Movement Disorders Flashcards
What is the most common movement disorder?
Parkinsons Disease
What percent of the population is affected by PD?
1-2%
Which gender is more commonly affected by PD?
Males
What is the second most common neurodegenerative disorder?
Alzheimers
What is the second most common neurodegenerative disorder?
Parkinsons Disease
Most common age group for the diagnosis of Parkinsons?
50-70
What genes have an association to Parkinsons?
LLRK2, PARK2, PARK7, PINK1 and SNCA
What are risk factors for Parkinsons Disease?
- Age
- Male
- Race (non-Asian)
- Family Hx (genes)
- Environmental
- Trauma
What are environmental risk factors to developing Parkinsons Disease?
- Herbicides, pesticides
- Metals (Mg, Fe) exposure
- Well Water, Farming and Rural Residence
- Wood Pulp Mills
- Steel and Alloy workers
Is smoking a risk or protective of PD?
Protective (possibly)
Describe the pathophysiology of PD
- Loss of dopaminergic cells within the substantial nigra
2. Accumulation of Lewy Bodies and Lewy Neurites (not specific to PD)
Where is the loss of dopaminergic neurons most prominent?
Ventral Lateral Substantia Nigra
What percent of dopaminergic neurons must be lost before motor signs of PD?
60-80%
What are Lewy Bodies?
Eosinophilic, round cytoplasmic inclusions
Where does the substantia nigra project?
Striatum
What is the major function of the striatum?
Regulation of posture and muscle tone
What are the motor features of PD?
Tremor
Rigidity
Akinesia (bradykinesia)
Postural instability
+ stooped posture, shuffling gait, enbloc turning and poor arm swing
What are the features of the tremor associated with PD?
- Asymmetric onset
- Rill rolling or supination/pronation
- Affects all limbs (late)
What are the non-motor features of PD?
- Mood
- Memory
- Autonomics
What are the mood symptoms of PD?
Depression, Anxeity and Hallucinations (late stage, well formed visual hallucinations)
What are the autonomic symptoms of PD?
- Constipation
- Sialhorhea
- Urinary frequency
- Sweating
- Orthostatic Hypotension
What disease may be associated with REM sleep disorder?
PD
What are the medications used to treat PD?
- Sinemet (Levadopa/Carbidopa)
- Dopamine Agonists (Ropiranole)
- MAOB Inhibitors (rasaGELINE ad seleGILINE)
- COMT Inhibitors (entaCapone)
Which Parkinsons medications have been shown to slow the disease progression?
MAO-B Inhibitors (rasaGILINE and seleGILINE)
Which PD drug inhibits dopa-decarboxylase?
Carbidopa
What is the function of dopa-decarboxylase?
Converts L-Dopa to Dopamine
Can Carbidopa cross the BBB?
No
Where does Entacapone work?
In the periphery to prevent the conversion of L-Dopa to 3OM-dopa
Which Parkinsons drug smoothes the on/off phenomena of the disease?
Entacapone
Where do rasagiline and seligiline work?
In the brain the prevent the breakdown of Dopamine
Where is a DBS placed in PD to treat dyskinesia and tremor?
Subthalamic Nucleus
Where is a DBS placed in PD to treat dystonia and rigidity?
Globus Pallidus Externus
Where is a DBS placed in PD to treat just tremor?
VIM
What can be used to treat the postural instability of PD?
PT and Occupational Therapy
What can be used to treat the constipation of PD?
Miralax and herbal remedies
What can be used to treat the orthostatic hypotension seen in PD?
- Fluids
2. Increased salt intake
What can be used for the depression in PD?
SSRIs
What can be used to treat the REM sleep disturbances in PD?
Melatonin or Clonazepam
What can be used to treat the dystonia and pain in PD?
Botulinum toxin
What is used for the dementia in PD?
Donepezil, Memantine and Rivastigmine
What is the triad seen in Normal Pressure Hydrocephalus?
- Abnormal Gait
- Urinary Incontinence
- Dementia
Which feature of NPH is earliest to present?
Abnormal Gait
Which feature of NPH is most responsive to treatment?
Abnormal Gait
Describe the gait in NPH
Bradykinetic, broad-based, magnetic and shuffling gait
How is NPH diagnosed?
CT or MRI; LP with the goal of removing large volumes of CSF
What will be seen on neuroimaging of NPH?
Dilation of the ventricles out of proportion to sulcal atrophy ; will see transependymal flow
What are the treatments for NPH?
- Ventriculoperitoneal or Lumboperitoneal CSF shunting
What are the symptoms of Lewy Body Dementia?
- Visual Hallucinations
- Fluctuations in Cognitive Function
- Parkinsonian motor features preceding cognitive features by less than 1 year
- Anterograde memory loss
- Prominent executive function
Describe the cognitive changes associated with Lewy Body Dementia
- Fluctuations in alertness/attention- Daytime drowsiness
- Staring into space
- Disorganized speech
When is the peak age of incidence for PSP?
6th decade
What are the clinical manifestations of PSP?
- Supranuclear Ophthalmoplegia (downward gaze first)
- Pseudobulbar Palsy
- Prominent Neck Dystonia
- Parkinsonism
- Behavioral and Cognitive disturbances
- Frequent backwards falls
Describe the parkinsonism in PSP
More bradykinesia and less tremor or rigidity symptoms compared to PD
What is the genetics of Huntington’s Disease?
Autosomal Dominant Chromosome 4
What trinucleotide repeat is associated with Huntington’s Disease?
CAG
What genetic feature is associated with HD?
Genetic Anticipation
Describe the Westphall Variant of Huntington’s Disease?
A variant of HD seen in younger children that has symptoms more similar to PD
What are the early symptoms of HD?
- Chorea
- Dystonia
- Motor Impersistance
- Gait Instability
What is the age of onset for HD?
40
What are the later cognate and psychiatric symptoms of HD?
- Depression
- Anxiety
- Apathy
- Impulsivity
- Dementia
How long do patients survive after being diagnosed with HD?
10-15 years
What region of the brain is most affected by HD?
Caudate Nucleus with “box car appearance”
What is the microscopic finding of HD?
Loss of the spiny striatal neurons with gliosis
What are the medical treatments for HD?
- Tetrabenazine (depleted dopamine)
- SSRIs
- Antipsychotics
What are the side effects of Tetrabenazine?
Depression and orthostatic hypotension
What is the most common cause of postural or action tremor?
Essential Tremor
What percent of the population has an essential tremor?
5%
Is Essential tremor symmetric?
No, but usually bilaterally
When is essential tremor most severe?
When arms are outstretched during goal-directed movements
How is Essential Tremor affected by Alcohol?
It improves
What are the 3 components of Essential Tremor?
- Bilaterally action tremor
- No other neuro signs (except cog wheeling)
- May have head tremor with no signs of dystonia
What are the secondary criteria for Essential Tremor?
- > 3 years
- Positive Family History
- Benefit from alcohol
What is the age of onset of Essential Tremor?
Bimodal (2nd Decade and 6th Decade)
Which has a higher correlation with family history, PD or ET?
Essential Tremor
Which type of tremor is usually asymmetric?
Parkinsons Disease
What are the frequencies of PD and ET?
PD- 4-6Hz
ET- 4-10Hz
What is the type of tremor in PD or ET?
PD- supination + pronation
ET- flexion + extension
What are associated features of ET?
Deafness, dystonia and parkinsonism
What is the treatment for ET?
- Propranolol
- Topiramate
- Primidone (Barbituate)
What is the treatment for ET when medical therapy fails?
DBP with electrodes to the VIM of the Thalamus