Lupus cerebritis is an inflammatory neurological condition secondary to systemic lupus erythematosus and is characterized by several neuropsychiatric manifestations. The diagnosis of lupus cerebritis can be challenging as there are no specific diagnostic tests. Therefore, the work up depends upon excluding other conditions with identical signs and symptoms, besides, confirming that the patient has systemic lupus erythematosus.
Presentation
Lupus cerebritis (LC) is a serious complication associated with the connective tissue disorder, systemic lupus erythematosus (SLE). Initial neuropsychiatric manifestations can be non-specific such as anxiety, depression, visual disturbances, behavioral changes, psychosis, stroke [1] [2], lethargy, dementia and even coma.
Psychiatric disorders in lupus cerebritis may result from thromboembolic events, electrolyte imbalance, opportunistic infections causing meningitis or encephalitis or may be due to prolonged steroid medication (steroid psychosis). Other neurological manifestations include parkinsonism with chorea and athetosis which is seen in approximately 4% patients [3], pseudotumor cerebri, and sinus thrombosis. Thromboembolic strokes occur in a small percentage of patients due to the involvement of blood vessels of all sizes.
Posterior reversible encephalopathy syndrome can present dramatically in patients on immunosuppressive medications and renal disease with hypertension [4] and typically resolves within a month after onset. All types of seizures have been reported in LC although partial and secondary general seizures are more common. Electrolyte imbalance and opportunistic intracranial infections can cause seizures while high dose steroid treatment can result in status epilepticus.
Transient cranial neuropathies are rare but have been reported [5] [6] while sensory peripheral neuropathy, mononeuritis multiplex, and acute demyelinating polyneuropathy are seen quite often. Although spinal cord is uncommonly involved, sudden onset spinal artery thrombosis, progressive demyelination and transverse myelitis have been reported.
Entire Body System
- Fever
The first case, presented with generalized seizure, severe metabolic acidosis, and shock, with a history of fever of one-month duration. [ncbi.nlm.nih.gov]
- Epilepsy
Managing epilepsy and co-existing disorders. Boston: Butterworth-Heinemann; 2002;135-154. With permission from Elsevier ( www.elsevier.com ). Back to top [epilepsy.com]
Epilepsy Behav 2016; 56: 165-9. [ Links ] 35. Irani SR, Stagg CJ, Schott JM, Rosenthal CR, Schneider SA, Pettingill P, et al. [scielo.cl]
Lil Wayne Lil Wayne's struggle with epilepsy has been well-documented over the years. In March of 2013 the Young Money rapper was hospitalized for over a week, which he later told Los Angeles’ Power 106 was because of multiple seizures. [mtv.com]
The central ones are further divided into neurological (aseptic meningitis, cerebrovascular disease, demyelinating syndrome, headache including migraine and benign intracranial hypertension, movement disorders, myelopathy, epilepsy), and psychiatric [f1000research.com]
Epilepsy is an important complication of lupus patients and can present as generalised "grand mal", focal, temporal lobe or "petit mal". [lupusuk.org.uk]
- Malaise
At today’s morning report at Parkland R2 Corbin Eule presented a case of a 68 year-old female with 2 weeks of confusion and lower extremity motor and sensory neuropathy, on a background of chronic fatigue, malaise and unintentional weight loss. [utswim.wordpress.com]
Clinical presentation and course are variable; common symptoms at presentation may include fever, fatigue, malaise, joint pain, abdominal pain, and stereotypical malar rash. 1 In severe cases, it can lead to lupus cerebritis, a neuropsychiatric manifestation [healio.com]
Hydroxychloroquine: Useful for skin lesions, arthralgia, myalgia and malaise. Cutaneous manifestations may respond within days but, more often, clinical improvement takes 6-12 weeks of treatment. [patient.info]
Anteriorly, they are: low cervical, second rib, lateral epicondyle, knee HIV infection Arthralgia, fever, lymphadenopathy, malaise, myalgia, peripheral neuropathy, rash Western blot assay for detection of HIV antibodies Inflammatory bowel disease Diarrhea [aafp.org]
- Multiple Organ Dysfunction Syndrome
acute lung injury, multiple organ dysfunction syndrome, and much more. [books.google.ro]
Respiratoric
- Pneumonia
