Acute stroke in familial Mediterranean fever: An analysis of 23 new cases and systematic review of the literature.

OBJECTIVE: The nature of neurovascular involvement in cases of familial Mediterranean fever (FMF) has not been adequately clarified. METHODS AND PATIENTS: Clinical features, infarct topography, vascular status, and stroke etiology were prospectively determined in 35 acute neurovascular events that occurred in 23 FMF patients. Clinicoradiological features were compared with an age- and gender-matched control group of 115 acute stroke patients. Characteristics of additional FMF and acute stroke cases (6 episodes in 6 patients) identified from a systematic literature review (PROSPERO registration no: CRD420212264820) were also analyzed. RESULTS: There were 27 acute ischemic stroke episodes in 19 patients, 7 transient ischemic attack episodes in 3 patients, and 1 patient with a single episode of parietal hematoma in our cohort. Twenty (74%) ischemic stroke episodes in 12 patients were cryptogenic. Ten of these 12 cases had a previous FMF diagnosis and were taking colchicine. There was no significant difference in the FMF group in terms of the presence of vascular risk factors and angiography-documented disease in comparison to controls. Cerebral distal artery involvement was significantly prevalent in FMF (78% vs 45%, P = .002). Especially, midbrain central deep perforating territory involvement was higher (30% vs 1%, P < .001). The long-term prognosis (median 8.5 years) under antiplatelet agents and colchicine is favorable. DISCUSSION: The acute stroke phenotype in FMF cases is herein described for the first time. Several clinicoradiological features such as thrombotic lacunar infarcts located in the central mesencephalon seem so typical that we recommend searching for FMF mutations in geographic regions where FMF is common.

Frequency and Types of Complications Encountered in Patients With Nonconvulsive Status Epilepticus in the Neurological ICU: Impact on Outcome.

Objectives. The frequency and types of complications in patients with nonconvulsive status epilepticus (NCSE) who are followed up in the intensive care unit (ICU), and the impact of these complications on outcome are not well-known. We investigated the complications and their effects on prognosis in NCSE patients. Methods. After reviewing the video-EEG monitoring (VEEGM) reports of all the consecutive patients who were followed up in our ICU between 2009 and 2019, we identified two groups of patients: 1-patients with NCSE (study group) and 2-patients who underwent VEEGM for possible NCSE but did not have ictal recordings (no-NCSE group). Electronic health records were reviewed to identify demographic and clinical data, duration of ICU care, medical and surgical complications, pharmacologic treatment, and outcome. These parameters were compared statistically between the groups. We also investigated the parameters affecting prognosis at discharge. Results. Thirty-two patients with NCSE comprised the study group. Infection developed in 84%. More than half were intubated, had tracheostomy or percutaneous endoscopic gastrostomy application. Refractory NCSE was associated with significantly more frequent complications and worse outcome. There was a higher tendency of infections in the study group (P = .059). Higher organ failure scores and prolonged stay in ICU predicted worse outcome (P < .05). Conclusion. The frequency of complications in patients with NCSE who are cared for in the ICU is considerable. Most of the complications are similar to the other patients in ICU, except for the higher frequency of infections. Increased physician awareness about modifiable parameters and timely interventions might help improve prognosis.

Spectrum of EEG Findings in Patients with Leptomeningeal Carcinomatosis and Seizures: Correlation with Neurodiagnostic Results and Outcome.

Purpose: Leptomeningeal carcinomatosis (LC) is a devastating condition in patients with systemic malignancies or primary brain tumors. Although much is known about neuro-radiologic investigations, there is very little information about EEG findings in these patients. Whether EEG is correlated with cranial magnetic resonance imaging (MRI) results and survival has not been investigated. Methods: Medical records of 2340 adult patients with the diagnosis of brain tumor, either metastatic (Group 1) or primary (Group 2), between 2000-2021 were reviewed for the presence of LC and seizures. Demographic and clinical features, laboratory results and Karnofsky performance scores of included patients were noted. Available routine EEG recordings were re-evaluated. Any possible correlation between EEG findings-MRI and EEG findings-survival were investigated statistically. Results: Sixty-six patients with LC and seizures were identified. The most common malignancies were lung cancer and glioblastoma multiforme. Twenty-six EEG recordings of 17 patients in Group 1, and 13 EEGs of 9 patients in Group 2 were available for final analysis. The most common EEG characteristic was background slowing (73%). The most frequent findings were rhythmic periodic patterns or spike wave activity (27%). Sporadic epileptiform discharges (8%) or ictal recordings (4%) were very rare. None of the EEG features correlated with MRI results or survival. Conclusion: There are various EEG patterns in patients with LC and seizures. The most common findings are related to background activity, with rhythmic periodic patterns or spike wave activity being observed less commonly. EEG characteristics do not predict MRI findings or survival.

Comparison of early- and late-phase CT angiography findings in brain death.

