Open main menu
Home
Random
Recent changes
Special pages
Community portal
Preferences
About Wikipedia
Disclaimers
Incubator escapee wiki
Search
User menu
Talk
Dark mode
Contributions
Create account
Log in
Editing
Progressive supranuclear palsy
(section)
Warning:
You are not logged in. Your IP address will be publicly visible if you make any edits. If you
log in
or
create an account
, your edits will be attributed to your username, along with other benefits.
Anti-spam check. Do
not
fill this in!
=== Signs and Symptoms of PSP-RS Subtype === PSP-RS is characterized by a combination of motor, ocular, cognitive, and speech-related impairments, that typically emerge in early stages of the disease. Symptoms of PSP-RS usually begin after 60 and steadily progress over time.<ref name=":0" /> Clinical symptoms of PSP-RS often include unexplained falls, unsteady gait, bradykinesia, apathy, disinhibition, cognitive dysfunction, difficulty planning or multitasking, slow speech, and impaired ocular movement.<ref name=":0" /><ref name=":3" /><ref name=":4">{{Cite journal |last1=Krzosek |first1=Patrycja |last2=Madetko |first2=Natalia |last3=Migda |first3=Anna |last4=Migda |first4=Bartosz |last5=Jaguś |first5=Dominika |last6=Alster |first6=Piotr |date=2022-02-09 |title=Differential Diagnosis of Rare Subtypes of Progressive Supranuclear Palsy and PSP-Like Syndromes—Infrequent Manifestations of the Most Common Form of Atypical Parkinsonism |journal=Frontiers in Aging Neuroscience |volume=14 |doi=10.3389/fnagi.2022.804385 |doi-access=free |pmid=35221993 |pmc=8864174 |issn=1663-4365}}</ref> PSP-RS is also characterized by unresponsiveness to dopamine therapies often prescribed for those with [[Parkinson's disease]].<ref name=":5">{{Cite journal |last=Armstrong |first=Melissa J. |date=March 2018 |title=Progressive Supranuclear Palsy: an Update |url=http://link.springer.com/10.1007/s11910-018-0819-5 |journal=Current Neurology and Neuroscience Reports |language=en |volume=18 |issue=3 |page=12 |doi=10.1007/s11910-018-0819-5 |pmid=29455271 |issn=1528-4042|url-access=subscription }}</ref> Patients can present initially with symptoms more characteristic of the PSP-Parkinson (PSP-P) subtype which is characterized by asymmetric rigidity, resting tremor, and are more responsive to dopamine therapies such as [[Levodopa|levadopa]] compared to PSP-RS.<ref>{{Cite journal |last1=Coughlin |first1=David G. |last2=Litvan |first2=Irene |date=April 2020 |title=Progressive supranuclear palsy: Advances in diagnosis and management |journal=Parkinsonism & Related Disorders |language=en |volume=73 |pages=105–116 |doi=10.1016/j.parkreldis.2020.04.014|pmid=32487421 |pmc=7462164 }}</ref> Clinical and pathological differences between PSP-P and PRSP-RS occur within the first year of the disease, with individuals with PSP-RS exhibiting faster progression of symptoms and lower survival rates after diagnosis. Diagnostic criteria distinguish between '''probable''' and '''possible''' PSP-RS, as definitive diagnosis requires post-mortem neuropathological confirmation.<ref name=":5" /> The NINDS-SPSP criteria define probable PSP-RS as requiring both vertical supranuclear gaze palsy and early postural instability with falls, while possible PSP-RS requires either vertical gaze palsy or slowed vertical saccades combined with early falls.<ref name=":5" /><ref name=":4" /> PSP-RS effects more Males than Females, with a Male to Female ratio of 1.8:1.<ref name=":3" /> Symptom progression tends to occur more quickly in PSP-RS than other sub-types with the average disease duration being 5.9 years and an average age of death of 72.1 years.