Encephalopathy with upper body hypertonia and myoclonus in patient with systemic lupus erythematosus and anti-CASPR2

Lupus ◽  
2016 ◽  
Vol 26 (1) ◽  
pp. 84-87 ◽  
Author(s):  
A Castro ◽  
J C Romeu ◽  
R Geraldes ◽  
J A Pereira da Silva
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nervous System ◽  
Lupus Erythematosus ◽  
Fdg Pet ◽  
Limbic Encephalitis ◽  
Brain Mri ◽  
Lower Limbs ◽  
Upper Body ◽  
Systemic Lupus ◽  
Specific Antibodies

Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET). Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies. Brain MRI may be normal and cortical brain hypometabolism on FDG PET scans has been reported. We have not found any report of limbic encephalitis or other SLE neurological manifestation associated to positive titres of anti-CASPR2 antibodies and this may warrant systematic investigation. In the rare cases of limbic encephalitis associated with SLE no specific antibodies were documented. Anti-CASPR2 antibodies have been associated not only with limbic encephalitis but also with neuromyotonia and Morvan syndrome. Although our patient had a specific pattern of tone abnormalities with an impressive cervical and upper limb hypertonicity and flaccid lower limbs, no myotonic discharges were found. We did not find any association between myoclonus and anti-CASPR2 antibodies. We cannot exclude that a non determined autoantibody could have played a role; however, clinical and FDG PET improvement supports an antibody-mediated injury, in this case of neuroSLE.

scholarly journals Electroencephalography in systemic lupus erythematosus patients with neuropsychiatric manifestations

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.

scholarly journals Autoantibodies in Neuropsychiatric Systemic Lupus Erythematosus (NPSLE): Can They Be Used as Biomarkers for the Differential Diagnosis of This Disease?

2021 ◽  
Author(s):  
Elias Manca
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Human Body ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Systemic Lupus ◽  
Neuropsychiatric Systemic Lupus Erythematosus ◽  
The Central Nervous System

AbstractSystemic lupus erythematosus is a complex immunological disease where both environmental factors and genetic predisposition lead to the dysregulation of important immune mechanisms. Eventually, the combination of these factors leads to the production of self-reactive antibodies that can target any organ or tissue of the human body. Autoantibodies can form immune complexes responsible for both the organ damage and the most severe complications. Involvement of the central nervous system defines a subcategory of the disease, generally known with the denomination of neuropsychiatric systemic lupus erythematosus. Neuropsychiatric symptoms can range from relatively mild manifestations, such as headache, to more severe complications, such as psychosis. The evaluation of the presence of the autoantibodies in the serum of these patients is the most helpful diagnostic tool for the assessment of the disease. The scientific progresses achieved in the last decades helped researchers and physicians to discover some of autoepitopes targeted by the autoantibodies, although the majority of them have not been identified yet. Additionally, the central nervous system is full of epitopes that cannot be found elsewhere in the human body, for this reason, autoantibodies that selectively target these epitopes might be used for the differential diagnosis between patients with and without the neuropsychiatric symptoms. In this review, the most relevant data is reported with regard to mechanisms implicated in the production of autoantibodies and the most important autoantibodies found among patients with systemic lupus erythematosus with and without the neuropsychiatric manifestations.

scholarly journals AB0383 EXTREME FATIGUE IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND NEUROPSYCHIATRIC SYMPTOMS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1491.2-1492
Author(s):  
R. Monahan ◽  
R. Fronczek ◽  
J. Eikenboom ◽  
H. Middelkoop ◽  
L. J. J. Beaart- van de Voorde ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nervous System ◽  
Standard Deviation ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Systemic Lupus ◽  
System Involvement ◽  
Vas Score ◽  
Age Related ◽  
Sf 36

