[Subacute sclerosing panencephalitis: fulminant form]. / Panencefalitis esclerosante subaguda: forma fulminante.

INTRODUCTION: Subacute sclerosing panencephalitis (SSPE) has become less frequent as a consequence of massive anti measles vaccination. Early infection or immunological factors could increase the risk of its appearing and of evolved forms of the disease. CASE REPORTS: We describe the cases of four patients with fulminating forms of SSPE: a girl who had measles at the age of eight months; a male who, without actually suffering the clinical disease, came into contact with measles in the family and, despite having been vaccinated, presented SSPE 18 months after the contact; a boy aged 4 years and 4 months who had measles at the age of 6 months, and a boy of a similar age who had the illness when he was one year old. They all developed ataxia, with focal and generalised neurological signs, myoclonic and atonic seizures with rapid deterioration of language and the cognitive functions. In the four cases, the computerised tomography scans were normal, the electroencephalograms showed bilateral paroxysms and periods of recurrent bioelectrical attenuation. The magnetic resonance images of the four patients revealed disseminated hyperintense lesions, and one of the patients presented hyperintense lesions in the cervical spinal cord. The anti measles IgG titres were high in the cerebrospinal fluid. Anti convulsive drugs were ineffective. In the third and fourth patients, intrathecal intraventricular treatment with interferon did not modify the course of the disease and neurological deterioration was seen in the subsequent follow up of all the cases. CONCLUSION: As a consequence of vaccination against measles, SSPE has become less frequent. Infection of infants, prior to the immunisation stage, can induce SSPE with periods of latency that are shorter than usual and with a fast progression of the disease.

[Behçet's disease in children: cortical calcifications]. / Enfermedad de Behçet en la infancia: calcificaciones corticoencefálicas.

INTRODUCTION: Between 5 and 48% of the patients with Behçet s disease have neurological abnormalities. In children, meningoencephalitis as an initial manifestation of the disease is not frequent. CASE REPORT: We present a four years old girl with a Behçet s disease. When she was six months of age, during an hospitalization caused by malnutrition and respiratory infection, she had developed an aseptic meningoencephalitis and laboratory test showed hypergammaglobulinemia. When she was 21 months old, she had seizures as epilepticus status, and good response was achieve treating with anticonvulsive drugs. She did not repeat episodes of epileptic seizures, but the patient showed language and neuromotor problems. CT was abnormal with asymmetric ventricular dilatation and increased subarachnoides cortical sulci with multiple calcifications in those areas. She also suffered respiratory and gastrointestinal infections that required hospitalization. At the age of 3 years and 6 months, she had occular affection with uveitis, oral and genital ulceration. The clinical history of the girl consisting of meningoencephalitis, gastrointestinal and respiratory disorders, later presentation of uveitis and oral and genital ulcers confirmed the Behçet s disease diagnosis. CONCLUSION: We present a girl with multisystemic disorders characteristic of the Behçet s disease. Initially she had an aseptic meningoencephalitis developing cortical calcifications.

Differential modulation of vascular cell integrin and extracellular matrix expression in vitro by TGF-beta 1 correlates with reciprocal effects on cell migration.

In vitro, BAEC and BASMC migratory phenotypes are known to be reciprocally modulated by both soluble factors and extracellular matrix proteins. In addition, integrin matrix receptors mediate endothelial and smooth muscle cell attachment and migration. To further elucidate these phenomena, we studied the effects of TGF-beta 1 on integrin expression by vascular BASMC and BAEC in tissue culture. TGF-beta 1 upregulated mRNA levels and surface pools of BASMC beta 3 integrin classes without modulating beta 1 integrin mRNA levels or expression of beta 1 integrin organization. In contrast to its effects on BASMC, TGF-beta 1 increased BAEC mRNA levels and surface expression of beta 1 and beta 3 integrins without altering their organization. Conversely, extracellular matrix components (fibronectin, laminin, and fibrinogen) organized cell surface integrins in both BASMC and BAEC without affecting the size of their cell surface pools. These data are consistent with the hypothesis that SMC and EC behavior in neointimal lesions may be modulated, in part, through a coordination of soluble factor and extracellular matrix protein regulation of integrin surface expression and organization.