A child with recurrent headache, fever and diffuse meningeal enhancement on MRI

Juvenile neurolupus presents primarily with neuropsychiatric manifestations which may also be the initial presentation. Such primary neuropsychiatric SLE (NPSLE) events are a consequence either of microvasculopathy and thrombosis, or of autoantibodies and inflammatory mediators. Diagnosis of NPSLE r...

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Bibliographic Details
Published in:Clinical rheumatology 2024-10, Vol.43 (10), p.3249-3252
Main Authors: Khatua, Pratiksha, Bathia, Jigna, De, Hriday, Pal, Priyankar
Format: Article
Language:English
Subjects:
anemia
Autoantibodies
brain
Brain - diagnostic imaging
Brain - pathology
Case Based Review
Cerebrospinal fluid
Child
children
Diagnosis
Diagnosis, Differential
DNA
electrophysiology
Female
Fever
Functional anatomy
Globulins
Headache
Headache - diagnostic imaging
Hemorrhage
Humans
Image processing
Inflammation
juveniles
Leukocytes (neutrophilic)
Lupus Vasculitis, Central Nervous System - diagnostic imaging
Magnetic Resonance Imaging
Male
Medicine
Medicine & Public Health
Meninges - diagnostic imaging
Meninges - pathology
Meningitis
Meningitis - diagnostic imaging
Migraine
Neuroimaging
patients
Recurrence
Rheumatology
Structure-function relationships
Thrombocytopenia
Thrombosis
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Description
Summary:Juvenile neurolupus presents primarily with neuropsychiatric manifestations which may also be the initial presentation. Such primary neuropsychiatric SLE (NPSLE) events are a consequence either of microvasculopathy and thrombosis, or of autoantibodies and inflammatory mediators. Diagnosis of NPSLE requires the exclusion of other causes, and clinical assessment directs the selection of appropriate investigations. These investigations include measurement of autoantibodies, analysis of cerebrospinal fluid, electrophysiological studies, neuropsychological assessment and neuroimaging to evaluate brain structure and function. In our patient, the disease presented with chronic headache initially diagnosed as migraine, followed by fever and paraparesis. Fundoscopy showed retinal haemorrhages. Investigations revealed anaemia, neutrophilic leucocytosis, thrombocytopenia and raised inflammatory markers (ESR 119 mm/h CRP 58 mg/L) and high globulin. MRI brain showed diffuse meningeal enhancement resembling meningitis but CSF analysis was normal. ANA and dsDNA were positive with low C3, C4. All diffuse meningeal enhancements may not be meningitis and one needs to corroborate all the clinical, biochemical and imaging analyses to come to a diagnosis.
ISSN:0770-3198
1434-9949
1434-9949
DOI:10.1007/s10067-024-07030-2