Hughes syndrome and Multiple sclerosis

Uthman, Imad; Noureldine, Mohammad Hassan A.; Berjawi, Ahmad; Skaf, Marie; Haydar, Ali A.; Merashli, Mira; Hughes, G. R. V.

doi:10.1177/0961203314555539

Cited by 20 publications

(25 citation statements)

References 76 publications

(200 reference statements)

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“…However, it should be noted that up to 60% of NPSLE patients may have oligoclonal bands in their CSF, and evidence suggesting demyelination on imaging is not rare [5]. On the other hand, autoantibodies, such as aPL antibodies, may be detected in patients with pure MS [47]. …”

Section: Reviewmentioning

confidence: 99%

Neuropsychiatric lupus: a mosaic of clinical presentations

Kivity

Agmon‐Levin

Zandman‐Goddard

et al. 2015

BMC Med

162

141

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Neuropsychiatric symptoms affect nearly half of the patients with systemic lupus erythematosus; however, the effect on disease severity, quality of life, and prognosis is tremendous. Symptoms of neuropsychiatric systemic lupus erythematosus may range from mild diffuse ones, to acute life threatening events. Although the underlying mechanisms are still largely unraveled, several pathogenic pathways are identified, such as antibody-mediated neurotoxicity, vasculopathy due to anti-phospholipid antibodies and other mechanisms, and cytokine-induced neurotoxicity. In the current review, we describe the old and the new regarding epidemiology, pathophysiology, diagnosis, and management of neuropsychiatric systemic lupus erythematosus. The possible link between neuropsychiatric symptoms and specific mechanisms may help to facilitate our understanding of the disease in the future, thus allowing for better treatment strategies.

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Section: Reviewmentioning

confidence: 99%

Neuropsychiatric lupus: a mosaic of clinical presentations

Kivity

Agmon‐Levin

Zandman‐Goddard

et al. 2015

BMC Med

162

141

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“…Several possible pathophysiological mechanisms could be raised, such as premature atherosclerosis, thrombosis, vasculopathy, emboli, dissection of the carotid artery, and antiphospholipid syndrome [1, 2, 4, 6, 7, 8, 9, 10]. There would have been atherosclerosis and thrombosis process following the chronic inflammation, and rarely, these pathological changes can result in ICA dissection [1, 8, 9].…”

Section: Discussionmentioning

confidence: 99%

“…The absence of significant pain in her neck or head does not exclude the possibility of an ICA dissection [9]. There was no evidence to support a diagnosis of antiphospholipid syndrome, which could potentially cause arterial stenosis or occlusion [4, 6, 10]. A similar previously reported case of SLE with ICA occlusion presented with positive antiphosphatidylserine/prothrombin antibodies [6]; however, we could not evaluate these antibodies and cannot rule out their presence in the present case [10].…”

Section: Discussionmentioning

confidence: 99%

“…One possibility is that the ICA occlusion could have caused an artery-to-artery embolism or a hemodynamic hypoperfusion with an acute cerebral infarction, but there was no evidence of diffusion-weighted imaging hyperintense lesions to support this hypothesis. Alternatively, local cerebral microangiopathy or demyelination of the brain under SLE pathology may be the cause of the white matter lesions [1, 4]. …”

Section: Discussionmentioning

confidence: 99%

“…1a, b). So, according to the 2010 McDonald criteria [4], it was difficult to make a diagnosis of MS.…”

Section: Case Presentationmentioning

confidence: 99%

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Internal Carotid Artery Occlusion in Systemic Lupus Erythematosus as a Potential Mimicker of Multiple Sclerosis

et al. 2017

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The diagnosis of neurological symptoms in patients with systemic lupus erythematosus (SLE) is often challenging, in part because they sometimes mimic features of multiple sclerosis (MS). Herein we report a case of a young female who presented with relapsing-remitting symptoms of unilateral visual loss and motor aphasia. Additionally, radiological findings revealed multiple white matter lesions on her brain that led to an initial diagnosis of MS based on the established diagnostic criteria. However, she was eventually diagnosed with neuropsychiatric SLE (NPSLE) presenting with extracranial internal carotid artery (ICA) occlusion. Her ICA occlusion had not been detected for 2 months until she underwent magnetic resonance angiography. Although exact underlying pathological mechanisms are unclear, both the ICA occlusion and MS-like brain white matter lesions could be attributed to SLE. This case demonstrated that both of these lesions can coexist in the same patient, suggesting that NPSLE with ICA occlusion can be a potential mimicker of MS, and vice versa. Care must be paid to avoid delay in the diagnosis.

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Prevalence and adverse consequences of delayed diagnosis and misdiagnosis in thrombotic antiphospholipid syndrome. An observational cohort study and a review of the literature

et al. 2023

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Obiectives This study aims to prospectively evaluate the frequency and adverse consequences of diagnostic delay and misdiagnosis in a cohort of patients with thrombotic antiphospholipid syndrome (TAPS). In addition, a systematic review of the literature concerning the diagnostic delay and misdiagnosis of TAPS was carried out. Methods Patient enrollment occurred between 1999 and 2022. The study group was formed by TAPS patients whose diagnosis was delayed and those who were misdiagnosed. The control group was made up of patients who were timely and correctly diagnosed with TAPS. Results The literature review showed 42 misdiagnosed patients, 27 of them were in one retrospective cohort study and 15 in 13 case reports. One hundred sixty-one out of 189 patients (85.2%) received a timely, correct diagnosis of TAPS; 28 (14.8%) did not. The number of patients with diagnostic issues was significantly higher for the first period (1999–2010), and the number of patients with a correct diagnosis was significantly higher for the second one (2011–2022). When the clinical and laboratory characteristics of the patients with delayed diagnosis were compared with those with misdiagnosis, there was a significantly higher number of severe adverse consequences characterized by permanent disability or death in the latter group. The two most common types of misdiagnoses were systemic lupus erythematosus (6 cases, 46.1%) and cardiovascular diseases (4 cases, 30.8%). Conclusions The study demonstrates that although knowledge about TAPS has improved over time, diagnostic delays and errors remains to be addressed as they are strongly associated to adverse consequences. Key Points•Although knowledge of thrombotic antiphospholipid syndrome has improved over time, it is still limited.•Diagnostic delay and misdiagnosis are still an important issue that remains to be addressed as they are strongly associated to adverse consequences.•The three more frequent misdiagnoses are multiple sclerosis, systemic lupus erythematosus and cardiovascular diseases.

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Hughes syndrome and Multiple sclerosis

Cited by 20 publications

References 76 publications

Neuropsychiatric lupus: a mosaic of clinical presentations

Neuropsychiatric lupus: a mosaic of clinical presentations

Internal Carotid Artery Occlusion in Systemic Lupus Erythematosus as a Potential Mimicker of Multiple Sclerosis

Prevalence and adverse consequences of delayed diagnosis and misdiagnosis in thrombotic antiphospholipid syndrome. An observational cohort study and a review of the literature

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