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Cranial neuropathy in neurosarcoidosis. (Imaging Clinic).


Sarcoidosis Sarcoidosis Definition

Sarcoidosis is a disease which can affect many organs within the body. It causes the development of granulomas. Granulomas are masses resembling little tumors. They are made up of clumps of cells from the immune system.
 is a multisystem granulomatous disease. Its neurologic manifestations usually occur as a part of the spectrum of the systemic disorder. Cranial neuropathy is a common manifestation of neurosarcoidosis, occurring in as many as 75% of patients. (1,2) Most patients have more than one cranial neuropathy. The cranial nerve most often involved is the facial nerve. The VIIIth cranial nerve is involved in 10 to 20% of cases. (2'3 )

The case presented here is that of a 44-year-old black man with mild bilateral sensorineural hearing loss Sensorineural hearing loss
Hearing loss caused by damage to the nerves or parts of the inner ear governing the sense of hearing.

Mentioned in: Tinnitus

sensorineural hearing loss 
 who had been monitored for several years. Coronal-section (figure 1) and axial-section (figure 2) magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures.  (MM) examinations of the brain had revealed symmetrical enhancing lesions that involved the internal acoustic canals and the trigeminal nerves. These findings suggested the presence of schwannomas and led to a presumptive diagnosis of neurofibromatosis type II. The patient also developed progressively worsening low back pain and experienced difficulty walking secondary to myelopathy myelopathy /my·elop·a·thy/ (mi?e-lop´ah-the)
1. any functional disturbance and/or pathological change in the spinal cord; often used to denote nonspecific lesions, as opposed to myelitis.

2.
 and peripheral neuropathy. Further evaluation of the spine by MRI revealed the presence of a space-occupying lesion at the level of the conus medullaris and involvement of the leptomeninges leptomeninges /lep·to·me·nin·ges/ (lep?to-me-nin´jez) sing. leptome´ninx   the pia mater and arachnoid taken together; the pia-arachnoid.leptomenin´geal

lep·to·me·nin·ges
n.
 in the thoracic and cervical areas. Biopsy analysis identified a granulomatous granulomatous /gran·u·lom·a·tous/ (-lom´ah-tus) containing granulomas.
Granulomatous
Resembling a tumor made of granular material.
 process that was consistent with sarcoidosis involving the distal spinal cord and leptomeninges.

Following surgical treatment of the spine, the patient recovered almost completely. Further treatment with corticosteroids was prescribed. On follow-up MRI examinations, a decrease in the enhancement of the cranial nerve lesions was observed. However, the patient experienced no significant alleviation of his sensorineural hearing loss.

References

(1.) Gullapalli D, Phillips LH II. Neurologic manifestations of sarcoidosis. Neurol Clin 2002;20:59-83.

(2.) Delaney P. Neurologic manifestations in sarcoidosis. Review of the literature, with a report of 23 cases. Ann Intern Med 1977;87:336-45.

(3.) Zouaoui A, Maillard JC, Dormont D, et al. [MRI in neurosarcoidosis]. J Neuroradiol 1992;19:271-84.

From the Department of Radiology (Dr. Palacios) and the Department of Otorhinolaryngology otorhinolaryngology /oto·rhi·no·lar·yn·gol·o·gy/ (-ri?no-lar?ing-gol´ah-je) the branch of medicine dealing with the ear, nose, and throat.

o·to·rhi·no·lar·yn·gol·o·gy
n.
 (Dr. Rigby and Dr. Smith), Lousiana State University Health Sciences Center, New Orleans.
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Article Details
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Author:Smith, Douglas L.
Publication:Ear, Nose and Throat Journal
Geographic Code:1USA
Date:Apr 1, 2003
Words:336
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