Treatment of progressive supranuclear palsy is symptomatic and supportive. The spectrum of the clinical phenotypes in these families was variable, including 34 typical cases of psp 12 probands plus 22 secondary cases, 3 patients with postural tremor, 3 with dementia, 1 with. Progressive supranuclear palsy psp is the most common atypical parkinsonian disorder, with a prevalence of approximately five to six individuals per 100,000. Other common early symptoms include changes in personality, mood and behavior. Wholebrain gray matter metaanalyses show atrophy in the midbrain in psp. Progressive supranuclear palsy psp is a rare progressive condition that can cause problems with balance, movement, vision, speech and swallowing. Another feature, the mickey mouse sign, describes selective atrophy of the. Progressive supranuclear palsy psp is a neurodegenerative disease that results in severe disability. Recent pathological studies of large numbers of patients diagnosed as having parkinsons disease in life have highlighted the fact that at least one in ten of such cases have some other condition. Sep 28, 2014 progressive supranuclear palsy psp also known as the steelerichardsonolszewski syndrome, a neuro degenerative disease with no treatment. Most commonly, the first symptom is losing balance while walking, which may cause unexplained falls. The histopathology is prominent in basal ganglia, subthalamic nucleus, brainstem nuclei, dentate nucleus of cerebellum, and frontal cortex.
Later symptoms and signs are dementia typically including loss of inhibition and ability to. Hummingbird sign and mickey mouse sign in psp brain support. Diversified psychiatric presentation in a case of progressive. Dec 18, 2019 progressive supranuclear palsy psp is a rare brain disease. It was first described as a clinicopathologic entity in 1964. This blog post from 20 is about the hummingbird sign and mickey mouse sign in progressive supranuclear palsy psp. Example of patient with progressive supranuclear palsy. May 09, 20 progressive supranuclear palsy psp is an atypical parkinsonian syndrome characterized by motor symptoms, postural instability, personality changes, and cognitive impairment. Progressive supranuclear palsy psp is a rare brain disorder that causes serious and progressive problems with control of gait and balance, along with complex eye movement and thinking problems. In some cases, drugs used to treat parkinson disease antiparkinsonian agents, such as levodopa, may be of some benefit in relieving symptoms of slowness, but the effect is usually limited and temporary. Progressive supranuclear palsy psp or steelerichardsonolszewski syndrome is a neurodegenerative disease of middle and late age. Progressive supranuclear palsy psp is a dementia associated with degeneration of the frontosubcortical neural networks, often resulting in apathy, decreased executive function, or impulsivity. If you have problems viewing pdf files, download the latest version of adobe reader. The psp association estimates there are around 4,000 people with psp living in the uk.
Progressive supranuclear palsy is characterized by decreased cognition, abnormal eye movements supranuclear vertical gaze palsy, postural instability and falls, as well as. Key emerging issues in progressive supranuclear palsy and. A 79yearold male with gait ataxia and frequent falls with forgetfulness and loss of interest in routine activity. Progressive supranuclear palsy psp is the second most common of the four atypical parkinsonisms and exists in two variants. Progressive supranuclear palsy genetic and rare diseases. Progressive supranuclear palsy psp is a rare degenerative brain disorder that affects movement, control of walking and balance, vision, speech, and cognition. Almost nine years after her mother passed away from a rare brain disease, rita clagett, author of killing mother. Most people with progressive supranuclear palsy survive 5 to 9 years after the disease first appears. One of the classic signs of the disease is an inability to aim the eyes properly, which occurs because of lesions in the area of the brain that. The disease results from damage to nerve cells in the brain. Progressive supranuclear palsy diagnosis progressive supranuclear palsy psp is a rare neurodegenerative condition that is a form of atypical parkinsonism, meaning that it shares some features with parkinsons disease. Although certain features help in favoring psp over alternative clinical diagnoses parkinson. Progressive supranuclear palsy psp and parkinsons disease. Progressive supranuclear palsy psp is a rare degenerative disorder of the brain.
Its caused by increasing numbers of brain cells becoming damaged over time. Progressive supranuclear palsy typically becomes clinically apparent in the 6 th decade of life, and progresses to death usually within a decade 217 years from diagnosis clinical presentation. The loss of nerve cells causes palsy, or paralysis, that slowly gets worse as the disease progresses. It is underdiagnosed not only by general physicians but also by neurologists. These include stiffness of neck and trunk muscles rigidity, slowness of movement bradykinesia, and impaired balance. The signs and symptoms of this disorder usually become apparent in mid to late adulthood, most often in a persons 60s.
