Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 29, 2019
EIGHTH NERVE NEUROPATHY SECONDARY TO SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
- Background
- Neuropsychiatric disease in systemic lupus erythematosus (NPSLE) occurs in 10-80% of the patients. The most common clinical manifestations include cognitive impairment, seizures, and cerebrovascular strokes.
- Cranial nerve involvement in SLE usually affects multiple nerves or is associated with other neurological deficits; the estimated prevalence varies from 2-7%. Isolated cranial neuropathy is very rare. The CN VIII is the most frequently affected while the oculomotor set (III, IV, VI), CN V and VII are less affected.
- Abnormal MRI findings range from 19-70% in patients with NPSLE.
- Clinical Presentation
- Hearing loss, tinnitus, and vertigo are the most frequently encountered clinical findings among patients with SLE. These symptoms occur in 25-67% of patients.
- Key Diagnostic Features
- Common brain MRI findings in NPSLE include small punctate hyperintense T2W focal lesions in subcortical and periventricular white matter, brain atrophy, and infarctions. However, these conditions are not specific to the disease and they can be asymptomatic.
- Most of the patients with SLE who experience these symptoms have normal MRI. Some case reports have described poor visualization of the semicircular canals on MRI. In this instance, our patient’s enhancing lesions have disappeared in correlation with the improvement of her clinical symptoms, suggesting NPSLE.
- Inflammatory cells and immune complex formed by cell-derived autoantigens and autoantibodies deposit in tissues such as the nerve causing the damage.
- Differential Diagnosis
- Vestibular Schwannoma: Most common vestibular nerve tumor. MRI shows a mass arising from the vestibular nerve, this lesion grows in the internal auditory canal and shows various patterns of enhancement depending on the size and the composition.
- Vestibular Neuritis: The vestibular nerve can be slightly enlarged and enhances with contrast. Vestibular neuritis is not associated with a mass like lesion.
- Neurosarcoidosis: MRI shows solitary or multiple lesions in the central nervous system (meningeal layer, subarachnoid space, brain parenchyma and spinal cord). It usually affects the optic nerve. MRI demonstrates hypointense lesions on T2WI with diffuse gadolinium enhancement.
- Granulomatosis with polyangiitis: Central nervous system involvement is uncommon, usually in the form of orbital masses with meningeal extension. It can produce mononeuritis multiplex and cranial neuropathy affecting the CN II, VI, and VII.
- Our patient was diagnosed with SLE (she met diagnostic criteria) and several of the previous diagnoses could be ruled out by her clinical information. The enhancing lesions disappeared with the improvement of her clinical symptoms after steroids, which is highly suggestive of NPSLE. This was a diagnosis of exclusion.
- Treatment
- Glucocorticoids are the treatment of choice. Cyclophosphamide, azathioprine, and mycophenolate mofetil are other options to be considered.
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