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Arch Neuropsychiatry 2017; 54: 282-283 • DOI: 10.5152/npa.2016.12654
A Very Rare Type of Neuralgia: Nervus Intermedius Neuralgia
Çok Nadir Bir Nevralji: İntermedius Nevraljisi
Turgay DEMİR, Miray ERDEM, Şebnem BIÇAKCI
Department of Neurology, Çukurova University School of Medicine, Adana, Turkey
Letter to the Editor / Editöre Mektup
Cite this article as: Demir T, Erdem M, Bıçakcı Ş. A Very Rare Type of Neuralgia: Nervus Intermedius Neuralgiai. Arch Neuropsychiatry
2017; 54:282-283.
Dear Editor,
The nervus intermedius, which is the peripheral part of the facial nerve, has visceral motor and special sensory fibers. First described in
1563, the nerve was referred to as “portio media inter comunicantem faciei et nervum auditorium” by Heinrich August Wrisberg in 1777
(1). The word “intermedius” is used because of the intermediate position of the nerve between the superior part of the vestibular
nerve and the facial nerve (2). The nervus intermedius enters the internal auditory meatus immediately after leaving the brainstem and
travels with the facial nerve through the facial canal (Figure 1). According to the diagnostic criteria of the International Classification of
Headache Disorders, 3rd
edition (beta version), nervus intermedius (facial nerve) neuralgia (NIN) is a rare disorder characterized by
brief paroxysms of pain felt deep in the auditory canal that sometimes radiates to the parieto-occipital region (3).
A 55 year-old woman presented to a hospital with very severe burning sensation and stinging pain attacks that persisted for seconds
and often occurred during the day in her right ear. From her past medical history, it was understood that she had diabetes mellitus,
hypertension, and hyperlipidemia. In addition, she was involved in a car accident seven years ago, which resulted in loss of consciousness
and bleeding in her right ear. Her neurological examina-
tion and high-resolution magnetic resonance image of the
brain and inner ear were normal. Hypoplasia of the anteri-
or cerebral artery on the right side was seen during brain
magnetic resonance angiography. Moreover, her temporal
bone computed tomography image was normal. She had
insignificant presbycusis bilaterally during her audiological
evaluation. Thus, in line with existing evidence, she was di-
agnosed with NIN, and following six months of treatment
with 1200 mg/day of gabapentin, her pain attacks stopped.
While the most common cause of cranial neuralgia is trigem-
inal neuralgia, limited data are available on the incidence of
NIN. However, NIN is very rare; the number of cases found
between 1932 and 2012 was less than 150 (4). Other neuro-
logical and otolaryngological etiologies should be excluded in
patients with NIN, which is mostly seen in middle-aged wom-
en. Therefore, it is essential to thoroughly examine the head,
ear, nose, throat, face, and neck of patients. When there is
no underlying etiology, it is referred to as classical NIN. Some
secondary reasons have been reported: herpes zoster, tem-
poromandibular joint dysfunction, nasopharyngeal carcino-
ma, petrous bone osteoma, and neuroborreliosis (5,6).
Depending on the patient’s condition, there are sever-
al treatment options such as surgical treatment, physical
Figure 1. Anatomy of the facial and intermedius nerve
Correspondence Address/Yazışma Adresi: Turgay Demir, Çukurova Üniversitesi Tıp Fakültesi, Nöroloji Anabilim Dalı, Adana, Türkiye
E-mail: drtdemir@gmail.com
Received/Geliş Tarihi: 30.10.2015 Accepted/Kabul Tarihi: 29.02.2016
©Copyright 2017 by Turkish Association of Neuropsychiatry - Available online at www.noropskiyatriarsivi.com
©Telif Hakkı 2017 Türk Nöropsikiyatri Derneği - Makale metnine www.noropskiyatriarsivi.com web sayfasından ulaşılabilir.
282
therapy, analgesic treatment, and nerve block. While antiepileptic drugs
such as carbamazepine, gabapentin, baclofen, or amitriptyline can be used
for medical treatment, radiofrequency gangliolysis, retrogasserian glycer-
ol injections, microvascular decompression surgery, and the gamma knife
system are other treatment modalities.
Nervus intermedius neuralgia should be considered as a differential diag-
nosis if a patient presents with paroxysmal localized pain in the ears.
Informed Consent: Written informed consent was obtained from patient who
participated in this study.
Peer-review: Externally peer-reviewed.
Author Contributions: Concept - T.D., M.E.; Design - T.D., Ş.B.; Supervision Ş.B.;
Resource - T.D., M.E.; Materials - T.D., M.E.; Data Collection and/or Processing - T.D.;
Analysis and/or Interpretation - T.D., Ş.B.; Literature Search - T.D., M.E.; Writing -
T.D.; Critical Reviews - Ş.B.
Acknowledgements: The authors thank to Derviş İlker Gül, faculty member of
the Faculty of Fine Arts, Çukurova University, for his contribution to the drawing
of the facial nerve anatomy.
