for focal and phonic tics
- 93% improved
- 50% tic free
- side effects: ptosis, neck weakness, hypophonia (80%)
Comprehensive Behavioral Intervention for Tic
- habit reversal training
- self awareness
- competing response
- most effective in older adolescents without ADHD
Deep brain stimulation for Tourette’s
- Not FDA approved
- Current lesion targets: thalamus and internal capsule of the globus pallidus
Essential tremor
-definition
-involuntary, rhythmic, oscillating movement of the head, upper extremity, or voice
Essential tremor
-etiology
- Genetic relation in an Autosomal Dominant pattern (doesn’t have to follow this pattern but it’s MC)
- no gene has been identified
HPI for essential tremor
- Is there a family history (50% with +FH)
- Age of onset (Median age: 45)
- Location (hands, head, and voice)
- Current medications
- Progression (unilateral and slow)
- Exacerbation: stress, fatigue, caffeine, postural, kinetic
Essential tremor
-characteristics
- MC tremor
- worsens with stress, fatigue, anxiety, movement
- interfere with writing, drinking, eating, working, social
- improved with modest ETOH consumption
- absent with sleep
Essential tremor exam
- Tremor noted with action
- Arms in extension and in flexion with fingers in front of the face without touching
- Vocalization of “e” and “ah”
- Handwriting skills
- Fine motor tasks
Occupational therapy for mild essential tremor
- vocational changes
- biofeedback and relaxation techniques
- avoidance of stimulants (like caffeine)
- mild ETOH consumption during stress situations (lasts up to 3 hrs)
- weight objects (wrist weights, writing, and utensils)
Essential tremor pharm - primidone
*first line: primidone (Mysoline)
- side effects: sedation and flu-like
- tolerance can occur
- caution: debilitated, OSA not on CPAP, impaired hepatic and renal function
- contraindications: porphyria, severe respiratory depression, pregnant or breast feeding
Essential tremor pharm - propanolol
*first line: propanolol (inderal) PRN or chronic
PRN: 20-80mg 30 minutes-1 hr prior to an event
- side effects: fatigue, bradycardia, decreased exercise tolerance, bronchospasm, constipation
- contraindications: heart failure, conduction disorders, asthma, diabetes
Essential tremor pharm - second line or combo
-benzo
Benzodiazapines
- Alprazolam (Xanax)
- Clonazepam (Klonopin)
- Lorazepam (Ativan): works best with anxiety induced tremor or in combination
Essential tremor pharm - second line or combo
-anticonvulsants
- Topiramate (Topamax): requires high doses and not tolerated well
- Gabapentin (Neurontin): minimal efficacy
Essential tremor pharm
-botox
Botox works well for head and vocal tremors, doesn’t work for upper extremity tremors.
Essential tremor
-surgery
Stereotactic/Gamma Knife Thalamotomy (unilateral)
- ventralis intermedius (VIM) nucleus of the thalamus
- functional improvement 78-100%
- recurrence 20-30%
Deep brain stimulation
- VIM of the thalamus
- reduce risk of dysarthria d/t ability to control
Huntington’s Disease
-genetics
- Autosomal Dominant
- Chromosome 4p16.3
- Gene codes for the protein Huntingtin
- Mutation results in the addition of CAG (glutamine) repeats
- Normal <27 repeats
- > 40 repeats = Huntington’s Disease
Huntington’s disease
-CAG repeats
- normal: <27
- 50% chance of disease if 37 repeats
- 100% chance of disease if >40 repeats
- childhood onset if >60 repeats (Westphal variant – death early)
Huntington’s disease
-pathophysiology
mHTT protein is responsible for cell loss and gliosis of the striatum (basal ganglia) and thalamus resulting in severe atrophy and loss of the inhibitory pathway (dopamine).
Huntington’s disease
-incidence
- 7-10 per 100,000 in the USA
- age of onset: 4th decade (usually between 35-39)
- death: usually 15-20 years from onset
Westphal variant (> 60 repeats of CAG):
- onset < 20 years old
- more severe with rapid progression
- death: 10-15 years from onset (by age 30-35)
Huntington’s disease
-triad of symptoms
- behavioral
- Depression (40%), Anxiety/OCD, Irritability, Impulsivity
- Increased rates of divorce, job loss, imprisonment, and suicide - cognitive
- executive function (attention, concentration, planning)
- memory deficit (progresses to dementia) - motor
- clumsiness, dropping items, wide base gait, slurred speech
- CHOREA: Involuntary, irregular, spasmodic movement, “dance like”
- progresses to rigidity, dyskinesia and immobility
Huntington’s disease
-physical exam
- Generalized flexion/extension movements
- Unable to perform rapid alternating movements
- Difficulty staying on task
- Limited eye movement on exam
- Unable to withstand a position over time
- Dysphagia
- Choke on food and water
- Awkward, clumsy, drop objects
Huntington’s disease
-referrals
- genetic counseling
- neurology
- psychology/psychiatry
- social services
Huntington’s disease
-treatments
- no cure
- only symptomatic/complex and require continuous monitoring and adjusting
- behavioral treatment (try to minimize meds)
Huntington’s disease
-cognitive treatment
Cholinesterase inhibitors and NMDA receptor antagonist have not provided any known improvement in cognitive deficit
Huntington’s disease
-motor treatment
- Tetrabenzine (Xenazine): FDA approved for HD [Black Box Warning: increased risk of depression and suicide]
- Haloperidol (Haldol)
- Risperidone (Risperdal)
- Quetiapine (seroquel)
Define Parkinson’s disease
Progressive neurodegenerative condition resulting from the death of the dopamine-containing cells of the substantia nigra
What other conditions are often mistaken for/are similar to Parkinson’s?
