Journal of the Neurological Sciences

BackgroundUnruptured intracranial aneurysms (UIAs) are rarely reported in primary central nervous system vasculitis (PCNSV). In this study we described the clinical findings, response to therapy, and outcomes of UIA in a large cohort of PCNSV patients. MethodsWe retrospectively studied 216 consecutive patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 40-year period. UIAs were identified on cerebral angiography. The clinical, laboratory, radiologic and pathologic findings, management, and outcomes of patients with UIA were described and compared with those without UIA. Results12/216 (5.5%) PCNSV patients had at least one UIA. In the only positive patient biopsy showed necrotizing vasculitis. Eleven patients had evidence of UIA on angiogram at diagnosis. One patient developed an aneurysm during the follow-up associated with a worsening of vasculitic radiological findings. The most common presenting symptom for PCNSV in the setting of UIA was headache (67%), followed by persistent neurologic deficit or stroke (50%). Most patients with UIA presented with multiple cerebral infarcts on MRI (67%), one patient had subarachnoid hemorrhage, and one left parieto-occipital intracerebral hematoma, both unrelated to the aneurysm. Black blood imaging was performed in 4 patients and 2 showed segmental circumferential mural enhancement involving multiple vessels. Two patients had 2 UIAs, while the other 10 had 1. The most frequent UIA location was internal carotid artery (50%), followed by anterior cerebral artery (21%). Ten of the UIAs were < 5 mm in diameter, and 3 were 5-7 mm in diameter; the size was not available for one. All UIAs were unchanged in size and configuration during follow-up and no new aneurysms were detected. Compared to the 204 patients with PCNSV without a UIA, no significant clinical differences were observed, except for a reduced disability at last follow-up (p = 0.038). ConclusionsUIAs uncommonly occur in PCNSV.

BackgroundSARS-CoV-2 induced coagulopathy can lead to thrombotic complications such as stroke. Cerebral venous sinus thrombosis (CVST) is a less common type of stroke which might be triggered by COVID-19. We present a series of CVST cases with SARS-CoV-2 infection. MethodsIn a multinational retrospective study, we collected all cases of CVST in SARS-CoV-2 infected patients admitted to nine tertiary stroke centers from the beginning of the pandemic to June 30th, 2020. We compared the demographics, clinical and radiological characteristics, risk factors, and outcome of these patients with a control group of non-SARS-CoV-2 infected CVST patients in the same seasonal period of the years 2012-2016 from the country where the majority of cases were recruited. ResultsA total of 13 patients fulfilled the inclusion criteria (62% women, mean age 50.9{+/-} 11.2 years). Six patients were discharged with good outcomes (mRS[&le;]2) and three patients died in hospital. Compared to the control group, the SARS-CoV-2 infected patients were significantly older (50.9 versus 36.7 years, p<0.001), had a lower rate of identified CVST risk factors (23.1% versus 84.2%, p<0.001), had more frequent cortical vein involvement (38.5% versus 10.5%, p: 0.025), and a non-significant higher rate of in-hospital mortality (23.1% versus 5.3%, p: 0.073). ConclusionCVST should be considered as potential comorbidity in SARS-CoV-2 infected patients presenting with neurological symptoms. Our data suggest that compared to non-SARS-CoV-2 infected patients, CVST occurs in older patients, with lower rates of known CVST risk factors and might lead to a poorer outcome in the SARS-CoV-2 infected group.

BackgroundIntracranial vessel wall enhancement (VWE) on high-resolution magnetic resonance imaging (HRMRI) is associated with the progression and poor prognosis of moyamoya disease (MMD). However, the genetic background and risk factors for VWE in MMD have not been investigated. Therefore, this study assessed potential risk factors for VWE in MMD. MethodsWe evaluated MMD patients using HRMRI and traditional angiography examinations. The participants were divided into VWE group and non-VWE group based on the presence or absence of VWE on HRMRI. Logistic regression was performed to compare the risk factors for VWE in MMD. The incidence of cerebrovascular events of the different subgroups according to risk factors were compared using Kaplan - Meier survival and Cox regression.. STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) guidelines were followed for reporting our cohort study. ResultsWe included 283 MMD patients, 84 of whom had VWE on HRMRI. The VWE group had higher modified Rankin Scale scores at admission (P = 0.014) and a higher incidence of ischemia and hemorrhage (P =0.002) than did the non-VWE group. Multiple logistics regression shows risk factors for VWE included the ring finger protein 213 (RNF213) pR4810K variant (OR: 2.01 [95% confidence interval (CI), 1.08 - 3.76]; P = 0.028), hyperhomocysteinemia (HHcy; OR: 5.08 [95% CI, 2.34 - 11.05], P < 0.001), and smoking history (OR, 3.49 [95% CI, 1.08-11.31], P = 0.037). During the follow-up of 63.9 {+/-} 13.2 months (medium 65 months), 18 recurrent stroke events occurred. Cox regression showed that VWE and RNF213 pR4810K variant were risk factors for follow-up stroke. ConclusionThe RNF213 p.R4810K variant is strongly associated with VWE and poor prognosis in MMD. HHcy and smoking are independent risk factors for VWE; both are likely to induce the VWE phenomenon by affecting vascular endothelial function or causing vascular endothelial damage.

