PSP and CBD folks –
A great new publication, Frontotemporal Disorders: Information for Patients, Families and Caregivers,has just been released by the National Institute on Aging and NIH. It is free and available by download or in hardcopy via the following link: [Editor’s note: link modified in 2017]
Jennifer Merrilees, RN, from UCSF’s MAC, assisted in the creation of this booklet along with people from Northwestern University and the Association for Frontotemporal Dementias.
I’ve copied below some excerpts that are on terminology, CBS, and PSP. I especially liked the terminology section (“FTD? FTLD? Understanding Terms”) and the section on symptoms (with a breakdown by type of symptom — behavioral, language, emotional, and movement). Of course not all of the symptoms listed occur in each disorder nor in each person with a given disorder.
Frontotemporal Disorders: Information for Patients, Families, and Caregivers
NIA and NIH
Published October 2010
FTD? FTLD? Understanding Terms
One of the challenges shared by patients, families, clinicians, and researchers is confusion about how to classify and label frontotemporal disorders. A diagnosis by one doctor may be called something else by a second, and the same condition or syndrome referred to by another name by a pathologist who examines the brain after death.
For many years, scientists and physicians used the term frontotemporal dementia (FTD) to describe this group of illnesses. After further research, FTD is now understood to be just one of several possible variations and is more precisely called behavioral variant frontotemporal dementia, or bvFTD.
This booklet uses the term frontotemporal disorders to refer to changes in behavior and thinking that are caused by underlying brain diseases collectively called frontotemporal lobar degeneration (FTLD). FTLD is not a single brain disease but rather a family of neurodegenerative diseases, any one of which can cause a frontotemporal disorder (see “Causes,” page 11). Frontotemporal disorders are diagnosed by physicians and psychologists based on a person’s symptoms. FTLD can be identified definitively only by brain autopsy after death.
Types of Frontotemporal Disorders
Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms physicians identify when they examine patients.
• Progressive behavior/personality decline—characterized by changes in personality, behavior, emotions, and judgment (e.g., behavioral variant frontotemporal dementia).
• Progressive language decline—marked by early changes in language ability, including speaking, understanding, reading, and writing (e.g., primary progressive aphasia).
• Progressive motor decline—characterized by various difficulties with physical movement, including shaking, difficulty walking, frequent falls, and poor coordination.
Types of Frontotemporal Disorders
Diagnostic Terms and Main Early Symptoms
Progressive Motor Decline
Corticobasal syndrome (CBS)
• Muscle rigidity
• Difficulty closing buttons, operating simple appliances
• Language or spatial orientation problems
Progressive supranuclear palsy (PSP)
• Progressive problems with balance and walking
• Slow movement, falling, body stiffness
• Restricted eye movements
FTD with parkinsonism
• Movement problems similar to Parkinson’s disease, such as slowed movement and stiffness
• Changes in behavior or language
FTD with amyotrophic lateral sclerosis (FTD-ALS)
• Combination of FTD and ALS (Lou Gehrig’s disease)
• Changes in behavior and/or language
• Muscle weakness, shrinkage, jerking
Two rare neurological disorders associated with FTLD, corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), occur when the nerves attached to muscles malfunction and cause problems with movement. The disorders may affect thinking and language abilities, too.
CBS is caused by corticobasal degeneration—gradual atrophy and loss of nerve cells in specific parts of the brain. This degeneration causes progressive loss of movement and muscle rigidity, typically beginning around age 60. Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with CBS first has language problems or trouble orienting objects in space and later develops movement symptoms.
PSP causes serious problems with balance and walking. People with the disorder typically move slowly, fall, and have body stiffness, especially in the neck and upper body—symptoms similar to those of Parkinson’s disease. A hallmark sign of PSP is trouble with eye movements, such as involuntary closing of the eyelids, difficulty opening and closing the eyes, trouble looking way up and way down, and limited blinking. These symptoms may give the face a fixed stare. Behavior problems can also develop.
Other movement-related frontotemporal disorders include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS).
