Extensive White Matter Lesions Research Articles

Anti-leucine-rich Glioma-inactivated 1 Protein-antibody Positive Encephalitis with Extensive Unilateral Cerebral Cortex and White Matter Lesions.

Encephalitis caused by antibodies targeting the leucine-rich glioma-inactivated 1 protein receptor, which belongs to the anti-voltage-gated potassium channel receptor complex, is characterized by hyponatremia, progressive cognitive impairment, seizures, and psychiatric disorders. The patient initially presented with faciobrachial dystonic seizures and subsequently developed encephalopathy. Brain magnetic resonance imaging revealed atypical unilateral hyperintense signals in the cerebral cortex and white matter. Intravenous corticosteroid pulse therapy effectively improved faciobrachial dystonic seizures and brain lesions.

Two rare cases of myelin oligodendrocyte glycoprotein antibody-associated disorder in children with leukodystrophy-like imaging findings

BackgroundChildren with acquired demyelinating syndromes (ADS) whose sera are positive for myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG) can be diagnosed with MOG-IgG associated disorder (MOGAD). Cases with leukodystrophy-like imaging findings with recurrent MOGAD have rarely been reported.Case presentationTwo children with MOGAD, whose onset age was 6 months and 3 years, respectively, were admitted to the hospital due to fever and altered consciousness. In both children, MOG-IgG was detected in the serum using live cell-based assay. Brain magnetic resonance imaging (MRI) revealed leukodystrophy-like lesions with diffuse bilateral white matter. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis with normal or slightly increased protein levels and no oligoclonal bands. Metabolic and inflammatory blood/CSF markers were all negative. Full exon gene testing revealed normal results, and nuclear and mitochondrial DNA were normal. Despite regular immunotherapy and reduction of lesions based on brain MRI results, the patients repeatedly relapsed and had residual neurological dysfunction at 3–4 years of follow-up.ConclusionsAlthough MOGAD is a monophasic and benign condition, certain MOGAD patients can experience multiple relapses and residual neurologic deficits. The spectrum of clinical manifestations in MOGAD is wider in children than in previously reported cases, including cases with leukodystrophy-like imaging findings. Such imaging findings along with MOG-IgG may occur recurrently and result in severe neurological prognosis. Patients with extensive and confluent white matter lesions should undergo early testing of MOG-IgG to ensure early therapy. In refractory cases, MOGAD treatment may need to be escalated beyond the current therapy, which means second-line immunotherapy should be performed as early as possible and hormone levels should not be rapidly reduced. Early diagnosis and appropriate treatment may improve the prognosis of children with MOGAD.

Association between Citrullinated Histone H3 and White Matter Lesions Burden in Patients with Ischemic Stroke.

Neutrophil extracellular traps play a role in the pathophysiology of stroke and are associated with severity and mortality. We aimed to investigate whether the citrullinated histone H3 (CitH3), a biomarker for neutrophil extracellular traps formation, is associated with the white matter lesion (WML) burden in ischemic stroke patients. Between September 2021 and April 2022, 322 patients were enrolled in this prospective observational cohort study. Serum CitH3 levels were measured after admission using an enzyme-linked immunosorbent assay. WMLs severity was graded according to the Fazekas scale and conceptually defined as mild (total Fazekas score 0-2) and severe (total Fazekas score 3-6). We used multivariable regression models to determine the relationship between CitH3 concentrations and the severity of WMLs burden. One-hundred and forty-eight (46.0%) patients were diagnosed with severe WMLs burden after admission. Increased CitH3 levels (first quartile vs. fourth quartile of H3Cit, odds ratio, 3.311, 95% confidence interval, 1.336-8.027; p = 0.011) were independently associated with a greater WML burden in the fully adjusted multivariable model. Similar results were found when the H3Cit was analyzed as a continuous variable. Furthermore, the multiple-adjusted spline regression model showed a linear association between H3Cit levels and severe WMLs (P = 0.001 for linearity). In the present study, increased CitH3 levels were positively associated with extensive WMLs in ischemic stroke patients, indicating a role of neutrophil extracellular traps formation in the pathogenesis of WMLs.

