Thymoma and myasthenia gravis - an observational study at a tertiary center.

INTRODUCTION: Acquired Myasthenia Gravis (MG) is a rare autoimmune neurological disorder characterized by fluctu- ating paresis of the skeletal muscle due to pathogenic antibodies against the acetylcholine receptor or other elements of the neuromuscular plaque. There is a close relation between MG and thymoma. We aimed to characterize a population of patients with Myasthenia Gravis associated thymoma (MGAT). METHODS: Retrospective and longitudinal study in all patients with MGAT observed at a tertiary center between 2009 and 2020. We assessed epidemiological, clinical, laboratory and therapeutic features of both MG and thymoma. RESULTS: We found 18 patients with an average age of 53 ± 16.2, 13 of them females. Most patients (n=15) presented the generalized MG form. Most frequent Masaoka staging was II (n=7). Regarding the WHO histopathological classification of thymoma, most patients (n=11) presented with type B2 or B3. Thirteen patients underwent extended thymectomy (12 by median sternotomy and 1 by VATS). Of the remaining 5 patients, 4 of them underwent a CT scan guided biopsy, and 1 patient did not accept further work-up. Seven patients were classified as R0 for surgical resection margins and only one of them had recurrence of thymoma. Besides surgery, oncological treatment included radiotherapy and chemotherapy. Five patients expe- rienced a myasthenic crisis during the course of the disease. Three deaths occurred in the studied population. CONCLUSIONS: This study helped to pinpoint important aspects concerning therapeutic orientation of MGAT patients, such as the clinical impact of thymectomy in the course of MGAT, the oncological prognostic value of surgical resection mar- gins, and the importance of preoperative intravenous immunoglobulin. Management of MGAT patients is only possible with a multidisciplinary approach.

Cytomegalovirus optic neuropathy in a young immunocompetent patient.

Cytomegalovirus (CMV) infection is common worldwide, but is usually a subclinical or self-limited infection in immunocompetent patients. On the contrary, most of the ocular and central nervous system involvement occurs in immunosuppressed patient, and usually has severe consequences. Ocular manifestations of CMV infection are frequent in immunosuppressed patients (notably keratouveitis, retinitis and retinal branch angiitis), but a few cases of optic neuropathy (mostly papillitis) have been reported in the literature in immunocompetent patients. We report a case of a young and previously healthy female patient who developed a CMV optic neuropathy after a presumed CMV reinfection. Viral copies were detected in both blood and cerebrospinal fluid, as well as a high IgG titre and no evidence of immunosuppression was found. Clinical improvement was seen after high-dose corticosteroids and ganciclovir.

Neurologists' Diagnostic Accuracy and Interspecialties' Diagnostic Concordance of Acute Vertigo: Observational Study at the Emergency Department in a Tertiary Center.

BACKGROUND: Acute vertigo (AV) is often a challenging condition. Because of its multiple causes, patients are frequently observed by neurologists and physicians from other areas of specialites, particularly Ear, Nose, and Throat (ENT). We aimed to assess the diagnostic accuracy of AV in patients observed by Neurology and other medical specialties. MATERIALS AND METHODS: Retrospective cross-sectional study with the selection of all patients with AV observed by Neurology at the Emergency Department (ED) of a tertiary center in 2019, regarding demographic data, imaging studies, diagnosis by Neurology and ENT at the ED, and diagnosis after ED discharge by different medical specialties. RESULTS: In all, 54 patients were selected, 28 (52%) of them were women. The mean age was 59.96±14.88 years; 48% had a history of AV and 89% underwent imaging studies (computed tomography scan and/or magnetic resonance imaging scan). The most frequent diagnosis established by Neurology was benign paroxysmal positional vertigo, followed by vestibular neuronitis; 28 patients were also observed by ENT with an overall concordance rate of diagnosis of 39%. After ED discharge, most patients were observed at the Balance Disorders Outpatient Clinic. Diagnosis by Neurology at the ED was not significantly different from observation by other medical specialties after ED discharge regarding the distinction between peripheral and central causes of AV (κ=0.840, 95% confidence interval: 0.740 to 0.941, P<0.005). CONCLUSIONS: Neurologists can effectively differentiate central and peripheral causes of AV at the ED. Patients with AV should be primarily evaluated by Neurology at the ED, avoiding redundant observations and allowing faster patient management.

Prognostic significance of specific EEG patterns after cardiac arrest in a Lisbon Cohort.

OBJECTIVE: To evaluate if EEG patterns considered highly malignant are reliable predictors not only of poor neurological outcome but also reliable predictors of death. METHODS: Retrospectively, EEGs from Cardiac Arrest (CA) patients of two teaching hospitals in Lisbon were classified into 3 groups: highly malignant, malignant, and benign groups. Outcome was assessed at 6 months after CA by CPC (Cerebral Performance Categories) scale. We evaluated the accuracy of these patterns to predict poor neurological outcome and death. RESULTS: We included 106 patients for analysis. All patients with a highly malignant EEG (n = 37) presented a poor neurological outcome. Those patterns were also associated with death. Malignant EEG patterns were not associated with poor neurological outcome. Benign EEG patterns were associated with good neurological recovery (p < 0.0001). CONCLUSION: Highly malignant EEG patterns were strongly associated with poor neurological outcome and can be considered to be predictors of death. SIGNIFICANCE: This study increased the knowledge about the value of EEG as a tool in outcome prediction of patients after cardiac arrest.

