A case of neuropsychiatric lupus with myelopathy successfully treated with corticosteroids

Salmi, Homeed H; Shalaby, Medhat; Ageely, Hussein; Moustafa, Fayza

doi:10.4314/wajm.v23i2.28113

Cited by 3 publications

(4 citation statements)

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“…This patient showed recovery from hypotonia grade 1-2/5 right and 4/5 left to 4/5 bilaterally at hospital discharge (partial improvement). 17 No information on MRI at follow-up is available in this report. In the other MRI-/CSFpatient, sMRI showed abnormalities at follow-up: spinal atrophy was visible after 1 year.…”

Section: Case Reportsmentioning

confidence: 99%

“…Of the 14 patients with normal sMRI, CSF was available for 11 patients. [11][12][13][14][15][16][17][18][19][20][21][22] 9/11 showed abnormalities in CSF: pleocytosis (4/10), increased protein (7/11), low glucose (3/9), oligoclonal bands (2/6) and an increased IgG index (3/3). Only two patients were MRI-/CSF-.…”

Section: Case Reportsmentioning

confidence: 99%

“…Only two patients were MRI-/CSF-. 17,22 One MRI-/CSF-patient was treated with MP 1000mg IV for 3 days followed by prednisone 1mg/kg for 1 month. This patient showed recovery from hypotonia grade 1-2/5 right and 4/5 left to 4/5 bilaterally at hospital discharge (partial improvement).…”

Section: Case Reportsmentioning

confidence: 99%

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Suspected Transverse Myelitis with Normal MRI and CSF Findings in a Patient with Lupus: What to Do? A Case Series and Systematic Review

Monahan

Beaart

Fronczek

et al. 2020

NDT

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Purpose To evaluate the use of immunosuppressive treatment, clinical outcome and diagnostic strategy in patients with systemic lupus erythematosus (SLE) presenting with clinical features of transverse myelitis (TM), but normal MRI of the spinal cord (sMRI) and normal cerebrospinal fluid (CSF) assessment, and to suggest a clinical guideline. Patients and Methods All patients with SLE and clinical features compatible with (sub)acute TM visiting the NPSLE clinic of the LUMC between 2007 and 2020 were included. Information on baseline characteristics, investigations, treatment and outcomes was collected from electronic medical records. In addition, a systematic review of individual participant data was performed up to April 2020 in PubMed, Embase and Web of Science, identifying all patients with TM, SLE and sMRI assessment. Data regarding sMRI, CSF analysis, treatment and outcome were extracted, and outcome was compared between patients with normal sMRI and CSF (sMRI-/CSF-) and patients with abnormalities. Results Twelve SLE patients with a clinical diagnosis of TM were identified: four sMRI-/CSF- and one sMRI- with CSF not available. All patients received immunosuppressive treatment, but outcome in sMRI-/CSF- patients was worse: no recovery (n=1) or partial recovery (n=3) compared to partial recovery (n=4) and (nearly) complete recovery (n=3) in MRI+ patients. The systematic literature review yielded 146 articles eligible for inclusion, 90% case reports. A total of 427 SLE patients with TM were identified, of which only four cases were sMRI-/CSF- (1%), showing no improvement (n=1), partial improvement (n=2) and complete recovery (n=1) after immunosuppressive treatment. Conclusion Outcome in SLE patients presenting with clinically suspected TM with normal sMRI and CSF is less favorable, despite treatment with immunosuppressive therapy. Taking a functional neurological disorder into consideration may be helpful in order to start other therapeutic strategies. We suggest prescribing immunosuppressive treatment for a restricted period of time to evaluate its effect in cases where a functional disorder initially is considered unlikely.

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Section: Case Reportsmentioning

confidence: 99%

Section: Case Reportsmentioning

confidence: 99%

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Suspected Transverse Myelitis with Normal MRI and CSF Findings in a Patient with Lupus: What to Do? A Case Series and Systematic Review

Monahan

Beaart

Fronczek

et al. 2020

NDT

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“…Overall transverse myelitis, peripheral neuropathy and aseptic meningitis each occur in approximately 1-5% of patients. 5,7,36 Aseptic meningitis must be considered in the differential diagnosis of all patients who present with headache. Less commonly, PNS involvement includes polyneuropathy, mononeuritis, myasthenia gravis, cranial neuropathy, demyelinating disease, and Guillain-Barrè syndrome.…”

Section: Peripheral Nervous Systemmentioning

confidence: 99%

Review: Neuropsychiatric involvement in pediatric systemic lupus erythematosus

Benseler

2007

Lupus

153

111

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Neuropsychiatric (NP) manifestations are found in approximately 25% of children and adolescents with pediatric SLE (pSLE). In 70% of those, NP involvement will occur within the first year from the time of diagnosis. Headaches (66%), psychosis (36%), cognitive dysfunction (27%) and cerebrovascular disease (24%) are the most common presentations. The support of a psychiatrist is often required. Anti-phospholipid antibodies are associated with distinct NP disease entities and may be implicated in the pathogenesis of several manifestations of NP-pSLE including chorea, cerebrovascular disease and seizures. The role of novel auto-antibodies and imaging modalities is currently explored. The treatment of NP-pSLE is not based on prospective studies; however, an immunosuppressive combination therapy consisting of high doses of prednisone and a second line agent such as cyclophosphamide or azathioprine is commonly suggested for children with NP-pSLE. The role of novel therapies is currently studied. The outcome of children with NP-pSLE is relatively good. The overall survival is 95-97%, 20% of children experience a disease flare during childhood and 25% have evidence of permanent neuropsychiatric damage.

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Clinical value of autoantibodies for lupus myelitis and its subtypes: A systematic review

Oiwa

Kuriyama

Matsubara

et al. 2018

Seminars in Arthritis and Rheumatism

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A case of neuropsychiatric lupus with myelopathy successfully treated with corticosteroids

Cited by 3 publications

References 0 publications

Suspected Transverse Myelitis with Normal MRI and CSF Findings in a Patient with Lupus: What to Do? A Case Series and Systematic Review

Suspected Transverse Myelitis with Normal MRI and CSF Findings in a Patient with Lupus: What to Do? A Case Series and Systematic Review

Review: Neuropsychiatric involvement in pediatric systemic lupus erythematosus

Clinical value of autoantibodies for lupus myelitis and its subtypes: A systematic review

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