Accepted Manuscript Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion Elizabeth A. Yu, MD, PhD Laura K. Snydman, MD Joseph Rencic, MD PII:
S0002-9343(14)00672-X
DOI:
10.1016/j.amjmed.2014.07.034
Reference:
AJM 12635
To appear in:
The American Journal of Medicine
Received Date: 21 July 2014 Revised Date:
24 July 2014
Accepted Date: 25 July 2014
Please cite this article as: Yu EA, Snydman LK, Rencic J, Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion, The American Journal of Medicine (2014), doi: 10.1016/j.amjmed.2014.07.034. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Title Page Title of manuscript: Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion
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Article type: Clinical Communication to the Editor Authors: Elizabeth A. Yu, MD, PhDa,b, Laura K. Snydman, MDa, Joseph Rencic, MDa a Department of Medicine, Tufts Medical Center, Boston, MA, USA b Corresponding author: 800 Washington Street, Boston, MA, USA 02111 [email protected], (617)636-5000
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To the best of our knowledge, no conflict of interest, financial or other, exists. All authors had access to the data and a role in writing the case report.
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Keywords: Ramsay Hunt syndrome, ataxia, syndrome of inappropriate antidiuretic hormone secretion, SIADH, cerebellitis, varicella zoster
ACCEPTED MANUSCRIPT
To the Editor: Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion
SC
RI PT
A 64-year-old immunocompetent Cantonese-speaking man with no significant past medical history presented with altered mental status and gait disturbance. He complained of sore throat, subjective fevers, headache, right ear pain, and dysgeusia six days prior to admission for which he drank two liters of water daily. His wife reported that he developed confusion and repeatedly fell to his right side when walking without associated lightheadedness or vertigo. The patient also had new symptoms of difficulty urinating and constipation.
M AN U
Examination revealed two aphthous ulcers on the right side of his hard palate that did not cross the midline and a peripheral 7th nerve palsy. His right ear canal was erythematous, there was dried blood on his tympanic membrane without vesicular lesions, and he had mild right sensorineural hearing loss. His prostate was enlarged (3+). He fell to the right when performing tandem gait, but had a negative Romberg test and an otherwise normal neurologic exam.
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Laboratory studies revealed a sodium of 123 mEq/L (normal 135-145). Pertinent normal values included a serum creatinine, thyroid stimulating hormone, morning cortisol, urine sodium, urine osmolality, serum osmolality and human immunodeficiency virus. Magnetic resonance imaging (MRI) of his auditory canal showed intracanalicular segment enhancement of the right facial nerve extending to the fundus, tympanic, and mastoid segments of the right facial nerve, with hyperintense T2 signal and enhancement with the right mastoid air cells suggesting mastoiditis (Figure 1).
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Hyponatremia with normal urine studies was consistent with syndrome of inappropriate antidiuretic hormone (SIADH) and he was treated with fluid restriction. While his confusion improved with normalization of his sodium, his neurological findings and urinary retention remained unchanged. Foley catheter placement yielded one liter of urine. His peripheral 7th nerve palsy, erythematous ear canal with sensorineural hearing loss, and right-sided palatal ulcers suggested Ramsay Hunt syndrome and acyclovir 760 mg IV every 8 hours was started empirically. Lumbar puncture revealed a normal opening pressure, 60 white blood cells per mm3 (60% lymphocytes of which 5% were atypical), and polymerase chain reaction was positive for varicella zoster virus (VZV). The patient developed acute kidney injury from acyclovir, which resolved with IV fluids and transition to valacyclovir 1000 mg three times daily. He was started on prednisone 60 mg daily for one week followed by a one week taper. Eight weeks later, his ataxia resolved and hearing loss and facial droop improved. He ultimately underwent transurethral resection of the prostate for his urinary retention. Ramsay Hunt syndrome (aka Herpes zoster oticus) is a polycranial neuropathy due to reactivation of varicella zoster virus in the geniculate ganglion, causing the triad of
ACCEPTED MANUSCRIPT
ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle1. Our patient demonstrated the polycranial neuropathy including features of zoster-related cerebellitis.
