Acute inflammatory demyelinating polyneuropathy as the initial presentation of lupus

Hsu, Tai-Yi; Wang, Shih-Hao; Kuo, Chang‐Fu; Chiu, Te-Fa; Chang, Yu‐Che

doi:10.1016/j.ajem.2008.11.006

Cited by 31 publications

(20 citation statements)

References 14 publications

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“…Guillain-Barré syndrome as the initial presentation of SLE has been reported in only a few cases. [ 3 , 4 ]. Some were treated with plasmapheresis and steroids with full recovery [ 3 ] or steroids, plasmapheresis, and cyclophosphamide with partial recovery [ 4 ] or had full recovery to only cyclophosphamide and steroids after failed initial treatment with IVIG and plasmapheresis [ 5 ] or had full recovery with cyclophosphamide and steroids after failed treatment with IVIG [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

Okoh

Lubana

Langevin

et al. 2015

Case Reports in Rheumatology

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Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

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Section: Discussionmentioning

confidence: 99%

A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

Okoh

Lubana

Langevin

et al. 2015

Case Reports in Rheumatology

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“…Review of the literature reveals that cases of GBS in the context of SLE have predominantly presented with acute inflammatory demyelinating polyneuropathy followed by the acute motor axonal neuropathy variant 6–19. Rare cases of acute motor sensory axonal neuropathy,14 20 Miller-Fisher syndrome21 and pharyngeal-cervical-brachial syndrome22 have also been reported.…”

Section: Discussionmentioning

confidence: 99%

Fulminant Guillain-Barré syndrome in a patient with systemic lupus erythematosus

Coomes¹,

Haghbayan

Spring

et al. 2019

BMJ Case Rep

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A 45-year-old man with a history of systemic lupus erythematosus presented with progressive weakness and areflexia. Electromyogram revealed reduced motor and sensory amplitudes without demyelinating features. He was clinically diagnosed with the acute motor and sensory axonal neuropathy variant of Guillain-Barré syndrome. Despite intravenous immunoglobulin therapy, he deteriorated with loss of all voluntary motor function and cranial nerve reflexes. Concomitant investigations revealed class V lupus nephritis. Therapy was initiated with plasma exchange, glucocorticoids and further immunosuppression, with gradual neurological recovery. We present the first documented case of fulminant Guillain-Barré syndrome as a neuropsychiatric manifestation of systemic lupus erythematosus, highlighting how immune-mediated polyneuropathy via diffuse deafferentation may mimic the outward appearance of brain death. While glucocorticoids are not indicated in idiopathic Guillain-Barré, when this neurological disorder is a consequence of systemic lupus erythematosus, immunomodulatory treatment should be initiated to prevent neurological deterioration.

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“…[3,4] Common polyneuropathy presentation in SLE includes multiple mononeuritis, acute inflammatory demyelinating polyneuropathy and chronic inflammatory demyelinating polyneuropathy [2]. Albeit rare, Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is the commonest neurological entity associated with SLE and is being increasingly recognized [5,6]. CIDP is a chronic, acquired, immune-mediated condition affecting the peripheral nervous system and is characterized by progressive limb weakness, sensory loss and areflexia with a relapsing or progressive course [7,8].…”

Section: Introductionmentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyneuropathy in Systemic Lupus Erythematosus: A Rare Entity

Mohd¹,

Nordin²,

Cader³

2018

MEDJ

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Background: Neurological manifestations in Systemic Lupus Erythematous (SLE) varies and commonly affects the Central Nervous System (CNS) rather than the peripheral nervous system. Neuropsychiatric or CNS manifestation can be as high as 24-54%, whereas the peripheral nervous system involvement is lower around 5-27%. Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP) is one of the three commonest peripheral nervous system involvements in SLE patients and results with severe debilitating effects. However, it is rarely reported. Methods: A retrospective review of all SLE patients that were diagnosed with CIDP between 2000 and 2015 was done under follow up at our center that were diagnosed with CIDP between 2000 and 2015. We reviewed their medical records and analyzed their clinical presentation, investigations, treatment instituted, response to therapy and any neurological sequealae. Results: A total of 512 case notes were reviewed. Of these 4 patients presented with CIDP (3 females, 1 male) aged between 26 to 46 years old. Three presented with transverse myelitis and the other one with acute motor and sensory axonal neuropathy. All patients were treated with high dose corticosteroids, three patients received cyclophosphamide whilst the other patient was induced with mycophenolate mofetil. Complete recovery was seen in one patient, two had persistent but improving numbness and the other one had a residual weakness. Conclusion: Peripheral nervous system involvement in SLE can result in serious debilitating effects. Early diagnosis and treatment are crucial in limiting the neurological sequealae.

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Acute inflammatory demyelinating polyneuropathy as the initial presentation of lupus

Cited by 31 publications

References 14 publications

A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

Fulminant Guillain-Barré syndrome in a patient with systemic lupus erythematosus

Chronic Inflammatory Demyelinating Polyneuropathy in Systemic Lupus Erythematosus: A Rare Entity

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