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

Abstract

OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%): seizures (17 / 36.2%), intractable headache (7 / 14.9%), mood disorders (5 / 10.6%), cerebrovascular disease (4 / 8.5%), acute confusional state (3 / 6.4%), aseptic meningitis (3 / 6.4%), psychosis (3 / 6.4%), chorea (3 / 6.4%), Guillain-Barré syndrome (2 / 4.3%) and cranial neuropathy (1 / 2.1%). Morbidity indexes (SLEDAI and SLICC) were higher among patients with neuropsychiatric manifestations (p<0.05). CONCLUSION: Neuropsychiatric syndromes are frequent, and add significant morbidity to juvenile systemic lupus erythematosus.

Antiphospholipid-Related Chorea

“…In Cervera’s study, 58% of patients suffered from defined SLE, 12% had lupus-like syndrome, and 30% were affected by PAPS (Cervera et al, 1997). Among SLE patients, 1–3% of adults and 9% of children present choreic movements (Meyer and Kahn, 2000; Joseph et al, 2007; Spinosa et al, 2007). Chorea generally starts within the first year after the onset of SLE, and often precedes the clinical diagnosis (Asherson et al, 1987; Baizabal-Carvallo et al, 2011).…”

Mixed movements disorders as an initial feature of pediatric lupus

“…Acute psychiatric features are seen in 46.7-59% of pSLE [6]. Mood disorders and psychotic events may reveal the disease or appear later.…”

An Unusual Presentation of Lupus in a Pediatric Patient

“…Neuropsychiatric features though not so common in adult population are much more prevalent in Juvenile onset SLE [10, 11] with headache and seizures being the most common presenting features.…”

“…Case reports of chorea as a presenting feature of SLE have been documented in the literature [12, 13]. The prevalence of chorea is variable with different studies and can account for 2% to 6% of neurological manifestations in SLE [10, 11]. …”