Peripheral nervous system involvement in patients with sickle cell disease

Peripheral nervous system involvement is rare in sickle cell disease (SCD). The aim of this study is to determine the peripheral nerve involvement electrophysiologically in SCD patients without clinically evident neurological signs, symptoms and to determine the relationship between the frequency of sickle cell crisis and peripheral neuropathy.

Fifty-one patients with SCD and fifty-one healthy controls were enrolled to the study. Conventional electrophysiological studies of peripheral nerves were performed to all subjects. The data about the frequency of sickle cell crisis were obtained.

Peripheral nervous system involvement was detected in ten (19.6%) patients. Five (9.8%) patients had sensorimotor axonal neuropathy, two (3.9%) sensory axonal neuropathy, one (2%) patient had ulnar sensory neuropathy and two (3.9%) had median sensory neuropathy. Sural nerve sensorial action potential was unobtainable in eight (15.7%) patients and prolonged F latencies were observed in three (5.9%). The frequency of neuropathy was higher in SCD patients when compared with the controls. The frequency of sickle cell crisis was not significantly correlated with peripheral neuropathy.

Subclinical peripheral nerve involvement may be seen in SCD patients. Electrophysiological examinations are recommended in routine examination to diagnose early neuropathy in SCD patients without neurologic symptoms.