Magnitude of Involvement of Peripheral Nervous System in Sickle Cell Anemia Patients in Vaso-Occlusive Crisis

镰状细胞贫血患者血管阻塞危象中周围神经系统受累程度

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Abstract

Sickle cell disease is an inborn genetic blood disorder that is usually found among residents of Africa, Middle-East Gulf countries, and India. There has been increased knowledge and awareness of the range of neurological complications of Sickle cell disease (SCD) over the past 20 years. Silent neurologic involvement may occur in SCD and there's an opportunity of subclinical involvement in these patients presenting with vasoocclusive crisis. The case-control study was carried out after getting the institutional ethical clearance and obtaining consent from the patients and the controls. They were subjected to nerve conduction studies. Nerve conduction study was performed on median and tibial nerves for motor nerves, and median and sural for sensory nerves unilaterally. The nerves of the upper limb showed changes in neuropathy. The lower limb nerves were completely normal. In our study, we found out that Distal motor latency (DML) is prolonged in the Median motor nerve in cases as compared to controls suggesting motor neuropathy, and Sensory nerve action potential (SNAP) is reduced in the median sensory nerve suggesting sensory neuropathy. The nerve conduction studies in the tibial nerve were normal, suggesting subclinical neuropathy could be present in the patients with sickle cell presenting as mononeuropathy or mononeuritis multiplex. Our study also found a statistically significant correlation of neuropathy with the increasing number of hospital admissions for the vaso-occlusive crisis. Subclinical neuropathy is another manifestation of nervous system in patients with SCD and one should give adjuvant therapy of membrane stabilizing agents for the relief of pain. Detailed studies of this complication must be required further to evaluate its significance in clinical practice. We also conclude the aches and the pain the patient of sickle cell disease complains could not be always due to vaso-occlusive crisis, it could be the neuropathic pain which might mimic the ischemic pain.

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