RI PT
To our knowledge, this is the first report of Ramsay Hunt syndrome in an immunocompetent patient presenting with atypical external ear manifestations, palatal ulceration, and cerebellitis.2 There are two cases of varicella zoster virus infection with ataxia, but those occurred in the absence of facial nerve palsy, vesicular rash, or dermatomal-distribution of pain.3,4 Clinicians with awareness of these uncommon manifestations are less likely to miss the diagnosis of Ramsay Hunt syndrome, leading to expedited treatment and a reduced potential for long-term sequelae.
SC
Acknowledgements
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We acknowledge Dr. Daniel Do-Dai of the radiology department at Tufts Medical Center. References 1
Adour, KK. Otological complications of herpes zoster. Ann Neurol 1994; 35: Suppl S62–4.
2
Park, HK and Lee, JH. A case of Ramsay Hunt syndrome complicated by cerebellitis J Clin Neurol 2006; 2: 198–201.
3
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Ratzka, P, Schlachetzki, JCM, Bahr, M, Nau, R. Varicella zoster virus cerebellitis in a 66-year-old patient without herpes zoster. The Lancet 2006; 367: 182.
4
EP
Suzuki, Y, Kanno, A, Minami, M, Ogawa, K, Oishi, M, Kamei, S. Case of acute cerebellitis as a result of varicella zoster virus infection without skin manifestations Geriatr Gerontol Int 2012; 12: 756–7. Authors: Elizabeth A. Yu, MD, PhD, Laura K. Snydman, MD, Joseph Rencic, MD
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Figure 1. MRI internal auditory canal. Abnormal enhancement of the intracanalicular, tympanic and mastoid segments of the right facial nerve (white arrow) likely representing sequela of an infectious or inflammatory process.
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EP
TE D
M AN U
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RI PT
ACCEPTED MANUSCRIPT
S0002-9343(14)00672-X
DOI:
10.1016/j.amjmed.2014.07.034
Reference:
AJM 12635
To appear in:
The American Journal of Medicine
Received Date: 21 July 2014 Revised Date:
24 July 2014
Accepted Date: 25 July 2014
Please cite this article as: Yu EA, Snydman LK, Rencic J, Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion, The American Journal of Medicine (2014), doi: 10.1016/j.amjmed.2014.07.034. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Title Page Title of manuscript: Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion
RI PT
Article type: Clinical Communication to the Editor Authors: Elizabeth A. Yu, MD, PhDa,b, Laura K. Snydman, MDa, Joseph Rencic, MDa a Department of Medicine, Tufts Medical Center, Boston, MA, USA b Corresponding author: 800 Washington Street, Boston, MA, USA 02111 [email protected], (617)636-5000
SC
To the best of our knowledge, no conflict of interest, financial or other, exists. All authors had access to the data and a role in writing the case report.
AC C
EP
TE D
M AN U
Keywords: Ramsay Hunt syndrome, ataxia, syndrome of inappropriate antidiuretic hormone secretion, SIADH, cerebellitis, varicella zoster
ACCEPTED MANUSCRIPT
To the Editor: Ramsay Hunt syndrome presenting with ataxia and syndrome of inappropriate antidiuretic hormone secretion
SC
RI PT
A 64-year-old immunocompetent Cantonese-speaking man with no significant past medical history presented with altered mental status and gait disturbance. He complained of sore throat, subjective fevers, headache, right ear pain, and dysgeusia six days prior to admission for which he drank two liters of water daily. His wife reported that he developed confusion and repeatedly fell to his right side when walking without associated lightheadedness or vertigo. The patient also had new symptoms of difficulty urinating and constipation.
