This is the classic symptom of the disorder, but it is not always present at initial presentation and can take a number of years to develop. [lecturio.com]

(Table) 3) Pure akinesia with gait freezing (PAGF) is a rare presentation of PSP-tau pathology. [movementdisorders.org]

Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia. / Nagao, Shigeto; Yokota, Osamu; Nanba, Reiko; Takata, Hiroshi; Haraguchi, Takashi; Ishizu, Hideki; Ikeda, Chikako; Takeda [okayama.pure.elsevier.com]

Presentation Variable presentation, depending on subtype. [patient.info]

Less commonly, patients present with cognitive loss and no motor signs. [rarediseases.org]

• Falling

  Unlike Parkinson Disease, people often fall backward instead of forward. They may also develop severe stiffness in the neck. [brain.northwestern.edu]

  Devices that make walking safer, such as a walker that is weighted in the front, can be helpful to prevent falls. Shoes with built-up heels can also help decrease falling backwards. [bcm.edu]

  I am falling frequently. What should I do? Falls are one of the leading causes of injury, disability, and even death in the elderly. [movementdisorders.ufhealth.org]

  The beginning stages of PSP include the inability to walk, falling spells, and stiffness. Falls experienced by a PSP patient are often described as having a state of dizziness, prior to actually falling. [webmd.com]

  PSP-Parkinsonism (PSP-P) is characterized by prominent early parkinsonism (tremor, limb bradykinesia, axial and limb rigidity) rather than falls and cognitive change. [orpha.net]

• Movement Disorder

  Movement Disorders, Second Edition vigorously examines the important contributions and application of animal models to the understanding of human movement disorders, and serves as an essential resource for basic neuroscientists engaged in movement disorders [books.google.com]

  Since the early 1960s when many astute movement disorder specialists were unable to recall having seen progressive supranuclear palsy, this disorder has become commonly recognized at movement disorder referral centers, comprising 4% to 5% of their cases [medlink.com]

  Movement Disorders: Official Journal of the Movement Disorder Society. 2004 ; 19 ( 2 ): 181–9. 69. Litvan, I, Agid, Y, Jankovic, J, Goetz, C, Brandel, JP, Lai, EC, et al. [cambridge.org]

  Diagnosis of Movement Disorders Progressive Supranuclear Palsy There a number of different clinical presentations of this disorder. The most classic phenotype is known as Richardson phenotype. [lecturio.com]

  To be sure of your diagnosis, seek a neurologist familiar with Parkinsons and ask for specialist with movement disorders training, such as you will find here at the UF Center for Movement Disorders & Neurorestoration. [movementdisorders.ufhealth.org]

• Progressive Dementia

  […] vertical gaze palsy) and mild progressive dementia. [lecturio.com]

  "Relationship between frontotemporal dementia and corticobasal degeneration/progressive supranuclear palsy". Dementia and Geriatric Cognitive Disorders. 17 (4): 282–6. doi : 10.1159/000077155. [ipfs.io]

  Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54 Suppl 5: S5-19. [omicsonline.org]

• Aspiration

  The main concern is aspiration, or swallowing something down the wind-pipe, which can cause a reactive (and even infectious) pneumonia. Pneumonias of course are a leading cause of death in patients. [movementdisorders.ufhealth.org]

  Because of swallowing problems some patients require placement of a feeding tube directly into the stomach in order to maintain adequate nutrition and prevent aspiration pneumonia. [bcm.edu]

  Patients present with disturbance of balance, a disorder of downward gaze and L-DOPA-unresponsive parkinsonism and usually develop progressive dysphagia and dysarthria leading to death from the complications of immobility and aspiration. [pmj.bmj.com]

  "Impact of Aspiration Pneumonia on the Clinical Course of Progressive Supranuclear Palsy: A Retrospective Cohort Study.". PLOS ONE. 10 (8): e0135823. doi : 10.1371/journal.pone.0135823. PMC 4536232. PMID 26270456. ↑ "K. [ipfs.io]

  "Impact of Aspiration Pneumonia on the Clinical Course of Progressive Supranuclear Palsy: A Retrospective Cohort Study". PLOS One. 10 (8): e0135823. Bibcode : 2015PLoSO..1035823T. doi : 10.1371/journal.pone.0135823. PMC 4536232. [en.wikipedia.org]

• Sweating

  Shy-Drager syndrome is an extrapyramidal syndrome that combines autonomic system dysfunction (orthostatic hypotension, impotence, disturbances of sweat and salivary gland secretion, papillary abnormalities) and parkinsonism. [lecturio.com]

• Diplopia

  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]

  Additional, and important symptoms to note, include: Diplopia Bradykinesia Burning eyes Blurred vision Sensitivity to light Rigidity of the spine Eye movement paralysis The eyelids of people with PSP might be held wide open with eye movement paralysis [disabled-world.com]

  Despite her limited eye movements, the patient had not noted diplopia and denied vision changes. [webeye.ophth.uiowa.edu]

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

• 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]

  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]