PS-R04-1: A CASE OF DRUG-INDUCED RENAL INJURY AND DRUG-INDUCED HYPERTENSION RESULTING IN HYPERTENSIVE EMERGENCIES

48 years old male. He had high blood pressure for 2 years and was treated at a Chinese herbal medicine shop, the prescription was changed each time. He began to have headaches and was taking triple doses of NSAIDs daily. Three months later, he developed lightheadedness and edema. He was diagnosed with hydrocephalus, Ommaya reservoir was inserted, but his loss of consciousness was prolonged. He was transferred to the neurosurgery department of our hospital on X + 3 for close examination of the tumor. At the time of transfer, his Cr was 9.32 mg/dl and he was referred to our department. He was suspected to have uremic symptoms, so Hemodialysis (HD) was started. Renal biopsy revealed nephrosclerosis and drug-induced kidney injury (DKI). Since the previous physician had discontinued NSAIDs and Chinese herbal medicine, so we mainly managed him with antihypertensive therapy. The Neurosurgical examination was negative for tumor, and the patient was transferred to the Department of Neurology on X + 11 for further examination of encephalopathy. Although his blood pressure was 230/140 mmHg immediately after transfer, the presence of endocrine hypertension was excluded, and the symptom improved by the elimination of the uremia and lowering of the blood pressure by ARB, CCB and HD. In addition, extensive white matter lesions on head CT resolved, so he was diagnosed Posterior reversible encephalopathy syndrome (PRES). He was weaned from HD on X + 29 after his urinary output recovered, but was transferred to our department on X + 31 for decision making including reintroduction of HD because of his Cr 5.08 mg/dl level. He was discharged from the hospital on X + 47 after starting half-pulse steroid therapy (0.5 mg/kg post therapy) for DKI, with a strong desire to avoid reintroduction of HD. Currently, his Cr is 2.87 mg/dl. This case of hypertensive emergency was caused by DKI and drug-induced hypertension. NSAIDs suppress renal prostaglandin production by inhibiting COX, resulting in decreased renal function and increased blood pressure. Although the details of the Chinese herbal remedy are unknown, we suspected that NSAIDs was the cause of the hypertension, because the RAA system is usually suppressed if licorice is the cause. Although the rate of conversion from hypertensive emergencies to maintenance dialysis has not been shown, it is considered that the early release of renal load by HD in this case led to the recovery of renal function.

An Atypical Case of Neonatal Hypoglycemic Encephalopathy with Extensive White Matter Lesions

An Atypical Case of Neonatal Hypoglycemic Encephalopathy with Extensive White Matter Lesions

A-279 Progressive Neuropsychological Deficits Secondary to an Acute Disseminated Encephalomyelitis Attack 11 Years Later Complicated by Possible New Pathology

Abstract Objective: Acute Disseminated Encephalomyelitis (ADEM) often presents as an abrupt, brief, and intense attack causing widespread inflammation and demyelination to the central nervous system. Most patients typically make a complete neurological recovery; however, a few demonstrate long-term, persistent impairments. Method: A 64-year-old white female presented for a neuropsychological evaluation (2021) following a progressive one-year decline in her memory and motor abilities, which per family, reportedly overlay persisting residual deficits from an ADEM attack she had in 2010. Nearly 11 years following the attack, she reported continued reliance on her family in completing most of her instrumental, and even some basic activities of daily living since the attack. Initial neuroimaging following the attack (2010) revealed extensive bilateral white matter (WM) lesions, whereas current neuroimaging (2021) (despite not explicitly commenting on the resolution status of her lesions), continued to reveal widespread hyperintense signals in WM regions, progressing cerebral atrophy, and the possibility of superimposed normal pressure hydrocephalus (i.e., ventriculomegaly). Results: Her neuropsychological profile (with no prior baseline) was marked by substantial impairments in immediate and delayed verbal/visual memory, attention, language, motor abilities, visuospatial skills, executive functions, along with mild anxiety. Conclusions: This neurocognitive profile displayed atypical findings in patient recovery following an ADEM attack. Given the test results and the continued presence of extensive hyperintense signals in WM regions, it is possible her initial brain lesions (due to ADEM) remained unresolved. This, along with the possible intervening effects of new pathology (e.g., NPH and possible vascular changes) are likely exacerbating her existing deficits related to ADEM.

