Progressive supranuclear palsy (PSP) is an uncommon brain disorder. It affects many areas of function, including a person's movement, balance, mood and behavior, speech, and thoughts. Damage done to nerve cells within the brain cause this condition. The illness worsens and causes weakness in the body through damaging the nuclei in the brain. In particular, these nuclei are in control of a person's eye movements. PSP's symptoms often mimic Parkinson's disease, but the two conditions are separate. While there is no current treatment for progressive supranuclear palsy, early detection can help patients manage their symptoms with medication. These are the main symptoms and warning signs that PSP is developing.
Loss Of Balance
Progressive supranuclear palsy is most likely to manifest in individuals over sixty. Those in this age group don't tend to think much of a loss of balance; it's just part of getting older. However, repeated losses of balance are unusual. If you've had multiple falls from losing your balance, this might be a sign something is neurologically amiss. PSP's balance issues usually result in a person falling straight backward rather than forward or to the side.
Falls can lead to further health complications, especially repeated falls. If you suffer any head trauma during a fall, you need to be examined by a medical professional. Your injury may not seem severe to you, but there could be unknown brain damage or bleeding in the brain. With a sensitive neurological condition like progressive supranuclear palsy, you need to be as kind to your brain as possible.
Continue to see how vision might be affected with this condition.
The progression of progressive supranuclear palsy can lead to vision problems. They will begin to develop difficulties controlling the muscles in their eyes, along with their eye movement, which can lead to blurred and unfocused vision. A loss of balance can be an indication of one of the numerous neurological disorders, but eye problems tend to offer the first diagnostic clue pointing to PSP.
Patients with progressive supranuclear palsy might have abnormally slow eye movements. In particular, they tend to experience trouble shifting their gaze up and down. Eyelid movement might also be difficult to control. Eyes may close involuntarily, be challenging to open and be subject to infrequent or prolonged blinking. Individuals may also need to move their entire head to see in a different direction, rather than just moving their eye muscles. One more potential problem is a sudden inability to keep eye contact throughout a conversation, which can make a person seem uninterested or hostile.
Continue reading to learn about symptoms related to speech.
Problems With Speech
Throughout the progression of the disease, progressive supranuclear palsy patients will have increasing problems with speech and language capabilities. Their speech tends to become slurred and slow, with frequent pauses between words. It might be difficult for an individual to swallow both liquids and foods, and they might have to give up difficult-to-swallow foods entirely. Speech might become monotone and facial expressions mask-like.
All of these speech problems match up with the typical progression of Parkinson's disease. Many PSP patients receive an initial misdiagnosis of Parkinson's disease. However, progressive supranuclear palsy has a more rapid onset time than Parkinson's. PSP speech problems tend to present in more severe ways than in Parkinson's.
Continue for the next sign of progressive supranuclear palsy.
Stiff & Awkward Movement
After movement of the eyes becomes difficult, movement of the entire body becomes difficult, and individuals may sit or stand in rigid, awkward positions. Progressive supranuclear palsy patients tend to stand perfectly straight and lean back slightly. There might also be awkward movements of the head and eyes. Monotone speech and mask-like facial expressions are common as the disease progresses.
Damage to the brain cells causes the stiff and awkward movement. In PSP patients, a protein called tau builds up in the brain. When broken down properly, it's a naturally-occurring protein. Progressive supranuclear palsy patients do not break it down correctly, so the tau becomes brain-damaging clumps. When abnormal tau clumps affect a person's central nervous system and muscle control, it leads to widespread full-body weakness and difficulty with movement.
Continue reading to learn what symptom related to memory is important to watch for.
As the disease continues to damage the brain, patients with progressive supranuclear palsy will begin to have an increase in memory difficulties. They may also have difficulty regulating their emotions and judgment. It might be difficult for patients to find words to express themselves or to understand the words being spoken to them. They may forget seemingly simple things, both about the world and those around them.
It's common for the memory problems to be accompanied by emotional volatility. Progressive supranuclear palsy can switch from hysterical laughter to hysterical crying for no reason. They might be increasingly irritable and snappish, and may even have unprompted angry outbursts. Alternatively, it's possible patients will become withdrawn and apathetic as they fail to relate to the world around them.
As with any serious illness, patients with PSP are subject to extreme fatigue and constantly feeling tired all the time. Out of all the symptoms an individual can experience with PSP, one study concluded that eighty percent reported feeling fatigued and experienced difficulty sleeping and sleep disorders. A study was performed on participants with PSP and their sleeping patterns to determine how the severity of the disease affected their sleep. Many individuals with PSP also suffer from severe insomnia and spend on average, two to six hours awake per night.
