Saturday, February 26, 2011

Pseudobulbar Affect - Inappropriate Laughing/Crying in Multiple Sclerosis and Alzheimer's Disease

While sitting having lunch with a friend, a woman begins laughing hysterically, and no one seems to know why.

While playing cards with his friends, a man breaks into tears in the middle of the game, and everyone assumes he's depressed over something.

While happily dancing with her sweetheart, an elderly woman has a sudden outburst of anger, fighting the spouse she was hugging only moments before.  

All of these people are neurological patients, and all these scenarios illustrate a phenomenon that happens in about 10% of MS sufferers, and a similar number of Alzheimer's and dementia patients.  In all it is estimated to affect approximately 2 million people worldwide.  Called the Pseudobulbar Affect (PBA), the condition is often overlooked by family, friends and physicians when they assume that the inappropriate behavior is not a symptom of their disease, but instead a symptom of emotional lability or even depression. PBA is also sometimes called Involuntary Emotional Expression Disorder (IEED).

PBA can actually be a symptom of many different neurological conditions.  This week the ABC television show Off the Map episode Es Un Milagro, a patient with a pressure inside her skull.  She began laughing hysterically and at first was seen as happy she had survived a dangerous surgery.  When the condition continued it was recognized as a symptom that something very wrong was actually happening in her brain.

In MS and AD patients, PBA is usually a symptom of advanced disease, but not always.  It can happen at any stage.  Damage is done within the pathways that control expression, resulting in inappropriate reactions.  Patients are usually aware that their emotions are not on par with their physical reaction, yet are powerless to control it.  Often these episodes do have what starts as a minor emotional trigger far less than the reaction would indicate.

PBA is difficult to diagnose in anyone if the patient doesn't tell their  physicians or caregivers that something seems wrong.  This implies choice on the part of the patient.  For some very advanced patients, this is almost impossible.

MS, AD and other neurological patients may have extreme difficulty communicating due to their diseases/conditions, making diagnosis more about educated assumptions and guesswork.  It's all too common for physicians, caregivers etc to simply assume the patient is depressed and not acting appropriately.  After-all, depression in chronic diseases is indeed a real problem.  It's important not to use the diagnosis of depression as a trashcan diagnosis however - throwing the symptom into the diagnostic trash bin because it is handy.  This diagnosis often takes work on the part of all involved.  In patients who do experience co-morbid depression, getting an appropriate diagnosis may be nearly impossible without an exquisitely intuitive physician.  This makes awareness about the condition super important for caregivers and loved ones of these patients.

Researchers don't know exactly what causes PDA, but assume that it involves the brain's physiology as well as neurotransmitters.  What they do understand is that the symptoms result from the loss of control in the cortical and subcortical pathways that lead to the brain stem.

Treatment may be possible and often includes the use of low doses of tricyclic antidepressant medicines like amitriptyline or SSRI's.  However, the best results seem to be coming from a medicine called Nuedexta which was just given FDA approval in January 2011.  The active ingredient in this compound is dextromethorphan - an OTC cough suppressant - which is thought to help regulate excitatory neurotransmitters, especially in patients whose primary symptom is crying.  The dextromethorphan is combined in this case with an enzyme called quinidine that increases the bioavailability of the dextromethorphan.

PBA is considered widely to be under-recognized and as a result under-treated.  Doctors often don't think of it when considering their patients.  This adds to the burden of the patient and caregiver to be proactive in recognizing and describing, and insisting upon appropriate treatment for the sufferer.

I think this is an important topic, but I haven't seen much in either the MS or AD communities in which I am a member.  Do you regularly talk about PBA?  Do you think it's important?

What can we do to make this widely known and discussed in these communities?  Will you share this post in order to help to that end?

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