Impact of Brain MRI Markers on Major and Mild Vascular Cognitive Impairment in CADASIL

Background: Cognitive impairment is the second most common clinical manifestation in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). However, understanding of cognitive impairment in CADASIL has been hampered by lack of consensus on diagnosis of vascular cognitive impairment (VCI). We used vascular impairment of cognition classification consensus study principles (VICCCS-1) and protocols (VICCCS-2) to assess the cognitive impairment in CADASIL. We also evaluated the impact of MRI markers on major and mild VCI in CADASIL.Methods: We prospectively recruited 64 patients who underwent standardized brain MRI and detailed neuropsychological test. MRI analysis included number of lacunes, number of cerebral microbleeds (CMB), normalized volume of white-matter hyperintensities (nWMH), and brain parenchymal fraction (BPF). BPF has been used to measure brain atrophy. The patients were divided into three groups: those with normal cognition (CADASIL-NC, n=14), those with mild VCI (CADASIL-mild VCI, n=38), and those with major VCI (CADASIL-major VCI, n=11).Results: The three groups differed according to age, with the major VCI group being older. The major VCI group had more lacunes, more CMB, more extensive white matter lesions and lower BPF than NC group. There were no significant differences between NC and mild VCI groups in BPF. BPF and age were the independent predictors of major VCI. There was a tendency that women were at higher risk for mild VCI, though it did not reach statistical significance. Women were older than men, but had lower number of lacunes in mild VCI.Conclusions: These findings suggest that brain atrophy and age are the main predictors of major VCI in CADASIL.

AARS2-Related Leukodystrophy: a Case Report and Literature Review.

Mutations in the alanyl-transfer RNA synthase 2 (AARS2) represent a heterogenous group of autosomal recessive leukodystrophy characterized by cognitive decline, ataxia, spasticity, and Parkinsonism. AARS2-related leukodystrophy (AARS2-L) is extremely rare. To date, only 45 genetically confirmed cases, explaining the frequent diagnostic delay. Here, we report a 21-year-old male presented with unsteady gait and weakness in the bilateral lower extremities. Examination revealed dysarthria, cerebellar ataxia, paraparesis, and Parkinsonism with generalized hyperreflexia. MRI findings showed extensive white matter lesions in bilateral frontoparietal lobes, immediate periventricular regions, and corpus callosum. Focused exome sequencing revealed compound heterozygous mutations in the AARS2 gene confirming the diagnosis of AARS2-L; two heterogeneous missense mutations (c.452T > C, p. M151T; c. 2557C > T, p. R853W) appeared together for the first time. We also reviewed phenotypic spectra of AARS2-related leukodystrophies from a total of 45 reported cases.

Reversible splenial lesions during febrile illness with or without white matter lesions

ObjectiveReversible splenium lesions during febrile illness (RESLEF) are found in a spectrum. There are two types of corpus callosum (CC) lesions: CC-only type, with limited lesions and the CC (+) type, with extensive white-matter lesions. This retrospective study aimed to describe the differences in clinical findings between CC-only and CC (+) lesions and the association between onset age and clinico-radiological features in RESLEF. MethodsFifty-two episodes of CC-only or CC (+) lesions accompanied by neurological symptoms, e.g., seizures, delirious behavior (DB), and disturbance of consciousness (DC), from January 2008 to October 2019 were included. We analyzed the etiology (pathogen), clinical course, laboratory data, magnetic resonance imaging and electroencephalography findings, therapy, and prognosis. ResultsThe rate of DC in the CC (+) was significantly higher than that in the CC-only group (5/6 [83%] vs 7/46 [15%]; p = 0.0016). The median number of seizures in the CC (+) was also significantly higher than that in the CC-only group (4 [0–7] vs 0 [0–7]; p = 0.034). Further, in RESLEF, the median onset age (months) in the seizure was significantly lower than that in the no-seizure group (39 [12–74] vs 83 [28–174]; p = 0.0007). The median onset age (months) in the DB was significantly higher than that in the no-DB group (74.5 [26–174] vs 28 [12–139]; p = 0.003). ConclusionsIn RESLEF, CC (+) is a more severe neurological symptom than CC-only. Furthermore, the onset age is related to the type of neurological symptoms that appear.

