Progressive Supranuclear Palsy, Corticobasal Degeneration and Multiple System Atrophy

by Carlo Raj, MD

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    Our topic now brings us to progressive supranuclear palsy. Progressive supranuclear palsy characterized by truncal rigidity and disequilibrium. There will be nuchal dystonia. What does dystonia mean? Contraction of muscle that’s taking place for long period of time. Pseudobulbar palsy and abnormal speech, ocular disturbance, and here, it’s vertical gaze palsy, which is extremely important, and mild progressive dementia. The clinical pearl here is going to be vertical gaze palsy. Progressive supranuclear palsy. Its onset is between the fifth and seventh decades of life and studies have shown that males and females could share equal preponderance. The pathology: So what’s happening in progressive supranuclear palsy? Neuronal loss in the globus pallidus. Think about that for one second. Subthalamic nucleus, substantia nigra, colliculi, periaqueductal gray matter and dentate nucleus of the cerebellum. There’s neural loss in many of these structures that we’ve identified here. Neurofibrillary tangles could also be found, but remember neurofibrillary tangles is something that we find in every individual who gets older, elderly, okay? However, if the patient starts showing you signs of Alzheimer’s disease, then the number of neurofibrillary tangle is going to then correlate with the severity of the disease itself. Our topic here is corticobasal type of degeneration. The definition. It’s a disease of the elderly characterized by extrapyramidal rigidity. Asymmetric motor disturbances. In other words, a multifocal type of myoclonus and sensory cortical dysfunction. By cortical dysfunction, you should be thinking about higher order type of activity including apraxia, disorder of language, and then here, alien hand type of phenomenon. All of this is referred to as being your corticobasal type of degeneration. Allow the name to speak to you. The pathology here will be cortical atrophy of motor, premotor, or perhaps even anterior parietal lobes. Hence, we talked about the language disorder that...

    About the Lecture

    The lecture Progressive Supranuclear Palsy, Corticobasal Degeneration and Multiple System Atrophy by Carlo Raj, MD is from the course Movement Disorders. It contains the following chapters:

    • Progressive Supranuclear Palsy
    • Corticobasal Degeneration
    • Multiple System Atrophy

    Included Quiz Questions

    1. Vertical gaze palsy.
    2. Horizontal gaze palsy.
    3. Miosis.
    4. Internuclear ophthalmoplegia.
    5. Ptosis.
    1. Corticobasal Degeneration.
    2. Parkinson's disease.
    3. Multiple Sclerosis.
    4. Stroke.
    5. Alzheimer's Disease.
    1. Shy-Drager Syndrome.
    2. Parkinson's Disease.
    3. Upper motor neuron disease.
    4. Huntington's Disease.
    5. Supranuclear palsy.
    1. Cerebellar degeneration.
    2. Nigrostriatal pathway degeneration.
    3. Cerebral degeneration.
    4. Neurofibrillary tangles.
    5. Upper motor neuron disease.

    Author of lecture Progressive Supranuclear Palsy, Corticobasal Degeneration and Multiple System Atrophy

     Carlo Raj, MD

    Carlo Raj, MD

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