Multiple cranial nerve dysfunction caused by neurosarcoidosis




Abstract


Neurosarcoidosis is a rare identity and occurs in only 5% to 15% of patients with sarcoidosis. It can manifest in many different ways, and therefore, diagnosis may be complicated. We report a case presented in a very unusual manner with involvement of 3 cranial nerves; anosmia (NI), facial palsy (NVII), and hearing loss (NVIII). When cranial nerve dysfunction occurs, it is very important to take neurosarcoidosis into consideration.



Introduction


Sarcoidosis is a multisystem disorder of unknown etiology. Despite its predilection for the lungs, sarcoidosis can target every organ. However, neurosarcoidosis is rare and occurs in only 5% to 15% of patients .


Neurosarcoidosis can manifest in many different ways, and any part of the nervous system can be affected . Therefore, the diagnosis may be complicated. In neurosarcoidosis, the cranial nerves are commonly involved, being affected in as many as 75% of those patients. In most patients, more than 1 cranial nerve is involved .


Because of its infrequency, there is a paucity in published articles . Here, a case of cranial nerve dysfunction caused by sarcoidosis is described, presenting very unusual involvement of 3 cranial nerves; anosmia (NI), facial palsy (NVII), and hearing loss (NVIII).





Case report


A healthy 26-year-old woman developed a left-sided facial palsy a few days after a flu-like illness. She was treated with valacyclovir 1000 mg 3 times a day and prednisolone 25 mg 2 times a day for a week. However, a month hereafter, there was no improvement, and the patient was referred to our hospital. At that time, she also had hearing loss of the left ear, headache, and malaise.


Physical examination showed a facial nerve palsy on the left side, grade III (House-Brackmann grading system ), and a painful left parotic gland. Audiometry revealed a sensorineural hearing loss of approximately 40 to 50 dB on both sides ( Fig 1 A and B). Other cranial nerves functioned normally, and further neurologic examination was without abnormalities. Lues, human immunodeficiency syndrome, and Borrelia were tested, but serology proved negative. Angiotensin-converting enzyme level was slightly increased (89 IU/L; reference range, 7.0–21.4 IU/L), and the sedimentation rate of the erythrocytes was 25 mm/h. In addition, the chest x-ray showed evident hilar lymphadenopathy ( Fig 2 ). The result of cerebrospinal fluid examination was normal. Postcontrast magnetic resonance imaging (MRI) examinations of the brain demonstrated marked enhancement of the seventh left cranial nerve and both eighth cranial nerves ( Fig 3 A and B). The diagnosis of neurosarcoidosis was made. The patient was treated with methylprednisolone 1000 mg daily during the first 3 days. Hereafter, prednisolone 40 mg was given daily, and this was tapered during the next several months. There appeared to be no involvement of the eye and the heart. However, the patient complained of a rapidly progressive joint pain while on corticosteroid treatment. The diagnosis oligoarthritis caused by sarcoidosis was made.




Fig. 1


(A and B) The sensorineural hearing loss of approximately 40 to 50 dB at the right (A) and left (B) side at presentation. (C and D) The reconvalescence of hearing after treatment with high dose steroids for the right (C) and left ear (D).



Fig. 2


Chest x-ray demonstrating evident hilar lymphadenopathy.



Fig. 3


(A) An axial T1-weighted gadolinium-enhanced MRI image shows marked enhancement of the left ganglion geniculi of the seventh cranial nerve (arrow). (B) A coronal T1-weighted gadolinium-enhanced MRI image demonstrates marked enhancement of the seventh and eighth cranial nerve in the left acoustic canal.

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Aug 25, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on Multiple cranial nerve dysfunction caused by neurosarcoidosis

Full access? Get Clinical Tree

Get Clinical Tree app for offline access