<ref name=":3" /><ref name=":7" /> ==== Motor Symptoms in PSP-RS ==== One of the key features of PSP-RS that occurs within the first year of symptom onset, is early postural instability which often leads to unexplained falls.<ref name=":6" /><ref name=":5" /><ref name=":8">{{Cite journal |last1=Alster |first1=Piotr |last2=Madetko |first2=Natalia |last3=Koziorowski |first3=Dariusz |last4=Friedman |first4=Andrzej |date=2020-03-10 |title=Progressive Supranuclear Palsy—Parkinsonism Predominant (PSP-P)—A Clinical Challenge at the Boundaries of PSP and Parkinson's Disease (PD) |journal=Frontiers in Neurology |volume=11 |page=180 |doi=10.3389/fneur.2020.00180 |doi-access=free |pmid=32218768 |pmc=7078665 |issn=1664-2295}}</ref> Additionally, patients present with axial rigidity, which is characterized by stiffness in the neck and body.<ref name=":5" /> This is often accompanied with [[bradykinesia]] which is slowness of movement. Although PSP-RS is often misdiagnosed as Parkinson's disease, tremors are uncommon in PSP-RS.<ref name=":3" /> Motor symptoms are often symmetrical in PSP-RS with both sides of the body being affected.<ref name=":10">{{Cite journal |last1=Chunowski |first1=Patryk |last2=Madetko-Alster |first2=Natalia |last3=Alster |first3=Piotr |date=2024-09-28 |title=Asymmetry in Atypical Parkinsonian Syndromes-A Review |journal=Journal of Clinical Medicine |volume=13 |issue=19 |pages=5798 |doi=10.3390/jcm13195798 |issn=2077-0383 |pmc=11477316 |pmid=39407856 |doi-access=free}}</ref> ===== Ocular Symptoms in PSP-RS ===== A defining feature of PSP-RS is vertical supranuclear gaze palsy, which is difficulty with voluntary downward gaze.<ref name=":9">{{Cite journal |last1=Krzosek |first1=Patrycja |last2=Madetko |first2=Natalia |last3=Migda |first3=Anna |last4=Migda |first4=Bartosz |last5=Jaguś |first5=Dominika |last6=Alster |first6=Piotr |date=2022-02-09 |title=Differential Diagnosis of Rare Subtypes of Progressive Supranuclear Palsy and PSP-Like Syndromes—Infrequent Manifestations of the Most Common Form of Atypical Parkinsonism |journal=Frontiers in Aging Neuroscience |volume=14 |doi=10.3389/fnagi.2022.804385 |doi-access=free |pmid=35221993 |pmc=8864174 |issn=1663-4365}}</ref> Vertical supranuclear gaze palsy, a symptom characterized by decreased velocity and amplitude of vertical eye movements (saccades) is often the prominent diagnostic feature of PSP-RS. Approximately 40% of patients with PSP-RS experiencing supranuclear gaze palsy, but it may not present until 3–4 years after disease onset.<ref name=":0" /><ref name=":9" /> Individuals with PSP-RS also display other ocular motor symptoms such as dry, red and sore eyes, blurred vision, and difficulty focusing.<ref name=":7" /> They may also experience spontaneuous and involuntary eye-lid closure or apraxia of the eyelid opening.<ref name=":7" /> ===== Cognitive and Behavioral Symptoms in PSP-RS ===== Cognitive changes are frequent in PSP-RS compared to other PSP subtypes and include slowed thinking (bradyphrenia), executive dysfunction, and difficulty with planning or problem-solving.<ref name=":5" /><ref name=":8" /> Some patients exhibit apathy, emotional blunting, or involuntary episodes of laughing or crying unrelated to mood.<ref name=":7" /> Some studies report that around half the individuals with PSP-RS develop these personality changes within 2-years of diagnosis.<ref name=":7" /> Dementia is not typically a dominant feature early on but may develop in later stages.
Edit summary
(Briefly describe your changes)
By publishing changes, you agree to the
Terms of Use
, and you irrevocably agree to release your contribution under the
CC BY-SA 4.0 License
and the
GFDL
. You agree that a hyperlink or URL is sufficient attribution under the Creative Commons license.
Cancel
Editing help
(opens in new window)