Combined Central and Peripheral Demyelination in a Case With Sjogren Syndrome

Abstract

Introduction: Combined central and peripheral demyelination (CCPD) is a rare entity in which central and peripheral nervous system demyelination coexist. Herein, we present a patient with coexistence of Sjögren syndrome (SS) and CCPD. Case Report: A 58-year-old female patient was admitted to our neurology clinic with paraparesis, difficulty walking, imbalance, and paresthesia. Neurological examination showed paraparesis, absence of lower extremity deep tendon reflex, sensory deficit at the T8 level, loss of deep sensory position, and vibration. Spinal magnetic resonance imaging revealed multiple focal T2-hyperintense and contrast-enhancing cord lesions. Fat-suppressed imaging disclosed T2 hyperintensity in lumbar nerve roots, diffuse linear enhancement of the cauda equina, and diffuse increased enhancement in lumbar nerve roots. Electrodiagnostic findings fulfilled the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. Extensive laboratory workup excluded all possible pathologies. The Schirmer test detected positive in both eyes and minor salivary gland biopsy resulted in grade 3. These results were consistent with SS. The patient received intravenous methylprednisolone, azathioprine hydroxychloroquine. Approximately 2 years later, her complaints had completely disappeared, except for mild sensory complaints. Conclusion: It is unclear whether the association of central nervous system and peripheral nervous system demyelination and SS is a coincidence or a consequence. Our patient shows that patients with SS can have CCPD, and a significant clinical response can be obtained with early treatment. We hope that this unique case sheds light on the pathophysiology of CCPD.

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“…At present, there is no unified diagnostic standard for CCPD. The diagnosis of CCPD should admit the following conditions: (1) there are manifestations of central nerve damage such as intracranial, spinal cord or optic nerve MRI T2 high signal or abnormal visual evoked potential; (2) At least two nerves of the median nerve, ulnar nerve or tibial nerve had evidence of demyelination of peripheral nervous systems, such as nerve conduction delay, conduction block and abnormal F-wave; (3) Except direct infection [syphilis, human immunodeficiency virus (HIV), human T cell virus infection, etc. ), systemic immune diseases (sarcoidosis, Behcet's disease, Sjogren's syndrome, vasculitis, connective tissue disease, etc.…”

“…Combined central and peripheral demyelination (CCPD) is a rare entity in which central and peripheral nervous system demyelination coexist. It also is an entity with heterogeneous immune pathogenesis and clinical characteristics, overlapping between multiple sclerosis (MS) and chronic inflammatory demyelinating polyneuropathy (CIDP) [1][2]. CCPD with NF -155 has been reported in the past, but CCPD with NF -186 is rare.…”