Pseudobulbar affect

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Pseudobulbar affect (PBA), emotional lability, labile affect or emotional incontinence refers to a neurologic disorder characterized by involuntary crying or uncontrollable episodes of crying and/or laughing, or other emotional displays.[1] PBA occurs secondary to neurologic disease or brain injury. Patients may find themselves crying uncontrollably at something that is only moderately sad, being unable to stop themselves for several minutes. Episodes may also be mood-incongruent: a patient might laugh uncontrollably when angry or frustrated, for example.

Terminology

Historically there have been a variety of terms used, including pseudobulbar affect, pathological laughter and crying, emotional lability, emotionalism, emotional dysregulation, or, more recently, involuntary emotional expression disorder (IEED).[2]

Terms such as forced crying, involuntary crying, pathological emotionality, and emotional incontinence have also been used, although less frequently.[3]

Clinical presentation

The cardinal feature of the disorder is a pathologically lowered threshold for exhibiting the behavioral response of laughter, crying, or both. An affected individual exhibits episodes of laughter and/or crying without an apparent motivating stimulus or in response to stimuli that would not have elicited such an emotional response before the onset of their underlying neurologic disorder. In some patients, the emotional response is exaggerated in intensity but is provoked by a stimulus with an emotional valence congruent with the character of the emotional display. For example, a sad stimulus provokes a pathologically exaggerated weeping response instead of a sigh, which the patient normally would have exhibited in that particular situation.

However, in some other patients, the character of the emotional display can be incongruent with, and even contradictory to, the emotional valence of the provoking stimulus or may be incited by a stimulus with no clear valence. For example, a patient may laugh in response to sad news or cry in response to stimuli with no emotional undertone, or, once provoked, the episodes may switch from laughing to crying or vice versa.[4]

Characteristics

The symptoms of PBA can be severe, with persistent and unremitting episodes.[3] Characteristics include:

  • the onset can be sudden and unpredictable, and has been described by some patients as coming on like a seizure;
  • the outbursts have a typical duration of a few seconds to several minutes; and,
  • the outbursts may happen several times a day.

Background and current understanding

Many patients with neurologic disorders exhibit uncontrollable episodes of laughing, crying, or both that are either exaggerated or contradictory to the context in which they occur. Where patients have significant cognitive deficits (e.g., Alzheimer's) it can be unclear whether it is true PBA as opposed to a grosser form of emotional dysregulation, but patients with intact cognition often report the symptom as disturbing. Patients report that their episodes are at best only partially amenable to voluntary control, and unless they experience a severe change of mental status, they often have insight into their problem and judge their emotional display as inappropriate and out of character. The clinical impact of PBA can be severe, with unremitting and persistent symptoms that can be disabling to patients, and may significantly impact quality of life for caregivers.

Causes

PBA was first described in the medical literature more than 130 years ago. Charles Darwin described elements of the condition in his seminal text The Expression of the Emotions in Man and Animals.[5] Although the symptoms of PBA have been recognized for more than a century, the specific pathophysiology involved in this frequently debilitating condition is still under investigation; the primary pathogenic mechanisms of PBA remain controversial.[6] One hypothesis, established by early researchers such as Wilson and Oppenheim, placed emphasis on the role of the corticobulbar pathways in modulating emotional expression in a top-down model, and theorized that PBA occurs when bilateral lesions in the descending corticobulbar tract cause failure of voluntary control of emotion, which leads to the disinhibition, or release, of laughing/crying centers in the brainstem.[7] Other theories implicate the prefrontal cortex.[8]

Pseudobulbar affect is a secondary condition

Pseudobulbar affect is a condition that occurs secondary to neurological disease or brain injury, and is thought to result from disruptions of neural networks that control the generation and regulation of motor output of emotions. PBA is most commonly observed in people with neurologic injuries such as traumatic brain injury (TBI) and stroke,[9][10] and neurologic diseases such as dementias including Alzheimer's disease, attention deficit/hyperactivity disorder (ADHD),[11][12][13] multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS), Lyme Disease, PANDAS in children and adults, and Parkinson's disease (PD).

PBA has also been observed in association with a variety of other brain disorders, including brain tumors, Wilson’s disease, syphilitic pseudobulbar palsy, and various encephalitides. Rarer conditions associated with PBA include gelastic epilepsy, dacrystic epilepsy, central pontine myelinolysis, olivopontinocerebellar atrophy, lipid storage diseases, chemical exposure (e.g., nitrous oxide and insecticides), fou rire prodromique, and Angelman syndrome.

