Juvenile systemic lupus erythematosus with primary neuropsychiatric presentation.
Systemic 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.
Department of Pediatrics, Hospital Nossa Senhora do Rosário, Centro Hospitalar Barreiro-Montijo, Barreiro, Portugal. neliaferraria@gmail.com
Nélia
N
Ferraria
Susana
S
Rocha
Vera Silva
VS
Fernandes
Teresa
T
Correia
Elisabete
E
Gonçalves
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000097:blood; D001259:Ataxia / Q000209:etiology; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D003072:Cognition Disorders / Q000209:etiology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004172:Diplopia / Q000209:etiology; D004660:Encephalitis / Q000175:diagnosis; D005260:Female; D006261:Headache / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020945:Lupus Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D001523:Mental Disorders / Q000209:etiology*; D008775:Methylprednisolone / Q000627:therapeutic use; D014839:Vomiting / Q000209:etiology
D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine; D008775:Methylprednisolone