Atypical absence is less understood than typical absence. Several conditions that produce atypical absence are known including Lennox-Gastaut syndrome, myoclonic astatic epilpsy and epileptic encephalopathy with continuous spike and waves in slow wave sleep. A 17-year-old girl with mental retardation had developed frequent loss of consciousness and occasional falling attack with traumatic facial injury for 2 years. The interictal EEG showed 2 Hz slow spike-and-wave complex with maximum over right frontotemporal area and the brain MRI was normal. Carbamazepin was prescribed initially but the drug seemed to worsen the seizures. Long term video-EEG monitoring showed very frequent atypical absence seizures consisting of sudden hypotonia of head and oral automatism with or without secondary generalization. Generalized 2 to 2.5 Hz slow spike-and-wave complexes with duration of 10 to 40 seconds were seen during ictal period. About 10% to 20% of the non REM sleep was occupied with generalized slow spike-and-wave complex and/or polyspikes or polyspikes-and-wave complex with duration of within 1 second. Valprorate monotherapy had failed, then lamotrigin was added. In spite of polytherapy, the seizure was intractable. We think this intractable atypical absence might be associated with juvenile onset Lennox-Gastaut syndrome.
Adolescent ; Automatism ; Brain ; Electroencephalography ; Epilepsy, Absence ; Facial Injuries ; Female ; Generalization (Psychology) ; Head ; Humans ; Intellectual Disability ; Magnetic Resonance Imaging ; Muscle Hypotonia ; Seizures ; Sleep, REM ; Unconsciousness

Metronidazole-induced encephalopathy (MIE) can be caused by excessive dose or prolonged metronidazole administration. The signal abnormalities in the cerebellar dentate nuclei, midbrain, dorsal pons and corpus callosum on magnetic resonance imaging are considered as the characteristic feature of MIE. Although the mechanism of MIE remains to be elucidated, various hypothesis have been proposed including the role of metronidazole as a thiamine antagonist. Here we report a 58-year-old woman with MIE who coincidentally presented with thiamine deficiency.
Brain Diseases ; Corpus Callosum ; Female ; Humans ; Magnetic Resonance Imaging ; Mesencephalon ; Metronidazole ; Middle Aged ; Pons ; Thiamine Deficiency ; Thiamine

A 67-year-old male visited Pusan Veterans Hospital due to general weakness and weight loss for 6 months. Physical examination showed non-tender 4 finger breaths sized splenomegaly and both inguinal and cervical lymphadenopathy. The white blood cell count was 25,300/uL with 91% morphologically mature lymphocytes. Bone marrow aspirate revealed hypercellularity with 74.5% lymphocytes morphologically similar to peripheral lymphocytes. The immunophenotpying study of lymphocytes displayed the phenotype of CD19(+), CD20(+), HLA-DR(+), sIg(+) but CD5(-). We concluded that this patients's diagnosis is CD 5 negative B-cell chronic lymphocytic leukemia.
Aged ; B-Lymphocytes* ; Bone Marrow ; Busan ; Diagnosis ; Fingers ; Hospitals, Veterans ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell* ; Leukocyte Count ; Lymphatic Diseases ; Lymphocytes ; Male ; Phenotype ; Physical Examination ; Splenomegaly ; Weight Loss

The type III histone deacetylase silent information regulator 1 (SIRT1) is an enzyme that is critical for the modulation of immune and inflammatory responses. However, the data on its role in rheumatoid arthritis (RA) are limited and controversial. To better understand how SIRT1 regulates adaptive immune responses in RA, we evaluated collagen-induced arthritis (CIA) in myeloid cell-specific SIRT1 knockout (mSIRT1 KO) and wild-type (WT) mice. Arthritis severity was gauged on the basis of clinical, radiographic and pathologic scores. Compared with their WT counterparts, the mSIRT1 KO mice exhibited less severe arthritis, which was less destructive to the joints. The expression levels of inflammatory cytokines, matrix metalloproteinases and ROR-γT were also reduced in the mSIRT1 KO mice compared with the WT mice and were paralleled by reductions in the numbers of Th1 and Th17 cells and CD80- or CD86-positive dendritic cells (DCs). In addition, impaired DC maturation and decreases in the Th1/Th17 immune response were observed in the mSIRT1 KO mice. T-cell proliferation was also investigated in co-cultures with antigen-pulsed DCs. In the co-cultures, the DCs from the mSIRT1 KO mice showed decreases in T-cell proliferation and the Th1/Th17 immune response. In this study, myeloid cell-specific deletion of SIRT1 appeared to suppress CIA by modulating DC maturation. Thus, a careful investigation of DC-specific SIRT1 downregulation is needed to gauge the therapeutic utility of agents targeting SIRT1 in RA.
Animals ; Arthritis ; Arthritis, Experimental* ; Arthritis, Rheumatoid ; Coculture Techniques ; Cytokines ; Dendritic Cells* ; Down-Regulation ; Histone Deacetylases ; Joints ; Matrix Metalloproteinases ; Mice* ; T-Lymphocytes ; Th17 Cells