Mild encephalopathy with reversible extensive white matter lesions in a child with acute adenoviral infection and a literature review

Mild encephalopathy with a reversible splenial lesion (MERS) is a known clinical-radiological description. However, MERS with extensive lesions (MERS type-2) is rarely associated with adenovirus. There are only three published cases of MERS type-2 associated with adenovirus infection. We present a 10-year-old previously healthy girl who presented with speech difficulty and mild encephalopathy after three days of prodromal illness. The magnetic resonance imaging (MRI) revealed bilateral diffusion restriction in the parietal white matter, splenium and genu of the corpus callosum without mass effect and slight thickening at the splenium of corpus callosum with no contrast enhancement. With empirical and support treatment, her neurological examination was completely normal by the 18th hour. The nasopharyngeal respiratory adenoviral PCR resulted positive. She was discharged with total clinical and radiological resolution on the 10th day of admission. The case was diagnosed with MERS type-2 which is rarely associated with adenoviral infection. This report is the first case of adenovirus related MERS type-2 in a Turkish child. Pediatricians, child neurologists, child infection specialists and radiologists should recognize this condition to ensure appropriate diagnosis.

EEG Synchronization-Parameters in Patients With Subcortical Arteriosclerotic Encephalopathy and Gait Disorder.

Subcortical arteriosclerotic encephalopathy (SAE) is characterized by extensive white matter lesions in the MRI. Clinical symptoms are cognitive impairment, ranging from mild deficits to vascular dementia, impaired executive functioning, and gait disorders. In the EEG of SAE patients with vascular dementia, the lower frequencies are increased. However, it is unclear whether EEG changes also exist in SAE patients with gait disorders but without vascular dementia. The authors analyzed the EEGs of 50 nondemented patients with SAE and gait disorders and 50 healthy controls applying pointwise transinformation as a measure of synchronization. Hundred seconds of waking EEG that appeared unaltered in visual analysis were sufficient to prove changes in synchronization. The authors found a decrease in the mean level of synchronization, combined with an elongation of synchronization time in all examined brain areas. These effects correlated slightly with the extent of subcortical lesions. Changes in EEG synchronization in patients with SAE and gait disorders seem to occur independently of cognitive function. The causal relationship of the changes in EEG synchronization and gait disorders remains to be clarified. The results of this study might point to a decrease in coupling efficiency in these patients, with the increase in synchronization duration as a possible compensatory mechanism. Because a time-efficient signal transmission particularly during gait execution is crucial, reduced efficiency might contribute to an impairment of postural stabilization. The study results might indicate a neuronal network for planning and execution of motor activity and particularly gait, extending from the frontal over the central to the parietal cortex.

Acute Focal Bacterial Nephritis Associated With Reversible Splenial Corpus Callosum Lesion

Acute focal bacterial nephritis (AFBN) is a localized bacterial infection of the kidney, which presents as an inflammatory mass without formation of a frank abscess. Rare cases of AFBN are accompanied by neurological symptoms such as meningeal irritation, unconsciousness, and seizures. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is characterized by transient high signal intensity splenial lesions on diffusion-weighted magnetic resonance imaging. MERS can be divided into two types: type 1 with an isolated splenium of the corpus callosum and type 2 with extensive white matter and/or entire callosal lesions. We experienced three rare cases of pediatric AFBN associated with neurological symptoms, including unconsciousness and reversible splenial corpus callosum lesion. The first case was an 8-year-old girl who had neurological symptoms, including unconsciousness, and AFBN was associated with MERS type 2. The second (5-year-old boy) and third (5-year-old girl) cases had neurological symptoms, including unconsciousness and AFBN with MERS type 1. Two of the three cases had AFBN caused by Escherichia coli . All three cases showed high levels of urinary beta 2 -microglobulin (B2MG). AFBN should be suspected in children with fever, rapid clinical deterioration, neurological symptoms including unconsciousness, and high urinary B2MG level. We recommend that abdominal enhanced computed tomography should be performed for the diagnosis of AFBN. Int J Clin Pediatr. 2020;9(3):82-86 doi: https://doi.org/10.14740/ijcp395

Cerebral Amyloid Angiopathy Related Inflammation With Prominent Meningeal Involvement. A Report of 2 Cases

Cerebral amyloid angiopathy related inflammation (CAA-RI) is a rare form of CAA characterized by subacute encephalitic symptoms (cognitive decline, seizures, focal deficits) associated with extensive and confluent white matter lesions co-localizing with lobar microbleeds on brain MRI. We report two cases of unusual CAA-RI mimicking meningoencephalitis but without typical brain lesions on FLAIR and T2* sequences. These 2 cases may extend the clinical spectrum of CAA-RI by suggesting the possible occurrence of quite purely meningeal forms of CAA-RI.

