An Overview of CBD and PSP

 As the Baby-Boom generation ages, the number of people with brain disease will increase substantially.  This will include corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), as well as  Parkinson’s disease, Alzheimer’s disease, and other related diseases.  Some scientists believe CBD is a  variant of PSP.

 Both CBD and PSP are devastating neurodegenerative diseases that ultimately prove fatal. New research  has shown that there may be a significant genetic component to these diseases. To develop methods for  prevention and therapy, it is essential to understand this genetic component.

 Approximately 20,000 people in the United States have PSP and a smaller number have CBD. CBD and  PSP lead to profound disability and eventual death by impairing movement, balance, vision, speech,  swallowing, and some aspects of thinking and decision making. Persons with CBD and PSP may also  exhibit alterations of mood and behavior. Their spouses and other family caregivers must devote  enormous amounts of time, expense, and emotional energy to their care.

  What is Corticobasal Degeneration (CBD)? • CBD is a neurodegenerative brain disease in which parts of the  brain deteriorate. Several regions of the brain degenerate in CBD. The cortex, or outer layer of the brain, is  severely affected, especially the fronto-parietal regions, located near the centertop of the head. Other deeper  brain regions are also affected, including parts of the basal ganglia, hence the name “corticobasal”  degeneration.

  What is Progressive Supranuclear Palsy (PSP)? • PSP is a neurodegenerative brain disease that has no  known  cause, treatment or cure. It affects nerve cells that control walking, balance, mobility, vision, speech, and  swallowing. Symptoms begin, on average, when an individual is in the early 60s, but may start as early as in  the 40s. But PSP has no known geographical, occupational or racial preference.

  What are the symptoms of CBD? • Stiffness, shakiness, jerkiness, slowness, and clumsiness, in either the  upper or lower extremities. • Other initial symptoms may include dysphagia (difficulty with speech generation),  dysarthria (difficulty with articulation), and difficulty controlling the muscles of the face and mouth. • Symptoms  usually begin on one side of the body, and spread gradually to the other. Some patients may have memory or  behavioral problems as the earliest or presenting symptoms.

  What are the causes of CBD and PSP? • Both are caused by a gradual deterioration and death of brain cells in  an important place in the base of the brain. • The cause of CBD and PSP is unknown. There is currently no  strong evidence to suggest that they are inherited diseases and no other risk factors, such as toxins or  infections, have been identified to date. • Studies of brain tissue of individuals with CBD and PSP show certain  characteristic cell changes. These changes, involving a brain protein called tau, have provided researchers initial  clues in their search for the causes of CBD and PSP. • Both CBD and PSP are progressive diseases, meaning the  symptoms worsen over time.

  How are CBD and PSP diagnosed? • Early in the course of the disease, it is often difficult to distinguish CBD  and PSP from similar neurodegenerative diseases. Diagnosis of CBD and PSP involves a careful neurological  exam, combined with one or more types of laboratory evaluations. Electrophysiological studies, including an  EEG,  may show changes in brain function over time that are consistent with the neurodegeneration. CT or MRI  scans can also be used in this way, providing images of asymmetric atrophy (in CBD) of the fronto-parietal  regions of the brain’s cortex, the regions most frequently involved in the disease.

  How are PSP and CBC treated? • Unfortunately, there are no drugs or other therapies that can slow the  progress of the diseases, although a few may offer symptomatic relief. Tremor may be controlled somewhat with  certain medications. Levodopa and other dopaminergic drugs used in Parkinson’s disease are rarely beneficial,  but may help some patients. • Physical therapy exercises may be useful to maintain range of motion of stiff  joints. This may prevent pain and contracture, and help maintain mobility. Occupational therapy may be used to  design adaptive equipment that supports the activities of daily living, thus helping to maintain more functional  independence. Speech therapy is used to improve articulation and volume.

  What is the usual course of CBD and PSP? • Persons with PSP or CBD will usually become immobile due to  rigidity within five years of symptom onset, and may require a gastrostomy (PEG) tube for feeding. Most often,  within ten years of onset, pneumonia may lead to life-threatening complications.

  Support for patients and families: Support groups or support networks provide great value to CBD and PSP  patients and their loved ones. Patients and caregivers can exchange ideas on ways to cope with the physical  and psychological limitations of CBD and PSP and can learn about problems and treatments from healthcare  professionals such as physical therapists, speech and language pathologists, and occupational therapists.

  Further information about CBD and PSP is available at: CurePSP Executive Plaza III 11350 Hunt Valley,               Maryland, 21031 McCormick Road, Suite 906

  Telephone  410-785-7004 Toll-Free  800-457-4777 Canada 866-457-4777

  www.curepsp.org