  Imaging did not support this, however, and on formal testing, abnormal nystagmus and eye movements were detected. A sagittal view of the CT / MRI scan shows atrophy of the midbrain, with preservation of the volume of the pons. [en.wikipedia.org]

• Anhedonia

  Patients can also have anhedonia, anxiety, and dysphoria. Ask about sleep, as they may have sleep disturbances even in early PSP. On examination, one expects to eventually find a Supranuclear ophthalmoparesis. [lecturio.com]

• Inappropriate Laughter

  Ups and downs, “roller coaster” emotions In PSP emotional lability, or pseudobulbar affect, may occur and is characterized by sudden or inappropriate laughter or inconsolable crying. [movementdisorders.ufhealth.org]

• Incontinence

  There's urinary incontinence and bowel incontinence," says Swankie. [theguardian.com]

  It can also cause behavioural changes - leading the patient to cry and laugh too readily and at inappropriate times - and incontinence. As the condition progresses, speech becomes slurred and movement becomes jerkier and less stable. [dailymail.co.uk]

  Image : “This patient presented with progressive dementia, ataxia and incontinence. A clinical diagnosis of normal pressure hydrocephalus was entertained. [lecturio.com]

  Micturition disturbances, including urinary incontinence, are common in the later stages. Signs The classic gaze palsy usually involves looking down before looking up. It affects horizontal, as well as vertical, eye movements. [patient.info]

  It has been described as a tauopathy. [3] Symptoms and signs This patient presented with progressive dementia, ataxia and incontinence. A clinical diagnosis of normal pressure hydrocephalus was entertained. [ipfs.io]

• Apraxia

  However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. [okayama.pure.elsevier.com]

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

  PSP-PNFA is characterized by speech anomalies (apraxia of speech, agrammatism, phonemic errors). Motor disturbances may occur later. [orpha.net]

  Botulinum toxin injections into the eyelids and eyebrows can be effective for treating patients with blepharospasm and apraxia of eyelid opening. [bcm.edu]

• Dysarthria

  A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. [okayama.pure.elsevier.com]

  Ex: Goals, sentences etc PSP cognitive symptoms: Language -Processing and comprehension relatively spared -Slowed speech -dysarthria difficult or unclear articulation of speech that is otherwise linguistically normal. [quizlet.com]

  Lack of spontaneous and associative movements, dysarthria and facial immobility may suggest the diagnosis of PD during conversation and history taking. [pmj.bmj.com]

  They may lose the ability to control their mouth to speak, called apraxia of speech, or progressive slurring of speech, called dysarthria. People with CBD can develop loss of inhibition. [brain.northwestern.edu]

  Results in ataxia, eye and somatic movement abnormalities, dysarthria and rigidity. [lecturio.com]

• Motor Symptoms

  Motor symptoms appear later in the course of the disease. PSP is characterized neuropathologically by neuronal loss, gliosis with astrocytic plaques and accumulation of tau-immunoreactive neurofibrillary tangles in specific brain areas. [orpha.net]

  Motor symptoms come first and always precede cognitive changes. The progression of the disease is slow between 5 to 10 years. The age of onset is typically over 50 years old. The duration of the disease is 7 years. [alzheimer-europe.org]

  One of these areas, the substantia nigra, is also affected in Parkinson's disease, and damage to this region of the brain accounts in part for the motor symptoms that PSP and Parkinson's have in common. [ninds.nih.gov]

  In patients with prominent motor symptoms and a suspicion of CBD, levodopa can often be administered as a screening test. Multiple System Atrophy Patients with MSA typically have an akinetic parkinsonism with marked rigidity. [lecturio.com]

  Introduction Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome, characterized by motor symptoms, such as postural instability, rigidity, akinesia, and behavioral and cognitive symptoms. [dovepress.com]

• Irritability

  Then came new symptoms: falls, tremors, difficulty speaking, troubled sleep, irritability. It was only when Swankie's GP was at a conference where a geriatrician was talking about PSP that the pieces started to fall into place. [theguardian.com]

  As regards behavioral symptoms, an interview with relatives revealed depression, anxiety, apathy, irritability, and sleep disorders. [dovepress.com]

  Cognitive symptoms include forgetfulness and personality changes, such as loss of interest in formerly pleasurable activities (apathy), impaired attention and concentration, depression, and increased irritability. [rarediseases.org]

  They may lose interest in ordinary pleasurable activities or show increased irritability and forgetfulness. [ninds.nih.gov]

  Behavior or personality changes - Irritation, grouchiness Memory loss, forgetfulness Apathy (indifference) Slowed thinking, reasoning, planning Inappropriate laughing or crying Angry or aggressive outbursts Slurred speech Swallowing problems - Solids [emedicinehealth.com]

• Resting Tremor

  Resting tremor is unusual. Differential diagnosis Other causes of dementia, and other movement disorders - eg, Parkinson's disease. [patient.info]

  Some have a jerky postural tremor and even a 4 to 6Hz rest tremor.6-8 A "moderate" or "good" improvement in parkinsonism follows initiation of L-dopa in a proportion of the patients and secondary unresponsiveness occurring over a few years is usual.6 [movementdisorders.org]

