Cerebral Infarction

Fig 3. Contrast computed tomographic scan at 6% months of age. Transverse section through the midbrain shows bilateral teg- mental lucencies.

scribed basal ganglia and thalamic involvement, one should be aware that radiolucent changes limited to the posterior fossa and hemispheric white matter are not specific for SNE.

An additional clue to the diagnosis of SNE by CT before death is contained in a recent report of waxing and waning of the CT findings of SNE 141. Until a more definitive test is discovered, however, the diag- nosis must rest on a combination of clinical, laboratory, and radiological findings.

References 1. Andriola MR: Computed tomography in the diagnosis of Cana-

van’s disease. Ann Neurol 11:323-324, 1982 2. Bottshauser E, Isler W: CAT scan in spongy degeneration. Lancet

1:1123, 1976 3. KendaIl B: Cranial CT scans in metabolic diseases involving the

CNS of children. Resid Staff Physician 28:33-42, 1982 4 . Koch TK, Lo WD, Berg BO: Variability of serial CT scans in

subacute necrotizing encephalomyelopathy (Leigh disease). Pediatr Neurol 1:48-51, 1985

5. Lane B, Carroll BA, Pedley TA: Computerized cranial tomog- raphy in cerebral diseases of white matter. Neurology (NY)

6. Mall K, Gardner-Medwin D: CT scan appearance in Leigh‘s dis- ease (subacute necrotizing encephalomyelopathy). Neuroradi-

7. Rushton AR, Shaywitz BA, Duncan CC, et al: Computed tomog- raphy in the diagnosis of Canavan’s disease. Ann Neurol 10:57- 60, 1981

8. Schwartz WJ, Hutchinson HT, Berg BO: Computerized tomog- raphy in subacute necrotizing encephdomyelopathy (Leigh dis- ease). Ann Neurol 10:268-271, 1981

28~534-544, 1978

010gy 16~48-50, 1978

in Sickle Cell Trait Joel Greenberg, MD, and E. Wayne Massey, M D

Sickle cell disease is known to predispose patients to the risk of cerebral infarction. However, only scattered re- ports exist of the neurological sequelae of the sickle cell trait. Only 8 cases are reported in the English literature, in some of which the sickle cell trait was not docu- mented by hemoglobin electrophoresis. This report de- scribes 2 men, age 35 and 24 years, who developed acute cerebral infarction. Investigation revealed no apparent cause for the lesion other than the sickle cell trait.

Greenberg J, Massey EW: Cerebral infarction in sickle cell trait. Ann Neurol 18:354-355, 1985

The neurological consequences of sickle cell disease are widely appreciated. However, neurological se- quelae associated with the sickle cell trait are less well known. Two patients with this disorder following strokes are described.

Case Reports Patient 1 A 24-year-old, right-handed black man had a 10-minute epi- sode of weakness in his left hand. Five days earlier a similar episode had occurred. He described a recent automobile accident which occurred because he was unable to see a car approaching on the right. One year earlier the patient had had a two-week episode of aphasia, diagnosed as a left hemis- pheric stroke. An arteriogram done at that time was normal. At the same time, an evaluation for abdominal pain and hematuria documented sickle cell trait with evidence of in- farction in the spleen and liver. H e was treated with aspirin and dipyridarnole. There was no history of heart disease, hypertension, diabetes mellitus, or drug abuse.

Physical examination revealed a right homonymous hemianopia and diminished sensation to pinprick over the left arm. Results of motor examination were normal. Hema- tocrit, white blood cell count, sedimentation rate, electrocar- diogram, chest x-ray study, and arterial blood gas and cere- brospinal fluid values were all normal. Fibrinogen and fibrin split product levels, platelet functions, and the serologic panel were all normal. Serum protein electrophoresis, glucose-6-phosphate dehydrogenase (G6PD), and red cell volume were normal. Hemoglobin electrophoresis demon- strated 36.3% hemoglobin S; the remainder was hemo- globin A.

From the Division of Neurology, Duke University Medical Center, Durham, NC. Received Oct 19, 1983, and in revised form Mar 25, 1985. Ac- cepted for publication Mar 28, 1985. Presented at the American Neurological Association Meeting, New Orleans, LA, October 2-5, 1983.

