FRI0327 Brain mri findings in patients with diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus

We report a case of 40-year-old female who is known to have systemic lupus erythematosus (SLE) presenting with severe cognitive impairment with lymphopenia, proteinuria, and evidence of SLE serological activity. Initially, she was managed as a case of neuropsychiatric systemic lupus erythematosus (NPSLE) with Cyclophosphamide and pulse steroids. However, she has been deteriorating clinically in forms of right sided hemiparesis, blindness, and aphasia despite normalization of complements and anti-double stranded DNA antibodies levels. Diagnosis of progressive multifocal leukoencephalopathy (PML) was made based on her clinical manifestations with brain MRI findings of subcortical white matter lesions and detection of John Cunningham virus (JCV) in cerebrospinal fluid analysis. Immunosuppressive agents were discontinued aiming for immune system restoration.

Download Full-text

Case of Acute Disseminated Encephalomyelitis Associated with Cytomegalovirus Reactivation in an Immunocompromised Systemic Lupus Erythematosus Patient

Medicina ◽

10.3390/medicina57090882 ◽

2021 ◽

Vol 57 (9) ◽

pp. 882

Author(s):

Mirela Pavicic Ivelja ◽

Kresimir Dolic ◽

Daniela Marasovic Krstulovic ◽

Gordana Glavina ◽

Ivo Ivic

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Brain Mri ◽

Acute Disseminated Encephalomyelitis ◽

Cognitive Disability ◽

Systemic Lupus ◽

Mri Findings ◽

Cns Lupus ◽

Ischemic Cerebrovascular Disease ◽

Wide Range

We present a case of an immunocompromised systemic lupus erythematosus female patient admitted to our hospital for general impairment, monoparesis, and temporary cognitive disability. The case represented a significant diagnostic and therapeutic challenge primarily because of a wide range of differential diagnosis options (CNS lupus, ischemic cerebrovascular disease, viral meningoencephalitis, progressive multifocal leukoencephalopathy, limbic encephalitis, and acute disseminated encephalomyelitis—ADEM). Brain MRI findings were compatible with ADEM, and microbiological tests showed a cytomegalovirus infection (CMV) which is rarely associated with ADEM despite the increasing number of immunocompromised patients prone to symptomatic CMV reactivation. Our patient was treated with intravenous methylprednisolone, immunoglobulin (IVIG), along with antiviral therapy resulting in a favorable therapeutic effect. In conclusion, only a few described ADEM cases have been associated with CMV, and none of them, to the best of our knowledge, in an immunocompromised patient. In this case, a multidisciplinary approach and broad diagnostic considerations were decisive for successful treatment and outcome.

Download Full-text

Electroencephalography in systemic lupus erythematosus patients with neuropsychiatric manifestations

The Egyptian Journal of Internal Medicine ◽

10.1186/s43162-020-00012-1 ◽

2020 ◽

Vol 32 (1) ◽

Author(s):

Howaida E. Mansour ◽

Reem A. Habeeb ◽

Noran O. El-Azizi ◽

Heba H. Afeefy ◽

Marwa A. Nassef ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Epileptiform Activity ◽

Brain Mri ◽

Periventricular White Matter ◽

Normal Brain ◽

Systemic Lupus ◽

Specific Test ◽

Eeg Abnormalities ◽

Neuropsychiatric Manifestations

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.

Download Full-text

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

Modern Rheumatology ◽

10.1080/14397595.2016.1193966 ◽

2016 ◽

Vol 27 (2) ◽

pp. 278-283 ◽

Cited By ~ 11

Author(s):

Gakuro Abe ◽

Hirotoshi Kikuchi ◽

Yoshiyuki Arinuma ◽

Shunsei Hirohata

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Brain Mri ◽

Acute Confusional State ◽

Systemic Lupus ◽

Confusional State

Download Full-text

Acute hypomania in systemic lupus erythematosus, differential diagnosis. A case report

European Psychiatry ◽

10.1016/j.eurpsy.2016.01.1414 ◽

2016 ◽

Vol 33 (S1) ◽

pp. S393-S393 ◽

Cited By ~ 1

Author(s):

D.K. Ochoa García ◽

G.M. Chauca Chauca ◽

L. Carrión Expósito

Keyword(s):

Systemic Lupus Erythematosus ◽

Bipolar Disorder ◽

Differential Diagnosis ◽

Case Report ◽

Lupus Erythematosus ◽

Depressive Episode ◽

Psychiatric Symptoms ◽

Brain Mri ◽

Systemic Lupus ◽

Neuropsychiatric Sle

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.

Download Full-text

Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology

Case Reports in Neurology ◽

10.1159/000444874 ◽

2016 ◽

Vol 8 (1) ◽

pp. 59-65 ◽

Cited By ~ 9

Author(s):

Shala Ghaderi Berntsson ◽

Evangelos Katsarogiannis ◽

Filipa Lourenço ◽

Maria Francisca Moraes-Fontes

Keyword(s):

Systemic Lupus Erythematosus ◽

Progressive Multifocal Leukoencephalopathy ◽

Lupus Erythematosus ◽

Jc Virus ◽

Brain Mri ◽

Fatal Outcome ◽

Disease Onset ◽

Unmet Need ◽

Human Polyomavirus ◽

Systemic Lupus

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.

Download Full-text