Altered amplitude of low-frequency fluctuation in basal ganglia correlates to pulmonary ventilation function in COPD patients: A resting-state fMRI study.

IntroductionPatients under chronic obstructive pulmonary disease (COPD) has been reported to be associated with a higher prevalence of cognitive impairment (CI). However, it is still largely unknown whether the aberrant resting‐state spontaneous neuronal activity pattern reflected by the amplitude of low‐frequency fluctuation (ALFF) analysis will be associated with the CI in COPD patients.MaterialsA total of 28 COPD patients and 26 healthy controls were enrolled in this study. Of all the subjects, structural and functional MRI data, spirometry tests performance and neuropsychological assessments of different cognitive domains were collected. Voxel‐based two‐sample t tests were used to detect brain regions showing differences in the ALFF value between COPD patients and healthy controls. An additional fMRI runs with supplementary oxygen delivery were employed to explore the impact of elevated partial pressure of oxygen (PaO2) or moderate hyperoxia on ALFF in COPD patients and healthy controls respectively.ResultsMore extensive white matter lesion was detected in COPD patients. COPD patients exhibit decreased ALFF value in bilateral basal ganglia areas and right thalamus, and aberrant ALFF value is correlated with PaO2 and pulmonary ventilation function (FEV1%pred). COPD patients performed worse in the Digit Span Test (reverse), Digit Symbol Substitution Test, Trail‐making test (A and B) than controls. After supplementary oxygen inhalation, the ALFF value of basal ganglia and right thalamus significantly increased in the controls, but not in the COPD patients.ConclusionsCOPD patients mainly exhibit impaired executive function but not long‐term memory in cognitive function assessment. Aberrant ALFF alteration in the deep brain may be directly related to lower PaO2 in COPD patients.

IL-1α and IL-6 predict vascular events or death in patients with cerebral small vessel disease—Data from the SHEF-CSVD study

PurposeThe natural clinical course of cerebral small vessel disease (CSVD) was not thoroughly described. The aim of this single center cohort study was to establish biochemical predictors of vascular events and death in CSVD patients during a 24-month follow-up. Patients and methodsA total of 130 functionally independent patients with marked MRI features of CSVD and recent lacunar stroke (n = 52,LS), vascular Parkinsonism (n = 28,VaP) or dementia (n = 50,VaD) were prospectively recruited. Serum markers of endothelial dysfunction, inflammation and hemostasis were determined at baseline. The primary outcome was defined as occurrence of death or any vascular events during the observation. ResultsThe mean age was 72 ± 8.1 years, and 37.6% of the patients were women. The mean follow-up time was 22.3 ± 4.3 months, and 84.6% of patients had extensive white matter lesions on baseline MRI. The overall mortality rate was 6.9%, and vascular events or death occurred in 27% of the patients. Kaplan-Meier survival curves revealed no significant differences between CSVD groups (log rank p = 0.49). Cox regression analysis revealed that IL-1α (HR 1.4; 95%CI 1.09–1.8), IL-6 (1.4;1.1–2.2), hs-CRP (1.1;1.06–1.9), homocysteine (1.4;1.1–1.8), fibrinogen (1.4;1.05–2), and d-dimer (2.7;1.6–4.5) were significantly associated with the primary outcome. IL-1α (1.3;1.07–1.8), IL-6 (1.4;1.02–2.2), d-dimer (2.8;1.6–5) and homocysteine (1.4;1.1–1.8) remained significant after adjusting for age, sex and CSVD radiological markers. ConclusionsOur study demonstrated the important prognostic role of various circulation markers of inflammation in individuals with different clinical signs and radiological markers of CSVD. The strongest association occurred between IL-1α, IL-6 and recurrent stroke, other vascular events and death.

Brain imaging can predict neurodevelopmental outcome of Group B streptococcal meningitis in neonates.

