PSP presents gradually and is insidious. The age of presentation is usually between 55-70 years. Typical symptoms may include the following key features

• The early symptoms of PSP may be dysphagia and dysarthria.
• Progressive difficulty with walking (gait) and balance resulting in frequent falls. Postural instability is a key feature of the disorder. Patients often have slow unsteady gait. The bradykinesia is usually symmetrical and the rigidity is axial. Both these symptoms fail to respond to levodopa. Motor exam will reveal difficulty that often involves the axial rather than limb muscles. In the axial musculature, the neck muscles are more affected than trunk muscles. Motor symptoms are slow and upper body appears stiff and rigid. Rigidity with abnormal posturing of the neck (retrocollis).
• Visual symptoms are obvious and characteristic for PSP. There is progressive loss of voluntary control of eye movements. Earlier findings are slowing of vertical saccades, blepharospasm, vertical gaze palsy, difficulty with eye-opening and closing, reduced blinking, constantly raised eyebrows facial expression and a fixed stare. The gaze abnormalities can make it difficult to have eye contact and scan or read a page. Other ocular symptoms that also tend to occur in the early phase include blurred vision, diplopia, light sensitivity and burning eyes.
• Difficulty eating because of inability to look down at food.
• Conversations are impossible because of delay in responses and pronunciations.
• Eventually patients are unable to swallow and even eating, motions become uncoordinated.
• Aspiration pneumonia is common due to difficulty swallowing, coughing and drooling.
• Cognitive and behaviour problems due to frontal lobe involvement. There is progressive decline in concentration, concrete thinking, difficulty planning, impaired reasoning and shifting to another task.
• Behaviours changes include lack of motivation, apathy, withdrawal, perseveration and impulsivity. Depression is common.
• Unlike Parkinson disease, patients with PSP can experience falls early in disease. This is due to stiff upper body posture and tendency to fall backwards.
• Dysarthria (slow or slurred speech) is an early feature and may be mixed with a combination of hypokinetic, spastic and ataxic features. Pyramidal signs occur in about 30% of patients
• Because of the development of frontal lobe dysfunction, the occasional individual with PSP may suddenly jump on his or her feet from a sitting position without thinking only to fall backwards because of postural instability. This is known as the “rocket sign”.[8]

Differential diagnosis of PSP:

• Alzheimer's disease
• Corticobasal degeneration
• Multiple system atrophy
• Parkinson's disease
• Pick's disease
• Postencephalitic parkinsonism

Differentiating PSP from Parkinson disease

• Sometimes it can be very difficult to differentiate PSP from Parkinson disease during the early stages of the disease. Some PSP patients may not develop postural instability and ophthalmoplegia early in the course of the disease.
• In general, features of PD are asymmetric whereas this is not so with PSP. In addition, treatment with L-dopa will only have a limited response in patients with PSP.
• Dysphagia occurs much early in PSP whereas in PD it is usually seen in advanced cases and is delayed.
• The progression of PSP is much faster clinically
• PD patients generally do not have dementia, which is a common feature of PSP

• Falling

  Of 198 clinical parameters, we hypothesized 38 to be correlated with an increasing risk of falls. These 38 parameters were analyzed via univariate regression analysis to determine the strength of their association with fall frequency. [ncbi.nlm.nih.gov]

  The falls of PSP are often called “unexplained falls” because the person seems to fall out of the blue, for no obvious reason. [brainsupportnetwork.org]

  Progressive Supranuclear Palsy (PSP) PSP average age of onset is around 60 years with an average duration of 6 years PSP features: • Rigidity • Slowness of movement • Early falls or tendency to fall backwards • Unsteady gait • Vertical supranuclear gaze [ftlda.org]

• Malaise

  The majority of diseases known to man often list fatigue or malaise as possible associated symptoms. [emedicinehealth.com]

• Dysphagia

  Dysphagia is a well-recognized complication of PSP [ 3 ], occurring in up to 80% of patients [ 4 ]. Both the time to development of dysphagia and that from dysphagia to death are shorter in PSP than in Parkinson disease (PD) [ 5 ]. [doi.org]

