Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases

Sato, Satoshi; Nakajima, Junya; Shimura, Masaru; Kawashima, Hisashi; Yoshio, Taku; Hara, Yuko

doi:10.1111/1756-185x.12235

Cited by 14 publications

(4 citation statements)

References 18 publications

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“…Strikingly, cerebral vasculitis was reported in 9.7% of the PULSE cohort. This rate is high, as cerebral vasculitis is considered a rare entity in both children and adults with SLE [54–56]. Our study was not designed to determine the cause of this high rate in our cohort, but further research is required to investigate and explain this interesting finding.…”

Section: Discussionmentioning

confidence: 92%

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

Harrison¹,

Zühlke²,

Lewandowski³

et al. 2019

Pediatr Rheumatol

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BackgroundPediatric onset of systemic lupus erythematosus (SLE) is associated with major organ involvement, and African patients tend to develop more aggressive disease than patients of European descent. Although cardiovascular involvement is common in pediatric SLE, there are few published reports on the subject. This study describes the frequency and characteristics of cardiac and vascular manifestations of pediatric SLE in a multi-ethnic South African cohort.MethodsDemographic, clinical, and echocardiographic data were collected from pediatric SLE patients at two centers in Cape Town, South Africa. At the time of investigation, this cohort consisted of 93 participants diagnosed with SLE according to international classification criteria prior to the age of 19. Individuals with cardiac and/or vascular involvement were identified by retrospective chart review. Cardiac manifestations were defined as presence of pericardial effusion, myocarditis, cardiomyopathy, cardiac failure, Libman-Sacks endocarditis, myocardial infarction, and arrhythmia. Vascular manifestations included deep vein thrombosis, pulmonary embolism, sinus thrombosis, stroke, critical limb ischemia, cerebral vasculitis and systemic vasculitis. Statistical analysis was performed using R (v3.4.1).ResultsCardiac and vascular involvement was present in 47% of the cohort. Previous studies have reported prevalence of 5%—50%. Demographic features of those with cardiac/vascular involvement did not differ from the overall cohort. Echocardiographic data were available for 23 participants. The most common cardiac manifestations were pericardial effusion (n = 24) and cardiac failure (n = 8), while the most common vascular manifestations were cerebral vasculitis (n = 9), stroke (n = 7), and pulmonary embolism (n = 7). Cardiovascular manifestations were frequently severe; one third of pericardial effusion cases required intervention, including three cases of cardiac tamponade. Cardiac and vascular involvement conferred an increased risk of mortality (31.1% versus 10.4%).ConclusionsCardiac and vascular involvement were highly prevalent in this South African cohort. The mortality rate was high, and severe manifestations were frequent. Prospective research is needed to improve knowledge of pediatric SLE in Africa and to improve outcomes for this high-risk population.

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Section: Discussionmentioning

confidence: 92%

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

Harrison¹,

Zühlke²,

Lewandowski³

et al. 2019

Pediatr Rheumatol

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“…It has been suggested that these findings may represent vasogenic edema and vascular changes occurring due to a vasculitic process localized in the basal ganglia probably due to immune-mediated underlying pathogenesis or effect of inflammation. Moreover, these MRI findings have been described to be reversible after starting immunosuppressive therapy (30). SLE-associated vasculitis may be associated with the deposition of immune complexes (ICs) in the endothelium.…”

Section: Discussionmentioning

confidence: 99%

C1q Deficiency and Neuropsychiatric Systemic Lupus Erythematosus

et al. 2016

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C1q deficiency is a rare immunodeficiency, which is strongly associated with the development of systemic lupus erythematosus (SLE). A mutation in one of the C1q genes can either lead to complete deficiency or to low C1q levels with C1q polypeptide in the form of low-molecular weight (LMW) C1q. Patients with C1q deficiency mainly present with cutaneous and renal involvement. Although less frequent, neuropsychiatric (NP) involvement has also been reported in 20% of the C1q-deficient patients. This involvement appears to be absent in other deficiencies of early components of the complement classical pathway (CP) (C1r/C1s, C2, or C4 deficiencies). We describe a new case with C1q deficiency with a homozygous G34R mutation in C1qC-producing LMW-C1q presenting with a severe SLE flare with NP involvement. The serum of this patient contained very low levels of a LMW variant of C1q polypeptides. Cell lysates contained the three chains of C1q, but no intact C1q was detected, consistent with the hypothesis of the existence of a LMW-C1q. Furthermore, we provide a literature overview of NP-SLE in C1q deficiency and hypothesize about the potential role of C1q in the pathogenesis of NP involvement in these patients. The onset of NP-SLE in C1q-deficient individuals is more severe when compared with complement competent NP-SLE patients. An important number of cases present with seizures and the most frequent findings in neuroimaging are changes in basal ganglia and cerebral vasculitis. A defective CP, because of non-functional C1q, does not protect against NP involvement in SLE. The absence of C1q and, subsequently, some of its biological functions may be associated with more severe NP-SLE.

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“…In a few cases of NPSLE, brain MRI revealed a reversal of abnormal findings in the bilateral basal ganglia through diffusion weighted imaging, and tiny hyperintensities in frontal periventricular white matter through T2WI and FLAIR images. 14,15 However, in many patients with obvious CNS manifestations, MRI may not show abnormalities, especially for affective disorders, confusional states, and headache. More than 40% of SLE patients with various neuropsychiatric manifestations have MRI scans that show no findings.…”

Section: Discussionmentioning

confidence: 99%

Reversible Amygdala and Parahippocampal Lesions of Brain ¹⁸Fluorodeoxy Glucose-Positron Emission Tomography in Neuropsychiatric Systemic Lupus Erythematosus

Yoon

Park

Lee

et al. 2015

Dement Neurocognitive Disord

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Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases

Abstract: The reported cases indicate that reversible vasculopathies represent vasogenic edema localized in basal ganglia lesions and that activation of the autoimmune system and inflammation could lead to NP manifestations in SLE.

Cited by 14 publications

References 18 publications

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

C1q Deficiency and Neuropsychiatric Systemic Lupus Erythematosus

Reversible Amygdala and Parahippocampal Lesions of Brain ¹⁸Fluorodeoxy Glucose-Positron Emission Tomography in Neuropsychiatric Systemic Lupus Erythematosus

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Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases

Abstract: The reported cases indicate that reversible vasculopathies represent vasogenic edema localized in basal ganglia lesions and that activation of the autoimmune system and inflammation could lead to NP manifestations in SLE.

Cited by 14 publications

References 18 publications

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

C1q Deficiency and Neuropsychiatric Systemic Lupus Erythematosus

Reversible Amygdala and Parahippocampal Lesions of Brain 18Fluorodeoxy Glucose-Positron Emission Tomography in Neuropsychiatric Systemic Lupus Erythematosus

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Reversible Amygdala and Parahippocampal Lesions of Brain ¹⁸Fluorodeoxy Glucose-Positron Emission Tomography in Neuropsychiatric Systemic Lupus Erythematosus