Involuntary emotional expression disorder (IEED) is a condition that manifests as episodes of crying, laughing, or both and is a result of structural brain damage (Cummings et al., 2006; Schiffer & Pope, 2005). It is common with several neuro-logical disorders, including stroke (Robinson, Parikh, Lipsey, Starkstein, & Price, 1993), amyotrophic lateral sclerosis (ALS; McCullagh, Moore, Gawel, & Feinstein, 1999), traumatic brain injury (Tateno, Jorge, & Robinson, 2004), multiple sclerosis (MS; Feinstein, Feinstein, Gray, & O'Connor, 1997), and dementias such as Alzheimer's disease (Starkstein et al., 1995). Although the prevalence of IEED is likely to be underestimated, approximately 1 million cases exist in the United States (Schiffer & Pope). Associated with undeniable distress for patients and family members, IEED can result in embarrassment and withdrawal from social and occupational situations (Choi-Kwon & Kim, 2002; Lantz, 2005).

The prevalence of IEED is underestimated because this disease is often confused with other mood or affective disorders, such as major depressive disorder (Arciniegas et al., 2005). Diagnosis is further complicated by lack of understanding about the specific etiology or underlying pathology associated with IEED. Given the significant effect IEED can have on a patient's quality of life (Choi-Kwon & Kim, 2002; Green, 1998), its differential diagnosis is a critical aspect of its overall management.

Although useful treatments for IEED exist (Brooks et al., 2004), misdiagnosis leads to improper treatment. The condition frequently comes to the attention of the nurse on inpatient clinical units by patient presentation, but family members and patients may report symptoms in inpatient or outpatient settings. Therefore, nurses must be well-educated regarding the presentation of IEED, the measures available to quantify it, and the interventions used as treatments. This article defines IEED as it presents in stroke and other neurological disorders and provides a brief review of the differential diagnosis and the various options available for appropriate treatment and nursing care of patients who suffer from this debilitating condition.

Defining the Condition

IEED is characterized by episodes of uncontrollable crying, laughing, or both that may be inappropriate and independent of mood (Dark, McGrath, & Ron, 1996). Various terms have been previously used to describe this condition, including pseudobulbar affect, affective and emotional lability, pathologic laughing and crying, emotional incontinence, and forced crying (Dark et al.; Feinstein et al., 1997). The term IEED is intended to unify and eliminate the confusion surrounding the varied nomenclature used in the literature (Cummings et al., 2006).

Regardless of the name used, understanding the distinction between mood and affect helps in the recognition of this condition. The American Psychiatric Association (APA), in the Diagnostic and Statistical Manual of Mental Disorders (4th ed.; APA, 1994), presents the useful analogy of climate versus weather; this analogy is also often used by Arciniegas and colleagues (Arciniegas et al., 2005; Arciniegas & Topkoff, 2000) to explain IEED. Mood is an emotional state that is sustained during a relatively long period of time; it may be compared to climate. In contrast, affect is the "emotional weather," relatively short in duration and superimposed on the prevailing mood. That is, mood is the underlying feelings of a patient, and affect is the outward appearance of the patient's mood. During episodes of IEED, affect and mood become dysregulated. The manifestations of emotional motor output that accompany this syndrome occur independently of mood, but they may or may not appear to correlate with mood.

Since episodes often result in response to specific stimuli, they may or may not be present during an office or clinic visit. Nurses must be aware of the potential appearance of this condition in neurological patients and actively pursue its diagnosis in a clinical setting. Patients report sudden and shortlived episodes, when they occur. Most patients report that they cannot resist, prevent, or inhibit the episodes of crying or laughing. Following the episode, the patient returns to the affective display observed previously, demonstrating no residual changes in mood or affect. Patients describe no mood change after an occurrence of crying or laughing, but the episodes often result in embarrassment (Arciniegas & Topkoff, 2000).

Although the classic example involves episodes of crying or laughing that are incongruent to mood, studies of stroke survivors have shown that an appropriate stimulus may provoke an episode that is congruent with mood but excessive in nature (House, Dennis, Molyneux, Warlow, & Hawton, 1989; Morris, Robinson, & Raphael, 1993). For example, patients may cry for longer than normal when they see a family member who lives in a distant state, or patients may cry when viewing an emotional television commercial that previously would not have affected them.