It is hypothesized that these primary neurologic injuries and diseases impact chemical signaling in the brain, which in turn disrupts the neurologic pathways that control emotional expression.[14][15][16]

Other effects

Impact on social life

While not as profoundly disabling as the physical symptoms of these diseases, PBA can have a significant impact on individuals' social functioning and their relationships with others. Such sudden, frequent, extreme, uncontrollable emotional outbursts may lead to social withdrawal and interfere with activities of daily living, social and professional pursuits, and have a negative impact on overall healthcare. For example, patients with ALS and MS are often cognitively normal; however, the appearance of uncontrollable emotions is commonly associated with learning disabilities; this may lead to severe embarrassment and avoidance of social interactions for the patient, which in turn has an impact on their coping mechanisms and their careers.[3][17][18][19][20]

Depression: distinct or coexisting

PBA may often be misdiagnosed as clinical depression; however, many clear distinctions exist.

Several criteria exist to differentiate between PBA and depression

In depression and grief syndromes, crying is typically a sign of sadness, whereas the pathological displays of crying which occur in PBA are often in contrast to the underlying mood, or greatly in excess of the mood or eliciting stimulus. In addition, a key to differentiating depression from PBA is duration: PBA episodes are sudden, occurring in a brief episodic manner, while crying in depression is a more sustained presentation and closely relates to the underlying mood state. The level of control that one has over the crying episodes in PBA is minimal or nonexistent, whereas for those suffering from depression, the emotional expression (typically crying) can be modulated by the situation. Similarly, the trigger for episodes of crying in patients with PBA may be nonspecific, minimal or inappropriate to the situation, but in depression the stimulus is specific to the mood-related condition. These differences are outlined in the adjacent Table.

In some cases, depressed mood and PBA may co-exist. In fact, depression is one of the most common emotional changes in patients with neurodegenerative disease or post-stroke sequelae. As a result, it is often comorbid with PBA. Comorbidity implies that depression is distinct from PBA and is not necessary for, nor does it exclude, a diagnosis of PBA.[2]

Prevalence of PBA symptoms

Prevalence estimates place the number of people with PBA between 1.5 and 2 million in the United States alone. However, given the fact that PBA is a relatively common disorder among patients with various neurologic conditions, its actual prevalence may be higher. Furthermore, PBA is generally thought to be under-recognized and undertreated because clinicians are unfamiliar with the disorder.[21][22]

Prevalence in patients with stroke

PBA is one of the most frequently reported poststroke behavioral syndromes, with a range of reported prevalence rates from 28% to 52%.[23][24][25] The higher prevalence rates tend to be reported in stroke patients who are older and/or who have a history of prior stroke.[26][27] The relationship between poststroke depression and PBA is complicated, because the depressive syndrome also occurs with high frequency in stroke survivors. Poststroke patients with PBA are more depressed than poststroke patients without PBA, and the presence of a depressive syndrome may exacerbate the weeping side of PBA symptoms.[23][28]

Prevalence in patients with MS

Recent studies suggest a lifetime prevalence of PBA of approximately 10% in patients with MS.[29][30] PBA is generally associated with later stages of the disease (chronic progressive phase).[25] PBA in MS patients is associated with more severe intellectual deterioration, physical disability, and neurological disability.[31]

Prevalence in patients with ALS

A study designed specifically to survey for prevalence found that 49% of patients with ALS also had PBA.[17] PBA does not appear to be associated with duration of amyotrophic lateral sclerosis.[32][33] It is a symptom of ALS that many patients are unaware of and do not get information about by their physician.[34]

Prevalence in patients with TBI

One study of 301 consecutive cases in a clinic setting reported a 5% prevalence. PBA occurred in patients with more severe head injury and coincided with other neurological features suggestive of pseudobulbar palsy.[35]

Brain Injury Association of America (BIAA) indicates that approximately 80% of survey respondents experience symptoms of an additional neurologic condition called pseudobulbar affect (PBA).[36]

Results from a recent investigation estimates the prevelance of pseudobulbar affect associated with traumatic brain injury to exceed more than 55% of survivors.[37]

Treatment

Recognition is crucial for the treatment of PBA. Education of patients, families, and caregivers is an important component of the appropriate treatment of PBA. Crying associated with PBA may be incorrectly interpreted as depression; laughter may be embarrassing. It is therefore critical for families and caregivers to recognize the pathological nature of PBA and the reassurance that this is an involuntary syndrome that is manageable. Traditionally, antidepressants such as sertraline,[38] fluoxetine,[39][40] citalopram,[41] nortriptyline[42] and amitriptyline[43] have been prescribed with some efficacy.

