Visual snow syndrome in patients with migraine: widening the clinical spectrum of the syndrome with permanent and episodic manifestations.

PURPOSE: Migraine is the comorbidity most frequently associated with visual snow syndrome (VSS), but the prevalence of VSS in patients with migraine (PWM) has not been studied. Our objective was to evaluate the frequency of VSS in PWM and to analyze if symptoms of VSS happened in a permanent or episodic manner (eVSS) in this population. METHODS: We conducted a multicenter observational cross-sectional study. PWM was recruited from headache units, and a survey about the presence of visual snow symptoms was administered. The frequency and characteristics of patients that met current VSS criteria were analyzed. Demographic and clinical features of patients with VSS, eVSS, and PWM with no visual snow were compared. RESULTS: A total of 217 PWM were included. Seventeen patients (7.8%) met the VSS criteria. VSS patients had visual aura more frequently (58.8% vs. 31%; p = 0.019) and a higher MIDAS score (96.6 vs. 47.7; p = 0.014). Fifty-eight PWM (26.7%) showed visual snow and associated features in an episodic way and were classified as eVSS. Patients with VSS showed a tendency towards a higher frequency of visual symptoms than patients with eVSS (p > 0.05). No statistically significant differences in sociodemographic characteristics and comorbid conditions were found between VSS and eVSS. CONCLUSION: The prevalence of VSS in PWM may be higher than that described for the general population. Some PWM may present similar visual symptoms to patients with VSS but in an episodic manner. Our study reinforces the observation that the clinical spectrum of visual snow is likely to be broader than previously described.

Altered ioflupane single-photon emission computed tomography in anti-IgLON5 disease: A new case mimicking probable progressive supranuclear palsy and review of the literature.

BACKGROUND AND PURPOSE: Anti-IgLON5 disease is a rare disorder characterized by a heterogeneous myriad of symptoms that may include sleep disorders, bulbar dysfunction, gait problems, movement disorders, cognitive impairment, oculomotor abnormalities, and nervous system hyperexcitability. Its physiopathology remains unknown, with a combination of both autoimmune and neurodegenerative findings. METHODS: We describe clinical, cerebrospinal fluid (CSF), and ioflupane single-photon emission computed tomography (SPECT) findings of a positive case of anti-IgLON5 disease mimicking probable progressive supranuclear palsy (PSP). We performed a literature review of previous publications reporting on anti-IgLON5 disease and ioflupane SPECT. RESULTS: We report the case of a 66-year-old male who met clinical criteria for probable PSP, in whom ioflupane SPECT showed an alteration of the left presynaptic dopaminergic pathway. However, the presence of atypical neurological symptoms for PSP led to further complementary tests, and IgLON5 antibodies were detected in CSF. According to our literature review, ioflupane SPECT findings have been previously described in only three other patients with anti-IgLON5 disease, with a reduced uptake in the striatum in two of them. CONCLUSIONS: Ioflupane SPECT abnormalities, though scarcely described, are not uncommon in anti-IgLON5 disease. They could be related to nigrostriatal dopaminergic degeneration in the context of the tauopathy component of the disease, but further case descriptions are necessary.

Acutely altered mental status as the main clinical presentation of multiple strokes in critically ill patients with COVID-19.

BACKGROUND AND AIMS: Cerebral infarction in COVID-19 patients might be associated with a hypercoagulable state related to a systemic inflammatory response. Its diagnosis might be challenging. We present two critically ill patients with COVID-19 who presented acutely altered mental status as the main manifestation of multiple strokes. METHODS: Clinical presentation and diagnostic work-up of the patients. RESULTS: Two patients in their sixties were hospitalized with a bilateral pneumonia COVID-19. They developed respiratory failure and were admitted to ICU for mechanical ventilation and intense medical treatment. They were started on low-molecular-weight heparin since admission. Their laboratory results showed lymphopenia and increased levels of C-reactive protein and D-dimer. Case 1 developed hypofibrinogenemia and presented several cutaneous lesions with biopsy features of thrombotic vasculopathy. Case 2 was performed a CT pulmonary angiogram at ICU showing a bilateral pulmonary embolism. When waking up, both patients were conscious but with a remarkable global altered mental status without focal neurological deficits. A brain MRI revealed multiple acute bilateral ischemic lesions with areas of hemorrhagic transformation in both patients (case 1: affecting the left frontal and temporal lobes and both occipital lobes; case 2: affecting both frontal and left occipital lobes). Cardioembolic source and acquired antiphospholipid syndrome were ruled out. COVID-19-associated coagulopathy was suspected as the possible main etiology of the strokes. CONCLUSION: Acutely altered mental status might be the main manifestation of multiple brain infarctions in critically ill COVID-19 patients. It should be specially considered in those with suspected COVID-19-associated coagulopathy. Full-dose anticoagulation and clinical-radiological monitoring might reduce their neurological consequences.