Conflict of Interest: No conflict of interest was declared by the authors.
Financial Disclosure: The authors declared that this study has received no fi-
nancial support.
Hasta Onamı: Yazılı hasta onamı bu çalışmaya katılan hastadan alınmıştır.
Hakem Değerlendirmesi: Dış Bağımsız.
Yazar Katkıları: Fikir - T.D., M.E.; Tasarım - T.D., Ş.B.; Denetleme - Ş.B.; Kaynaklar
- T.D., M.E.; Malzemeler - T.D., M.E.; Veri Toplanması ve/veya İşlemesi - T.D.; Analiz
ve/veya Yorum - T.D., Ş.B.; Literatür Taraması - T.D., Ş.B.; Yazıyı Yazan - T.D.; Eleştirel
İnceleme - Ş.B.
Teşekkür: Yazarlar, Çukurova Üniversitesi Güzel Sanatlar Fakültesi Grafik Bölümü
Öğretim Görevlisi Derviş İlker Gül’e Fasial sinir anatomisi çizimindeki katkılarından
dolayı teşekkür ederler.
Çıkar Çatışması: Yazarlar çıkar çatışması bildirmemişlerdir.
Finansal Destek: Yazarlar bu çalışma için finansal destek almadıklarını beyan et-
mişlerdir.
REFERENCES
1.	 Alfieri A, Strauss C, Prell J, Peschke E. History of the nervus intermedius of
Wrisberg. Ann Anat 2010; 192:139-144. [CrossRef]
2.	 Rhoton AL Jr, Kobayashi S, Hollinshead WH. Nervus intermedius. J Neurosurg
1968; 29:609-618. [CrossRef]
3.	 Headache Classification Committee of the International Headache Society
(IHS). The International Classification of Headache Disorders, 3rd edition
(beta version). Cephalalgia 2013; 33:629-808.
4.	 Tang IP, Freeman SR, Kontorinis G, Tang MY, Rutherford SA, King AT, Lloyd SK.
Geniculate neuralgia: A systematic review. J Laryngol Otol 2014; 128:394-399.
[CrossRef]
5.	 Rupa V, Saunders RL, Weider DJ. Geniculate neuralgia: the surgical manage-
ment of primary otalgia. J Neurosurg 1991; 75:505-511. [CrossRef]
6.	 Frese A, Lüttmann RJ, Husstedt IW, Ringelstein EB, Evers S. Geniculate neu-
ralgia as a manifestation of neuroborreliosis. Headache 2002; 42:826-828.
[CrossRef]
Arch Neuropsychiatry 2017; 54: 282-283	 Demir et al. Nervus Intermedius Neuralgia
283

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A very rare type of neuralgia

  • 1. Arch Neuropsychiatry 2017; 54: 282-283 • DOI: 10.5152/npa.2016.12654 A Very Rare Type of Neuralgia: Nervus Intermedius Neuralgia Çok Nadir Bir Nevralji: İntermedius Nevraljisi Turgay DEMİR, Miray ERDEM, Şebnem BIÇAKCI Department of Neurology, Çukurova University School of Medicine, Adana, Turkey Letter to the Editor / Editöre Mektup Cite this article as: Demir T, Erdem M, Bıçakcı Ş. A Very Rare Type of Neuralgia: Nervus Intermedius Neuralgiai. Arch Neuropsychiatry 2017; 54:282-283. Dear Editor, The nervus intermedius, which is the peripheral part of the facial nerve, has visceral motor and special sensory fibers. First described in 1563, the nerve was referred to as “portio media inter comunicantem faciei et nervum auditorium” by Heinrich August Wrisberg in 1777 (1). The word “intermedius” is used because of the intermediate position of the nerve between the superior part of the vestibular nerve and the facial nerve (2). The nervus intermedius enters the internal auditory meatus immediately after leaving the brainstem and travels with the facial nerve through the facial canal (Figure 1). According to the diagnostic criteria of the International Classification of Headache Disorders, 3rd edition (beta version), nervus intermedius (facial nerve) neuralgia (NIN) is a rare disorder characterized by brief paroxysms of pain felt deep in the auditory canal that sometimes radiates to the parieto-occipital region (3). A 55 year-old woman presented to a hospital with very severe burning sensation and stinging pain attacks that persisted for seconds and often occurred during the day in her right ear. From her past medical history, it was understood that she had diabetes mellitus, hypertension, and hyperlipidemia. In addition, she was involved in a car accident seven years ago, which resulted in loss of consciousness and bleeding in her right ear. Her neurological examina- tion and high-resolution magnetic resonance image of the brain and inner ear were normal. Hypoplasia of the anteri- or cerebral artery on the right side was seen during brain magnetic resonance angiography. Moreover, her temporal bone computed tomography image was normal. She had insignificant presbycusis bilaterally during her audiological evaluation. Thus, in line with existing evidence, she was di- agnosed with