Multiple Cranial Nerve Palsies as the First Presentation of Sarcoidosis

Rose, Oliver; Ahmad, Zahoor; Snow, Barry

doi:10.1155/2014/592510

Cited by 6 publications

(10 citation statements)

References 5 publications

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“…However, Bridel et al [2015] found CSF-ACE to have low specificity and sensitivity in diagnosing neurosarcoidosis, providing a cohort of 440 patients with suspected neurosarcoidosis and 9 with a confirmed diagnosis by histology. Despite the controversy regarding diagnosis, there seems to be consensus that any patient with multiple cranial neuropathies, whether they are simultaneous, or sequential as in our patients, should raise suspicion for neurosarcoidosis [Carlson et al, 2015;Loor et al, 2012;Rose et al, 2014].…”

Section: Differential Diagnosismentioning

confidence: 80%

“…There is no single test to diagnose neurosarcoidosis [Carlson et al, 2015], and even laboratory evidence of systemic sarcoidosis can suggest multiple etiologies, as serum ACE can be elevated in other disorders such as TB, multiple sclerosis, and Guillain-Barré syndrome [Rose et al, 2014]. In the study by Zajicek et al [1999], elevated CSF ACE was noted in 6 of the 18 patients tested, but the authors felt that an elevated CSF ACE was only helpful if it was raised out of proportion to serum ACE.…”

Section: Differential Diagnosismentioning

confidence: 98%

“…Neurosarcoidosis presented as leptomeningeal enhancement on T1-weighted postcontrast MRI in 67% of patients, or as distinct from multiple granulomas that mimicked neoplastic masses in 17% [Carlson et al, 2015]. In other case reports of neurosarcoidosis, the hearing loss was initially unilateral and then became bilateral [Loor et al, 2012;Rose et al, 2014]. Zajicek et al [1999] described the diagnostic criteria for neurosarcoidosis: "definite" which includes positive neural tissue histology, "probable" which includes evidence of CSF inflammation (increased protein or oligoclonal bands), MRI findings of neurosarcoidosis (white matter lesions, leptomeningeal enhancement, or CN enhancement) and evidence of systemic sarcoidosis (positive histology, elevated serum ACE, and at least 2 of the following: a positive Gallium scan, and chest imaging demonstrating bilateral hilar lymphadenopathy or serum ACE), and "possible" where evidence suggests neurosarcoidosis but the previous criteria are not met.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…methylprednisolone is used for a few days [Hoitsma et al, 2010;Jardine et al, 2015;Loor et al, 2012;Rose et al, 2014;Zajicek et al, 1999]. In many cases, patients experience stable, improved, or even complete resolution of disease although they may require long term corticosteroids [Carlson et al, 2015;Jardine et al, 2015;Loor et al, 2012].…”

Section: Etiology/pathophysiologymentioning

confidence: 99%

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The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

Greene

Naumann²,

Poulik³

et al. 2017

Audiol Neurotol

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Background: A rare subset of sarcoidosis, neurosarcoidosis, is reported to occur in 5-7% of sarcoid patients and can manifest in a variety of ways. The most common are facial paralysis and optic neuritis, less commonly causing cochleovestibulopathy, blindness, anosmia, and other cranial nerve (CN) palsies. The sensory deficit may be severe and psychiatric symptoms may result from the effects of the disease or steroid treatment. Although MRI-compatible cochlear implants are now available, concerns about the feasibility of recoverable hearing with cochlear implantation in these patients as well as the practical difficulty of disease monitoring due to implant artifact must be considered. Results: We present 3 recent cases from different institutions. The first is a 39-year-old man with a history of progressively worsening hearing loss, followed by visual loss, delusions, agitation, ataxia, and musical auditory hallucinations, diffuse leptomeningeal enhancement on MRI with a normal serum angiotensin-converting enzyme (ACE) level but elevated cerebrospinal fluid (CSF) ACE levels, suggesting neurosarcoidosis, was treated with corticosteroids, and underwent successful cochlear implantation. The second is a 36-year-old woman with rapid-onset horizontal diplopia, left mixed severe sensorineural hearing loss (SNHL) and tinnitus, diffuse leptomeningeal enhancement on MRI, and progressive palsy of the left CNs IV, VI, VII, IX, X and XI, with altered mental status requiring admission following high-dose intravenous corticosteroids. The third is a 15-year-old boy who presented with sudden, bilateral, profound SNHL, recurrent headaches, and left facial weakness refractory to antivirals, ultimately diagnosed with neurosarcoidosis following an aborted cochlear implantation where diffuse inflammation was found, and histopathology revealed Schaumann bodies; he was treated with methotrexate and later underwent successful cochlear implantation. Conclusions: Neurosarcoidosis is an elusive diagnosis and can cause hearing loss and psychiatric symptoms. Cochlear implantation for patients with severe hearing loss should be considered once the diagnosis is confirmed, as it is possible to achieve a successful level of hearing. Psychiatric symptoms can manifest with the onset of neurosarcoidosis, result from CN deficits, or develop as a side effect from long-term, high-dose corticosteroids, and should be monitored carefully in patients with neurosarcoidosis.

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Section: Differential Diagnosismentioning

confidence: 80%

Section: Differential Diagnosismentioning

confidence: 98%

Section: Differential Diagnosismentioning

confidence: 99%

Section: Etiology/pathophysiologymentioning

confidence: 99%

See 2 more Smart Citations

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

Greene

Naumann²,

Poulik³

et al. 2017

Audiol Neurotol

View full text Add to dashboard Cite

show abstract

“…MR enhancement and thickening of cisternal portions of the cranial nerves denotes diseases like intrinsic tumors of the nerves, infiltrative malignancies, inflammatory and infectious diseases [ Table 1 ]. [ 2 3 4 5 6 7 8 ] As can be seen from Table 1 , when the nerves are significantly thickened, neoplasms (Primary/metastatic) dominate the etiologies followed by conditions like sarcoidosis, CIDP, and at times chronic infections like syphilis, schistosomiasis, chronic meningitis.…”

Section: Discussionmentioning

confidence: 99%