Movement Disorders Flashcards
What is the most common movement disorder?
Which sex does it more frequently affect?
- Parkinson’s Disease
- More common in men
- 1-2% general population 65+ (most cases start bw 50-70 yoa)
Note it is the #2 neurodegenerative disorder
PD is less common in which ethnicities (3):
- Asian
- African American
What are 5 genes associated with parkinson’s disease?
- LRRK2
- PARK2
- PARK 7
- PINK1
- SNCA** also associated with hyperkalemic epilepsy
List 6 risk factors and one protective factor associated with parkinson’s disease:
Risk
- Age
- Male sex
- Family hx
- Herbicide, manganese, Fe, rural living, well water, welders by trade
- Trauma
Smoking may be protective…
What are the motor sx of parkinson’s disease? (4)
TRAP
- Tremor (asymmetric onset), pill rolling, supination/pronation
- Rigidity
- Akinesia/bradykinesia
- Postural disturbance (festinating gait)
What are the non-motor sx of parkinson’s disease? (5)
MMA
Mood, memory, autonomic dysfunction
- Cognitive (LB dementia)
- Psychiatric (Depression, anxiety, hallucinations)
- REM sleep bx disorder
- ANS dysfunction (constipaiton, urinary frequency, sweating, orthostatic HypoTN)
- Pain
Describe the pathophysiology go Parkinson’s Disease (2):
- Loss of pigmented dopaminergic neurons in ventrolateral substantial nigra (60-80% loss = sx)
- Lewy bodies and Lewy neurites
Meds to treat parkinsons? (6)
- Carbadopa/ Levodopa
- DA Agonists
(Ropiranole, pramipexole) - MAOBi’s
(Rasagelinie, selegiline) - Amantadine
- COMTi’s
(Entacapone) - Stalevo
(Sinemet + Entacapone)
Which drugs are the only ones proven to slow the course of PD?
Which drug is used to smooth out dyskinesia associated with DA agonist and Sinemet?
Which drug helps smooth on/off sx of sine met dosing?
- MAOBi’s are only ones shown to slow course of PD
- Amantadine helps smooth out dyskinesia caused by other meds
- Entacapone and other COMTi’s help with smoothing out on/off sx
When does MS typically present?
Typically females between 20-40 yoa
What are some risk factors predisposing patients to MS? (3)
- Northern climate (low Vit D?)
- Female sex
- First degree relative with MS (^ risk 20-40x)
Where is the DBS electrode placed to treat the following disorders:
- Dyskinesia and tremor
- Dystonia
Dyskinesia and tremor: sub thalamic nucleus
Dystonia: Gpe
Normal Pressure Hydrocephalus:
What is the classic triad and which feature appears first?
1) Magnetic gait
2) Urinary incontinence (^ frequency, urgency)
3) Dementia (Bradyhrenia, memory loss, forgetfulness, inattention)
How do we diagnose NPH? (2)
1) CT or MRI (transependymal flow)
2) LP with goal to remove lots of CSF **walking speed over 10 steps and MOCA will both improve with large volume drainage
How do we treat NPH?
Ventriculoperitoneal or lumboperitoneal CSF shunting
What are the features of Lewy Body Dementia? (4)
- Well formed visual hallucinations (non-persecutory)
- Parkinsonian motor features preceding Dementia by 1 year
- Prominent executive and visuospatial impairment
**Anterograde memory loss (less prominent than Alzheimers)
What are the clinical features of Progressive Supranuclear Palsy? (6)
- Supranuclear ophthalmoplegia (downward–> upward)
- Pseudobulbar palsy
- Neck dystonia
- Parkinsonism
- Gait disturbance w/ BACKWARDS falls + impaired postural reflexes
- Bx, cognitive disturbance
What are the early manifestations of huntington’s disease (4)
What is the typical age of onset?
- Chorea
- Dystonia
- Motor impersistance
- Gait instability
Typical onset in 40’s
What are the later manifestations of Huntington’s disease?
How long do patients with HD typically live?
Cognitive and Psych:
- Depression
- Anxiety
- Apathy
- Impulsivity
- Dementia
Patients typically live for 10-15 yrs
Describe the macro and microscopic pathological features of a brain w HD:
- Atrophy of caudate nucleus
- Loss of spiny stratal neurons w gliosis
How does HD present in a pediatric patient?
“Westfall Variant” presents like PD in child
What do we use to treat choreaform movements in HD patients? What is its MOA? What are 2 ADRs?
Tetrabenzine (Xenaxine):
Profound DA depleting agent; asstd with depression and orthostatic HypoTN
What do we use to treat depression and psychosis in HD patients?
Depression: #1 SSRI
Psychosis: Antipsychotics
What is the most common neurologic case of postural or action tremor?
Benign essential tremor
Describe the tremor of Benign ET: (3)
- Symmetric flexion-extension tremor commonly at wrist; increases w goal directed movement
- Foreign implicates voice, head and neck
- Internal tremors are common
Describe how a patient with benign ET will typically present:
- How long have sx persisted?
- What makes it better?
- What is evident in history?
Sx must persist more than 3 yrs; better w ETOH; family hx of tremor
Potential treatments for benign essential tremor (4):
What is one non-medicinal treatment?
- Beta blocker (propranolol)
- Primidone (sedative, decreases cognition)
- Topirimate
- BDZs
- DBS in the face of drug failure (apply to the VIM nucleus of the thalamus)
Three sx associated with benign essential tremor?
- Deafness
- Dystonia
- Parkinsonism
Unilateral tremor, leg tremor, rigidity, bradykinesia, resting tremor…
PD
Gait disturbance (2 pathologies)
PD
Cerebellar tremor
Focal tremor pathology…
dystonic tremor
Isolated head tremor with abnormal posture (head tilt or turning)
dystonic tremor
Sudden rapid onset tremor
Psychogenic or toxic
Current drug treatment that may cause or exacerbate tremor
Drug-induced or toxic tremor
