Neurologic Symptoms in Children With Systemic Lupus Erythematosus

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.

Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system

“…Prior series have reported comparative rates of 0-28% 27,31 . To the best of our knowledge, seizure semiology has not been described in detail in pediatric SLE series.…”

“…Pediatric series report prevalences of 7-10% 26,31 , 5% 25 and 2-3.5% 26,31 respectively at disease presentation. These rates rise to 10-20% for headache and 6.6-12% for stroke during the course of the disease 27,30,35 .…”

Generalized chorea as the first presentation of systemic lupus erythematosus: case report and review of literature

“…4,[7][8][9] Other less common features include cranial neuropathies, transverse myelitis, meningitis, and movement disorders which occur in less than 5% of patients and include chorea, ataxia, choreoathetosis, dystonia, and hemiballismus. [10][11][12] Chorea accounts only for about 2% of all NPSLE manifestations, which qualified it as a clinical diagnostic challenge. 12 In practice, the differential diagnosis of chorea is wide and include a long list of hereditary and non-hereditary disorders.…”

“…[10][11][12] Chorea accounts only for about 2% of all NPSLE manifestations, which qualified it as a clinical diagnostic challenge. 12 In practice, the differential diagnosis of chorea is wide and include a long list of hereditary and non-hereditary disorders. [13][14][15] Acquired causes include drugs and toxins, Sydenham chorea, SLE and antiphospholipid (APL) syndrome, chorea gravidarum, stroke and hyperthyroidism.…”

Introduction and Physiology of Lupus

“…In this disease, major organ systems are involved and the patient presents with neuropsychiatric complications [88,[94][95][96][97][98][99].…”