Síndrome de Miescher-Melkersson-Rosenthal: Presentación de tres casos y revisión de la literatura / Miescher-Melkersson-Rosenthal Syndrome: Three cases reports and literature review

RESUMEN El síndrome de Miescher-Melkerson-Rosenthal es una condición clínica poco frecuente caracterizada por un edema orofacial episódico, paresia facial periférica recurrente, lengua geográfica (lingua plicata) y granulomas en biopsia de piel o labial. Se ha reportado más frecuentemente en mujeres, y su incidencia es probablemente subestimada por el desconocimiento del síndrome, su presentación clínica incompleta y la plétora de diagnósticos diferenciales. Se debe considerar en pacientes que presenten al menos dos de las cuatro características mencionadas. Su tratamiento, con esteroides vía oral, está dirigido a los episodios de edema orofacial y/o paresia facial. A continuación se describen tres casos del síndrome valorados en el servicio de neurología de la Fundación Cardioinfantil y se presenta una breve revisión de la literatura.

SUMMARY Miescher-Melkerson-Rosenthal syndrome is an uncommon clinical condition characterized by episodic orofacial oedema, recurrent peripheral facial paralysis, geographic tongue (lingua plicata) and granulomas in skin or labial biopsy. It is reported more frequently in women, and its incidence is probably underestimated due to lack of knowledge of the syndrome, incomplete clinical presentation and the plethora of differential diagnosis. It should be considered in patients with at least two of the four mentioned characteristics. Its treatment, with oral steroids, is directed at the episodes of orofacial oedema and/or the facial paralysis. We described three cases of the syndrome seen at the Fundacion Cardioinfantil's neurology service and present a short review of the literature.

Fatal reversible cerebral vasoconstriction syndrome: A systematic review of case series and case reports.

OBJECTIVE: To describe patient characteristics, radiological findings and the clinical course of adults with fatal reversible cerebral vasoconstriction syndrome (RCVS). METHODS: A systematic literature search from January 1, 2000, until December 31, 2018, was performed using PubMed, EMBASE, Scopus, Cochrane reviews, LILACS and Scielo. Studies reporting RCVS in adult patients with fatal outcomes were included. RESULTS: 430 studies were initially identified, 179 full-text articles were reviewed, and 9 publications describing 12 subjects were included. The vast majority of the reports were from the U.S. Most of the female cases occurred during postpartum. All patients had a headache on initial presentation, although only 42% had thunderclap headache. A CT scan was performed on 67% of the patients. Imaging results were diverse, with a tendency toward cerebral hemorrhage followed by mixed cases. The main course of treatment included steroids (58% of the patients), with only 42% receiving nimodipine. The time to death ranged from 4 to 14 days, with a median of 9.2 days (SD ±â€¯3.2). CONCLUSION: We found that the majority of fatal cases reported in the literature are most likely related to postpartum angiopathy. We established a tendency in the onset of brain hemorrhage and the combination of infarction and brain hemorrhage. We described various markers for poor prognosis, including focal signs, the presence of hemorrhage and infarct in the first diagnostic image obtained and the need for invasive interventions. The majority of fatal cases in our report occurred in women, with over half of those cases during the puerperium period.

Pylephlebitis with splenic abscess following transrectal prostate biopsy: rare complications of intra-abdominal infection.

Pylephlebitis is a rare complication of intra-abdominal infection involving septic thrombosis of the portal system. If the splenic vein is compromised, this can lead to a splenic abscess, an extremely rare complication of pylephlebitis. The pathophysiology behind these clinical entities remains unclear. In both cases, symptoms are highly nonspecific and include fever, malaise and abdominal pain. Here, we discuss a case in which a patient develops both pylephlebitis and a subsequent splenic abscess following a transrectal prostate biopsy. Diagnosis was made by computerized tomography scan; the treatment included broad spectrum antibiotics and laparoscopic splenectomy, after which the patient made a full recovery.