الفهرس 
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Abstract
Summary
SCD is an autosomal recessive hemoglobinopathy resulting from a
structural change in the sequence of amino acids on the beta globin chain of
the hemoglobin molecule due to a point mutation. SCD is characterized by
hemoglobin polymerization, erythrocyte stiffening, and subsequent vasoocclusion.
Acute and chronic organ dysfunction, chronic hemolytic anemia, and
recurrent painful episodes are the main features of sickle cell disease. In
addition, chronic cerebral ischemia and cerebral vascular anomalies are
considered among the most disabling problems in sickle cell disease.
Neurological complications of sickle cell disease include: headache,
”soft neurological signs’’, seizures, neurocognitive impairment, visual loss
ischemic stroke, hemorrhagic stroke, transient ischemic attack, silent cerebral
infarction, coma, altered mental status, moyamoya disease and neuropathic
pain.
Early diagnosis and proper management of SCD neurological
complications require specialized hematological and neurological expertise.
The newly used medications under ongoing research foster the hope to
overcome this devastating disease and its complications.
The aim of this work was to assess the neurological disorders
including: stroke, transient ischemic attacks, seizures, cognitive impairment,
visual loss and silent infarction in pediatric patients with sickle cell disease
using multimodal approach through clinical, laboratory, neuroimaging and
neurophysiological studies in a trial to detect etiological risk factors.
This study was carried out on 50 children suffering from sickle cell
disease ’’diagnosed by hemoglobin electrophoresis’’ aged from 2 to 18 years
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Summary & Conclusion
old including 27 males and 23 females. There was also a control group of 25
healthy children matched with the age (3 to 16 years old) and gender
including 16 males and 9 females who attended general outpatient clinic of
Pediatric Department for a comparative study.
All children were subjected to:
1) Full medical history taking.
2) Thorough neurological examination using pediatric neurological
sheet.
3) Laboratory investigations including: hemoglobin electrophoresis,
complete blood picture count with differential, reticulocyte count,
serum electrolytes, proteins C and S, renal function and hepatobiliary
function tests.
4) Neuroimaging including: CT and /or MRI of the brain. Also, MRA
and /or CT angiography of cerebral blood vessels when needed in
some patients. Besides, MRV when needed in some patients.
5) Transcranial color coded duplex (TCCD).
6) Electrophysiological studies including electroencephalogram (EEG).
7) Stanford-Binet Intelligence scales-Fifth Edition as an evaluation tool
for intellectual functioning.
This study revealed that:
Many patients with SCD showed positive family history for the
disease. They were also positive for consanguinity.
Most patients presented with headache, cognitive decline, seizures
and visual