Multiple System Atrophy (MSA)
|Parkinson Plus syndromes
|Idiopathic Parkinson disease
|Progressive Supranuclear Palsy
|Drug Induced Parkinson disease
Consider if Parkinsonism and early falls. Mona lisa stare ! Mickey Mouse and Hummingbird MRI findings
- There may be bradykinesia and rigidity - Parkinsonism
- One of the frontotemporal dementias
- Described in 1963 by Steele Richardson and Olszewski.
- Tau positive neurofibrillary tangles
- Tangles found in brainstem and basal ganglia
- Neuronal depletion of substantia nigra.
- The frontal cortex also involved
- Seen in those over age > 50
- Symmetrical akinetic-rigid Parkinsonism.
- Marked Neck and axial rigidity.
- Postural instability. Unexplained falls usually backwards
- Vertical gaze supranuclear palsy - difficulty looking down
- May also have saccadic slowing seen early
- Rocket sign as patients get up or sit down suddenly
- Dysarthria and dysphagia
- Preserved Doll's head movement of eyes.
- Eye signs appear late and can delay the diagnosis.
- Open mouth patient has what is called a "reptilian" stare.
- Associated subcortical dementia.
- The posture is more axial rigidity and extensor
- MRI: midbrain atrophy 'Mickey mouse ears' as IIrd ventricle enlarges and interpeduncular fossa. T2W shows enhancement around the periaqueductal area. Sagittal plane - humming bird-like appearance. Frontal and temporal atrophy
- Anal sphincter EMG: abnormal
Humming bird appearance of midbrain
- Classically there is a poor transient response to Levodopa. Consider amantadine.
- PEG tube may be indicated with severe dysphagia
- Death occurring at a median of six years after onset due to immobility and pneumonia