Varicella with
Delayed Hemiplegia
Takashi Ichiyama, MD*,
Sadataka Houdou, MD*,
Toshiro Kisa, MD*, Kousaku Ohno, MD*,
and Kenzo Takeshita, MD*

We report 4 children who developed acute hemiplegia
7 w e e k s to 4 m o n t h s a f t e r v a r i c e l l a i n f e c t i o n . In 2 patients, c a r o t i d a n g i o g r a p h y d e m o n s t r a t e d s e g m e n t a l
n a r r o w i n g a n d occlusion of t h e m i d d l e c e r e b r a l a r t e r y .
T h e i r clinical and a n g i o g r a p h i c f e a t u r e s w e r e s i m i l a r
to t h o s e a s s o c i a t e d w i t h c o n t r a l a t e r a l h e m i p l e g i a a f t e r
h e r p e s z o s t e r o p h t h a l m i c u s , t h e p a t h o g e n e s i s of w h i c h
c o m p r i s e s c e r e b r a l angiitis d u e to v a r i c e l l a z o s t e r v i r a l
i n f e c t i o n . W e believe t h a t o u r p a t i e n t s h a d t h e s a m e
p a t h o g e n e s i s . In a s u r v e y o f infectious diseases in o u r
region, the f r e q u e n c y of v a r i c e l l a w i t h d e l a y e d h e m i p a r e s i s was r o u g h l y 1:6,500 v a r i c e l l a p a t i e n t s .
l c h i y a m a T, Houdou S, Kisa T, O h n o K, Takeshita K.
Varicella with delayed hemiplegia. Pediatr Neurol 1990;6:
279-81.

Introduction

Herpes zoster ophthalmicus (HZO) with delayed contralateral hemiparesis is a distinct central nervous system
s y n d r o m e caused by the varicella zoster virus (VZV). It is
characterized by unilateral vasculopathy affecting larger
cerebral arteries and appears a few days to 6 months after
H Z O [1-11 ]. The pathogenesis comprises cerebral angiitis
due to direct V Z V infection [4-6].
We report 4 children with varicella infection and delayed hemiplegia. Because the clinical and angiographic
features were similar to those of hemiplegia after H Z O ,
the pathogenesis of both syndromes may be the same.
Case Reports
Patient 1. This 4-year-old boy suddenly experienced fight-sided
hemiconvulsions and then developed gait disturbance 11 weeks after
varicella infection which included generalized vesicular exanthema.
The course had been uncomplicated.
On admission, neurologic examination revealed marked fight hemiplegia and motor aphasia. Computed tomography (CT) performed 10

From the *Division of Child Neurology; Institute of Neurological
Sciences; Tottofi University School of Medicine; Yonago, Japan;
*Department of Pediatrics; Shimane Prefectural Central Hospital;
Izumo, Japan.

~
~
=:~:_,,...I a ~lightly low-density lesion in the left
striatocapsular region. The serum antivaricella virus antigen IgG titer
determined by an indirect fluorescent antibody (IFA) method was positive at 1:40 dilution: however, the IgM titer was not elevated. Neither
titer was elevated in the cerebrospinal fluid (CSF). CSF contained 3
leukocytes/mm3 Call mononuclear cellsL 17 mg/dl protein. 70 mg/dl
glucose, and 1.28 mg/dl lgG. Antinuclear antibody was negative. The
coagulation studies were normal, including platelets (286.000/mm3),
bleeding time (3 minL fibrinogen (255 mg/dlL prothrombin time
(107%). activated partial thromboplastin time (35 sec; control: 28-37
secl. and fibfinogen degradation products (< I mg/dl). Echocardiography disclosed no abnormality.
Three weeks later, the low-density lesion had resolved on CT (Fig
IAI. Carotid angiography revealed segmental narrowing of the left
middle cerebral artery (MCA: Fig 2A). Eight months later he was normal except for clumsiness of the right arm.
Patient 2. This l-year-old girl developed acute right-sided weakness
4 months after vaficella infection. Vesicular exanthema had been mainly on the trunk with a little on the face and head.
On admission, she exhibited mild right hemiparesis. CT revealed a
low-density lesion in the left anterior limb of the internal capsule (Fig
I B). CSF contained 2 leukocytes/mm3 (mononuclear cells), glucose
54 mg/dl, and protein 8 mg/dl. The serum complement-fixation titer for
varicella was positive at 1:16 dilution. Electrocardiography revealed no
arrhythmia. Carotid angiography disclosed occlusion of the proximal
segment of the left MCA (Fig 2B). Five months later, her neurologic
deficits had disappeared completely.
Patient 3. This 7-year-old boy developed varicella which had an uncomplicated, typical course. Several vesicles appeared on the face. Two
months later, he developed acute right-sided weakness with aphasia
lasting about 5 rain. Subsequently, the same type of attack occurred 4
times. He developed slurred speech and fight-sided weakness within I
day. On admission, he had a right hemiparesis. Tendon reflexes were
normal. On day 19 of hospitalization, c'r revealed a low-density lesion
in the left lenticular nucleus (Fig IC). CSF contained I leukocyte/mm3
(mononuclear), glucose 63 mg]dl, and protein 19 mg/dl. Results of
bleeding time (3.5 rain), coagulation time (7 mio), and aggregation of
blood platelets by adenosine diphosphate, collagen, and epinephrine
were normal. Carotid angiography and echocardiography did not reveal
any abnormalities. One month later he was asymptomatic.
Patient 4. This 2-year-old girl developed varicella following measles.
On recovery from varicella, she was believed to be normal. Seven
weeks after the onset of varicella, she complained of fatigue and dizziness. She rapidly developed weakness in the right arm and leg and
could not walk. On admission, she exhibited fight hemiplegia. CT revealed a hypodensity in the left striatocapsular region (Fig ID). Carotid
angiography was not performed. Four months later, she was discharged
with residual hemiparesis and dystonia of the fight upper extremity.
Discussion

