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ALS Multidisciplinary Clinic
Clinic: 617-724-3914 Neurology Access Center: 1-855-MGH-NEUR (644-6387)
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ALS research at Mass General
Overview As with any serious disease, ALS has an immense impact on a person’s life as well as their family members or caregivers.
Depression and anxiety are common psychological reactions to the disease, although there are other emotion-related challenges that can be directly related to the disease itself.
How Common Is Depression in People With ALS? Clinical depression is not as prevalent or severe as might be expected (ALS Journal vol 8: 4, 2007. 243-54). Researchers have found, for example, that depression is found less frequently in individuals with ALS than in another neurological disease, multiple sclerosis (MS) (Shifner and Babigian, 13). However, depression, when present, has the ability to significantly affect the life of any one individual and is more commonly seen in situations involving loss or threat of loss.
In general terms, individuals experience depressive symptoms mainly as a reaction following the communication of an ALS diagnosis. There is a higher tendency for depression to appear at the beginning of disease than towards the end. A high number of people with ALS tend have a more positive attitude toward life, even as their disease advances.
Researchers have also found that the presence of depression in patients with ALS doesn’t seem to be related to a person’s spiritual beliefs, the presence of a spouse, caregiver/partners, their financial situation, the psychological health of their partners or their participation in hospice care (Neurology 2005; 65:62-67).
Another finding is that depression in individuals with ALS is not associated with disease severity, although a worsening of their physical functioning is clearly related to levels of their psychological distress.
The presence or absence of depression was not more common among those individuals who expressed a wish to have a hastened death, and the authors of the study concluded that this desire may be part of a broader phenomenon they call end-of-life despair, which is characterized by hopelessness, but not other depressive symptoms.
Because ALS affects everyone in the family, family members may also be affected by depression. This may or may not include the patient. In a study of depression in ALS subjects and their caregivers, depression increased much more for caregivers than for patients. Around 10 percent of caregivers had depression at the beginning of the study, whereas 9 months later, it almost doubled.
Recognizing Depression It is not unusual that the person who is depressed is the last to realize it. Symptoms may come on slowly and almost appear to be normal after awhile.
People who are depressed usually exhibit a very low mood that is part of all aspects of their life. There is the inability to enjoy activities that formerly were enjoyed. Depressed persons may be preoccupied with, or constantly have thoughts and feelings of worthlessness, inappropriate guilt or regret, helplessness, hopelessness and self-hatred. Other symptoms include poor concentration and memory, withdrawal from social situations and activities, reduced sex drive and thoughts of death or suicide.
The inability to sleep normally is common and often a person wakes very early and is unable to get back to sleep. Oversleeping may occur, but is less common. Appetite often decreases, with resulting weight loss, although increased appetite and weight gain occasionally occur. The person may report multiple physical symptoms such as fatigue, headaches or digestive problems. However, very often, the patient may not admit to feeling sad, as they may consider it another burden to the family members or a weakness of character. The following two questions can help identify depression:
If the answer is yes to both questions, depression might be present. Diagnosis and treatment can help restore one’s ability to enjoy life again even as they deal with the ongoing difficult events in their lives.
Managing Depression Identifying depression in people with ALS offers considerable hope. In contrast to many symptoms and complications of the disease, the episodes of depression are not permanent and frequently respond to treatment.
The management of depression must be tailored for each individual patient. Treatment is built on a combination of addressing precipitating problems, antidepressant medication and therapeutic counseling.
Antidepressants are often part of the therapy. No one antidepressant has been shown to be superior than another, and any one medication is often chosen on a personal or family history of use of antidepressants or on the presence or absence of possible side effects. An example of this is the use of tricyclic antidepressants to help reduce saliva production, to help a person sleep better at night or to help with controlling nerve pain. A serotonin uptake receptor inhibitor (SSRI) can sometimes be avoided because of the potential to affect sexual desire, although this class of medication is frequently prescribed when anxiety is also a major symptom.
