Advertisement

Advances in Therapy

, Volume 28, Issue 7, pp 586–601 | Cite as

Pseudobulbar affect: an under-recognized and under-treated neurological disorder

  • Susan S. Work
  • Jennifer A. Colamonico
  • Walter G. Bradley
  • Randall E. Kaye
Open Access
Original Research

Abstract

Introduction

Pseudobulbar affect (PBA) is a neurologic syndrome of emotional affect disinhibition, characterized by uncontrollable, exaggerated, and often inappropriate emotional outbursts, which may cause severe distress, embarrassment, and social dysfunction. However, the US prevalence of PBA remains unknown.

Methods

An online survey was conducted primarily to estimate the US prevalence of PBA in patients with the six most commonly associated conditions: Alzheimer’s disease, amyotrophic lateral sclerosis, multiple sclerosis, Parkinson’s disease, stroke, and traumatic brain injury. Invitations to participate were randomly sent online to adults (aged ≥18 years) registered in the Harris Poll Online Panel who were patients or belonged to a household with a patient diagnosed with one of the six conditions (identified through previous screening by Harris Interactive). Participants were screened for PBA using the Pathological Laughing and Crying Scale (PLACS) and the Center for Neurologic Study-Lability Scale (CNS-LS). PBA estimates were made using a cut-off score of ≥13 on the PLACS and two different cut-off thresholds on the CNS-LS, a lower one of ≥13 and a more rigorous one of ≥21. Existing US prevalence data for the six underlying conditions were used to estimate US prevalence of PBA.

Results

Of 38,000 individuals invited to participate, 8876 responded (23%) and 2318 (26%) completed the questionnaire. Mean prevalence of PBA across all six conditions was 10.1%, 9.4%, and 37.5% with the PLACS ≥13, CNS-LS ≥21, and CNS-LS ≥13 thresholds, respectively. Using disease population estimates from government agencies and professional organizations, the estimated US population with PBA ranged from 1.8 to 7.1 million. Among patients who discussed their laughing and/or crying episodes with a physician, 41% were diagnosed, and about half received a medication for their episodes.

Conclusions

The overall prevalence of PBA was estimated to be about 10% across these commonly associated underlying neurological conditions and appears to be under-recognized.

Keywords

Alzheimer’s disease amyotrophic lateral sclerosis diagnosis multiple sclerosis Parkinson’s disease prevalence pseudobulbar affect stroke traumatic brain injury treatment 