BackgroundAt the end of December 2019, a novel respiratory infection, initially reported in China, known as COVID-19 initially reported in China, and later known as COVID-19, led to a global pandemic. Despite many studies reporting respiratory infections as the primary manifestations of this illness, an increasing number of investigations have focused on the central nervous system (CNS) manifestations in COVID-19. In this study, we aimed to evaluate the CNS presentations in COVID-19 patients in an attempt to identify the common CNS features and provide a better overview to tackle this new pandemic. MethodsIn this systematic review and meta-analysis, we searched PubMed, Web of Science, Ovid, Embase, Scopus, and Google Scholar. Included studies were publications that reported the CNS features between January 1st, 2020, to April 20th, 2020. The data of selected studies were screened and extracted independently by four reviewers. Extracted data analyzed by using STATA statistical software. The study protocol registered with PROSPERO (CRD42020184456). ResultsOf 2353 retrieved studies, we selected 64 studies with 11282 patients after screening. Most of the studies were conducted in China (58 studies). The most common CNS symptom of COVID-19 were Headache (8.69%, 95%CI: 6.76%-10.82%), Dizziness (5.94%, 95%CI: 3.66%-8.22%), and Impaired consciousness (1.9%, 95%CI: 1%-2.79%). ConclusionsThe growing number of studies have reported COVID-19, CNS presentations as remarkable manifestations that happen. Hence, understanding the CNS characteristics of COVID-19 can help us for better diagnosis and ultimately prevention of worse outcomes.

Background and aimsRecent reports reveal incidences of spinal cord involvement in form of para-infectious or post-infectious myelitis raising potential concerns about the possibilities of SARS-CoV-2 behind the pathogenesis of spinal cord demyelination. In this study, we intend to summarise so far available pieces of evidence documenting SARS-CoV-2 mediated spinal demyelination in terms of clinical, laboratory parameters and imaging characteristics. MethodologyThis review was carried out based on the existing PRISMA (Preferred Report for Systemic Review and Meta-analyses) consensus statement. Data was collected from four databases: Pubmed/Medline, NIH Litcovid, Embase and Cochrane library and Preprint servers up till 10th September, 2020. Search strategy comprised of a range of keywords from relevant medical subject headings which includes "SARS-COV-2", "COVID-19", "demyelination" etc. ResultsA total of 21 cases were included from 21 case reports after screening from various databases and preprint servers. Biochemical analysis reveals that the majority of cases showed elevated CSF protein as well as lymphocytic pleocytosis. Interestingly, a majority of cases were found to be associated with long extensive transverse myelitis (LETM), and remaining cases were found to be associated with isolated patchy involvement or isolated short segment involvement or combined LETM and patchy involvement. Few cases were also found with significant co-involvement of the brain and spine based on the imaging data. ConclusionIt can be interpreted that SARS-CoV-2 may play a potential role in spinal demyelinating disorders in both para-infectious and post-infectious forms. HighlightsO_LIImaging data reveals LETM, short and patchy involvements C_LIO_LIPara infectious myelitis precedes post-infectious manifestation C_LIO_LIAltered CSF parameters and myelitis-like symptoms at the onset of COVID-19 C_LIO_LISimilar spinal cord involvements in related HCoVs infections C_LI

