In our other posts, we’ve discussed Alzheimer’s disease, the most common form of dementia, and vascular dementia, the second most common. This post focuses on some of the less common forms of dementia. Rarer dementias include Lewy body dementia (LBD), normal pressure hydrocephalus (NPH), and frontotemporal dementia (FTD or FTLD). Other rare types are multiple system atrophy (MSA) and corticobasal degeneration (CBD).
Rare Forms of Dementia: Motor and Autonomic Symptoms
When we think of dementia and its first signs, most of us think of cognitive, thinking, problems. Unlike Alzheimer’s and vascular dementia, the first signs of some rare forms of dementia are often motor symptoms, autonomic symptoms, or significant behavior changes.
Motor symptoms: Instead of the first signs being the classic brain slips we all think of, some dementias begin with motor symptoms, which can include tremors, balance issues, and challenges walking.
Autonomic symptoms: Other early signs might involve changes in the autonomic nervous system. This system controls body functions like heart rate, respiration, digestion, and bladder control.
Behavior/personality symptoms: For certain dementias, like behavioral-variant frontotemporal dementia, the first signs might be significant personality and behavior changes, such as becoming much less empathic, beginning to use socially inappropriate humor or other behaviors, or making painful decisions with severe consequences that are out of character for someone.
Lewy Body Dementia
Lewy body dementia is closely related to Parkinson’s disease. Classic Parkinson’s symptoms include tremors, stiffness, shuffling steps, reduced facial expression, and changes in vocal tone. Lewy body dementia shares these symptoms but also can include:
- Fluctuations in Alertness: A person might be lucid and clear one moment and severely confused the next.
- Visual Hallucinations: These hallucinations are often highly vivid and complex, and look as real as day to the person experiencing them. They can be distressing. A member of the Michael J. Fox Foundation shared some of her husband’s hallucinations if you’d like to read more.
- REM Sleep Behavior Disorder: This disorder involves acting out dreams due to a lack of normal muscle paralysis during REM sleep, often leading to injuries.
Lewy body dementia is also associated with changes in autonomic functions like heart rate and digestion.
Normal Pressure Hydrocephalus
Normal pressure hydrocephalus (NPH) usually starts with balance and walking challenges. Patients might develop a shuffling, “duck waddle” and have difficulty initiating steps or appearing frozen in place. They often also experience urinary incontinence. They’ll have a strong urge to urinate and then immediately go.
Other cognitive symptoms of NPH include:
- Decreased Attention: Difficulty with focus.
- Slowed Processing Speed: Slower thinking and problem-solving.
- Memory Issues: Short-term memory problems.
- Executive Functioning: Difficulty planning and organizing.
Frontal Temporal Dementia
Frontotemporal dementia (FTD or FTLD) is another less common type and is covered in more detail in this post due to its complexity. FTD often involves big changes in personality and behavior and language difficulties.
Need for Support
Caregivers and families of patients with rare dementias face unique challenges. Here are some important things to keep in mind:
Delayed Diagnosis
Diagnosing these rare types of dementia can take years and usually involves many specialists such as neurologists, gerontologists, and neuropsychologists.
Rapidly Changing Symptoms
Symptoms can change quickly. This can make it difficult for families to adapt.
Emotional Burden
The ups and downs of symptoms and large shifts in behavior can be exhausting and frustrating for caregivers.
Recommendations for Caregivers
Support groups and professional education are crucial for both patients and caregivers. Groups such as the Normal Pressure Hydrocephalus Society, the Association for Frontotemporal Degeneration, and the Lewy Body Dementia Association offer valuable resources and support. Connecting with a group can provide:
- Emotional Support: Sharing experiences with others who going through the same thing can be comforting. No one going through these dementias should do it alone, and no one needs to.
- Practical Advice: Learn from people who’ve been there how to manage symptoms and daily tasks.
- Educational Resources: Gain a better understanding of the disease and the way it progresses.
Rare Forms of Dementia
Early recognition, proper diagnosis, and connecting with support groups can make a big difference in the quality of life for everyone involved.
If you or a loved one is concerned about any form of dementia, including these rare types, contact Idaho Neuropsychology. We are here to provide expert guidance and compassionate support.