Generalised autonomic failure as a prognostic factor in systemic light-chain (AL) amyloidosis.

BACKGROUND: In the present study, it was investigated whether autonomic dysfunction could predict prognosis in light-chain (AL) amyloidosis patients. PATIENTS AND METHODS: Seventy-two patients with biopsy-proven AL amyloidosis were included and underwent an autonomic function test (AFT) between January 2016 and June 2019. Autonomic failure was evaluated using the Composite Autonomic Severity Score (CASS). Survival curves and the three-year overall survival (OS) rate were estimated using the Kaplan-Meier curve, and the Cox proportional hazards regression method was used to evaluate the variables that influenced survival. RESULTS: Autonomic dysfunction was observed in 69 (96%) patients with AL amyloidosis, and the three-year OS rate was 67%. Generalised autonomic failure (GAF) was observed in 31 (43%) patients. In the Kaplan-Meier curve, the three-year OS rates in patients with sudomotor dysfunction or GAF were lower than that in control patients (35 vs. 84%, and 33 vs. 81%, respectively). In Cox proportional hazards regression model, female, bone marrow plasma cell percentage, left ventricular systolic dysfunction, and GAF were significant independent variables associated with survival. CONCLUSION: The results of this study indicate that GAF on the AFT is an independent adverse prognostic factor for survival in AL amyloidosis patients.

Comparison of MOG and AQP4 antibody seroprevalence in Korean adults with inflammatory demyelinating CNS diseases.

We aimed to compare seroprevalence of anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin-4 (AQP4) antibodies in Korean adults with inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS), based on a multicenter nationwide database. Sera were analyzed using a live cell-based assay for MOG and AQP4 antibodies. Of 586 Korean adults with IDDs of the CNS, 36 (6.1%) and 185 (31.6%) tested positive for MOG and AQP4 antibodies, respectively. No participant showed double positivity. Seroprevalence of MOG antibodies was about five times lower than that of AQP4 antibodies in a large cohort of Korean adults with IDDs of the CNS.

Recurrence of clinical events at the same anatomical location in patients with MOG antibody-associated disease.

OBJECTIVES: Likelihood of clinical events occurring within the same anatomical location in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) was retrospectively investigated. METHODS: A total of 236 clinical events in 90 patients with MOGAD from nine referral hospitals were analyzed via logistic regression, and odds ratios (ORs) were calculated. Anatomical lesion location was divided into four groups; optic nerve, spinal cord, cerebral hemisphere, and brainstem/cerebellum. RESULTS: At all locations, there was an increased likelihood of a second attack occurring at the same location as the initial event (cerebral hemisphere OR = 22.14, brainstem/cerebellum OR = 18.4, spinal cord OR = 9.1, and optic nerve OR = 7.8). There was an increased likelihood of a third attack occurring at the same location as the initial event in the optic nerve (OR = 14.9), cerebral hemisphere (OR = 11.7), and spinal cord (OR = 6.7). There were positive trends toward a third clinical event occurring at the same location as the first and/or second events if the event was in the optic nerve (OR = 13.5), cerebral hemisphere (OR = 6.9), or spinal cord (OR = 5.7). CONCLUSIONS: The current study suggests that clinical relapses of MOGAD during early stage tend to recur at the same anatomical locations in the central nervous system.

Value of Area Postrema Syndrome in Differentiating Adults With AQP4 vs. MOG Antibodies.

Objectives: To compare the frequency of area postrema syndrome (APS) in adults with anti-aquaporin-4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. Methods: APS is defined as acute or subacute, single or combined, episodic or constant nausea, vomiting, or hiccups, persisting for at least 48 h, which cannot be attributed to any other etiology. The presence of APS was investigated in 274 adults with AQP4 antibodies and 107 adults with MOG antibodies from 10 hospitals. Results: The study population comprised Korean adults (≥18 years). At the time of disease onset, 14.9% (41/274) adults with AQP4 antibodies had APS, while none of the participants with MOG antibodies developed APS (p < 0.001). During the course of the disease, 17.2% (47/274) adults with AQP4 antibodies had APS in contrast to 1.9% (2/107) adults with MOG antibodies with APS (p < 0.001). Conclusions: APS, one of the core clinical characteristics of individuals with AQP4 antibodies, is an extremely rare manifestation in Korean adults with MOG antibodies.

Volume and morphology of left atrial appendage as determinants of stroke subtype in patients with atrial fibrillation.