In the study, among these patients, sleep latency became shorter and the number of times an individual would awaken during the night increased with greater motor impairment, and total time spent sleeping declined as dementia worsened. The findings of the study indicated that in PSP, insomnia is related to the severity of the condition, and sleeping patterns overall worsened as the disease progressed. Insomnia is so incredibly common for PSP patients that it is worse for those with PSP than patients with Parkinson’s disease or Alzheimer’s, and researchers believe it is due to the degenerative changes in brain structures that are responsible for sleep maintenance.
Behavioral & Personality Changes
PSP patients typically have deficits in their cognitive functions and difficulties with most daily activities, but also display notable personality changes such as they become apathetic, depressed, impulsive, and irritable. One study performed on 154 patients showed that more than half displayed signs of behavioral abnormalities, and experienced apathy, depression, and sleeping problems, with approximately one third, showed signs of agitation, irritability, disinhibition or recklessness, and eating problems. Further studies proved that patients with PSP who experienced negative symptoms or emotions, such as apathy, were similar to apathetic patients with dementia of the frontal lobe, with apathy being the most common negative symptom experienced by PSP patients.
Depression is another common symptom for those with PSP but varies with severity amongst patients. In addition to these negative symptoms, about twenty percent of individuals with PSP have exhibited impaired interpersonal functioning, which affected their engagement in activities with family and friends, poor relationships with family members, issues with communication, and impaired emotional expression. Given the prevalence of many of these symptoms, patients in general who experience behavioral changes are expected to see psychiatrists and other mental health professionals regularly to help manage their symptoms and increase their quality of life.
Another common symptom of PSP, as the disease progresses, is photophobia. Photophobia is the experience of discomfort or pain to the eyes due to light exposure or due to the presence of actual physical sensitivity of the eyes. In relation to PSP, a clinical study was performed on 187 patients, which concluded that forty-three percent of these patients showed symptoms of photophobia. Another study showed that photophobia is significantly greater in clinically diagnosed PSP than in corticobasal degeneration (CBD), which is a progressive neurological disorder that is characterized by nerve cell loss and atrophy of multiple parts of the brain. These study results can help doctors differentiate between both of these diseases due to the presence of photophobia.
Dysphagia is an early sign of PSP but can become more of a problem as the disease progresses. Dysphagia is the medical term that describes difficulty swallowing. This symptom includes difficulty starting a swallow, called oropharyngeal dysphagia, and the sensation of food being lodged in the neck or chest called esophageal dysphagia. Some signs of oropharyngeal dysphagia include difficulty controlling food in the mouth, inability to control saliva, difficulty initiating a swallow, coughing, choking, frequent pneumonia, unexplained weight loss, gurgly or wet voice after swallowing, nasal regurgitation. The most common symptom of esophageal dysphagia is the inability to swallow solid foods, and these foods often become stuck before it passes into the stomach. Most patients dealing with PSP will experience any one of these symptoms and difficulty swallowing in general.
One particular study examined the abnormalities patients with PSP experience during the multiple stages of ingestion, and their symptoms included uncoordinated lingual movements, impaired posterior lingual displacement, copious pharyngeal secretions, and absent velar retraction or elevation. PSP patients also had significantly fewer continuous swallows and required a longer duration to complete their swallows than those with healthy controls.
Loss Of Bladder & Bowel Function
In advanced stages of PSP, unfortunately, many patients experience difficulties with controlling their bladder and bowel functions. Constipation and difficulties urinating are quite common, as well as uncontrollable urination, especially at night, as many patients in the advanced stage report having to urinate multiple times throughout the night. Some patients even experience urinary incontinence, which is the unintentional passing of urine, and there are multiple types of urine incontinence, such as stress incontinence, urge incontinence, overflow incontinence, and total incontinence. Researchers have also determined that patients with PSP experience a variety of urinary dysfunctions, such as problems with the bladder storing and withholding urine and issues with expelling urine from the bladder, which leads to either frequent urination or leaking or issues with urinating itself.
Diagnosing a person with progressive supranuclear palsy can be quite difficult, as its symptoms are incredibly similar to other movement disorders, such as Parkinson’s disease, and some of the common symptoms develop in later stages or not at all. There are no specific laboratory tests or imaging that can diagnose this condition. An initial diagnosis is generally based on the individual’s medical history and a physical and neurological exam. Diagnostic scans, such as magnetic resonance imaging, may show shrinkage at the top of the brain stem and degeneration, along with other imaging tests that look at brain activity. Unfortunately, PSP is often misdiagnosed as it is quite rare and mimics many of the symptoms of other neurological conditions. The best way to diagnose PSP is by identifying early gait instability and difficulty moving the eyes, speech and swallowing abnormalities, as well as ruling out Parkinson’s disease and other similar disorders.