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Case Reports
. 2009 Apr;12(2):124-6.
doi: 10.4103/0972-2327.53083.

Primary Sjögren's syndrome manifesting as multiple cranial neuropathies: MRI findings

V V Ashraf  1 , Ramesh Bhasi, R Praveen Kumar, A S Girija
Affiliations
Case Reports

Primary Sjögren's syndrome manifesting as multiple cranial neuropathies: MRI findings

V V Ashraf et al. Ann Indian Acad Neurol. 2009 Apr.

Abstract

We report a case of primary Sjögren's syndrome presenting with multiple cranial nerve palsies and radiological evidence of cranial pachymeningitis and hypophysitis. A 47-year-old woman developed right sensory neural hearing loss followed, 2 months later, by right facial palsy. Cranial magnetic resonance imaging showed features of pachymeningitis and pituitary gland infiltration. The diagnosis of primary Sjögren's syndrome was confirmed by demonstrating positive SS-A and SS-B antibodies and histological evidence of lymphocytic infiltration of the sublabial salivary gland. During the 2-year follow-up, the patient had transient VI(th), IX(th), X(th), and XII(th) cranial nerve palsies. Sjögren's syndrome should be considered in the differential diagnosis of patients presenting with multiple recurrent cranial nerve palsies, even if prominent sicca symptoms are absent.

Keywords: Cranial pachymeningitis; multiple cranial nerve palsies; primary Sjögren syndrome.

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Conflict of interest statement

Conflict of Interest: Nil

Figures

Figure 1
Figure 1
(a) Sagittal gadolinium-enhanced T1-weighted MR images showing thickening and enhancement of cerebellar tentorium (short thick arrow). It also shows a mildly enlarged pituitary gland. (b) Coronal gadolinium-enhanced T1-weighted MR images showing the enlarged and enhancing pituitary gland. (c) Sagittal T1-weighted MR image (post contrast) showing reduction in size of pituitary gland swelling and dural thickening following immunotherapy. (d) Histopathology section of lower lip biopsy showing diffuse infiltration of lymphocytes and plasma cells
See this image and copyright information in PMC

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