Overview

Other Dementia Disorders

Lewy Body Dementia

Lewy Body Dementia (LBD) is a disease that is related to Parkinson Disease, but has significant involvement of memory and thinking as well.

Symptoms
Like Parkinson Disease, people with LBD get slowed movements, stooped unsteady shuffling gait, loss of facial expression, and tendency to fall. Additionally, people with LBD also develop significant changes to memory and thinking. Commonly, they will have major fluctuations in their attention and awareness, which can last for significant periods of the day, or can be momentary. This may manifest as staring spells, spending most of the day sitting or sleeping, or difficulty following events. Oftentimes, the types of thinking changes will look like psychiatric disease like major depression, odd false beliefs (delusions) or seeing things that are not there (visual hallucinations).

Progression
Over time, both the movement and cognitive symptoms get worse. These symptoms tend to develop slowly, but somewhat quicker than in dementia of the Alzheimer type. Rarely, the symptoms may develop extremely rapidly.

Late in the course, people may develop problems with injuries from falls, immobility, or swallowing problems.

Diagnosis and Treatment
How it’s diagnosed
LBD is diagnosed based on the signs and symptoms above by a qualified doctor. Oftentimes, an MRI and basic bloodwork is done to make sure that there are no other explanations for the symptoms.

Medications
The movement problems in LBD can be treated with Parkinson Disease medication, such as carbidopa-levodopa. Depression and motivation problems may respond to antidepressants. Antipsychotic medications such as quetiapine can help behavior, psychiatric problems such as delusions and hallucinations, and with agitation, but can also worsen movements, so need to be used with caution. Alzheimer’s medications such as donepezil seem to help with the fluctuations in many with LBD.

Non-pharmacological interventions
Supportive treatment for problems with behavior, swallowing, speech and communication, falls and mobility are available. Many people with LBD benefit immensely from occupational therapy, physical therapy, and speech therapy.  

Corticobasal Degeneration (CBD) & Progressive Supranuclear Palsy (PSP)

Corticobasal Degeneration (CBD)  and progressive supranuclear palsy (PSP) are Parkinson’s-plus syndrome, meaning that they are diseases that share some of the symptoms of Parkinson Disease such as slowing of movements, stiffness, tremors, falls, and shuffling of the feet. They may both also cause changes in memory and thinking.

Symptoms
In addition to these symptoms, people with PSP also develop problems moving their eyes, called supranuclear palsy, a wide-eyed appearance, and difficulty swallowing. Unlike Parkinson Disease, people often fall backward instead of forward. They may also develop severe stiffness in the neck.

There are several variations on the name of CBD, such as corticobasal syndrome or disease, and corticobasal ganglionic degeneration. It is named after the parts of the brain that are damaged, the cortex, which is the outer part of the brain, and the basal ganglia, which are deep within the brain. Like Parkinson Disease, slowing of movements, stiffness, tremors, falls, and shuffling of the feet can be seen. Problems with movements occur on one side of the body, such as stiffness, shaking, or loss of control. People with CBD may be unable to get their arm to do what they want even if they know how. Sometimes, the arm on that side might move on its own, called alien limb syndrome.

Not all people with PSP and CBD develop problems with memory, thinking, behavior or language. However, in some people, these problems do arise. In fact, sometimes these problems are the first symptoms, and the more classic symptoms listed above develop later.

People with PSP can develop a loss of motivation. They may lose interest in their everyday activities. Problems may arise with attention and concentration. They may lose the ability to control their mouth to speak, called apraxia of speech, or progressive slurring of speech, called dysarthria.

People with CBD can develop loss of inhibition. They may act or speak rudely or crudely, or seem to lose empathy for others. They may also have difficulty with attention and concentration. A type of language difficulty where understanding grammar and speaking in full sentences can develop, or can be the first symptom.

Progression
All the symptoms of PSP and CBD get worse as the diseases progress. Eventually, people may become completely bedbound and unable to safely walk. Swallowing problems may lead to choking on food or getting food in the lungs, leading to pneumonia. On average, people with these diseases die within 5 years of diagnosis. Death is usually from complications of falls or swallowing problems.

Diagnosis and Treatment
How it’s diagnosed
CBD and PSP are diagnosed based on the signs and symptoms above by a qualified doctor. Oftentimes, an MRI and basic bloodwork is done to make sure that there are no other explanations for the symptoms.

Medications
There are currently no drugs that are proven to reverse the damage in either PSP or CBD. However, some medications can be helpful for symptom control in these conditions. Some people have improvement of their movements with Parkinson Disease medication, such as carbidopa-levodopa, but in general, they are less effective than in Parkinson Disease. For people with severe stiffness, muscle relaxants and Botox may be useful. Depression and motivation problems may respond to antidepressants. In severe disease, antipsychotic medications can help treat agitation, but should not be used except when absolutely needed.

Non-pharmacological interventions
Supportive treatment for problems with behavior, swallowing, speech and communication, falls and mobility are available. Many people with PSP or CBD benefit immensely from occupational therapy, physical therapy, and speech therapy.

