Neurol Sci DOI 10.1007/s10072-014-1818-1
LETTER TO THE EDITOR
Ramsay Hunt syndrome after cervical zoster in an immunocompetent patient Dong-Woo Shin • Jeong-Min Kim • Suk-Won Ahn Young Chul Youn • Oh-Sang Kwon
•
Received: 17 January 2014 / Accepted: 3 May 2014 Ó Springer-Verlag Italia 2014
Keywords Ramsay Hunt syndrome Varicellar zoster virus Cervical zoster Dear Sir, Ramsay Hunt syndrome is the reactivation of varicellar zoster virus (VZV) involving geniculate ganglion which is associated with unilateral facial paralysis, vertigo and hearing difficulty [1]. We report a case of Ramsay Hunt syndrome in an otherwise healthy girl who previously had had cervical zoster. The patient had typical clinical manifestation of Ramsay Hunt syndrome, but notable finding is that her symptoms were preceded with cervical zoster, suggesting distinct viral spreading mechanism by anatomical connection between upper cervical roots and lower cranial nerves. A 16-year-old girl visited neurology clinic complaining of left side facial palsy which started 3 days ago. She also complained of left ear fullness and tinnitus. She did not complain of vertigo or hyperacusis. Two weeks ago she had had neuropathic pain and vesicular skin eruptions involving third cervical dermatomes. She was diagnosed as varicella zoster, and cervical lesions and pain were improved after topical steroid and analgesics treatment. She had a history of chickenpox but did not have any other past medical history which could compromise immunity. Neurological examination revealed left side peripheral type facial palsy, hypogeusia and left sensorineuronal
D.-W. Shin J.-M. Kim (&) S.-W. Ahn Y. C. Youn O.-S. Kwon Department of Neurology, Chung-Ang University Hospital, Chung-Ang University Medical Center, Chung-Ang University College of Medicine, 102, Heukseok-ro, Dongjak-gu, Seoul 156-755, South Korea e-mail: [email protected]
hearing loss. The severity of facial weakness scored five points on the House–Brackmann facial disability score. Other cranial nerve and motor examination was normal, and meningeal irritation sign was absent. She had multiple healing vesicular lesions involving second and third cervical dermatomes due to cervical zoster (Fig. 1a), but she did not have any skin lesion on her face or ear. Brain MRI showed edematous inflammatory change of left facial nerve and normal brainstem (Fig. 1b). Cerebrospinal fluid (CSF) analysis disclosed normal opening pressure with clear color, no red blood cell (0/mm3) and borderline increase of white blood cells (5/mm3) with normal protein (22.2 mg/dL, normal range 8–43 mg/dL) and glucose level (58 mg/dL, normal range 40–70 mg/dL, serum glucose 98 mg/dL). The blink test showed absent electrical activity of left facial nerve after direct and indirect stimulation. Viral antibody study showed positive anti-VZV immunoglobulin M from serum but negative from CSF. Other antiviral immunoglobulin M against herpes simplex type 1 and 2, cytomegalovirus, and Epstein Barr virus were negative. Polymerase chain reaction test for VZV and herpes simplex virus from CSF was also negative. With an impression of Ramsay Hunt syndrome by VZV, she was treated with 50 mg/day oral prendnisolone and 750 mg/day famciclovir for 7 days. Left ear fullness, left facial weakness and taste change were relieved after treatment but tinnitus remained with fluctuation. After initial VZV infection, the virus remains in cranial nerve, dorsal root ganglia and autonomic nervous system ganglia [2, 3]. Reactivation of VZV occurs in less than 5 % of individuals but more frequently in immunocompromised patients [1, 2]. When zoster reactivation involves multiple dermatomes, disseminated zoster by systemic viremia should be suspected because it is potentially lethal for those
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Neurol Sci
Fig. 1 The cervical skin lesion and Brain MR finding of the patient with Ramsay Hunt syndrome. She had had neuropathic pain and vesicular skin eruptions involving upper cervical dermatomes
2 weeks ago (a). On admission brain MRI revealed edematous change of left facial nerve with normal brain parenchyma (b)
patients with compromised immunity, thereby needing prompt evaluation and treatment [3]. However, Ramsay Hunt syndrome followed by nearby cervical or cranial varicella zoster reactivation has been reported in immunologically healthy subject, which suggests different viral spreading mechanism from immunocompromised host [4]. There are three hypotheses explaining the sequential involvement of cranial and cervical sensory ganglia by VZV [4]. First, reactivated VZV would propagate to nearby ganglia via anatomical connection between lower cranial nerves and upper cervical roots [1, 4]. Spinal accessory nerve plexus is the main network connecting lower cranial and upper cervical nerves [5]. Another theory is CSF/ hematogenous spread. Middle meningeal artery supplying geniculate ganglia and anterior and posterior spinal arteries serving cervical ganglia are located in subarachnoid space and provide arterial supply to dura matter. The interconnection of CSF and vessels located in subarachnoid space may contribute to viral spreading between cervical roots and cranial nerves. Finally, simultaneous activation of multiple ganglia may be another possible mechanism. This case showed that Ramsay Hunt syndrome which occurred after cervical zoster had a benign clinical course. Compared to Bell’s palsy, Ramsay Hunt syndrome has higher House–Brackmann facial disability score and shows poor recovery, but early treatment within 72 h, combination of steroid and antiviral agent, and young onset age under 20 years are known to be good prognostic factors [6,
7]. Initial prompt treatment and young age may be the reason of favorable clinical response in this case.
