A cry for help: Treating involuntary emotional expression disorder Pharmacotherapy can lessen the impact of uncontrollable laughing or crying rs. R, a 68-year-old retired teacher,® isDowden referred Health Media to you for suspected mania after a closed head Minjury from a car accident. The referring physician reports that Mrs. R experiencedCopyright mild anterogradeFor personal amnesia use only that has resolved, but she continues to suff er from “persistent mood swings as evidenced by substantial inappropriate laughter.” Mrs. R is not manic. Her mood is normal, with a relatively euthymic aff ect. When asked about her accident or injury, however, she breaks into bouts of laughter PSYCHIATRY that appear to be uncontrollable and last up to several minutes. These episodes include respiratory changes CURRENT that make her laughter nearly indistinguishable from FOR crying. Mrs. R explains that the episodes occur every time she discusses the accident—regardless of her eff orts ORVIDAS KEN to prevent them—and complains they are extremely frustrating and embarrassing. She avoids situations that Joshua D. Grill, PhD might trigger the episodes. Director, recruitment and education core Alzheimer Disease Center, Department of neurology Jeff rey L. Cummings, MD Patients with involuntary emotional expression dis- Augustus S. Rose Professor of Neurology order (IEED)—a neurologic disorder that manifests Director, Alzheimer Disease Center, Department of neurology as brief bouts of uncontrollable crying, laughing, or Professor of psychiatry and biobehavioral science both—may appear to have bipolar disorder, schizo- phrenia, depression, or another psychiatric disorder. David Geff en School of Medicine at UCLA Careful evaluation, however, can distinguish IEED Los Angeles from other conditions. Managing the disorder requires an understanding of IEED phenomenology, including: • neurologic conditions that result in IEED • underlying pathology • diagnostic criteria Current Psychiatry • effective treatments. Vol. 7, No. 3 101 continued For mass reproduction, content licensing and permissions contact Dowden Health Media. Table 1 that eventually can affect mood and often Neurologic conditions cause patients to avoid social interaction.3 associated with IEED IEED can occur in any condition that damages and affects the brain areas criti- Amyotrophic lateral sclerosis cal to emotional motor output (Box 1).4-6 Multiple sclerosis The broad pattern of lesions that can result in IEED stems from many disease states. Traumatic brain injury Treating IEED IEED is often observed in amyotrophic Stroke lateral sclerosis (ALS), multiple sclerosis Alzheimer’s disease (MS), stroke, and traumatic brain injury. It Frontotemporal dementia also may occur in dementia, Parkinson’s 7 Parkinson’s disease disease, and other disorders (Table 1). Progressive supranuclear palsy Multiple systems atrophy Diagnosis can be elusive Wilson’s disease Although IEED is not included in DSM-IV- Clinical Point Normal pressure hydrocephalus TR, recently developed diagnostic criteria If a patient presents Olivopontine cerebellar atrophy can help distinguish it from other disor- ders (Table 2).1 As with DSM-categorized with symptoms Source: Reference 7 disorders, IEED must result in clinically that suggest IEED, signifi cant distress or impairment in social Table 2 fi rst determine the or occupational function and must not be neurologic condition Is it IEED? Diagnostic criteria better accounted for by another disorder or caused by a physiologic substance. that is causing them Presence of brain damage The patient must present with symp- Episodes of involuntary emotional motor toms caused by brain dysfunction from output that: brain injury or neurodegenerative disease. • represent a change from normal emotional reactivity Underlying brain damage might not be • are independent or in excess of provoking apparent when the patient fi rst presents, stimuli but to our knowledge no case of idiopathic • result in clinically signifi cant distress or social IEED has been described. If a patient pres- or functional impairment ents with symptoms thought to be IEED, Disorder is not: fi rst determine what underlying neurolog- • better accounted for by another neurologic ic condition is causing the symptoms and or psychiatric disorder optimally manage this disorder. • caused by a physiologic substance To be considered IEED, the patient’s Source: Reference 1 symptoms must represent a change from his or her normal emotional reactivity. Brain dysfunction alters aff ect When interviewing patients and their fam- IEED was introduced as an inclusive term, ilies, compare the patient’s current emo- replacing previous nomenclature such as tional reactivity with that from when he pathologic laughing and