The association between cranial ultrasound (CUS) or magnetic resonance imaging (MRI) lesions and neonatal Group B streptococcal (GBS) meningitis outcome has not been studied in detail. This retrospective study assessed CUS, cranial MRI and neurodevelopmental outcome in 50 neonates with GBS meningitis admitted to three neonatal intensive care units in the Netherlands between 1992 and 2014. Death, cognitive outcome and motor outcome below -1 SD were considered as adverse outcomes. CUS was available in all and MRIs in 31 infants (62%) with 28 CUS (56%) and 27 MRIs (87%) being abnormal. MRI lesions were multifocal (n=10, 37%), bilateral (n=22; 82%) and extensive (n=11; 41%). A total of 10 died in the neonatal period. Median age at assessment was 24months. Among survivors, abnormal cognitive outcome and motor outcome were seen in 23 and 20 patients, respectively. Abnormal CUS [odds ratio (OR) 5.3, p=0.017], extensive bilateral deep grey lesions (OR 6.7, p=0.035) and white matter lesions (OR 14.0, p=0.039) correlated with abnormal motor outcome. Extensive bilateral deep grey matter lesions correlated with abnormal cognitive outcome (OR 8.1, p=0.029). Abnormal CUS and the most severely affected MRIs were associated with poor neurodevelopmental outcome in neonatal GBS meningitis.

High-sensitivity cardiac troponin T and severity of cerebral white matter lesions in patients with acute ischemic stroke.

Cardiac troponin (hs-cTnT) is a sensitive marker of myocardial injury and has been linked to incident dementia. The underlying mechanism of that observation is still unknown. Given that severity of cerebral small vessel disease is a predictor of cognitive decline, we aimed to explore whether there is an association between hs-cTnT and severity of white matter lesions (WML) as a marker of cerebral small vessel disease in patients with ischemic stroke. We analyzed consecutive acute ischemic stroke patients admitted to Charité-University Hospital, Berlin from 2011 to 2013. Severity of WML was graded on 3T-MRI using the age-related white matter severity score (ARWMS). Patients with hs-cTnT elevation suggestive of acute coronary syndrome (ACS) were excluded (hs-cTnT > 52ng/l or dynamic change of hs-cTnT > 50%, ESC guideline). We performed unadjusted and adjusted quantile regression models to assess the association between increased hs-cTnT (dichotomized at the 99th percentile, 14ng/l) and severity of WML. A total of 860 patients was analyzed (median age 73years, 44.8% female, median ARWMS 6). Patients with elevated hs-cTnT had more extensive WML than those without (median ARWMS 8 vs. 5, adjusted beta for 50th percentile 1.12, 95% CI 0.41-1.84). The association between WML and hs-cTnT elevation was strongest in patients with severe WML (adjusted beta 1.77, 95% CI 0.26-3.27 for 80th WML percentile). Elevated hs-cTnT levels were associated with extent of WML in acute stroke patients. Further studies are needed to assess whether hs-cTnT can be used to identify stroke patients at risk for cognitive decline.

Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome

To further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS). We compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled). Patients with RCVS were predominantly female (p < 0.0001), with migraines (p < 0.0001), and were more often exposed to vasoactive substances (p < 0.0001) or postpartum (p = 0.002) than patients with PACNS. Headache, especially thunderclap headache, was more frequent in RCVS (both p < 0.0001). Thunderclap headache was absent in only 6% of patients with RCVS and was mainly recurrent (87%) and provoked (77%) mostly by sexual intercourse, exertion, or emotion. All other neurologic symptoms (motor deficit, seizure, cognitive disorder, or vigilance impairment, all p < 0.0001) were more frequent in PACNS. At admission, brain CT or MRI was abnormal in all patients with PACNS and in 31% of patients with RCVS (p < 0.0001). Acute ischemic stroke was more frequent in PACNS than in RCVS (p < 0.0001). Although intracerebral hemorrhage was more frequent in PACNS (p = 0.006), subarachnoid hemorrhage and vasogenic edema predominated in RCVS (p = 0.04 and p = 0.01, respectively). Multiple small deep infarcts, extensive deep white matter lesions, tumor-like lesions, or multiple gadolinium-enhanced lesions were observed only in PACNS, whereas cervical artery dissection was found only in RCVS. Our study confirms that careful analysis of clinical context, headache features, and patterns of brain lesions can distinguish PACNS and RCVS within the first few days of admission in most cases. However, diagnosis remains challenging in a few cases.

Cerebral and Extracerebral Vasoreactivity in Patients With Different Clinical Manifestations of Cerebral Small-Vessel Disease: Data From the Significance of Hemodynamic and Hemostatic Factors in the Course of Different Manifestations of Cerebral Small-Vessel Disease Study.

