Síndrome del ocho y medio secundario a vasculopatía lúpica: presentación de un caso / Eight-and-a-half syndrome secondary to lupus vasculopathy: a case presentation

RESUMEN Introducción: El manejo diagnóstico y terapéutico en los pacientes con lupus eritematoso sistémico que desarrollan una afectación neuropsiquiátrica representa un reto, debido a la heterogeneidad de las formas en que puede presentarse y la ausencia de criterios diagnósticos. Objetivo: Reconocer las formas clínicas de presentación de los síndromes neuroftalmológicos que traducen afectación pontina. Presentación del caso: Hombre de 71 años con antecedente de lupus eritematoso sistémico con afectación neuopsiquiátrica, que de forma aguda desarrolla un cuadro emético en el curso de una emergencia hipertensiva seguido de una parálisis de la mirada horizontal hacia la izquierda, una oftalmoplejía internuclear posterior derecha y una parálisis facial izquierda. En la neuroimagen se constata una afectación multifocal con marcado daño pontino. Conclusiones: Reconocer las formas clínicas de presentación de estos trastornos neuroftalmológicos raros que generalmente se presentan de forma aguda/subaguda permite al neurólogo realizar el diagnóstico topográfico de la lesión a nivel protuberancial con elevada precisión desde la Sala de Urgencias, así como reducir los posibles diagnósticos diferenciales a una etiología vascular, desmielinizante u ocupativa de espacio(AU)

ABSTRACT Introduction: The diagnostic and therapeutic management of patients with systemic lupus erythematosus who develop a neuropsychiatric involvement represents one of the biggest challenges due to the heterogeneity of the ways in which it can occur and the absence of diagnostic criteria. Objective: To recognize the clinical forms of presentation of neurophthalmological syndromes that express pontine involvement. Case presentation: Seventy-one-year-old man with history of systemic lupus erythematosus with neuropsychiatric involvement who acutely develops an emetic episode in the course of a hypertensive emergency followed by a paralysis of the horizontal gaze to the left, a right-sided posterior internuclear ophthalmoplegia and a left facial palsy. In the neuroimaging, a multifocal involvement with marked pontine damage is observed. Conclusions: Recognizing the clinical forms of presentation of these rare neurophthalmological disorders that generally occur in an acute or subacute form allows the neurologist to perform the topographic diagnosis of the lesion at a protuberancial level with high precision from the time when the patient attends the Emergency Department and reduces the possible differential diagnoses to a vascular, demyelinating or occupational etiology of space(AU)

Vascular endothelial growth factor G1612A (rs10434) gene polymorphism and neuropsychiatric manifestations in systemic lupus erythematosus patients / Polimorfismo genético do fator de crescimento vascular endotelial G1612A (rs10434) e manifestações neuropsiquiátricas em pacientes com lúpus eritematoso sistêmico

Abstract Aim: To investigate the relation between vascular endothelial growth factor (VEGF) gene polymorphism in systemic lupus erythematosus (SLE) patients and lupus related neuropsychiatric manifestations. Patients and methods: Sixty adult SLE patients recruited from the Rheumatology and Neurology departments of Cairo University hospitals were classified into two groups; Group A: 30 patients with neuropsychiatric manifestations (NPSLE) and Group B: 30 patients without. For both groups the SNP G1612A (rs10434) of the VEGF gene was genotyped by real time polymerase chain reaction (RT-PCR). Results: Statistically significant difference was found in genotype and allele frequencies between both groups (AA [70% vs 13.3%, p < 0.001] and GG [10% vs 66.7%, p < 0.001]). Conclusion: Polymorphism in the gene coding for VEGF may be associated with increased incidence of neuropsychiatric lupus in SLE patients.

Resumo Objetivo: Investigar a relação entre o polimorfismo genético do fator de crescimento vascular endotelial (VEGF) em pacientes com lúpus eritematoso sistêmico (LES) e manifestações neuropsiquiátricas relacionadas com o lúpus. Pacientes e métodos: Foram recrutados 60 pacientes adultos com LES nos departamentos de Reumatologia e Neurologia de hospitais universitários do Cairo e classificados em dois grupos; grupo A: 30 pacientes com manifestações neuropsiquiátricas (LESNP) e grupo B: 30 pacientes sem manifestações neuropsiquiátricas. Genotipou-se o SNP G1612A (rs10434) do gene VEGF em ambos os grupos por reação em cadeia da polimerase em tempo real (RT-PCR). Resultados: Foi encontrada diferença estatisticamente significativa nas frequências genotípicas e alélicas entre os dois grupos (AA [70% vs. 13,3%, p < 0,001] e GG [10% vs. 66,7%, p < 0,001]). Conclusão: O polimorfismo no gene que codifica o VEGF pode estar associado ao aumento na incidência de lúpus neuropsiquiátrico em pacientes com LES.

