Estudo das complicações neurológicas em hemofílicos da região norte do estado do Ceará

Abstract

Patients with hemophilia may suffer from several types of central and peripheral nervous system diseases. Intracranial bleeding is a leading cause of death, but data about the prevalence of those conditions is limited. We prospectively evaluated, from January 2011 to December 2018, the prevalence of central and peripheral nervous system disorders in all patients with hemophilia seen at the Hemocentro Regional Norte, Brazil. Seventy-five hemophilia A patients (no type B patient was found) were prospectively evaluated (prevalence of 4.61 hemophiliacs/100.000 inhabitants in this area). 13.3% (N=10) had either central (N=5) or peripheral nervous system disorders (N=5) secondary to bleeding. Patients with CNS disease had their neurological event at a significantly earlier age than patients with PNS disorders: 12.2±6.6 versus 32.5±5 (P<0.05). Three patients had subdural hematomas, one intracerebral and one subarachnoid hemorrhage. Overall, the outcome of those conditions was good, although one patient underwent intracerebral hematoma drainage (patient #3) and 3 were left with epilepsy or behavior disorders. Prophylactic factor VIII therapy replacement prevented all new cases of CNS disease, and decreased the prevalence of PNS dysfunction. The most commonly affected peripheral nerve was the femoral (N=3). One patient had a right sciatic neuropathy and one tardive right ulnar neuropathy related to right elbow hemophilic arthropathy. Most of the patients had predominant residual sensory involvement, although 2 had more significant motor involvement. In summary, to our knowledge, this is the first prospective description of clinically relevant central and peripheral nervous system disorders in hemophiliac patients prior and after the prophylactic factor VIII replacement era.