The effect of intensive statin therapy in non-symptomatic intracranial arteries: The STAMINA-MRI sub-study.

Background and aims: Pleiotropic effects of statins result in the stabilization of symptomatic intracranial arterial plaque. However, little is known about the effect of statins in non-symptomatic cerebral arteries. We hypothesized that intensive statin therapy could produce a change in the non-symptomatic cerebral arteries. Methods: This is a sub-study of a prospective observational study under the title of "Intensive Statin Treatment in Acute Ischemic Stroke Patients with Intracranial Atherosclerosis: a High-Resolution Magnetic Resonance Imaging (HR-MRI) study." Patients with statin-naive acute ischemic stroke who had symptomatic intracranial artery stenosis (above 50%) were recruited for this study. HR-MRI was performed to assess the patients' cerebral arterial status before and 6 months after the statin therapy. To demonstrate the effect of statins in the non-symptomatic segment of intracranial cerebral arteries, we excluded symptomatic segments from the data to be analyzed. We compared the morphological changes using cerebrovascular morphometry. Results: A total of 54 patients (mean age: 62.9 ± 14.4 years, 59.3% women) were included in this study. Intensive statin therapy produced significant morphological changes of overall cerebral arteries. Among the morphological features, the arterial luminal area showed the highest number of significant changes with a range from 5.7 and 6.7%. Systolic blood pressure (SBP) was an independent factor associated with relative changes in posterior circulation bed maximal diameter percentage change (beta -0.21, 95% confidence interval -0.36 to -0.07, p = 0.005). Conclusion: Intensive statin therapy produced a favorable morphological change in cerebral arteries of not only the target arterial segment but also non-symptomatic arterial segments. The change in cerebral arterial luminal diameter was influenced by the baseline SBP and was dependent on the topographic distribution of the cerebral arteries.Clinical Trial Registration: ClinicalTrials.gov, identifier NCT02458755.

Association of Systolic Blood Pressure and Cerebral Collateral Flow in Acute Ischemic Stroke by Stroke Subtype.

Background and Purpose: Collateral flow in acute ischemic stroke is known as a predictor of treatment outcome and long-term prognosis. However, factors determining the initial collateral flow remain unclear. We investigated factors related to collateral flow in patients with acute ischemic stroke caused by large vessel occlusion (AIS-LVO) and further analyzed the results according to stroke etiology. Methods: This was a retrospective study using prospective stroke registry data from a single university hospital from October 2014 to May 2021. AIS-LVO with middle cerebral artery M1 occlusion identified by pre-treatment multiphasic computed tomography angiography was included. Collateral flow score was graded on a 6-point ordinal scale according to pial arterial filling. Results: A total of 74 patients [cardioembolism (CE): 57; large artery atherosclerosis (LAA): 17] was included. The mean age of all patients was 72.2 ± 11.7 years, and 37.8 % (n = 28) were men. Multivariate regression analysis showed that initial SBP [odds ratio (OR): 0.994; 95% confidence interval (CI): 0.990-0.998; p = 0.002] and stroke etiology (OR: 0.718; 95% CI: 0.548-0.940; p = 0.019) were independent factors of the collateral flow grade. Collateral flow grade was independently associated with initial SBP in the CE group (OR: 0.993; 95% CI: 0.989-0.998; p = 0.004) but not in the LAA group (OR: 0.992; 95% CI: 0.980-1.004; p = 0.218). Initial SBP was significantly correlated with NIHSS score in the CE group but not in the LAA group (r 2= 0.091, p = 0.023; r 2 = 0.043, p = 0.426, respectively). Conclusions: Elevated initial SBP was associated with poor cerebral collateral flow and more severe symptoms in the CE group, but not in the LAA group in patients with AIS-LVO. These findings suggest differential effects of initial SBP elevation on collateral flow by stroke subtypes.

Correlation Between Hippocampal Enlarged Perivascular Spaces and Cognition in Non-dementic Elderly Population.

