Chronic inflammatory demyelinating polyneuropathy (CIDP) has been rarely reported in systemic sclerosis (SSc). We describe the clinical, electrophysiological, and pathologic findings in a 73-year-old female with established limited cutaneous SSc who later developed CIDP. The patient had progressive limb weakness, sensory loss, and slow nerve conduction velocities. Sural nerve biopsy showed evidence of demyelination and remyelination. This case demonstrates that chronic demyelinating polyneuropathy can be an unusual manifestation of SSc, presumably resulting from an immune-mediated process.
Aged ; Autoimmunity ; Biopsy ; Demyelinating Diseases ; Extremities ; Female ; Humans ; Neural Conduction ; Polyneuropathies* ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Scleroderma, Systemic* ; Sural Nerve

BACKGROUND: There is strong evidence for the efficacy of stroke unit (SU) care, which has become a common practice in other developed countries but is available only in a small number of centers in Korea. This late introduction of SU care in Korea can be at least partly attributed to doubts about its benefits on patient outcomes compared to general ward (GW) care in our routine practice. METHODS: Based on a prospective stroke registry, we identified 1,300 acute stroke patients hospitalized in either SUs (n=195) or GWs (n=1,105) from January 2004 to June 2008. Clinical outcomes such as fatality and disability rates were compared using propensity score matching in the total cohort. Additional statistical adjustments were performed using stepwise logistic regression analyses with the backward elimination method for potential confounders. RESULTS: The baseline characteristics did not differ significantly between patients admitted to SUs (n=195) and their matched controls (n=386). The 3-month dependency rate was 17.4% in SUs and 21.0% in GWs (p=0.31), and the case fatality rates were 1.5% and 8.0% (p<0.001), respectively. Adjusted odds ratios for SU care were 0.49 for dependency (95% confidence interval [CI]=0.22-1.08) and 0.17 for death (95% CI=0.04-0.83). CONCLUSIONS: This study showed that SU care reduces fatality rates and improves functional outcomes, which indicates the need to implement more SUs in Korea.
Cohort Studies ; Dependency (Psychology) ; Developed Countries ; Humans ; Korea ; Logistic Models ; Odds Ratio ; Patients' Rooms ; Propensity Score ; Prospective Studies ; Stroke

BACKGROUND: Acute intracranial large-artery occlusions (AILAOs) are detected frequently and cause severe neurological disabilities. Most studies in this field do not focus on the natural history of AILAOs, but on the individual prognosis for each intervention. The aim of this study was to elucidate the clinical profiles and outcomes of AILAOs. METHODS: A consecutive series of patients hospitalized between January 2004 and October 2007 due to AILAO within 24 hours from onset were recruited. Based on a prospective stroke registry, their clinical profiles were collected. AILAO was defined as an intracranial internal carotid artery (ICA), middle cerebral artery (MCA), or basilar artery (BA) occlusion that could be confirmed by angiography, with relevant lesions on diffusion-weighted imaging (DWI). The modified Rankin Scale (MRS) score at 3 months and the recanalization rate within 14 days were recorded as outcomes. RESULTS: Among 1,047 patients with acute ischemic stroke who were examined within 24 hours of onset, 189 [18.1%; 101 men, 88 women; age 68.6+/-13.0 years (mean+/-SD); median National Institutes of Health Stroke Scale (NIHSS) score=11]. Occlusion sites were MCA M1, MCA M2, distal ICA, and BA in 99 (52.4%), 50 (26.5%), 20 (10.6%), and 20 (10.6%) of cases,respectively. Embolic sources were found in 103 (54.5%) cases. MRS scores were available for 184 (97.4%) of the patients, of which 78 had a favorable outcome (MRS: 0.2). Follow-up angiography was performed in 122 (64.6%) cases, with recanalization observed in 88 (72.1%) of these. Thrombolysis, occlusion site, presence of an embolic source, and initial glucose level were predictors of early recanalization (p<0.001). Recanalization status and initial NIHSS score were strongly correlated with a favorable outcome (p<0.005). CONCLUSIONS: This is the first report of the clinical profiles, outcomes, and their predictors in a cohort of Korean patients with AILAO.
Angiography ; Arteries ; Basilar Artery ; Carotid Artery, Internal ; Cerebrovascular Disorders ; Cohort Studies ; Follow-Up Studies ; Glucose ; Humans ; Intracranial Embolism and Thrombosis ; Male ; Middle Cerebral Artery ; National Institutes of Health (U.S.) ; Natural History ; Prognosis ; Prospective Studies ; Stroke