ObjectiveTo report three patients infected with COVID-19 with severe respiratory syndrome requiring intubation, who developed acute demyelinating encephalomyelitis (ADEM). MethodPatient data were obtained from medical records from the North Memorial Health Hospital, Robbinsdale, MN, USA ResultsThree patients (two men and one woman, aged 38 - 63) presented with fatigue, cough and fever leading to development of acute respiratory distress syndrome secondary to COVID-19 infection requiring intubation and ventilatory support. Two patients were unresponsive, one with strong eye deviation to the left and the third patient had severe diffuse weakness. MRI in all patients showed findings consistent with ADEM. CSF showed elevated protein in all patients with normal cell count and no evidence of infection, including negative COVID-19 PCR. All three of the patients received Convalescent plasma therapy for COVID-19. All patients were treated with intravenous corticosteroids and improved, although two responded minimally. Two patients treated with IVIG showed no further improvement. ConclusionNeurological complications from COVID-19 are being rapidly recognized. Our three cases highlight the occurrence of ADEM as a postinfectious/immune mediated complication of COVID-19 infection, which may be responsive to corticosteroid treatment. Early recognition of this complication and treatment is important to avoid long term complications.

Structured AbstractO_ST_ABSImportanceC_ST_ABSCerebral microvascular lesions are common in patients with severe COVID-19. Radiologic-pathologic correlation in one case suggests a combination of microvascular hemorrhagic and ischemic lesions that may reflect an underlying hypoxic mechanism of injury, which requires validation in larger studies. ObjectiveTo determine the incidence, distribution, and clinical and histopathologic correlates of microvascular lesions in patients with severe COVID-19. DesignObservational, retrospective cohort study: March to May 2020. SettingSingle academic medical center. ParticipantsConsecutive patients (n=16) admitted to the intensive care unit with severe COVID-19, undergoing brain MRI for evaluation of coma or focal neurologic deficits. ExposuresNot applicable. Main Outcome and MeasuresHypointense microvascular lesions identified by a prototype ultrafast high-resolution susceptibility-weighted imaging (SWI) MRI sequence, counted by two neuroradiologists and categorized by neuroanatomic location. Clinical and laboratory data (most recent measurements before brain MRI). Brain autopsy and cerebrospinal fluid PCR for SARS-CoV-2 in one patient who died from severe COVID-19. ResultsEleven of 16 patients (69%) had punctate and linear SWI lesions in the subcortical and deep white matter, and eight patients (50%) had >10 SWI lesions. In 4/16 patients (25%), lesions involved the corpus callosum. Brain autopsy in one patient revealed that SWI lesions corresponded to widespread microvascular injury, characterized by perivascular and parenchymal petechial hemorrhages and microscopic ischemic lesions. Conclusions and RelevanceSWI lesions are common in patients with neurological manifestations of severe COVID-19 (coma and focal neurologic deficits). The distribution of lesions is similar to that seen in patients with hypoxic respiratory failure, sepsis, and disseminated intravascular coagulation. Collectively, these radiologic and histopathologic findings suggest that patients with severe COVID-19 are at risk for multifocal microvascular hemorrhagic and ischemic lesions in the subcortical and deep white matter. Key Points SectionO_ST_ABSQuestionC_ST_ABSWhat is the prevalence and pathophysiology of cerebral microvascular injury in patients with severe COVID-19? FindingsIn this retrospective cohort study of 16 patients undergoing MRI for neurologic complications of severe COVID-19, microvascular lesions were observed in 11 patients and showed an anatomic distribution similar to that seen in patients with hypoxic respiratory failure and sepsis. In one patient who died, brain autopsy revealed widespread microvascular injury, including perivascular microhemorrhages and microscopic ischemic lesions. MeaningMicrovascular injury is common in patients with severe COVID-19. Radiologic-pathologic correlation, though limited to a single case, provides insights into possible mechanisms of injury.

BackgroundNeurological disability associated with multiple sclerosis and immunosuppressive or immunomodulatory therapy which is administered for it may increases the risk of SARS-CoV-2 infection and its morbidity/mortality. ObjectiveIn this study, we evaluated the infection rate and the severity of SARS-CoV-2 infection in patients with multiple sclerosis (MS) MethodsOne thousand and three hundred and sixty one MS patients from Fars province, south of Iran, were interviewed by phone from April 3 to June 20, 2020. Basic demographic data, information about MS disease and any symptoms or laboratory results relevant to COVID-19 were gathered and reviewed by treating neurologist and MS nurses. SPSS version 22 was used for data analysis. Results68 (5%) of MS patients were suspected cases and 8 (0.58%) of all patients with positive real-time reverse transcription polymerase chain reaction (RT-PCR) or chest CT were in the confirmed group. 5 cases of the confirmed group needed hospitalization. Two patients died while both of them had PPMS and were taking rituximab. The frequency rate of suspected cases with RRMS was 57 (87.7%), followed by PPMS 5 (7.7%) and CIS 2(3.1%). In the confirmed group 37.5% had RRMS, 50% had PPMS, 25% use corticosteroid drug, and 50% were on rituximab. 62.5% of confirmed cases had high disability level and need assistance to walk. 36.8% of suspected and 25% of the confirmed cases were on IFN-{beta}1; eventually all of them recovered well from COVID-19 infection. ConclusionThe present study showed that rate of developing COVID-19 in MS patients are similar to the general population and the frequency of PPMS phenotype, rituximab therapy and corticosteroid therapy were higher in the confirmed group.