Revisión clínica y diagnóstica de la enfermedad de Kawasaki: estudio descriptivo, retrospectivo y analítico / Review of the diagnosis and clinical features of Kawasaki disease: retrospective descriptive and analytical study

Introducción: la enfermedad de Kawasaki es una vasculitis aguda, febril y autolimitada, que puede complicarse con alteraciones cardiovasculares. Su diagnóstico se basa en criterios clínicos. A pesar de un tratamiento eficaz, es la principal causa de cardiopatía adquirida en niños de países desarrollados. Objetivos: analizar la prevalencia de alteraciones coronarias en pacientes pediátricos diagnosticados en nuestro medio; evaluar las características demográficas, clínicas y analíticas de la población de estudio y describir los factores de riesgo de alteración coronaria en niños con enfermedad de Kawasaki. Métodos: se revisaron de forma retrospectiva las historias clínicas de los niños diagnosticados de enfermedad de Kawasaki desde enero de 1997 hasta diciembre de 2016 en el Hospital Clínico San Carlos (Madrid, España). Se consideró diagnóstico de enfermedad de Kawasaki la presencia de los criterios clínicos propuestos por la Academia Americana de Pediatría en 2017. Resultados: la enfermedad de Kawasaki se presentó fundamentalmente durante el invierno (n = 13; 56,5%) en mujeres (n = 12; 52,2%) de cinco años o menos (n = 18; 78,3%), que desarrollaron fiebre (n = 23; 100%) junto con cambios en la mucosa orofaríngea (n = 21; 91,3%) y presentaron proteína C reactiva y velocidad de sedimentación globular elevadas (n = 20; 86,95% y n = 13; 86,6%). Fueron tratados con inmunoglobulina intravenosa (n = 22; 95,7%) y ácido acetilsalicílico a dosis antiinflamatoria (n = 22; 95,7%) o antiagregante (n = 20; 87%). Tres pacientes (13%) desarrollaron alteraciones coronarias, que terminaron remitiendo. Conclusiones: en nuestra cohorte, la prevalencia de alteraciones coronarias fue similar a la reportada en nuestro medio y superior a la descrita en estudios internacionales. Los factores de riesgo de alteración coronaria son predominantemente niños con retraso en el diagnóstico y tratamiento, leucocitosis >12 000/ul, proteína C reactiva >3 mg/dl y trombocitosis

Introduction: Kawasaki disease is an acute, febrile and self-limiting vasculitis that may be complicated by cardiovascular changes. Its diagnosis is based on clinical criteria. Although effective treatment is available, it is the leading cause of acquired heart disease in children in developed countries. Objectives: to assess the prevalence of coronary artery abnormalities in paediatric patients diagnosed in our area, and the demographic, clinical and laboratory characteristics of the population under study, describing the risk factors for coronary involvement in children with Kawasaki disease. Methods: we performed a retrospective review of the medical records of children given a diagnosis of Kawasaki disease between January 1997 and December 2016 at the Hospital Clínico San Carlos (Madrid, Spain). We defined Kawasaki disease as the presence of the clinical criteria proposed for its diagnosis by the American Academy of Pediatrics in 2017. Results: cases of Kawasaki disease were most frequent in the winter (n = 13; 56.5%), in girls (n = 12; 52.2%) and in children aged 5 years or less (n = 18; 78.3%). Patients presented with fever (n = 23; 100%) and changes in the oropharyngeal mucosa (n = 21; 91.3%), with elevation of C-reactive protein and the erythrocyte sedimentation rate (n = 20; 86.95% and n = 13; 86.6%). Treatment consisted of intravenous immunoglobulin (n = 22; 95.7%) and acetylsalicylic acid at anti-inflammatory doses (n = 22; 95.7%) or antithrombotic doses (n = 20; 87%). Three patients (13%) developed coronary artery abnormalities that eventually resolved. Conclusions: in our cohort, the prevalence of coronary complications was similar to the prevalence reported for Spain and higher than the prevalence reported in international studies. The main risk factors for coronary artery abnormalities were delays in diagnosis or treatment, a white blood cell count greater than 12 000 cells/µl, a serum level of C-reactive protein greater than 3 mg/dl and thrombocytosis