RESUMO
INTRODUCTION: As an immune-mediated disease of the central nervous system, multifaceted aspects of a humoral immune response are widely described during multiple sclerosis (MS). However, the prevalence of different auto-antibodies, such as antinuclear antibodies (ANA), during MS is very variable and their clinical relevance remains controversial. Our aim was to evaluate the prevalence and clinical correlations of ANA positivity in South Tunisian MS patients. MATERIAL AND METHODS: We performed ANA screening using indirect immunofluorescence (IIF) on HEp-2 cells (Biosystems®) in 82 MS patients. For ANA positive samples (titer ≥1/160), anti-ds-DNA detection (IIF on Crithidia luciliae (Biosystems®)) and extractable nuclear antigen typing (immunodot (Euroimmun®)) were performed. RESULTS: ANA were positive in 35/82 MS patients (42.7%). The titer was ≥1/320 in 16/35 patients. The antigenic specificity of ANA was identified in 7/35 patients. None of the patients had extra-neurological manifestations. No correlation was found between ANA and age, gender, MS course, disease duration, disability, annual relapse rate nor IgG index. ANA positivity was more frequent in patients with IgG oligoclonal bands (OCB) (47.1%) than in patients without IgG OCB (16,6%) (p=0.049). Regarding disease activity, ANA positivity was significantly more frequent in patients with relapse (52.6%) than in patients in remission (25.9%) (p=0.031). CONCLUSION: Our results showed that ANA positivity in MS disease is not rare. This positivity was not associated with clinical expression of any connective tissue disease. ANA occurrence in MS was associated with IgG OCB+ profile and relapsing status, probably reflecting an ongoing immune dysregulation.
Assuntos
Esclerose Múltipla , Bandas Oligoclonais , Anticorpos Antinucleares , Antígenos Nucleares , DNA , Epitopos , Humanos , Imunoglobulina G , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/epidemiologia , RecidivaRESUMO
Megalencephalic leukoencephalopathy with subcortical cysts or Van der Knapp disease is a rare entity that has recently been identified. It is characterized by the presence of macrocephaly, epilepsy and a slowly progressive spastic cerebellar syndrome. The culprit MLC1 gene is located on chromosome 22. MRI provides valuable data for diagnosis characterized by diffuse white matter lesions with subcortical cysts. We report four cases of megalencephalic leukoencephalopathy with subcortical cysts from two different families.
Assuntos
Gânglios da Base/patologia , Encefalopatias/patologia , Cerebelo/patologia , Cistos/patologia , Atrofia , Criança , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
Moyamoya syndrome has rarely been reported in association with Down syndrome. We report on 2 cases in 3-year-old and 6-year-old female children with Down syndrome, who presented with neurological deficit. Imaging (magnetic-resonance angiography and digital-subtraction angiography) revealed the classical Moyamoya pattern. The neurological deficits persisted in both cases. One patient has developed epilepsy.
Assuntos
Síndrome de Down/complicações , Doença de Moyamoya/complicações , Angiografia Digital , Angiografia Cerebral , Criança , Pré-Escolar , Feminino , Humanos , Angiografia por Ressonância Magnética , Doença de Moyamoya/diagnóstico , Doença de Moyamoya/diagnóstico por imagemAssuntos
Imunocompetência , Leishmaniose Visceral/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/etiologia , Adulto , Humanos , Imunocompetência/fisiologia , Leishmaniose Visceral/imunologia , Linfo-Histiocitose Hemofagocítica/imunologia , Masculino , Pessoa de Meia-IdadeRESUMO
We report a case of Candida glabrata perinephric abscess in a patient with diabetes mellitus who recently underwent ureteropelvic surgery for lithiasic urinary tract obstruction. Surgical drainage and amphotericin B treatment led to resolution of the infection. C. glabrata urinary infection has become more prevalent over the last decade in immunocompromised patients. Drainage is indicated for development of a fungal abscess in the perinephric area. Most authors recommend administration of an antifungal adjuvant treatment.