Lewy body dementia strikes hockey giant Stan Mikita

Stan Mikita

Chicago Blackhawks legend Stan Mikita

On Friday, Jan. 30, a family spokesman released a statement announcing that the legendary Blackhawks hockey player Stan Mikita, “is facing some serious health issues.” The statement added: “Stan has been diagnosed with suspected Lewy body dementia, a progressive disease, and is currently under the care of compassionate and understanding care givers.”

Mikita, 74, spent his entire 22-year professional career with the Hawks. He is the franchise’s all-time leading scorer. He was honored as the NHL’s most valuable player in 1967 and 1968, and as the league’s leading scorer in 1964, 1965, 1967 and 1968.

Science Life spoke with James Mastrianni, MD, PhD, Associate Professor of Neurology and Director of the Center for Comprehensive Care and Research on Memory Disorders at the University of Chicago Medicine about this little known but surprisingly common disease.

When we hear about dementia in someone in his 70s, most of us think of Alzheimer’s disease. What’s the difference between Alzheimer’s and Lewy body dementia?

James Mastrianni, MD, PhD

Lewy body dementia is a different form of dementia than Alzheimer’s disease. It causes similar problems, but it has some distinguishing features that differ from Alzheimer’s. Memory problems occur with Lewy Body Dementia, but these may be somewhat less than what is seen in Alzheimer’s disease and there are other, more prominent features, that are not typical in Alzheimer’s disease. Parkinsonian features are common—not so much the tremor that you see in a Parkinson’s patient—but stiffness and rigidity of muscles, slowness of movement, smaller steps, and sometimes a reduction of facial expression.

Another characteristic feature is hallucinations. Patients with Lewy Body Dementia typically develop what are termed “benign hallucinations”, that are commonly of non-threatening visions of children or animals. Some patients will actually interact with them; for instance putting down food for a dog, or giving a toy to a child, or have conversations with them. In early stages of disease, patients often recognize that they are hallucinations and not real, but despite this, they can’t resist them.

The third characteristic is day-to-day fluctuations in thinking. One day they might be very foggy and can’t concentrate or focus on things, and the next day they may be quite sharp.

How common is this illness?

It’s the second most common form of degenerative dementia, right behind Alzheimer’s disease. It affects an estimated 1.3 million individuals and their families in the United States, according to the Lewy Body Dementia Association, which stresses that many medical professionals still are not familiar with LBD. About 10 to 25 percent of patients with dementia will develop Lewy Body Dementia.

Does this disease affect older people, like Alzheimer’s does?

The statistics are similar to Alzheimer’s disease as far as gender and age at onset. Although they are both insidious-onset diseases, because they tend to “sneak up” on people, the abrupt onset of hallucinations or unexplained falling might be a sudden change that the family members recognize.

How is it treated?

Treatment is similar to Alzheimer’s disease. We treat the memory disorder with a class of drugs that increases the level of acetylcholine, a neurotransmitter in the brain that is reduced in dementia, especially Alzheimer’s disease. Because patients with Lewy Body Dementia also have symptoms similar to those with Parkinson’s disease, they are sometimes treated with anti-Parkinson’s medications. However, these can worsen the hallucinations, so they are used cautiously. If the hallucinations do become a problem, we might treat with newer forms of antipsychotic medications, but again, we minimize their use because of potential side effects.

One seldom hears about LBD. Is this a diagnosis that is often missed?

It can often be misdiagnosed. Because Alzheimer’s disease is so common in the elderly, this is the diagnosis most often applied to patients with dementia. Sometimes, the clinical diagnosis of LBD is not so obvious, a feature that may be explained by the fact that the pathology of Alzheimer’s disease and LBD coexist in up to 50 percent of cases. Pathology of the brain is used to make the definite diagnosis.

Hockey is a contact sport. Players get hit in the head. In fact, Mikita was a pioneer in bringing helmets into the game. Could this have been caused or hastened by head injuries or multiple concussions during his time as a professional athlete?

People who have had head injuries are at higher risk for dementia later in life. Although this association has been linked primarily to Alzheimer’s disease, it is well known that boxers are at higher risk for developing a syndrome historically known as dementia pugilistica, and recently relabeled as Chronic Traumatic Encephalopathy (CTE). This condition, which is currently under intense study, is often associated with Parkinson’s-like features, especially in later stages. The controversy with CTE is whether all contact sports, including hockey, enhances its risk.

Because it involves movement abnormalities as well as memory, is LBD more serious than Alzheimer’s?

They are both serious neurodegenerative diseases, with no cure. However, when thinking about a patient with LBD, you can imagine a person suffering from both Alzheimer’s disease and Parkinson’s disease simultaneously. Alzheimer’s disease is mainly about memory and behavior; it is not typically accompanied by impaired movement, whereas LBD is. Thus, the disability associated with Lewy Body Dementia may develop faster than in a patient with Alzheimer’s disease. Its duration, from onset to end stage, is similar to other neurodegenerative dementias, typically 5 to 8 years, but extremes at either end of that range are common.

Is there much research underway in Lewy body dementia?

Researchers for any disease always say there’s not enough and they are usually correct. A major focus of our laboratory has been to understand this family of diseases better and to use that knowledge to develop more effective strategies to diagnose and treat them. That takes time and funding. As you know, funding resources for the past several years have not grown with the need, so private donations have become much more critical. Without a lot of treatment options currently available, we’ve tended to focus on quality-of-life issues in our management of patients. That’s an important aspect of our current research program and it can make a difference in the day-to-day care and well being of patients and their families. But we have a lot to learn about this disease and better approaches to treatment are desperately needed.

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