Maqbool Alam, Munir A Lodhi, Durab Khan.
Cerebro Vascular Accident in Sickle Cell Disease.
J Coll Physicians Surg Pak Jan ;13(1):55-6.

An 11-year-old Saudi female reported in pediatric outpatient department with complaint of weakness of left side of the body for last two days. The child was a known case of sickle cell disease and was diagnosed at an age of 6 months. Two other siblings were also sicklers. She had an episode of left sided hemiparesis 2 years back and on CT scan, diagnosis of cerebral infarct was made (Figure 1). Exchange transfusion was done at that time. Patient recovered completely. She was receiving blood transfusion every 6-8 weeks. During summer vacations, she went to her native town and did not receive blood transfusion for about 13 weeks. She developed weakness on left side of the body. On examination, she was fully conscious and well oriented in time, place and person. She was pale and her vital signs were stable. On central nervous system examination, her speech was normal. No cranial nerve deficit was noted. Power in left upper limb was grade IV/V with normal reflexes. Power in left lower limb around hip and knee was grade IV/V and power around left ankle was grade II. Plantar extensor and plantar clonus was present in the left side. No signs of cerebeller lesion or meningial irritation were noted. Rest of the systemic examination did not reveal any abnormality. Her blood counts revealed hemoglobin 7.2 g/dl, platelets 203 x 109/l and total leukocyte count 13.5 x 109/l with differential showing neutrophils 51%, lymphocytes 45%, monocytes 2% and eosinophils 2%. Reticulocyte counts were 13%. Biochemistry profile revealed glucose, urea, creatinine, electrolytes, calcium, aspartate transaminase and alanin transaminase within normal range. Total bilirubin was 46.7 umol/l (normal range 0.0-24 umol/l); out of this unconjugated bilirubin was 41 umol/l. Lactate dehydrogenase (LDH) levels were 1543 U/l (normal value 297-537 U/l). CT scan of the brain revealed right temporo-parietal infarct. Her hemoglobin S was 83% on hemoglobin electrophoresis. Exchange transfusion was done as per protocol of this hospital and child was maintained on adequate hydration. Child was discharged from hospital after two weeks. She was advised physiotherapy for weakness of left lower limb and hyper-transfusion protocol in outpatient department regularly.

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