  CONCLUSIONS: This is the first study comparing clinical profile of dysphagia in patients with PD, MSA, and PSP. [ncbi.nlm.nih.gov]

  Some may progress to anarthria. 46% are affected by dysphagia with by 5 years. Sleep disturbance is common. Autonomic failure is less common than in Parkinson's disease with the exception of incontinence. [cmdg.org]

• Choking

  Minimally invasive surgery to place a feeding tube into the stomach may be necessary if choking becomes a hazard. [houstonmethodist.org]

  As PSP progresses, patients are at greater risk for complications, such as choking, pneumonia, head injury and fractures caused by falls. The most common cause of death is pneumonia. [ucsfhealth.org]

  Problems swallowing, which can lead to choking or inhaling food or liquid into your airway (aspiration). Aspiration can develop into pneumonia, the most common cause of death in people with progressive supranuclear palsy. [mayoclinic.org]

  Dysphagia Difficulty swallowing, including gagging or choking. This can lead to aspiration pneumonia. Dysarthria Slurred or slowed speech due to difficulty moving the muscles controlling the lips, tongue and jaw. [theaftd.org]

  Complications that result from worsening symptoms, such as pneumonia (from breathing in food particles while choking during eating), can be life-threatening. [stanfordhealthcare.org]

• Gagging

  Dysphagia Difficulty swallowing, including gagging or choking. This can lead to aspiration pneumonia. Dysarthria Slurred or slowed speech due to difficulty moving the muscles controlling the lips, tongue and jaw. [theaftd.org]

  He then developed a severe pseudo‐bulbar palsy, with dysarthria, dysphagia, brisk palmo‐mental reflex, jaw jerks and an absent gag reflex. [doi.org]

• Vertical Gaze Palsy

  Supranuclear vertical gaze palsy, gait instability and the absence of delusions distinguished PSP from diffuse Lewy body disease. Supranuclear vertical gaze palsy and increased age at symptom-onset distinguished PSP from MSA. [doi.org]

• Blepharospasm

  Blepharospasm is a form of dystonia affecting the muscles around the eyes. [rarediseases.org]

  Additional ocular findings include photophobia, blepharospasm, and eyelid opening apraxia ( 2 ). [webeye.ophth.uiowa.edu]

  Botulinum toxin A may be useful in the treatment of blepharospasm associated with PSP-RS. Physical therapy has been found to be helpful. [patient.info]

  Associated ocular findings are horizontal square-wave jerks, slow and hy pometric saccades, apraxia of eyelid opening, blepharospasm, or a severely reduced blink rate and a progressive paresis of gaze in all directions. [cmdg.org]

  Earlier findings are slowing of vertical saccades, blepharospasm, vertical gaze palsy, difficulty with eye-opening and closing, reduced blinking, constantly raised eyebrows facial expression and a fixed stare. [symptoma.com]

• Diplopia

  We report the case of a 75-year-old ex-professional boxer who developed diplopia and eye movement abnormalities in his 60's followed by memory impairment, low mood and recurrent falls. [ncbi.nlm.nih.gov]

  Common symptoms at disease onset include postural instability and falls, dysarthria, bradykinesia and visual disturbances such as diplopia, blurred vision, burning eyes, and light sensitivity. [patient.info]

  Because the eyes have trouble coming together to focus at short distances, the patient may complain of diplopia (double vision) when reading. [en.wikipedia.org]

  It can be characterized by parkinsonism, ataxia, diplopia, horizontal gaze palsy, sluggish eye movements, and occasionally optic atrophy. OPCA does not respond well to L-dopa. [web.archive.org]

  Other ocular symptoms that also tend to occur in the early phase include blurred vision, diplopia, light sensitivity and burning eyes. Difficulty eating because of inability to look down at food. [symptoma.com]

• Abnormal Eye Movement

  Progressive supranuclear palsy is characterized by decreased cognition, abnormal eye movements (supranuclear vertical gaze palsy), postural instability and falls, as well as parkinsonian features and speech disturbances 1-3. [radiopaedia.org]