References

  1. 1.
    Schiffer R, Pope LE. Review of pseudobulbar affect including a novel and potential therapy. J Neuropsychiatry Clin Neurosci. 2005;17:447–454.PubMedCrossRefGoogle Scholar
  2. 2.
    Parvizi J, Coburn KL, Shillcutt SD, et al. Neuroanatomy of pathological laughing and crying: a report of the American Neuropsychiatric Association Committee on Research. J Neuropsychiatry Clin Neurosci. 2009;21:75–87.PubMedCrossRefGoogle Scholar
  3. 3.
    Wortzel HS, Oster TJ, Anderson CA, Arciniegas DB. Pathological laughing and crying: epidemiology, pathophysiology and treatment. CNS Drugs. 2008;22:531–545.PubMedCrossRefGoogle Scholar
  4. 4.
    Moore SR, Gresham LS, Bromberg MB, Kasarkis EJ, Smith RA. A self-report measure of affective lability. J Neurol Neurosurg Psychiatry. 1997;63:89–93.PubMedCrossRefGoogle Scholar
  5. 5.
    Strowd RE, Cartwright MS, Okun MS, Haq I, Siddiqui MS. Pseudobulbar affect: prevalence and quality of life impact in movement disorders. J Neurol. 2010;257:1382–1387.PubMedCrossRefGoogle Scholar
  6. 6.
    Duda JE. History and prevalence of involuntary emotional expression disorder. CNS Spect. 2007;12(suppl. 5):6–10.Google Scholar
  7. 7.
    Wilson SA. Some problems in neurology. Pathological laughing and crying. J Neurol Psychopathol. 1924;4:299–333.PubMedCrossRefGoogle Scholar
  8. 8.
    Dark FL, McGrath JJ, Ron MA. Pathological laughing and crying. Aust N Z J Psychiatry. 1996;30:472–479.PubMedCrossRefGoogle Scholar
  9. 9.
    Calvert T, Knapp P, House A. Psychological associations with emotionalism after stroke. J Neurol Neurosurg Psychiatry. 1998;65:928–929.PubMedCrossRefGoogle Scholar
  10. 10.
    Tateno A, Jorge RE, Robinson RG. Pathological laughing and crying following traumatic brain injury. J Neuropsychiatry Clin Neurosci. 2004;16:426–434.PubMedCrossRefGoogle Scholar
  11. 11.
    Siddiqui MS, Fernandez HH, Garvan CW, et al. Inappropriate crying and laughing in Parkinson disease and movement disorders. World J Biol Psychiatry. 2009;10:234–240.PubMedCrossRefGoogle Scholar
  12. 12.
    Tang WK, Chen Y, Lam WW, et al. Emotional incontinence and executive function in ischemic stroke: a case-controlled study. J Int Neuropsychol Soc. 2009;15:62–68.PubMedCrossRefGoogle Scholar
  13. 13.
    Choi-Kwon S, Kim JS. Poststroke emotional incontinence and decreased sexual activity. Cerebrovasc Dis. 2002;13:31–37.PubMedCrossRefGoogle Scholar
  14. 14.
    Petracca GM, Jorge RE, Ación L, Weintraub D, Robinson RG. Frequency and correlates of involuntary emotional expression disorder in Parkinson’s disease. J Neuropsychiatry Clin Neurosci. 2009;21:406–412.PubMedCrossRefGoogle Scholar
  15. 15.
    Caroscio JT, Mulvihill MN, Sterling R, Abrams B. Amyotrophic lateral sclerosis. Its natural history. Neurol Clin. 1987;5:1–8.PubMedGoogle Scholar
  16. 16.
    Miller RG, Rosenberg JA, Gelinas GF, et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology. 1999;52:1311–1323.PubMedGoogle Scholar
  17. 17.
    Gallagher JP. Pathologic laugher and crying in ALS: a search for their origin. Acta Neurol Scand. 1989;80:114–117.PubMedCrossRefGoogle Scholar
  18. 18.
    Zimmerman EK, Eslinger PJ, Simmons Z, Barrett AM. Emotional perception deficits in amyotrophic lateral sclerosis. Cogn Behav Neurol. 2007;20:79–82.PubMedCrossRefGoogle Scholar
  19. 19.
    Ziegler LH. Psychotic and emotional phenomena, associated with amyotrophic lateral sclerosis. Arch Neurol Psychiatry. 1930;24:930–936.Google Scholar
  20. 20.
    Gubbay SS, Kahana E, Zilber N, et al. Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol. 1985;232:295–300.PubMedCrossRefGoogle Scholar
  21. 21.
    Pratt RT. An investigation of the psychiatric aspects of disseminated sclerosis. J Neurol Neurosurg Psychiatry. 1951;14:326–335.PubMedCrossRefGoogle Scholar
  22. 22.
    Starkstein SE, Migliorelli R, Tesón A, et al. Prevalence and clinical correlates of pathological affective display in Alzheimer’s disease. J Neurol Neurosurg Psychiatry. 1995;59:55–60.PubMedCrossRefGoogle Scholar
  23. 23.
    Data on file. Avanir Pharmaceuticals, Inc.; 2006.Google Scholar
  24. 24.
    Feinstein A, Feinstein K, Gray T, O’Connor P. Prevalence of neurobehavioral correlates of pathological laughing and crying in multiple sclerosis. Arch Neurol. 1997;54:1116–1121.PubMedGoogle Scholar
  25. 25.
    Langworthy OR, Kolb LC, Androp S. Disturbances of behavior in patients with disseminated sclerosis. Am J Psychiatry. 1941;98:243–249.Google Scholar
  26. 26.
    Cottrell SS, Wilson SAK. The affective symptomatology of disseminated sclerosis: a study of 100 cases. J Neurol Psychopathol. 1926;7:1–30PubMedCrossRefGoogle Scholar
