Foix-Chavany-Marie Syndrome (FCMS) is characterized by anarthria and bilateral facio-pharyngo-glosso-masticatory paralysis with an automatic-voluntary dissociation, which usually develops in bilateral opercular lesions. We present a case of FCMS caused by unilateral subcortical lesion. A 54-year-old man was admitted due to acute right hemiparesis with anarthria. He had voluntary facial paresis but automatic-involuntary facial movements were preserved. MRI showed an acute left corona radiata infarction and PET revealed decreased glucose metabolism in left basal ganglia and fronto-parietal lobe.
Basal Ganglia ; Cerebral Infarction ; Cranial Nerve Diseases ; Cranial Nerves ; Dissociative Disorders ; Facial Paralysis ; Glucose ; Humans ; Infarction ; Middle Aged ; Paralysis ; Paresis

Increasing concern is being given to the association between risk of cancer and exposure to low-dose bisphenol A (BPA), especially in young-aged population. In this study, we investigated the effects of repeated oral treatment of low to high dose BPA in juvenile Sprague-Dawley rats. Exposing juvenile rats to BPA (0, 0.5, 5, 50, and 250 mg/kg oral gavage) from post-natal day 9 for 90 days resulted in higher food intakes and increased body weights in biphasic dose-effect relationship. Male mammary glands were atrophied at high dose, which coincided with sexual pre-maturation of females. Notably, proliferative changes with altered cell foci and focal inflammation were observed around bile ducts in the liver of all BPA-dosed groups in males, which achieved statistical significance from 0.5 mg/kg (ANOVA, Dunnett’s test, p<0.05). Toxicokinetic analysis revealed that systemic exposure to BPA was greater at early age (e.g., 210-fold in C(max), and 26-fold in AUC at 50 mg/kg in male on day 1 over day 90) and in females (e.g., 4-fold in C(max) and 1.6-fold in AUC at 50 mg/kg vs. male on day 1), which might have stemmed from either age- or gender-dependent differences in metabolic capacity. These results may serve as evidence for the association between risk of cancer and exposure to low-dose BPA, especially in young children, as well as for varying toxicity of xenobiotics in different age and gender groups.
Animals ; Area Under Curve ; Bile Ducts* ; Bile* ; Body Weight ; Child ; Female ; Humans ; Inflammation ; Liver Neoplasms* ; Liver* ; Male ; Mammary Glands, Human ; Rats* ; Rats, Sprague-Dawley ; Toxicokinetics ; Xenobiotics

BACKGROUND AND PURPOSE: The Literacy-Independent Cognitive Assessment (LICA) has been developed for a diagnosis of dementia and is a useful neuropsychological test battery for illiterate populations as well as literate populations. The objective of this study was to develop the short form of the LICA (S-LICA) and to evaluate the reliability and validity of the S-LICA. METHODS: The subtests of the S-LICA were selected based on the factor analysis and validation study results of the LICA. Patients with dementia (n=101) and normal elderly controls (n=185) participated in this study. RESULTS: Cronbach's coefficient alpha of the S-LICA was 0.92 for illiterate subjects and 0.94 for literate subjects, and the item-total correlation ranged from 0.63 to 0.81 (p<.01).The test-retest reliability of the S-LICA total score was high (r=0.94, p<.001), and the subtests had high test-retest reliabilities (r=0.68-0.87, p<.01). The correlation between the K-MMSE and S-LICA total scores were substantial in both the illiterate subjects (r=0.837, p<.001) and the literate subjects(r=0.802, p<.001). The correlation between the S-LICA and LICA was very high (r=0.989, p<.001). The area under the curve of the receiver operating characteristic was 0.999 for the literate subjects and 0.985 for the illiterate subjects. The sensitivity and specificity of the S-LICA for a diagnosis of dementia were 97% and 96% at the cutoff point of 72 for the literate subjects, and 96% and 93% at the cutoff point of 68 for the illiterate subjects, respectively. CONCLUSIONS: Our results indicate that the S-LICA is a reliable and valid instrument for quick evaluation of patients with dementia in both illiterate and literate elderly populations.
Aged ; Dementia ; Literacy ; Humans ; Neuropsychological Tests ; Reproducibility of Results ; ROC Curve ; Sensitivity and Specificity