354

Cerebrovascular Compkcations in Sichle Cell Trait

Age W , Hemoglobin Other Reference Sex Diagnosis Electrophoresis Organs

Thompson et al, 1948 [ lo] 20, M Cortical vein thrombosis Not done Not reported Diggs and Jones, 1952 [37 48, F Diffuse cortical hemorrhages, no occlusion Not done Liver, spleen Ende et al, 1955 [ 4 ] 46, M Venous occlusion Not done Gdney, lung

26, M Right MCA hemorrhagic infarct SA Not reported 25, M (?) Posterior fossa lesion-unknown pathology SA Bowel

Schenk, 1964 [ B ] 12, M Sagittal sinus thrombosis SA Not reported Dalal et al, 1974 [27 12, M Sagittal sinus thrombosis and infarction SA Not reported Handler and Perkin, 1982 167 22, M Bilateral infarcts SA Not reported Present report 24, M Bilateral infarcts SA Liver, spleen

35, M Right MCA infarct SA No

M = male; F = female; MCA = middle cerebral artery; SA = sickle cell trait.

Head computed tomographic (CT) scan showed enhancing low-density lesions in the left occipital and left frontotem- poral regions. Nonenhancing lesions were seen in both parietal lobes. The patient was begun on anticoagulant treat- ment with warfarin and has been without further incident for ten months.

Patient 2 A 35-year-old, right-handed black man developed a mild headache, followed the next day by weakness in his left arm. The following morning he was found to have left hemiparesis that within hours progressed to a flaccid hemiplegia. He smoked one pack of cigarettes per day. There was no history of stroke, heart disease, hypertension, or diabetes mellitus.

Laboratory data, including serological and clotting studies, were normal except for hemoglobin electrophoresis that showed 37% hemoglobin S and 63% hemoglobin A.

Initial head CT scan showed decreased density involving a large area of the right cerebral hemisphere. A repeat scan at 48 hours showed effacement of the right lateral ventricle. An arch arteriogram and selective right arteriogram were nor- mal.

The cerebral edema resolved and the patient’s condition gradually improved. At two months follow-up, neurological examination revealed only a minimal decrease in left-sided rapid alternating movements.

Discussion Sickle cell disease is an important risk factor in the development of stroke (Table), with a prevalence as high as 17% in some studies [ l l ] . Most strokes occur in subjects under age 15 1111, and patients may have hemogiobin S values as low as 17 to 33.5% [l}. Sick- ling, followed by impairment of flow, is the presumed mechanism of vascular occlusion [S}..

By contrast, sickle cell trait is considered a benign condition. Of 8 cases previously reported with cere- brovascular problems, only 3 offer evidence of both infarction and confirmation by hemoglobin elec-

trophoresis 12, 4, 61. In one review of 175 patients with sickle cell trait, 11 had neurological manifesta- tions but none experienced stroke 151. In 2 cases 12, 91, anesthesia or perioperative hypoxia, or both, were suggested as precipitating causes. Four patients had evidence of infarction in other organs. In our 2 pa- tients the onset of symptoms followed moderate exer- tion.

Since the prevalence of sickle cell trait in American blacks is 7 to 9% 171, hemoglobin electrophoresis should be carried out routinely in young patients with unexplained strokes. The discovery of sickle cell trait in a stroke patient should prompt a search for visceral infarction.

References 1. Buchanan GR, Bowman WP, Smith SJ: Recurrent cerebral isch-

emia during hypertransfusion therapy in sickle cell anemia. J Pediatr 103921-923, 1983

2. Dalal FX, Schmidt GB, Bennett EJ, Ramamorthy S: Sickle cell trait: a report of a postoperative neurologic complication. Br J Anaesth 46:387-388, 1974

3. D i g s L, Jones RS: Clinicoparhologic conference. Am J Clin Pathol 22:1194-2000, 1952

4. Ende N, Pizzolato P, Ziskind J: Sicklemia. Ann Intern Med

5 . Greer M, Schotland D: Abnormal hemoglobin as a cause of 42:1065-1075, 1955

6.

7.

8.

9.

10.

11.

neurologic disease. Neurology (NY) 12:114-123, 1962 Handler CE, Perkin GD. Sickle cell trait and multiple cerebral infarctions. J R SOC Med 75:550-555, 1982 Helzsover KJ, Hayden FG, Rogol AD: Severe metabolic com- plications in a cross-country runner with sickle cell trait. JAMA

Powars D, Wilson B, Imbus C, et al: The natural history of stroke in sickle cell disease. Am J Med 65:461-471, 1978 Schenk EA: Sickle cell trait and superior longitudinal sinus thrombosis. Ann Intern Med 60:465-470, 1964 Thompson RK, Wagner SA, Macleod M: Sickle cell disease: report of a case with cerebral manifestations in the absence of anemia. Ann Intern Med 29921-928, 1948 Wood DH: Cerebrovascular complications of sickle cell anemia. Stroke 12:23-27, 1975

249:777-779, 1983

Brief Communication: Greenberg and Massey: Cerebral Infarction 355