Rare Case of Central Pontine Myelinolysis: Etiological Dilemma

Abstract

Central nervous system (CNS) involvement in Sjogren's syndrome (SS) has a broad spectrum of presentations. We present a 33-year-old with sudden onset, rapidly progressive quadriplegia, severe dysarthria, bilateral facial palsy, bulbar palsy, and hypernatremia. The MRI of the brain revealed hyperintensity in the central pons diffusion-weighted imaging, T2-weighted imaging, and fluid-attenuated inversion recovery (FLAIR) without abnormal contrast enhancement, consistent with central pontine myelinolysis. However, there was no antecedent history of hyponatremia with rapid correction. The patient responded excellently to sodium correction and pulse methylprednisolone therapy and was erroneously diagnosed as idiopathic hypernatremic osmotic demyelination. One year later, she presented with vague constitutional symptoms, renal tubular acidosis type-1 (distal), hypokalemia with associated myopathy. Subsequent testing for anti-Sjögren's-syndrome-related antigen A (SSA)/Ro autoantibodies and a biopsy of the minor salivary gland established the diagnosis of primary Sjogren syndrome (pSS). Remission was achieved with oral prednisolone after her discharge. Neurological signs can be the initial presentation that precedes the classical systemic manifestations of multisystem autoimmune disorders like pSS. In the event of osmotic demyelination, when antecedent hyponatremia with rapid correction is not there, we suggest evaluating for possible autoimmune etiology.

Primary Sjögren’s syndrome with renal tubular acidosis and central pontine myelinolysis: An unusual triad

“…The neurological manifestations include poly- or mononeuropathies. Less frequently, the central nervous system (CNS) may manifest with a wide array of neurological presentations (e.g., focal deficits, meningoencephalitis, encephalopathy, myelopathy, optic neuritis, brainstem lesions, seizures, and mood disorders) [ 8 ]. However, CPM is a rare manifestation.…”

“…Central pontine myelinolysis (CPM) is a rare, severe neurological manifestation of pSS. Several case reports have demonstrated pontine lesions, which are usually reversible [ 8 , 9 , 10 ].…”

Occurrence of osmotic demyelination syndrome in diabetes mellitus: A case report and literature review of various etiologies for osmotic demyelination syndrome

“…Furthermore, ODS has been found in autoimmune liver disease, 27 Sjogren’s syndrome, 28 and non-Hodgkin’s lymphoma. 29 The pathophysiology for ODS occurring in case of autoimmune diseases, that is, autoimmune hepatitis and Sjogren’s syndrome, has not been cleared but in case of non-Hodgkin lymphoma, it is supposed to be due to systemic and metabolic stress induced by non-Hodgkin lymphoma.…”

Psychosis Due to Osmotic (Pontine) Myelinolysis in Non-Hodgkin’s Lymphoma: Case Report

“…В дальнейшем, по мере дегидратации глиальных клеток, начинается процесс их апоптоза [18]. Также дополнительным компонентом демиелинизации является появление антител к миелиновой оболочке с ее аутоимунным поражением [19].…”