Neurological complications of sickle cell anemia.

A variety of neurological complications occur with sickle cell hemoglobinopathy. The most striking and most common is cerebral infarction. It is also the most devastating. Because of the high recurrence rate in untreated patients, cerebral infarction is the most debilitating neurological complication. The observed frequency varies from 6% to as high as 34% in different reports. The pathogenesis, clinical features, and demography are discussed. Diagnostic procedures should include a spinal tap to rule out potentially treatable lesions. CT scan and cerebral angiography may also be helpful in certain cases. Transfusion therapy remains the mainstay of management in the acute phase of cerebral infarction. There is considerable evidence to indicate that long-term transfusion programs are effective in the prevention of recurrences. Intracranial hemorrhage, spinal cord infarction, isolated neuropathies due to anatomical proximity to infarcted bones, lead neuropathy, auditory problems, and ocular manifestations are other neurological problems which can affect patients with sickle cell disease.