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Classic Progressive Supranuclear Palsy Syndrome


Presentation

  • 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]
  • Presentation Variable presentation, depending on subtype.[patient.info]
  • Less commonly, patients present with cognitive loss and no motor signs.[rarediseases.org]
  • Most notably, a presentation with Parkinson syndrome and absent gaze palsy has been identified, with less severe PSP-tau pathology.Conclusions: In recognition of his seminal observations, we propose that the classic clinical presentation of PSP-tau pathology[discovery.ucl.ac.uk]
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]
Pathologist
  • If swallowing issues are identified, such as coughing or choking on food or liquids, it is advisable to undergo evaluation with a speech-language pathologist, who can help advise on dietary modifications to aid in safe swallowing.[bcm.edu]
Painter
  • ") (1931-2007) Rae Ferren, American impressionist painter (1929-2016) Richard Frank, past leader of nonprofit Population Services International and first husband of Jane Harman, died 20 April 2014, age 77 [43] Phyllis Frelich, actress (1944-2014) Bob[ipfs.io]
Progressive Dysphagia
  • 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]
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]
  • […] supranuclear vertical gaze limitation after extravolitional pathway activation with the vestibular ocular reflex (VOR) or the Bell phenomenon Nearly continuous square wave jerks commonly observed with fixation Impairment of convergence eye movements (may cause diplopia[emedicine.medscape.com]
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]
Startled Facial Expression
  • Obvious facial signs may be present with examples including decreased blink rate and a “startledfacial expression, which results from rigid facial muscles, but these signs may not be present in the early stages of the disease.[bcm.edu]
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]
  • 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]
  • As regards behavioral symptoms, an interview with relatives revealed depression, anxiety, apathy, irritability, and sleep disorders.[dovepress.com]
  • 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]
Axial Dystonia
  • dystonia Behavior changes: apathy, disinhibition, anxiety Frontal release signs, including grasp reflex and the applause sign May exhibit cross-over with other Tauopathies (e.g., Progressive Non-Fluent Aphasia) Diagnosis Clinical (see above) MRI shows[webeye.ophth.uiowa.edu]
  • Over a period of 2 years, he progressed to complete vertical gaze palsy, axial dystonia, retrocollis, and generalized severe akinesia.[emedicine.medscape.com]
Spastic Gait
  • Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs.[okayama.pure.elsevier.com]
Palmomental Reflex
  • Glabellar and palmomental reflexes in parkinsonian disorders. Neurology. 2004;63:1096-8. Burrell JR, Hodges J.R, Rowe J.B. Cognition in corticobasal syndrome and progressive supranuclear palsy: A review. Mov Disord. 2014;29:684–93.[bcm.edu]
Broad-Based Gait
  • The gait has a characteristic reeling or staggering quality, due to the stiff posture of the trunk and neck with irregular large steps forwards, which allow a distinction to be made from the veering broad based gait of cerebellar ataxia.[pmj.bmj.com]

Workup

  • […] 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]
Ischemic Changes
  • Symptomatic characteristics of parkinsonism and the width of substantia nigra pars compacta on MRI according to ischemic changes in the putamen and cerebral white matter: implications for the diagnosis of vascular parkinsonism.[ajnr.org]

Treatment

  • 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

  • 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]
  • 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]
  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • Levodopa is the component that helps the disease symptoms; carbidopa simply helps prevent the nausea that levodopa can cause.[psp.org]

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