he Role of Electrophysiological Studies in Assessment of Peripheral and Trigeminal Neuropathy in Patients with Sickle Cell

Abstract

Sickle cell disease (SCD) is the most common genetic disease of the blood. It is an autosomal recessive inherited hemoglobinopathy. It is characterized by chronic hemolytic anemia and episodes of painful vaso-occlusive crises (VOC).

VOC resulting in occlusion of vasanervosum and infarction of nerves, can lead to cranial nerve neuropathies particularly, trigeminal neuropathy, mental neuropathy, as well as, peripheral nerves mononeuropathy multiplex or distal sensorimotor demyelinating polyneuropathy.

Peripheral nervous system involvement in SCD is under-appreciated, as silent subclinical peripheral neuropathy involvement does occur. Moreover, SCD peripheral neuropathic involvement may be obscured by the severity of central nervous system complications.

Emphasizing peripheral and cranial neuropathy in SCD will increase the recognition of such complications and ultimately providing new insights into their prevention and treatment.

Electrophysiological studies (EPS) are objective, noninvasive and potential useful methods for diagnosis of symptomatic trigeminal and peripheral neuropathy, as well as, for detecting subclinical involvement of these nerves. Application of orthodromic recording of mental sensory nerve action potential (SNAP) in concert with trigeminal evoked