  The main clinical manifestations are resting tremor, rigidity, impaired postural reflexes and sustained response to levodopa (L-DOPA) treatment. [journal.frontiersin.org]

  […] be diagnosed premortem.[16] PSP results in hallmark clinical signs including vertical gaze palsy (especially downgaze) and loss of balance.[16] In addition, patients with PSP may experience dysarthria, dysphagia, muscle rigidity, a Parkinsonian like resting [eyewiki.aao.org]

[…] patients with PSP show the following abnormalities, although these are not specific for PSP: Diminished total sleep time Increased awakenings Progressive loss of rapid-eye-movement (REM) sleep [7, 8] Decreased REM–to–non-REM (NREM) quotient [9, 10] See Workup [emedicine.medscape.com]

However, the introduction of MR imaging into the routine workup of patients with suspected PSP or other parkisonian syndromes has been hampered by its low sensitivity and poor specificity and also by high variability, which can be heavily influenced by [ajnr.org]

• Neurofibrillary Tangle

  Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a [okayama.pure.elsevier.com]

  Tau neurofibrillary tangles appear most commonly as globose tangles under the light microscope, in contrast to the flame-shaped tangles of Alzheimer's disease. [pmj.bmj.com]

  Neuropathologically, all forms are characterized by neuronal loss, gliosis with astrocytic plaques and accumulation of tau-immunoreactive neurofibrillary tangles in specific brain areas. [orpha.net]

Management and treatment There is no treatment curing the disease. Classical PSP patients do not respond to levodopa treatment. [orpha.net]

Is there any treatment? There is currently no effective treatment for PSP, although scientists are searching for better ways to manage the disease. [my.clevelandclinic.org]

Prognosis Progressively, patients become wheel-chair dependant due to the frequent falls. Difficulties in breathing and swallowing, and infections are the main causes of death, generally 4-8 years after onset of the disease. [orpha.net]

However, patients will benefit from accurate, early diagnosis that allows them to understand the prognosis, to avoid unnecessary tests and useless treatments, and to avail themselves of disease-specific support services. [medlink.com]

What is the prognosis? PSP gets progressively worse but is not itself directly life-threatening. It does, however, predispose patients to serious complications such as pneumonia secondary to difficulty in swallowing (dysphagia). [my.clevelandclinic.org]

The prognosis for patients with PSP is poor, with the average time from onset of symptoms to death ranging from 5-9 years. [eyewiki.aao.org]

Etiology PSP is a 4R tauopathy composed of a preponderance of four-repeat tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). The MAPT H1-clade specific sub-haplotype, H1c, is a risk factor for this disease. [orpha.net]

Summary Epidemiology The prevalence is conservatively estimated at about 1/16,600. [orpha.net]

[…] to be described and their seminal report was followed by many case reports and case series from around the world. 8 The documentation of these individual cases and case series through the 1960s, 1970s and 1980s have been followed by more recently by epidemiological [pmj.bmj.com]

Epidemiology [ 1, 9 ] The mean age of onset is 65 years. Virtually no autopsy-confirmed cases have been confirmed earlier than 40 years. PSP is a rare disease. [patient.info]

Academic clinicians, translational researchers and basic scientists are brought together to connect experimental findings made in different animal models to the clinical features, pathophysiology and treatment of human movement disorders. [books.google.com]

It is also useful for the investigation of the pathophysiology of diseases. [touchneurology.com]

The role of heredity in the pathophysiology of PSP remains elusive. Although there are anecdotal reports in the literature that describe apparent familial PSP, several larger series have not noted this association. [emedicine.medscape.com]

Pathophysiology of Movement Disorders Progressive Supranuclear Palsy It causes neuronal degeneration and loss in the globus pallidus, subthalamic nucleus, substantia nigra, colliculi, periaqueductal gray matter and the dentate nucleus of the cerebellum [lecturio.com]

In addition there must be a low to high density in at least three of the following structures: oculomotor complex, dentate nucleus, striatum, or medulla.[16, 33] Pathophysiology As stated previously, PSP is a known tauopathy. [eyewiki.aao.org]

Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. ‎ [books.google.es]

Evaluation early on by a trained physical therapist, knowledgeable in Parkinsonism, is critical to help with gait, balance, and prevent falls. [movementdisorders.ufhealth.org]

Because of swallowing problems some patients require placement of a feeding tube directly into the stomach in order to maintain adequate nutrition and prevent aspiration pneumonia. [bcm.edu]

Is There A Way to Prevent Progressive Supranuclear Palsy? There is no known way to prevent PSP. Which Specialties of Doctors Diagnose and Treat PSP Symptoms? The medical care of a person with PSP usually involves a team of health professionals. [emedicinehealth.com]

(NNIPPS) Terminated NCT00211224 Phase 3 Riluzole 6 Effects of Coenzyme Q10 in PSP and CBD Completed NCT00532571 Phase 2, Phase 3 CoQ10 7 Study About Efficacy and Safety to Treat Multi-System-Atrophy Completed NCT00146809 Phase 3 Minocyline 8 Relapse Prevention [malacards.org]