Meningitis in familial mediterranean fever

Abstract

Familial Mediterranean fever (FMF) is an inherited autosomal recessive disorder characterized by recurrent, self-limited attacks of fever and serosal inflammation [1-4]. The disease is most commonly encountered in patients of Armenian, Jewish, and Arab origin, but has also been sporadically found in many other ethnic groups [4]. In addition to the characteristic febrile paroxysms of peritonitis, pleuritis, and arthritis, a number of less common manifestationsnamely, pericarditis, myalgia, bowel obstruction, renal disease, and a variety of cutaneous lesions--have been described. Meningeal involvement has not been widely recognized as a manifestation of FMF, although occasional reports of meningeal irritation, meningismus, and frank meningitis have appeared in the literature. The purpose of this report is to re'view the clinical and laboratory findings of the reported cases of meningitis allegedly associated with FMF and to describe the course of another patient extensively studied during several epsiodes. CASE REPORT The patient is a 32-year-old, non-Ashkenazic Jewish man, in whom meningitis first developed in Morocco at the age of one year. Details of his hospital admission at that time are not available, but a streptococcal infection was suspected as the cause. At the age of six years, he began to experience recurrent attacks of fever accompanied by either pleuritic chest pain or unrelenting abdominal pain. Each episode lasted 24 to 48 hours and was followed by periods of complete wellbeing, varying from a week to several months. At the age of 14 years, during an episode of fever and abdominal pain, a laparotomy was performed. Except for splenomegaly, no significant abnormalities were found. A normal appendix was removed. His paroxysms of fever and abdominal pain continued. Temperatures usually ranged from 38 to 39°C. The abdominal pain accompanied by anorexia, nausea, vomiting, cough, tenderness, and rigidity required bed rest for one to two days. He also had occasional episodes of fever and arthralgias or frank, acute arthritis involving the knees and ankles. At the age of 20 years, while in France, he began taking colchicine and observed a decrease in the frequency of episodes. However, he discontinued the use of this drug after two months, because of diarrhea. At the age of 24 years, the episodes of fever and peritonitis began to occur every seven to 10 days, and colchicine therapy along with a psyllium seed preparation was reinstituted. He was well for the next 12 months and then gradually decreased his compliance.'