BackgroundNew Daily Persistent Headache (NDPH) is an enigmatic syndrome characterized by a distinct onset headache that becomes continuous and unremitting within 24 hours and persists for at least 3 months without underlying structural cause. Patients may experience years or decades of pain with negative impacts on functionality and quality of life. Although characteristically treatment resistant, case reports of response to pharmacologic therapies exist, though with great heterogeneity and without generalizability. Given NDPH is incompletely characterized and displays phenotypic variability, it is possible that better sub-population identification might yield more targeted therapeutic directions. MethodsWe conducted a retrospective chart review of adult patients seen within the University of Pittsburgh Medical Center System who received an NDPH International Classification of Diseases 9 or 10 code between 2011-2019. 5,926 patient charts were reviewed and only those who satisfied International Classification of Headache Disorders, 3rd edition criterion were further analyzed. Two independent data collectors reviewed each chart. Results466 individuals satisfied NDPH diagnostic criteria. Median age at first consultation was 37.1 years, with 66.7% of the population identifying as female. Length of NDPH duration at the last consultation was 1.75 years. Headache onset exhibited no obvious seasonality. Headaches were unilateral in 12.5% of cases and severe pain was reported in 79.1% of cases with documented pain severity. Thunderclap onset was observed in 5.8% of cases. Further categorization into migraine (MP) and tension (TP) phenotypes found approximately even distribution, 207 and 214 respectively. Both MP and TP exhibited demographics, precipitating factors, and prognostic types reflective of the general population. In comparison to TP, MP patients described increased prevalence of severe pain (48.3% vs 14.0%), nausea (68.6% vs 33.6%), vomiting (25.1% vs 7.0%), light-sensitivity (85% vs 27.1%), noise-sensitivity (80.7% vs 24.8%), and osmophobia (19.3% vs 3.7%). Comparison of MP and TP exacerbating factors found increased reporting of physical activity (23.7% vs 10.3%). Chronicity of MP and TP found similar breakdowns with 26.1% vs 23.4% reporting 5+ years of headache. ConclusionsNDPH is a refractory, severe pain headache disorder that exhibits no seasonality for onset, with equal breakdown of MP and TP subtypes.

IntroductionSevere SARS-CoV-2 infection, responsible for COVID-19, is accompanied by venous thromboembolic events particularly in intensive care unit. In non-severe COVID-19 patients affected by neurovascular diseases, the prevalence of deep venous thrombosis (DVT) is unknown. The aim of or study was to report data obtained after systematic Doppler ultrasound scanning (DUS) of lower limbs in such patients. MethodsBetween March 20 and May 2, 2020, consecutive patients with neurovascular diseases with non-severe COVID-19 were investigated with a systematic bedside DUS. ResultsThirteen patients were enrolled including 10 acute ischemic strokes, one transient ischemic attack, one cerebral venous thrombosis and one haemorrhagic stroke. At admission the median National Institute of Health Stroke Scale (NIHSS) was of 6 (IQR, 0-20). We found a prevalence of 38.5% of asymptomatic calves DVT (n = 5) during the first week after admission despite thromboprophylaxis. Among them, one patient had a symptomatic pulmonary embolism. Two patients died during hospitalization but the outcome was favourable in the others with a discharge median NIHSS of 1 (IQR, 0-11). Discussion/ConclusionDespite thromboprophylaxis, systematic bedside DUS showed a high prevalence of 38.5% of DVT in non-severe COVID-19 patients with neurovascular diseases. Therefore, we suggest that this non-invasive investigation should be performed in all patients of this category.

