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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
  • 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]
  • In patients with systemic lupus erythematosus (SLE) presenting with headache and fever, SLE cerebritis must be differentiated from acute bacterial meningitis by lumbar puncture and cerebrospinal fluid (CSF) analysis.[deepdyve.com]
  • […] additionally had cerebral lesions consistent with neutrophilic vasculitis and cerebritis, and that it may all have started at least 10 years prior with nonspecific musculoskeletal manifestations subsequently followed by a rash as well as intractable fevers[pubfacts.com]
Epilepsy
  • She presented with an atypical movement disorder and partial continuous epilepsy. A brain biopsy excluded cerebral vasculitis and tumoral processes. We discuss the differential diagnosis of status epilepticus in a patient with SLE.[reumatologiaclinica.org]
  • 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 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]
  • […] following six drugs: hydralazine (Apresoline) is used for high blood pressure ; quinidine (Quinidine Gluconate, Quinidine Sulfate) and procainamide (Pronestyl; Procan-SR; Procanbid) are used for abnormal heart rhythms ; phenytoin (Dilantin) is used for epilepsy[rxlist.com]
Malaise
  • 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]
Amyloidosis
  • T. 1999 122 Secondary Amyloidosis has Decreased in Patients with Inflammatory Joint Disease in Finland Laiho, K. / Tiitinen, S. / Kaarela, K. / Helin, H. / Isomäki, H. 1999 124 Optic Neuropathy in Systemic Lupus Erythematosus and Antiphospholipid Syndrome[tib.eu]
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.com]
  • […] 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]
Blurred Vision
  • S ir , We describe a 22‐yr‐old female admitted with a 1‐month history of blurred vision. Five weeks prior to presentation, the patient started to experience headaches, diplopia and squint followed a week later by blurred vision.[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]
  • What is seizure disorder? Learn about seizure disorder symptoms, seizure disorder causes, diagnosis, and more.[latestlupusupdate.review]
  • The patient reported here was a 12-year-old girl with ANA negative lupus cerebritis who presented with left hemiparesia after a generalized seizure, with neutropenia observed during its course.[journals.sbmu.ac.ir]
  • 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.[journals.lww.com]
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]
  • 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.[journals.lww.com]
  • . - Role of imaging Assessment of acute focal (stroke-like) neurologic deficits. CNS vasculitis. Reported in up to 7% of SLE patients.[neuroradiologyonthenet.blogspot.com]
  • Keep current with the latest developments in palliative care, mass casualty/epidemic disease, acute respiratory failure, non-invasive ventilation, neurocritical care, neuroimaging, hypoxic-ischemic encephalopathy, stroke and intracerebral hemorrhage,[books.google.ro]
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]
  • Mayo Clinic Proceedings 74(6): 575-578 1999 A 48-year-old woman with a recent diagnosis of systemic lupus erythematosus (SLE) was admitted to hospital in Israel because of a fever, confused state, and convulsive episode.[eurekamag.com]
Dizziness
  • 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]
  • 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 didn’t feel dizzy anymore. When it was time for my periodic lab test to check my hemoglobin count, the doctor was in for a big surprise. My hemoglobin count went up from 8.1 g/dl to 12 g/dl (normal level) and my creatine level was normal.[philstar.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.com]

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]
  • 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]
  • 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.com]

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]
  • Thus, these biomarkers are potentially powerful adjuncts to current standards for the diagnosis, prognosis, and treatment of acute and chronic HF.[donaldwilliams.club]

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 193 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]
  • The secondary headaches include a diverse and fascinating array of etiologies which can mimic primary headache disorders ranging from the rare to the mundane and from the well established to the highly controversial.[books.google.ro]
  • […] cerebral lesions consistent with neutrophilic vasculitis and cerebritis, and that it may all have started at least 10 years prior with nonspecific musculoskeletal manifestations subsequently followed by a rash as well as intractable fevers of unknown etiology—much[pubfacts.com]
  • She had serologic evidence of lupus activity and all infectious etiology was ruled out. She had a protracted course but symptoms were eventually controlled with intravenous steroids, cyclophosphamide, rituximab and intravenous immunoglobulin.[omicsonline.org]

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 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]
  • Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann Rheum Dis . 2013 Apr. 72 Suppl 2:ii76-9. [Medline] .[emedicine.medscape.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]
  • 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]
  • 5] Causes [ edit ] 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[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]
  • […] often glomerulonephritis (in up to 50% of cases) cardiac effects: pericarditis or myocarditis pulmonary effects: pleurisy, pneumonitis hematologic effects: anemia, leukopenia, or thrombocytopenia constitutional symptoms: fever, fatigue, and myalgia Pathophysiology[epilepsy.com]

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]
  • Prevention Treating the metabolic disorder may reduce the risk of developing this type of dementia. The high mineral content can soak directly into the skin and reduce inflammation and pain, which are two extremely common symptoms of lupus.[sheilaobrien.club]
  • 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]

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.

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Last updated: 2018-06-22 08:37