Background:Fatigue is commonly described in chronic illnesses, especially auto-immune disorders such as systemic lupus erythematosus (SLE).Objectives:We aim to study the prevalence of fatigue in SLE patients with NP symptoms and compare fatigue in SLE patients with NP symptoms attributed to major organ involvement due to SLE (NPSLE) with SLE patients with NP symptoms not caused by major nervous system involvement (non-NPSLE).Methods:All patients visiting the tertiary referral center for NPSLE in the LUMC between 2007-2019 with the clinical diagnosis of SLE and age >18 years that signed informed consent were included in this study. Patients underwent a standardized multidisciplinary assessment, including two questionnaires: SF-36 (2007-2019) and multidimensional fatigue index (MFI, 2011-2019). Patients were classified as NPSLE in this study if NP symptoms were attributed to SLE and immunosuppressive or anticoagulant therapy was initiated, otherwise patients were classified as non-NPSLE. The vitality (VT) domain of the SF-36 domain was used to assess fatigue, which generates a score from 0-100, 100 representing the complete absence of fatigue. Patients with a score more than 1 standard deviation (SD) removed from age-related controls of the Dutch general population were classified as fatigued; patients more than 2 SD removed were classified as extremely fatigued1. The MFI was also used, which consists of 5 subdomain scores between 0-20, leading to a total score between 0-100, 100 representing the most extreme fatigue. All scores are presented as mean and standard deviation.Results:373 patients fulfilled the inclusion criteria and SF-36 questionnaires of 328 patients were available (88%). The majority of these patients was female (87%) and 98 were classified as NPSLE (30%). In NPSLE patients, average age was 41 ± 13 years and in non-NPSLE the average age was 45 ± 14 years. The average score of the SF-36 vitality domain was 36.0 ± 20.7 in NPSLE vs 33.9 ± 18.8. in non-NPSLE. Overall, 73.5% of the patients were fatigued and 46.9% extremely fatigued in NPSLE vs 77.8% fatigued and 45.7% extremely fatigued in non-NPSLE.The MFI questionnaire and VAS score were available for 222 patients, of which 65 patients were classified as NPSLE (29.3%). Table 1 depicts the scores of NPSLE and non-NPSLE patients on the MFI subdomains and the VAS score.Table.Patient characteristics at registry entry.NPSLE(N = 65)Non-NPSLE (N = 157)MFI(mean, sd)General Fatigue10.8 (1.8)11.1 (1.5)Physical Fatigue11.4 (2.4)12.3 (1.9)Reduced Activity9.6 (2.9)10.7 (2.2)Reduced Motivation10.7 (2.6)11.1 (1.9)Mental Fatigue9.5 (3.0)9.8 (2.7)Total score51.8 (9.9)54.9 (6.9)SF-36 Vitality (mean, sd)35 (20.7)32.7 (18.2)Conclusion:Nearly half of patients with SLE and NP symptoms are as extremely fatigued as only 2.5% of the general Dutch population. Extreme fatigue is not influenced by major nervous system involvement.References:[1]Aaronsonet al.J Clin Epidemiol. Vol. 51, No. 11, pp. 1055–1068, 1998Disclosure of Interests:Rory Monahan: None declared, Rolf Fronczek: None declared, Jeroen Eikenboom: None declared, Huub Middelkoop: None declared, L.J.J. Beaart- van de Voorde: None declared, Gisela Terwindt: None declared, Nic van der Wee: None declared, Thomas Huizinga Grant/research support from: Ablynx, Bristol-Myers Squibb, Roche, Sanofi, Consultant of: Ablynx, Bristol-Myers Squibb, Roche, Sanofi, Margreet Kloppenburg: None declared, G.M. Steup-Beekman: None declared

scholarly journals A report of chronic intestinal pseudo-obstruction related to systemic lupus erythematosus

Open Medicine ◽  
2018 ◽  
Vol 13 (1) ◽  
pp. 562-564 ◽  
Author(s):  
Ruiqiang Wang ◽  
Bowen Zheng ◽  
Biyue Wang ◽  
Pupu Ma ◽  
Fengmei Chen ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Symptoms ◽  
Gastrointestinal Disorder ◽  
Lower Limbs ◽  
Class Iii ◽  
Systemic Lupus ◽  
Intravenous Corticosteroid ◽  
Clinical Presentations ◽  
Intestinal Pseudo Obstruction

AbstractChronic intestinal pseudo-obstruction (CIPO) is a functional gastrointestinal disorder with symptoms of ileus. CIPO can either be idiopathic or secondary to other diseases such as systemic lupus erythematosus (SLE). SLE is involved in many parts of the gastrointestinal system with variable clinical presentations. Reports about reduplicated CIPO as a complication of SLE is infrequent. A 49-year-old female suffering from clinical symptoms of ileus has been hospitalized 3 times over 1 year. Her examination results showed no observation of mechanical obstruction. In August 2017, she came to the nephrology department due to edema in both lower limbs along with symptoms of ileus. After thorough examination, she was diagnosed with secondary CIPO related to SLE. Results of renal biopsy confirmed to be lupus nephritis (Class III-(A) + V). The symptoms of ileus are gradually improved after treatment of full-dose intravenous corticosteroid for 5 days.

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