![]() Ī characteristic facial appearance known as “procerus sign”, with a wide-eye stare, furrowing of forehead with a frowning expression and deepening of other facial creases is diagnostic of PSP. Because the eyes have trouble coming together to focus at short distances, the patient may complain of diplopia (double vision) when reading. Difficulties with convergence (convergence insufficiency), where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Assessment of these square-wave jerks and diminished vertical saccades is especially useful for diagnosing progressive supranuclear palsy, because these movements set PSP patients apart from other parkinsonian patients. Although healthy individuals also make square-wave jerk movements, PSP patients make slower square-wave jerk movements, with smaller vertical components. These are fine movements, that can be mistaken for nystagmus, except that they are saccadic in nature, with no smooth phase. On close inspection, eye movements called "square-wave jerks" may be visible when the patient fixes at distance. Involuntary eye movement, as elicited by Bell's phenomenon, for instance, may be closer to normal. This vertical gaze paresis will correct when the examiner passively rolls the patient's head up and down as part of a test for the oculocephalic reflex. Patients tend to have difficulty looking down (a downgaze palsy) followed by the addition of an upgaze palsy. The ophthalmoparesis experienced by these patients mainly concerns voluntary eye movement and the inability to make vertical saccades, which is often worse with downward saccades. Patients typically complain of difficulty reading due to the inability to look down well. The visual symptoms are of particular importance in the diagnosis of this disorder. Some patients retain full cognitive function up to the end. Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence, and constipation. The latter accounts for some of the falls experienced by these patients, as they find it difficult to look up or down. ![]() Later symptoms and signs can include, but do not necessarily include dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, particularly in the vertical direction. The most common behavioural symptoms in patients with PSP include apathy, disinhibition, anxiety, and dysphoria. Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms. Dementia symptoms are also initially seen in about one in five cases of frontotemporal dementia. The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls. No association has been found between PSP and any particular race, location, or occupation. Males are slightly more likely to be affected than females. The first symptoms typically occur at 60–70 years of age. PSP affects about six people per 100,000. Medications such as levodopa and amantadine may be useful in some cases. The cause of the condition is uncertain, but involves accumulation of tau protein within the brain. PSP may be mistaken for other neurodegenerative diseases such as Parkinson's, frontotemporal dementia and Alzheimer's. The condition leads to symptoms including loss of balance, slowing of movement, difficulty moving the eyes, and cognitive impairment. Progressive supranuclear palsy ( PSP) is a late-onset degenerative disease involving the gradual deterioration and death of specific volumes of the brain. Medication, physical therapy, occupational therapy Parkinson's disease, corticobasal degeneration, FTDP-17, Alzheimer's disease Impaired balance, slowed movements, difficulty moving eyes, dementia These findings suggest the diagnosis of progressive supranuclear palsy. Also, atrophy of the tectum is seen, particularly the superior colliculi. This appearance has been called the "hummingbird sign" or "penguin sign". A sagittal view of the CT/ MRI scan shows atrophy of the midbrain, with preservation of the volume of the pons. ![]() Imaging did not support this, however, and on formal testing, abnormal nystagmus and eye movements were detected. ![]() A clinical diagnosis of normal-pressure hydrocephalus was entertained. ![]() Steele–Richardson–Olszewski syndrome, frontotemporal dementia with parkinsonismĪ person with progressive dementia, ataxia, and incontinence. Medical condition Progressive supranuclear palsy ![]()
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