Endothelial dysfunction has been implicated in the pathogenesis of cerebral small-vessel disease (SVD). Little is known about the relationship between SVD and measures of endothelium-dependent vasodilatation and cerebral vasomotor reactivity. The aim of this study was to evaluate cerebral and extracerebral endothelial dysfunction in patients with different manifestations of SVD and to assess the relationship between endothelial dysfunction and radiologic markers of SVD. The vasomotor reactivity reserve (VMRr), breath-holding index (BHI) of the middle cerebral arteries, and brachial artery flow-mediated dilatation (FMD) were measured with ultrasound techniques in 90 patients (30 in each group) older than 60 years with extensive white matter lesions (Fazekas grade ≥ 2) with a history of lacunar stroke, vascular dementia, or parkinsonism and 30 individuals with normal magnetic resonance imaging findings (control group). All groups were matched for age, sex, hypertension, and diabetes. The mean age ± SD (71.8 ± 3.4 versus 71.7 ± 3.4 years), sex distribution, and prevalence of the main vascular risk factors were similar in the SVD and control groups. The VMRr (56.6% ± 18.3% versus 77.1% ± 16.9%), BHI (0.8 ± 0.3 versus 1.1 ± 0.4), and FMD (5.8% ± 4 versus 12.1% ± 5.2%) were severely impaired in the SVD groups compared to the control group (P < .01). The vascular responses to all tests was similar in the SVD groups, but they were significantly decreased in patients with severe white matter lesions, marked brain atrophy, and enlarged perivascular spaces. This study was the first that simultaneously evaluated cerebral and extracerebral vasodilator responses in a well-phenotyped cohort of patients with lacunar stroke, vascular dementia, or parkinsonism. The VMRr, BHI, and FMD were more severely impaired in patients with SVD, regardless of its clinical manifestation, than in control participants. All measures were significantly lower in patients with severe white-matter lesions, brain atrophy, or enlarged perivascular spaces.

Different features between pediatric-onset and adult-onset patients who are seropositive for MOG-IgG: A multicenter study in South China

BackgroundImmunoglobulin against myelin oligodendrocyte glycoprotein (MOG-IgG) is a potential demyelinating disease-associated autoantibody. Whether clinical features of MOG antibody-associated demyelinating diseases change with age remains unclear. ObjectTo investigate the different clinical features between pediatric-onset and adult-onset MOG-IgG-seropositive patients in a relatively large cohort. MethodsA total of 816 consecutive patients with suspected demyelinating disease were prospectively enrolled from three tertiary academic centers in South China from February 2016 to December 2016. Sixteen pediatric-onset cases (≤14 years old) and 34 adult-onset cases (>14 years old) seropositive for MOG-IgG were identified. Differences in clinical features between the two groups were investigated. ResultsThere was a significant difference in the cumulative incidence of first relapse among the two groups (P = .008). Cerebral symptoms were significantly higher in pediatric-onset patients than in adult-onset patients, either at disease onset (pediatric-onset group, 10/16(62.5%); adult-onset group, 8/34(23.53%); P = .007) or throughout the course of disease (pediatric-onset group, 11/16(68.8%); adult-onset group, 10/34(29.4%); P = .009). Optic nerve symptoms were more common in adult-onset groups, but no significant difference was found between the two groups. A significantly higher rate of pediatric-onset patients (9/16, 56.3%) met the acute disseminated encephalomyelitis criteria compared with adult-onset patients (2/34, 5.9%) (P = .0003), and isolated optic neuritis was mainly diagnosed in adult-onset patients (pediatric-onset group, 2/16(12.5%); adult-onset group, 14/34(41.2%); P = .043). The MOG-IgG titer showed a significant positive correlation with total protein levels in cerebrospinal fluid, but only in adult-onset patients (r = 0.95; P = .0004). On magnetic resonance imaging, extensive white matter lesions were observed in both groups, and the number was much higher in pediatric-onset (7/15, 46.7%) than in adult-onset patients (4/29, 13.8%) (P = .043). At the last follow-up, more pediatric-onset patients (10/16, 62.5%) experienced complete recovery (EDSS 0.0 at last follow up) compared with adult-onset patients (9/34, 26.5%) (P = .014). ConclusionsDistinctive features are present between pediatric-onset and adult-onset patients with MOG-IgG. Further studies are required to determine the different underlying pathogenesis of MOG antibody at different ages.