Acute motor-sensory axonal neuropathy associated with systemic lupus erythematosus

Thakolwiboon, Smathorn; Sohn, Gyeongmo

doi:10.1080/08998280.2019.1647715

Cited by 7 publications

(7 citation statements)

References 20 publications

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“…This case report highlights the complexity of treating concurrent autoimmune diseases in the case of ongoing pregnancy. Even though the main therapeutic options for GBS are either IVIG or plasma exchange, 24,25,[35][36][37] in our patient, IVIG in combination with corticosteroids did not result in neurological improvement. Termination of pregnancy and the addition of TPE and cyclophosphamide led to eventual recovery of the patient, both from GBS and SLE nephritis.…”

Section: Discussioncontrasting

confidence: 62%

Therapeutic plasma exchange in a patient with acute motor axonal neuropathy subtype of Guillain‐Barre syndrome and systemic lupus erythematosus

Akgun

Langlie

Huberman

et al. 2022

J of Clinical Apheresis

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A young female in her early 20s with a history of systemic lupus erythematosus presented to the emergency department due to 4 days of progressive bilateral extremity weakness and numbness. The patient reported flu-like symptoms that had spontaneously recovered 2 weeks prior to her presentation. She was 10 weeks pregnant at presentation. Lumbar puncture study and electrical muscle stimulation (EMS) were consistent with acute motor axonal neuropathy subtype of Guillain-Barre syndrome (GBS). Patient also had increased proteinuria and renal biopsy performed that was consistent with lupus nephritis.Despite treatment with pulse dose corticosteroids and IVIG, the patient had minimal neurological improvement and with continued decline required intubation. Her pregnancy was terminated at this point and a course of therapeutic plasma exchange (TPE) was started. Patient was also treated with cyclophosphamide. The patient responded to the combination of therapy and had slow but gradual neurologic recovery as well as improvement of proteinuria. Here we describe a case of an acute motor axonal neuropathy (AMAN) subtype of GBS in a young woman with active SLE and current pregnancy at the time of the presentation. Concurrent GBS and active SLE in the setting of pregnancy may be more treatment resistant, and combination therapy including TPE, immunosuppression, and termination of pregnancy may be indicated.

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Section: Discussioncontrasting

confidence: 62%

Therapeutic plasma exchange in a patient with acute motor axonal neuropathy subtype of Guillain‐Barre syndrome and systemic lupus erythematosus

Akgun

Langlie

Huberman

et al. 2022

J of Clinical Apheresis

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“…Current treatment recommendations for pediatric (non-SLE) GBS emphasize the use of IVIG over PLE ( 39 , 40 ). However, this approach appears to be inadequate for patients with severe SLE-GBS as inferred from available experience in children and adults ( 5 , 7 , 16 – 18 , 27 , 29 , 37 , 38 ), including our patient.…”

Section: Discussionmentioning

confidence: 84%

“…There are no published clinical trials or contemporary therapeutic guidelines for patients with SLE-GBS. Anecdotally, adult patient were treated with high-dose glucocorticoids, IV or oral cyclophosphamide, PLEX, IVIg, and mycophenolate mofetil, in various combinations ( 6 , 7 , 27 , 29 , 37 , 38 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl

et al. 2022

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Peripheral nervous system involvement accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. Guillain-Barré syndrome (GBS) as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. A 14-year-old, previously healthy female teenager developed classic features of GBS with ascending bilateral muscle weakness leading to respiratory insufficiency, associated with protein-cell dissociation in cerebro-spinal fluid, nerve root enhancement by MRI and reduction in compound muscle action potential amplitude. SLE was diagnosed serologically and histologically (lupus nephritis WHO class II). Despite immediate treatment with intravenous immunoglobulin (IVIg), methylprednisolone pulses and subsequently, rituximab, the patient required prolonged mechanical ventilation. She achieved full recovery following 14 PLEX treatments and two more rituximab infusions. Anti-dsDNA, C3, C4 and urinalysis normalized while anti-Smith and Sjögren antibodies persisted 15 months after disease onset, with no other lupus manifestations. Review of the literature revealed two pediatric cases of GBS at the onset of SLE and a third case with GBS 6 years after the diagnosis of SLE. Conventional GBS therapy may not be adequate to treat SLE-GBS. SLE should be included in the differential diagnosis of GBS. Importantly, treatment experiences and outcomes of such cases need be reported to inform future treatment recommendations.

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“…Acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN) are two variants of GBS rarely reported in SARDs, notably in LES (Santiago-Casas et al, 2013;Thakolwiboon et al, 2019). Acute motor axonal neuropathy presents as acute ascending symmetrical quadriparesis with occasional preservation of deep tendon reflexes, whereas AMSAN is a more severe form of AMAN, with superimposed sensory symptoms.…”

Section: Clinical Patterns Of Immune Axonal Neuropathy In Systemic Autoimmune Rheumatic Diseasesmentioning

confidence: 99%

“…Neuropathy is rarely the inaugural manifestation in SLE and usually occurs in the advanced stages of the disease (Ropper et al, 2014) and correlates with disease activity (Mizisin and Weerasuriya, 2011). Interestingly, AMAN and AMSAN have been reported as initial manifestations of SLE in 4 cases and preceding the diagnosis of SLE in 2 cases (Thakolwiboon et al, 2019).…”

Section: Types Of Immune Axonal Neuropathy In Systemic Autoimmune Rheumatic Diseasesmentioning

confidence: 99%

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases

et al. 2021

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Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.

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Acute motor-sensory axonal neuropathy associated with systemic lupus erythematosus

Cited by 7 publications

References 20 publications

Therapeutic plasma exchange in a patient with acute motor axonal neuropathy subtype of Guillain‐Barre syndrome and systemic lupus erythematosus

Therapeutic plasma exchange in a patient with acute motor axonal neuropathy subtype of Guillain‐Barre syndrome and systemic lupus erythematosus

Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases

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