IntroductionAutoimmune encephalitis (AE), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are complex and debilitating neurological disorders. MethodsThis study uses the Vaccine Adverse Event Reporting System (VAERS) to investigate the potential relationship between vaccinations and the incidence of NMOSD, AE, and MOGAD. Potential risk factors, such as age, sex, type of vaccine, and previous history of autoimmune diseases, were examined using multivariate logistic regression analysis. ResultsOur analysis included 161 cases: 72 NMOSD, 82 AE, and 7 MOGAD. The COVID-19 vaccine was implicated in 19/72 (26.3%) NMOSD, 43/82 (52.4%) of AE and 6/7 (85.7%) of MOGAD. The subacute temporal profile (OR 24.4, p = 0.004) and the presence of any comorbidity (OR 12.49, p = 0.004) were significantly associated with hospitalization for those with NMOSD. The subacute onset of symptoms and encephalopathy was statistically significant for hospitalization (OR 6.15, p = 0.048) and (OR 10.3, p = 0.005) respectively for patients with AE. Anti-NMDAR (N-methyl D-aspartate) antibodies were observed in 16/24 (66.7%) of AE. Treatment often involved high-dose corticosteroids or intravenous immunoglobulin (IVIG). ConclusionsMost cases of vaccine induced NMOSD, AE, and MOGAD occurred secondary to the SARS-CoV-2 vaccine. The subacute onset of symptoms and the presence of encephalopathy was most associated with hospitalization.

IntroductionDespite the new SARS-CoV-2 (COVID-19) be the seventh of the coronavirus family viruses known to cause human disease, little is known about potential symptoms and syndromes secondary to the compromise of the central and peripheral nervous systems. We reviewed neurological manifestations due to the new coronavirus, thus far published in the literature, as well as guidelines issued by sub-specialties in Neurology, to tackle the disease. Methodswe searched medical databases, such as PubMed, PubMed Central, LILACS and Google scholar for papers (case reports, short letters, case series, etc) describing neurological symptoms in patients with confirmed or suspect COVID-19 diagnosis and also searched webpages of associations and organizations that deal with neurological disorders. Resultswe describe briefly each article considered for this review. Forty-one papers were found associating neurological conditions and COVID-19. Cases are divided by disease groups and, within each disease group, results are listed in chronological order or publication date. We also discuss briefly recommendations for neurological patients, according to disease group. ConclusionAlthough there is evidence of neurological manifestations with previous coronaviruses, COVID-19 is assuring a volume of published papers not seen before for other coronavirus infections. Most neurological cases are not life-threatening, but 10 to 20% of cases will require hospitalization and are in risk for sequelae and death. Although a lot of data coming from these papers is amassing, researchers must bear in mind that many papers currently published are not yet peer-reviewed, and thus are prone to further corrections.

Neurological manifestations of COVID-19 are increasingly described in the literature. There is uncertainty whether these occur due to direct neuroinvasion of the virus, para-infectious immunopathology, as result of systemic complications of disease such as hypercoagulability or due to a combination of these mechanisms. Here we describe clinical and radiological manifestations in a sequential cohort of patients presenting to a district hospital in South Africa with neurological symptoms with and without confirmed COVID-19 during the first peak of the epidemic. In these patients, where symptoms suggestive of meningitis and encephalitis were most common, thorough assessment of presence in CSF via PCR for SARS-CoV2 did not explain neurological presentations, notwithstanding very high rates of COVID-19 admissions. Although an understanding of potential neurotropic mechanisms remains an important area of research, these results provide rationale for greater focus towards the understanding of para-immune pathogenic processes and the contribution of systemic coagulopathy and their interaction with pre-existing risk factors in order to better manage neurological disease in the context of COVID-19. These results also inform the clinician that consideration of an alternative diagnosis and treatment for neurological presentations in this context is crucial, even in the patient with a confirmed diagnosis COVID-19.