BACKGROUND: The aims of this study were to develop and internally and externally validate a prognostic model that can predict the benefit and harm of thrombolysis in patients with acute ischemic stroke and that may be used promptly in an emergency setting. METHODS: The data of a consecutive series of patients who were hospitalized to Seoul National University Bundang Hospital within 12 hours of stroke onset between January 2004 and March 2008 and with relevant ischemic lesions on diffusion-weighted MRI were used to develop and internally validate the prognostic model. The external validation was performed using the data of patients from five participating centers of the Clinical Research Center for Stroke that had been collected between April 2008 and September 2009. The score on the modified Rankin Disability Scale at 3 months was selected to determine the efficacy outcome, and the occurrence of symptomatic hemorrhagic transformation was used to evaluate the safety outcome. Prognostic models were constructed with logistic regression, and both internal and external validations were performed. RESULTS: The discriminative abilities of the efficacy model (C statistic=0.880) and the safety prognostic model (C statistic=0.864) were confirmed. External validation of both models revealed remarkably little degradation in the discrimination power (C statistic=0.835 and 0.822 for the efficacy and safety models, respectively). CONCLUSIONS: This study shows that the efficacy and safety prognostic models developed with basic clinical variables were reliably validated with independent data. Both models may be helpful to clinicians in the emergency setting to identify patients who would benefit from thrombolysis.
Discrimination (Psychology) ; Emergencies ; Humans ; Logistic Models ; Stroke

BACKGROUND: For the improvement in stroke care, we have campaigned for stroke warning signs and its symptoms. However, few reports on the validation of symptom selection in the campaign have been published till now. METHODS: Based on the prospective stroke registry, patients were identified, who were hospitalized with stroke within 7 days from the stroke onset and had relevant lesions on their brain images. One presenting symptom was selected in each patient through review of electronic medical records by an investigator, and those collected symptoms were classified into 5 symptom categories or the unclassified. Those 5 symptom categories were developed and are being used in the stroke awareness campaign in 2009 by the Korean Stroke Society (KSS). RESULTS: A total of 3027 patients (age, 66.57+/-12.6 years; male, 58.2%) were enrolled in our study. The rate of frequency of each categorized symptom was 54.9% for unilateral numbness or weakness, 27.5% for confusion or speech disturbance, 2.8% for visual disturbance, 10.5% for dizziness or gait disturbance, 2.3% for severe headache, and 2.0% for the unclassified. Ninety-eight percent of stroke patients were classified into one of the 5 symptom categories. Confusion or speech disturbance was associated with the shorter pre-hospital delay, whereas dizziness or gait disturbance with the longer delay. Dizziness was the most frequent symptom in TIA, and so was severe headache in hemorrhagic stroke. CONCLUSIONS: Our study shows that the 5 stroke warning symptoms of the KSS campaign represent well the presenting symptoms of Korean patients with acute stroke or TIA.
Brain ; Dizziness ; Electronic Health Records ; Gait ; Headache ; Humans ; Hypesthesia ; Male ; Prospective Studies ; Research Personnel ; Stroke

Moraxella is an aerobic, Gram-negative coccobacillus, which is rarely associated with serious and invasive infections. Because of its rarity, the clinical significance and appropriate therapy for infections due to Moraxella are not well understood. We report a case of meningoencephalitis caused by Moraxella. The patient presented with fever and confusion and was successfully treated with beta-lactam and aminoglycoside antibiotics. We also review 8 previously published cases of Moraxella meningitis or meningoencephalitis.
Anti-Bacterial Agents ; Fever ; Humans ; Meningitis ; Meningoencephalitis ; Moraxella

Degenerative pannus arising from the atlanto-axial articulation is a rare entity, which is associated with chronic atlanto-axial instability in degenerative arthropathies. Due to cervical instability and the mass effect on the spinal cord, it can produce severe neck pain and compressive myelopathy. We report a case of cervical myelopathy resulting from non-rheumatoid atlanto-axial subluxation. The posterior stabilization provided excellent neurological improvement and pannus regression. Early recognition of this condition is important as the clinical condition will deteriorate without surgical interventions.
Neck Pain ; Osteoarthritis ; Spinal Cord ; Spinal Cord Compression ; Spinal Cord Diseases

