Carbamazepine-induced toxic effects and HLA-B*1502 screening in Taiwan.

BACKGROUND: Carbamazepine, an anticonvulsant and a mood-stabilizing drug, is the main cause of the Stevens-Johnson syndrome (SJS) and its related disease, toxic epidermal necrolysis (TEN), in Southeast Asian countries. Carbamazepine-induced SJS-TEN is strongly associated with the HLA-B*1502 allele. We sought to prevent carbamazepine-induced SJS-TEN by using HLA-B*1502 screening to prospectively identify subjects at genetic risk for the condition. METHODS: From 23 hospitals in Taiwan, we recruited 4877 candidate subjects who had not taken carbamazepine. We genotyped DNA purified from the subjects' peripheral blood to determine whether they carried the HLA-B*1502 allele. Those testing positive for HLA-B*1502 (7.7% of the total) were advised not to take carbamazepine and were given an alternative medication or advised to continue taking their prestudy medication; those testing negative (92.3%) were advised to take carbamazepine. We interviewed the subjects by telephone once a week for 2 months to monitor them for symptoms. We used the estimated historical incidence of SJS-TEN as a control. RESULTS: Mild, transient rash developed in 4.3% of subjects; more widespread rash developed in 0.1% of subjects, who were hospitalized. SJS-TEN did not develop in any of the HLA-B*1502-negative subjects receiving carbamazepine. In contrast, the estimated historical incidence of carbamazepine-induced SJS-TEN (0.23%) would translate into approximately 10 cases among study subjects (P<0.001). CONCLUSIONS: The identification of subjects carrying the HLA-B*1502 allele and the avoidance of carbamazepine therapy in these subjects was strongly associated with a decrease in the incidence of carbamazepine-induced SJS-TEN. (Funded by the National Science Council of Taiwan and the Taiwan Drug Relief Foundation.).

Isolated sleep paralysis linked to impaired nocturnal sleep quality and health-related quality of life in Chinese-Taiwanese patients with obstructive sleep apnea.

PURPOSE: Isolated sleep paralysis (ISP) is a rapid eye movement (REM) sleep parasomnia and has a special meaning in Chinese population. Worsening of obstructive sleep apnea (OSA) occurs especially during REM sleep. The relationship between ISP and OSA is unclear. The aim of this study was to investigate the impact of ISP on sleep and life quality in Chinese-Taiwanese OSA patients. METHODS: We recruited 107 OSA patients diagnosed by polysomnography (PSG) in Southern Taiwan. ISP was evaluated by self-reported sleep questionnaire. We used Chinese version of Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), and Short-Form 36 (SF36) to evaluate daytime sleepiness, nocturnal sleep quality, and health-related quality of life, respectively for OSA patients. Student's t-test was used to compare PSG parameters, ESS, PSQI, physical and mental component of SF-36 (P-SF36 and M-SF36) between OSA patients with and without ISP. Stepwise multiple regression was used to find out the factors independently associated with ESS, PSQI, P-SF36, and M-SF36. RESULTS: Forty-one of 107 patients (38.3%) had ISP. It showed no significant difference in PSG parameters between OSA patients with and without ISP. OSA patients with ISP had significantly higher ESS (P = 0.010), higher PSQI (P = 0.007), lower P-SF36 (P = 0.020), and lower M-SF36 (P = 0.001) than those without ISP. ISP was an independent factor associated with ESS (P = 0.017), PSQI (P = 0.001), and M-SF36 (P = 0.030) after adjusting for other confounding variables. CONCLUSIONS: ISP was independently associated with excessive daytime sleepiness, worse sleep quality, and impaired mental health-related quality of life in Chinese-Taiwanese OSA patients.

Subclinical white matter integrity in subjects with cumulative lead exposure.

PURPOSE: To evaluate microstructural changes in the white matter of patients who were exposed to lead and to compare differences in fractional anisotropy (FA) between these patients and control subjects. MATERIALS AND METHODS: Institutional review board approval and subject informed consent were obtained for this HIPAA-compliant study. Nineteen factory workers who had been exposed to lead and 18 healthy volunteers who had not were enrolled. FA values and T2-weighted fluid-attenuation inversion-recovery magnetic resonance images were obtained at several regions of interest (the bilateral parietal, frontal, occipital, and temporal white matter and the genu and splenium of the corpus callosum). Lead levels were measured in the blood, midtibia, and patella. The Student t test was used to compare the difference in continuous variables between the two groups. Pearson correlation coefficients were used to assess the association between two variables. RESULTS: There were no significant differences in sex, age, body mass index, smoking history, betel nut consumption, or alcohol consumption between the factory workers and the volunteers. The number of milk drinkers among factory workers was significantly higher than that among volunteers (P < .001). The factory workers had significantly higher blood (P < .001), patella (P < .001), and midtibia (P = .005) lead levels than did the volunteers. Mean FA in the factory workers was lower than that in the volunteers at the same anatomic location; significant differences between the groups were noted bilaterally in the parietal, frontal, occipital, and temporal white matter. There was no significant difference in mean diffusivity values and mean T2 ratios between the factory workers and the volunteers. CONCLUSION: Decreased FA was associated with exposure to lead. Negative correlations between FA and blood, midtibia, and patella lead levels suggest that FA may be a useful index of early white matter damage. SUPPLEMENTAL MATERIAL: http://radiology.rsnajnls.org/cgi/content/full/2522080653/DC1.