Assessment Tools

Nurses are in a unique position to identify and assess IEED because they have direct contact with patients in inpatient settings. Differential diagnosis relies heavily on documenting and monitoring clinical changes related to involuntary crying and laughing; nurses must ask about these symptoms because patients may not volunteer the information. Scales used to assess IEED include the Pathological Laughing and Crying Scale (PLACS; Robinson et al., 1993); the Center for Neurologic Study-Lability Scale (CNS-LS; Moore, Gresham, Bromberg, Kasarkis, & Smith, 1997); and the Affective Lability Scale (ALS; Harvey, Greenberg, & Serper, 1989). Initially, nurses may use scale items to educate themselves about how to ask about IEED. Although nurses may choose to not use these scales formally, items from the scales can guide the patient interview. PLACS questions include (1) "Have you recently experienced sudden episodes of crying or laughing?" (2) "Have these episodes occurred without any cause in your surroundings?" and (3) "Have these episodes been uncontrollable by you?" CNS-LS items include (1) "I find myself crying very easily," (2) "There are times when I feel fine one minute, and then I'll become tearful the next over something small or for no reason at all," and (3) "I find that even when I try to control my crying or laughter I am often unable to do so." ALS items include (1) "One minute I can be feeling OK and then I feel tense, jittery, and nervous," (2) "There are times when I'm so mad that my heart is pounding and then shortly afterwards I feel quite relaxed," and (3) "My sleeping patterns frequently shift from times when I have difficulty falling asleep to times when I don't have much of a desire to sleep at all."

During episodes of IEED, affect and mood become dysregulated.

A neuroscience nurse who is aware of IEED can use these scales to quickly and efficiently evaluate patients with diseases or injuries commonly associated with IEED. These measures also provide an excellent first step to properly diagnose IEED in the clinic. Regardless of the setting, care must be taken to not confuse IEED with other disorders that present with episodes of laughing and crying.

Delineating IEED

Although both depression and IEED may present with excessive crying, they are separate disorders. Robinson and colleagues (1993) found no significant correlation between results of the PLACS and the Hamilton Depression Scale when these instruments were administered to a group of patients hospitalized with acute stroke and referred for treatment of involuntary crying. Additionally, these authors reported that improvement in PLACS score after treatment with a tricyclic antidepressant was independent of improvement in depression, as shown by two-way analysis of variance. This finding was supported by successful treatment of IEED in traumatic brain injury patients without corresponding changes in depression (Tateno et al., 2004).

Many patients with serious neurological disorders experience reactive depression in response to their diagnosis. Reactive depression is self-limiting and most often abates as the patient copes with the illness. Unfortunately, patients also may develop major depression or dysthymic symptoms after being diagnosed with a neurological disorder (Ranga et al., 2002). The hallmark of major depression is a depressed mood that exists for most or all of every day. Changes in appetite and sleep also occur (APA, 1994). In addition, patients experience anhedonia, problems with motivation, and difficulty in carrying out daily work and activities (Ebert, Loosen, & Nurcombe, 2000). The onset of major depression is slow and insidious, and without treatment it becomes chronic. Suicide risk is a primary concern with patients who suffer from major depression. In contrast, although patients with IEED often describe embarrassment in social situations and decreased quality of life, they do not describe depressive or suicidal thoughts.

One primary distinction between IEED and major depressive disorder is duration. As a mood disorder, depression is characterized by symptoms that persist for weeks or months. As an affective disorder, IEED consists of shorter episodes of altered affect only. The patient's baseline mood is unaltered by these episodes. Table 1 compares IEED to major or unipolar depression.

Comparison of IEED and Depression

However, a single patient can suffer from both IEED and depression. Thus, assessment for both conditions is essential, using clinical interview questions and established tools such as the IEED tools previously discussed and depression tools including the Beck Depression Inventory (Beck, Ward, Mendelson, Mock, & Erbaugh, 1961), Hamilton Depression Scale (Hamilton, 1967), Center for Epidemiology Studies Depression Scale (CES-D; Radloff, 1977), and the Geriatric Depression Scale (Sheikh & Yesavage, 1986).

