The Neurologist Is In
Monthly insights and advice from a stroke specialist, a movement disorder expert, and a neuromuscular physician.

Wednesday, February 1, 2017

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BY SARAH SONG, MD, MPH

The stroke center where I work just got a mobile stroke unit—one of a handful in the country—and I am so excited. This miniature emergency department on wheels lets us bring stroke care right to your door by ambulance, and helps us reduce the time between the appearance of symptoms and medical care. That, in turn, increases the opportunity of administering intravenous (IV) tissue plasminogen activator (tPA), the only FDA-approved medication to treat acute stroke, which must be used four-and-a-half hours after symptoms start. Some patients also require endovascular therapy, a procedure that removes blood clots using a thin catheter and other devices, such as stents, to restore blood flow to the brain. Early treatment with both IV tPA and endovascular therapy decreases the risk of disability in patients who would benefit from clot removal.

Mobile Magic

Everything that happens in the hospital can happen in this special ambulance, which is staffed with a nurse trained in stroke care, a technician proficient in computed tomography (CT) imaging, and a monitor screen connected to a neurologist, who can beam in remotely from almost anywhere via mobile technologies. After picking up the patient, the ambulance driver parks and levels the vehicle using special feet that emerge from below. While the neurologist examines the patient via video screen—asking him or her to smile, read cards, look left and right, and more—the nurse starts intravenous lines, assesses vitals, and draws blood. Once the CT technician is ready, the patient undergoes a CT scan, which is sent to a waiting radiologist, who interprets it and confers with the neurologist.

The patient is treated within minutes and transferred to a primary stroke center if symptoms are less serious, , or to a comprehensive stroke center, if the patient's symptoms are more severe, or if the patient needs endovascular treatment to remove larger blood clots.

Saving Lives

Early evidence shows that mobile stroke units shorten treatment times in half, with the average patient being treated within 30 minutes. Given that patients are more likely to have a better outcome—to walk, return to work, and become more independent—if they receive IV tPA (and endovascular therapy), it is paramount that stroke centers find new and innovative ways to reach patients with stroke more quickly.

With every minute that passes, 1.9 million brain cells are destroyed during an acute stroke! Although expensive—a mobile unit costs just under $1 million plus another $1 million to operate—it's worth it if it helps save more of those precious minutes.

For more about mobile stroke units and stroke care, go to bit.ly/NN-Telemedicine.​

Dr. Sarah Song is an assistant professor of neurology at Rush University Medical Center in Chicago, IL, and a member of Neurology Now's editorial advisory board.


Friday, December 2, 2016

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BY BORNA BONAKDARPOUR, MD, FAAN

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.

Managing Symptoms

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.

Furthering Research

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.​


Thursday, November 10, 2016

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BY MELISSA ARMSTRONG, MD, MSC, FAAN

One of my patients has spent a lifetime "exercising" her brain. She worked as an elementary school teacher for more than 30 years. Since retiring five years ago, she has stayed mentally active doing daily Sudoku and crossword puzzles, and she babysits her grandchildren twice a week. Ten years into her Parkinson's disease, a time when memory and thinking are commonly affected, she has only mild difficulties multitasking and finding words.

Coincidence? Maybe not. Research suggests that people who stay mentally active may develop memory and thinking problems later in life than those who are not mentally active. More years of education, jobs that stimulate the brain, and intellectually challenging hobbies all build what researchers call "cognitive reserve," which may help the brain cope with changes due to different neurologic diseases.

Mental activity can't prevent brain disease, but it may help people experience fewer memory problems, at least at the beginning. For those who already notice changes in memory and thinking, mental exercise might still be helpful. The analogy is that as physical exercise strengthens muscles, mental exercise may help strengthen memory and thinking.

Researchers are still trying to identify the best types of mental exercise and how—or how much—they help. In the meantime, I encourage my patients to incorporate mental exercise into their daily routine. Here are some brain training tips I share with them:

  1. Stimulate your brain as often as possible. If mental exercise is going to help, it has to be something you do regularly.
  2. Set reasonable goals. If you like puzzles or Sudoku, start with small, easy ones before tackling the Saturday puzzle of the New York Times. Otherwise, you'll get frustrated and give up. Pick activities that make you think, and start at a level that is challenging but doable.
  3. Strengthen your weaknesses. If you have trouble concentrating or doing math or thinking logically, pick exercises such as doing a jigsaw puzzle that force you to work on that weakness. That's how mental exercise can be most helpful, research suggests.
  4. Choose activities you enjoy. If you love reading books, join a book club. If you've always wanted to learn a new language or instrument, sign up for a class. Find your passion and pursue it. You're more likely to stick with it, if you're having fun.
  5. Stay physically active. Mental exercise is not a substitute for physical exercise. Research shows that physical exercise can improve memory and thinking.

