An Overlap Between Als And Ftd
These two distinct disorders FTD and ALS are merging, and scientists are coming to understand that theres tremendous overlap between the two, says Stanley H. Appel, chairman of the Department of Neurology at the Methodist Neurological Institute, in Houston, and director of the MDA/ALS clinic there.
Scientists have identified several genes that appear to be associated with both ALS and FTD. This fall, researchers uncovered a gene, C9ORF72, which they identified as the most common cause to date of familial ALS, FTD and ALS-FTD.
In addition, a 2005 study found FTD-associated changes in the brains of all ALS participants, whether or not they showed signs of cognitive impairment.
We did a study in ALS patients who were cognitively normal, and they had changes in the frontal and temporal regions of the brain consistent with FTD, says Catherine Lomen-Hoerth, who directs the MDA Clinic at the University of California at San Francisco Medical Center. Its just to a much lesser degree, so there are no obvious symptoms.
Causes Of Motor Neurone Disease
Motor neurone disease occurs when specialist nerve cells in the brain and spinal cord progressively lose their function. It’s not clear why this happens.
In most cases, a person with motor neurone disease won’t have a family history of the condition. This is known as sporadic motor neurone disease. Researchers believe that the cause is probably a series of steps involving a mixture of damaging genetic and environmental factors. As we get older, we may gradually lose the ability to keep this damage under control, triggering irreversible neurodegeneration.
About 5% of people with motor neurone disease have a close family relative with the condition or a related condition known as frontotemporal dementia. This is called familial motor neurone disease which can be hereditary or linked to a problem with genes that can cause problems at a younger age.
Next Steps For Dementia And Als
People with fronto-temporal dementia-like symptoms require regularly scheduled follow-up visits with the medical professional coordinating his or her care. These visits will give the coordinator a chance to check progress and monitor behavioral changes. The coordinator can make recommendations for changes in treatment if any are necessary.
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What Are The Causes Of Als
ALS can be classified as either sporadic or familial. Most cases are sporadic. That means no specific cause is known.
The Mayo Clinic estimates that genetics only cause ALS in about 5 to 10 percent of cases. Other causes of ALS arent well understood. Some factors that scientists think might contribute to ALS include:
- free radical damage
- an immune response that targets motor neurons
- imbalances in the chemical messenger glutamate
- a buildup of abnormal proteins inside nerve cells
The Mayo Clinic has also identified smoking, lead exposure, and military service as possible risk factors for this condition.
The onset of symptoms in ALS usually occurs between the ages of 50 and 60, although symptoms can occur earlier. ALS is slightly more common in men than in women.
What Happens In Als
The primary symptom of lower motor neuron disease is weakness. The weakness usually begins in one hand, one foot, or the tongue. As weakness becomes more severe, the involved muscles become smaller, and weakness spreads to other muscles. Cramping and twitching of muscles are common however, these symptoms are also common in healthy people. Clinicians typically use electromyography to diagnose lower motor neuron disease.
The primary symptoms of upper motor neuron disease are stiffness, slowness and clumsiness of movement. The symptoms usually begin in one limb or in the mouth or throat, later spreading to other parts of the body. There are no standard laboratory tests for upper motor neuron disease, but spasticity , abnormally brisk tendon reflexes, Babinskis sign and diminished fine motor coordination are seen as diagnostic signs on examination.
The earliest symptoms of amyotrophic lateral sclerosis may be so slight that they are frequently overlooked. All patients with ALS will experience progressive muscle weakness and paralysis, but not all people will share the same symptoms or the same patterns of progression. From the time of diagnosis, a patient with ALS typically survives three to five years.
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Who Gets Ftd Symptoms
Approximately half of all people with ALS wont exhibit any cognitive or behavioral disruptions throughout the course of their disease.
The other half will exhibit some signs of FTD at some stage in their disease. In most cases, the signs will be extremely subtle.
Its estimated that between 10 to 30 percent of people with ALS will have more severe FTD symptoms.