A further distinction must be made between IEED and bipolar disorder. Bipolar disorders I and II are diagnosed by the presence of both depression and mania or hypomania. Bipolar disorders are often mistaken for depression because of the prominence of the depressive phase in the illness and because symptoms of mania may be initially ignored. Diagnosis of bipolar disorder may not occur for up to 10 years after symptoms begin, and only one-third of patients are properly diagnosed (Lewis, 2004). Patients with mania display symptoms of elation, humorousness, flight of ideas, and grandiosity. Behavioral signs associated with mania include aggressiveness, increased motor or social activity, and inattentiveness to hygiene, sleep, and eating (Stuart & Laraia, 2005). Mood lability is a sign of bipolar disorder. Patients who experience four or more episodes of depression and mania in 12 months fit the criteria for rapid-cycling bipolar disorder (APA, 1994). Between 5% and 15% of patients with bipolar disorder have rapid-cycling episodes; these episodes are more common in women (Leibenluft, 1997). Because antidepressants can precipitate the rapid-cycling phase of bipolar disorder, proper diagnosis is essential (Goldberg, 2003). These patients may have periods of crying during the depressive phase of the illness and laughing episodes when mania is present. Correct diagnosis can be aided by asking family members to corroborate patient reports of major depression or bipolar disorder and by asking about family history of major depression or bipolar disorder. Many factors that distinguish IEED from major depressive disorder also apply to bipolar disorder, including the persistent nature of symptoms and the relation between affect and underlying mood.

Inappropriate laughing and crying may also be in response to the psychotic and disorganized thoughts present in schizophrenia, characterized by delusions and hallucinations. The possibility of substance abuse must also be evaluated, because patients may cry or laugh when under the influence of psychoactive drugs such as cocaine and other opiates, methamphetamines and comparable stimulants, or hallucinogens such as LSD and ecstasy. Episodes of crying and laughing when the patient is under the influence of such substances are likely to be appropriate in the mind of the patient, even if socially they are not.

Perhaps the single greatest distinction between IEED and the conditions described above is the nature of the underlying pathology. Although the primary effects of the conditions above are believed to be related to changes in neurotransmitter levels, IEED is precipitated by structural brain damage (Arciniegas et al., 2005; Schiffer & Pope, 2005). Neurotransmitter dysfunction as a result of cellular brain damage is likely to contribute to the etiology of IEED, but this dysfunction only occurs secondary to some underlying structural brain damage that occurs as a result of injury or disease.

Pathology

The original description of pathological laughing and crying explained it as a behavioral phenomenon resulting from pseudobulbar palsy. In these patients, lesions to the corticobulbar motor pathways, rather than to the bulbar motor nuclei themselves, resulted in pathological affect. As currently defined, IEED includes lesions to several brain regions beyond the brainstem and even beyond the corticobulbar circuitry. Cortical, subcortical, and limbic areas of the brain are all involved in the generation of emotion (Mega, Cummings, Salloway, & Malloy, 1997), and lesions to any of these areas could in theory result in IEED. Some researchers have suggested that the right hemisphere is associated with negative emotions, such as crying, while the left hemisphere is involved with positive emotions, such as laughing (Arciniegas & Topkoff, 2000). Similarly, damage to the right hemisphere has been suggested as more likely to produce manic and euphoric states, and damage to the left hemisphere is more likely to produce depression and pathological affect (Sackeim et al., 1982). Examples of single-lesion patients, however, suggest that there are no absolute rules of lateralization in IEED (Arroyo et al., 1993; Garg, Misra, & Verma, 2000).

