BACKGROUNDS: Though K-ADL was developed and validated, weighting each item of K-ADL is needed to evaluate the severity of functional disability as a whole. METHODS: Nominal group technique was used to weight individual items of K-ADL. Relevant experts were recruited; one specialist in geriatrics, one expert in elderly health service, one doctor expert in questionnarie, one nurse and two social welfare workers and one caregiver who work in nursing home for elderly, one nurse working at dementia care center. At the first round, each expert assigned the weights of each item. At the second round, those experts met, discussed, and re-evaluated each weighting. Using the newly developed weighted score, we measured its validity compared to brain-disability grade and three experts' decision of severe dysfunction. RESULTS: For inability to perform task, 7 points were given to 'dressing'and 'washing face', 6 points to 'bathing', 9 points to 'feeding', 8 points to 'transfer and toileting'. If any assistance is needed, 7 points were scored to 'dressing', 4 points were scored to 'washing face', 'feeding', 'transfer' and 'toileting', 2 points were scored to 'dressing'. Correlation coefficient between weighted K-ADL sum and brain-disability grade was -0.665(p=0.000). CONCLUSIONS: We assigned differential weighting to each of K-ADL and found that summing weighted K-ADL was excellent in some validity test.
Aged ; Caregivers ; Dementia ; Geriatrics ; Health Services ; Humans ; Nursing Homes ; Social Welfare ; Specialization ; Weights and Measures

No abstract available.
Animals ; Mice* ; Oenanthe*

Coenzyme Q therapy has been used to support metabolic derangements in patients with mitochondrial encephalomyopathies. Biochemical analysis of the living human brain can be performed by magnetic resonance spectroscopy (MRS). We report upon a KSS patient who was serially imaged with localized proton MRS to monitor the efficacy of CoQ treatment. A 17-year-old girl with KSS was serially imaged with localized proton MRS performed on a GE 1.5 T SIGNA MRI/MRS system. The elevated lactate contents of lesions decreased after one month of CoQ therapy but were re-elevated 10 months after treatment. We conclude that MRS presents us with a powerful tool for monitoring the effects of therapeutic trials in mitochondrial encephalomyopathies.
Adolescence ; Brain/metabolism ; Brain/drug effects ; Case Report ; Female ; Human ; Kearns Syndrome/metabolism* ; Kearns Syndrome/drug therapy* ; Kearns Syndrome/diagnosis ; Lactic Acid/metabolism ; Magnetic Resonance Spectroscopy/diagnostic use* ; Pyruvic Acid/metabolism* ; Treatment Outcome ; Ubiquinone/therapeutic use*

'Num-chin syndrome', isolated mental neuropathy, is a rare manifestaion of malignant hernatologic diseases, but important sign for early diagnosis and prediction of clinical course and prognosis. Here we report 7 cases of numb-chin syndrome; one in leukemic transformation of malignant lymphoma, one in blastic crisis of chronic myelogenous leukemia and 5 in acute leukemia Two cases of acute leukemia revealed the 'numb-chin sign' in early course of disease before the diagnosis of leukemia and other three in aggravating state of acute leukemia. The therapeutic response and prognosis was poor and 5 cases expired in a few months.
Diagnosis ; Early Diagnosis ; Hematologic Diseases* ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Lymphoma ; Prognosis