Dextromethorphan/quinidine

Dextromethorphan/quinidine (trade name: Nuedexta) is the first FDA-approved drug for the treatment of PBA. Treatment with dextromethorphan/quinidine significantly decreased laughing and crying episodes in patients with amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS) as compared with placebo in a 12-week, randomized, double-blind study (n=326).[44]

See also

References

  1. ↑ Arciniegas DB, Topkoff; Topkoff, J (2000). "The neuropsychiatry of pathological affect:an approach to evaluation and treatment". Semin Clin Neuropsychiatry 5 (4): 472–479. doi:10.1053/scnp.2000.9554. PMID 11291026. 
  2. ↑ 2.0 2.1 Cummings J, Arciniegas D, Brooks B, Herndon R, Lauterbach E, Pioro E, Robinson R, Scharre D, Schiffer R, Weintraub D (2006). "Defining and diagnosing involuntary emotional expression disorder". CNS Spectr 11 (6): 1–7. PMID 16816786. 
  3. ↑ 3.0 3.1 3.2 Dark FL, McGrath JJ, Ron MA (1996). "Pathological laughing and crying". Aust N Z J Psychiatry 30 (4): 472–479. doi:10.3109/00048679609065020. PMID 8887697. 
  4. ↑ Parvizi J, Archiniegas DB, Bernardini GL, Hoffman MW et al. (2006). "Diagnosis and management of pathological laughter and crying". Mayo Clin Proc 81 (11): 1482–1486. doi:10.4065/81.11.1482. PMID 17120404. 
  5. ↑ Darwin C (1872). The Expression of the Emotions in Man and Animals. New York and London: D. Appleton and Company. ISBN 0-8014-1990-5. 
  6. ↑ Archiniegas DB, Topkoff J (2000). "The neuropsychiatry of pathologic affect: an approach to evaluation and treatment". Seminal Clinical Neuropsychiatry 5 (4): 290–306. doi:10.1053/scnp.2000.9554. PMID 11291026. 
  7. ↑ Parvizi J, Anderson SW, Martin CO, Damasio H, Damasio AR (2001). "Pathological laughter and crying: a link to the cerebellum". Brain 124 (Pt 9): 1708–1719. doi:10.1093/brain/124.9.1708. PMID 11522574. 
  8. ↑ McCullagh S, Moore M, Gawel M, Feinstein A. 1999. Pathological laughing and crying in amyotrophic lateral sclerosis: an association with prefrontal cognitive dysfunction. Journal of Neurological Sciences 169:43–48
  9. ↑ Parvizi J, Anderson SW, Martin CO et al. (2001). "Pathological laughter and crying:a link to the cerebellum". Brain 124 (Pt. 9): 1708–1719. doi:10.1093/brain/124.9.1708. PMID 11522574. 
  10. ↑ Robinson RG, Parikh RM, Lipsey JR, Starkstein SE, Price TR (1993). "Pathological laughing and crying following stroke: validation of a measurement scale and a double-blind treatment study". Am J Psychiatry 150 (2): 286–293. PMID 8422080. 
  11. ↑ Skirrow, C; Asherson, P (2013). "Emotional lability, comorbidity and impairment in adults with attention-deficit hyperactivity disorder". Journal of Affective Disorders 147 (1–3): 80–6. doi:10.1016/j.jad.2012.10.011. PMID 23218897. 
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  17. ↑ 17.0 17.1 Moore SR, Gresham LS, Bromberg MB, Kasarkis EJ, Smith RA (1997). "A self report measure of affective lability". J Neurol Neurosurg Psychiatry 63 (1): 89–93. doi:10.1136/jnnp.63.1.89. PMC 2169647. PMID 9221973. 
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  21. ↑ Archiniegas DB, Lauterbach EC, Anderson KE, Chow TW et al. (2005). "The differential diagnosis of pseudobulbar affect (PBA). Distinguishing PBA among disorders of mood and affect. Proceedings of a roundtable meeting". CNS Spectr 10 (5): 1–16. PMID 15962457. 
  22. ↑ http://www.cnsspectrums.com/aspx/articledetail.aspx?articleid=378[]
  23. ↑ 23.0 23.1 House A, Dennis M, Molyneux A, Warlow C, Hawton K (1989). "Emotionalism after stroke". BMJ 298 (6679): 991–994. doi:10.1136/bmj.298.6679.991. PMC 1836312. PMID 2499390. 