Progressive supranuclear palsy psp brain support network. Humming bird sign, mickey mouse sign, and morning glory sign in. Psp develops because of the deterioration of brain cells in a few small, but very important areas. Progressive supranuclear palsy psp is a disorder characterized by symptoms similar to parkinsons disease including unsteady gait, stiff movements, and mild dementia. Progressive supranuclear palsy nord national organization. Progressive supranuclear palsy radiology reference article.
Progressive supranuclear palsy psp is the second most common of the four atypical p arkinsonisms and exists in two variants. Progressive supranuclear palsy psp is a rare brain disease. Symptoms usually begin between ages 50 and 60, with a range from the early forties to the late eighties and is more common in men than in women. Animals in the brain mulroy 2019 movement disorders clinical. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Progressive supranuclear palsy psp, also known as the. Pspa is a national charity providing support and information to people living with psp and cbd while funding research into treatments and ultimately a cure. The condition leads to symptoms including loss of balance, slowing of movement, difficulty moving the eyes, and dementia. Because of its rarity, psp is mostly unknown by the general public. Progressive supranuclear palsy psp causes a wide range of symptoms that. Psp is progressive, which means that it gets worse over time. People with progressive supranuclear palsy psp develop a range of difficulties with balance, movement, vision, speech and swallowing.
Progressive supranuclear palsy psp, also known as the steelerichardson olszewski syndrome, is a form of parkinsonplus syndrome. Causes psp is a condition that causes symptoms similar to those of parkinson disease. Progressive supranuclear palsy psp, previously known as. Much has been learnt about this sporadic illness of middle and late life although its cause remains unknown, and its treatment continues to be difficult. Progressive supranuclear palsy, or psp, is a rare neurodegenerative disease that is often misdiagnosed as parkinsons disease because it carries similar symptoms. It destroys nerve cells in the parts of the brain that control eye movements, breathing and muscle coordination.
Based on the clinical and radiographic features, he was diagnosed as a case of progressive supranuclear palsy steele. Pathologic diagnosis was confirmed by internationally agreed criteria. Progressive supranuclear palsy psp and corticobasal degeneration cbd are neurodegenerative diseases first described almost half a century ago 1, 2 that are both characterized by the deposition of tau, a microtubule associated protein, in an altered aberrant form. The neuropathological hallmark of psp is a biochemical alteration in the tau protein, which. Mar 17, 2020 progressive supranuclear palsy psp is an uncommon brain disorder that affects movement, control of walking gait and balance, speech, swallowing, vision, mood and behavior, and thinking. The main neuropathologic characteristics of psp are neuronal degeneration and loss in the tegmentum of the midbrain, atrophy of the. Progressive supranuclear palsy psp represents the most common form of atypical parkinsonism with a prevalence of 6. Mean age of onset is approximately 63 years, and mean survival from symptom onset is 9 years. Progressive supranuclear palsy psp is a degenerative condition of unknown aetiology that produces an akineticrigid form of parkinsonism characterised by early falls and abnormalities of extraocular movements. Psp is a very individual condition and every case is unique. This page is dedicated to sharon rae mueller franke 19442010.
Progressive supranuclear palsy psp, also known as the steelerichardsonolszewski syndrome, is a form of parkinsonplus syndrome. The condition tends to develop gradually, which means it can be mistaken for another, more common, condition at first. A particular mr finding in progressive supranuclear palsy article in magnetic resonance in medical sciences 33. See more ideas about psp, alzheimer care and mri brain. It happens because of damage to nerve cells in the brain.
Progressive supranuclear palsy is one of the commonest alternative diagnoses. Progressive supranuclear palsy, reflects on her experiences as a caregiver and what it all means compassionate journeys. Progressive supranuclear palsy psp is an uncommon brain disorder that affects movement, control of walking gait and balance, speech, swallowing, vision, mood and behavior, and thinking. Hummingbird sign also known as the penguin sign on mid sagittal sections as in our case. Shows saccadic intrusions in forward gaze, pursuit. Progressive supranuclear palsy dementia nuchal dystonia. Progressive supranuclear palsy psp is a movement disorder that occurs from damage to certain nerve cells in the brain. Shobha m itolikar, santosh b salagre, chetan r kalal. When steele, richardson and olszewski described progressive supranuclear palsy in archives of neurology in 1964, it was thought to be a rare disease. Progressive supranuclear palsy richardson syndrome 060419. When my friend chris told me that she still missed her mother and thought about her every day, nine years after she died, i thought, hmmm.