H Z O with delayed contralateral hemiparesis was first
recognized in 1919 [1]. Since then, there have been reports
o f more than 30 patients [2-11]. T h e r e is an interval of a
few days to 6 months (average: 8 weeks) between H Z O
onset and the neurologic disorder [2]. In most instances,
C T demonstrates small, low-density lesions in the deep
central region (i.e., basal ganglia,, internal capsule, and
thalamus), occasionally referred to as lacunar infarcts [3,4,

Communications should be addressed to:
Dr. Ichiyama; Division of Child Neurology;
Institute of Neurological Sciences;
Tottori University School of Medicine; Nishi-machi 86;
Yonago 683, Japan.
Received February 6. 1990; accepted March 19, 1990.

Ichiyama et al" Varicella and Hemjplegia 279

Figure 1. CT scans of Patients 1-4 reveal low-density lesions in the left basal ganglia (at'rows). (A) Patient 1. (B) Patient 2. (C) Patient 3. (D) Patient 4.

8,11]. Carotid angiography demonstrates segmental constriction and/or occlusion of the proximal middle and
anterior cerebral arteries [2,3,7,8]. Pathologic studies
reveal virus-like particles and viral antigens in vessel
walls [4-6], which suggest vasculitis due to direct VZV
infection.
Acute hemiplegia, appearing 1-3 months after varicella
infection, was first reported by Eda et al., but without
angiographic evidence [12]. A patient was reported with
varicella followed by hemiparesis with angiographic
evidence of vasculitis [13]. In previously reported patients,
lacunar infarcts were detected on CT. All of our patients
also had attacks of acute hemiplegia 7 weeks to 4 months
after varicella infection; infarcts were also revealed on CT.
In addition, carotid angiography demonstrated segmental
narrowing and occlusion of the MCA in 2 patients. Clinical
and radiographic findings in our patients and others are
very similar to those with HZO and delayed contralateral
hemiparesis in terms of the interval from the onset of the
eruption to the onset of hemiparesis, CT findings, and
carotid angiographic findings. Considering that varicella
and herpes zoster are different clinical manifestations of
the same causative agent, the occasional association of
acute hemiparesis with antecedent varicella strongly suggests a causal relationship.

280

PEDIATRIC NEUROLOGY

Vol. 6 No. 4

The cerebral angiographic findings, which are similar to
those in HZO with delayed hemiparesis, suggest that the

n.

Figure 2. (A) A left internal carotid angiogram of Patient 1
demonstrates segmental narrowing of the left MCA (arrowhead). (B)
A left internal carotid angiogram of Patient 2 reveals occlusion of the
left MCA (arrowhead).

pathogenesis o f acute hemiplegia after varicella results
from vasculitis due to V Z V infection. Although the distribution of vascular lesions in patients with H Z O is consistent with what is known about patterns o f trigeminovascular connections, cerebral vasculopathies have been
reported in patients with segmental herpes zoster infection
without clinical e v i d e n c e of trigeminal or disseminated
herpes zoster infection [3,4,91. This finding suggests other
m e c h a n i s m s for viral access to the cerebral vessels, including trigeminal infection without a rash, hematogenous
seeding o f nerves, or spreading via sympathetic nervous
system pathways [4].
In our region (the San-in district, Japan), 4 patients were
observed between 1979 and 1986. A survey of infectious
diseases in this area disclosed that there were 26,001
varicella patients in the same period; therefore, the frequency of varicella with delayed hemiplegia is roughly
1:6,500 varicella patients.

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Ichiyama et ai: Varic¢lla and Hemiplegia 281