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Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis.

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Computerized Tomography
as a Diagnostic Aid in
Acute Hemorrhagic
Ted L. Rothstein, MD," and Cheng-Mei Shaw, MD1~~~
Computerized tomography (CT) in a pathologically
proven case of acute hemorrhagic leukoencephalitis
(AHL) showed a mass effect and increased absorption
coefficient i n the right hemisphere within 18 hours of
the onset of neurological symptoms. The changes
corresponded to the site of white matter edema, necrosis,
and petechial hemorrhages demonstrated postmortem.
The early changes on CT reflect the hyperacute nature
of AHL and differ from those of herpes simplex
Rothstein TL, Shaw C-M. Computerized
tomography as a diagnostic aid in acute
hemorrhagic leukoencephalitis.
Ann Neurol 13:331-333, 1983
Acute hemorrhagic leukoencephalitis (AHL) is a rare,
hyperacute, inflammatory disease of cerebral white
matter with a rapid and catastrophic course. These features make diagnosis during life difficult.
A 25-year-old, right-handed man was admitted to the hospital nine hours after he developed progressive weakness and
paresthesias of the left arm and leg. Five days previously he
had developed a productive cough and decreased breath
sounds in his left lung, and was treated with erythromycin.
From the "Department of Neurology, Northwest Hospital, Seattle,
WA 98133, and the tDepartment of Pathoiogy and Child Development and the Mental Retardation Center, University of Washington
Medical School, Seattle, WA 98195.
Received Apr 30, 1982, and in revised form June 2. Accepted for
publication June 27, 1982.
Address reprinc requescs to Dr Rotbsrein, 1570 N 11Sth St, Suite 2,
Seattle, WA 98133.
Examination on admission revealed slight irritability but an
otherwise normal mental state. The temperature was 37°C
and the pulse 134 beats per minute. Blood pressure was l6Oi
100 mm Hg. The neck was not rigid. A grade 214 systolic
ejection murmur was present at the left sternal border.
Breath sounds were reduced at the left lung base and axilla.
The disc margins were flat, but retinal venous pulsations were
absent. The peripheral visual fields were full to confrontation.
The pupils were equal and reacted briskly to light and on
convergence. Extraocular movements were limited in upward
gaze. The left lower side of the face was slightly weak. Function of the other cranial nerves was normal. There was mildly
diminished strength and tone in the left arm and leg. Occasional involuntary pincer-like movements of the left thumb
and forefinger were observed. The reflexes were hypoactive
and symmetrical. The right plantar response was flexor, the
left neutral. Reaction to pinprick was impaired over the left
side of the trunk and extremities.
While under observation the patient developed a generalized tonic-clonic seizure, greater on the left. The episode
lasted five minutes, after which he was obtunded and had
more severe left-sided paralysis.
Laboratory findings inc1uded.a white cell count of 15,500
with 84% neutrophils and 8% band neutrophils. The urine,
hemoglobin, erythrocyte sedimentation rate, serum glucose,
nonprotein nitrogen, and electrolytes were all normal. Lumbar puncture yielded cloudy cerebrospinal fluid (CSF) under
a pressure of 300 mg HZO.The fluid contained 3,143 white
cells per cubic millimeter, 95% polymorphonuclear, and no
red blood cells; total protein was 160 mgidl, and glucose 66
mgidl. N o organisms were seen on smear, and culture \;as
negative. Serum and CSF serology results were negative. An
electrocardiogram showed diffuse loss of T-wave voltage and
S-T segment sagging. An electroencephalogram was diffusely
abnormal with delta activity throughout and rare runs of
spike waves and sharp and slow waves in the right central and
parietal regions.
A roentgenogram of the chest showed a left perihilar
infiltrate. A computerized tomographic (CT)scan performed
18 hours after the onset of neurological symptoms showed a
mass effect of the right hemisphere with partial obliteration
and shift of the right lateral ventricle and septum pellucidum
to the left of midline (Fig 1). Areas of low absorption
coefficient involved the white matter of the temporal and
parietal regions, more marked on the right, with generalized
contrast enhancement of gray matter (Fig 2).