Frontotemporal dementia with parkinsonism is an inherited disease caused by a genetic mutation. Symptoms include movement problems similar to those of Parkinson’s disease, such as slowed movement, stiffness, and balance problems, and changes in behavior or language.
FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease. Symptoms include the behavioral and/or language changes seen in bvFTD as well as the muscle weakness, shrinking, and jerking seen in ALS. Symptoms of either disease may appear first, with other symptoms developing over time. Mutations in certain genes have been found in some patients with FTD-ALS.
Carol had a tingling sensation and numbness in her upper right arm. Then her arm became stiff. She had to change from cursive handwriting to printing. Carol, 61, told her doctor that she had trouble getting her thoughts out and described her speech as “stumbling.” She had increasing trouble talking but could still understand others. Eventually, she was diagnosed with CBS.
Trouble with walking
For a year and a half, John had trouble walking and fell several times. He also had trouble concentrating. He couldn’t read because the words merged together on the page. John, 73, also seemed less interested in social activities and projects around the house. His wife noticed that he was more irritable than usual and sometimes said uncharacteristically inappropriate things. John’s primary care doctor did several tests, then referred him to a neurologist, who diagnosed PSP.
Symptoms of frontotemporal disorders vary from person to person and from one stage of the disease to the next as different parts of the frontal and temporal lobes are affected. In general, changes in the frontal lobe are associated with behavioral symptoms, while changes in the temporal lobe lead to language and emotional disorders.
Symptoms are often misunderstood. Family members and friends may think that a person is misbehaving, leading to anger and conflict. For example, a person with bvFTD may neglect personal hygiene or start shoplifting. It is important to understand that people with these disorders cannot control their behaviors and other symptoms.
• Problems with executive functioning—Problems with planning and sequencing (thinking through which steps come first, second, third, and so on), prioritizing (doing more important activities first and less important activities last), multitasking (shifting from one activity to another as needed), and self-monitoring and correcting behavior.
• Perseveration—A tendency to repeat the same activity or to say the same word over and over, even when it no longer makes sense.
• Social disinhibition—Acting impulsively without considering how others perceive the behavior. For example, a person might hum at a business meeting or laugh at a funeral.
• Compulsive eating—Gorging on food, especially starchy foods like bread and cookies. People may take food from other people’s plates.
• Utilization behavior—Difficulty resisting impulses to use or touch objects that one can see and reach. For example, a person picks up the telephone receiver while walking past it when the phone is not ringing and the person does not intend to place a call.
• Aphasia—A language disorder in which the ability to use or understand words is impaired but the physical ability to speak properly is normal.
• Dysarthria—A language disorder in which the physical ability to speak properly is impaired (e.g., slurring) but the message is normal.
• Apathy—A lack of interest, drive, or initiative. Apathy is often confused with depression, but people with apathy may
not be sad. They often have trouble starting activities but can participate if others do the planning.
• Emotional changes—Emotions are flat, exaggerated, or improper. Emotions may seem completely disconnected from a situation or are expressed at the wrong times or in the wrong circumstances. For example, a person may laugh at sad news.
• Social-interpersonal changes—Difficulty “reading” social signals, such as facial expressions, and understanding personal relationships. People may lack empathy—the ability to understand how others are feeling—making them seem indifferent, uncaring, or selfish. For example, the person may show no emotional reaction to illnesses or accidents that occur to family members.
• Changes in insight—An inability to understand changes in one’s condition and how they affect others. This lack of awareness is hard for caregivers to deal with because the person may reject efforts to help.
• Dystonia—Abnormal postures of the hands or feet. A limb may be bent stiffly or not used when performing activities that are normally done with two hands.
• Gait disorder—Abnormalities in walking, such as walking with a shuffle, sometimes with frequent falls.
• Tremor—Shakiness, usually of the hands.
• Clumsiness—Dropping of small objects or difficulty manipulating small items like buttons or screws.
Managing Movement Problems
No treatment can slow down or stop frontotemporal-related movement disorders, though medications and physical and occupational therapy may provide modest relief.