- Shy-drager syndrome
- Progressive supranuclear palsy
- Lewy body dementia
- Normal pressure hydrocephalus
- Essential tremor
- Huntington’s disease
- Wilson’s disease
- Enecephalopathy
- Drug-induced (neuroleptics)
Which neuroleptics can cause Parkinson-isms?
- antipsychotics
- phenergan
- metaclopramide
Parkinson’s Incidence
- Progressive neurodegenerative disorder
- 160 per 100,00 prevalence
- Mean age of onset over 60 years old
- M>F
Parkinson’s diagnosis criteria
Bradykinesia with at least one of the following:
- rigidity
- resting tremor
- postural instability
With at least 3 supportive criteria:
- Unilateral onset
- Progressive signs and symptoms
- Persistent asymmetry of affected side
- Response to levodopa
- Levodopa dyskinesia
- Clinical course for more than 10 years
Primary vs. Secondary Classification
Primary = Parkinson’s disease
Secondary:
- Drug induced: anti-psychotics, anti-emetics, dopamine depleting (reserpine, tetrabenzaprine), meperidine
- Toxins- MPTP
- Multi-infarct into substantia nigra or deep brain tissues
- Dementia-puglistica: post traumatic
Parkinson’s early symptoms
- Fatigue
- Sleep disorder: insomnia
- Dystonia: foot cramps mostly at night
- Restless Leg Syndrome
- Depression/Anxiety
- Minor cognitive deficit: mostly slowness
- Loss of smell
Parkinson’s
-cardinal features
- Slow ocular movements
- Positive Doll’s eye
- Myerson’s sign: glabellar tap = blinking
- Masked facies
- Finger tapping
- Pronation/supination
- Finger to nose
- Sit to stand
- Gait-limited arm swing*
- Block turning*
- Micrographia*
- Hypophonia*
*occur in later stages
Parkinson’s resting tremor
- “Pin rolling”
- Attenuates with movement
- Low frequency
- Absent in 25% of patients
Parkinson’s rigidity on exam
- axial (head and neck ROM decreased
- distal: upper extremity (elbow ROM decreased in flexion/extension and wrist “cogwheel” rigidity)
*exacerbated by distraction
Parkinson’s postural instability on exam
- pull test positive: retropulsion
- forward flexion on gait*
- shuffling gait, fenestrated gait*
*later in disease progression
What is the pull test (retropulsion) for Parkinson’s?
- it is a cerebellar test
- you pull someone back towards you and as you pull them back their instinct should be to correct that and come forward
- but these patients don’t have that stability, so they’ll come back with you
Parkinson’s exam
-cognition
in later stages, patients develop dementia.
*important to do mini mental exam at every important so you can track progression
Parkinson’s imaging
- typically not needed
- if under 55 or other neuro finding, you can get CT or MRI
-DaT scans: SPECT scan looking at dopamine in the brain
Treatment for Parkinson’s
- physical therapy
- speech therapy
- medications
- surgery
Anticholinergics - early stages < 60
- Trihexyphenol (Artane): therapeutic 6-10mg/day
- Benzotropine (Cogentin): max 6mg/day
Side effects: memory loss, psychosis, orthostasis, constipation, dry mouth
Anticholinergics - early stages > 60
Amantadine (Symmetrel): max 400mg/day
Less side effects
Parkinson’s tx
-dopamine
Carbidopa/Levodopa (Sinemet)
- Levodopa able to cross the BB barrier*
- Causes nausea
- Addition of carbidopa improves nausea
- Side effects: dyskinesia
When should you start dopamine meds for Parkinson’s? Why?
Don’t start initially until symptoms have become refractory and debilitating or significant instability issues.
Once you start, you continuously titrate up until you reach max dose.
Parkinson’s tx
-dopamine agonist
- Pramipexole (Mirapex)
- Ropinirole (Requip)
- Bromocriptine (Parlodel)
Adjuvant to Sinemet
Parkinson’s tx
-COMT inhibitors
- Entacapone (Comtan)
- Tolcapone (Tasmar)
Parkinson’s tx
-MAO-B inhibitors
- Rasagiline (Azilect)
- Selegiline (Zelapar)
Parkinson’s tx for psychosis
Quetiapine (Seroquel)
Surgery for Parkinson’s
Thalamotomy/Pallidotomy: unilateral
- impaired language learning: dominant
- impaired visuospatial ability: non dominant
Deep Brain Stimulator: bilateral
-STN vs globus pallidus
What do you call a pig that does karate?
Pork chop
:)
