Hypokalemic Paralysis due to Primary Sjögren Syndrome: Case Report and Review of the Literature

Garza-Alpirez, Alejandro; Arana-Guajardo, Ana Cecilia; Esquivel‐Valerio, Jorge Antonio; Villarreal-Alarcón, Miguel Ángel; Galarza‐Delgado, Dionicio Ángel

doi:10.1155/2017/7509238

Cited by 16 publications

(11 citation statements)

References 42 publications

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“…Motor impairment due to neuropathy has not been widely recognized until now in patients with Sjögren's syndrome (3, 4). However, this severe complication of Sjögren's syndrome with fulminant development of limb weakness but favorable outcome after immunosuppressive therapy was described in several case reports (5–9). Since the prevalence of peripheral neuropathy associated with Sjögren's syndrome increases with age (10), underestimation of paralysis as a neurologic complication of Sjögren's syndrome is a great risk.…”

Section: Introductionmentioning

confidence: 99%

Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome

et al. 2019

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Objective: Sjögren's syndrome is a heterogeneous inflammatory disorder frequently involving peripheral nerves with a wide spectrum of sensory modalities and distribution patterns. The objective of this cross-sectional study was to determine characteristics of Sjögren's syndrome as a cause for severe neuropathy with limb weakness. Methods: One hundred and eighty four patients with polyneuropathy associated with limb weakness underwent routine diagnostics including investigations for Sjögren's syndrome. Forty-four patients with Sjögren's syndrome (ACR-EULAR classification criteria) and severe neuropathy were identified. Results: Sjögren's syndrome was found at a median age of 63 years and the gender distribution showed a balanced female-male ratio of 1:1. Anti-SSA(Ro) antibodies were detected in 48% while seronegative patients were diagnosed with Sjögren's syndrome based on sialadenitis on minor salivary gland biopsy with a focus score ≥1. The majority of patients (93%) were diagnosed with Sjögren's syndrome after neurological symptoms appeared. Limbs were symmetrically involved in 84% of patients (57% tetraparesis, 27% paraparesis). Sensory function was not affected in 11% of patients indicating that Sjögren's syndrome associated neuropathy can present as a pure motor syndrome. Electrophysiological measurements did not reveal pathognomonic findings (23% demyelinating pattern, 36% axonal pattern, 41% both demyelinating and axonal damage signs). More than half of our patients fulfilled the European Federation of Neurological Societies (EFNS) diagnostic criteria for CIDP indicating that distinction between Neuro-Sjögren and other causes of neuropathy such as CIDP is challenging. Interpretation: Our findings show that severe neuropathy with limb weakness is often associated with Sjögren's syndrome. This is of great importance in identifying and understanding the causes of immune mediated polyneuropathy.

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Section: Introductionmentioning

confidence: 99%

Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome

et al. 2019

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“…In PubMed Central ® (PMC), a free archive of biomedical and life sciences journal literature for example, we founded the case of a patient with a hypokalemia as a primary manifestation of Sjögren syndrome with a reference to similar cases. There were reported 52 cases of hypokalemic paralysis in Sjögren syndrome patients, and in some cases, distal renal tubular acidosis was present before the diagnosis of primary Sjögren syndrome (pSS) [7]. It must be emphasized that the underlying problem in distal renal tubular acidosis is a reduced excretion of H + ion in the distal part of the nephron.…”

Section: Resultsmentioning

confidence: 99%

Hypokaliemia in Primary Sjogren’s Syndrome

Nevenka¹,

Bulatović²,

Natasa³

et al. 2018

Rheumatology

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Sjögren syndrome (SS) is classified as an autoimmune disorder where the immune system primarily attacks the lacrimal and salivary glands impairing the glands ability to secrete fluids. Immune system may also attack other organs and tissues. Sjogren syndrome is classified as primary or secondary, depending on coexistance of other autoimmune disease. It affects predominantly middle-aged women, with a female to male ratio reaching 9:1 [1]. We present a 35 year old female patient with suspected diagnose of Sjögren's syndrome and flakcid quadriparesis caused by hypokaliemia in RTA type 1.

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“…There are case reports describing hypokalemic paralysis as a first presentation in SS. [ 7 8 9 10 11 ] Our patient had previously one episode of mild weakness of all the four limbs with hypokalemia and diagnosed as hypokalemic periodic paralysis and now presented with severe hypokalemic paralysis with RTA.…”

Section: Discussionmentioning

confidence: 96%

Hypokalemic paralysis as an initial presentation of Sjogren syndrome

et al. 2020

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Sjogren syndrome (SS) is a systemic autoimmune disorder with predominant exocrine gland involvement leading to sicca symptoms. Among extraglandular manifestations, renal disease is the most common. Tubular interstitial nephritis and renal tubular acidosis (RTA) are the common presentations. Mild hypokalemia associated with distal RTA is common in SS, however, severe hypokalemia causing paralysis is unusual. We report the case of a 26-year-old female who presented with hypokalemic paralysis. On evaluation, distal RTA was diagnosed. Further evaluation showed positive SS-a/SS-b antibodies in high titer, which confirms the diagnosis of primary SS. Our report illustrates that SS is a rare but important cause of hypokalemic paralysis.

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Hypokalemic Paralysis due to Primary Sjögren Syndrome: Case Report and Review of the Literature

Cited by 16 publications

References 42 publications

Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome

Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome

Hypokaliemia in Primary Sjogren’s Syndrome

Hypokalemic paralysis as an initial presentation of Sjogren syndrome

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