Armstrong_Melissa (1).JPGDr. Armstrong is a movement disorders specialist at the University of Florida Health Center for Movement Disorders and Neurorestoration in Gainesville. She is also involved in the American Academy of Neurology's evidence-based guideline program.


Wednesday, November 2, 2016

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BY RAGHAV GOVINDARAJAN, MD

Susan Schneider Williams, wife of the late and beloved Robin Williams, recently joined the board of the American Brain Foundation, in part because of her newfound understanding of her husband's health problems prior to his suicide in 2014. She also published "The Terrorist inside my Husband's Brain," an essay about her experience with Robin's Lewy body dementia, in Neurology (bit.ly/WilliamsEssay). She was also interviewed in a podcast where she discussed her experience in more detail (bit.ly/NN-WilliamsPodcast).

I had the opportunity to interview Susan in April at the Annual Meeting of the American Academy of Neurology in Vancouver, BC, Canada.

She described Robin's struggle with Lewy body dementia, her own journey toward a better understanding of the disease, and her efforts to raise awareness about it, including the fact that the disease affects an estimated 1.4 million Americans and their families. Symptoms such as fluctuating cognition, visual hallucinations, and mood and behavioral changes are caused by the build-up of Lewy bodies—accumulated bits of alpha-synuclein protein inside the nuclei of neurons—in areas of the brain that control behavior, memory, and movement. The disease continues to be widely underdiagnosed.

In excerpts from our conversation, Susan describes what else she has learned since Robin's death.

What were some of the changes you noticed in Robin related to Lewy body dementia?

I vividly remember Robin having a sudden spike of fear and anxiety very different from the normal anxieties and fears we all face. There was a dramatic change in his personality with paranoia and delusions and a repetitive aspect to his behavior. He also seemed depressed and couldn't sleep. Many of his symptoms were attributed to depression or anxiety, and he could never get a clear answer from his doctors.

How did Robin change as the disease progressed?

He had difficulty focusing, and sometimes he seemed confused. After his death, I learned that he told his doctors he was having visual hallucinations. I also noticed tremors in his left hand. His face also lost expression and even his voice weakened, which was devastating for him. This constellation of symptoms made it seem like he was a hypochondriac or that he was depressed, but the symptoms came on suddenly and were more severe and vivid than before.

Research is critical for Lewy body dementia. How are you supporting that? As a member of the board of the American Brain Foundation (americanbrainfoundation.org​), I'm helping to raise awareness and increase funding for finding a cure by speaking to the public and doing interviews about Lewy body dementia and other brain diseases. I am confident that as more people become aware of this disease there will be quicker and more accurate diagnoses (unlike what happened to Robin). Importantly a lot of resources will be invested in research and finding a cure.

What are some helpful thoughts you can share with families?

The symptoms of Lewy body dementia can be very confusing and complex. Many symptoms are psychiatric in nature, and there is a certain level of stigma associated with it that prevents patients and families from opening up. My biggest piece of advice to families is these patients need you. They did not choose this disease. Just because you cannot see physical ailments does not mean they are not struggling. The chemical warfare going on in their brains means they need you to be on their side helping them fight the biggest battle of their lives.

Which movie character Robin played was closest to the man you knew?

I have not seen all of Robin's movies, and each movie reflected a different aspect of his life. But my favorite movie is What Dreams May Come in 1998 (bit.ly/Williams-Movie) in which Robin plays a character named Chris Nielsen who goes to great lengths to be reunited with his wife after they both die. The character's willingness to share and partner with his wife depicts the Robin I knew and the bond we shared as a couple. I am captivated by the depth and beauty of love portrayed in the movie, which is something Robin and I shared deeply.

Dementia Resources

For more information about Lewy body dementia and other types of dementia, consult these organizations.

