Evidence-Based Reviews

A cry for help: Treating involuntary emotional expression disorder

Current Psychiatry. 2008 March;7(3):101-111

References

1. Cummings JL, Arciniegas DB, Brooks BR, et al. Defining and diagnosing involuntary emotional expression disorder. CNS Spectr 2006;11:1-7.

2. 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;37:156-61.

3. Lieberman A, Benson DF. Control of emotional expression in pseudobulbar palsy. A personal experience. Arch Neurol 1977;34:717-9.

4. Caroscio JT, Mulvihill MN, Sterling R, Abrams B. Amyotrophic lateral sclerosis. Its natural history. Neurol Clin 1987;5:1-8.

5. Arciniegas DB, Lauterbach EC, Anderson KE, et al. The differential diagnosis of pseudobulbar affect (PBA). Distinguishing PBA among disorders of mood and affect. Proceedings of a roundtable meeting. CNS Spectr 2005;10:1-14; quiz 15-16.-

6. Rabins PV, Arciniegas DB. Pathophysiology of involuntary emotional expression disorder. CNS Spectr 2007;12:17-22.

7. Arciniegas DB, Topkoff J. The neuropsychiatry of pathologic affect: an approach to evaluation and treatment. Semin Clin Neuropsychiatry 2000;5:290-306.

8. Green RL, McAllister TW, Bernat JL. A study of crying in medically and surgically hospitalized patients. Am J Psychiatry 1987;144:442-7.

9. Dark FL, McGrath JJ, Ron MA. Pathological laughing and crying. Aust N Z J Psychiatry 1996;30:472-9.

10. Wilson S. Some problems in neurology. II. Pathological laughing and crying. J Neurol Psychopathol 1924;16:299-333.

11. Diagnostic and statistical manual of mental disorders. 4th ed, text rev. Washington, D.C: American Psychiatric Association; 2000.

12. Robinson RG, Parikh RM, Lipsey JR, et al. Pathological laughing and crying following stroke: validation of a measurement scale and a double-blind treatment study. Am J Psychiatry 1993;150:286-93.

13. Schiffer RB, Herndon RM, Rudick RA. Treatment of pathologic laughing and weeping with amitriptyline. N Engl J Med 1985;312(23):1480-2.

14. Anderson G, Vestergaard K, Riis JO. Citalopram for post-stroke pathological crying. Lancet 1993;342(8875):837-9.

15. Müller U, Murai T, Bauer-Wittmund T, von Cramon DY. Paroxetine versus citalopram treatment of pathological crying after brain injury. Brain Inj 1999;13(10):805-11.

16. Burns A, Russell E, Stratton-Powell H, et al. Sertraline in stroke-associated lability of mood. Int J Geriatr Psychiatry 1999;14(8):681-5.

17. Kim SW, Shin IS, Kim JM, et al. Mirtazapine treatment for pathological laughing and crying after stroke. Clin Neuropharmacol 2005;28:249-51.

18. Udaka F, Yamao S, Nagata H, et al. Pathologic laughing and crying treated with levodopa. Arch Neurol 1984;41:1095-6.

19. Brooks BR, Thisted RA, Appel SH, et al. Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology 2004;63:1364-70.

20. Panitch HS, Thisted RA, Smith RA, et al. Randomized, controlled trial of dextromethorphan/quinidine for pseudobulbar affect in multiple sclerosis. Ann Neurol 2006;59:780-7.

21. Werling LL, Keller A, Frank JG, Nuwayhid SJ. A comparison of the binding profiles of dextromethorphan, memantine, fluoxetine and amitriptyline: treatment of involuntary emotional expression disorder. Exp Neurol 2007;207(2):248-57.

22. Starkstein SE, Migliorelli R, Teson A, et al. Prevalence and clinical correlates of pathological affective display in Alzheimer’s disease. J Neurol Neurosurg Psychiatry 1995;59:55-60.

23. Tateno A, Jorge RE, Robinson RG. Pathological laughing and crying following traumatic brain injury. J Neuropsychiatry Clin Neurosci 2004;16:426-34.

24. Moore SR, Gresham LS, Bromberg MB, et al. A self report measure of affective lability. J Neurol Neurosurg Psychiatry 1997;63:89-93.

25. 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;10:679-85.