Studies have shown that older age, symptoms beginning in the speaking or swallowing muscles , and a high degree of bulbar involvement correlate with an increased incidence of FTD.
FTD and ALS cause a wide range of different presentations and severities, says Walter Bradley, former director of the Kessenich Family MDA/ALS Center at the University of Miami School of Medicine.
You have people with absolutely pure ALS, who never show any signs of cognitive impairment whatsoever, or any behavioral abnormality, or any other of the hallmarks of frontotemporal degeneration, says Bradley. And you have people who have pure frontotemporal dementia, who never get any motor neuron disease, no ALS.
And then you get people who are somewhere in between the two ends of that spectrum.
What Are The Main Types
There are two types of ALS:
- Sporadic ALS is the most common form. It affects up to 95% of people with the disease. Sporadic means it happens sometimes without a clear cause.
- Familial ALS runs in families. About 5% to 10% of people with ALS have this type. FALS is caused by changes to a gene. Parents pass the faulty gene to their children. If one parent has the gene for ALS, each of their children will have a 50% chance of getting the gene and having the disease.
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Treating Motor Neurone Disease
There’s currently no cure for motor neurone disease. Treatment aims to:
- make the person feel comfortable and have the best quality of life possible
- compensate for the progressive loss of bodily functions such as mobility, communication, swallowing and breathing
For example, a breathing mask can greatly help the symptoms of breathing and weakness, and a feeding tube helps maintain nutrition and overall comfort. If necessary, medication can be used to help control excessive drooling.
A medication called riluzole has shown a very small improvement in patients’ overall survival, but it isn’t a cure and doesn’t stop the condition progressing.
Read more about treating motor neurone disease
Testing To Diagnose Ftd
Lab tests usually arent used to make a definitive diagnosis of FTD, although blood or other biological samples may be tested to rule out other factors that can cause dementia-like symptoms.
Anxiety, depression, sleeping problems, insufficient blood-oxygen levels and medication side effects can cause symptoms that mimic FTD-related symptoms, and must be ruled out before an FTD diagnosis can be made.
Imaging studies are useful tools, as they are able to pinpoint brain abnormalities that can be associated with FTD. Computed tomography scans are able to detect atrophy in the frontal lobe, magnetic resonance imaging can help clinicians observe brain abnormalities in finer detail, and single-photon emission computed tomography images are able to indicate brain-function problems.
In 2011, an international consortium developed revised guidelines for the diagnosis of behavioral variant frontotemporal dementia the most common type associated with ALS based on the available literature and collective experience. The criteria guide physicians toward, or away from, an FTD diagnosis based on the presence and severity of symptoms.
Neuropsychological tests designed to assess different thinking functions also can be used to evaluate cognitive changes in people with ALS.
As far as symptoms go, people can start with ALS symptoms, they can start with FTD-type symptoms, or they can start with both at the same time, Lomen-Hoerth noted.
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How Can I Be Involved In Research
National ALS RegistryThe National ALS Registry is a program to collect, manage, and analyze data about people with ALS in the United States. Developed by the Center for Disease Control and Preventions Agency for Toxic Substances and Disease Registry , this registry establishes information about the number of ALS cases, collects demographic, occupational and environmental exposure data from people with ALS to learn about potential risk factors for the disease, and notifies participants about research opportunities. The Registry includes data from national databases as well as de-identified information provided by individuals with ALS. All information is kept confidential. People with ALS can add their information to the Registry by visiting www.cdc.gov/als.
Clinical trialsMany neurological disorders do not have effective treatment options. Clinical trials offer hope for many people and an opportunity to help researchers find better ways to safely detect, treat, or prevent disease. For information about finding and participating in a clinical trial, visit NIHClinical Research Trials and You at www.nih.gov/health/clinicaltrials. For more information abuot finding clinical trials on ALS, visit www.clinicaltrials.gov. Use the search terms amyotrophic lateral sclerosis or ALS AND to locate trials in your area.
