Makindo Medical Notes

Related Subjects: Multiple System Atrophy (MSA) | Parkinson Plus syndromes | Parkinsonism | Idiopathic Parkinson disease | Progressive Supranuclear Palsy | Drug Induced Parkinson disease

Consider Progressive Supranuclear Palsy if experiencing Parkinsonism symptoms and early falls. Notable for the "Mona Lisa" stare and unique "Mickey Mouse" and "Hummingbird" MRI findings. Early symptoms may include difficulty looking down, affecting the ability to read or navigate stairs.

About Progressive Supranuclear Palsy

• Characterized by bradykinesia and rigidity, typical of Parkinsonism.
• Classified as one of the frontotemporal dementias.
• First described in 1963 by Steele, Richardson, and Olszewski.

Etiology

• Presence of tau-positive neurofibrillary tangles.
• Tangles are primarily found in the brainstem and basal ganglia.
• Notable neuronal depletion in the substantia nigra.
• Significant involvement of the frontal cortex.

Clinical Presentation

• Typically affects individuals over the age of 50.
• Features symmetrical akinetic-rigid Parkinsonism.
• Marked neck and axial rigidity.
• Frequent unexplained falls, usually backwards due to postural instability.
• Vertical gaze palsy, notably difficulty looking downward.
• Early presence of saccadic slowing.
• "Rocket sign" observed as patients rise or sit down abruptly.
• Experiences dysarthria and dysphagia.
• Preservation of Doll's head eye movement.
• Characteristic "reptilian" stare due to an open mouth and delayed eye signs that can complicate diagnosis.
• Associated with subcortical dementia, featuring more axial rigidity and extensor posture.

Diagnostic Imaging

Investigations

• MRI findings include midbrain atrophy resembling 'Mickey Mouse ears' as the third ventricle enlarges and the interpeduncular fossa is affected. T2-weighted images show enhancement around the periaqueductal area with a sagittal plane resembling a 'hummingbird' appearance. Notable frontal and temporal atrophy.
• Anal sphincter EMG typically shows abnormal results.

Management Strategies

• Levodopa often results in a poor, transient response; consider amantadine instead.
• Antidepressants to manage mood disturbances such as depression and anxiety.
• Botulinum toxin injections for the management of blepharospasm or dystonia.
• PEG tube placement may be necessary for severe dysphagia.
• Typical progression leads to mortality within six years from onset, often due to complications from immobility and pneumonia.

Additional Resources

• Guideline for the Diagnosis and Management of Paget's Disease of Bone in Adults