10) Serositis (8) Neurological (6) Mucocutaneous (3) Pulmonary haemorrhage (1) Infection 16.2 Types of infection ( n ) Vasculitic ulcer infection (1) Septic joint (2) Haemodialysis line sepsis (4) Cellulitis (2) Urosepsis/urinary tract infection (4) Pneumonia [academic.oup.com]
(nonspecific) (usual) due to collagen vascular disease Interstitial pneumonia (nonspecific) (usual) in diseases classified elsewhere Organizing pneumonia due to collagen vascular disease Organizing pneumonia in diseases classified elsewhere Code First [icd10data.com]
In very rare cases, cerebritis may occur as a result of a Klebsiella pneumoniae infection.[10] One other reason to develop cerebritis is an infection caused by bacteria, viruses, or other organisms. [en.wikipedia.org]
- Pleural Effusion
286 888 Pregnancy Trauma 898 Foreign Body Esophageal 902 Procedural Sedation 904 Pseudotumor Cerebri 910 Pulmonary Contusion 920 Purpura 926 Peritonsillar Abscess 928 Phalangeal Injuries Hand 934 Phenytoin Poisoning 940 Pityriasis Rosea 946 Red 948 Pleural [books.google.ro]
The diagnosis of SLE in this case was based on the presence of serositis (pleural effusion, pericardial effusion, ascites), anemia, pancytopenia, discoid rash, and neuropathy, with very high ANA and anti-DsDNA levels. [ncbi.nlm.nih.gov]
Cardiovascular
- Hypertension
Coverage includes practical information on renal transplant and dialysis, the main classes of drugs used for hypertension and renal protection, and how to prescribe for patients with renal diseases. [books.google.ro]
Posterior reversible encephalopathy syndrome can present dramatically in patients on immunosuppressive medications and renal disease with hypertension and typically resolves within a month after onset. [symptoma.com]
Eyes
- Diplopia
Five weeks prior to presentation, the patient started to experience headaches, diplopia and squint followed a week later by blurred vision. The headaches and diplopia gradually subsided, whereas her blurred vision worsened progressively. [rheumatology.oxfordjournals.org]
Urogenital
- Kidney Failure
In severe cases, organs damage and failure can occur. Over 90 percent of people with lupus are women between the ages of 15 and 45. Historically, lupus caused people to die young, primarily from kidney failure. [healthline.com]
Todaro/Filmmagic Nick Cannon is opening up about his ongoing health problems that came to light after he was hospitalized for kidney failure in January and then hospitalized again, weeks later, with blood clots in his lungs. [people.com]
Kidney problems occur in 30% to 50% of lupus patients and may progress to kidney failure. Kidney disease due to lupus occurs more frequently in African-Americans and Hispanics. [clinicaltrials.gov]
Some people develop lupus nephritis, an inflammation of the kidneys that can cause them to leak protein and blood cells into urine. Left untreated, this can lead to kidney failure. [nyulangone.org]
Complications Inflammation caused by lupus can affect many areas of your body, including your: Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. [mayoclinic.org]
Neurologic
- Seizure
All types of seizures have been reported in LC although partial and secondary general seizures are more common. [symptoma.com]
She presented with new-onset generalized seizures. High-dose steroids were given for the cerebral edema. [radiopaedia.org]
The range of neurological symptoms varies from non-specific features like depression, anxiety, headaches, and seizures, while severe symptoms (major seizures, vision problems, dizziness, behavior changes, stroke, or psychosis) are commonly encountered [ncbi.nlm.nih.gov]
- Stroke
or to patients who suffered a minor stroke event, to better understand the mechanism responsible for the increased stroke risk in SLE patients. [lupusnewstoday.com]
Thromboembolic strokes occur in a small percentage of patients due to the involvement of blood vessels of all sizes. [symptoma.com]
This may result in stroke or other symptoms including headaches, seizures, psychosis, dementia, or peripheral neuropathy. Stroke occurs in 5–20% of all patients with systemic lupus. [link.springer.com]
The symptoms of lupus cerebritis may be mild or severe and these may include depression, anxiety, headaches, stroke and seizures among others. [simstat.com]
- Confusion
A 48-year-old woman with a recent diagnosis of SLE was admitted to the hospital because of a fever, confused state, and convulsive episode. Her symptoms were interpreted as being compatible with lupus cerebritis. [unboundmedicine.com]