Imaging Markers For Prognostication
The average life expectancy of patients with ALS is 24 years, but a subgroup of patients survive for more than 10 years. Clinical predictors of shorter survival time include older age at onset, bulbar onset, and respiratory muscle weakness. Neuroimaging may be able to provide important additional information to help determine prognosis. One 1H-MRS study demonstrated that lower NAA:Cho ratios in the motor cortex were predictive of shorter survival time in patients with ALS. Using DTI, another study showed that lower FA in the CST predicted shorter survival. FA values in the PLIC correlate with rate of disease progression, with patients with lower FA values having faster disease progression. Although the number of studies is small, these results suggest that advanced neuroimaging methods could provide important prognostic information to help guide clinical decisions and stratify patients for clinical research trials.
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Cognitive Changes In Als
Traditionally, ALS has been described as a neurodegenerative disease affecting the motor neurons that control muscles. Involvement of other non-motor systems was thought to be rare.
But cognitive and associated behavioral symptoms associated with ALS have been noted in studies dating as far back as 1889. Today, it is known that approximately 50 percent of the time, ALS is accompanied by some degree of frontotemporal lobar degeneration , a type of progressive deterioration in the frontal and temporal lobes of the brain .
ALS is now known to be a multisystem disorder. Loss of motor neurons still is the hallmark of the disease, but non-motor involvement, including degeneration in parts of the brain responsible for cognitive function, also is recognized.
Although roughly half of all people with ALS exhibit at least some symptoms of cognitive impairment at some stage of their disease, only a small percentage are like John Shearer, whose severe cognitive and behavioral difficulties meet the criteria for an official diagnosis of frontotemporal dementia .
Usually, cognitive and behavioral symptoms in ALS range from mild to moderate. Symptoms include atypical behaviors, apathy and poor judgment, anger and irritability.
The simultaneous occurrence of both disorders is called ALS-FTD.
Contrary To Popular Belief Als Does Affect The Mind
The American Academy of Neurology is the world’s largest association of neurologists and neuroscience professionals, with 36,000 members. The AAN is dedicated to promoting the highest quality patient-centered neurologic care. A neurologist is a doctor with specialized training in diagnosing, treating and managing disorders of the brain and nervous system such as Alzheimer’s disease, stroke, migraine, multiple sclerosis, concussion, Parkinson’s disease and epilepsy.
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Als Effects On Body Function
Oral Motor Effects
While people with ALS do not overproduce saliva, their swallowing problems can create sialorrhea, or excessive salivation and drooling. It may take trials of several medications until one provides relief without undesirable side effects.
Difficulty with swallowing and chewing food increases over time. Coordination of muscles for effective chewing can increase difficulty in digestion. Weight should be carefully monitored to be sure loss is not too aggressive. Because of diminished oral motor skills, choking problems increase. In later stages of the disease, feeding though a tube placed through the abdominal wall into the stomach may need to be considered.
The loss of the ability to communicate is not life-threatening or painful but being unable to verbally and physically communicate is a very frustrating aspect of ALS. Assistive devices can help. Much of technology today relies on a simple touch, eye movement or even brain waves to activate devices. Individuals might use self-made communication boards with letters and pictures or mechanical devices that are high-level in sophistication but ease of use.
Obtaining a communication devise early, before it is needed allows time for the individual to record their own voice to be used by the machine. It can be quite satisfying to sound like yourself instead of a mechanical voice.
Muscle and Tone Effects
Why Did Stephen Hawking Live So Long With Als
Some medical experts have suggested Hawking lived so long because he developed the disease so early in his life, a theory yet to be proven, Bruijn said. Theres no real evidence for that, she said. One could imagine that obviously if you are younger your body is able to cope with something that might go awry.
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What Part Of The Brain Does Als Affect
ALSparts of the brainALS affectspart of the brain
. In this regard, what parts of the cell does ALS affect?
