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• W2607750650 abstract "[ILLUSTRATION OMITTED] Disruption of neural circuitry undermines voluntary control of affect Pseudobulbar affect (PBA) is a disorder of affective expression that manifests as stereotyped and frequent outbursts of crying (not limited to lacrimation) or laughter. Symptoms are involuntary, uncontrolled, and exaggerated or incongruent with current mood. Episodes, lasting a few seconds to several minutes, may be unprovoked or occur in response to a mild stimulus, and patients typically display a normal affect between episodes. (1) PBA is estimated to affect 1 to 2 million people in the United States, although some studies suggest as many as 7 million, (1,2) depending on the evaluation method and threshold criteria used. (3) Many terms have been used to describe aspects of PBA (Table 1 (4) and Box, page 58 (5-10)). This abundance of often conflicting terminology is thought to have impeded efforts to categorize emotional expression disorders, determine their prevalence, and evaluate clinical evidence of potential therapeutic options. (11) Where to look for pseudobulbar affect PBA has been most commonly described in major neurologic disorders: * Alzheimer's disease * amyotrophic lateral sclerosis (ALS) * multiple sclerosis (MS) * Parkinson's disease * stroke * traumatic brain injury (TBI). Of these disorders, most studies have found the highest PBA prevalence in patients with ALS and TBI, with lesser (although significant) prevalence in Parkinson's disease (Table 2, page 59). (1,12) These big 6 diagnoses are not a comprehensive list, as many other disease states are associated with PBA (Table 3, page 59). (12-14) As PBA has become better defined and more widely recognized, additional sequelae have been described. PBA's sporadic and unpredictable nature and the potential embarrassment and distress of public outbursts may lead to an agoraphobia-like response. (15) People with PBA report a significantly worse subjective assessment of general health, quality of life, relationships, and work productivity compared with people with similar primary underlying diagnoses without PBA. (16) 2 Pathways: 'Generator' and 'governor' Despite the many and varied injuries and illnesses associated with PBA, Lauterbach et al (10) noted patterns that suggest dysregulation of 2 distinct but interconnected brain pathways: an emotional pathway controlled by a separate volitional pathway. Lesions to the volitional pathway (or its associated feedback or processing circuits) are thought to cause PBA symptoms. To borrow an analogy from engineering, the emotional pathway is the generator of affect, whereas the volitional pathway is the governor of affect. Thus, injury to the governor results in overspill, or overflow, of affect that usually would be suppressed. The emotional pathway, which coordinates the motor aspect of reflex laughing or crying, originates at the frontotemporal cortex, relaying to the amygdala and hypothalamus, then projecting to the dorsal brainstem, which includes the midbrain-pontine periaqueductal gray (PAG), dorsal tegmentum, and related brainstem. The volitional pathway, which regulates the emotional pathway, originates in the dorsal and lateral frontoparietal cortex, projects through the internal capsule and midbrain basis pedunculi, and continues on to the anteroventral basis pontis. The basis ponds then serves as an afferent relay center for cerebellar activity. Projections from the pons then regulate the emotional circuitry primarily at the level of the PAG. (10) Lesions of the volitional pathway have been correlated with conditions of PBA, whereas direct activation of the emotional pathway tended to lead to emotional lability or the crying and laughing behaviors observed in dacrystic or gelastic epilepsy. (10) The pivotal nature of the regulation occurring at the PAG has guided treatment options. …" @default.
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• W2607750650 date "2016-09-01" @default.
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• W2607750650 title "Pseudobulbar Affect: When Patients Laugh or Cry, but Don't Know Why" @default.
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