Lupus fog can be frustrating but there are a variety of coping skills that can help you learn to improve your concentration and lessen confusion. [lupus.org]
Confused at how we got to here so quickly? You probably did not know or realise but Sodium Valproate has controlled my life for the last few years. It has. You may have noticed along the way that I am kind of interested in art. [thislupuslife.com]
- Dizziness
The serious problems caused because of inflammation include headaches, seizures, vision problems, dizziness, behavior changes and even stroke. In recent times a number of cases with some mild form of lupus cerebritis have been identified. [simstat.com]
Symptoms can include mild to severe headaches, dizziness, involuntary movements, seizures, stroke, or dementia. Tests can help to determine if a patient has lupus cerebritis, such as blood tests or imaging tests, like a CAT scan or MRI scan. [drgaryslupustreatment.org]
The types of symptoms you might experience include: Vision problems Facial pain Ringing in the ears Dizziness Drooping of an eyelid Carpel tunnel syndrome Autonomic Nervous System (ANS) impacts The autonomic nervous system regulates many of the body’s [lupus.org]
Other symptoms include dizziness, chest pain and kidney inflammation. The strain of dealing with this condition can also greatly affect the state of mind, making depression common in lupus sufferers. [wisegeekhealth.com]
I have horrible fatigue, nausea, headaches almost daily, dizziness, no appetite, problems walking and moving in general, cognitive problems, brain fog, memory problems,concentration problems,anxiety or depression problems, urinary problems, neuropathy [mdjunction.com]
- Cognitive Impairment
That condition is characterized by cognitive impairment and dementia, depression, and balance problems. [lupusnewstoday.com]
The various studies of cognitive function suggest considerable diversity in the type of cognitive impairment, including attention and concentration, various aspects of verbal and non-verbal memory including working memory, verbal fluency, visuo-spatial [lupusuk.org.uk]
Although dementia is not considered one of the most common presentations, formal neuropsychological testing has suggested that 66% of SLE patients experience cognitive impairment. 6 The true incidence of seizures in SLE is difficult to determine, owing [epilepsy.com]
One must emphasize that the anti-NMDAR antibody has been categorized as a mediator for cognitive impairment [17]. [journals.plos.org]
Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 2013; 136 (10): 3151-62. [ Links ] 36. Soler B, Godoy J, Mellado P. [scielo.cl]
Workup
Diagnosis of lupus cerebritis can be challenging as the clinical features are non-specific, resemble other psychiatric and neurological disorders and there is no particular laboratory or imaging study available to confirm the condition. The disorder should be suspected in young, female patients presenting with unexplained neuropsychiatric signs and symptoms. Clinical suspicion, history, examination findings along with serological detection of lupus antibodies and cerebrospinal fluid (CSF) analysis are required to make a provisional diagnosis.
A thorough neurological examination may reveal movement disorders, mononeuropathy or cranial nerve involvement, features of intracranial thromboembolism like stroke and even spinal cord infarction. A complete blood count will show hemolytic anemia with decreased platelets and neutropenia. Inflammatory markers like erythrocyte sedimentation rate and C-reactive protein are elevated. Serological tests for lupus antibodies include antinuclear antibodies (ANA), antiphospholipid antibodies, and anticardiolipin antibodies will be positive in patients with LC [7]. Assessment of serum electrolytes is essential in patients presenting with confusion or coma to exclude electrolyte imbalance as the underlying etiology. CSF finding of an immunological SLE marker is more specific for LC compared to a serum marker [2] with lymphocytotoxic antibodies being seen in a majority of patients with LC [8]. Other CSF findings in these patients are lymphocytosis with an elevated cell count, high levels of proteins [9], interleukin-6 and interferon alfa. With severe central nervous system involvement, CSF levels of nitric oxide are found to be elevated and can help to monitor LC progression [10].
An electroencephalogram is likely to be abnormal in more than 50% of the patients.