  Patients usually do not complain of abnormal eye movements, although they may notice blurring of vision and problems reading. As the disease progresses, horizontal eye movements may also become affected. [bcm.edu]

  Speech and swallowing difficulties are more common and severe with PSP and the abnormal eye movements of PSP are essentially absent with PD. A poor response to levodopa along with symmetrical onset can also help differentiate PSP from PD. [en.wikipedia.org]

  Abnormal eye movements develop several years after other movement dysfunctions and often present as an inability to move the eyes up and down. [physio-pedia.com]

  August 29, 2012 A case of an 81-year-old woman with limited eye movements Chief complaint : Abnormal eye movements History of present illness The patient was accompanied by her husband, who provided a substantial portion of the history. [webeye.ophth.uiowa.edu]

• Strabismus

  Amblyopia and Strabismus Most studies of fixational eye movements in ophthalmic disease to date have centered on amblyopia and strabismus. [doi.org]

  2016 2017 2018 2019 Non-Billable/Non-Specific Code Type 2 Excludes internal ophthalmoplegia ( H52.51- ) internuclear ophthalmoplegia ( H51.2- ) progressive supranuclear ophthalmoplegia ( G23.1 ) Ophthalmoplegia - see also Strabismus, paralytic supranuclear [icd10data.com]

• Behavior Disorder

  INTRODUCTION: Beside motor symptoms, patients with progressive supranuclear palsy syndrome (PSPs) commonly present cognitive and behavioral disorders. In this study we aimed to assess the structural brain correlates of cognitive impairment in PSPs. [ncbi.nlm.nih.gov]

  In REM sleep behavior disorder, patients talk and move during dream sleep, and the movement can result in personal injury or injury to a bed partner. [rarediseases.org]

  Patients can also develop REM sleep behavior disorder consisting of abnormal motor activity with vivid dreams during REM sleep. [encyclopedia.com]

  Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: a prospective study. Parkinsonism Relat Disord. 2009 Jan. 15(1):59-61. [Medline]. [emedicine.medscape.com]

• Postural Instability

  Postural instability and supranuclear gaze palsy represent the key symptoms of Richardson's syndrome, the most frequent clinical manifestation of progressive supranuclear palsy (PSP). [ncbi.nlm.nih.gov]

  Supranuclear vertical gaze palsy, moderate or severe postural instability and falls during the first year after onset of symptoms classified the sample with 9% error using logistic regression analysis. [doi.org]

• Bradykinesia

  UPDRS total motor scores and subscores for bradykinesia and axial motor deficits did not correlate with R2* values of the five brain regions. [ncbi.nlm.nih.gov]

  PSP-corticobasal syndrome (PSP-CBS) is characterized by progressive, asymmetric dyspraxia, limb rigidity, bradykinesia and progressive postural instability. [orpha.net]

  Bradykinesia was present in 98% of cases, rigidity in 98% and falls in 94%. [doi.org]

• Dystonia

  In this mini-review, I reviewed effects of zolpidem as movement disorders including Parkinsons disease, progressive supranuclear palsy, dystonia and so on. In addition, I reviewed a possible mechanism of zolpidem for movement disorders. [doi.org]

  (xv) Extra axial-dystonia: the presence of dystonia in any body part apart from trunk and neck. (xvi) Pyramidal signs: pathologically brisk reflexes and/or extensor plantar response(s). [dx.doi.org]

  G24.01 Drug induced subacute dyskinesia G24.02 Drug induced acute dystonia G24.09 Other drug induced dystonia G24.1 Genetic torsion dystonia G24.2 Idiopathic nonfamilial dystonia Reimbursement claims with a date of service on or after October 1, 2015 [icd10data.com]

  CONSUMERS: Click here for the Consumer Version Progressive supranuclear palsy is a rare, degenerative CNS disorder that progressively impairs voluntary eye movements and causes bradykinesia, muscular rigidity with progressive axial dystonia, pseudobulbar [msdmanuals.com]

