Chickenpox with
Delayed- Contralateral
Hemiparesis Caused by
Cerebral Angiitis
John Kamholz, MD, and Gerald Tremblay, M D
Chickenpox and herpes zoster ophthalmicus are caused
by the same virus. Herpes zoster ophthalmicus can be
followed by contralateral hemiparesis, which is thought
to be caused by spread of varicella-zoster virus to blood
vessels contiguous to the trigeminal nerve and its
branches. We report what we believe to be the first case
of a patient with chickenpox followed by hemiparesis in
whom there was angiographic evidence of an associated
vasculitis similar to that found with herpes zoster
ophthalmicus.
Kamholz J, Tremblay G: Chickenpox with
delayed contralateral hemiparesis caused by
cerebral angiitis. Ann Neurol 18:358-360, 1985

Herpes zoster ophthalmicus (HLO)with delayed contralateral hemiparesis is a well-defined syndrome.
Thirty-four cases have been reported in the Englishlanguage literature, and characteristic historical, angiographic, and pathological features have been described
(2, 51. A focal cerebral angiitis ipsilateral to the herpes
zoster eruption is the cause of the hemiparesis (91. The
pathogenesis of the vasculitis is unknown but is
thought to be a direct extension of viral infection to
the blood vessels on the affected side via the
ophthalmic nerve f3, 81. Although both varicella and
zoster infections are caused by the same agent, no case
of delayed hemiparesis appears to have been reported
in association with primary varicella infection.

Case Report
Six weeks after the onset of chickenpox, a 6-year-old girl
developed tingling and weakness in her left arm that resolved
within several minutes. Another brief episode of tingling and
weakness in the left arm associated with slurred speech occurred a few days later. Three days prior to admission she
began to drag her left leg and developed slurred speech and
clumsiness of her left hand. Upon admission, healing scars
from chickenpox were evident on her face, extremities, and

From the Department of Neurology, Hospital of the University of
Pennsylvania and Childrens’ Hospital of Philadelphia, Philadelphia,
PA 19104.
Received Nov 19, 1984, and in revised form Feb 28, 1985. Accepted for publication Feb 28, 1985.
Address reprint requests to Dr Kamholz, Laboratory of Molecular
Genetics, NINCDS, National Institutes of Health, Bethesda, MD
20205.

358

lower right eyelid. Findings of the general physical examination were normal. She had a left hemiparesis, greater in her
face and arm than in her leg, and a left Babinski’s response.
Tendon reflexes and sensory tests were normal. Normal laboratory studies included a complete blood count, urinalysis,
electrocardiogram, echocardiogram, liver function tests, and
measurements of serum electrolytes, erythrocyte sedimentation rate, rapid plasma reagin, and antinuclear antibody. Cerebrospinal fluid (CSF) contained 50 red blood cells per mm3
and 13 white blood cells per mm3 (all mononuclear),a glucose value of 59 mgldl, and a protein level of 16 mgldl.
Computed tomography (CT) showed a hypodensity in the
region of the right basal ganglia (Fig 1A) and several small
areas of enhancement in the right centrum semiovale (Fig
1B). An electroencephalogram showed slowing over the
right hemisphere. Arteriography demonstrated marked stenosis of the proximal middle and anterior cerebral arteries on
the right side (Fig 2), with evidence of distal branch occlusions in both territories. The left carotid artery and its
branches were normal.

Discussion
The clinical course of the hemiparesis and the radiographic and CSF findings in our patient are typical of
those found in HZO with delayed hemiparesis (2, 5 ,
91. Neurological symptoms began six weeks postexanthem, and CT showed both hypodense and enhancing lesions. There was a mild CSF mononuclear
cell pleocytosis, and the arteriogram demonstrated segmental narrowing of the MI and A2 branches of the
middle cerebral and anterior cerebral arteries, respectively.
Central nervous system (CNS) complications of primary varicella infection are rare [7] but include diffuse
meningoencephalitis, acute cerebellar ataxia, transverse myelitis, polyradiculoneuropathy, optic neuritis,
and Reye’s syndrome [l, 4, 7, lo]. The pathophysiological basis of these disorders is not known, although
both direct viral infection and immune mechanisms
have been postulated (61. Immune dysfunction is not
associated with the CNS complications of chickenpox
(4, lo], unlike some of the CNS complications of
herpes zoster infection (61. Both meningoencephalitis
and transverse myelitis have been shown to be associated with a necrotizing inflammation of small vessels
[lo]. However, pathological or angiographic evidence
for a large vessel vasculitis as a complication of primary
varicella infection is lacking (4, lo].
In our patient, there was no clinical evidence for an
alteration in immune function, althdugh a subclinical
immunological deficiency has not been excluded. Until
the presentation of her neurological symptoms, there
was nothing unusual about the course of her varicella
infection. However, there may have been accidental
corneal inoculation with virus from varicella lesions on
the lower eyelid on the side of the vasculitis. No eyelid
or eye lesions were found in the opposite side. Corneal

A

B

Fig I . Contrast computed tomographic images showing (A) hypodensity in the area of the right basal ganglia and (B) enhancing lesions in the centrum semiwale.