Amyotrophic lateral sclerosis kills motor neurons, the large cells of the spinal cord that send nerve fibers out to control the muscles. Also, motor neurons in the part of the brain governing voluntary movements are destroyed in ALS.
does ALS affect the frontal lobe? ALS patients with frontal lobe abnormalities and poor swallowing ability may have difficulty following medical advice to limit solid foods, or they may place too much food in their mouth.
Also question is, how does ALS affect the central nervous system?
ALS greatly affects the brain and central nervous system. In a healthy body, motor neurons in the brain send signals throughout the body, telling muscles and body systems how to work. ALS slowly destroys those neurons, preventing them from working correctly. Eventually, ALS destroys the neurons completely.
Has anyone ever recovered from ALS?
ALS currently has no known cure. The U.S. Food and Drug Administration has only approved two drugs that slow down the disease, albeit modestly: riluzole and edaravone. Clinical trials have shown that riluzole extends survival by a few months, while edaravone improves the daily functioning of people with ALS.
What Are The Complications Of Als
There is no cure for ALS. Over a period of 3 to 5 years, the disease will progress, making voluntary movements of arms and legs impossible. In time, you will need help with personal care, eating, and mobility. Movement of the diaphragm for breathing is also impaired. You may need a ventilator for breathing. Most people with ALS die from respiratory failure.
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Support Groups And Counseling
Living with motor neuron disease presents many new challenges, both for the affected person and for family and friends.
- You will naturally have many worries about the disabilities that come with the disease. You worry about how your family will cope with the demands of your care. You wonder how they will manage when you are no longer able to contribute. You may feel anxious about loss of independence and death.
- Your loved ones also feel anxious about future uncertainties, issues about your quality of life and need for physical care, and the prospect of death. They wonder how they will care for you through your illness. Money is almost always a concern.
- Many people in this situation feel anxious and depressed, at least sometimes. Some people feel angry and resentful others feel helpless and defeated.
If you are affected by motor neuron disease, talking about feelings and concerns may help.
- Your friends and family members can be very supportive. They may be hesitant to offer support until they see how you are coping. Don’t wait for them to bring it up. If you want to talk about your concerns, let them know.
- Some people worry that they will “burden” their loved ones, or they prefer talking about their concerns with a more neutral professional. A social worker, counselor, or member of the clergy can be helpful if you want to discuss your feelings and concerns about having a motor neuron disease. Your primary care provider or neurologist should be able to recommend someone.
Medications For Dementia In Als
No specific treatment is available for frontal lobe dementia in motor neuron disease.
- The few available treatments for motor neuron diseases have had little effect on dementia. Riluzole is currently the only approved medication for motor neuron diseases. No strong evidence shows that this drug improves dementia due to motor neuron disease.
- Another drug widely used to treat motor neuron disease, gabapentin , has shown no positive effects on dementia.
- Drugs called cholinesterase inhibitors used in Alzheimer’s disease may worsen irritability in people with frontal lobe dementia. These include donepezil ,rivastigmine , and galantamine/galanthamine .
Behavior disturbances may improve with medication.
- Antidepressants that alter brain chemistry in certain ways can improve mood and/or calm agitation.
- Another type is selective dopamine blockers, such as olanzapine .
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Living With Lou Gehrig’s Disease
Living with Lou Gehrig’s disease is physically difficult, but it is reassuring to know that the mind usually is not affected. Most people with ALS can think as clearly as ever, are able to maintain relationships with friends and family, and should be treated respectfully and normally.
It’s normal for family members to feel upset, overwhelmed, and sad if a loved one has ALS. Counseling, as well as support from other family members and friends, can make it easier to deal with the challenges they face.
Communication can be difficult because the disease affects breathing and the muscles needed for speech and arm movement. With patience, the families of patients with ALS can learn to communicate effectively with their loved one.
Researchers continue to study ALS as they try to understand why it happens, and how the disease damages the motor neurons in the brain and spinal cord. As they learn more about the disease, researchers can continue to develop new and better treatments.