Computed tomography (CT) scan features can vary from normal to cerebral atrophy, cerebral infarction, intracranial hemorrhage, to intracranial abscesses. In patients with chronic lupus cerebritis, CT scan may show calcifications. Magnetic resonance imaging (MRI) is a more sensitive imaging study to observe neurological features of LC [1]. Other imaging studies such as magnetic resonance spectroscopy, diffusion and perfusion weighted imaging, and magnetization transfer imaging are being studied currently to enable rapid confirmatory diagnosis of LC [11].
Biopsy of the brain is indicated if imaging studies are unable to differentiate between LC and intracranial lesions due to other etiologies. On histology, findings in lupus cerebritis are small vessel vasculitis with or without inflammatory cells and features of embolic infarction.
Pleura
- Pleural Effusion
286 888 Pregnancy Trauma 898 Foreign Body Esophageal 902 Procedural Sedation 904 Pseudotumor Cerebri 910 Pulmonary Contusion 920 Purpura 926 Peritonsillar Abscess 928 Phalangeal Injuries Hand 934 Phenytoin Poisoning 940 Pityriasis Rosea 946 Red 948 Pleural [books.google.ro]
The diagnosis of SLE in this case was based on the presence of serositis (pleural effusion, pericardial effusion, ascites), anemia, pancytopenia, discoid rash, and neuropathy, with very high ANA and anti-DsDNA levels. [ncbi.nlm.nih.gov]
Treatment
Treatment Options for Lupus Cerebritis Since lupus cerebritis can cause long-lasting and debilitating problems, it is important to get treatment for the condition as soon as possible. Treatment will be based on different factors. [drgaryslupustreatment.org]
It is necessary to find the exact cause of a lupus cerebritis flare up before treatment can begin. [wisegeekhealth.com]
Treatment: – Treatment of SLE depends on the severity of the flare, with systemic corticosteroids and immunosuppressants forming the mainstay of treatment. – Determining dosing intensity and duration of corticosteroid therapy for treatment of SLE is challenging [utswim.wordpress.com]
Current concepts in the pathophysiology plants treat lupus treatment of aplastic anaemia. What i should have to use. Recall is generally 3 weeks for the initial phase of your treatment plan. [sheilaobrien.club]
Early detection and treatment is the key to a better health outcome and can usually lessen the progression and severity of the disease. [books.google.de]
Prognosis
Prognosis of Lupus glomerulonephritis Treatment leading to normalization of proteinuria, HTN and renal dysfunction indicate a good prognosis. Glomerulosclerosis usually predicts ESRD (Kumar and Clarks’s, Clinical Medicine 2009) 19. [slideshare.net]
Prognosis The prognosis has improved with earlier recognition and improved management. The five-year survival rate is over 90%. [patient.info]
What is the prognosis for patients with involvement of the brain? Overall, the prognosis is quite good. Dr. Steven’s study showed that patients with CNS disease as a group survived as well as patients without neurological disease. [lupusinternational.com]
Treatment and prognosis Management is predominantly with immunotherapy, options including high-dose glucocorticoids, intravenous immunoglobulin, plasmapharesis, cyclophosphamide, and rituximab 11,12. [radiopaedia.org]
Learn mri lupus erythematosus lupus symptoms (butterfly rash), treatment options, diagnosis, prognosis, causes, and pregnancy flares. Cerebritis about diet. [latestlupusupdate.review]
Etiology
Biopsy of the brain is indicated if imaging studies are unable to differentiate between LC and intracranial lesions due to other etiologies. [symptoma.com]
Cerebritis and Neutropenia in A Child with ana Negative Lupus Abstract 204 Objective Systemic lupus erythematosus (SLE), an autoimmune systemic disease with unknown etiology, affects virtually every part of the body; involvement of the central nervous [journals.sbmu.ac.ir]
* Comprehensive current coverage of basic models of etiology and pathogenesis * Complete catalog of autoantibodies produced * Includes an outstanding review of imaging techniques for the diagnosis of lupus and its complications for each organ system [books.google.ro]
Epidemiology