OBJECTIVE: The goal of this study was to examine the colposcopic findings underlying referred cytologic abnormalities classified according to the Bethesda system. Further determine the strength of correlations between the referral smear performed at colposcopy, and the histologic diagnosis. METHODS: The charts of 462 patients who had referred to our hospital due to abnormal cytology at another hospitals from Jan. 1997 to Dec. 2001 at Soonchunhyang University Chunan Hospital were retrospectively reviewed. According to the necessities, repeat Pap, colposcopy-guided biopsy and conization was done. Results were compared both with referral Pap. One way ANOVA and chi-square test were used for statistical analysis. RESULTS: The results were as follows. 1. Mean age of the patients was 46.3 +/- 3.7 years. 2. Mean interval between referral smear and repeat smear or colposcopy was 3-82 days. 3. The agreement between referral and repeat Pap smears was 48.4%, no AGUS smears were conformed by repeat cytology and abnormal referral smears reverted to negative in 36.7%: 28.6% for the women with ASCUS, 50% with AGUS, 60% with LSIL, 36.4% with HSIL, and 13% with cancer. 4. The likelihood of a negative colposcopy guided biopsy of referral Pap smears was 14.3% for the women with ASCUS, 50% with AGUS, 25% with LSIL, 15.4% with HSIL, and 0% with cancer and more severe lesion than LSIL was found in 71.4% with ASCUS, 100% with AGUS, 68.8% with LSIL, 84.6% with HSIL, 100% with cancer. 5. The likelihood of a negative colposcopy guided biopsy of repeat Pap smears was 9.1% for the women with ASCUS, 50% with LSIL, 9.5% with HSIL, and 0% with cancer and more severe lesion than LSIL was found in 90.9% with ASCUS, 50% with LSIL, 90.5 with HSIL, 100% with cancer. 6. The likelihood of a negative conization of referral Pap smears was 12.5% for the women with ASCUS, 33.3% with AGUS, 0% with LSIL, 16.4% with HSIL, and 0% with cancer and more severe lesion than LSIL was found in 87.5% with ASCUS, 66.6% with AGUS, 100% with LSIL, 83.6% with HSIL, 100% with cancer. CONCLUSION: The agreement of referral smears with colposcopic biopsy and conization was 54.3% and 51.9%, respectively. Since the cytology classified according to the Bethesda system does not accurately predict histologic diagnosis, cytologic findings should not be used to determine treatment. Further we should use other methods as colposcopy, punch biopsy and conization to determine treatment.
Biopsy* ; Chungcheongnam-do ; Colposcopy ; Conization ; Diagnosis ; Female ; Humans ; Referral and Consultation ; Retrospective Studies

BACKGROUND AND PURPOSE: Chemokines participate in the regulation of immune and inflammatory responses by interacting with their receptors, which are primarily expressed on immune and inflammatory cells such as B- and T-lymphocytes and antigen-presenting cells. Chemokines and their receptors are therefore considered to mediate inflammation and tissue damage in autoimmune disorders. Chemokine receptor (CCR) genotypes were recently identified, and the importance of their genetic polymorphisms in some autoimmune and infectious disorders has been demonstrated. To define the roles of the polymorphism of the CCR2 gene at codon 64 (CCR2-64I) and the 32-bp deletion in the coding region of CCR5 (CCR5Delta32) in Korean patients with myasthenia gravis (MG), we compared these genotypes in MG cases and healthy controls and investigated the clinical features associated with these genotypes. METHODS: One hundred and fifteen healthy controls (51 men and 64 women) and 109 MG patients (44 men and 65 women) from three University hospitals were included. We examined each patient for clinical features using electrophysiology tests, laboratory tests, and thymic pathology. The CCR2-64I and CCR5Delta32 polymorphisms were determined by the PCR-RFLP method. RESULTS: We detected no difference in the frequencies of CCR2-64I polymorphism between MG patients and healthy controls. All of the MG patients and the healthy controls were homozygous for the wild-type CCR5 genotype. The results of electrophysiological tests and thymic pathologies were not influenced by the type of CCR2-64I polymorphism. However, the anti-acetylcholine-receptor (AChR) antibody titer was higher in the CCR2 G/G genotype (13.34+/-12.71 nmol/L) than in the CCR2 A/A genotype (5.83+/-2.56 nmol/L). CONCLUSIONS: We found no evidence of an increased risk for MG associated with the CCR2-64I and CCR5Delta32 polymorphisms. However, the increased anti-AChR antibody titer in the patients with the CCR2 G/G genotype suggests that the CCR2 gene play a role in the pathophysiology of MG.
Antigen-Presenting Cells ; Chemokines ; Clinical Coding ; Codon ; Electrophysiology ; Genotype ; Hospitals, University ; Humans ; Inflammation ; Male ; Myasthenia Gravis* ; Pathology ; Polymorphism, Genetic ; T-Lymphocytes

