An 86-year-old woman admitted in our geriatric inpatient unit exhibited frequent episodes of involuntary, uncontrolled, particularly disturbing outbursts of crying and, less frequently, uncontrolled episodes of laughing. She also had episodes of wandering at night. The situation was becoming untenable and could not be managed by her husband anymore. Past medical history was positive for hypertension, osteoporosis, gait abnormality, nocturnal urinary incontinence, and Alzheimer's disease (AD) with related behavioral problems. Her medications included amlodipine (2.5 mg daily), venlafaxine (75 mg daily), quetiapine (25 mg at bedtime), and calcium/vitamin D (500 mg/400 units daily). Parkinsonism was present on physical examination.
The initial workup included biochemical and laboratory investigation to exclude metabolic, inflammatory, hormonal, and toxic causes for dementia. All results were within normal limits. This patient had an obvious adverse impact on activities of daily living and, as she had a progressive and severe multi-domain cognitive decline, she could not perform an MMSE (Mini-Mental State Examination). Brain computerized tomography (CT) scan performed 4 years earlier showed mild cerebral atrophy and leukoaraiosis in the white matter regions of the brain (Figure 1). An electromyogram was performed to rule out motoneuron disease, which was negative. Magnetic resonance imaging (MRI) revealed nonspecific subcortical signal abnormalities of the white matter associated with subcortical ischemic changes and a left-sided cerebellar lacuna (Figure 2). Brain positron emission tomography (PET) with fluoro-2-deoxy-d-glucose (FDG) was in favor of a mixed neurodegenerative disorder, showing an altered metabolic pattern suggestive of AD dementia or/and dementia with Lewy bodies (DLB). DLB could explain the parkinsonism symptoms observed in the patient. She was seen by a psychiatrist who diagnosed dysregulation of affective expression without evidence of significant depressive or anxious symptoms. Following evaluation by a neurologist, PBA secondary to a mixed neurodegenerative disorder was considered the likely diagnosis. Imaging with dopamine transporter agents would have been helpful to obtain a more precise diagnosis, but it is not available at our center.
Venlafaxine was stopped to try different medications. Table 1 describes the medication trials received by the patient during her hospitalization in order to reduce her crying and laughing symptoms. DM/Q was determined to be the most effective medication. A mild improvement was observed by the husband following 3 days of treatment. After 1 week, the effect was more apparent, as crying episodes were less frequent and of shorter duration. After 2 weeks, the crying episodes were reduced by 50% and wandering at night was less disturbing. At that point, the patient was discharged home with DM/Q and then 1 year later moved to a nursing home, where she died a few weeks after admission.