Juvenile systemic lupus erythematosus with primary neuropsychiatric presentation

Abstract

SUMMARYSystemic lupus erythematosus (SLE) is an autoimmune disease with multiple manifestations in several organs and systems. Neuropsychiatric manifestations can occur in 22-95% of paediatric cases, being much less frequent as an initial clinical event. We report a case of SLE, presenting primarily with neuropsychiatric symptoms. An African-descendant 7-year-old girl was admitted with a 4-day history of ataxia, diplopia and morning vomiting, as well as severe headache, psychiatric symptoms and cognitive dysfunction beginning 1 year prior to admission. Brain MRI was suggestive of encephalitis. Investigation excluded infectious aetiology. Immunological markers revealed high titre of antinuclear and anti-double-stranded DNA antibodies. Neuropsychiatric lupus (NPL) was considered, and cyclophosphamide and methylprednisolone pulses were started, with good initial response. Clinical deterioration motivated therapy with azathioprine with subsequent clinical stabilisation and a latent cognitive dysfunction. In unusual encephalitis presentation, a wide range of differential diagnosis has to be considered. Primary NPL presents difficult diagnostic and therapeutic challenges. BACKGROUND

Depression as the First Manifestation in a Young Girl With Juvenile Systemic Lupus Erythematosus

“…Neuropsychiatric manifestations can occur in 22-95% of pediatric cases, but are much less frequent as an initial clinical event, rarely leading to a diagnosis of JSLE. [1][2][3] Depression can be a symptom of active JSLE or it can occur as a reaction following diagnosis of chronic illness. [4][5][6][7] A 12-year-old girl was admitted with a history of sadness, decreased interest in activities, poor communication, and absences from school, fatigue, irritable moods, and arthralgia in her hands.…”

Early-onset neurolupus: A challenge for pediatricians

“…Depending on the part of the CNS involved, symptoms and manifestations are variable and can occur in 22-95% of pediatric cases. However, they are rare as an initial presenting complaint [2,6]. Our patient, five-year old female, is interesting in that her disease started at quite an early age.…”

“…The median age at onset of symptoms in pediatric SLE is between 11-12 years, with a female predominance being pronounced after puberty [1]. Neuropsychiatric manifestations can occur in 22-95% of pediatric cases, being much less frequent as an initial presenting symptom [2]. Symptoms of central nervous system (CNS) involvement range from poor school performance, depressive moods and difficulty to concentrate up to seizures, psychosis and stroke.…”

Leukoencephalopathy and cerebral edema as the presenting manifestations of SLE in an ANA-negative adolescent female: A case report and review of literature.

“…Instead, she spiked fevers intermittently. According to the literature, the finding of diffuse cerebral edema with or without leukoencephalopathy in NPSLE is extremely rare, and if present, As shown in Table 1, most of the case reports reviewed described intracranial hypertension with or without leukoencephalopathy (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). Of the three cases that were children (ages, 7y, 11y, and 14y), Interestingly, our patient is also unique in that she was diagnosed with SLE but had a negative ANA test noted on two occasions during her hospitalization and again after discharge.…”