Progressive supranuclear palsy, also known as Steele-Richardson-Olszewski syndrome, is a degenerative disease involving the gradual loss and deterioration of cells in areas of the brain. The condition causes symptoms including imbalance, slowed movement, difficulty moving the eyes, and dementia. Progressive supranuclear palsy is similar to other neurodegenerative diseases such as Parkinson's and Alzheimer's. The cause of the condition is not yet certain, but it is related to the buildup of a type of protein called tau in the brain. Medications such as levodopa and amantadine may help in some cases. Supranuclear palsy affects approximately six people in every 100,000 people, and symptoms usually begin in people between the ages of 60 and 70. Males are slightly more likely to be affected than females. No association has been found between supranuclear palsy and any particular race, location, or occupation. The initial symptoms in two-thirds of cases are loss of balance, lurching forward when moving, walking quickly, bumping into objects or people, and frequent falls. Dementia is also the first symptom in one-fifth of cases. Other common early symptoms include personality changes and general slowing of movement, in addition to visual symptoms. Later symptoms and signs are dementia, slowing of speech, difficulty swallowing, and difficulty moving the eyes, especially in a vertical direction. The latter symptom explains some of the falls experienced by these patients, as they are unable to look up or down. Other signs include poor eyelid function, facial muscle contraction with head tilted backwards, neck muscle spasms, sleep disturbances, urinary incontinence, and constipation. Visual symptoms are of particular importance in the diagnosis of this disorder. Patients often complain of difficulty reading due to the inability to see well. It is worth noting here that the eye disorders experienced by these patients are mainly related to voluntary eye movement and the inability to make vertical saccadic movements, which are often worse with downward saccadic movements. Patients complain of difficulty looking down due to neck formation, followed by upper gaze palsy, which is corrected when the examiner rotates the patient's head up and down as part of the glioblastoma test. The involuntary eye movements may be close to normal, as in Bell's phenomenon, and on close inspection, eye movements called "square-wave tremors" are visible when the patient fixates at a distance. These are subtle movements, which can be mistaken for nystagmus, but they are rapid in nature. Although healthy individuals also make square-wave tremors, patients with PNP make slower square-wave tremors with smaller vertical movements. Evaluation of these square-wave tremors and decreased vertical movement is particularly useful in diagnosing PNP because these movements distinguish PNP patients from other Parkinson's patients. Convergence difficulties, where the eyes converge when focusing on a nearby object such as the pages of a book, are typical. Because the eyes have difficulty converging to focus at short distances, the patient may complain of diplopia when reading. Primary Pathology: The cause of PNP is not known. Less than 1% of people with PSP have a family history of the disorder, meaning that a genetic factor is unlikely. A variant in the tau gene called haplotype H1, located on chromosome 17, has been linked to PSP. Almost all people with PSP have received a copy of this variant from their parents, but two-thirds of the general population have received a copy of this variant! So it seems that the H1 haplotype is necessary but not sufficient to cause PSP. Other genes and environmental toxins are being investigated as other contributing factors to PSP. Mitochondria and complex I inhibitors are particularly implicated in PSP-like brain lesions. Affected brain cells include both neurons and glial cells. Neurofibrillary tangles, which are clumps of tau protein, are a normal part of the internal structure of brain cells. These tangles are often different from those seen in Alzheimer's disease, but may be structurally similar when they occur in the cerebral cortex. Its chemical composition is usually different, similar to the tangles seen in corticobasal atrophy. The presence of tau protein chains in astrocytes or in astrocyte bundles is also a diagnostic factor. Unlike globular neurofibrillary tangles, they may be more widespread in the cortex. Lewy bodies are seen in some cases, but it is not clear whether this is a distinct or independent process. In some cases, PSP can coexist with cortical atrophy, Parkinson's disease, and/or Alzheimer's disease, especially in older patients. The main areas of the brain affected are: MRI is often performed to diagnose PSP. MRI may show atrophy of the midbrain with preservation of the pons, giving the "hummingbird" and Mickey Mouse signs. Progressive supranuclear palsy is often misdiagnosed as Parkinson's disease because it presents with slowed movements and difficulty walking, and in these symptoms it is similar to progressive supranuclear palsy as it is one of a group of diseases referred to as Parkinsonian syndromes. Both Parkinson's and progressive supranuclear palsy begin in late middle age and involve slowed and rigid movement. However, there are several distinguishing features between them. Tremor is very common in people with Parkinson's disease, but rare in people with progressive supranuclear palsy. Difficulty speaking and swallowing are more common and severe in progressive supranuclear palsy, and the abnormal eye movements of progressive supranuclear palsy are essentially absent in people with Parkinson's disease. Poor response to levodopa can help distinguish between Parkinson's and progressive supranuclear palsy. Patients with Richardson type of progressive supranuclear palsy tend to stand upright or with a hunched back, unlike the forward-bending posture seen in other Parkinsonian disorders. Frequent falls are also more common with progressive supranuclear palsy, especially Richardson type. Progressive supranuclear palsy can be misdiagnosed as Alzheimer's disease because of behavioral changes. Chronic traumatic encephalopathy (CTEP) shows many similarities to PSP. There is no cure for PSP, and treatment is primarily supportive. PSP is often divided into two subgroups, Richardsonian PSP, which is the classic type, and parkinsonian PSP, in which a short-term response to levodopa can be obtained. Dyskinesia is an occasional but rare complication of treatment. Amantadine is also sometimes helpful. After a few years, PSP tends to take on the characteristics of Richardson's disease. Botox can be used to treat neck dystonia and blepharospasm, but this can worsen dysphagia. Two studies have suggested that rivastigmine may help with cognitive aspects, but the authors of both studies suggest using large sample sizes. There is some evidence that the hypnotic zolpidem may improve motor and eye function, but these studies have been small. Patients with PSP typically seek or are referred to occupational therapy, speech-language pathologists for speech changes, and physical therapy for balance and gait problems and history of frequent falls. Reports of rehabilitation in PSP are lacking in scientific evidence, and currently the majority of research on this topic consists of reports involving only a small number of patients. Reports of rehabilitation programs for patients with PSP include limb coordination activities, balancing on incline boards, gait training, strength training with progressive resistance exercises, isokinetic exercises, and neck muscle stretching. While some reports suggest that physical therapy can provide improvements in balance and gait for patients with PSP, the results cannot be generalized to all patients with PSP, as each report only involved one or two patients. However, the observations obtained from these case studies may be useful in helping to guide future research on the effectiveness of balance and gait training programs in the rehabilitation of patients with PSP. Individuals with progressive supranuclear palsy are often referred to occupational therapists to help manage their condition and to help promote independence. Treatment may include teaching them to use transportation. A walker, especially one that leans forward, is recommended because of their tendency to fall backwards frequently. Using an appropriate mobility aid can help reduce the risk of falls and make the individual safer in the community. Due to balance problems and irregular movements, individuals need to spend time learning how to get around safely in their homes and in the community. This may include getting up from a sitting position and then sitting in a chair safely. Due to the progressive nature of the disease, most individuals eventually lose the ability to walk and will eventually need to use a wheelchair for mobility. This is often followed by severe difficulty swallowing, at which point death is often only a matter of months. Although some symptoms can respond to some measures, no cure has been found for progressive supranuclear palsy. The average age of onset is 63 and the average survival time from onset is 7 years, with wide variation between patients. In these patients, pneumonia is a common cause of death. Progressive supranuclear palsy was first described by neurologists John Still, John Richardson, and Jerzy Olszowski in 1963.