Evidence-Based Reviews

A cry for help: Treating involuntary emotional expression disorder

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

Pharmacotherapy can lessen the impact of uncontrollable laughing or crying.

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.

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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.

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

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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.

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Mrs. R, a 68-year-old retired teacher, is referred to you for suspected mania after a closed head injury from a car accident. The referring physician reports that Mrs. R experienced mild anterograde amnesia that has resolved, but she continues to suffer from “persistent mood swings as evidenced by substantial inappropriate laughter.”

Mrs. R is not manic. Her mood is normal, with a relatively euthymic affect. When asked about her accident or injury, however, she breaks into bouts of laughter that appear to be uncontrollable and last up to several minutes. These episodes include respiratory changes that make her laughter nearly indistinguishable from crying. Mrs. R explains that the episodes occur every time she discusses the accident—regardless of her efforts to prevent them—and complains they are extremely frustrating and embarrassing. She avoids situations that might trigger the episodes.

Patients with involuntary emotional expression disorder (IEED)—a neurologic disorder that manifests as brief bouts of uncontrollable crying, laughing, or both—may appear to have bipolar disorder, schizophrenia, depression, or another psychiatric disorder. Careful evaluation, however, can distinguish IEED from other conditions. Managing the disorder requires an understanding of IEED phenomenology, including:

neurologic conditions that result in IEED
underlying pathology
diagnostic criteria
effective treatments.

Brain dysfunction alters affect

IEED was introduced as an inclusive term, replacing previous nomenclature such as pathologic laughing and crying, pseudobulbar affect, affective lability, and emotional incontinence.¹

IEED can present as episodes of laughter, as in Mrs. R’s case, but more commonly manifests as bouts of crying. Other presentations include a combination of laughing and crying, but episodic outbursts of other emotions that are out of the patient’s control—such as anger—can be included in this syndrome.² IEED episodes can lead to embarrassment, frustration, and anger that eventually can affect mood and often cause patients to avoid social interaction.³

IEED can occur in any condition that damages and affects the brain areas critical to emotional motor output (Box 1).^4-6 The broad pattern of lesions that can result in IEED stems from many disease states. IEED is often observed in amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), stroke, and traumatic brain injury. It also may occur in dementia, Parkinson’s disease, and other disorders (Table 1).⁷

Table 1

Neurologic conditions associated with IEED

Amyotrophic lateral sclerosis
Multiple sclerosis
Traumatic brain injury
Stroke
Alzheimer’s disease
Frontotemporal dementia
Parkinson’s disease
Progressive supranuclear palsy
Multiple systems atrophy
Wilson’s disease
Normal pressure hydrocephalus
Olivopontine cerebellar atrophy
Source: Reference 7

Diagnosis can be elusive

Although IEED is not included in DSM-IV-TR, recently developed diagnostic criteria can help distinguish it from other disorders (Table 2).¹ As with DSM-categorized disorders, IEED must result in clinically significant distress or impairment in social or occupational function and must not be better accounted for by another disorder or caused by a physiologic substance.

The patient must present with symptoms caused by brain dysfunction from brain injury or neurodegenerative disease. Underlying brain damage might not be apparent when the patient first presents, but to our knowledge no case of idiopathic IEED has been described. If a patient presents with symptoms thought to be IEED, first determine what underlying neurologic condition is causing the symptoms and optimally manage this disorder.

Clinical Point

If a patient presents with symptoms that suggest IEED, first determine the neurologic condition that is causing them

To be considered IEED, the patient’s symptoms must represent a change from his or her normal emotional reactivity. When interviewing patients and their families, compare the patient’s current emotional reactivity with that from when he or she was free of all disease symptoms. Such considerations are important because a patient may have a life-long condition in which he or she is prone to emotional displays—such as essential crying—that is distinct from IEED.⁸

Symptoms must be incongruent with or in excess of the person’s underlying mood and independent or in excess of the provoking stimulus. Inappropriateness of the emotional response is the hallmark of IEED.

Clinical Point

Inappropriate emotional response is the hallmark of IEED

IEED episodes have characteristic clinical features (Table 3). They are brief—lasting seconds to minutes—and sudden in onset and conclusion. Episodes are likely to be stereotyped in severity and presenting type within patients, as well as in the triggering stimulus or set of stimuli. For example, patients often experience episodes when asked about the syndrome.⁹ In severe cases, patients experience episodes with any interpersonal contact.¹⁰

Some characteristics support—but are not essential for—an IEED diagnosis:

autonomic symptoms, such as flushing of the face and increased salivary production during episodes
pseudobulbar signs, such as increased jaw jerk, exaggerated gag reflex, dysarthria, and dysphagia
other emotional outbursts.