Progressive supranuclear palsy
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Medpedia on Progressive supranuclear palsy (Less technical, good quality control)
Wikipedia on Progressive supranuclear palsy (Less technical, ? quality control)
Progressive Supranuclear Palsy (PSP) is also known as Steele-Richardson-Olszewski syndrome after the authors of the 1964 description of the condition.[1] Patients fitting its characterstic features are not as rare (1/100000) as originally described. This is no doubt because of misdiagnoses, typically as Parkinson's Disease, although few experienced at making that diagnosis will be caught out, as they will tend to reserve the diagnosis.
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- Postural instability, often more prominent than in others with Parkinsonism. Tremor is rare.
- A poor response to treatment of Parkinsonism
- Features of a subcortical dementia
- Personality changes
- Apathy
- Cantankerous
- Irritability
- Personality changes
- Emotional lability
- Suddenly laughing or crying for no reason
- Spontaneous angry outbursts
- Voluntary gaze palsy
- Care:Paresis of voluntary upwards gaze is common, and more characteristic of generalised white matter damage than PSP.
- Loss of eye contact in conversation
- Dysphagia
- Speech slurring
Aetiology
Unknown
Subtypes
Subtypes may exist:[2]
- Richardson's syndrome (RS)
- 50% of cases
- Early onset of postural instability and falls
- Supranuclear vertical gaze palsy
- Cognitive dysfunction
- 50% of cases
- PSP-parkinsonism (PSP-P)
- 33% have Parkinsonism with:
- Asymmetric onset
- Tremor
- Moderate initial therapeutic response to levodopa
- 33% have Parkinsonism with:
References
- ↑ Steele JC, Richardson JC, Olszewski J. PROGRESSIVE SUPRANUCLEAR PALSY. A HETEROGENEOUS DEGENERATION INVOLVING THE BRAIN STEM, BASAL GANGLIA AND CEREBELLUM WITH VERTICAL GAZE AND PSEUDOBULBAR PALSY, NUCHAL DYSTONIA AND DEMENTIA. Arch Neurol. 1964 Apr;10:333-59.
- ↑ Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. Brain : a journal of neurology 2005;128:1247-58. (Direct link – subscription may be required.)
