In Parts Two and Three of this series we looked at different types of dementia: Alzheimer’s Disease, Vascular Dementia, Lewy Body Dementia, Parkinson’s Disease and Frontotemporal Dementia (FTD) and discussed their etiology (cause), characteristics and symptoms. In this Post I would like to look at three other types. Progressive Supranuclear Palsy, Corticobasal Degeneration and Creutzfeldt-Jakob Disease. All of these are considerably rarer forms of dementia than those previously discussed.
Progressive Supranuclear Palsy
Progressive supranuclear palsy (PSP) is a form of dementia that is characterized by problems with eye movements. Generally the problems begin with difficulty moving the eyes up and/or down (vertical gaze palsy). Since difficulty moving the eyes upward can sometimes happen in normal aging, problems with downward eye movements are the key in PSP. Other key symptoms of PSP include falls backwards, balance problems, slow movements, rigid muscles, irritability, apathy, social withdrawal and depression.
The person may also have certain “frontal lobe signs” such as, a grasp reflex and utilization behavior (the need to use an object once you see it) and perseveration. In psychological terms perseveration is the repetition of a particular response, such as a word, phrase, or gesture, despite the absence or cessation of a stimulus. Symptoms include “lacking ability to transition or switch ideas appropriately with the social context, as evidenced by the repetition of words or gestures after they have ceased to be socially relevant or appropriate,” or the “act or task of doing so,”[ People with PSP often have progressive difficulty eating and swallowing, and eventually with talking as well. Because of the rigidity and slow movements, PSP is sometimes misdiagnosed as Parkinson’s disease. On scans of the brain, the midbrain of people with PSP is generally shrunken (atrophied), but there are no other common brain abnormalities visible on images of the person’s brain.
Corticobasal degeneration is a rare form of dementia that is characterized by many different types of neurological problems that get progressively worse over time. This is because the disease affects the brain in many different places, but at different rates. One common sign is difficulty with using only one limb. One symptom that is extremely rare in any condition other than corticobasal degeneration is the “alien limb.” The alien limb is a limb of the person that seems to have a mind of its own, it moves without control of the person’s brain.
Other common symptoms include involuntary jerky movements of one or more limbs (myoclonus), symptoms that are different in different limbs (asymmetric), difficulty with speech that is due to not being able to move the mouth muscles in a coordinated way, numbness and tingling of the limbs and neglecting one side of the person’s vision or senses. In neglect, a person will ignore the opposite side of the body than the one that has the problem. For example, a person may not feel pain on one side, or may only draw half of a picture when asked. In addition, the person’s affected limbs may be rigid or have muscle contractions causing strange repetitive movements (dystonia). The area of the brain most often affected in corticobasal degeneration is the posterior frontal lobe and parietal lobe. Still, many other parts of the brain can be affected.
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 300 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements (myoclonus), blindness, weakness of extremities, and coma may occur. The brain develops holes and the texture changes to resemble that of a kitchen sponge. As such CJD is at times called a human form of mad cow disease (bovine spongiform encephalopathy or BSE). Pneumonia and other infections often occur in these individuals and can lead to death.
CJD is caused by an agent called a “prion”. Prions are misfolded proteins that replicate by converting their properly folded counterparts, in their host, to the same misfolded structure they possess. CJD causes the brain tissue to degenerate rapidly, and the disease destroys the brain. Most victims die six months after initial symptoms appear, often of pneumonia due to impaired coughing reflexes. About 15% of patients survive for two or more years.
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