Brain MRI in patients with acute confusional state of diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus

2016 ◽  
Vol 27 (2) ◽  
pp. 278-283 ◽  
Author(s):  
Gakuro Abe ◽  
Hirotoshi Kikuchi ◽  
Yoshiyuki Arinuma ◽  
Shunsei Hirohata
2018 ◽  
Vol 5 (1) ◽  
pp. e000281 ◽  
Author(s):  
Yu Matsueda ◽  
Yoshiyuki Arinuma ◽  
Tatsuo Nagai ◽  
Shunsei Hirohata

ObjectiveRecent studies have demonstrated that autoantibodies directed against glucose-regulated protein 78 (GRP78) on endothelial cells promote blood–brain barrier (BBB) damages. The present study examined whether serum anti-GRP78 antibodies might be involved in the pathogenesis of neuropsychiatric SLE (NPSLE).MethodsSerum samples were obtained from 129 patients with SLE (58 patients with diffuse psychiatric/neuropsychological syndromes of NPSLE (diffuse NPSLE), 30 with neurological syndromes (focal NPSLE), 21 with lupus nephritis (LN), 20 without NPSLE or LN (SLE alone)), from 35 patients with non-SLE rheumatic diseases (non-SLE RD) and from 24 healthy controls (HC). Anti-GRP78 levels were measured with an ELISA, using recombinant GRP78 as antigens. Cerebrospinal fluid (CSF) samples were also obtained from 88 patients with NPSLE. The BBB function was evaluated by Q albumin ((CSF albumin/serum albumin)×103).ResultsSerum anti-GRP78 levels were significantly elevated in SLE compared with non-SLE RD or HC. There were no significant differences in serum anti-GRP78 levels among NPSLE, LN and SLE alone. Of note, serum anti-GRP78 levels were significantly higher in acute confusional state (ACS) than in non-ACS diffuse NPSLE (p=0.0001) or in focal NPSLE (p=0.0002). Finally, serum anti-GRP78 levels were significantly correlated with Q albumin (r=0.294, p=0.0054) in NPSLE.ConclusionThese results indicate that anti-GRP78 antibodies are associated with the development of diffuse NPSLE, especially ACS. Thus, the data suggest that anti-GRP78 antibodies might contribute to the development of ACS through the damages of BBB.


2020 ◽  
Vol 32 (1) ◽  
Author(s):  
Howaida E. Mansour ◽  
Reem A. Habeeb ◽  
Noran O. El-Azizi ◽  
Heba H. Afeefy ◽  
Marwa A. Nassef ◽  
...  

Abstract Background Neuropsychiatric manifestations are frequently reported in systemic lupus erythematosus (SLE) patients. This study was done to describe electroencephalographic (EEG) findings in SLE patients with neuropsychiatric manifestation (NPSLE). Results Among 60 SLE patients, there were 50 females (83.3%) and 10 males (16.7%). EEG abnormalities were reported in 12 patients out of 30 (40%) with NPSLE, while all patients with non-NPSLE (n = 30) had no EEG abnormalities; diffuse slowing (20%) was the most common abnormalities, followed by generalized epileptiform activity (13.3%), and lastly temporal epileptiform activity (6.7%). Seizure was the most reported neuropsychiatric disorder in 13 patients (43.3%); 8 of them had abnormal EEG (61.5%). Periventricular white matter lesion (23.3%) followed by infarction (13.3%) were the most common MRI brain findings among 53.3% of NPSLE group. Half of the cases with EEG abnormality had normal brain MRI. SLEDAI score and ACL IgM positivity were higher in the NPSLE group than the non-NPSLE group. EEG is not a sensitive or specific test for detecting NPSLE with sensitivity (37.5%) and specificity (57.1%). Conclusion Not all patients with NPSLE must have abnormal brain MRI or EEG. EEG is a useful assistant tool in the assessment of different manifestations of NPSLE, but it cannot be used as a screening test alone and must be supplemented by neuroimaging studies.


