ALS has a wide range of associated symptoms and most of them are physical. They include the weakness of muscle groups, muscle twitching (fasciculation), overall reduced mobility as well as difficulty swallowing, speaking and breathing.
But for the most part, over the last few decades, it has been understood that the disease does not impair cognitive ability.
This was amply demonstrated by the life of Stephen Hawking, who struggled with the disease for 55 years before his recent death, but maintained his mental acuity and is considered as one of the most respected scientific figures in history.
Recent research, however, suggests that ALS may actually increase the likelihood of cognitive impairment and conditions such as dementia.
In the past, the development of dementia and ALS were considered separate entities. The fact that ALS occurs in many people later in life, as does dementia, meant that it was often assumed that there was no link between the two. Some people would be diagnosed with ALS. More people would be diagnosed with dementia. Some people would be diagnosed with both.
Recent studies have shown that as many as 50% of people diagnosed with ALS will demonstrate mild cognitive impairment. A further 20% of these developed what is considered full dementia. When associated with ALS, the type of dementia we see is invariably Frontal Temporal Dementia (FTD) syndrome.
Dementia is a catch-all description used when someone’s thinking or behavior is impaired and has a direct impact on their ability to conduct their life. For example, it might impair their ability to drive. They may get lost walking in a location that was previously familiar. They might forget how to dress or when to cook food or eat.
FTD is a specific type of dementia that results in the degeneration of the frontal and temporal lobe and often presents with problems with language and behavior. Symptoms can include poor decision making, loss of empathy, and a lack of inhibition. There may also be speech difficulties and sudden mood changes.
The clinical presentation of ALS often makes it difficult to conduct accurate diagnosis and measure cognitive impairment, especially in the latter stages of the disease. Because of this, diagnosis of dementia requires a slightly different mechanism than with other patients who do not have ALS.
Someone may have difficulty speaking because of damage to the nerves serving the vocal cords, for example, which can make assessment difficult.
Early evidence of FTD, however, may be exhibited by a lowering of social and personal conduct, emotional response changes and a loss of the ability to express insights. Screening tools that can be used include word generation tests and neurobehavioral questionnaires.
Caregivers and family members will probably be the first to notice if there are changes in behavior, though this is not always the case.
When an individual is trying to adapt to living with a disease such as ALS, having to also cope with a condition like dementia will of course present even more challenges.
How this issue is communicated to the individual can be crucial and the support provided will play a significant role. Caregivers will most certainly want to know what their next step is and may even have to face the potential of taking out power of attorney when it comes to making decisions for their loved one.
There are a range of tools that can be used to manage dementia, including:
There may be a range of different circumstances that occur which require careful monitoring by caregivers and no one individual is the same as another. If they are uncertain on their feet and have difficulty walking, for example, poor decision making because of FTD may mean they either walk too far or go off in the wrong direction.
Simple activities such as eating can become problematic, for instance, if the individual tries to put too much food in their mouth at one time and has difficulty swallowing.
Management of cognitive and behavioral impairment as a result of ALS can be highly challenging and requires caregivers to be informed and supported as much as possible.