As a dementia specialist who sees many patients with primary progressive aphasia (PPA) and their families, I was immediately interested in reading Where the Light Gets In (Crown Archetype, 2016), a memoir by Kimberly Williams-Paisley about the effect her mother's diagnosis of PPA had on the family. I was struck by Williams-Paisley's candor and frankness about the difficulties of adjusting to her mother's constant shifts in behavior as the disease progressed. Her mother died on November 17, 2016, at age 73, 10 years after her diagnosis. I thought Williams-Paisley's book exactly captured the myriad challenges patients and families face.
The experience of PPA is different for everyone, but it is always a worsening condition. In sharing her family's struggles, Williams-Paisley can help other families prepare for possible changes in the future. Her memoir also reminds us that people with PPA have strengths that can be enriched, enjoyed, and cherished at any stage of the disease. Over time, Williams-Paisley was able to see the light through the darkness of the disease.
Symptoms of Primary Progressive Aphasia
About 55 percent of PPA cases are caused by frontotemporal lobar degeneration (FTLD), a condition marked by the shrinking of frontal and temporal lobes of the brain; about 45 percent are caused by Alzheimer's disease. People with FTLD typically have behavioral problems and trouble with executive function (the ability to perform a sequence of steps to accomplish a task). People with Alzheimer's disease usually have noticeable problems with memory. With PPA, degenerative diseases such as FTLD or Alzheimer's disease start by affecting the language regions of the brain causing aphasia.
Aphasia, or difficulty speaking or understanding words and sentences, can also be caused by a stroke, traumatic brain injury, brain tumors, or infections. PPA starts with trouble finding words or following conversations, then progresses to an inability to communicate or understand conversations. Eventually, other areas of brain function are affected, causing problems with memory and difficulty handling daily activities unrelated to language.
As Williams-Paisley describes in her book, her mother's problems first became evident when she had difficulty reading verses from the Bible during Williams-Paisley's wedding. As months went by, her mother had more trouble finding her words. The disease eventually affected her ability to drive, and she needed more help with activities of daily living.
How to Diagnose
To assess for PPA, I conduct a series of tests to evaluate language functions. For example, I ask the patient to describe a picture, tell me the names of objects, or to repeat words and sentences. I also evaluate whether he or she can comprehend words and sentences, or read and write. In addition, I evaluate memory, attention, problem solving, and visual processing. Patients may undergo imaging tests to see if any irregularities show up in the brain in areas associated with language and cognition or to check for certain spinal fluid proteins, which may indicate whether someone has Alzheimer's disease or not.
At the moment, there is no cure for PPA. Instead, neurologists treat symptoms and offer strategies for managing communication and behavioral problems. If the underlying cause is Alzheimer's disease, I might prescribe medications approved for use with Alzheimer's disease, which may slow progression. If the cause is FTLD, I try to manage the symptoms through speech or language therapy, since no approved drug exists to treat underlying FTLD. In both FTLD and Alzheimer's disease, if psychiatric problems develop, I may prescribe antidepressants or antipsychotics along with recommendations for family members and care partners for how to adjust to behavioral changes.
Addressing Speech Problems
Since trouble speaking is the first symptom in PPA, sessions with a speech pathologist are crucial—either in person or via the internet or telehealth (treatment sessions offered through the internet) if a local speech therapist with PPA expertise isn't available. Evidence suggests that speech therapy interventions can stabilize symptoms for a while. When the ability to speak worsens, the speech pathologist can update or modify exercises accordingly. Check with Northwestern University Cognitive Neurology Center (brain.northwestern.edu) and the aphasia laboratory at the University of Texas, Austin (moody.utexas.edu/aphasialab), for specialized treatment programs, as well as the Association for Frontotemporal Dementia (aftd.org) or the Alzheimer's Association (alz.org) for more information.
Maintaining Quality of Life
Physical exercise and social engagement and enrichment may also help slow the decline of PPA. Art or music therapy, for example, may reveal a new interest in or affinity for art or music, which can be an important outlet for emotional expression as the ability to communicate with words declines. Art and music therapy sessions, either one-on-one or in groups, are available through specialized centers.
Supporting the Family
Degenerative diseases are tough for families, especially spouses and adult children or other members closely involved in caregiving. As PPA progresses, families will need help from knowledgeable care partners or assisted living facilities where experts can manage psychiatric symptoms, if they develop. Later in the course of the disease, I may consult with family members about palliative or hospice care, if necessary.
The search for a cure or better treatment for degenerative diseases continues, with the collaboration of affected individuals, their family members, primary care physicians, neurologists, and clinician-scientists. Active participation in clinical trials is a vital step toward this goal.
Through her book and speaking about PPA, Williams-Paisley helped raise awareness of the condition, which continues to be underdiagnosed in minority and underserved communities. The fight against this disease is possible only if patients and family members collaborate with health care professionals and investigators to better understand the underlying causes and find an effective treatment.
To read our feature on Kimberly Williams-Paisley and her mother, go to bit.ly/NN-ShiningALight.
Dr. Bonakdarpour, a specialist in primary progressive aphasia, is assistant professor of neurology at Cognitive Neurology and Alzheimer Disease Center, Northwestern University Feinberg School of Medicine in Chicago.