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Cerebral infarction in sickle cell trait.

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Cerebral Infarction
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 reports 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 documented by hemoglobin electrophoresis. This report describes 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
Fig 3. Contrast computed tomographic scan at 6% months of
age. Transverse section through the midbrain shows bilateral tegmental 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 diagnosis 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 tomography in cerebral diseases of white matter. Neurology (NY)
28~534-544, 1978
6. Mall K, Gardner-Medwin D: CT scan appearance in Leigh‘s disease (subacute necrotizing encephalomyelopathy). Neuroradi010gy 16~48-50,1978
7. Rushton AR, Shaywitz BA, Duncan CC, et al: Computed tomography in the diagnosis of Canavan’s disease. Ann Neurol 10:5760, 1981
8. Schwartz WJ, Hutchinson HT, Berg BO: Computerized tomography in subacute necrotizing encephdomyelopathy (Leigh disease). Ann Neurol 10:268-271, 1981
The neurological consequences of sickle cell disease
are widely appreciated. However, neurological sequelae 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 episode 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 hemispheric 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 infarction 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. Hematocrit, white blood cell count, sedimentation rate, electrocardiogram, chest x-ray study, and arterial blood gas and cerebrospinal 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 demonstrated 36.3% hemoglobin S; the remainder was hemoglobin A.
From the Division of Neurology, Duke University Medical Center,
Durham, NC.
Received Oct 19, 1983, and in revised form Mar 25, 1985. Accepted 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
Reference
Thompson et al, 1948 [ l o ]
Diggs and Jones, 1952 [37
Ende et al, 1955 [ 4 ]
Age W ,
Sex
Diagnosis
Hemoglobin
Other
Electrophoresis Organs
20, M
48, F
46, M
26, M
Not done
Not done
Not done
SA
SA
SA
SA
SA
SA
SA
25, M
Schenk, 1964 [ B ]
12, M
Dalal et al, 1974 [27
12, M
Handler and Perkin, 1982 167 22, M
Present report
24, M
35, M
M
=
male; F = female; MCA
=
Cortical vein thrombosis
Diffuse cortical hemorrhages, no occlusion
Venous occlusion
Right MCA hemorrhagic infarct
(?) Posterior fossa lesion-unknown pathology
Sagittal sinus thrombosis
Sagittal sinus thrombosis and infarction
Bilateral infarcts
Bilateral infarcts
Right MCA infarct
Not reported
Liver, spleen
Gdney, lung
Not reported
Bowel
Not reported
Not reported
Not reported
Liver, spleen
No
middle cerebral artery; SA = sickle cell trait.
Head computed tomographic (CT) scan showed enhancing
low-density lesions in the left occipital and left frontotemporal regions. Nonenhancing lesions were seen in both
parietal lobes. The patient was begun on anticoagulant treatment 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. H e
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 normal.
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 [ll]. Most strokes occur
in subjects under age 15 1111, and patients may have
hemogiobin S values as low as 17 to 33.5% [l}. Sickling, 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 cerebrovascular 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 manifestations 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 patients the onset of symptoms followed moderate exertion.
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 ischemia 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
42:1065-1075, 1955
5. Greer M, Schotland D: Abnormal hemoglobin as a cause of
neurologic disease. Neurology (NY) 12:114-123, 1962
6. Handler CE, Perkin GD. Sickle cell trait and multiple cerebral
infarctions. J R SOCMed 75:550-555, 1982
7. Helzsover KJ, Hayden FG, Rogol AD: Severe metabolic complications in a cross-country runner with sickle cell trait. JAMA
249:777-779, 1983
8. Powars D, Wilson B, Imbus C, et al: The natural history of
stroke in sickle cell disease. Am J Med 65:461-471, 1978
9. Schenk EA: Sickle cell trait and superior longitudinal sinus
thrombosis. Ann Intern Med 60:465-470, 1964
10. 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
11. Wood DH: Cerebrovascular complications of sickle cell anemia.
Stroke 12:23-27, 1975
Brief Communication: Greenberg and Massey: Cerebral Infarction
355
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