Abstract
Progressive supranuclear palsy (PSP) is a clinical syndrome which includes supranuclear gaze palsy, postural instability, and mild frontal-subcortical dementia. PSP is characterized neuropathologically by midbrain atrophy with accumulation of neurofibrillary tangles and glial tau pathology in a widespread topographic distribution. The most common form has been designated as Richardson’s syndrome (RS) and includes postural instability with falls, gait disorder, a supranuclear vertical gaze palsy, eyelid opening apraxia (see Chap. 17), axial rigidity, and a frontal type of cognitive impairment. Early clinical signs of PSP are often subtle, thereby delaying the diagnosis.
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References
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16 Progressive Supranuclear Palsy.mp4 (MP4 17,174KB)
Clip 1: the patient exhibits limited downward gaze and characteristic retrocollis while walking. Facial expression is absent. Gait is slow with a tendency to fall backwards when attempting to turn. Arm swing is absent bilaterally. Pull testing revealed marked postural instability. (Video contribution from Dr. Kammant Phanthumchinda, Chulalongkorn Center of Excellence on Parkinson’s disease and Related Disorders, Thailand.) Clip 2: another patient shows nearly complete vertical and horizontal supranuclear ophthalmoplegia with relative preservation of horizontal and vertical eye movements during passive head manipulation.
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Bhidayasiri, R., Tarsy, D. (2012). Progressive Supranuclear Palsy. In: Movement Disorders: A Video Atlas. Current Clinical Neurology. Humana, Totowa, NJ. https://doi.org/10.1007/978-1-60327-426-5_16
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DOI: https://doi.org/10.1007/978-1-60327-426-5_16
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