Fig 2. Right carotid arteriograms showing segmental narrowing
in the proximal branches of both the middle and anterior cerebral
arteries (arrows).

Brief Communication: Kamholz and Tremblay: Chickenpox with Delayed Hemiparesis

359

inoculation could provide a more efficient route of
viral spread into the brain than inoculation from a
cutaneous source and could have led to a higher local
viral concentration near the bifurcation of the internal
carotid artery, thus causing vasculitis by contiguous
spread to these vessels. A less likely possibility is that
our patient had early reactivation of latent varicellazoster virus in the trigeminal ganglion, which could
also have led to an increase in local viral concentration
and vasculitis. The clinical circumstances in this patient
thus support the current notion that varicella-zosterassociated angiitis is due to contiguous spread of the
virus to adjacent cerebral blood vessels. The rarity of
vasculitis in large vessels in chickenpox could be explained by the usually relatively low viral concentration
near the large cerebral vessels. Unusual circumstances
such as corneal inoculation with varicella-zoster virus
may thus be necessary to cause cerebral angiitis. This
patient represents what we believe to be the first
documented case of primary varicella-associated vasculitis in whom the radiographic findings are typical of
the adult syndrome of HZO associated with contralateral hemiparesis.

References
1. Appelbaum E, Rachelson MH, Dolgopol VB: Varicella encephalitis. Am J Med 15:223-230., 1953
2. Bourdette D N , Rosenberg NL, Yatsu FM: Herpes zoster
ophthalmicus and delayed ipsilateral cerebral infarction. Neurology (Cleveland) 33:1428-1432, 1983
3. Doyle PW, Gibson G , Dolman CL: Herpes zoster ophthalmicus
with contralateral hemiplegia: identification of cause. Ann
Neurol 14:84-85, 1983
4. Griffith JF, Salam MV, Adams RD: The nervous system diseases associated with varicella. Acta Neurol Scand 46:279-300,
1970
5. Hilt DC, Buchholz D, Krumholz A, et al: Herpes zoster
ophthalmicus and delayed contralateral hemiparesis caused by
cerebral angiitis: diagnosis and management approaches. Ann
Neurol 14:543-553, 1983
6. Jamsek J, Greenberg SB, Taber L, et al: Herpes zosterassociated encephalitis: clinicopathologic report of 12 cases and
review of the literature. Medicine G2:81-97, 1983
7. Johnson R, Milbourn PE: Central nervous system manifestations of chickenpox. Can Med Assoc J 1022331-834, 1970
8. Linnemann CC, Alvira MM: Pathogenesis of varicella-zoster angiiris in the CNS. Arch Neurol 37:239-240, 1980
9. MacKeozie RA, Forbes GS, Karner, WE: Angiographic findings
in herpes zoster arteritis. Ann Neurol 10:458-464, 1981
10. McKendall RR, Klawans HL: Nervous system complications of
varicella-zoster virus. In Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology, Vol 34. New York, Elsevier, 1978,
pp 161-184

The Auditory Pathology
of Brain Death as
Revealed bv Auditorv
Evoked Po&ntials
J

Kimitaka Kaga, MD,* Akiyu Takamori, MD,"
Toshio Mizutani, MD,? Taiji Nagai, MD,?
and Roger R. Marsh, PhDf
A case of brain death is reported in which the auditory
brainstem response, middle latency component, and
slow vertex response were recorded before and after the
cessation of cortical activity, and in which histological
examination of the temporal bone and central auditory
pathways was performed post mortem. Brain death of at
least 48 hours' duration was demonstrated by neurological examination, flat electroencephalographic recording, and persistent absence of auditory evoked responses. The postmortem examination was performed 3
hours after death. The pathological studies of the whole
length of the auditory pathway revealed total autolysis
of the organ of Corti, marked cell loss in the dorsal
cochlear nucleus, and moderate cell loss in other nuclei
of the central auditory pathway. It should be considered
that total autolysis of the organ of Corti and severe
cell loss of the cochlear nucleus may occur if the auditory brainstem response becomes absent in comatose
patients.
Kaga K, Takamori A, Mitutani T, Nagai T,
Marsh RR: The auditory pathology of brain
death as revealed by auditory evoked potentials.
Ann Neurol 18:360-364, 1985

In the evaluation of brain death, auditory brainstem
response (ABR) testing, unlike the electroencephalogram (EEG), has the potential to define brainstem
death. Generally the appearance of only waves I and I1
on ABR testing implies lesions at the level of the cochlear nucleus of the brainstem, and the absence of an
ABR implies loss of cochlear function or lower brainstem function, or both [I, 3 , 61. Although knowledge
of the correlates of auditory disorders and ABR is very
important for understanding brain death, little information is available.

From the *Department of Otolaryngology, Teikyo University
School of Medicine, Tokyo, the t Department of Pathology, Tokyo
Metropolitan Institute for Neurosciences, Tokyo, Japan, and the
$Department of Otolaryngology and Human Communication, Children's Hospital of Philadelphia, Philadelphia, PA.
Received Feb 1, 1985. Accepted for publication Feb 4 , 1985.
Address reprint requests to Dr Kaga, Department of Otolaryngology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashiku, Tokyo 173, Japan.

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