Peripheral nervous system involvement in patients with sickle cell disease

Area of Science:

• Neurology
• Hematology
• Genetics

Background:

• Sickle cell disease (SCD) is a genetic blood disorder.
• Peripheral nervous system (PNS) involvement is considered rare in SCD.
• Neurological complications in SCD are typically studied in symptomatic patients.

Purpose of the Study:

• To electrophysiologically determine peripheral nerve involvement in SCD patients without clinical neurological signs.
• To investigate the relationship between sickle cell crisis frequency and peripheral neuropathy in SCD patients.

Main Methods:

• Fifty-one SCD patients and fifty-one healthy controls were enrolled.
• Conventional electrophysiological studies of peripheral nerves were performed on all participants.
• Data on the frequency of sickle cell crises were collected.

Main Results:

• Peripheral nervous system involvement was detected in 19.6% of SCD patients.
• Neuropathy types included sensorimotor axonal, sensory axonal, ulnar sensory, and median sensory neuropathies.
• The frequency of neuropathy was significantly higher in SCD patients compared to controls, but not correlated with crisis frequency.

Conclusions:

• Subclinical peripheral nerve involvement is prevalent in SCD patients.
• Electrophysiological examinations are recommended for early diagnosis of neuropathy in asymptomatic SCD patients.
• Early detection of neuropathy can guide management and potentially prevent further neurological damage.