The patient's seizures were treated with intravenous
diphenylhydantoin and diazepam, and he received dexamethasone and mannitol for cerebral edema. O n the second hospital day he was unresponsive, had decerebrate responses to
stimuli, and had blurred disc margins and sluggish pupillary
reactions. Intermittent seizure activity of the left leg persisted. A brain biopsy obtained through a right temporal
craniotomy contained no inflammation or inclusion bodies.
Neurons showed loss of cytoplasmic and nuclear detail,
suggesting degeneration. Fluorescent antibody studies and
viral cultures for herpes simplex were negative. Adenine
arabinoside therapy was begun empirically, but the patient
deteriorated with loss of brainstem reflexes and two successive isoelectric electroencephalograms. H e died on the eighth
hospital day.
33 1
Fig I . CT Scan obtained 18 hours after onset of neurologicaf
symptom.r, area of decreaseddensity in the right hemispherejJ
lateralf,entrjclrand &ift
of midline structures from right to 14.There ;.F coyitrajt enha,tc-ement of peripheral gray matter.
F i g 2 . Brain substance near the vertex is decreased in density bilaterah). u i t h more extensi&einvohement on the righi side and
lhqt of midline from right to
Di//use contrart enhancement
0fgra.y matter is again seen.
Nezlropathological Findings
The whole brain weighed 1,310 gm, including 185 gm of the
brainstem and cerebellum after fixation. The leptomeninges
were normal. The cerebral arteries showed no atherosclerosis. There was caudal herniation of cerebellar tonsils, which
were necrotic and friable. The cerebrum was dusky and
mildly friable because of autolysis. It was sectioned in a fresh
state, so the cut surfaces were irregularly shrunken and brain
slices were distorted. The degree of transtentorial herniation
could not be evaluated. Coronal sections of the cerebral
hemispheres revealed flattening of convexities and obliteration of sulci. The third and lateral ventricles appeared to be
displaced to the left but were normal in size. There was
diffuse swelling with softening, gray discoloration, and
numerous petechial hemorrhages of the right frontoparietal
white matter (Fig 3). This lesion was relatively sharply demarcated from the overlying cerebral cortex, but the ventral border was poorly defined. The arcuate or U fibers were grossly
preserved (Fig 4 ) . The cerebral cortex was congested, with
focal gray discoloration in the depth of the sulci. The brainstem and cerebellum showed diffuse swelling with obliteration of the aqueduct and fourth ventricle. The spinai cord was
not studied.
Microscopically, the lesion in the right frontoparietal white
matter showed diffuse edema and marked demyelination
with focal necrosis and numerous ring-and-ball hemorrhages.
Nerve fibers were well preserved except in foci of necrosis.
Blood vessels showed thickened walls. Vascular necrosis and
332 Annals of Neurology
Vol 1 3 N o 3 March 1983
Fig 3. Coronal section through the puhinav. There is difluse
swelling ojthe right cerebral hemzsphere with peteshial hemorrhage.r, gray discoloration,and necrosir of the superior parietal
white matter.
perivenous fibrinous exudates were also present. A small
number of lymphocytes were found in the wall of some blood
vessels, but inflammatory exudates were inconspicuous. O n
the other hand, microglia cells were abundant and had a tendency to cluster around blood vessels but also infiltrated diffusely into the parenchyma in a patchy fashion. Necrosis and
demyelinatiori were more pronounced in the subcortical re-
Fig 4. Magnified view of the lesion, disclosing widespread petechial hemorrhage and necrosis in the white matter sparing the
cortex and U-Jzbers.
gions and became less marked in deeper white matrer. The
subcortical arcuate fibers were unstained with myelin stain
but were free of necrosis, gitter cell infiltration, and edema.
The cerebral cortex over the lesion was well preserved except
for occasional small foci of ischemic neuronal changes and
edema. No lesions except for edema and autolytic changes
were found in other parts of the brain.
Though this brain showed a moderate degree of autolysis
because the patient had been placed on a respirator for six
days, both the macroscopic and microscopic findings are characteristic of AHL.