Once prescribed an antidepressant, it is important to take it each day and to take it at the same time to keep blood levels of the medications stable. Antidepressants are generally started at lower doses and then steadily increased. Many antidepressants can safely be increased every week or two. It is common for an antidepressant to take three to four weeks to begin working and longer to become fully effective (six to eight weeks); however, some individuals respond much earlier. It is important to give any antidepressant an adequate trial.
Although individuals may not respond to any one antidepressant, adding or switching to another antidepressant can increase the chances of success. Many individuals who are successfully treated remain on treatment for at least six months.
Antidepressants should not be discontinued abruptly. Most antidepressants, when they are discontinued, should be gradually tapered over a two to four week period. Patients should call their doctor if they feel a need to discontinue taking the medication, before stopping any antidepressant medication.
Psychotherapy is also an integral part of the care and management of depression. Counseling attempts to address the way that a person sees themselves and to help patients get in control of their disease and their lives. Psychotherapy can be very effective, but it requires time and commitment. Often, a person will also start on an antidepressant medication in addition to seeing a therapist.
Behavioral and Cognitive Changes in ALS ALS may be accompanied by changes in the person’s behavior and overall cognitive functioning or their ability to process information. These features may occur to different degrees in any one individual, from barely noticeable to seriously affecting their ability to stay safe and manage their affairs.
The disease causes these symptoms because of its effect on the frontal-temporal part of the brain. Like other aspects of the disease, symptoms may not be present or noticeable earlier in the disease process, though they may become more apparent in time. It is not well understood how often these symptoms occur in ALS, but as the disease is better understood, it is being more recognized than previously reported.
There do not appear to be any particular risk factors for why it might develop in some patients, but not others. Psychological testing is frequently used to determine the presence of cognitive changes. These tests try to determine if there are changes in personality towards a more rigid thinking process, slow psychological processes (like in decision making and answering questions), difficulties understanding new information, problem solving, dividing one’s attention (the inability to do two things at the same time) and emotional changes. It is important to determine if one or some of the above changes are due to the disease process or with a person’s attempt to cope in a stressful situation.
Helping the individual manage when there are personality changes can be very difficult for family members. The degree of the changes will, to a greater or lesser degree, affect their degree of flexibility and ability to understand information that is important in making medical decisions and decisions centered around safety.
Calling upon the medical, social services and nursing staff involved in the care of the person, as well as relying upon the psychological supports of a psychiatrist or therapist, may prove helpful. Most importantly, involving other family members in the care and support of the patient should be considered. The emotional support of the immediate family members involved in providing care is essential.
Inappropriate Crying and Laughing Involuntary emotional expression disorder (IEED) is a syndrome in which a person’s affect and expressions of emotions occurs independently from the real emotions the person is feeling. This syndrome in ALS is often referred to as a Pseudobulbar Affect (PBA).
A typical presentation of IEED is the occurrence of uncontrollable and unpredictable crying and/or laughing, and the lack of an apparent cause to trigger such responses. These episodes may alternate between tears and laughing quite rapidly, and they can start and stop quite abruptly. The syndrome may sometimes be under-recognized or misunderstood as depression or bipolar disease. IEED should be managed differently than other mood disorders.
The cause of the disorder remains unclear, although there is general agreement that it is the result of an injury to the brain pathways that control expression of emotions. This syndrome can present in a number of neurological diseases like stroke or traumatic brain injury, multiple sclerosis (MS), as well as dementias, Parkinson’s disease and ALS. Both patients and those close to them should be aware of this syndrome, since it can be very disruptive and as distressing as the physical symptoms of the disease.
Treatment is available for PBA. Results from clinical trials support the use of Nuedexta, an FDA approved drug for emotional lability (Annals of Neurology 2010; 68:693-702) Although the syndrome is not a symptom of depression, antidepressants have also been used in the clinical setting and appear helpful.
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