  27. 27.
    Kim JS, Choi-Kwon S. Poststroke depression and emotional incontinence: correlation with lesion location. Neurology. 2000;54:1805–1810.PubMedGoogle Scholar
  28. 28.
    Morris PL, Robinson RG, Raphael B. Emotional lability after stroke. Aust N Z J Psychiatry. 1993;27:601–605.PubMedCrossRefGoogle Scholar
  29. 29.
    Tang WK, Chan SS, Chiu HF, et al. Emotional incontinence in Chinese stroke patients — diagnosis, frequency, and clinical and radiological correlates. J Neurol. 2004;251:865–869.PubMedGoogle Scholar
  30. 30.
    House A, Dennis M, Molyneux A, Warlow C, Hawton K. Emotionalism after stroke. BMJ. 1989;298:991–994.PubMedCrossRefGoogle Scholar
  31. 31.
    Robinson RG, Parikh RM, Lipsey JR, Starkstein SE, Price TR. Pathological laughing and crying following stroke: validation of a measurement scale and a double-blind treatment study. Am J Psychiatry. 1993;150:286–293.PubMedGoogle Scholar
  32. 32.
    Zeilig G, Drubach DA, Katz-Zeilig M, Karatinos J. Pathological laughter and crying in patients with closed traumatic brain injury. Brain Inj. 1996;10:591–597.PubMedCrossRefGoogle Scholar
  33. 33.
    Brooks BR, Thisted RA, Appel SH, et al. for the AVP-923 ALS Study Group. Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine. A randomized trial. Neurology. 2004;63:1364–1370.PubMedGoogle Scholar
  34. 34.
    Faul M, Xu L, Wald MM, Coronado VG. Traumatic brain injury in the United States: emergency department visits, hospitalizations and deaths 2002–2006. Atlanta, GA: Centers for Disease Control and Prevention, National Center for Injury Prevention and Control; 2010.Google Scholar
  35. 35.
    Smith RA, Berg JE, Pope LE, et al. Validation of the CNS emotional lability scale for pseudobulbar affect (pathological laughing and crying) in multiple sclerosis patients. Mult Scler. 2004;10:1–7.CrossRefGoogle Scholar
  36. 36.
    Panitch HS, Thisted RA, Smith RA, et al. for the Pseudobular Affect in Multiple Sclerosis Study Group. Randomized, controlled trial of dextromethorphan/quinidine for pseudobulbar affect in multiple sclerosis. Ann Neurol. 2006;59:780–87.PubMedCrossRefGoogle Scholar
  37. 37.
    Smith RA, Licht JM, Pope LE, Berg JE, Arnold RP. Combination dextromethorphan and quinidine in the treatment of frustration and anger in patients with involuntary emotional expression disorder (IEED). Ann Neurol. 2006; 60(suppl. 3):S50.Google Scholar
  38. 38.
    The ALS Association. Who gets ALS? http://www.alsa.org/als/who.cfm. Accessed February 2, 2010.
  39. 39.
    National Institute of Neurological Disorders and Stroke (NINDS). Dementia: hope through research. http://www.ninds.nih.gov/disorders/dementias/detail_dementia.htm. Accessed February 2, 2010.
  40. 40.
    National MS Society. About MS: who gets MS? http://www.nationalmssociety.org/about-multiplesclerosis/what-we-know-about-ms/who-gets-ms/index.aspx. Accessed February 2, 2010.
  41. 41.
    Parkinson’s Disease Foundation. Frequently-asked questions about Parkinson’s disease. http://www.pdf.org/en/pins_res_center. Accessed February 2, 2010.
  42. 42.
    American Stroke Association. Heart disease and stroke statistics — 2005 update. http://www.americanheart.org/downloadable/heart/1105390918119HDSStats2005Update.pdf. Accessed March 23, 2011.
  43. 43.
    Centers for Disease Control (CDC) and National Center for Injury Prevention and Control (NCIPC). What are the long-term outcomes of TBI? 2006. http://www.cdc.gov/traumaticbraininjury/outcomes.html. Accessed February 2, 2010.
  44. 44.
    Wallin MT, Kurtzke JF. Neuroepidemiology. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Neurology in Clinical Practice. 4th edition. Philadelphia, PA: Butterworth Heinemann Elsevier, 2004:776–779.Google Scholar
  45. 45.
    United States Census Bureau. The Population Division of the United States Census Bureau. U.S. POPClock projection. http://www.census.gov/population/www/popclockus.html. Accessed February 2, 2010.
  46. 46.
    Rowland LP. Neurology in Clinical Practice, 3rd edition. Arch Neurol. 2000;57:1084.Google Scholar
  47. 47.
    Hier DB. Neurology in Clinical Practice e-dition: text with continually updated online references/Edition 4. Doody Review Services. http://search.barnesandnoble.com/Neurology-in-Clinical-Practicee-dition/Walter-G-Bradley/e/9780750674690#TABS. iAccessed January 6, 2011.
  48. 48.
    Deming WE, Stephan FF. On a least squares adjustment of a sampled frequency table when the expected marginal totals are known. Ann Math Stat. 1940;11:427–444.CrossRefGoogle Scholar
  49. 49.
