CASE 17020 Published on 29.10.2020

Focal pachymeningitis in association with Sjögren’s syndrome

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Yue Zhang, MD, PhD

Department of Neurology, Huashan Hospital, Fudan University, No.12 Wulumuqi Road, Jing’an District. Shanghai, China

Patient

37 years, male

Categories
Area of Interest CNS ; Imaging Technique MR
Clinical History

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.

Imaging Findings

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).

Discussion

Background

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%,[1] pachymeningitis is extremely rare.[2-4]

Clinical Perspective

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.

Imaging Perspective

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.

Outcome

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.

Differential Diagnosis List
Sjögren’s syndrome related pachymeningitis
Meningeal lymphoma
Meningeal metastasis
Meningioma
Idiopathic hypertrophic pachymeningitis
IgG4 related hypertrophic pachymeningitis
ANCA related hypertrophic pachymeningitis
Final Diagnosis
Sjögren’s syndrome related pachymeningitis
Case information
URL: https://www.eurorad.org/case/17020
ISSN: 1563-4086
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