Acute neuropathic complications rarely develop in patients with inflammatory bowel disease. A case of acute peripheral neuropathy that developed only 4 weeks after a diagnosis of Crohn's disease (CD) is reported herein. Peripheral neuropathy can be a rare extraintestinal manifestation of CD. Early recognition and proper management of the neurologic complications are crucial for preventing the associated morbidity.
Crohn Disease* ; Diagnosis ; Humans ; Inflammatory Bowel Diseases ; Peripheral Nervous System Diseases* ; Polyneuropathies

BACKGROUND: Thrombolysis becomes an emerging therapeutic option for acute ischemic stroke. However, few reports exist on its use in the very elderly. We investigated whether there is a difference in the effectiveness and safety of intravenous (IV) and/or intra-arterial (IA) thrombolysis between patients aged > or =80 years and those aged <80 years. METHODS: A consecutive series of patients hospitalized within 12 hours from stroke onset were selected. We evaluated the effectiveness of thrombolysis with modified Rankin scale (mRS) at 3 months, and the safety with symptomatic hemorrhagic transformation (sHT). The odds ratio (OR) of thrombolysis on the favorable mRS (0-2) was calculated using multivariable logistic regression analysis with adjustments for potential confounders. Whether the effectiveness of thrombolysis is age-dependent or not was analyzed by introducing an interaction term (thrombolysisxage group) into multivariable models. RESULTS: Among 219 patients > or =80 years, 21% of patients received IV or IA or combined thrombolysis while 23% of 980 patients <80 years received thrombolysis (P=0.51). With respect to the favorable mRS, the adjusted ORs of thrombolysis were 1.70 (95% confidence interval, 1.08-2.68) in all subjects, 1.61 (0.58-4.49) in those > or =80 years, and 1.71 (1.05-2.78) in those <80 years. There was no significant interaction between age group and thrombolysis (P=0.91). With respect to sHT, the adjusted OR was 4.72 (1.94-11.45) in all subjects with no significant interaction (P=0.86). CONCLUSION: This study suggests that thrombolysis may be equally safe and effective in stroke patients aged > or =80 years versus <80 years.
Aged ; Electrolytes ; Humans ; Logistic Models ; Odds Ratio ; Stroke ; Treatment Outcome

BACKGROUND AND PURPOSE: Ischemic lesion recurrence on diffusion-weighted imaging (DWI-LR) is a frequently observed phenomenon after acute ischemic stroke. However, no study has elucidated the impact of DWI-LR on functional outcome. METHODS: Among a consecutive series of patients who presented with focal symptoms or signs compatible with stroke within 48 hours from the onset over a 50-month period, those who had relevant ischemic lesions on initial DWI and underwent follow-up DWI within 14 days after the onset were enrolled in this study. As outcome variables, the scores on the modified Rankin Disability Scale (mRDS) at 3 months and 1 year were measured prospectively and dichotomized into good (0-2) vs. poor (3-6). When calculating odds ratios (ORs), adjustment was performed for age, previous stroke, initial score on the NIH Stroke Scale, stroke subtype, and IV thrombolysis. RESULTS: Among those 786 patients finally enrolled in this study, 221 (28.1%) had DWI-LR. For a poor outcome at 3 months, the crude ORs of any, symptomatic, and asymptomatic DWI-LR were 2.70 [95% confidence interval (CI), 1.96 to 3.72], 10.03 (95% CI, 4.39 to 22.96), and 2.04 (95% CI, 1.44 to 2.88), respectively. With adjustment, the OR of symptomatic DWI-LR was 6.44 (95% CI, 2.50 to 16.57), whereas those of any and asymptomatic DWI-LR lost their statistical significance: 1.44 (95% CI, 0.94 to 2.20) and 1.04 (95% CI, 0.65 to 1.65), respectively. Analyzing with the 1-year outcome produced similar results. CONCLUSIONS: This study shows that symptomatic early lesion recurrence can affect functional outcome after acute ischemic stroke, whereas an asymptomatic one may not.
Cerebral Infarction ; Diffusion Magnetic Resonance Imaging ; Electrolytes ; Follow-Up Studies ; Humans ; Odds Ratio ; Prospective Studies ; Recurrence ; Stroke