Background and aims: The pathophysiology of hippocampal enlarged perivascular spaces (H-EPVS) and its relationship to cognitive impairment is largely unknown. This study aimed to investigate the relationship between H-EPVS and cognition in non-dementic elderly population. Methods: A total of 109 subjects were prospectively enrolled. The eligibilities for inclusion were age from 55 to 85 years and Mini-Mental Status Examination score of ≥26. The Alzheimer's Disease Assessment Scale-Cognitive Subscale (ADAS-Cog), Montreal Cognitive Assessment, transcranial Doppler (TCD), and brain magnetic resonance imaging results were evaluated. H-EPVS was categorized in a three-degree scale: degree 0 (no), degree 1 (1,2), and degree 2 (>2). The associations between H-EPVS and TCD parameters/cognitive test profiles were analyzed. Results: The mean age was 65.2 years, and 52.3% subjects were men. H-EPVS was found to be associated with age (degree 2 vs. degree 1 vs. degree 0, 69.20 ± 6.93 vs. 65.70 ± 5.75 vs. 63.80 ± 5.43; p = 0.030) and ADAS-Cog memory score (degree 2 vs. degree 1 vs. degree 0, 14.88 ± 4.27 vs. 12.49 ± 4.56 vs. 11.4 ± 4.23; p = 0.037). However, the pulsatility index was not related to the degree of H-EPVS. Multivariate analysis revealed medial temporal atrophy (MTA) scale score was independently associated with ADAS-Cog memory score (MTA scale sum ≥4, p = 0.011) but not with the degree of H-EPVS. MTA scale score showed correlation with H-EPVS (r = 0.273, p = 0.004). Conclusions: Aging was associated with the development of H-EPVS in non-dementic elderly population. Memory function was found to be associated with MTA but not with the degree of H-EPVS.

A Case of GNE Myopathy Presenting a Rapid Deterioration during Pregnancy.

BACKGROUND: GNE myopathy is characterized by early-adult-onset distal myopathy sparing quadriceps caused by mutations in the GNE gene encoding UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase, an enzyme in the sialic-acid synthesis pathway. CASE REPORT: A 27-year-old Korean woman presented a rapid deterioration in strength of the distal lower limbs during her first pregnancy. She was diagnosed with GNE myopathy and carrying the compound heterozygous mutations of the GNE gene (D208N/M29T). CONCLUSIONS: This is a representative case implying that an increased requirement of sialic acid during pregnancy might trigger a clinical worsening of GNE myopathy.

Clinical characteristics and molecular genetic analysis of Korean patients with GNE myopathy.

PURPOSE: Glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE) myopathy is an autosomal recessive neuromuscular disorder characterized by early adult-onset weakness of the distal muscles of the lower limbs. The clinical spectrum of GNE myopathy varies, and it is not clear how the same GNE gene mutations can result in different phenotypes. Here, we present clinical, pathological and genetic characteristics of twenty-one Korean patients with GNE myopathy. MATERIALS AND METHODS: Twenty-one GNE myopathy patients were included in this study, conducted from 2004 to 2011. Based on medical records, patients' gender, onset age, family history, clinical history, serum creatine kinase (CK) level, neurologic examination, findings of muscle biopsy, muscle imaging findings and electrophysiologic features were extensively reviewed. Mutation of the GNE gene (9p13.3) was confirmed by DNA direct sequencing analysis in all patients. RESULTS: The mean onset age was 23.8±8.8 years (mean±SD). Patient serum CK levels were slightly to moderately elevated, ranging from 41 to 2610 IU. Among the patients, twelve patients were female and nine patients were male. Except for eight patients, all of the patients presented initially with only distal muscle weakness in the lower extremities. The most common mutation was V572L, followed by C13S. CONCLUSION: The clinical manifestations of our patients with GNE mutations varied. Among twenty-one patients, thirteen patients showed the typical GNE myopathy phenotype. There was no relationship between clinical features and site of mutation. Therefore, we suggest that neither homozygous nor compound heterozygous models are correlated with disease phenotype or disease severity.

A case of acute disseminated encephalomyelitis associated with hepatitis C virus infection.

Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system, which typically follows acute viral or bacterial infection or vaccination. We report a case of ADEM associated with hepatitis C virus (HCV) infection with positive serum and cerebrospinal fluid (CSF) anti-HCV antibody. After steroid treatment, neurologic symptoms were improved. Virus triggers autoimmunity or direct viral invasion plays a part in the genesis of ADEM. This is the first reported case of ADEM with anti-HCV antibody in the CSF.

Facial Diplegia in Plasmodium vivax Malaria.

BACKGROUND: Facial diplegia has diverse etiologies, including viral and bacterial infections such as diphtheria, syphilis and Lyme disease, and also protozoal infection in very rarely cases. CASE REPORT: A 20-year-old male patient was admitted to our hospital due to bilateral weakness of the upper and lower facial muscles. Examination revealed that the patient had a facial diplegia of the peripheral type. A peripheral blood smear demonstrated the presence of the asexual trophozoite stage of Plasmodium vivax with ring-form trophozoites, which led to a diagnosis of malaria. A serum work-up revealed increased IgG titers of antibodies to myelin-associated glycoprotein and ganglioside GD1b. The patient was administered antimalarial treatment, 1 week after which he showed signs of recovery. To our knowledge, this is the first case of facial diplegia after malaria infection, providing evidence that the mechanism underlying the condition is related to immune-mediated disease. CONCLUSIONS: Facial diplegia can manifest after P. vivax infection.