OBJECTIVES: It is increasingly thought that the human cerebellum plays an important role in emotion and cognition. Although recent evidence suggests that the cerebellum may also be implicated in fear learning, only a limited number of studies have investigated the cerebellar abnormalities in panic disorder. The aim of this study was to evaluate the cerebellar gray matter deficits and their clinical correlations among patients with panic disorder. METHODS: Using a voxel-based morphometry approach with a high-resolution spatially unbiased infratentorial template, regional cerebellar gray matter density was compared between 23 patients with panic disorder and 33 healthy individuals. RESULTS: The gray matter density in the right posterior-superior (lobule Crus I) and left posterior-inferior (lobules Crus II, VIIb, VIIIa) cerebellum was significantly reduced in the panic disorder group compared to healthy individuals (p < 0.05, false discovery rate corrected, extent threshold = 100 voxels). Additionally, the gray matter reduction in the left posterior-inferior cerebellum (lobule VIIIa) was significantly associated with greater panic symptom severity (r = -0.55, p = 0.007). CONCLUSIONS: Our findings suggest that the gray matter deficits in the posterior cerebellum may be involved in the pathogenesis of panic disorder. Further studies are needed to provide a comprehensive understanding of the cerebro-cerebellar network in panic disorder.
Cerebellum* ; Cognition ; Humans ; Learning ; Panic ; Panic Disorder*

4-Hydroxy-2-(4-hydroxyphenethyl)isoindoline-1,3-dione (PD1) is a synthetic phthalimide derivative of a marine compound. PD1 has peroxisome proliferator-activated receptor (PPAR) γ agonistic and anti-inflammatory effects. This study aimed to investigate the effect of PD1 on allergic asthma using rat basophilic leukemia (RBL)-2H3 mast cells and an ovalbumin (OVA)-induced asthma mouse model. In vitro, PD1 suppressed β-hexosaminidase activity in RBL-2H3 cells. In the OVA-induced allergic asthma mouse model, increased inflammatory cells and elevated Th2 and Th1 cytokine levels were observed in bronchoalveolar lavage fluid (BALF) and lung tissue. PD1 administration decreased the numbers of inflammatory cells, especially eosinophils, and reduced the mRNA and protein levels of the Th2 cytokines including interleukin (IL)-4 and IL-13, in BALF and lung tissue. The severity of inflammation and mucin secretion in the lungs of PD1-treated mice was also less. These findings indicate that PD1 could be a potential compound for anti-allergic therapy.
Animals ; Asthma* ; Basophils ; Bronchoalveolar Lavage Fluid ; Cytokines ; Eosinophils ; In Vitro Techniques ; Inflammation ; Interleukin-13 ; Interleukins ; Leukemia ; Lung ; Mast Cells ; Mice ; Mucins ; Ovalbumin ; Peroxisomes ; Rats ; RNA, Messenger

BACKGROUND: Cognitive intervention (CI) is a nonpharmacological approach used to compensate for cognitive impairment. It is categorized into cognitive training, cognitive stimulation and cognitive rehabilitation. Several studies showed that CI could induce cognitive enhancement and reduction of risk for future cognitive decline in patients with brain injury. We investigated effects of CI on cognitive functions and brain glucose metabolism based on serial cognitive assessments and [18F]-Fluorodexoxyglucose positron emission tomography (FDG-PET) in a patient with carbon monoxide (CO) intoxication. METHODS: A 40-year-old man presented with memory impairment and abnormal behaviors such as apathy, indifference, and perseveration 2-month after CO intoxication. Brain magnetic resonance image (MRI) demonstrated high signal changes in the bilateral basal ganglia, hippocampus and the subcortical white matter on T2 weighted images. FDG-PET also showed glucose hypometabolism in the bilateral hippocampus, basal ganglia, and the subcortical white matter. A detailed neuropsychological evaluation revealed multiple cognitive impairments in memory, language and frontal functions. He received twice a week sessions of 60-minute group-based cognitive intervention for 12 weeks. Several neuropsychological examinations and FDG-PETs were conducted at baseline and after CI. RESULTS: After CI, he showed improvements in memory and frontal functions compared with baseline performances. These cognitive improvements persisted by the 7-month follow-up. The extent of glucose hypometabolism was decreased 1-month after CI, however increased 8-month after CI. CONCLUSIONS: This case study suggested that CI could enhance cognitive functions and improve glucose metabolism in a patient with CO intoxication. Also, the effects of CI on cognitive functions seem to be last at least 7-month after training.
Adult ; Apathy ; Basal Ganglia ; Brain ; Brain Injuries ; Carbon Monoxide* ; Follow-Up Studies ; Glucose ; Hippocampus ; Humans ; Memory ; Metabolism ; Neuronal Plasticity ; Neuropsychology ; Positron-Emission Tomography ; Rehabilitation