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Conflict of interest
All authors report no conflict of interest.
Ethical standard The authors declare that they acted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.
References 1. Sweeney CJ, Gilden DH (2001) Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 71:149–154 2. Dworkin RH, Johnson RW, Breuer J, Gnann JW, Levin MJ, Backonja M et al (2007) Recommendations for the management of herpes zoster. Clin Infect Dis 44:S1–S26 3. Gilden DH, Mahalingam R, Cohrs RJ, Tyler KL (2007) Herpesvirus infections of the nervous system. Nat Clin Pract Neurol 3:82–94 4. Worme M, Chada R, Lavallee L (2013) An unexpected case of Ramsay Hunt syndrome: case report and literature review. BMC Res Notes 6:337 5. Brown H (2002) Anatomy of the spinal accessory nerve plexus: relevance to head and neck cancer and atherosclerosis. Exp Biol Med (Maywood) 227:570–578 6. Coulson S, Croxson GR, Adams R, Oey V (2011) Prognostic factors in herpes zoster oticus (Ramsay Hunt syndrome). Otol Neurotol 32:1025–1030 7. Donati D, De Santi L, Ginanneschi F, Cerase A, Annunziata P (2012) Successful response of non-recovering Ramsay Hunt syndrome to intravenous high dose methylprednisolone. J Neurol Sci 318:160–162
LETTER TO THE EDITOR
Ramsay Hunt syndrome after cervical zoster in an immunocompetent patient Dong-Woo Shin • Jeong-Min Kim • Suk-Won Ahn Young Chul Youn • Oh-Sang Kwon
•
Received: 17 January 2014 / Accepted: 3 May 2014 Ó Springer-Verlag Italia 2014
Keywords Ramsay Hunt syndrome Varicellar zoster virus Cervical zoster Dear Sir, Ramsay Hunt syndrome is the reactivation of varicellar zoster virus (VZV) involving geniculate ganglion which is associated with unilateral facial paralysis, vertigo and hearing difficulty [1]. We report a case of Ramsay Hunt syndrome in an otherwise healthy girl who previously had had cervical zoster. The patient had typical clinical manifestation of Ramsay Hunt syndrome, but notable finding is that her symptoms were preceded with cervical zoster, suggesting distinct viral spreading mechanism by anatomical connection between upper cervical roots and lower cranial nerves. A 16-year-old girl visited neurology clinic complaining of left side facial palsy which started 3 days ago. She also complained of left ear fullness and tinnitus. She did not complain of vertigo or hyperacusis. Two weeks ago she had had neuropathic pain and vesicular skin eruptions involving third cervical dermatomes. She was diagnosed as varicella zoster, and cervical lesions and pain were improved after topical steroid and analgesics treatment. She had a history of chickenpox but did not have any other past medical history which could compromise immunity. Neurological examination revealed left side peripheral type facial palsy, hypogeusia and left sensorineuronal
D.-W. Shin J.-M. Kim (&) S.-W. Ahn Y. C. Youn O.-S. Kwon Department of Neurology, Chung-Ang University Hospital, Chung-Ang University Medical Center, Chung-Ang University College of Medicine, 102, Heukseok-ro, Dongjak-gu, Seoul 156-755, South Korea e-mail: [email protected]
hearing loss. The severity of facial weakness scored five points on the House–Brackmann facial disability score. Other cranial nerve and motor examination was normal, and meningeal irritation sign was absent. She had multiple healing vesicular lesions involving second and third cervical dermatomes due to cervical zoster (Fig. 1a), but she did not have any skin lesion on her face or ear. Brain MRI showed edematous inflammatory change of left facial nerve and normal brainstem (Fig. 1b). Cerebrospinal fluid (CSF) analysis disclosed normal opening pressure with clear color, no red blood cell (0/mm3) and borderline increase of white blood cells (5/mm3) with normal protein (22.2 mg/dL, normal range 8–43 mg/dL) and glucose level (58 mg/dL, normal range 40–70 mg/dL, serum glucose 98 mg/dL). The blink test showed absent electrical activity of left facial nerve after direct and indirect stimulation. Viral antibody study showed positive anti-VZV immunoglobulin M from serum but negative