Research
Currently, a clinical trial is underway to see if a new medication called davunetide will be useful in treatment of PSP. We do not yet know what the results will be.

Posterior Cortical Atrophy (PCA)

Posterior Cortical Atrophy (PCA) is a rare disorder where people lose the ability to interpret what they are looking at.  It is also called Progressive Visuospatial Dysfunction.

Signs and Symptoms
People with PCA slowly develop difficulty with visual tasks. They may complain that their vision is blurry, but does not get better with new glasses. Common symptoms include:
• Having difficulty with tasks like telling time or reading
• Trouble finding things on shelves
• Difficulty driving within the lane
• Running into door frames
• Getting lost, even indoors
• Thinking that items are popping in and out of view
• Difficulty recognizing what or who one is looking at

Progression
When these symptoms progress, some people develop difficulty with attention and concentration and difficulty using objects correctly even if they know how. Others may develop memory loss or other cognitive difficulties. Depression is common as well.

Diagnosis and Treatment
How it’s diagnosed
PCA is diagnosed based on the signs and symptoms above by a qualified doctor. Oftentimes, an MRI and basic bloodwork is done to make sure that there are no other explanations for the symptoms. The MRI may also show atrophy (shrinkage) of the parts of the brain near the back of the head, called the parietal and occipital lobes.

Medications
There are no medications for PCA, but sometimes Alzheimer’s medications can be tried. Depression related to PCA can also be treated with medications.

Non-pharmacological interventions
People with PCA and their caregivers can often use strategies to help get around problems with visuospatial processing. For instance, items can be color coded for easier identification. Large text may actually be harder to read, so small labels and print may be more useful. Arranging things neatly and in the same place at all times is helpful.

Occupational therapists can help minimize risk of tripping or running into things in the home. Additionally, resources for people with low vision or blindness might be useful despite the problem not being with the eyes in this case.
 

Vascular Dementia (VaD)

Vascular Dementia (VaD) is the term for significant change in cognition (memory and thinking) and behavior from damage to the brain from vascular disease – problems with the bloodflow to the brain. It is one of the most common forms of dementia.

Symptoms
The signs and symptoms of VaD vary depending on where the brain is damaged. A common pattern is when narrowing and stiffening of the smallest vessels in the brain cause damage to the deep parts of the brain. This can lead to difficulty with attention and concentration, loss of motivation and interest, shuffling gait, crying or laughing that seems to come out of the blue (called pseudobulbar affect).

Progression
Classically, VaD gets worse over time in a “stepwise” manner, meaning distinct periods where the symptoms get suddenly worse. However, this is not always the case.

Diagnosis and Treatment
How it’s diagnosed
Vascular dementia is diagnosed based on the signs and symptoms above by a qualified doctor, as well as brain imaging such as an MRI or CT scan.

Medications
Treatment of vascular dementia should focus on two things: the underlying cause, and the symptoms. Since VaD is caused by unhealthy blood vessels, treating blood pressure, blood sugar, cholesterol and other general health maintenance (such as not smoking, staying active, taking an aspirin as directed) are important for slowing or stopping the progression of VaD. Alzheimer’s medications such as donepezil may help as well, as can treatment of mood.
 

Creutzfeld-Jakob Disease (CJD)

Creutzfeld-Jakob Disease, or CJD, is of the most rapid and devastating forms of dementia. It is caused by an abnormal prionprotein. It is rare.

Signs and Symptoms
CJD usually progresses over the course of just a few months. All types of memory and thinking may be affected. Additionally, CJD and other prion diseases can cause jerky involuntary movements, seizures, and major disturbances in sleep.  CJD is fatal.

Diagnosis and Treatment
CJD is diagnosed by a combination of clinical features (the signs and symptoms) and other evidence from tests such as spinal fluid tests (protein 14-3-3, tau), genetic testing, and brain MRI changes. There are no treatments at the current time.


Neurobehavior and Memory Clinic
676 North Saint Clair Street
Arkes Pavilion, Suite 945
Chicago, Illinois 60611

Tel: 312.695.9627
Fax: 312.695.6072

How we can help

The Neurobehavior and Memory Clinic is staffed by physicians from the Northwestern Medical Group, a multispecialty group practice of the full-time faculty at Northwestern's Feinberg School of Medicine.  Offering a comprehensive array of diagnostic, therapeutic, and innovative care, the clinic has a multidisciplinary staff that includes behavioral neurologists, neuropsychologists, neuropsychiatrists, and licensed clinical social workers. 

Through the Neurobehavior and Memory Clinic, we treat the whole patient and offer support services for both patients and their families.  Our treatment plan includes:

  • Evaluation of memory and other thinking abilities using specialized tests
  • Psychiatric evaluation and treatment for associated mood and behavior disorders
  • Assessment and follow-up care by behavioral neurologists dedicated to treating those with dementia
  • Disease education, counseling, and referrals to community support services
  • Research opportunities to participate in cutting edge research on Alzheimer's dementia, frontotemporal dementia and primary progressive aphasia