Chiari malformation type I(CMI) is a common condition. It is a subject of controversy from diagnosis to the management (16). Classically the diagnosis is made on clinical basis and radiological measurement of cerebellar tonsils herniation of 5mm or more below the opisthion-basion line in mid-sagittal plane(Mc Rae line.) The aim of our study was to determine the relationship between clinical presentation of CMI and cerebellar tonsil herniation measured in three dimensions, cerebellar tonsils volume and the volume ratio (cerebellar tonsils volume/Foramen magnum volume) within foramen magnum. Can the volume of cerebellar tonsils herniation and the volume ratio(cerebellar tonsils volume/volume foramen magnum) reflect better the severity of patients with CMI? the study is the first in current literature eliciting the relationship between myelopathy severity and headache severity in CMI patients; cerebellar tonsils volume and T/F volume ratio (cerebellar tonsils volume /Foramen magnum volume) MethodsWe conducted an observational cross sectional analytical study. Patients with clinical and radiological confirmation of CMI evaluated on cranial cervical MRI were enrolled. Three dimension morphometric measures of cerebellar tonsils was made, the volume of cerebellar tonsils was calculated using ellipsoid volume formula. The transverse diameter of foramen magnum was measured and the volume of foramen magnum was calculated using sphere formula. We computed various non-parametric statistical tests and hypothesis testing to analyze variation of cerebellar tonsils uniplanar measurements, cerebellar tonsils volume, and T/F volume ratio (Cerebellar tonsils volume/foramen magnum volume), and to analyze correlation between these measurements with the severity of myelopathy using modified Japanese orthopedics association score(mJOA) and headache severity using pain numeric rating scale. We did all the calculations in python 3 using scipy. stats, Wilcoxon, Pearson, seaborn, and matplotlib.pyplot packages and pandas library ResultsChiari malformation type I was more common in female with 61.5% and male patients with CMI was 38.5%. The majority of patients with CMI were in fourth and fifth decade. Occipital headache was the most presenting symptom followed by limb paraesthesia, vertigo, difficulty walking and bulbar symptoms. Scoliosis associated with CMI was found in 5% while syringomyelia associated with CMI was found in 8%. According to numeric pain scale; patients with CMI mostly presented with severe headache and moderate headache with 58.3% and 41.7 % respectively. There is difference between right and left sagittal tonsils measurement; the left median sagittal measurement is 7.8 mm while the right median sagittal measurement is 8.8 mm with P-value <0.001 The coronal and sagittal cerebellar tonsils measurements are statistically different. The median difference and interquartile range(IQR) between coronal and sagittal measurements were 0.6(-0.4 1.8) and p-value <0.001 respectively The finding showed a correlation between myelopathy severity and the volume of herniated cerebellar tonsils as well as correlation between myelopathy severity and T/F volume ratio (Cerebellar tonsils volume/Foramen magnum volume). There was no correlation between headache severity and sagittal measurement as we failed to reject hypothesis p=0.661 Spearmans correlation coefficient: -0.045 In contrast there was a correlation between headache severity and cerebellar tonsils volume as well as T/F volume ratio with P-value 0.03 in our study. ConclusionTwo dimensions radiological measurements in assessment of CMI is not reflecting the clinical severity of patients with CMI. Consideration of both clinical presentation and radiological measurement in assessment of severity of CMI is of great importance rather than only considering the cut off 5 mm descent of cerebellar tonsils herniation in midsaggital plan. Cerebellar tonsils volume and T/F volume ratio(cerebellar tonsils volume /foramen magnum volume) are the indicators of severity of myelopathy and headache severity as shown in our study.

Background and aimsWith the growing number of COVID-19 cases in recent times, the varied range of presentations is progressively becoming an addressing issue among clinicians. A significant set of patients with extra pulmonary symptoms has been reported worldwide. Neurological involvement in the form of altered mental status, loss of consciousness in considerable amounts has drawn attention of physicians all across the globe. Here we venture out to summarise the clinical profile, investigations and radiological findings among patients with SARS-CoV-2 associated meningoencephalitis in the form of a systematic review, which may aid clinicians in early diagnosis and prognostic evaluation of the disease. MethodologyThis review was carried out based on the existing PRISMA (Preferred Report for Systemic Review and Meta analyses) consensus statement. The data for this review was collected from four databases: Pubmed/Medline, NIH Litcovid, Embase and Cochrane library and Preprint servers up till 10th June, 2020. Search strategy comprised of a range of keywords from relevant medical subject headings which includes "SARS-COV-2", "COVID-19", "meningoencephalitis" etc. All peer reviewed, case control, case report, pre print articles satisfying our inclusion criteria were involved in the study. The inclusion prerequisites comprised of confirmed SARS-CoV-2 cases with neurological manifestations, previous cases of SARS-CoV, MERS-CoV with neurological involvement provided all the studies were published in English language. Quantitative data was expressed in mean+/-SD, while the qualitative date in percentages. Paired t test was used for analysing the data based on differences between mean and respective values with a p value of <0.05 considered to be statistically significant. ResultsA total of 43 cases were involved from 24 studies after screening from databases and preprint servers, out of which 29 of them had completed investigation profile and were included in the final analysis. Clincial and Laboratory findings as well as neuroimaging findings (CT, MRI and MRS) revealed consistent presentations towards association of COVID-19 with meningoencephalitis. Epileptogenic pictures were also evident on EEG (electroencephalogram) findings. ConclusionSARS-CoV-2 has been isolated from CSF as well as cerebrum of cases with meningoencephalitis depicting the natural tendency of the virus to invade the central nervous system. Speculations about retrograde olfactory transport or alternative haematogenous spread seem to be correlating with above findings. This review may raise the index of suspicion about COVID-19 among clinicians while attending patients with neurological manifestations.

BackgroundCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic hereditary cerebral small vessel disease (CSVD). Existing studies have confirmed that it is caused by mutations in the NOTCH3 gene, but the specific mechanisms underlying its pathogenesis and progression remain elusive. Existing research indicates that inflammation plays a critical role in the development and progression of CSVD. The Systemic Immune-Inflammation Index (SII) has revealed to be a reliable new marker to assess immune status and inflammatory response intensity. This study reveals the relationship of SII with cognitive impairment and magnetic resonance imaging (MRI) markers of CSVD in patients with CADASIL. MethodsThis cross-sectional investigate included patients diagnosed with CADASIL who had confirmed NOTCH3 gene mutations and complete clinical data. Cognitive function in patients with CADASIL was appraised by the Mini-Mental State Examination (MMSE). SII is obtained by calculating the number of platelets, neutrophils and lymphocytes in blood routine examination. Summary SVD score and imaging markers of CSVD, including cerebral microbleeds, lacunae, white enlarged perivascular space and matter hyperintensity were evaluated based on magnetic resonance imaging. The association between cognitive impairment and SII and MRI markers in CADASIL were evaluated using logistic regression models and Spearman correlation. ResultsAt baseline, A total of 96 Patients with CADASIL were enrolled in this cross-sectional study. the median age of patients with CADASIL was 59.00 (interquartile range 52.25-66.75) years, and 58.3% of patients were male. The correlation analysis results indicate that the SII level was negatively correlated with MMSE scores in patients with CADASIL (rs=-0.336, P <0.001). An elevated SII was statistically significantly linked with the risk of cognitive impairment (Q4 vs. Q1: OR 5.230, 95% CI 1.040-26.297; P=0.045) after adjusting for age, sex and education. In contrast, there was no considerable difference between SII and summary SVD score or MRI imaging markers. ConclusionsElevated SII was linked with cognitive impairment in CADASIL patients. Nevertheless, there were no significant differences between SII and summary SVD score or MRI imaging markers.

ObjectiveLumbar puncture opening pressure (LPOP) exceeding 250mmH2O stands as a pivotal criterion in diagnosing idiopathic intracranial hypertension (IIH), according to the revised Friedmans criteria. However, many studies discuss the variability of LPOP, while others highlight the accuracy of radiological findings as a credible diagnostic tool for IIH. We have encountered many symptomatic patients who did not meet the LPOP criteria (with or without papilledema), despite having IIH-related symptoms and neuroimaging findings. This study aimed to investigate the radiological findings and clinical symptoms in patients suspected of having IIH without meeting the LPOP criteria. MethodsWe retrospectively evaluated cerebral venous sinus stenosis using the conduit Farb score (CFS) as well as other radiological findings suggestive of IIH by computed tomography venography and magnetic resonance venography in female patients [&ge;]18 years of age with chronic headaches and suspected IIH with an LPOP <250mm. ResultsOur cohort comprised eighty-eight women (56 with LPOP < 200 mm H2O and 32 with LPOP ranging between 200-250 mmH2O). Among patients with LPOP in the 200-250 mmH2O range, 40% (12 patients) exhibited three or more radiological findings supporting IIH, compared to 17% (8 patients) in the LPOP < 200 mmH2O group (p=0.048). Furthermore, cerebral venous stenosis, as measured by a Conduit Farb Score (CFS) of 5 or lower, was observed in 80% (24 patients) of the LPOP 200-250 mmH2O group, contrasting with 40% (19 patients) in the LPOP < 200 mmH2O group (p<0.001). ConclusionCerebral venous stenosis, as well as other supportive radiological findings, were significantly more common in patients with LPOP 200-250 mmH2O than LPOP<200 mmH2O. These findings suggest that given supportive clinical and radiological evidence, patients with LPOP between 200-250 mmH2O, with or without papilledema, may benefit from treatment for IIH.