Epidemiologic, twin, and human leukocyte antigen data suggest a strong genetic contribution to the etiology of SLE, but the exact cause is unknown. [pedsinreview.aappublications.org]
The epidemiology of tumor-related autoimmune encephalitis mimics that of the underlying malignancy. Those with non-tumor related autoimmune encephalitis have a variable epidemiology but are mostly young patients with a female predilection 8. [radiopaedia.org]
Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus 2006;15:308-18. D'Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet 2007;369:587-96. Munoz LE et al. [eurodiagnostica.com]
[…] the Head of, the Sjögren's Syndrome Clinic, National Institute of Dental and Craniofacial Research, Gene Therapy and Therapeutics Branch Nikolay P Nikolov, Janine A Smith, Nicholas J Patronas & Gabor G Illei Deputy Clinical Director of the Division of Epidemiology [nature.com]
Pathophysiology
Through a patient case presentation, this article covers the pathophysiology, medication administration, and nursing considerations of a patient with lupus cerebritis. [ncbi.nlm.nih.gov]
Through a patient case presentation, this article covers the pathophysiology, medication administration, and nursing considerations of a patient with lupus cerebritis. © Williams & Wilkins 1996. All Rights Reserved. Related Articles [journals.lww.com]
The exact pathophysiological process of lupus cerebritis is unknown. The proposed mechanisms are likely due to the assault of several autoimmune system changes, including the following: Circulating immune complexes. [en.wikipedia.org]
10-15%. [5] Causes Lupus systemic erythematosus is one of the most common causes of cerebritis as it is believed that more than half of the patients with lupus from the United States suffer from a degree or another of lupus cerebritis. [6] The exact pathophysiological [ipfs.io]
Prevention
Q: What are some ways a person with lupus could prevent infections, or prevent an existing infection from becoming severe? A: It’s important to catch infections early. [healthline.com]
Neonatal prevention IVIg is not supported. Therapy with intravenous immune globulin had no effect on the outcomes of suspected or proven neonatal sepsis (Brockelhurst et al 2011). [blood.gov.au]
Moreover, the effect seemed independent of immune suppression, implying that Plaquenil can directly act to prevent the blood clots. [medicinenet.com]
Preventing a flare Treatment is available for lupus, but understanding how to prevent flares of this disease is very important to improve a person's overall quality of life. [medicalnewstoday.com]
The most desirable of these would be a heightening of awareness of the mental health needs of "pregnant families" and of the risks they incur in transition from non parenthood to parenthood, and a more effective level of primary and secondary prevention [books.google.ro]
References
- Raj R, Murin S, Matthay RA, Wiedemann HP. Systemic Lupus Erythematosus in the intensive care unit. Crit Care Clin. 2002;18:781–803.
- Kajs-Wyllie M. Lupus cerebritis: A case study. J Neurosci Nurs. 2002;34:176–83.
- Sanna G, Bertolaccini ML, Cuadrado MJ, et al. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatol. 2003 May; 30(5):985-92.
- Ishimori ML, Pressman BD, Wallace DJ, Weisman MH. Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE?. Lupus. 2007; 16(6):436-43.
- Keane JR. Eye movement abnormalities in systemic lupus erythematosus. Arch Neurol. 1995 Dec; 52(12):1145-9.
- Lee JH, Sung IY, Park JH, Roh JL. Recurrent laryngeal neuropathy in a systemic lupus erythematosus (SLE) patient. Am J Phys Med Rehabil. 2008 Jan; 87(1):68-70.
- Greenwood DL, Gitlits VM, Alderuccio F, Sentry JW, Toh BH. Autoantibodies in neuropsychiatric lupus. Autoimmunity. 2002;35:79–86.
- Bruyn GA. Controversies in lupus: Nervous system involvement. Ann Rheum Dis. 1995; 54: 159– 67.
- Calabrese LV, Stern TA. Neuropsychiatric manifestations of systemic lupus erythematosus. Psychosomatics. 1995;36:344–59.
- Brundin L, Svenungsson E, Morcos E, et al. Central nervous system nitric oxide formation in cerebral systemic lupus erythematosus. Ann Neurol. 1998;44:704–6.
- Govoni M, Castellino G, Padovan M, Borrelli M, Trotta F. Recent advances and future perspective in neuroimaging in neuropsychiatric systemic lupus erythematosus. Lupus. 2004;13:149–58.