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  25. ↑ 25.0 25.1 Feinstein A, Feinstein K, Gray T, O'Connor P (1997). "Prevalence and neurobehavioral correlates of pathological laughing and crying in multiple sclerosis". Arch Neruol 54 (9): 1116–1121. doi:10.1001/archneur.1997.00550210050012. PMID 9311355. 
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  27. ↑ Harris Y, Gorelick PB, Cohen D, Dollear W et al. (1994). "Psychiatric symptoms in dementia associated with stroke: a case-control analysis among predominantly African-American patients". J Natl Med Assoc 86 (9): 697–702. PMC 2607578. PMID 7966434. 
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  29. ↑ Haupt (1996). "Emotional lability, intrusiveness, and catastrophic reactions". Int Psychogeriatr 8 (Suppl 3): 409–414. doi:10.1017/S1041610297003736. PMID 9154598. 
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  31. ↑ Surridge D (1969). "An investigation into some psychiatric aspects of multiple sclerosis". Br J Psychiatry 115 (524): 749–764. doi:10.1192/bjp.115.524.749. PMID 5806869. 
  32. ↑ Caroscio JT, Mulvihill MN, Sterling R, Abrams B (1987). "Amyotrophic lateral sclerosis". Neurol Clin 5 (1): 1–8. PMID 3561382. 
  33. ↑ Gallagher JP (1989). "Pathologic laughter and crying in ALS: a search for their origin". Acta Neurol Scand 80 (2): 114–117. doi:10.1111/j.1600-0404.1989.tb03851.x. PMID 2816272. 
  34. ↑ Wicks P Frost J (2008). "ALS patients request more information about cognitive symptoms". Eur J Neurol 15 (5): 497–500. doi:10.1111/j.1468-1331.2008.02107.x. PMID 18325023. 
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  36. ↑ http://www.biausa.org/_literature_77385/1-7-2011_PBA_Survey_Results[]
  37. ↑ Fellus JL, Kantor D, Kaye RE (2012). "PRISM registry: A novel research tool to determine the prevalence of pseudobulbar affect". Eur Neurol 19 (2): 85–90. PMID 6156071. 
  38. ↑ Burns, Alistair; Russell, Eve; Stratton-Powell, Hilary; Tyrell, Pippa; O'Neill, Paul; Baldwin, Robert (1999). "Sertraline in stroke-associated lability of mood". International Journal of Geriatric Psychiatry 14 (8): 681–5. doi:10.1002/(SICI)1099-1166(199908)14:8<681::AID-GPS49>3.0.CO;2-Z. PMID 10489659. 
  39. ↑ Brown, K. W.; Sloan, R. L.; Pentland, B. (1998). "Fluoxetine as a treatment for post-stroke emotionalism". Acta Psychiatrica Scandinavica 98 (6): 455–8. doi:10.1111/j.1600-0447.1998.tb10119.x. PMID 9879787. 
  40. ↑ Choi-Kwon, S.; Han, S. W.; Kwon, S. U.; Kang, D.-W.; Choi, J. M.; Kim, J. S. (2005). "Fluoxetine Treatment in Poststroke Depression, Emotional Incontinence, and Anger Proneness: A Double-Blind, Placebo-Controlled Study". Stroke 37 (1): 156–61. doi:10.1161/01.STR.0000190892.93663.e2. PMID 16306470. 
  41. ↑ Andersen, G (1993). "Citalopram for post-stroke pathological crying". The Lancet 342 (8875): 837–9. doi:10.1016/0140-6736(93)92696-Q. PMID 8104273. 
  42. ↑ Robinson, RG; Parikh, RM; Lipsey, JR; Starkstein, SE; Price, TR (1993). "Pathological laughing and crying following stroke: Validation of a measurement scale and a double-blind treatment study". The American Journal of Psychiatry 150 (2): 286–93. PMID 8422080. 
  43. ↑ Schiffer, Randolph B.; Herndon, Robert M.; Rudick, Richard A. (1985). "Treatment of Pathologic Laughing and Weeping with Amitriptyline". New England Journal of Medicine 312 (23): 1480–2. doi:10.1056/NEJM198506063122303. PMID 3887172. 
  44. ↑ Pioro, Erik P.; Brooks, Benjamin Rix; Cummings, Jeffrey; Schiffer, Randolph; Thisted, Ronald A.; Wynn, Daniel; Hepner, Adrian; Kaye, Randall (2010). "Dextromethorphan Plus Ultra Low-Dose Quinidine Reduces Pseudobulbar Affect". Annals of Neurology 68 (5): 693–702. doi:10.1002/ana.22093. PMID 20839238. 
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