• Akinesia

  In subgroup analysis of PSP, volume loss in the whole and segmental cerebellum was more pronounced in patients with PSP-RS than in those with pure akinesia with gait freezing, PD or control subjects. [ncbi.nlm.nih.gov]

  PSP-Pure akinesia with gait freezing (PSP-PAGF) is characterized by progressive freezing of gait, speech and writing early in the course of the disease. [orpha.net]

• Apraxia

  Five presenting clinical syndromes of PSP are well-described: (i) the classic Richardson's syndrome; (ii) asymmetric parkinsonism with tremor; (iii) freezing of gait; (iv) asymmetric limb apraxia, and (v) apraxia of speech. [ncbi.nlm.nih.gov]

  Additional ocular findings include photophobia, blepharospasm, and eyelid opening apraxia ( 2 ). [webeye.ophth.uiowa.edu]

  In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP. [oadoi.org]

If PSP is suspected the following investigations are necessary:

• Electro oculographic recordings may be useful in differentiating PSP from other related disorders. Individuals with PSP have horizontal hypometric voluntary saccades while individuals with corticobasal degeneration have the opposite findings. Parkinsonian patients tend to have saccades of normal amplitude and latency.
• MRI will show thinning of the quadrigeminal plate and atrophy of the midbrain and area around the 3rd ventricle, supporting a diagnosis of PSP.
• MRI can also help exclude other disorders such as hydrocephalus, multi infarct dementia, and tumors.
• Other research tools used to support early diagnosis of PSP include polysommnography, evoked potentials and PET scan.

Diagnostic criteria
Criteria have been established to help improve diagnostic accuracy of PSP and include the following [9]:

• Age onset at age 40 or later
• Gradual progression
• Vertical supranuclear palsy and slowing of vertical saccades
• Prominent postural instability with falls in the first year

Supportive features to help diagnosis include:

• Symmetric akinesia or rigidity
• Proximal muscle involvement more than distal
• Retrocollis
• Poor or absent response to levodopa therapy
• Early dysarthria or dysphagia
• Early cognitive impairment including at least 2 of the following features: impaired abstract thought, apathy, decreased verbal fluency, frontal release signs and imitation behaviour.

• Neurofibrillary Tangle

  PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. [ncbi.nlm.nih.gov]

A Neurologist consultation is vital when evaluating a patient with PSP. The disorder is not easy to diagnose and can often be mistaken for Parkinson disease. The history should be obtained from the family or primary caregiver as it may reveal changes in behavior, daily functional activity and mobility.
So far there is no effective treatment of PSP [10].  All treatments are supportive. Even though dopaminergic agents have been tried, they only work in mild cases and the symptom relief is short-lived. Only a few patients see benefit from these agents. Even though there is significant loss of cholinergic neurons in the brain of PSP patients, administration of cholinergic agents like physostigmine has not been helpful.
Other anecdotal reports indicate slight improvement after treatment with methysergide, trazodone, amitriptyline and idazoxan. However, the benefits are often short-lived and inconsistent.
Botulinum toxin has been used to treat levator inhibition and blepharospasm but the success rates are low. To prevent exposure keratopathy, artificial tears are recommended. In patients who have depression, have emotional apathy or pseudo bulbar crying episodes, antidepressants may help.
Supportive therapies include use of ambulatory devices like a wheelchair, communication aids and walker. Refer to occupation and physical therapies to help with posture and daily living activities. Speech therapist may help some patients with difficulty in swallowing.

PSP is progressive disorder without a cure. Eventually all patients become wheelchair bound and many require a feeding tube. The median survival time from symptom onset is about 5 years. Individuals who fall during the first year, have early dysphagia and incontinence with negative prognostic signs. In most cases aspiration pneumonia leads to death. The quality of life is very poor and the disorder can create stress in the family or caregiver.