Table 2

Is it IEED? Diagnostic criteria

Presence of brain damage
Episodes of involuntary emotional motor output that: represent a change from normal emotional reactivity are independent or in excess of provoking stimuli result in clinically significant distress or social or functional impairment
*Disorder is not:* better accounted for by another neurologic or psychiatric disorder caused by a physiologic substance
Source: Reference 1

Table 3

Characteristics of IEED episodes

Paroxysmal, sudden onset with rapid offset
Brief (up to several minutes)
Stereotyped across patients (may manifest in similar fashion from patient to patient)
Stereotyped within patients (episodes often have similar type, severity, and eliciting stimuli)

Box 1

IEED: A consequence of brain pathology

Damage to the descending inputs to the pontomedullary area once referred to as the faciorespiratory center is most likely to result in release of bulbar function and, subsequently, involuntary emotional expression disorder (IEED). Therefore, because of the progressive upper motor neuron degeneration associated with amyotrophic lateral sclerosis (ALS), nearly 50% of ALS patients will eventually demonstrate pathological affect.⁴

The lesions that can result in IEED are diffuse, however, and have been described in a review of IEED neuroanatomy as including a cortico-limbic-subcortico-thalamo-ponto-cerebellar network.⁵ Single lesions to white matter structures—such as the internal capsule—and gray matter structures—such as the thalamus, hypothalamus, basal ganglia, cerebellum, and several cortical locations—have been associated with IEED. Bilateral lesions are more likely to produce the disorder than single lesions.

With such varied neuroanatomic substrates, predicting the underlying neurochemical pathology of IEED is difficult. Among the neurotransmitters considered in IEED pathology and treatment are serotonin, glutamate, and dopamine. The sigma-1 receptor system may also play a role.⁶

CASE CONTINUED: Reaching a diagnosis

After thoroughly interviewing Mrs. R, you exclude mood disorders such as depression or bipolar disorder. The paroxysmal, episodic nature of her emotional outbursts and the consistency of the eliciting stimulus, suggest IEED.

Distinguishing IEED from depression. Physicians may be quick to diagnose a patient with consistent, recurrent crying as having a depressive disorder. In IEED, the patient’s family commonly (and inappropriately) will confirm this misperception, even if the patient claims otherwise. The hallmark distinctions between depression and IEED are:

duration of crying
associated mood state.

Major depressive disorder (MDD) is a persistent change in a patient’s mood lasting weeks to months, accompanied by feelings of guilt, helplessness, hopelessness, and worthlessness, apathy, and anhedonia.¹¹ IEED is paroxysmal, with uncontrollable changes in affect without a corresponding sudden mood change. Patients may report mood changes during episodes, but between episodes return to an euthymic affect.

Patients who suffer from MDD, however, are not excluded from an IEED diagnosis. In 1 small study, almost one-half of patients with IEED also had major depression.¹² Differentiating these syndromes—even in patients who suffer from both—is important to ensure proper management and patient and family understanding of the condition. Lastly, although IEED is not a mood disorder, the embarrassment and frustration it causes can change a patient’s mood over time.

Recommended treatment

Education. In our experience, education is critical to help patients and family members understand IEED and deal with embarrassment and other normal reactions they may experience. Explain that these emotional displays are not manic or psychotic episodes but periods of motor dyscontrol caused by a neurologic condition.

Teach them to cope with IEED by:

identifying and avoiding stimuli that provoke IEED episodes
ignoring the episodes and continuing with usual activities.

Antidepressants are first-line pharmacotherapy for IEED. Studies and case reports have shown efficacy for tricyclic antidepressants (TCAs) such as nortriptyline and selective serotonin reuptake inhibitors (SSRIs) such as fluoxetine (Table 4).^2,12-16

Clinical Point

Education can help patients and families understand IEED and deal with embarrassment and other normal reactions

These agents have IEED-specific therapeutic effects through a mechanism independent of their antidepressant action. In patients with IEED and depression, antidepressants may resolve IEED while depression remains refractory.^2,12 Potential drawbacks include anticholinergic effects with TCAs and sexual and gastrointestinal side effects with SSRIs. Nevertheless, these agents are the optimal first-line therapy for IEED among currently available options.

Other agents. Small studies have investigated other agents, but the data are insufficient to warrant recommendations for clinical practice. One study found that the novel antidepressant mirtazapine improved symptoms in 2 patients who did not respond to SSRIs.¹⁷ In another study, levodopa therapy resulted in improvement in 10 of 25 patients.¹⁸

Box 2

2 scales for measuring IEED treatment efficacy

Among scales that measure involuntary emotional expression disorder (IEED) severity, 2 have been used in studies of IEED therapeutic efficacy (see Related Resources):^12,19,20

Pathological Laughing and Crying Scale (PLACS) developed by Robinson et al¹² is an interviewer-administered, 18-item tool that has been validated in IEED patients with stroke,¹² dementia,²²and traumatic brain injury.²³
7-item Center for Neurologic Study-Lability Scale (CNS-LS) is a self-report measure that has been validated in IEED patients with amyotrophic lateral sclerosis²⁴ and multiple sclerosis.²⁵

Although these scales have been used primarily for research, you can use them clinically to establish a baseline of IEED severity and gauge treatment efficacy. Improved scores generally correlate with successful treatment; if a patient fails to show adequate response on 1 of these scales, consider changing treatment.