Mr imaging of the superior profile of the midbrain. Rita clagett is a naturalist, writer and photographer who lives in western colorado with two dogs, an orange cat, and a tortoise, keeper of slow time. The clinical diagnosis of progressive supranuclear palsy psp and multiple system atrophy msa remains challenging due to heterogeneity. Your support means we can be a port in the storm that people can turn to in their darkest hours, connecting them to a network of support, providing. Depending on whom you are talking to, it is classified as an atypical parkinsonism disorder, a nonalzheimers dementia, a type of frontotemporal degeneration, or a subcortical dementia. Progressive supranuclear palsy radiology reference. Progressive supranuclear palsy psp is a degenerative disease involving the gradual. Progressive supranuclear palsy psp is an atypical parkinsonian syndrome characterized by motor symptoms, postural instability, personality changes, and cognitive impairment.
It is characterized by supranuclear vertical gaze palsy, pseudobulbar palsy, dystonic rigidity of the neck and upper extremities, and frequent falls. The nerve cell loss causes palsy or paralysis that slowly gets worse as the disease progresses. Significant advancements have been made over the past half century for both psp and cbd. Patients with psp show interesting radiological signs such as the. Progressive supranuclear palsy psp is an unusual neurodegenerative disorder with variant clinical phenotypes. Progressive supranuclear palsy is a rare brain disease affecting only a few thousand people in the u. Progressive supranuclear palsy definition of progressive. Recent work suggests that the disease is at least partly genetic. The key is a flattening or concave outline to the superior aspect of the midbrain which should be upwardly convex.
Hummingbird sign is a rather interesting sign seen in midsagital view of mri brain in patient with progressive supranuclear palsy psp ref1. 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. Progressive supranuclear palsy psp is a degenerative disease involving the gradual deterioration and death of specific volumes of the brain. The symptoms typically become more severe over several years, although the speed at which. Mri may show atrophy in the midbrain with preservation of the pons giving a hummingbird sign appearance and mickey mouse sign. Catalog home health topics progressive supranuclear palsy progressive supranuclear palsy 2 products local navigation. Progressive supranuclear palsy progressive supranuclear palsy psp, also known as steelerichardsonolszewski syndrome, is a neurodegenerative disease that affects cognition, eye movements, and posture. The cause of progressive supranuclear palsy is not known, but it is a form of tauopathy, in which abnormal phosphorylation of the protein tau leads to destruction of vital protein filaments in nerve cells, causing their death. Jan 28, 2016 progressive supranuclear palsy psp causes a wide range of symptoms that differ among affected people. Progressive supranuclear palsy an overview sciencedirect. Psp affects your movement, including control of your walking and balance.
Progressive supranuclear palsy genetics home reference nih. Progressive supranuclear palsy psp is a neurological condition caused by the premature loss of nerve cells in certain parts of the brain. Psp may be mistaken for other neurodegenerative diseases such as parkinsons and alzheimers. Progressive supranuclear palsy corticobasal degeneration.
For language access assistance, contact the ncats public information officer. Richardsons syndrome psprs and pspparkinsonism pspp. Discussion of difference between saccadic movement in supranuclear palsy and nystagmus. Progressive supranuclear palsy baylor college of medicine. Progressive supranuclear palsy is available on amazon and other internet booksellers. Progressive supranuclear palsy fact sheet national. We are thrilled to launch our new online interactive resource aimed at helping healthcare professionals involved in caring for people. Progressive supranuclear palsy is a brain disorder that affects movement, vision, speech, and thinking ability cognition. Hummingbird sign, penguin sign and mickey mouse sign. Bradley boeve, a mayo clinic neurologist, will explain progressive supranuclear palsy psp, a rare brain disease. Psp typically becomes clinically apparent in the 6th decade of life, and progresses to death usually within next 2 decades from diagnosis. The palsy affects your ability to move the eyes, relax. Ways to give progressive supranuclear palsy psp and related prime of life brain diseases cause devastation, heartache, and tremendous suffering to those afflicted and their families.