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Neurologic symptoms in children with systemic lupus erythematosus
A I Quintero-Del-Rio1, Van Miller
1Department of Pediatrics, The University of Texas Southwestern Medical Center at Dallas, USA. Ana-Quintero@ouhsc.edu
Insights
Neurologic complications in childhood systemic lupus erythematosus (SLE) are common, with seizures and headaches being frequent symptoms. Early diagnosis and treatment, often with high-dose steroids, generally lead to good outcomes in pediatric lupus cerebritis.
Area of Science:
- Pediatric Neurology
- Rheumatology
- Systemic Lupus Erythematosus (SLE)
Background:
- Neurologic complications of systemic lupus cerebritis are less understood in pediatric patients compared to adults.
- This study reviewed medical records of 86 children with SLE to identify those with neurologic complications.
Observation:
- Twenty-five children experienced neurologic complications, with 28% showing symptoms at SLE diagnosis.
- The median time from SLE diagnosis to neurologic complication onset was 1 month.
- Seizures were the most common neurologic symptom, particularly in those with established SLE diagnoses.
Findings:
- Headaches were the most frequent initial neurologic manifestation in children without a prior SLE diagnosis.
- Other observed complications included lupus cerebritis, stroke, cranial neuropathies, chorea, and pseudotumor cerebri.
- Most seizures were manageable, with no cases of status epilepticus.
Implications:
- High-dose intravenous methylprednisolone showed good response in 18 children.
- Cyclophosphamide and plasmapheresis were used in some cases.
- While outcomes were generally favorable, one case of fulminant cerebritis resulted in death, highlighting the potential severity.
Abstract:
Neurologic complications of systemic lupus cerebritis are not as well known in children as in adults. Twenty-five children with neurologic complications were identified after reviewing the hospital medical records of 86 children with systemic lupus erythematosus. Seven children (28%) had neurologic symptoms at the time of initial diagnosis of systemic lupus erythematosus; median time between diagnosis of systemic lupus erythematosus and onset of neurologic complications was 1 month (range 0-5 years). Seizures were the most common neurologic symptoms overall, but headaches were the most frequent neurologic manifestation in children without a previous diagnosis of systemic lupus erythematosus. Sixteen children had seizures, and 12 children had seizures as the initial central nervous system involvement. Almost all children who developed seizures had an established diagnosis of systemic lupus erythematosus; only one child had seizures that led to the diagnosis of systemic lupus erythematosus. No patient had status epilepticus, and, in general, seizures were not difficult to control. In six children, headache was the initial symptom of central nervous system involvement. Five children had lupus cerebritis, three children had stroke, and two had isolated cranial neuropathies. Chorea was seen in only two cases, and three children had pseudotumor cerebri. Treatment with high-dose intravenous methylprednisolone led to a good response in 18 children; cyclophosphamide was required in 6 patients and plasmapheresis in 1 child. Outcome was generally good, although one child developed fulminant cerebritis with intracranial hypertension and died.