BACKGROUND AND PURPOSE: Chemokines participate in the regulation of immune and inflammatory responses by interacting with their receptors, which are primarily expressed on immune and inflammatory cells such as B- and T-lymphocytes and antigen-presenting cells. Chemokines and their receptors are therefore considered to mediate inflammation and tissue damage in autoimmune disorders. Chemokine receptor (CCR) genotypes were recently identified, and the importance of their genetic polymorphisms in some autoimmune and infectious disorders has been demonstrated. To define the roles of the polymorphism of the CCR2 gene at codon 64 (CCR2-64I) and the 32-bp deletion in the coding region of CCR5 (CCR5Delta32) in Korean patients with myasthenia gravis (MG), we compared these genotypes in MG cases and healthy controls and investigated the clinical features associated with these genotypes. METHODS: One hundred and fifteen healthy controls (51 men and 64 women) and 109 MG patients (44 men and 65 women) from three University hospitals were included. We examined each patient for clinical features using electrophysiology tests, laboratory tests, and thymic pathology. The CCR2-64I and CCR5Delta32 polymorphisms were determined by the PCR-RFLP method. RESULTS: We detected no difference in the frequencies of CCR2-64I polymorphism between MG patients and healthy controls. All of the MG patients and the healthy controls were homozygous for the wild-type CCR5 genotype. The results of electrophysiological tests and thymic pathologies were not influenced by the type of CCR2-64I polymorphism. However, the anti-acetylcholine-receptor (AChR) antibody titer was higher in the CCR2 G/G genotype (13.34+/-12.71 nmol/L) than in the CCR2 A/A genotype (5.83+/-2.56 nmol/L). CONCLUSIONS: We found no evidence of an increased risk for MG associated with the CCR2-64I and CCR5Delta32 polymorphisms. However, the increased anti-AChR antibody titer in the patients with the CCR2 G/G genotype suggests that the CCR2 gene play a role in the pathophysiology of MG.
Antigen-Presenting Cells ; Chemokines ; Clinical Coding ; Codon ; Electrophysiology ; Genotype ; Hospitals, University ; Humans ; Inflammation ; Male ; Myasthenia Gravis* ; Pathology ; Polymorphism, Genetic ; T-Lymphocytes

No abstract available.
Arteries* ; Infarction* ; Spinal Cord Vascular Diseases ; Spinal Cord*

We report two cases of porphyric peripheral neuropathy in a 19-year-old male with variegate porphyria and in a 39 year-old male with intermittent acute prophyria. Clinically, there were sensory, motor disturbance and autonomic symptoms including decreased sweating, urinary and sphinctor distrubances. Variegate porphyria showed facial diplegia and positive family history inherited by autosomal dominent trait. Intermittent acute porphyria was combined-with SIADH. Both cases were expired due to respiratory failure. Nerve conduction studies were carried out in two cases and both cases showed slow motor, sensory nerve conductlon velocity ,and significant low CMAPs(Compound Muscle Action Potentials). Sural nerve biopsy was carried out in a variegate prophyria compared with one normal control. Decreased large myelinated fibers was found. In nerve fiber teased study. 8.5% of nerve fibers showed axonal degenration and only 2.3% of the segmental demyelination. There findings are suggesting that the porphyric neuro might be the axonal type.and severe neuropathy in a sign of poor prognosis.
Adult ; Axons ; Biopsy ; Demyelinating Diseases ; Humans ; Inappropriate ADH Syndrome ; Male ; Myelin Sheath ; Nerve Fibers ; Neural Conduction ; Peripheral Nervous System Diseases ; Porphyria, Acute Intermittent ; Porphyria, Variegate ; Prognosis ; Respiratory Insufficiency ; Sural Nerve ; Sweat ; Sweating ; Young Adult

Postpartum cerebral angiopathy is a benign, reversible clinicoradiologic syndrome. The clinical significance of this syndrome is the difficulty in differentiating it from other severe disorders such as subarachnoid hemorrhage and cere-bral vasculitis. A 25-year-old woman who developed a severe headache, transient total blindness ,and generalized tonic clonic seizures 14 days postpartum was admitted to our hospital. A MRI of the brain revealed a bilateral anterior cere-bral artery (ACA) infarction. A cerebral angiography showed the narrowing of the M1 and M2 segments of the left middle cerebral artery (MCA), the right MCA M1 segment, and the A1 and proximal A2 segments of the right ACA. Also, it showed the narrowing of the proximal basilar artery portion from which the anterior inferior cerebellar artey arose, P1, P2, and P3 segments of the left posterior cerebral artery (PCA), and the right PCA P1 segment. With the treatment of nimodipine, the abnormalities on these studies were markedly improved two weeks later.
Adult ; Arteries ; Basilar Artery ; Blindness ; Brain ; Cerebral Angiography ; Cerebrovascular Disorders ; Female ; Headache ; Humans ; Infarction ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Nimodipine ; Passive Cutaneous Anaphylaxis ; Posterior Cerebral Artery ; Postpartum Period* ; Pregnancy Complications ; Seizures ; Subarachnoid Hemorrhage ; Vasculitis