2016 ◽  
Vol 33 (S1) ◽  
pp. S393-S393 ◽  
Author(s):  
D.K. Ochoa García ◽  
G.M. Chauca Chauca ◽  
L. Carrión Expósito

IntroductionIt is well known that seizures and psychosis are diagnostic criteria for systemic lupus erythematosus (SLE), however, there could be many other neuropsychiatric symptoms. The American College of Rheumatology Nomenclature provides case definitions for 19 neuropsychiatric syndromes seen in SLE (NPSLE), including cognitive impairment, psychosis, mood and anxiety disorders. Lack of specific manifestations difficult diagnosis and treatment.ObjectivesTo address the diagnostic difficulties that involve the appearance of hypomanic symptoms in the course of SLE treated with high doses of corticoids in a patient with a depressive episode history.MethodDescription of case report and literature revision. We report the case of a 22-year-old woman who presented irritable mood, sexual disinhibition, insomnia and inflated self-esteem. The patient was recently diagnosed with SLE and was on treatment with 50 mg/d prednisone. She had familiar history for bipolar disorder and was taking 20 mg/d paroxetine since the last 6 months after being diagnosed with major depressive episode.ResultsWe proposed differential diagnosis between psychiatric symptoms secondary to central nervous system SLE involvement, a comorbid bipolar disorder or prednisone-induced mood symptoms. Fluctuation of hypomanic symptoms during hospitalization, poor relationship with variation in corticosteroid doses, findings on brain MRI compatible with vasculitis and positive antibodies, oriented this case to a neuropsychiatric manifestation of LES.ConclusionsWe should keep in mind that symptoms of neuropsychiatric SLE may vary from more established manifestations of NPSLE to mild diffuses ones. More studies are needed to expand knowledge in the relationship between mood disorders and neuropsychiatric SLE.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2016 ◽  
Vol 8 (1) ◽  
pp. 59-65 ◽  
Author(s):  
Shala Ghaderi Berntsson ◽  
Evangelos Katsarogiannis ◽  
Filipa Lourenço ◽  
Maria Francisca Moraes-Fontes

Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.


2013 ◽  
Vol 72 (Suppl 3) ◽  
pp. A485.1-A485
Author(s):  
Y. Arinuma ◽  
H. Kikuchi ◽  
E. Ogawa ◽  
T. Wada ◽  
T. Nagai ◽  
...  

2017 ◽  
Vol 4 (1) ◽  
pp. 50
Author(s):  
Shalini Sri Kumaran ◽  
Annie Hui Nee Law

Systemic lupus erythematosus (SLE) is a multisystem connective tissue disorder with varied clinical presentations with late onset SLE occurring after the age of 50. Involvement of the central nervous system (CNS) in SLE may range from non-focal symptoms of cognitive dysfunction, an acute confusional state, to focal symptoms of transient ischaemic attacks and strokes.  We report a case of an elderly male who presented with confusion, functional decline and fevers. On admission, he was febrile with a Glasgow Coma Scale (GCS) of 11 with no other focal neurological or systemic examination findings. Initial investigations pointed towards a diagnosis of possible tuberculous meningitis with a differential diagnosis of unidentified sepsis and malignancy still being entertained. However, a newly developed purpuric skin rash helped clinch the diagnosis of SLE and he was finally diagnosed as a case of late-onset neuropsychiatric systemic lupus erythematosus (NPSLE). He was started on intravenous (IV) hydrocortisone which was later switched to a tapering dose of oral prednisolone and made a significant improvement. 


2003 ◽  
Vol 33 (3) ◽  
pp. 299-303 ◽  
Author(s):  
Katsuji Nishimura ◽  
Masako Omori ◽  
Naoshi Horikawa ◽  
Eiichi Tanaka ◽  
Takefumi Furuya ◽  
...  

Acute confusional state (delirium) appears as one of the common neuropsychiatric manifestations of systemic lupus erythematosus (SLE). It has been suggested that neuropsychiatric SLE (NP-SLE) could promote the occurrence of drug-induced extrapyramidal symptoms (EPS). Atypical antipsychotics have been thought to be useful in management of delirium because of their low incidence of adverse effects including EPS. However, no reports of the use of atypical antipsychotics in delirium due to NP-SLE have been published. Here we report a case of NP-SLE presenting an acute confusional state (delirium), which was successfully managed by risperidone.


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