Abstract. The main objective of this study was to measure the quality of life (QoL) during a dengue episode. We conducted a facility-based survey in central Brazil in 2005 and recruited 372 laboratory-confirmed dengue patients greater than 12 years of age in hospital and ambulatory settings. We administered the World Health Organization QoL instrument approximately 15 days after the onset of symptoms. We used principal component analysis with varimax rotation to identify domains related to QoL. The median age of interviewees was 36 years. Most (85%) reported their general health status as very good or good before the dengue episode. Although ambulatory patients were mainly classified as having dengue fever, 44.8% of hospitalized patients had dengue hemorrhagic fever or intermediate dengue. Principal component analysis identified five principal components related to cognition, sleep and energy, mobility, self-care, pain, and discomfort, which explained 73% of the variability of the data matrix. Hospitalized patients had significantly lower mean scores for dimensions cognition, self-care, and pain than ambulatory patients. This investigation documented the generally poor QoL during a dengue episode caused by the large number of domains affected and significant differences between health care settings.
A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. A simultaneous improvement of the cognitive abilities allowed for the detection of sensory ataxia of the lower limbs. Sensory ganglionopathy was then detected with electrophysiological studies. A diagnosis of Sjögren syndrome was suspected and confirmed by salivary gland scintigraphy, Schirmer's test, and submaxillary gland biopsy. We report a case of Sjögren syndrome associated with central and peripheral nervous system involvement, without sicca symptoms preceding the neurological clinical picture. The coexistence of ganglionopathy and a favourable response to immunosuppression are key features that can lead to the correct diagnosis in cases with atypical CNS symptoms, mimicking a rapidly progressive dementia.
STX1B is a gene that encodes syntaxin-1B. STX1B mutations have recently been implicated in fever-associated epilepsy syndromes. However, these have not previously been reported in sleep-related hypermotor epilepsy. A 20-year-old man with a strong family history of epilepsy was investigated in our epilepsy monitoring unit due to uncontrolled epilepsy, compatible with sleep-related hypermotor epilepsy. Electroclinical and polygraphic physiological recordings revealed left frontal epileptiform discharges and prominent peri-ictal hypotension. Normal MRI using an epilepsy protocol prompted a search for a genetic epilepsy, which revealed a likely pathogenic mutation in the STX1B gene. The patient remained seizure-free after treatment optimization with carbamazepine. This case suggests that a sleep-related hypermotor epilepsy phenotype can be associated with syntaxin-1B gene mutation, and testing for this gene should be considered in such patients. Furthermore, it may also be concluded that autonomic dysfunction, characterized by peri-ictal hypotension, can also occur in this discorder. [Published with video sequences on www.epilepticdisorders.com]
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