Vascular Accident and Hemiplegia in a Patient With Sickle Cell Anemia

Abstract

History.\p=m-\Acolored boy, aged 6, entered Duke Hospital on Aug. 13, 1933, because of headache, dizziness and unconsciousness. He had been well until two days before admission. At that time, he complained of "stomach ache," which disappeared after catharsis. The next morning his head ached and, shortly afterward, he became dizzy and drowsy, and was too weak to climb into bed. His left arm and leg made spasmodic movements and his right limbs were powerless. Speech was only slightly impaired until later in the day, when he became stuporous. He had taken no food or water for twenty-four hours preceding admission to the hospital. The family history was interesting in that a brother had died several months previously after the onset and course of symptoms similar to those of this patient. A sister, aged 12, had had "rheumatism" for several years and her blood smears showed 25 per cent immediate sickling. Smears of blood from the father and mother did not contain any sickle cells. The patient's past history was unimportant.Physical Examination.-The boy's temperature on admission was 38 C. (100.5 F.) ; the pulse rate was 120; the respiratory rate was 28, and the blood pres¬ sure was 140 systolic and 75 diastolic. The patient was well developed, moderately dehydrated and in a semistuporous state. The skin was dry, and there was gen¬ eral glandular enlargement. The nail beds and mucous membranes were pale, while the sclerae were a deep greenish yellow. The eyegrounds were normal. The tonsils were large, cryptic and moderately injected. The lungs were clear as noted on percussion and auscultation. There was a systolic murmur heard best at the apex and transmitted into the axilla. The right arm and leg were very weak and flaccid. The deep reflexes were hyperactive and the Babinski sign was present on both sides, but remained permanent only on the right side. The cranial nerves were not involved. The spleen was not palpable.Laboratory Tests.-The blood count showed the following : hemoglobin, 9.8 Gm. (64 per cent) Sahli ; red blood cells, 2,740,000; white blood cells, 17,450; polymorphonuclear leukocytes, 80 per cent ; large lymphocytes, 8 per cent ; small lymphocytes, 8 per cent, myelocytes and transitionals, 4 per cent. There was 25 per cent immediate sickling, and 75 per cent within four hours. Bleeding time was one and one-half minutes and clotting time, one minute. Examinations of the stool and urine and intradermal tuberculin (0.1 mg. with old tuberculin), Wasser¬ mann and Schick tests gave negative results.Course.-Immediately after the patient was admitted to the ward, a lumbar puncture was made and bloody fluid obtained. Clear spinal fluid was collected the next day ; however, numerous red blood cells were found microscopically. The From the

The emerging understanding of sickle cell disease

“…This review established the early age of involvement and the high frequency of recurrence, features stressed in subsequent reviews (Greer & Schotland, 1962; Powars et al , 1978). A thrombotic mechanism was proposed (Arena, 1935; Kampmeier, 1936) and confirmed by Hughes et al (1940), and partial or complete occlusion of major cerebral arteries occurred in six out of seven children (Stockman et al , 1972). Major clinical features of stroke in SS disease included the young age of involvement (median age 6 years), the predominance of cerebral infarction in children and of haemorrhage in adults, and a 50–70% tendency to recurrence within 3 years of the initial episode (Powars et al , 1978; Balkaran et al , 1992).…”

Sickle cell disease: The neurological complications

“…Sickle cell disease (SCD) was recognized by West Africans centuries ago,1, 2 and sickled red cells were described in 1910,3 but the significance of these observations was not yet fully understood. By 1935, 3 patients with SCD and acute hemiplegia had been reported, including 1 with an autopsy finding of cerebral infarction 3–5. In the 1920s, the pathological basis of the abnormal hemoglobin was considered,6 and in 1949, Pauling and colleagues determined that SCD was caused by an abnormal hemoglobin molecule distinct from normal hemoglobin A (HbA) 7.…”

Brain infarction in sickle cell anemia: Magnetic resonance imaging correlates

“…Imaging was carried out with a multislice technique allowing the simultaneous observation of 18 sec-on published criteria [13]. Borderzone, or so-called watershed (WS), infarcts were of three types: (1) involving the superficial territory between the middle (MCA) and the anterior (ACA) cerebral arteries (anterior WS infarct); (2) involving the superficial territory between the MCA and the posterior cerebral arteries (posterior WS infarct); and (3) involving the borderzone between the superficial and the deep territories of the MCA in the region of the centrum semiovale (deep WS infarct) Ill]. Thus, the diagnosis of a WS infarct was based on MRI findings.…”