Clinical Analysis Of 157 Cases With Crow-Fukase Syndrome

Objective To analyse the clinical characteristics and prognosis of Crow-Fukase syndrome in order to improve the level of diagnosis and treatments for this disease. Methods 157 cases with Crow-Fukase syndrome were retrospectively reviewed. Results The major clinical characteristics were peripheral polyneuropathy (99.4%), hyperpigmentation (93%), elevated cerebrospinal fluid protein (86.7%), peripheral edema (82.1%) , hepatomegaly and splenomegaly (72% and 65%) , menoxenia or menostasia (72.2%) and impotence (61.2%) . Peripheral polyneuropathy was the initial manifestation in 59.2% of the patients. The treatments of CFS included different combinations of corticosteroid, immunodepressants and chemotherapy. The general symptoms improved in 80 patients. 27 cases continued to deteriorate while 20 patients died. Therapeutic efficacy was unknown in 30 cases. Conclusions Clinical manifestations of Crow-Fukase syndrome are too complicated and easily misdiagnosed. Traditional therapeutic regimens are difficult to achieve complete remission. It is still a potential fatal disease.