Traditionally, IEED has been described as resulting from a disconnection between the circuitry that produces emotion and the circuitry that controls motor expression (Moore et al., 1997; Wilson, 1924). As such, most cases describe lesions to the midbrain and pontine nuclei (Ceccaldi & Milandre, 1994; Kataoka, Hori, Shirakawa, & Hirose, 1997; Okuda, Chyung, Chin, & Waubant, 2005), and some report lesions to higher cortical regions (Arroyo et al., 1993). Finally, recent investigations suggest that both areas also receive cerebellar input and modulation; thus, damage to the cerebellum also may produce IEED (Parvizi, Anderson, Martin, Damasio, & Damasio, 2001). Regardless of the pathology, IEED does not occur as a primary disorder but only as a result of brain damage associated with neurological disease or injury. Because the type of brain damage involved seems to disinhibit static input to brainstem nuclei that regulate emotional motor output, treatment for IEED must reverse this lost inhibition and bring the emotional motor expression system back into balance.

Tailoring Treatment to Manage IEED Effectively

Diagnosis of IEED in patients with neurological disorders can be assumed when involuntary crying or laughing persists and other neurological conditions have been ruled out. Once diagnosis is confirmed, treatment should be focused on patient and family education, self-management skills, and pharmacological intervention. Evaluations must be repeated and interventions changed according to the results of the evaluations.

Education and Self-Management Skills

Education is essential to the clinical management of this condition. Patients and family members must be counseled about the general nature of IEED, and nurses are in a good position to teach patients about symptoms. Teaching that IEED is a discrete disorder, distinct from depression and other psychiatric disorders, can minimize the emotional stress on patients and family members.

Nurses can work with their patients to identify activities or factors that exacerbate the syndrome- such as excessive stress-and ways to avoid these factors, although astute patients and family members are likely to have already identified causative stimuli. Nurses must assess social withdrawal, because stress and embarrassment associated with episodes may result in isolation; in this case, nurses must explore alternatives for social interaction and support from others and emphasize quality relationships. Nurses should give careful thought to choosing the most effective coping techniques for each patient.

Patients who experience IEED may also be adjusting to a major illness such as ALS, multiple sclerosis, stroke, or traumatic brain injury. Working within a wellness model, nurses can focus on helping patients integrate physical, mental, and spiritual practices in daily life after diagnosis with these disorders (Dossey, Keegan, & Guzzetta, 2005). Because anxiety may increase when episodes occur, nurses may suggest or explore a variety of anxietyreducing techniques with patients. For example, a nurse might teach patients relaxation techniques such as breathing exercises, guided imagery, or distraction to decrease the worry. Likewise, spiritual practices may help patients deal with the impact of their condition.

Medication Management

Although educational efforts and behavioral modification can be useful, these strategies have no effect on the syndrome itself. The only documented mechanism to alleviate episodes of IEED is pharmacological treatment. Several treatment options have been examined. One small study reported clinical success with levodopa at doses up to 1.5 grams per day (Udaka, Yamao, Nagata, Nakamura, & Kameyama, 1984). Improvement has also been observed with multiple doses of tricyclic antidepressants (Robinson et al., 1993; Schiffer, Herndon, & Rudick, 1985). In addition, various selective serotonin reuptake inhibitors have been examined as potential treatments for IEED, including fluoxetine at a dose of 20 mg per day (Seliger, Hornstein, Flax, Herbert, & Schroeder, 1992) and citalopram at 10-20 mg per day (Andersen, Vestergaard, & Riis, 1993). More recently, the combination of dextromethorphan and quinidine (DM/Q; Brooks et al., 2004; Panitch et al., 2006) has been used in a twicedaily compound, consisting of 30 mg of each component. Of these options, only DM/Q is likely to be approved by the U.S. Food and Drug Administration for treatment of IEED. Large, well-controlled studies have confirmed this agent's safety and efficacy (Brooks et al.; Panitch et al.).

Summary

Involuntary emotional expression disorder is a condition that can severely affect a patient's quality of life. The impact of IEED often includes the family and caregiver. The neuroscience nurse is in a unique and opportune position to help patients afflicted by this underrecognized and often misdiagnosed syndrome and intervene for the benefit of the patient. Awareness and recognition of IEED are the first steps to improving quality of life for patients suffering from stroke, dementia, ALS, MS, and a variety of other neurological conditions.

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