Alzheimer's Association, alz.org​; 800-272-3900

Lewy Body Dementia Association; lbda.org; 404-935-6444; 800-539-9767

The National Institute of Neurological Disorders and Stroke; bit.ly/DementiaLewyBody

Govindarajan_Raghav[1].jpgDr. Govindarajan is a neurologist at the University of Missouri in Columbia, MO.


Wednesday, October 26, 2016

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BY MELISSA ARMSTRONG, MD, MSC, FAAN

I recently evaluated a patient—I'll call him "John" to protect his privacy—who came to my clinic with his wife. They described three years of memory problems starting around John's 65th birthday, when he retired from his job as an accountant. On any given day he had moments where he was like his "old self" and moments where he was very confused.

Over the last few years, he had slowed down more and had fallen twice in his home, thankfully without getting injured. Six months earlier he started hallucinating, seeing strange children running around his house. He was diagnosed with a urinary tract infection, which was treated at the time, but the hallucinations never went away. A doctor told the couple that John had "dementia" but offered no other explanation.

In their search for a more complete explanation, the couple ended up at my clinic where I took a full history. Based on John's decline in memory and thinking, problems with walking and movement, fluctuating confusion, and hallucinations, I was able to diagnose him with Lewy Body dementia. I also explained that even though Lewy body dementia is the second-most common dementia after Alzheimer's disease dementia it remains poorly recognized. People often experience long delays and many evaluations before they get a diagnosis—and some people are never diagnosed. For example, in 2014, comedian and actor Robin Williams was diagnosed with the disease only after his death.

Important Terminology

Lewy body dementia is an umbrella term that includes two related conditions that can be diagnosed by physicians: dementia with Lewy bodies and Parkinson's disease dementia. These two conditions overlap in terms of symptoms and what is happening in the brain. Both have Lewy bodies, particular protein clumps, in the brain which are seen on autopsy and which give Lewy body dementia its name.

Characteristic Symptoms

Symptoms of dementia with Lewy bodies and Parkinson's disease dementia include difficulties in memory and thinking severe enough to impact day-to-day life, ups and downs in attention and concentration, hallucinations, and physical difficulties such as slowness, stiffness, and trouble walking. In dementia with Lewy bodies, the difficulties with memory and thinking typically start before or at the same time as the movement problems.

In Parkinson's disease dementia, memory and thinking problems often develop many years after the diagnosis of Parkinson's disease.

Lewy body dementia affects more than just movement and thinking. It can affect sleep, behavior, mood, and other aspects of health. The diagnosis is made based on the history, exam, and sometimes other tests (blood tests or brain scans) to make sure there isn't another cause of the problems. Finding the right diagnosis helps patients and families know what to expect.

How LBD Is Treated

Sometimes patients with Lewy body dementia respond particularly well to drugs approved for use in Alzheimer's disease, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne). Only rivastigmine is approved by the US Food and Drug Administration (FDA) for people with Lewy body dementia. These medications may improve thinking to a modest extent; in Lewy body dementia, they can ease symptoms like hallucinations, too. In 2016, the FDA approved pimavanserin, an atypical antipsychotic that does not work through dopamine pathways, to treat hallucinations in patients with Parkinson's disease dementia. This medication has not been formally studied in people with dementia with Lewy bodies. It is important for patients with Lewy body dementia to avoid antipsychotic medications that work through dopamine pathways, when possible, as these drugs can cause worsening of some symptoms.

A Push for More Research

More research is needed to understand the brain changes in Lewy body dementia, to improve recognition and diagnosis, and to find treatments.

If you think that you or a loved one may have unrecognized Lewy body dementia, request a consultation with a neurologist, dementia specialist, or movement disorders specialist.

For more information on Lewy body dementia, see "A Different Kind of Dementia" (April/May 2015, bit.ly/NN-DifferentDementia),  "Dementia 101" (December 2015/January 2016, bit.ly/NN-dementia101), or contact the Lewy Body Dementia Association—a patient organization that provides outreach and support for families affected by LBD as well as educational programs and resources—at lbda.org.

Armstrong_Melissa (1).JPGDr. Armstrong is a movement disorders specialist at the University of Florida Health Center for Movement Disorders and Neurorestoration in Gainesville. She is also involved in the American Academy of Neurology's evidence-based guideline program.