My Beautiful Mother

This is a picture of my Mother taken during her 50th anniversary trip with my Father. She is beautiful, certainly to me. It was also a week or so after she was diagnosed with Corticobasal ganglionic degeneration also known as CBGD or CBD. In the photo she appears to be sitting rather coyly left arm and leg tipped up but she is not a coy person. She is holding her arm and leg in the only manner she could at the time to hold herself upright.

I use the term diagnosed very loosely, the only way to accurately diagnose CBGD is during an autopsy. In the mean time if she does not have any neurodegenerative disease that has an actual test for diagnosis then she has CBGD, unless she has PSP or a couple of other similar diseases, none of which have possible treatments.

Basically the brain stops talking to the rest of your body. Actions that were done autonomously in the past such as taking a step or swallowing must be done with great concentration. Requiring so much of the brain to do actions that were previously automatic causes difficulty in concentration which can appear as a mental dementia. Mental dementia is not a typical outcome of this disease which sounds great on teh surface but in the long run it is sad because a day may come when she is an active mind in a box with no movement or communication skills.

Although she works very hard and had until recently gone to the gym with my Father's help to try to keep atrophy from setting in the months since this photo was taken (April) her abilities have degenerated significantly.

She uses a walker around the house now and does not go out except on their back deck where she tries to continue her excersizes and physical therapy. The falls she has taken due to her inability to balance and move her limbs has caused both a torn rotator cuff and frozen shoulder. She is starting to get more movement in her left arm although the disease is starting to affect it in the same way as her leg (so far her left side is most affected) so the repair of the shoulder will mean slightly less than it would have a few months ago. We are planning to get her a motorized chair so that she can once again join us at ball games and picnics. Right now walking far with her walker is too tiring for more than around the house. We have installed a stairlift which made her happy as she can get down stairs to do her own laundry. The thing she hates most about this disease is her loss of independence.

I teased her about finding her on the street when the chair got here going down the road to the store and she said, "Don't think I won't!"

What is Corticobasal Degeneration?

Corticobasal degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body (unilateral), but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement), disequilibrium (impaired balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing) may also occur. An individual with corticobasal degeneration eventually becomes unable to walk.
Is there any treatment?
There is no treatment available to slow the course of corticobasal degeneration, and the symptoms of the disease are generally resistant to therapy. Drugs used to treat Parkinson disease-type symptoms do not produce any significant or sustained improvement. Clonazepam may help the myoclonus. Occupational, physical, and speech therapy can help in managing disability.
What is the prognosis?
Corticobasal degeneration usually progresses slowly over the course of 6 to 8 years. Death is generally caused by pneumonia or other complications of severe debility such as sepsis or pulmonary embolism.
What research is being done?
The NINDS supports and conducts research studies on degenerative disorders such as corticobasal degeneration. The goals of these studies are to increase scientific understanding of these disorders and to find ways to prevent, treat, and cure them.