Edit concept Question Editor Create issue ticket

Lupus Cerebritis

Cer System Lupus Erythematosus

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.

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]
  • 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]
  • The demographic characteristics (sex and age), clinical features (disease duration, NP-related manifestations, such as cerebrovascular disease, epilepsy, mental disorder), and general SLE-related symptoms (fever, erythema, dental ulcer, vasculitides,[archivesofrheumatology.org]
  • Herranz MT, River G, Kamashta MA, Blaser KU, Hughes G (1994) Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus. Arthritis Rheum 37: 568–571. View Article Google Scholar 15.[journals.plos.org]
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]
Bruxism
  • […] headaches; The neck and headaches; Drug-induced headaches; Trigeminal neuralgia and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and headaches; Post-traumatic headaches; Metabolic headaches; Temporomandibular joint disorders, bruxism[books.google.ro]
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]
  • […] topics that include: Headaches due to nasal and paranasal sinus disease; Headaches due to vascular disorders; Headaches and brain tumors; Low cerebrospinal fluid pressure syndromes; Painful ophthalmologic disorders and eye pain; Idiopathic intracranial hypertension[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]
  • Apply new evidence-based management options to treat genetic and constitutional factors leading to a high-risk pregnancy (such as diabetes, obesity, hypertension, and cardiac disease) through new and expanded coverage of these increasingly common presentations[books.google.com]
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]
Seizure
  • All types of seizures have been reported in LC although partial and secondary general seizures are more common.[symptoma.com]
  • Within 2 weeks she started to have headaches and was admitted for seizure and psychosis. Ultraviolet A exposure in the tanning salon is known to exacerbate lupus by modulation of the immune system at the level of the skin.[ncbi.nlm.nih.gov]
  • She presented with new-onset generalized seizures. High-dose steroids were given for the cerebral edema.[radiopaedia.org]
  • The symptoms of lupus cerebritis may be mild or severe and these may include depression, anxiety, headaches, stroke and seizures among others.[simstat.com]
  • 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
  • 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]
  • Lupus cerebritis with associated headache, seizures, stroke, and chorea is just one of a wide array of central nervous system disorders SLE patients can develop. It also is one of the most difficult manifestations of lupus to diagnose.[ncbi.nlm.nih.gov]
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]
  • Assessment of serum electrolytes is essential in patients presenting with confusion or coma to exclude electrolyte imbalance as the underlying etiology.[symptoma.com]
  • 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.[thislupuslife.com]
  • 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]
  • 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]
  • People who smoke who attempt to stop typically expertise complications, dizziness, and fidgeting, deep respiratory and meditation will help you to chill out your self from inside till the urge fades away.[ecigcanadazone.com]
Vertigo
  • Streptococcal Disease 1156 Tuberculosis 1158 Tympanic Membrane Perforation 1164 Suicide Risk Evaluation 1166 Gastritis 1168 Syncope 1172 Ovarian CystTorsion 788 1195 Ventilator Management 1198 Ventricular Peritoneal Shunts 1202 Urinary Tract Fistula 1174 Vertigo[books.google.ro]

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.

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]
  • 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]
  • The second form is a clotting disorder and once the type of condition is identified the relevant treatment can be given.[simstat.com]
  • Every medical procedure and treatment that might make any sense at all was tried on him as he lay in the ICU of the hospital.[books.google.ro]

Prognosis

  • Learn mri lupus erythematosus lupus symptoms (butterfly rash), treatment options, diagnosis, prognosis, causes, and pregnancy flares. Cerebritis about diet.[latestlupusupdate.review]
  • Systemic Lupus erythematosus: antibody symptomatology, other laboratory evidence, diagnosis, course and prognosis; discoid Lupus erythematosus; drug induced Lupus .[eurekamag.com]
  • Systemic lupus erythematosus in patients with end-stage renal disease: long-term follow-up on the prognosis of patients and the evolution of lupus activity . Am. J. Kidney Dis. 16 , 189–195 (1990). 11. Okano, K. et al .[nature.com]
  • 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]
  • Therapy and prognosis. Am J Med 1975;58:644-654 2. Abel T, Gladman D, Urowitz M. Neuropsychiatric lupus. J Rheumatol 1980;7:325-333 3. Hughes G. Central nervous system lupus-diagnosis and treatcment. J Rheumatol 1980;7:405-41 1 4.[iicsfl.com]

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]
  • . * 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]
  • 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]

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]
  • During this procedure, blood in removed from the body as in dialysis and certain blood cells monocytes are "washed" with a drug 8- methoxypsoralen; This week, we talk about a sickness called lupus and other autoimmune diseases; The application of genetic epidemiology[sarahwang.club]
  • 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]
  • Epidemiology There is a strong female predilection in adults, with women affected 9-13 times more than males. In children, this ratio is reversed, and males are affected two to three times more often.[radiopaedia.org]
  • JA Smith is a Deputy Clinical Director of the Division of Epidemiology and Clinical Research, National Eye Institute.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • From a clinical point of view, we argue that pseudotumour cerebri in this patient does not represent an idiopathic process, although the pathophysiology remains unknown.[rheumatology.oxfordjournals.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]
  • 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]

Prevention

  • 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]
  • The Prevention A. What to avoid Any thing which cause inflammation must be avoid. thyere is a report found that SLE patients and lupus-prone mice induces skin inflammation following intradermal injection into normal mice.[theplus-health.blogspot.com]
  • Furthermore, the present invention relates to a method of detecting the internalization behaviour of a fusion molecule of the invention, the composition of the invention for treating and/or preventing a condition selected from cancer, enzyme deficiency[glosbe.com]
  • A good treatment plan can help keep symptoms of lupus cerebritis under control or can at least help prevent further complications. I too have similar symptoms like tingling and numbness. My neurological responses are poor.[wisegeekhealth.com]
  • Review of the literature and guidelines for prevention . Int. J. Artif. Organs 17 , 5–13 (1994). 10. Cheigh, J. S. et al .[nature.com]

References

Article

  1. Raj R, Murin S, Matthay RA, Wiedemann HP. Systemic Lupus Erythematosus in the intensive care unit. Crit Care Clin. 2002;18:781–803.
  2. Kajs-Wyllie M. Lupus cerebritis: A case study. J Neurosci Nurs. 2002;34:176–83.
  3. 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.
  4. Ishimori ML, Pressman BD, Wallace DJ, Weisman MH. Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE?. Lupus. 2007; 16(6):436-43.
  5. Keane JR. Eye movement abnormalities in systemic lupus erythematosus. Arch Neurol. 1995 Dec; 52(12):1145-9.
  6. 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.
  7. Greenwood DL, Gitlits VM, Alderuccio F, Sentry JW, Toh BH. Autoantibodies in neuropsychiatric lupus. Autoimmunity. 2002;35:79–86.
  8. Bruyn GA. Controversies in lupus: Nervous system involvement. Ann Rheum Dis. 1995; 54: 159– 67.
  9. Calabrese LV, Stern TA. Neuropsychiatric manifestations of systemic lupus erythematosus. Psychosomatics. 1995;36:344–59.
  10. 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.
  11. 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.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2018-06-22 08:37