For people with corticobasal syndrome (CBS), movement difficulties are sometimes treated with medications for depression, Alzheimer’s disease, or Parkinson’s disease. But these medicines do not often lead to much improvement. Physical and occupational therapy can help a person with CBS move more easily. Speech therapy may delay the worsening of language symptoms.
For people with progressive supranuclear palsy (PSP), sometimes Parkinson’s disease drugs provide temporary relief for slowness, stiffness, and balance problems. Although physical therapy generally does not help, exercises can keep the joints limber, and weighted walking aids—such as a walker with sandbags over the lower front rung—can help maintain balance. Speech, vision, and swallowing difficulties usually do not respond to any drug treatment. Antidepressants have shown modest success. For people with trouble looking up or down, bifocals or special glasses called prisms are sometimes prescribed. …
For any movement disorder caused by FTLD, a team of experts can help patients and their families address difficult medical and caregiving issues. Physicians, nurses, social workers, and physical, occupational, and speech therapists who are familiar with frontotemporal disorders can ensure that people with movement disorders get appropriate medical treatment and that their caregivers can help them live as well as possible.
Caring for a Person with a Frontotemporal Disorder
In addition to managing the medical and day-to-day care of people with frontotemporal disorders, caregivers can face a host of other challenges. These challenges may include changing family relationships, loss of work, poor health, decisions about long-term care, and end-of-life concerns.
People with frontotemporal disorders and their families often must cope with changing relationships, especially as symptoms get worse. …
Frontotemporal disorders disrupt basic work skills, such as organizing, planning, and following through on tasks. Activities that were easy before the illness began might take much longer or become impossible. People lose their jobs because they can no longer perform them. As a result, the caregiver might need to take a second job to make ends meet—or reduce hours or even quit work to provide care and run the household.
Caregiver Health and Support
Caring for someone with a frontotemporal disorder can be very hard, both physically and emotionally. To stay healthy, caregivers can do the following:
• Get regular health care.
• Ask family and friends for help with child care, errands, and other tasks.
• Spend time doing enjoyable activities, away from the demands of caregiving. Arrange for respite care—short-term caregiving services that give the regular caregiver a break—or take the person to an adult day care center, a safe, supervised environment for adults with dementia or other disabilities.
• Join a support group for caregivers of people with frontotemporal disorders. Such groups allow caregivers to learn coping strategies and share feelings with others in the same position.
The organizations listed in the Resources section of this book (starting on page 27) can help with information about caregiver services and support.
For many caregivers, there comes a point when they can no longer take care of the person with a frontotemporal disorder without help. The caregiving demands are simply too great, perhaps requiring around-the-clock care. As the disease progresses, caregivers may want to get home health care services or look for a residential care facility, such as a group home, assisted living facility, or nursing home. The decision to move the person with a frontotemporal disorder to a care facility can be difficult, but it can also give caregivers peace of mind to know that the person is safe
and getting good care.
People with frontotemporal disorders typically live 6 to 8 years with their conditions, sometimes longer, sometimes less. Most people die of problems related to advanced disease. For example, as movement skills decline, a person can have trouble swallowing, leading to aspiration pneumonia, in which food or fluid gets into the lungs and causes infection. People with balance problems may fall and seriously injure themselves.
It is difficult, but important, to plan for the end of life. Legal documents, such as a will and durable powers of attorney for health care and finances, should be created or updated as soon as possible after a diagnosis of bvFTD, PPA, or a related disorder. Early on, many people can understand and participate in legal decisions. But as their illness progresses, it becomes harder to make such decisions.
A physician who knows about frontotemporal disorders can help determine the person’s mental capacity. An attorney who specializes in elder law, disabilities, or estate planning can provide legal advice, prepare documents, and make financial arrangements for the caregiving spouse or partner and dependent children. If necessary, the person’s access to finances can be reduced or eliminated.
National Institute on Aging
National Institute of Neurological Disorders and Stroke
Association for Frontotemporal Dementias
National Aphasia Association
Family Caregiver Alliance
National Academy of Elder Law Attorneys, Inc.
FTD Support Forum
PPA Support Group
Well Spouse Association
Diagnosis, Treatment, and Research Centers [many are listed, including…]