M AN U
Examination revealed two aphthous ulcers on the right side of his hard palate that did not cross the midline and a peripheral 7th nerve palsy. His right ear canal was erythematous, there was dried blood on his tympanic membrane without vesicular lesions, and he had mild right sensorineural hearing loss. His prostate was enlarged (3+). He fell to the right when performing tandem gait, but had a negative Romberg test and an otherwise normal neurologic exam.
TE D
Laboratory studies revealed a sodium of 123 mEq/L (normal 135-145). Pertinent normal values included a serum creatinine, thyroid stimulating hormone, morning cortisol, urine sodium, urine osmolality, serum osmolality and human immunodeficiency virus. Magnetic resonance imaging (MRI) of his auditory canal showed intracanalicular segment enhancement of the right facial nerve extending to the fundus, tympanic, and mastoid segments of the right facial nerve, with hyperintense T2 signal and enhancement with the right mastoid air cells suggesting mastoiditis (Figure 1).
AC C
EP
Hyponatremia with normal urine studies was consistent with syndrome of inappropriate antidiuretic hormone (SIADH) and he was treated with fluid restriction. While his confusion improved with normalization of his sodium, his neurological findings and urinary retention remained unchanged. Foley catheter placement yielded one liter of urine. His peripheral 7th nerve palsy, erythematous ear canal with sensorineural hearing loss, and right-sided palatal ulcers suggested Ramsay Hunt syndrome and acyclovir 760 mg IV every 8 hours was started empirically. Lumbar puncture revealed a normal opening pressure, 60 white blood cells per mm3 (60% lymphocytes of which 5% were atypical), and polymerase chain reaction was positive for varicella zoster virus (VZV). The patient developed acute kidney injury from acyclovir, which resolved with IV fluids and transition to valacyclovir 1000 mg three times daily. He was started on prednisone 60 mg daily for one week followed by a one week taper. Eight weeks later, his ataxia resolved and hearing loss and facial droop improved. He ultimately underwent transurethral resection of the prostate for his urinary retention. Ramsay Hunt syndrome (aka Herpes zoster oticus) is a polycranial neuropathy due to reactivation of varicella zoster virus in the geniculate ganglion, causing the triad of
ACCEPTED MANUSCRIPT
ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle1. Our patient demonstrated the polycranial neuropathy including features of zoster-related cerebellitis.
RI PT
To our knowledge, this is the first report of Ramsay Hunt syndrome in an immunocompetent patient presenting with atypical external ear manifestations, palatal ulceration, and cerebellitis.2 There are two cases of varicella zoster virus infection with ataxia, but those occurred in the absence of facial nerve palsy, vesicular rash, or dermatomal-distribution of pain.3,4 Clinicians with awareness of these uncommon manifestations are less likely to miss the diagnosis of Ramsay Hunt syndrome, leading to expedited treatment and a reduced potential for long-term sequelae.
SC
Acknowledgements
M AN U
We acknowledge Dr. Daniel Do-Dai of the radiology department at Tufts Medical Center. References 1
Adour, KK. Otological complications of herpes zoster. Ann Neurol 1994; 35: Suppl S62–4.
2
Park, HK and Lee, JH. A case of Ramsay Hunt syndrome complicated by cerebellitis J Clin Neurol 2006; 2: 198–201.
3
TE D
Ratzka, P, Schlachetzki, JCM, Bahr, M, Nau, R. Varicella zoster virus cerebellitis in a 66-year-old patient without herpes zoster. The Lancet 2006; 367: 182.
4
EP
Suzuki, Y, Kanno, A, Minami, M, Ogawa, K, Oishi, M, Kamei, S. Case of acute cerebellitis as a result of varicella zoster virus infection without skin manifestations Geriatr Gerontol Int 2012; 12: 756–7. Authors: Elizabeth A. Yu, MD, PhD, Laura K. Snydman, MD, Joseph Rencic, MD
AC C
Figure 1. MRI internal auditory canal. Abnormal enhancement of the intracanalicular, tympanic and mastoid segments of the right facial nerve (white arrow) likely representing sequela of an infectious or inflammatory process.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
