Sickle cell anemia is a hemolytic anemia due to haemoglobinopathy that had variable clinical presentation because it affects all body organs, all age groups but mainly younger age leading to considerable morbidity & mortality, as well as financial burden on health service and economy.[16,17,18] The pathologic lesions in the brains of patients with sickle cell disease who have neurological manifestations are varied and widespread. However, the principal changes are due to intravascular occlusion of blood vessels.[19,20]
The study was conducted in 100 Sudanese patients seen in the sickle cell clinic in Jaafar Ibn Aof pediatric hospital. Male to female ratio was 50:5o almost equally (1-1), this similar to what was mentioned in the literature by Ariel.[21] Age groups 11-20 years 53% resembling the bulk number of the patients. Geographical distribution of the patients revealed 71% of the total patients originated from the western Sudan, that was almost similar to what was mention in study done in Sudan 1996-2000 (Relationship of the sickle cell gene to the ethnic and geographic groups populating the Sudan).[9] SCA was found to be predominant among the Afro-Asiatic-speaking groups (68.4%) It includes nomadic groups of Arab and non-Arab descent who immigrated to Sudan at different times in history.Those patients clustered in western Sudan (Kordofan and Darfur) from where 73% of all cases originate.[9] About educational level, those who did not go for schooling either because of their illness or because of financial problem. Those who were studying at different educational levels, most of them had a delay in schooling because of their illness. Because of endogamy and the disease has an autosomal recessive inheritance, 58% of our patients had a positive family history of sickle cell anemia.
Non neurological clinical history and examination revealed that abdominal pain occurred more frequently in the age group 6-10 years (54.7% of the total number of the patients in this study), this is due to the fact that abdominal crisis is a well-known manifestations of the disease, this similar to what was mentioned in previous studies.[22,23] Abdominal pain is often so severe that the patient is thought to have an acute surgical emergency.[24] Extremities pain distributed mainly in the age group 11-20 years, similar to what was mentioned in the literature.[25,26] Mistaken diagnosis of acute poliomyelitis, scurvy or injury are made frequently because of pain in the muscles of the neck, back and legs. Unlike what was mentioned in the literature, priapism occurred only in 2% only, because it's often under diagnosed.[27,28,29] Almost 74% of our patients receive blood, because of the crisis or severe anemia. Systolic murmur was found in 35%, it had correlation with occurrence of low HB level. Abdominal examination showed that 32% of our studied group had hepatomegaly whereas only 2% had splenomegaly that indicate either splenic crisis or combination with other hemoglobinopathy.
It is important to recognize the fact that severe neurological disturbances in sickle cell disease may exist without anemia. This indicates that hemolysis of erythrocytes with subsequent anemia is not always the principal underlying process in sickle cell disease. Similar to what was mentioned in the literature the most common neurological symptoms was irritability which was distributed mainly in the age group 11-20 years, followed by headache.[30,31,32,33] Neurological manifestations observed included facial weakness, stiffness of the legs, nystagmus, transitory blindness, ptosis, generalized rigidity, dysphagia, anesthesia and analgesia of right side of body, nasal regurgitation, marked salivation, numbness, limbs weakness, seizure , drowsiness, photophobia , neck stiffness, deafness, night blindness, diplopia and delirium were similar to what was observed in a study which was conducted in John Gaston Children's Hospital.[34] The presence of S-hemoglobin appears to be a definite predisposing factor to the development of cerebral vascular disease, particularly in children. The types of cerebral vascular lesions that may occur with sickle cell disease include ischemic and/or hemorrhagic infarction, intracerebral hemorrhage, cortical venous and/or sinus thrombosis, and subarachnoid hemorrhage. Cerebral infarction is the most common cerebral vascular lesion. It also should be emphasized that not only can ischemic lesions occur in the brain and brainstem, they can also develop in the spinal cord. An alarming aspect of our study is younger age of presentation with neurological problems with major disability and tendency to recurrence hemiplegia .Many of the patients with recurrent strokes developed pseudo bulbar palsy-like picture associated with difficulty in speaking, swallowing, and ambulation, this is near to what was mentioned in Cooperative study, having high incidence in young age group below 20 years and reduce incidence above 20year.[35] In the Cooperative Study of Sickle Cell Disease, the incidence per 100 patient years of a first cerebral infarct in a series from the United States was 0.70 between ages two and five years, 0.51 between ages six and nine years, 0.24 between 10 and 19 years of age, and then fell to 0.04 between the ages of 20 and 29.[35] In many instances, however, the only manifestation of a crisis maybe the sudden development of a focal neurological deficit with or without seizures. A considerable number of sickle cell patients presenting with a stroke in our series had generalized or focal seizures at the onset. Subsequently, the majority of these patients developed a chronic seizure disorder requiring long-term anticonvulsant therapy. Seizure associated with hemiplegia in 11 patients (45.8%) P. value = 0.000, this may be due to post stroke seizure or due to sickling process per se , bilateral involvement indicating high risk of recurrence as mention in the literature.[36,37,38] Plantar reflex was up going in 13% resembling (54.2%) of the total hemiplegic. The rest of hemiplegic patient 11 patient (45.8%) had down going planter P. value = 0.000 .This result suggesting that it is not a good indicator for neurological assessment. The gait was spastic in 14% of our patients. There is no correlation between blood transfusion & occurrence of hemiplegia, this opposite to what was mention by STOP 1 trial that is because it was an episodic blood transfusion not a prophylactic chronic blood transfusion program due to screening program. [39] HB level was found to be low in most of our patients. This low readings not because of the disease alone but nutritional & financial factors had a role similar to what was mention in study done by Bayoumi (sickle cell disease in Sudan).[40]