Pseudobulbar Affect PBA Involuntary Outbursts of Crying and Laughing

1. Pseudobulbar Affect PBAInvoluntary Outbursts of Crying and Laughing Prepared by National Council of Certified Dementia Practitioners Sandra Stimson Executive Director Copy Right 2011 National Council of Certified Dementia Practitioners

2. How Do I Pronounce Pseudobulbar Affect? Soo-doh-BUHL-bahr AF-ekt Copy Right 2011 National Council of Certified Dementia Practitioners

3. PBA Definition “PBA is a disorder of disinhibition of emotional expression characterized by uncontrollable episodes of crying or laughing without an apparent stimulus to trigger such a response.” Source: Randall Kaye MD Copy Right 2011 National Council of Certified Dementia Practitioners

4. PBA is a Secondary Condition PBA is a secondary condition to an underlying neurologic condition or injury. Copy Right 2011 National Council of Certified Dementia Practitioners

5. No Known Cause • Not enough is known yet. Different Theories. • The common thought is that it is a result of an injury to neurologic pathways that controls emotional expression. • Some studies have implicated glutamatergic and monoaminergic neurotransmitters systems which appear to regulate expression and emotion. Copy Right 2011 National Council of Certified Dementia Practitioners

6. One of the Most Commonly Reported Syndromes • Reported that: • Estimated 10%-20% of Alzheimer’s disease Patients • 11 % Traumatic Brain Injury TBI • 11 % Stroke • 10 % Multiple Sclerosis Copy Right 2011 National Council of Certified Dementia Practitioners

7. PBA Also Associated with Other Illness • Wilson Disease • ALS Lou Gehrig’s Disease • Syphilitic pseudobular Palsy • Various encephalitides • Parkinson’s • Brain Tumors • Other dementia’s Copy Right 2011 National Council of Certified Dementia Practitioners

8. Rarer Conditions See PBA • Gelastic Epilepsy • Lipid Storage disease • Chemical Exposure • Insecticides • Nitrous Oxide • Angelman Puppet Like Syndrome • And others Copy Right 2011 National Council of Certified Dementia Practitioners

9. A Million or More Estimated over a million people have PBA although numbers could be higher because it is not understood or recognized by clinicians. Copy Right 2011 National Council of Certified Dementia Practitioners

10. Symptoms of PBA • Can be severe, persistent and unremitting episodes • Involuntary crying and or laughing outbursts • Can occur spontaneously • Can occur in response to stimuli such as events or questions. • May Laugh or Cry when frustrated or angry. Copy Right 2011 National Council of Certified Dementia Practitioners

11. May switch back and forth from crying to laughing. • Response may be exaggerated for example a “normal” response to “it’s raining again” might be a sigh, but with PBA they laugh or cry or even switch back and forth from laughing and crying. Copy Right 2011 National Council of Certified Dementia Practitioners

12. Characteristics • Onset is sudden and unpredictable • Can last a few minutes or seconds • May happen frequently through out the day • People with “intact” cognition may report the episodes as very disturbing for them. • Can not control sudden out bursts. Copy Right 2011 National Council of Certified Dementia Practitioners

13. Because this affects the patient socially which leads to isolation and impacts their quality of life. • May affect the way they cope on a day to day basis • May also affect their careers which in turn has a direct affect on their role as a provider. Copy Right 2011 National Council of Certified Dementia Practitioners

14. Disorder of disinhibition of emotional expressions vs. a disturbance of feelings. • Distinct from mood disorders Copy Right 2011 National Council of Certified Dementia Practitioners

15. Ongoing Continued Outbursts May Lead To • Depression • Anxiety • Embarrassment • Social Isolation • Which leads to negative impact on family, caregivers and patient Copy Right 2011 National Council of Certified Dementia Practitioners

16. Lack of Education Among Clinicians • Under treated and unrecognized because Clinicians are not familiar with disorder. Copy Right 2011 National Council of Certified Dementia Practitioners

17. Variety of Terms Used To Describe Patient Symptoms • Emotional Libility • Emotional Incontinence • Pathological Crying and Laughing • Forced Crying • Emotionalism • IEED Involuntary Emotional Expression Disorder Copy Right 2011 National Council of Certified Dementia Practitioners

18. FDA Approval • With the FDA Approval of a drug for treatment the term most likely to be used will be • PBA Pseudobulbar Affect Copy Right 2011 National Council of Certified Dementia Practitioners

19. Preferences in terminology varies among clinical specialties • Some terms imply Neurologic basis • Some terms imply a psychiatric basis • Today PBA is an umbrella that covers all of the language used to describe involuntary crying and laughing. Copy Right 2011 National Council of Certified Dementia Practitioners

20. Misdiagnosed • As Depression although it can co - exists • Generalized anxiety • Personality disorder • Sometimes even epilepsy Copy Right 2011 National Council of Certified Dementia Practitioners

21. Screen for PBA • “The most commonly used instruments to screen for and assess the severity of PBA symptoms is the Center for Neurological Study-Lability Scale (CNS-LS).” • As a class, take the test. • To obtain see Center for Neurologic Study-Lability Scale( CNS-LS) for Pseudobulbar Affect http://www.nuedexta.com/pdf/CNS%20LS%20Questionnaire.pdf Copy Right 2011 National Council of Certified Dementia Practitioners

22. Another Assessments • The Pathological Laughter and Crying Scale (PLACS) As a class, take the PLACS test and see how you score. Copy Right 2011 National Council of Certified Dementia Practitioners

23. Combination Therapy • In 2010 FDA approved in combination Quinidine and Dextromethorphan Copy Right 2011 National Council of Certified Dementia Practitioners

24. Treatment • 2011 Nuedexta (formerly referred to as "Neurodex" and "Zenvia" while in studies) is the first and only FDA-approved treatment for PBA at this time. Avanir Pharmaceuticals • Oral capsule • Taken twice a day Copy Right 2011 National Council of Certified Dementia Practitioners

25. Possible Side Effects • Change in heart rhythm • Dizziness-Take precautions to reduce falls. • Cough, vomiting, weakness • Swelling of feet and ankles • UTI • Elevated liver enzymes • Flatulence Copy Right 2011 National Council of Certified Dementia Practitioners

26. Help Patients Gain Some ControlFrom: www.PBAINFO.org • Be open about it. Let people know you can not always control your emotions. • Distract yourself. If you feel an emotional episode coming on, try to focus on something else. • Change your body position. Note the posture you take when having an episode. When you think you are about to cry or laugh, change it. Copy Right 2011 National Council of Certified Dementia Practitioners

27. Breathe. Inhale and exhale slowly until you are in control. • Relax. Release the tension in your forehead, shoulders and other muscle groups that tense up during an episode. • Always discuss with your doctor what else you can do. Copy Right 2011 National Council of Certified Dementia Practitioners

28. Resources • Center for Neurologic Study http://www.cnsonline.org/?page=1 • Avanir Pharmaceuticals • National Stroke Association • National Council of Certified Dementia Practitioners • National Multiple Sclerosis Society Copy Right 2011 National Council of Certified Dementia Practitioners

29. Pseudobulbar Affect Self Assessment Tool http://www.pbainfo.org/sites/default/files/file/printed/1/self_assessment_.pdf Wikipedia has a great fact sheet www.pbainfo.org Copy Right 2011 National Council of Certified Dementia Practitioners