Safety and Effectiveness of Fingolimod in Real-World Multiple Sclerosis Portuguese Patients.

OBJECTIVES: The aim of this study was to evaluate postmarketing fingolimod safety and effectiveness in a real-world clinical population. METHODS: This was a retrospective, single-center study with active multiple sclerosis patients treated with fingolimod with at least 12 months of follow-up. Demographic and clinical and imaging characteristics, including annualized relapse rate (ARR), Expanded Disability Status Score, previous treatment, adverse events, treatment duration, and reason for discontinuation, were analyzed. RESULTS: Sixty-three patients were included; 61.9% were females. Mean age and mean disease duration were 30.9 ± 9.3 years and 11.4 ± 6.9 years, respectively. Fifty-one patients received prior first-line disease-modifying therapies, 11 patients were previously treated with natalizumab, and 1 was treatment naive. The ARR decreased by 75.3% for the total population at the end of the first year of treatment (P < 0.0001). The proportion of relapse-free patients improved significantly. All patients previously treated with natalizumab switched because of safety concerns, although the ARR kept low after treatment initiation. Only 3 patients (4.8%) discontinued treatment because of adverse drug reactions, and 2 (3.2%) because of lack of effectiveness. CONCLUSIONS: In this real-world audit, fingolimod appeared to be effective after first-line treatment failure in reducing disease activity and progression of disability throughout the observational period and may be an effective option after natalizumab. Fingolimod was well tolerated with low rates of discontinuation and adverse events.

Parvovirus B19 infection associated with hemolytic anemia and cranial polyneuropathy.

Parvovirus B19 (PB19) is a common, widespread, small, single-stranded DNA virus which has been linked with a broad spectrum of clinical illnesses, including a variety of neurological complications such as encephalitis, meningitis, myelitis, stroke, cerebellar ataxia, and neuropathy. The authors describe a case of PB19 infection associated with hemolytic anemia and cranial polyneuropathy involving the second and third cranial nerves in a 23-year-old immunocompetent woman. The diagnosis of acute PB19 infection was established with detection of positive DNA and anti-PB19 IgM antibodies in blood samples. Antiganglioside antibody studies were performed and serum anti-GD1b IgG was strongly positive. Further investigation was normal or negative, excluding other infectious or autoimmune disorders. The patient was initially treated with a 5-day course of intravenous immunoglobulin (IGIV). Because of incomplete neurological recovery, methylprednisolone was also administered 7 days after IGIV therapy initiation. Complete resolution of clinical symptoms was observed 3 months after disease onset at follow-up visit, despite the persistence of PB19 DNA and anti-PB19 IgM antibodies in serum 5 months after the initial presentation. Our report provides evidence that PB19 could affect both the central and peripheral nervous system, possibly by triggering an autoimmune mechanism that leads to autoantibody production.

[Prevalence of carotid artery disease in an ischemic stroke population: role of Doppler ultrasonography]. / Prevalência de doença carotídea na patologia cérebro-vascular isquémica: o papel do eco-Doppler.

BACKGROUND: Atherosclerotic carotid disease represents approximately 20% of the causes of ischemic stroke. Effective treatment options, such as endovascular or surgical revascularization procedures, are available. Doppler Ultrasound (DUS) is a non-invasive, inexpensive, routine exam used to evaluate the presence of internal carotid artery (ICA) stenosis. We retrospectively analysed the prevalence of severe atherosclerotic carotid disease in a population of patients with acute ischemic stroke/transitory ischemic attacks (TIAs), and the role of DUS in the detection of ICA stenosis and treatment decisions in these patients. METHODS: A total of 318 patients with ischemic stroke or TIAs was admitted to our stroke unit, and 260 patients were studied by DUS. ICA stenosis was evaluated by DUS according to peak systolic velocity. All DUS exams were performed by the same operator. ICA stenosis was further assessed in 43 patients by digital subtraction angiography (DSA) using NASCET criteria. RESULTS: Of the total 318 patients, 260 (82%) had DUS evaluation. Of the total 520 ICAs studied by DUS, degrees of ICA stenosis were: 0-29% n= 438 (84%); 30-49% n= 8 (2%); 50-69% n= 27 (5%); 70-89% n= 15 (3%); 90-99% n= 20 (4%); oclusão n= 14 (2%). Of the total 260 patients studied, 43 (16.5%) underwent DSA. Sensibility and specificity of DUS in the diagnosis of carotid stenosis over 70% were, respectively, 91% e 84%. Of the total 31 patients with significant carotid stenosis (70-99%), 23 (74%) underwent subsequent carotid revascularization procedures. DISCUSSION: DUS is an important screening test in our stroke unit, justifying its use as a routine exam for all patients with ischemic stroke/TIAs. Moreover, our results show the relevance of severe carotid disease in a population with acute ischemic stroke/TIAs (16.5%), with a total of 9% of patients being submitted to carotid revascularization procedures.