Caracterización de una cohorte en el síndrome de Sjõgren primario: énfasis en el compromiso del sistema nervioso / Characterization of a cohort in the primary Sjõgren's syndrome: emphasis on the neurologic involvement

Con frecuencia evaluamos pacientes con el diagnóstico de síndrome de Sjõgren (SS), primario o secundario a diferentes patologías, especialmente artritis reumatoídea y lupus eritematoso sistémico, como también otras afecciones autoinmunes, que se manifiestan principalmente por síntomas de sicca. Sin embargo, en ocasiones, las manifestaciones extraglandulares del SS pueden dominar el cuadro clínico e incluso presentarse como su primera expresión. El compromiso del sistema nervioso central o periférico no es excepcional, alcanzando frecuencias de hasta 40 por ciento según las series publicadas, dependiendo de los distintos criterios de inclusión. por otro lado, el cuadro puede adquirir una gravedad con alto riesgo de secuelas e incluso de mortalidad si no es sospechado y reconocido tempranamente. La amplia y polimorfa forma de presentación del compromiso neurológico constituye un motivo para presentar una cohorte nacional prospectiva de 78 pacientes portadores de esta condición, con especial interés en la caracterización de la frecuencia y tipo de compromiso neurológico, que en esta serie se presentó en un 19,2 por ciento adquiriendo en general signos de severidad. Es una experiencia preliminar, que constituye un llamado de atención a la patología neurológica que puede acompañar al SS y que debe ser considerada en el diagnóstico diferencial con otra clase de patologías.

Frequently we evaluate patients with the diagnosis of Sjõgren's syndrome (SS), primary or secondary to various diseases, especially rheumatoid arthritis and systemic lupus erythematosus, as also other autoimmune conditions, manifested primarily by sicca symptoms. However, occasionally, extraglandular manifestations of the SS may dominate the clinical picture and even present as first expressed. The nervous central or peripheral system manifestations are not exceptional, reaching frequencies up to 40 percent according to the published series, depending on the different inclusion criteria. on the other hand, table can purchase a gravity high-risk consequences and even death if it is not suspected and recognized early. The vast and polymorphous form of presentation of the neurological manifestation constitutes grounds for presenting a prospective national cohort of 78 patients carriers of this condition, with special interest in the characterization of the frequency and type of neurological involvement, which in this series was presented in a 19.2 percent, gaining in general signs of severity. It's a preliminary experience, which is a call to attention to neurological pathologies that can accompany the SS and that and that it must be considered in the differential with another kind of pathology diagnosis.

Comparison of the Clinical Manifestations, Brain MRI and Prognosis between NeuroBehcet's Disease and Neuropsychiatric Lupus

BACKGROUND: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBehcet's disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients. METHODS: Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE. RESULTS: The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients. CONCLUSION: NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.

Henoch-schonlein Purpura Involving Cerebral Hemisphere

Henoch-Schonlein Purfrura is a generalized small vessel vasculitis of hypersensitivity type characterized primarily by a purpuric skin lesion, renal involvementwith hematuria and proteinuria, arthralgia, and colicky abdominal pain. A possible neurologic involvement was recognized in 1914 by Osler. A case of a 7 1/2-year-old male patient with Henoch-Schonlein purpura developed neurologic manifestations including seizure and mental status change. The cranial CT demonstrated a occipital hemorrhage of right side and cerebral edema with contrast enhancing scattered dilated vessels. The EEGchanges were slow wave activities in acute stage. The follow-up EEG shows that the slow wave activities were more improved than previous and that multiple spike waves were recorded at bothfronto-temporo-parietal areas with phase reversals. The vasculitis of HenochSch6nlein purpura can involve the nervous system.