Histologic and clinicopathologic findings of a woodchuck (Marmota monax) vertically infected with woodchuck hepatitis virus (WHV) are presented. The liver exhibits marked cirrhotic changes, which is characteristic of the pre-transformation phase of WHV. At necropsy, the woodchuck exhibited ascites and the liver had a grossly nodular appearance. Microscopically, focal hepatocyte necrosis and inflammatory cells were observed in midzonal and periportal areas in the liver. In Macchiavellos stained sections, cytoplasmic inclusion bodies appeared reddish granular materials. We believe that this may represent a new suitable and cost-effective cirrhotic model for the disease processes associated with hepadnaviruses in a number of other species, most notably Hepatitis B virus infection in man.
Alanine Transaminase/blood ; Alkaline Phosphatase/blood ; Animals ; Aspartate Aminotransferases/blood ; Hepatitis B/blood/pathology/*veterinary ; Hepatitis B Virus, Woodchuck/*isolation & purification ; Liver Cirrhosis/blood/pathology/*veterinary ; *Marmota ; Reference Values ; Rodent Diseases/blood/*pathology

The original version of this article contained wrong information of an author which should be changed.

BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type). METHODS: In total, 94 MRI scans (74 scans from bvFTD and 20 scans from age-matched normal controls) were classified into 35 D- and 39 F-type bvFTD cases based on an axial MRI visual rating scale. We compared baseline clinical characteristics, progression in motor and cognitive symptoms, and survival times between D- and F-types. Survival analyses were performed for 62 of the 74 patients. RESULTS: While D-type performed better on neuropsychological tests than F-type at baseline, D-type had higher baseline scores on the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Evaluations of motor progression showed that the disease duration with motor symptoms was shorter in D-type than F-type. Moreover, the survival time was shorter in D-type (6.9 years) than F-type (9.4 years). Cox regression analyses revealed that a high UPDRS Part III score at baseline contributed to an increased risk of mortality, regardless of the pattern of atrophy. CONCLUSIONS: The prognosis is worse for D-type than for those with F-type. Shorter survival in D-type may be associated with the earlier appearance of motor symptoms.
Atrophy* ; Disease Progression ; Frontal Lobe ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Magnetic Resonance Imaging ; Mortality ; Neurobehavioral Manifestations ; Neuropsychological Tests ; Parkinson Disease ; Prognosis*

OBJECTIVE: The aim of this study was to provide normative data on the Literacy Independent Cognitive Assessment (LICA) and to explore the effects of age, education/literacy, and gender on the performance of this test. METHODS: Eight hundred and eighty-eight healthy elderly subjects, including 164 healthy illiterate subjects, participated in this study. None of the participants had serious medical, psychiatric, or neurological disorders including dementia. Bivariate linear regression analyses were performed to examine the effects of age, education/literacy, and sex on the score in each of the LICA cognitive tests. The normative scores for each age and education/literacy groups are presented. RESULTS: Bivariate linear regression analyses revealed that total score and all cognitive tests of the LICA were significantly influenced by both age and education/literacy. Younger and more-educated subjects outperformed older and illiterate or less-educated subjects, respectively, in all of the tests. The normative scores of LICA total score and subset score were presented according to age (60-64, 65-69, 70-74, 75-80, and > or =80 years) and educational levels (illiterate, and 0-3, 4-6, and > or =7 years of education). CONCLUSION: These results on demographic variables suggest that age and education should be taken into account when attempting to accurately interpret the results of the LICA cognitive subtests. These normative data will be useful for clinical interpretations of the LICA neuropsychological battery in illiterate and literate elderly Koreans. Similar normative studies and validations of the LICA involving different ethnic groups will help to enhance the dementia diagnosis of illiterate people of different ethnicities.
Aged* ; Dementia ; Diagnosis ; Education ; Ethnic Groups ; Humans ; Linear Models ; Nervous System Diseases