NIN, and following six months of treatment with 1200 mg/day of gabapentin, her pain attacks stopped. While the most common cause of cranial neuralgia is trigem- inal neuralgia, limited data are available on the incidence of NIN. However, NIN is very rare; the number of cases found between 1932 and 2012 was less than 150 (4). Other neuro- logical and otolaryngological etiologies should be excluded in patients with NIN, which is mostly seen in middle-aged wom- en. Therefore, it is essential to thoroughly examine the head, ear, nose, throat, face, and neck of patients. When there is no underlying etiology, it is referred to as classical NIN. Some secondary reasons have been reported: herpes zoster, tem- poromandibular joint dysfunction, nasopharyngeal carcino- ma, petrous bone osteoma, and neuroborreliosis (5,6). Depending on the patient’s condition, there are sever- al treatment options such as surgical treatment, physical Figure 1. Anatomy of the facial and intermedius nerve Correspondence Address/Yazışma Adresi: Turgay Demir, Çukurova Üniversitesi Tıp Fakültesi, Nöroloji Anabilim Dalı, Adana, Türkiye E-mail: drtdemir@gmail.com Received/Geliş Tarihi: 30.10.2015 Accepted/Kabul Tarihi: 29.02.2016 ©Copyright 2017 by Turkish Association of Neuropsychiatry - Available online at www.noropskiyatriarsivi.com ©Telif Hakkı 2017 Türk Nöropsikiyatri Derneği - Makale metnine www.noropskiyatriarsivi.com web sayfasından ulaşılabilir. 282
  • 2. therapy, analgesic treatment, and nerve block. While antiepileptic drugs such as carbamazepine, gabapentin, baclofen, or amitriptyline can be used for medical treatment, radiofrequency gangliolysis, retrogasserian glycer- ol injections, microvascular decompression surgery, and the gamma knife system are other treatment modalities. Nervus intermedius neuralgia should be considered as a differential diag- nosis if a patient presents with paroxysmal localized pain in the ears. Informed Consent: Written informed consent was obtained from patient who participated in this study. Peer-review: Externally peer-reviewed. Author Contributions: Concept - T.D., M.E.; Design - T.D., Ş.B.; Supervision Ş.B.; Resource - T.D., M.E.; Materials - T.D., M.E.; Data Collection and/or Processing - T.D.; Analysis and/or Interpretation - T.D., Ş.B.; Literature Search - T.D., M.E.; Writing - T.D.; Critical Reviews - Ş.B. Acknowledgements: The authors thank to Derviş İlker Gül, faculty member of the Faculty of Fine Arts, Çukurova University, for his contribution to the drawing of the facial nerve anatomy. Conflict of Interest: No conflict of interest was declared by the authors. Financial Disclosure: The authors declared that this study has received no fi- nancial support. Hasta Onamı: Yazılı hasta onamı bu çalışmaya katılan hastadan alınmıştır. Hakem Değerlendirmesi: Dış Bağımsız. Yazar Katkıları: Fikir - T.D., M.E.; Tasarım - T.D., Ş.B.; Denetleme - Ş.B.; Kaynaklar - T.D., M.E.; Malzemeler - T.D., M.E.; Veri Toplanması ve/veya İşlemesi - T.D.; Analiz ve/veya Yorum - T.D., Ş.B.; Literatür Taraması - T.D., Ş.B.; Yazıyı Yazan - T.D.; Eleştirel İnceleme - Ş.B. Teşekkür: Yazarlar, Çukurova Üniversitesi Güzel Sanatlar Fakültesi Grafik Bölümü Öğretim Görevlisi Derviş İlker Gül’e Fasial sinir anatomisi çizimindeki katkılarından dolayı teşekkür ederler. Çıkar Çatışması: Yazarlar çıkar çatışması bildirmemişlerdir. Finansal Destek: Yazarlar bu çalışma için finansal destek almadıklarını beyan et- mişlerdir. REFERENCES 1. Alfieri A, Strauss C, Prell J, Peschke E. History of the nervus intermedius of Wrisberg. Ann Anat 2010; 192:139-144. [CrossRef] 2. Rhoton AL Jr, Kobayashi S, Hollinshead WH. Nervus intermedius. J Neurosurg 1968; 29:609-618. [CrossRef] 3. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629-808. 4. Tang IP, Freeman SR, Kontorinis G, Tang MY, Rutherford SA, King AT, Lloyd SK. Geniculate neuralgia: A systematic review. J Laryngol Otol 2014; 128:394-399. [CrossRef] 5. Rupa V, Saunders RL, Weider DJ. Geniculate neuralgia: the surgical manage- ment of primary otalgia. J Neurosurg 1991; 75:505-511. [CrossRef] 6. Frese A, Lüttmann RJ, Husstedt IW, Ringelstein EB, Evers S. Geniculate neu- ralgia as a manifestation of neuroborreliosis. Headache 2002; 42:826-828. [CrossRef] Arch Neuropsychiatry 2017; 54: 282-283 Demir et al. Nervus Intermedius Neuralgia 283