الفهرس 
Pathogenesis of SCDOnly 14 pages are availabe for public view
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Abstract
Sickle cell disease (SCD) is the most common genetic disease o
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 o
nerves, can lead to cranial nerve neuropathies particularly, trigemina
neuropathy, mental neuropathy, as well as, peripheral nerves
mononeuropathy multiplex or distal sensorimotor demyelinating
Ppolyneuropathy.
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 wil
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 involvemen of these nerves. Application of orthodromic recording of mental sensory nerve action potential (SNAP) in concert with trigeminal evoked potentials (TEP) are sensitive tools for the investigation of trigemina nerve sensory afferents and the central components, respectively. Computed tomographic (CT) of the mandible is an important too to assess patients with mental neuropathy. CT imaging of the mandible performed in several planes can yield an accurate anatomic image of the mandibular canal diameter, and the dimensions of the mental foramen on the buccal side of the mandible. The aim of our study was to detect the possible association between SCD (HbSS) and either trigeminal or peripheral neuropathy as well as, the pattern of trigeminal and peripheral neuropathic lesion in SCD patients, and correlate the results with clinical & laboratory results and also with CT findings of the mandible. We conducted our study on fifty adult patients with SCA and ten healthy subjects a control group. Our patients were divided into two groups. Group (I) included thirty SCA patients, who were clinically free of any symptoms or signs of peripheral or trigeminal neuropathy. Group II included twenty SCA patients, who had symptoms and/or signs o either peripheral or trigeminal neuropathy.
We performed EPS for trigeminal nerve (including nerve conduction study (NCS) of inferior alveolar nerve (IAN), TEP and blink reflex) and for peripheral nerves (motor and sensory NCS of bilatera median, ulnar, tibial, common peroneal and sural nerves and somatosensory evoked potentials (SSEP) of both median nerves) to detect either symptomatic or subclinical trigeminal and periphera neuropathy. Mental foramen (MF) dimensions; height and width and mandibular canal (MC) diameter were assessed in group II patients and controls using CT scan images in axial and cross-sectional views.