BACKGROUND: Atrial fibrillation (AF) is a leading cause of stroke, but not all cases of stroke in patients with AF are due to AF. OBJECTIVE: The purpose of this study was to determine whether morphometric or volumetric parameters of left atrial appendage (LAA) would be related to the development of cardioembolism in subjects with AF. METHODS: A total of 433 consecutive patients with acute ischemic stroke underwent multidetector cardiac computed tomography (MDCT). Of these patients, 88 with AF were divided into cardioembolic stroke (CES; n = 57) and non-CES (n = 31) groups, and 95 age- and sex-matched patients with non-CES without AF served as controls. Clinical factors, echocardiographic findings, and MDCT parameters were evaluated. RESULTS: Brain infarct volume, LAA orifice diameter, and LAA volume were larger in patients with CES with AF than in those with non-CES with AF (P<.05 in all cases), but no difference was observed between patients with non-CES with AF and those with non-CES without AF. MDCT and echocardiographic parameters of left atrial (LA) dysfunction were different depending on the presence of AF but not between patients with CES with AF vs non-CES with AF. After adjusting for covariates, LAA orifice diameter (odds ratio 1.19, 95% confidence interval 1.06-1.33, P = .004) and LAA volume (odds ratio 12.20, 95% confidence interval 2.58-57.79, P = .002) were independently associated with CES with AF, as was infarct volume. CONCLUSION: In patients with AF, LAA orifice diameter and LAA volume, but not left atrial dysfunction, were determinants of CES and were useful for stratifying noncardioembolic risk in patients with AF.

Mesial Temporal Lobe Epilepsy in Congenital Toxoplasmosis: A Case Report.

Toxoplasmosis is a rare disease caused by intracellular protozoan parasite, Toxoplasma gondii. Though most patients with toxoplasmosis are asymptomatic, congenital toxoplasmosis in the fetus can cause ocular involvement such as chorioretinitis and central nervous system disease including intracerebral calcification, nystagmus, hydrocephalus and microcephaly. Also, these brain lesions can cause seizure secondarily. Our patient was diagnosed with congenital toxoplasmosis, based on toxoplasma-specific serologic test with typical clinical symptoms, including chorioretinitis, nystagmus, hydrocephalus and cerebral palsy. Her brain imaging findings revealed not only the multifocal encephalomalacia, but also multifocal cerebral calcification including intracerebral calcification in left perihippocampal region. Her epileptogenic zone was defined as mesial temporal lobe including hippocampus on left side by seizure semiology, electroencephalogram and neuroimaging including single photon emission computed tomography and 18F-Fluorodeoxyglucose positron-emission tomography. Her seizures were refractory to multiple anti-epileptic drugs. We report a patient with congenital toxoplasmosis who showed intractable mesial temporal lobe epilepsy.

The Need for a Coagulation Assay after Initiation of New Oral Anticoagulants in Patients with Renal Dysfunction: A Case Report.

BACKGROUND: Dabigatran etexilate, a new oral anticoagulant, was recently approved as an efficacious alternative to warfarin for the prevention of first and recurrent stroke in patients with nonvalvular atrial fibrillation. Limited data are available for dabigatran use in patients with a creatinine clearance rate (CrCL) of 15-30 mL/min. Furthermore, current guidelines do not recommend frequent blood monitoring after dabigatran use. We report herein a patient with severe renal dysfunction who exhibited profound coagulopathy after 2 days of dabigatran use. CASE REPORT: An 87-year-old woman was admitted for altered mental status and left-side weakness. She was diagnosed with right middle cerebral artery infarction. The baseline assessment revealed a serum creatinine concentration of 1.29 mg/dL and a CrCL of 27.2 mL/min. Dabigatran therapy was started 5 weeks after admission at a dosage of 110 mg twice daily. After 2 days of dabigatran use, the patient developed multiple bruises and evidence of upper-gastrointestinal bleeding. Laboratory tests demonstrated a severe coagulopathy, with a prothrombin time of 85.9 sec, an international normalized ratio of 11.36, an activated partial thromboplastin time of 119.2 sec, and a thrombin time of 230.8 sec. Serial assessment of the patient's renal function revealed substantial fluctuation of the CrCL (range, 17.9-26.5 mL/min). CONCLUSIONS: The present case emphasizes the need for frequent checking of renal function and assessment using coagulation assays after commencing dabigatran therapy in patients with moderate-to-severe renal impairment.