Acute hemorrhagic leukoencephalitis is a hyperacute
disease of cerebral white matter characterized by progressive coma, pyramidal signs, seizures, and usually
death {3]. The C T scans in this patient mirrored the
violent effects of the disease on the brain and demonstrated the presence of a low absorption coefficient and
mass effect resulting from edema and necrosis of the
centrum semiovale. The presence of contrast enhancement reflected abnormal permeability of contrast from
damaged blood vessels.
One previous case report described C T findings in a
patient assumed to have had AHL [GI. That patient
presented with a subacute illness from which he eventually recovered. The brain biopsy was normal, as was
the initial C T scan. The presentation, clinical course,
and lack of pathological confirmation make a diagnosis
of AHL unlikely.
An important clinical feature of AHL is the frequent
unilaterality of cerebral involvement [3}. Of the primary viral encephalitides, only herpes simplex encephalitis (HSE) is likely to produce similar localized
abnormalities. HSE and AHL have parallel symptoms
early in the course, and clinical differentiation may be
The CT scan provides a point of distinction between
AHL and HSE. In HSE, the CT is usually normal in
the early stages [I}. Subsequently, the predominant
finding is an area of decreased attenuation in the temporal lobes that may involve the insular cortex but
spares the lenticular nucleus El, 4 , 71.These changes
were rarely present sooner than five days after the onset of neurological symptoms [l, S], and early in the
course C T often failed to reflect the full extent of the
disease process {S]. Mass effect, when present, increased with time and paralleled the increasing size of
the low-density lesion {2). Bilateral mass effect was an
early finding in two patients who eventually developed
low-absorption abnormalities 111. The <IT changes in
HSE conform to the development of angiographic and
technetium brain scan alterations, in which patients examined five days after the onset of neurological symptoms had abnormal findings [ S ] . The presence of a
mass effect or increased absorption coefficient on CT
within the first three days after the onset of hyperacute
encephalitis suggests AHL, and may permit diagnosis
during life.
Presented in part at the Twelfth World Congress of Neurology,
Kyoto, Japan, Sept 23, 1981.
D r Lawrence Cromwell helped in interpretation of the neuroradiological results, and D r Donald Nakonechny performed the
autopsy. The manuscript was prepared by Bobbit. J. Bennett and
Vernita S. Olson.
1. Davis JM, Davis KR, Kleinman GM, Ktrchner HS, Taveras JM:
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clinicopathological correlation. Radiology 129:409-4 17, 1978
2. Enxmann DR, Ranson B, Norman D, Talberth E: Computerized
tomography of herpes simplex encephalitis. Radiology 1294 19425, 1978
3. Gosztonyi G: Acute hemorrhagic leuko-encephalitis (Hurst’s disease). In Vinken PJ, Bruyn G W (eds): Handbook of Clinical
Neurology. Vol 3, Infections of the Nervous System, Part 11.
Amsterdam, Elsevier North-Holland, 1978, pp 587-604
4. Kauffman DM, Zimmerman RD, Leeds NE: Computerized tomography in herpes simplex encephalitis. Neurology (NY)
29:1392-1396, 1979
5. Pexman KHW: The angiographic and brain scan features of acute
herpes simplex encephalitis. Br J Radiol 47:179-184, 1974
6. Reich H, Shu-Ran L, Goldblatt D: Computerized tomography in
acute hemorrhagic leuko-encephalopathy: a case report. NeurolO ~ Y(NY) 29:255-258, 1970
7. Thomson JLG: The computerized axial tornograph in acute
herpes simplex encephalitis. Br J Radiol 49:86-87, 1076
8. Zimmerman RD, Russell EJ, Leeds NE, Kauffman DM: CT in the
early diagnosis of herpes simplex encephalitis. AJR 134:61-66,
Case Report: Rothstein and Shaw: CT in Acute Hemorrhagic Leukoencephalitis
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aid, computerized, leukoencephalitis, hemorrhagic, tomography, acute, diagnostika
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