LETTER TO THE EDI TORS

Trigeminal herpes zoster and Ramsay Hunt Syndrome

with a lesion in the spinal trigeminal nucleus and tract
Chia-Wei Hung

Shuu-Jiun Wang

Shih-Pin Chen

Jiing-Feng Lirng

Jong-Ling Fuh
Received: 6 November 2009 / Revised: 19 January 2010 / Accepted: 21 January 2010 / Published online: 13 February 2010
Springer-Verlag 2010
Abstract We report the case of a 77-year-old immuno-
competent man who developed herpes zoster in the maxillary
and mandibular branches of the trigeminal nerve. Within
3 weeks, he developed ipsilateral peripheral facial palsy,
hearing loss, vesicles over the external auditory canal, and
pain in the face and ear. A T
2
-weighted MRI of the brain
revealed a hyper-intense lesion at the right medulla corre-
sponding to the spinal trigeminal nucleus and tract. Gado-
linium enhancement was seen over the right facial nerve.
These lesions suggest a possibility of transaxonal spread of
the varicella zoster virus between the trigeminal nerve, the
facial nerve, and the spinal trigeminal nucleus and tract.
Keywords Varicella zoster virus Herpes zoster
Spinal trigeminal nucleus Ramsay Hunt Syndrome
Herpes zoster oticus Facial paralysis Magnetic resonance
imaging
Dear Sir or Madam;
We report on a healthy 77-year-old man with acute onset of
right facial pain. Two days after appearance of the symptom,
vesicles developed over the maxillary and mandibular
branches of the right trigeminal nerve. Decreased sensory
responses to pain, temperature, touch, and vibration, in
addition to allodynia throughout all three branches of the
right trigeminal nerve were noted. All other aspects of the
neurological examination were normal. The patient was
diagnosed with trigeminal herpes zoster. After 10 days of
treatment with acyclovir and steroids, the patient reported a
relief of pain symptoms, despite the persistence of facial
vesicles.
Right peripheral facial weakness, right hearing impair-
ment, and new vesicles in the right external auditory canal
developed on day 21 after onset of right facial pain.
Intermittent, but severe pain over the right chin, cheek, and
ear appeared on day 23. Cerebrospinal uid (CSF) was
normal and did not contain anti-varicella zoster virus
(VZV) antibodies. Accordingly, Ramsay Hunt Syndrome
was diagnosed. A T
2
-weighted MRI of the brain performed
on day 35 revealed gadolinium enhancement in the right
facial nerve (Fig. 1). A hyper-intense lesion on the right
posterior lateral portion of the medulla corresponding to
the right spinal trigeminal nucleus and tract (STNT) was
observed (Fig. 2).
The rostral part of STNT is lateral to the nucleus and
bers of the facial nerve, and the caudal part is indistin-
guishable from laminae IIV of the dorsal gray horn in the
cervical spinal cord. The STNT receives primary afferent
bers from the trigeminal, facial, glossopharyngeal, and
vagus nerves, which have general somatosensory functions.
Its efferent bers terminate in motor nuclei of the trigem-
inal and facial nerves, nucleus ambiguous, and hypoglossal
C.-W. Hung
Department of Neurology, Yangming Branch, Taipei City
Hospital, Taipei, Taiwan, ROC
C.-W. Hung S.-J. Wang S.-P. Chen J.-F. Lirng J.-L. Fuh
National Yang-Ming University School of Medicine,
Taipei, Taiwan, ROC
S.-J. Wang S.-P. Chen J.-L. Fuh (&)
Neurological Institute, Taipei Veterans General Hospital,
112 Taipei, Taiwan, ROC
e-mail: jlfuh@vghtpe.gov.tw
J.-F. Lirng
Radiology Department, Taipei Veterans General Hospital,
Taipei, Taiwan, ROC
C.-W. Hung S.-P. Chen
Institute of Clinical Medicine, National Yang-Ming University,
Taipei, Taiwan, ROC
1 3
J Neurol (2010) 257:10451046
DOI 10.1007/s00415-010-5487-6
nucleus. These areas mediate reexes in response to
stimulation of the trigeminal nerve [1]. The involvement of
the STNT and infection of two separate nuclei implies that
herpes zoster can be spread transaxonally in the central
nervous system. Anterograde transaxonal spread of VZV
along the trigeminal nerve bers to the STNT might occur
following reactivation of VZV in the gasserian ganglion.
This may have caused the subsequent spread to the adja-
cent nucleus and bers of the facial nerve or other struc-
tures. The absence of anti-VZV antibodies in the CSF of
our patient suggested that the virus was transmitted from
neuron to neuron rather than through the CSF.
Only two other patients with both trigeminal herpes
zoster and Ramsay Hunt Syndrome have been reported in
the literature [2, 3]. T
2
-weighted MRIs of both patients
revealed the same hyperintense abnormalities at the STNT,
but vesicles only developed in the region of one cranial
nerve. The subsequent symptoms were diagnosed as zoster
sine herpete [4]. Our patient had vesicles in the regions of
the trigeminal and facial nerves. Our case provides evidence
supporting transaxonal spread of VZV. The sequence of
clinical presentation for all three patients differed. One
patient [2] and our patient developed trigeminal herpes
zoster rst, and Ramsay Hunt Syndrome second. The other
patient [3], however, developed them in the reverse order.
The differences in clinical progression suggest that the
transaxonal spread of VZV may occur in either direction.
The interval between trigeminal herpes zoster and
Ramsay Hunt syndrome ranged from 12 days to 3 weeks in
these three patients. This range is similar to the incubation
period of VZV, which is 1021 days [5]. The interval
between the onset of each condition further supports the
hypothesis of transaxonal spread of VZV.
Acknowledgments This study was supported in part by grants from
the Taipei Veterans General Hospital (V99C1-047).
Conict of interest statement None.
References
1. Kiernan JA (1998) Barrs the human nervous system: an anatom-
ical viewpoint, 7th edn. Lippincott-Raven, Philadelphia, pp 126
160
2. Nagane Y, Utsugisawa K, Yonezawa H, Tohgi H (2001) A case
with trigeminal herpes zoster manifesting a long lesion of the
spinal trigeminal nucleus and tract on MR T2-weighted image.
Rinsho Shinkeigaku 41:5659 (in Japanese)
3. Nogueira RG, Seeley WW (2003) Ramsay Hunt Syndrome
associated with spinal trigeminal nucleus and tract involvement
on MRI. Neurology 61:13061307
4. Gilden DH, Kleinschmidt-DeMasters BK, LaGuardia JJ, Mahalin-
gam R, Cohrs RJ (2000) Neurologic complications of the
reactivation of varicella-zoster virus. N Engl J Med 342:635644
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In: Arvin A, Campadelli-Fiume G, Mocarski E et al (eds) Human
herpesviruses: biology, therapy, and immunoprophylaxis. Cam-
bridge University Press, New York, p 683
Fig. 1 Post-gadolinium T1-weighted sequences of a brain MRI
revealed abnormal enhancement in the right facial nerve (white
arrow)
Fig. 2 A T
2
-weighted brain MRI demonstrated a small hyperintense abnormality (white arrows) in the right posterior lateral portion of the
medulla
1046 J Neurol (2010) 257:10451046
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