Cerebral metabolic disturbance as detected with magnetic resonance spectroscopy in neuropsychiatric systemic lupus erythematosus

To evaluate the spectral brain changes using 1H-MR spectroscopy in Neuropsychiatric Systemic Lupus Erythematosus [NPSLE] patients: and, to correlate their neuropsychiatric clinical data with MRI and 1 PI-MRS abnormalities. Twenty three NPSLE patients and 8 age and sex matched control volunteers were included. Disease activity was measured by SLE Disease Activity Index [SEEDAI]. Patients were classified into two groups; the first included NPSEE patients with normal MRI, and the second included those with abnormal MRI. Single voxel proton MRS was evaluated in the parieto-occipital white matter in all patients and volunteers and in areas of MRI abnormal lesions. The metabolic peaks were fitted at known frequencies where N-acetylaspartate [NAA] at 2.02 ppm, creatine [Cr] at 3.02 ppm, and choline [Cho] at 3.22 ppm. The relative ratios of NAA to Cr and of Cho to Cr we measured. the most common neuropsychiatric features were headache and cognitive impairment with 39.1% for each of them, 9 patients had abnormal MRI imaging, while spectral changes were present in 20 patients. MRS changes were in agreement and statistically significant [P=0.001] with the neuropsychiatric manifestations, compared with MRI changes [P=0.8]. Neuropsychological tests were significantly affected in patients with abnormal MRI versus those with normal ones. In patients with abnormal MRI, the NAA/Cr ratio was significantly lower than those with normal MRL while the Cho/Cr ratio was significantly higher. In patients with NPSLE, 1H-MR spectroscopic findings seem to reflect the cerebral metabolic disturbances as functional abnormalities, related to the neuropsychiatric symptoms. It is a complementary tool to MRI in diagnosing the CNS involvement

Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system

OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7 percent): seizures (17 / 36.2 percent), intractable headache (7 / 14.9 percent), mood disorders (5 / 10.6 percent), cerebrovascular disease (4 / 8.5 percent), acute confusional state (3 / 6.4 percent), aseptic meningitis (3 / 6.4 percent), psychosis (3 / 6.4 percent), chorea (3 / 6.4 percent), Guillain-Barré syndrome (2 / 4.3 percent) and cranial neuropathy (1 / 2.1 percent). Morbidity indexes (SLEDAI and SLICC) were higher among patients with neuropsychiatric manifestations (p<0.05). CONCLUSION: Neuropsychiatric syndromes are frequent, and add significant morbidity to juvenile systemic lupus erythematosus.

OBJETIVO: Caracterizar o comprometimento neurológico no lupus eritematoso sistêmico juvenil. MÉTODO: Os prontuários dos pacientes com o diagnóstico de lupus eritematoso sistêmico antes dos 16 anos de idade, em acompanhamento na Unidade de Reumatologia do Hospital Pequeno Príncipe, de janeiro de 1992 a janeiro de 2006, foram revisados retrospectivamente enfatizando aspectos neuropsiquiátricos. RESULTADOS: Quarenta e sete pacientes foram incluídos. Síndromes neuropsiquiátricas foram encontradas em 29 (61,7 por cento): crises convulsivas (17 / 36,2 por cento), cefaléia intratável (7 / 14,9 por cento), distúrbios do humor (5 / 10,6 por cento), doença cerebrovascular (4 / 8,5 por cento), estado confusional agudo (3 / 6,4 por cento), meningite asséptica (3 / 6,4 por cento), psicose (3 / 6,4 por cento), coréia (3 / 6,4 por cento), síndrome de Guillain-Barré (2 / 4,3 por cento) e neuropatia craniana (1 / 2,1 por cento). índices de morbidade (SEDAI e SLICC) foram maiores em pacientes com manifestações neuropsiquiátricas (p<0,05). CONCLUSÃO: Síndromes neuropsiquiátricas são um achado freqüente que acrescenta morbidade significativa ao lupus eritematoso sistêmico juvenil.

Vasculite mesentérica como única manifestação de atividade lúpica: a importância de um diagnóstico precoce / Mesenteric vasculitis as the only manifestation of lupic activity: the importance of a premature diagnosis

A vasculite mesentérica é uma grave complicação do lúpus eritematoso sistêmico (LES), pois é considerada uma importante causa de morbidade e mortalidade nesta doença. Na verdade, ela é também uma preocupação diagnóstica e terapêutica, e seu reconhecimento e intervenção precoce afetam diretamente o prognóstico. Este relato descreve uma paciente de 28 anos com diagnóstico de lúpus eritematoso sistêmico, que subitamente apresentou forte dor abdominal na ausência de outras manifestações de atividade de doença. Os procedimentos diagnósticos são discutidos e revisados. Os autores também enfatizam a necessidade de estabalecer um diagnóstico precoce dessa entidade para evitar complicações e uma pior evolução.

Neuropsychiatric manifestations in Thai patients with systemic lupus erythematosus.

Neuropsychiatric (NP) manifestations in patients with systemic lupus erythematosus (SLE) [NPSLE] and prognostic factors were studied in 91 patients. There were 98 NP episodes, of which 78 (79.6%) occurred within the first year of the disease. Twenty-six patients (6.7%) had NPSLE as an initial presentation of the disease. There were seizures in 53 episodes (54.1%), psychosis in 13 (13.3%), acute confusion state in 11 (11.2%), abnormal consciousness in 6 (6.1%), transverse myelitis in 6 (6.1%), peripheral neuropathy in 5 (5.1%), cerebral infarction in 2 (2.0%) and aseptic meningitis in 2 (2.0%). Most forms of NPSLE responded well to high dose corticosteroids. Anti-convulsant therapy could be discontinued within a median duration of 3 months after the SLE activity was under control, and without significant recurrence of seizures. The 5-year and 10-year survival rates of patients with NPSLE were 75.9% and 50.6%, respectively. Patients with NPSLE had significantly more cutaneous vasculitis and less arthritis than those without.