Electroencephalography burst suppression in a patient with dengue encephalopathy: A case report.

OBJECTIVE: To report a rare case of dengue fever presenting with reversible encephalopathy and burst suppression in the EEG. METHODS: We describe an 85-year-old man with dengue encephalopathy and EEG burst suppression and review the related literature. RESULTS: The patient presented with an acute comatose state and absent brainstem reflexes. Blood pressure, pulse rate, temperature and oxygen saturation were 94/49mmHg, 82beats/min, 34.7 degrees C, and 83% respectively. Thrombocytopenia and skin rash were found initially. Oxygen saturation was 100% immediately after endotracheal intubation and mechanical ventilation. On the second day he remained comatose with EEG burst suppression but blood pressure and temperature were within normal limits. He regained consciousness and was alert on the 12th day and there was normalization of EEG and thrombocytopenia. CONCLUSIONS: Patients with dengue fever may present as encephalopathy with EEG burst suppression. SIGNIFICANCE: EEG burst suppression does not necessarily suggest a poor prognosis in patients with dengue fever.

Dengue fever with ischemic stroke: a case report.

BACKGROUND: Dengue fever, especially dengue hemorrhagic fever, often presents with thrombocytopenia and hemorrhagic phenomenon. Neurologic manifestations are uncommon. REVIEW SUMMARY: We report a case of dengue fever with thrombocytopenia and ischemic stroke. Only supportive treatment was instituted. The neurologic deficits of the patient were initially progressive and then improved. The platelet count correlated well with the disease severity and the neurologic deficits. The time course was compatible with the critical stage in dengue hemorrhagic fever. Some studies show that immunomodulators or immunosuppressants may reverse thrombocytopenia such that the bleeding episodes improve rapidly. CONCLUSION: Dengue fever may present as ischemic stroke.

Cognitive dysfunction after acute lacunar infarct.

Vascular dementia and vascular cognitive impairment have attracted more attention recently due to their association with increased risk of death and institutionalization. The purpose of the present study was to detect and identify the characteristics of cognitive impairments during the early stage of lacunar stroke. The subjects consisted of 23 consecutive first-ever acute lacunar infarction patients who were admitted to the Department of Neurology, Kaohsiung Municipal Hsiao-Kang Hospital, Taiwan, from November 2001 to October 2002. The National Institutes of Health Stroke Scale and Cognitive Abilities Screening Instrument (CASI) were used to evaluate stroke severity and cognitive function, and assessments were performed by a neurologist and psychologist, within 10 days of stroke onset. Of the 23 patients, 21 (91.3%) had CASI scores below their respective cutoff values and all patients had cognitive impairment in at least one cognitive domain in CASI. There were no significant correlations between CASI abnormality (below the cutoff value) and patient age, education, or the interval from stroke onset. Recent memory impairment was the most often impaired cognitive domain on CASI (19 patients, 82.6%). There were significant correlations between recent memory and "attention or concentration"(correlation coefficient, 0.52; p < 0.05), and "abstraction and judgment" (correlation coefficient, 0.44; p < 0.05). The correlations between recent memory and other domains were not significant. It was concluded that cognitive impairment after acute lacunar infarct is quite common and recent memory is the most often impaired cognitive domain. This may have been caused by the location of the specific lesion as well as by the impairment in "attention or concentration" or "abstraction and judgment".

Giant cell arteritis with multiple cranial nerve palsy and reversible proptosis: a case report.

Giant cell arteritis (GCA) often presents with symptoms of headache, jaw claudication, polymyalgia rheumatica, and blurred vision. GCA is relatively rare and may have atypical manifestations in Asians, including multiple cranial nerve palsy and reversible proptosis. A high suspicion of GCA is suggested when any older Asian suffers from headache that is new-onset or different from the previous pattern, even without other typical manifestations of GCA.