A 37-year-old male presented in the clinic with 3-month history of recurrent seizures which always started with left leg numbness and shaking, and then progressed to generalized tonic-clonic seizures. Lumbar puncture revealed opening pressure of 350mmH2O. CSF leukocytes count increased to 38×10^6/L. ANA was positive at 1:1000 dilution. Anti-SSA, Ro-52 and CCP antibodies were positive. RF, IgG4 and Anti-ANCA antibody were negative. Next generation sequencing of CSF for pathogen was negative. Malignant cells were not detected.
Figure. Head MRI. A and B. Coronal and sagittal FLAIR shows thickness of the pachymeninx above right paracentral lobule (arrow). C and D. Coronal and sagittal post-contrast T1WI shows thickness and enhancement of the dura matter (arrow).
We described a rare case of pachymeningitis associated with Sjögren’s syndrome. Sjögren’s syndrome is an autoimmune disease characterized by dryness of the mouth and eyes, but systemic involvement is very common. Although prevalence of CNS involvement in Sjögren’s syndrome reaches 67.5%, pachymeningitis is extremely rare.[2-4]
The previously reported symptoms of pachymeningitis associated with Sjögren’s syndrome include fever, fatigue, headache, diplopia, periorbital and temporomandibular joint pain, hypopituitarism, and central diabetes insipidus.
Since similar cases are very rare, the radiological characteristics of Sjögren’s syndrome-related pachymeningitis can not be concluded. The previous case reports showed diffuse dural matter thickening and enhancement. However, our case shows more localized pachymeningitis.
Past history investigation: The patient was diagnosed with Sjögren’s syndrome 10 years ago when he suffered from Reynaud phenomenon. ANA and SSA/Ro-52 were positive then. Schirmer’s test and salivary gland biopsy suggested Sjögren’s syndrome. He had been taking prednisone 5mg/d, leflunomide 10/d and penicillamine 0.125/d for 10 years.
Treatment: The patient was started on 500mg methylprednisolone IV for 5 days and the dose was gradually tapered. The seizures suspended and a repeat MRI revealed resolution of the dural lesion.
Take-Home Message / Teaching Points
Pachymeningitis is a rare complication of Sjögren’s syndrome.
Due to its rarity, other causes of dural thickness should be excluded first.
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 Nakano Y, Yamamoto M, Komatsu K, Yagita M, Fujita M. Hypertrophic Pachymeningitis in Sjogren's Syndrome. Intern Med 2018; 57(3): 413-5.
 Heo Y, Kim T. Case report: hypertrophic pachymeningitis associated with Sjogren's syndrome. Neurol Sci 2019; 40(4): 879-82.
 Li JY, Lai PH, Lam HC, et al. Hypertrophic cranial pachymeningitis and lymphocytic hypophysitis in Sjogren's syndrome. Neurology 1999; 52(2): 420-3.
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