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.
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  , 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 , 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  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   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
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]
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]
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 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]
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 second case manifested with an attack of generalized seizure after suffering from low-grade intermittent fever and joint pains for a duration of one-and-a-half months. [ncbi.nlm.nih.gov]
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]
Renal Artery Stenosis
Cardiology was consulted to help manage this complication and recommended imaging of her abdomen for possible pheochromocytoma and renal artery stenosis. [healio.com]
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]
Physical examination revealed slow speech and cognition, tachycardia with systolic murmur, nontender cervical adenopathy, splenomegaly, slight tremor noted in left hand, and decreased strength in both upper limbs. [healio.com]
The patient presented with fluctuating mentation, fatigue, psychomotor retardation, anorexia (with 16 lbs of weight loss), dysarthria with word-finding difficulty, slow speech, and unsteady gait. [healio.com]
On examination, the patient presented as nonverbal, unable to follow commands, and exhibiting hypokinesis, catalepsy, waxy flexibility, negativism, echopraxia. [healio.com]
Face, Head & Neck
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]
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]
Thromboembolic strokes occur in a small percentage of patients due to the involvement of blood vessels of all sizes. [symptoma.com]
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]
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]
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. You may have noticed along the way that I am kind of interested in art. [thislupuslife.com]
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]
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]
We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigitidy. [link.springer.com]
Imbalance while walking, dysarthria, nystagmus, and ataxic gait with positive Romberg's test suggested cerebellar involvement. Evaluation revealed CSF abnormalities indicative of aseptic meningitis. [ijpmonline.org]
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 . 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  with lymphocytotoxic antibodies being seen in a majority of patients with LC . Other CSF findings in these patients are lymphocytosis with an elevated cell count, high levels of proteins , 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 .
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 . 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 .
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.
- 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.