Editing Progressive supranuclear palsy (section)

==Signs and symptoms==
The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.<ref name=":2">{{Cite book|title=Bradley's neurology in clinical practice|vauthors=Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC|publisher=Elsevier Saunders|year=2012|isbn=978-1-4377-0434-1|edition=6th |pages=1778}}</ref><ref name=":0" /><ref name=":3" /> [[Dementia]] symptoms are also initially seen in about one in five cases.<ref name=":1">{{cite journal | vauthors = Finger EC | title = Frontotemporal Dementias | journal = Continuum | volume = 22 | issue = 2 Dementia | pages = 464–89 | date = April 2016 | pmid = 27042904 | pmc = 5390934 | doi = 10.1212/CON.0000000000000300 }}</ref>

Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms. The most common behavioural  symptoms in patients with PSP include [[apathy]], [[disinhibition|a lack of inhibition]], [[anxiety]], and [[dysphoria|a profound state of unease or dissatisfaction]].<ref name=":1" />

Later symptoms and signs can include, but do not necessarily include dementia (typically including loss of inhibition and ability to organize information), [[Dysarthria|slurring of speech]], [[difficulty swallowing]], and difficulty [[Eye movement (sensory)|moving the eyes]], particularly in the vertical direction.  The latter accounts for some of the falls experienced by these patients, as they find it difficult to look up or down.<ref name="NHS">{{cite web |title=Symptoms - Progressive supranuclear palsy |url=https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/symptoms/ |website=NHS |date=14 August 2018 |access-date=19 January 2021}}</ref>

Some of the other signs are poor [[eyelid]] function, [[contracture]] of the [[facial muscles]], a backward tilt of the head with stiffening of the [[Table of muscles of the human body: Neck|neck muscles]], [[Sleep disorder|sleep disruption]], [[urinary incontinence]], and [[constipation]].<ref name="NHS" />  Some patients retain full cognitive function up to the end.<ref name=":7">{{Cite journal |last1=Dunning |first1=Elise E. |last2=Decourt |first2=Boris |last3=Zawia |first3=Nasser H. |last4=Shill |first4=Holly A. |last5=Sabbagh |first5=Marwan N. |date=August 2024 |title=Pharmacotherapies for the Treatment of Progressive Supranuclear Palsy: A Narrative Review |journal=Neurology and Therapy |language=en |volume=13 |issue=4 |pages=975–1013 |doi=10.1007/s40120-024-00614-9 |pmid=38743312 |pmc=11263316 |issn=2193-8253}}</ref>

The visual symptoms are of particular importance in the diagnosis of this disorder.  Patients typically complain of difficulty reading due to the inability to look downwards. The [[ophthalmoparesis]] experienced by these patients mainly concerns voluntary eye movement and the inability to make vertical [[saccade]]s, which is often worse with downward saccades.  Patients tend to have difficulty looking down (a downgaze [[:wikt:palsy|palsy]]) followed by the addition of an upgaze palsy. This vertical gaze [[paresis]] will correct when the examiner passively rolls the patient's head up and down as part of a test for the [[oculocephalic reflex]].  Involuntary eye movement, as elicited by [[Bell's phenomenon]], for instance, may be closer to normal.<ref name=":7" />

On close inspection, eye movements called "square-wave jerks" may be visible when the patient fixes gaze at distance. These are fine movements, that can be mistaken for [[nystagmus]], except that they are saccadic in nature, with no smooth phase. Although healthy individuals also make square-wave jerk movements, PSP patients make slower square-wave jerk movements, with smaller vertical components.<ref name="Ophthalmic Disease 2018">{{cite journal | vauthors = Alexander RG, Macknik SL, Martinez-Conde S | title = Microsaccade Characteristics in Neurological and Ophthalmic Disease | journal = Frontiers in Neurology | volume = 9 | issue = 144 | pages = 144 | date = 2018 | pmid = 29593642 | pmc = 5859063 | doi = 10.3389/fneur.2018.00144 | doi-access = free }}</ref> Assessment of these square-wave jerks and diminished vertical saccades is especially useful for diagnosing progressive supranuclear palsy, because these movements set PSP patients apart from other parkinsonian patients.<ref name="Ophthalmic Disease 2018"/> Difficulties with [[Convergence (eye)|convergence]] (convergence insufficiency), where the eyes come closer together while focusing on something near, like the pages of a book, is typical.  Because the eyes have trouble coming together to focus at short distances, the patient may complain of [[diplopia]] (double vision) when reading.<ref name="NHS"/>

A characteristic facial appearance known as [[procerus sign]], with a wide-eye stare, furrowing of forehead with a frowning expression, and deepening of other facial creases, is also diagnostic of PSP.<ref name="Bradley">{{Cite book|title=Bradley's neurology in clinical practice|edition=7th|vauthors=Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL|publisher=Elsevier|year=2016|isbn=978-0-323-28783-8|volume=2|pages=1439 |oclc=932031625}}</ref>

=== 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.