Additional Basal Ganglia Pathologies Flashcards
What is the pathophysiology involved in Vascular Parkinsonism?
- Clinical features of PD that are caused by multiple small CVAs
What are the typical signs and symptoms of Vascular Parkinsonism?
Symmetrical lower-body parkinsonism
- Gait unsteadiness - +freezing, festinating
- Bradykinesia, akinesia, hypokinesia
- Absence of tremors
- Rigidity
- Pyramidal signs (weakness, spasticity, hyperreflexia, slow RAM)
How do we manage Vascular Parkinsonism and how is its prognosis?
Management
- CVA treatment – treat the cause
Prognosis
- Fair – multifocal strokes have the worst prognosis of strokes
- High morbidity
What is the average age of onset of Progressive Supranuclear Palsy (PSP)?
60s
Explain the pathophysiology behind PSP.
- Accumulation of tau protein that leads to neuronal death and astrogliosis
o Astrogliosis – abnormal accumulation of astrocytes (leads to neuronal death)
How is PSP diagnosed? What specific MRI findings are involved?
- MRI
o Frontal lobe subthalamic, and putamen atrophy
o Morning Glory sign (flower) – abnormal concavity in basis and tegmentum of midbrain
o Hummingbird sign – flattening of the brainstem
What is PSP commonly misdiagnosed as?
- Depression
- Dementia
- PD
What are the signs & symptoms associated with PSP
- Gait disturbances
- Loss of righting reactions – fall backwards suddenly
- Motor impulsivity – rocket sign
- Severe axial rigidity – extensors > flexors
- Supranuclear opthalmoplegia
- Dysphagia
- Dysarthria
- Frontal cognitive dysfunction
- Pseudobulbar affect
- Sleep disturbances
- Fixed Mona Lisa Stare (surprised expression)
Which direction do PSP patients typically fall? Why?
- fall backwards suddenly due to loss of righting reaction
- due to rocket sign (shoot right up) and axial rigidity that typically affects the extensors > flexors
What type of eye movement dysfunction do PSP patients have?
supranuclear opthalmopegia
- limited vertical eye movement
- loss of convergence
- impaired vertical saccades
- loss of visual acuity
- loss of eyelid control
How is PSP managed?
- No effective treatment
- Anti-PD meds – minimal and short lasting
How is the prognosis for PSP?
- Severe disability in 3-5 years of onset
- Mortality commonly seen 5-8 years
- Serious complications are common – Pneumonia, falls with injuries
What is the hallmark for multiple system atrophy? Why?
marked autonomic dysfunction
- thoracic and sacral spine involved which leads to autonomic dysfunction
What is the average age of onset of Multiple Systems Atrophy (MSA)?
- Early 50s
- Rarely diagnosed past 70 years
Explain the pathophysiology behind MSA.
- Unknown cause
- Proteins in glial cells that affect oligodendrocytes who create and maintain myelin in CNS
What are the two subtypes of MSA and how do they differ?
- MSA-P – resembles typical PD
- MSA-C – resembles cerebellar issues
How is MSA diagnosed? What specific MRI findings are involved?
- Clinical exam
- PET scan
- MRI – hot cross buns sign (atrophy in pons)
What is MSA commonly misdiagnosed as?
- PD but often corrected once patients do not respond to dopamine therapy
What are the signs & symptoms associated with MSA?
- Autonomic dysfunction
- Bradykinesia, rigidity, tremors
- Gait and limb ataxia
- Head/oral dyskinesias and dystonias – coat hanger pain
- Antecollis – looking down posture
- Pisa syndrome
- Oculomotor dusturbances
- Speech deficits
- Sleep disorders
- Cognitive deficits are RARE
How is MSA managed?
- No effective treatment
- Anti-PD meds effective for 1/3 of patients – can worsen OH
- Medications for symptoms
How is the prognosis for MSA?
- 50% need AD after 3 years
- 60% become wheelchair dependent in 5 years
- Mortality ranges from 5-10 years after onset
What predicts slower disease progression of MSA?
cerebellar phenotype and later onset of autonomic symptoms