BACKGROUND: Late-phase images on computed tomography angiography (CTA), traditionally used for assessing cerebral circulatory arrest in brain death, suffer from suboptimal diagnostic yield due to stasis filling. Herein, we assessed contrast filling in individual intracranial arteries and veins in the early and late phases of CTA in patients with clinically confirmed brain death. METHODS: Contrast opacification within 28 arterial/venous segments was evaluated in both phases of CTA in 79 patients. This information was combined with reports in the literature to calculate prevalence of contrast filling in different intracranial vessels. Additionally, diagnostic sensitivity of 4-point, 7-point, and 10-point scores defined for brain death were compared among ratings based on early, late, and both phases (arteries rated on early, veins rated on late phase) of imaging. RESULTS: The median (IQR) number of vessel segments with contrast opacification was 0 (0-2) in early phase and 6 (0-10) in late phase. All segments showed increased prevalence of opacification when evaluated in late phase (p < 0.05). The M4 segments of MCA, internal cerebral veins, and vein of Galen had the lowest percentage of opacification in both phases. The sensitivity of 4-, 7-, and 10-point scoring algorithms increased from 59-91% to 94-99% when ratings were performed using early-phase images rather than based solely on late-phase images. CONCLUSIONS: The incorporation of early-phase images might be considered as a strategy to improve the sensitivity of CTA as an ancillary test in confirming brain death, especially in patients without missing or questionable elements in clinical examination.

Life After Tetra Hit: Anti-NMDAR Encephalitis After HSV Encephalitis in a NMOSD Coexistent with Sjögren's Syndrome.

Herpes simplex encephalitis (HSE) and anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis are associated entities. On the contrary, although both are autoimmune diseases, the association of neuromyelitis optica spectrum disorder (NMOSD) and anti-NMDAR encephalitis is not well explained. Herein, we present consecutively developed post-herpetic anti-NMDAR encephalitis in a patient with the coexistence of NMOSD and Sjogren syndrome. In our patient evaluation with MRI and clinical findings, the diagnosis of HSE after immunosuppressive drug application for NMOSD was made. Whereupon, HSE triggered NMDAR encephalitis. Many authors demonstrated the induction of anti-NMDAR encephalitis over herpes encephalitis with the presence of movement disorders, psychiatric manifestations, and cognitive dysfunction. In our patient, without biphasic disease activity; the persistence of symptoms, new MRI findings, and the positivity of anti-NMDAR antibody confirmed the anti-NMDAR encephalitis diagnosis. Our patient is a representative case mentioning the importance of close follow-up of a patient in neuroimmunology.

Isolated spinal cord granulomatous angiitis: a case report and review of the literature.

INTRODUCTION: Isolated spinal cord angiitis (ISCA) is very rare disease. But, it is frequently encountered in the differential diagnosis of atypical spinal cord syndromes. CASE PRESENTATION AND REVIEW OF THE LITERATURE: We present a 31-year-old male who presented with progressive paraparesis, and diagnosed with pathologically confirmed ISCA. Longitudinal cystic transverse myelitis was documented in spinal MRI. He responded well to cyclophosphamide and steroid combination, and no relapse was noted during the 4-year follow-up. A standard systematic analysis of the germane literature disclosed 15 more ISCA cases. In total 16 cases (mean age: 46.5, 10 males), ISCA was diagnosed with pathological evaluation in all (Biopsy in 11, Autopsy in 5). MRI lesion is characterized by usually multisegmental longitudinal and sometimes cystic expansile lesions. In seven cases, it was described as "(pseudo)tumoral" by the authors. Albeit absence of elevation of CSF protein/WBC or "compatible" spinal MRI lesion may aid to exclude ISCA to some extent, pathological confirmation is currently necessary for the diagnosis. In 11 cases, ISCA was treated similar to primary supratentorial vasculitis. Mortality rate is 31%. DISCUSSION: ISCA diagnosis, a typical example of which we have presented here, can only be established by tissue examination. However, noninvasive diagnostic criteria are critically needed. Our data suggest that this can only be possible with multinational multicenter prospective registry.

Factors associated with fingolimod rebound: A single center real-life experience.

Background It is still controversial whether the relapse experienced after discontinuation of fingolimod treatment is a rebound. Increasing cases of rebound have been reported in the literature. The rate of fingolimod rebound in patients after fingolimod cessation is reported between 5% and 52%. The present study aims to determine the rate of rebound after discontinuation of fingolimod treatment and the factors affecting the rebound. Methods This retrospective cohort study consists of adult MS patients who have been admitted to the Hacettepe University Hospital Neurology MS Center outpatient clinic between 2012 and 2020. Results During the study period, 642 patients received fingolimod and 23.1% discontinued the fingolimod treatment. Thirteen of 126 patients had a rebound (10.3%) after fingolimod discontinuation. The patients in the rebound group were significantly younger and washout period were significantly longer than those in the non-rebound group. After discontinuation of fingolimod treatment, the EDSS score of the rebound group was significantly higher than the non-rebound group, while Annualized Relapse Rates were similar. Conclusion Younger age, longer washout time, and previous treatment preferences may increase the occurrence probability of rebound. It is recommended that patients should be closely monitored after fingolimod discontinuation and appropriate disease-modifying therapy should be initiated as soon as possible.