crying, pseudo- or she was free of all disease symptoms. bulbar affect, affective lability, and emo- Such considerations are important because tional incontinence.1 a patient may have a life-long condition IEED can present as episodes of laugh- in which he or she is prone to emotional ter, as in Mrs. R’s case, but more commonly displays—such as essential crying—that is manifests as bouts of crying. Other presen- distinct from IEED.8 tations include a combination of laughing Symptoms must be incongruent with and crying, but episodic outbursts of other or in excess of the person’s underlying emotions that are out of the patient’s con- mood and independent or in excess of the trol—such as anger—can be included in provoking stimulus. Inappropriateness of this syndrome.2 IEED episodes can lead the emotional response is the hallmark of Current Psychiatry 102 March 2008 to embarrassment, frustration, and anger IEED. Box 1 IEED: A consequence of brain pathology amage to the descending inputs cerebellar network.5 Single lesions to white Dto the pontomedullary area once matter structures—such as the internal referred to as the faciorespiratory center capsule—and gray matter structures—such is most likely to result in release of bulbar as the thalamus, hypothalamus, basal function and, subsequently, involuntary ganglia, cerebellum, and several cortical emotional expression disorder (IEED). locations—have been associated with IEED. Therefore, because of the progressive upper Bilateral lesions are more likely to produce motor neuron degeneration associated the disorder than single lesions. with amyotrophic lateral sclerosis (ALS), With such varied neuroanatomic nearly 50% of ALS patients will eventually substrates, predicting the underlying demonstrate pathological affect.4 neurochemical pathology of IEED is diffi cult. The lesions that can result in IEED are Among the neurotransmitters considered in diffuse, however, and have been described in IEED pathology and treatment are serotonin, a review of IEED neuroanatomy as including glutamate, and dopamine. The sigma-1 a cortico-limbic-subcortico-thalamo-ponto- receptor system may also play a role.6 Clinical Point Inappropriate Table 3 emotional response IEED episodes have characteristic clinical features (Table 3). They are brief—last- Characteristics of IEED is the hallmark of ing seconds to minutes—and sudden in episodes IEED onset and conclusion. Episodes are likely to be stereotyped in severity and present- Paroxysmal, sudden onset with rapid offset ing type within patients, as well as in the Brief (up to several minutes) triggering stimulus or set of stimuli. For Stereotyped across patients (may manifest example, patients often experience epi- in similar fashion from patient to patient) sodes when asked about the syndrome.9 In Stereotyped within patients (episodes often severe cases, patients experience episodes have similar type, severity, and eliciting stimuli) with any interpersonal contact.10 Some characteristics support—but are priately) will confi rm this misperception, not essential for—an IEED diagnosis: even if the patient claims otherwise. The • autonomic symptoms, such as fl ush- hallmark distinctions between depression ing of the face and increased salivary and IEED are: production during episodes • duration of crying • pseudobulbar signs, such as increased • associated mood state. jaw jerk, exaggerated gag refl ex, dys- Major depressive disorder (MDD) is a arthria, and dysphagia persistent change in a patient’s mood last- • other emotional outbursts. ing weeks to months, accompanied by feel- ings of guilt, helplessness, hopelessness, CASE CONTINUED and worthlessness, apathy, and anhedonia.11 Reaching a diagnosis IEED is paroxysmal, with uncontrollable After thoroughly interviewing Mrs. R, you ex- changes in affect without a corresponding clude mood disorders such as depression or bi- sudden mood change. Patients may report polar disorder. The paroxysmal, episodic nature mood changes during episodes, but between of her emotional outbursts and the consistency episodes return to an euthymic affect. of the eliciting stimulus, suggest IEED. Patients who suffer from MDD, however, are not excluded from an IEED diagnosis. Distinguishing IEED from depression. In 1 small study, almost one-half of patients Physicians may be quick to diagnose a pa- with IEED also had major depression.12 Dif- tient with consistent, recurrent crying as ferentiating these syndromes—even in pa- having a depressive disorder. In IEED, the tients who suffer from both— is important to Current Psychiatry patient’s family commonly (and inappro- ensure proper management and patient and Vol. 7, No. 3 103 Table 4 IEED: Evidence for antidepressants Drug Study design/population Dosage Outcome Tricyclics Amitriptyline