The cause of PSP is not known and it is thought to be a sporadic disorder. Very few familial cases have been reported but the mode of inheritance is not well understood. It is believed that the disorder has an autosomal dominant inheritance with reduced penetrance [5].
The role of viruses and toxins as a cause is pure speculative since there is no study that has even shown such a link. Currently there are studies indicating that perhaps genes leading to formation of neurofibrillary tangles may play an important role [6][7].

PSP tends to occur after the 5th decade of life. Based on small series, the annual incidence of PSP is about 2- 5.3 new cases per 100,000 people every year. It is believed that these numbers are underestimated because many healthcare workers either have not heard about the disorder or fail to make the diagnosis. It is more likely that most of the patients are not diagnosed from the disorder and probably die from an unrelated disorder that is present at the same time. Review of the UK Parkinson Disease Society Brain Bank revealed that nearly 6% of patients diagnosed with Parkinson disease were found to have PSP at autopsy. The incidence of PSP is known to increase with age and overall the disorder affects more males than females.

Unlike Alzheimer dementia, PSP is not associated with amyloid plaques in the brain but is associated with abnormal accumulation of tau protein. Some experts have classified PSP as a Pick complex disorder. The major neurotransmitter subsystems involved in PSP are the GABAergic, dopaminergic nigrostriatal pathway, and cholinoceptive striatal neurons including the cholinergic brainstem and basal forebrain nuclei.

At autopsy the chief macroscopic abnormality seen in the brain is the significant loss of pigment in the substantia nigra and locus coerulus which are often shrunken and discolored. There is also significant degeneration of cholinergic nucleic and loss of choline acetyltransferase activity in many parts of the brain.

Degeneration may be seen in the subthalamus, substantia nigra and pallidum. There is also widespread distribution of neurofibrillary tangles in the hippocampus, subthalamus, putamen caudate, frontal cortex, red nucleus, dentate and inferior olive regions. The National Institute of Neurological Disorders and Stroke (NINDS) criteria for PSP requires high density of neurofibrillary tangles and neuropil threads in at least three of the following anatomical sites: subthalamic nuclei, pallidum, pons or substantia nigra or pons, mild to moderate density in at least three of the following sites: medulla, striatum, dentate nucleus and oculomotor complex.

Because the cause of PSP is not known prevention is not possible. However, it is important that healthcare providers understand the clinical presentation of PSP so that an early diagnosis can be made. The  disorder is very disabling and of enormous stress to the caregiver, so an early diagnosis can mean prompt referral to physical, as well as speech and occupational therapy [11].

Progressive supranuclear palsy (PSP) also known as the Steel Richardson Olzewski syndrome is a neurodegenerative disorder very similar to Parkinson disease. The disorder occurs after the 5th decade of life and is progressive. Since the description of the disorder nearly 50 years ago, many small series have reported on the disorder. It is under-diagnosed not only by general physicians but also by neurologists. The cause of PSP is not known. Exposure to toxins and viruses has been proposed in the etiology of PSP without any concrete evidence. The features of PSP resemble those of Parkinson's disease and the two diseases are often confused. Despite certain common features with Parkinson's disease, PSP is a much aggressive disorder with many neuropsychiatric and motor abnormalities that it often leads to rapid decline and eventually to death.[1][2][3][4]

Progressive supranuclear palsy (PSP), also known as Steele Richardson Olzewski syndrome, is a degenerative motor disorder very similar to Parkinson disease.
Initially most patients have difficulty with visual difficulties, walking and balance but as the disorder advances, cognitive and behaviour changes may also develop. Late in the course of PSP, walking and eye movements become very difficult . The cognitive impairment progresses to full blown dementia
Use of ambulatory aids may help during the early phase of the disease but the risk of fall is always present. Because of difficulty with chewing and swallowing, most patients can only take in liquid or pureed foods. Physical and occupational therapy may help with mobility in the early stages. When swallowing difficulty is severe, patients should have a feeding tube placed in the stomach. The risk of aspiration is always present.
As family members and caregivers of patients with PSP may develop feelings of anger, frustration and guilt, it is important for them to seek support for these difficulties.

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