from CSF. Other antiviral immunoglobulin M against herpes simplex type 1 and 2, cytomegalovirus, and Epstein Barr virus were negative. Polymerase chain reaction test for VZV and herpes simplex virus from CSF was also negative. With an impression of Ramsay Hunt syndrome by VZV, she was treated with 50 mg/day oral prendnisolone and 750 mg/day famciclovir for 7 days. Left ear fullness, left facial weakness and taste change were relieved after treatment but tinnitus remained with fluctuation. After initial VZV infection, the virus remains in cranial nerve, dorsal root ganglia and autonomic nervous system ganglia [2, 3]. Reactivation of VZV occurs in less than 5 % of individuals but more frequently in immunocompromised patients [1, 2]. When zoster reactivation involves multiple dermatomes, disseminated zoster by systemic viremia should be suspected because it is potentially lethal for those
123
Neurol Sci
Fig. 1 The cervical skin lesion and Brain MR finding of the patient with Ramsay Hunt syndrome. She had had neuropathic pain and vesicular skin eruptions involving upper cervical dermatomes
2 weeks ago (a). On admission brain MRI revealed edematous change of left facial nerve with normal brain parenchyma (b)
patients with compromised immunity, thereby needing prompt evaluation and treatment [3]. However, Ramsay Hunt syndrome followed by nearby cervical or cranial varicella zoster reactivation has been reported in immunologically healthy subject, which suggests different viral spreading mechanism from immunocompromised host [4]. There are three hypotheses explaining the sequential involvement of cranial and cervical sensory ganglia by VZV [4]. First, reactivated VZV would propagate to nearby ganglia via anatomical connection between lower cranial nerves and upper cervical roots [1, 4]. Spinal accessory nerve plexus is the main network connecting lower cranial and upper cervical nerves [5]. Another theory is CSF/ hematogenous spread. Middle meningeal artery supplying geniculate ganglia and anterior and posterior spinal arteries serving cervical ganglia are located in subarachnoid space and provide arterial supply to dura matter. The interconnection of CSF and vessels located in subarachnoid space may contribute to viral spreading between cervical roots and cranial nerves. Finally, simultaneous activation of multiple ganglia may be another possible mechanism. This case showed that Ramsay Hunt syndrome which occurred after cervical zoster had a benign clinical course. Compared to Bell’s palsy, Ramsay Hunt syndrome has higher House–Brackmann facial disability score and shows poor recovery, but early treatment within 72 h, combination of steroid and antiviral agent, and young onset age under 20 years are known to be good prognostic factors [6,
7]. Initial prompt treatment and young age may be the reason of favorable clinical response in this case.
123
Conflict of interest
All authors report no conflict of interest.
Ethical standard The authors declare that they acted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.
References 1. Sweeney CJ, Gilden DH (2001) Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 71:149–154 2. Dworkin RH, Johnson RW, Breuer J, Gnann JW, Levin MJ, Backonja M et al (2007) Recommendations for the management of herpes zoster. Clin Infect Dis 44:S1–S26 3. Gilden DH, Mahalingam R, Cohrs RJ, Tyler KL (2007) Herpesvirus infections of the nervous system. Nat Clin Pract Neurol 3:82–94 4. Worme M, Chada R, Lavallee L (2013) An unexpected case of Ramsay Hunt syndrome: case report and literature review. BMC Res Notes 6:337 5. Brown H (2002) Anatomy of the spinal accessory nerve plexus: relevance to head and neck cancer and atherosclerosis. Exp Biol Med (Maywood) 227:570–578 6. Coulson S, Croxson GR, Adams R, Oey V (2011) Prognostic factors in herpes zoster oticus (Ramsay Hunt syndrome). Otol Neurotol 32:1025–1030 7. Donati D, De Santi L, Ginanneschi F, Cerase A, Annunziata P (2012) Successful response of non-recovering Ramsay Hunt syndrome to intravenous high dose methylprednisolone. J Neurol Sci 318:160–162