ObjectiveThe Circle of Willis (CoW) is often underdeveloped or incomplete, leading to suboptimal blood supply to the brain. As hypoperfusion is thought to play a role in the aetiology of white matter hyperintensities (WMH), the objective of this study was to assess whether incomplete CoW variants were associated with increased WMH volumes compared to the complete CoW. MethodsIn a cross-sectional population sample of 1864 people (age 40 - 84 years, 46.4% men), we used an automated method to segment WMH using T1-weighted and T2-weighted fluid-attenuated inversion recovery image obtained at 3T. CoW variants were classified from time-of-flight scans, also at 3T. WMH risk factors, including age, sex, smoking and blood pressure, were obtained from questionnaires and clinical examinations. We used linear regression to examine whether people with incomplete CoW variants had greater volumes of deep WMH (DWMH) and periventricular WMH (PWMH) compared to people with the complete CoW, correcting for WMH risk factors. ResultsParticipants with incomplete CoW variants did not have significantly higher DWMH or PWMH volumes than those with complete CoW when accounting for risk factors. Age, pack-years smoking, and systolic blood pressure were risk factors for increased DWMH and PWMH volume. Diabetes was a unique risk factor for increased PWMH volume. ConclusionIncomplete CoW variants do not appear to be risk factors for WMH in the general population.

Neuromyelitis optica is rare, autoimmune-mediated inflammation and demyelination of the central nervous system with a prevalence of 1-2 persons per 100,000 populations. We aim to generate a head-to-head comparison of these drugs with appropriate evidence to guide future trials and treatment guidelines in a patient with recurrent attacks of NMO. We searched the databases like PubMed, MEDLINE, Cochrane Central Register of Controlled Trials (CENTRAL) and Embase for studies published prior to April 2021 using the keywords. Over all 929 patients from 11 different publications were included in the study. Five studies were included for quantitative synthesis. Pooling of studies showed significant mean reduction of ARR in the monoclonal antibody group (-0.26 [-0.35, -0.17], P <0.00001, I2=0%) and the mean difference in EDSS score from baseline in monoclonal antibodies was - 0.23(95% CI [-0.43, -0.03], P=0.02, I2=0%). There was no significant difference in frequency of total reported adverse events between monoclonal antibody and the comparator arm (RR: 1.01 [0.95, 1.07], P=0.74, I2=14%). Our findings, particularly seen from the context of a few RCTs, support the pursuit of larger, multi-center RCTs that evaluate the effectiveness of each of the currently available monoclonal antibodies and better describe their adverse risk profile.

Background and ObjectivesFocal cerebral arteriopathy (FCA) is a recognized cause of pediatric stroke, but its presentation in adults is poorly defined, with limited cohorts and scarce advanced imaging data. We aimed to describe the clinical spectrum, radiological features, treatment strategies, and outcomes of adult FCA in the largest single-center cohort to date. MethodsWe retrospectively reviewed consecutive adults (>18 years) with ischemic stroke admitted between 2017 and 2024. FCA was defined as unilateral focal stenosis/irregularity of anterior circulation arteries (terminal ICA, M1, M2, A1) after excluding mimics such as vasculitis, dissection, embolic occlusion, and intracranial atherosclerosis. All underwent MRI with contrast-enhanced vessel wall imaging and confirmation on a second modality. Clinical, radiological, and outcome data were collected. Severity was scored using the FCA Severity Score (FCASS). ResultsOf 2,237 stroke admissions, 47 patients (2.1%) met criteria for FCA. Median age was 30 years, with near-equal sex distribution. Hemiparesis with or without aphasia predominated, and strokes were generally mild (median NIHSS 3, mRS 1.2). Preceding clustered TIAs occurred in one-third, and 32% reported new unilateral headache. Infarcts often involved lenticulostriate and MCA watershed territories. Median FCASS was 4. Vessel wall imaging showed concentric enhancement in 74.5%. All patients received antiplatelets; 49% received additional immunosuppression, most often IV methylprednisolone. Over a median 6-month follow-up (mean 12.4), no stroke recurrences occurred, though 5 patients showed radiological progression and 2 developed contralateral stenosis. Functional outcomes were favorable, with 87% achieving mRS 0-2. DiscussionAdult FCA is uncommon but clinically distinct, marked by mild strokes, clustered TIAs, and concentric vessel wall enhancement. The course was largely monophasic, with favorable functional outcomes and no recurrent strokes, regardless of immunosuppressive therapy. Radiological progression was rare but included contralateral involvement, raising the possibility of overlap with unilateral moyamoya disease. Vessel wall imaging is valuable for diagnosis, and longer follow-up is needed to clarify pathogenesis and treatment strategies.