    Pleis JR, Schiller JS, Benson V. Summary health statistics for U.S. adults: National Health Interview Survey, 2000. Vital Health Stat 10. 2003;215:1–132.PubMedGoogle Scholar
  50. 50.
    Langlois JA, Rutland-Brown W, Thomas KE. Traumatic brain injury in the United States: emergency department visits, hospitalizations, and deaths. Atlanta, GA: Centers for Disease Control and Prevention, National Center for Injury Prevention and Control; 2004.Google Scholar
  51. 51.
    Schafer JL. Analysis of incomplete multivariate data: monographs on statistics and applied probability. 1st edition. Boca Raton, FL: Chapman & Hall/CRC, 1997.CrossRefGoogle Scholar
  52. 52.
    Miller RG, Anderson Brooks BR, Mitsumoto H, Bradley WG, Ringel, SP, for the ALS CARE Study Group. Outcomes research in amyotrophic lateral sclerosis: lessons learned from the Amyotrophic Lateral Sclerosis Clinical Assessment, Research, and Education Database. Ann Neurol. 2009;65(suppl.):S 24–S28.Google Scholar
  53. 53.
    Tariot PN, Mack JL, Patterson MB, et al. Behavioral Pathology Committee of the Consortium to Establish a Registry for Alzheimer’s Disease. Am J Psychiatry. 1995;152:1349–1357.PubMedGoogle Scholar
  54. 54.
    Haupt M. Emotional lability, intrusiveness, and catastrophic reactions. Int Psychogeriatr. 1996;8:409–414.PubMedCrossRefGoogle Scholar
  55. 55.
    Poeck K. Pathological laughter and crying. In: Fredericks JAM, ed. Handbook of Clinical Neurology. Volume 45. Amsterdam, Netherlands: Elsevier Science Publishers; 1985.Google Scholar
  56. 56.
    Poeck K. Pathological laughing and weeping in patients with progressive bulbar palsy. Ger Med Mon. 1969;14:394–397.PubMedGoogle Scholar
  57. 57.
    Surridge D. An investigation into some psychiatric aspects of multiple sclerosis. Br J Psychiatry. 1969;115:749–764.PubMedCrossRefGoogle Scholar
  58. 58.
    Phuong L, Garg S, Duda JE, Stern MB, Weintraub D. Involuntary emotional expression disorder (IEED) in Parkinson’s disease. Parkinsonism Relat Disord. 2009;15:511–515.PubMedCrossRefGoogle Scholar
  59. 59.
    Cummings JL, Arciniegas DB, Brooks DB, et al. Defining and diagnosing involuntary emotional expression disorder. CNS Spectr. 2006;11(suppl. 6):1–7.PubMedGoogle Scholar
  60. 60.
    Kim JS. Post-stroke emotional incontinence after small lenticulocapsular stroke: correlation with lesion location. J Neurol. 2002;249:805–810.PubMedCrossRefGoogle Scholar
  61. 61.
    Green RL, McAllister TW, Bernat JL. A study of crying in medically and surgically hospitalized patients. Am J Psychiatry. 1987;144:442–447.PubMedGoogle Scholar
  62. 62.
    Sato T, Bottlender R, Kleindienst N, Möller H-J. Syndromes and phenomenological subtypes underlying acute mania: a factor analytic study of 576 manic patients. Am J Psychiatry. 2002;159:968–974.PubMedCrossRefGoogle Scholar
  63. 63.
    Rosen H. Dextromethorphan/quinidine sulfate for pseuduobulbar affect. Drugs Today (Barc). 2008;44:661–668.CrossRefGoogle Scholar
  64. 64.
    Choi-Kwon S, Han SW, Kwon SU, et al. Fluoxetine treatment in poststroke depression, emotional incontinence, and anger proneness: a double-blind, placebo-controlled study. Stroke. 2006;37:156–161.PubMedCrossRefGoogle Scholar
  65. 65.
    Müller U, Murai T, Bauer-Wittmund T, von Cramon DY. Paroxetine versus citalopram treatment of pathological crying after brain injury. Brain Inj. 1999;13:805–811.PubMedCrossRefGoogle Scholar
  66. 66.
    Andersen G, Vestergaard K, Riis JO. Citalopram for post-stroke pathological crying. Lancet. 1993;342:837–839.PubMedCrossRefGoogle Scholar
  67. 67.
    Barnes R, Veith R, Okimoto J, Raskind M, Gumbrecht G. Efficacy of antipsychotic medications in behaviorally disturbed dementia patients. Am J Psychiatry. 1982;139:1170–1174.PubMedGoogle Scholar
  68. 68.
    Fava M. Psychopharmacologic treatment of pathologic aggression. Psychiatr Clin North Am. 1997;20:427–451.PubMedCrossRefGoogle Scholar
  69. 69.
    Nuedexta™ (dextromethorphan hydrobromide and quinidine sulfate) capsules prescribing information. Aliso Viejo, CA: Avanir Pharmaceuticals, Inc.; October 2010.Google Scholar
  70. 70.
    Pioro EP, Brooks BR, Cummings J, et al. Dextromethorphan plus ultra low-dose quinidine reduces pseudobulbar affect. Ann Neurol. 2010;68:693–702.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Healthcare 2011

Authors and Affiliations

  • Susan S. Work
    • 1
  • Jennifer A. Colamonico
    • 2
  • Walter G. Bradley
    • 3
  • Randall E. Kaye
    • 1
  1. 1.Avanir Pharmaceuticals, Inc.Aliso ViejoUSA
  2. 2.Healthcare & Policy Research, Harris InteractiveNew YorkUSA
  3. 3.Department of Neurology, Miller School of MedicineUniversity of MiamiMiamiUSA

Personalised recommendations