Background and Aims Central nervous system involvement in paediatric systemic lupus erythematosus (SLE) is common, however, normal-pressure hydrocephalus is unusual.

Methods The medical records of a nine-year old Filipino female with SLE was reviewed.

Results Patient presented with prolonged fever, weight loss, generalised weakness, lapses in memory, and mood lability. She was poorly nourished, non-ambulatory, pale, febrile, and ill-looking. She was tachycardic, with normal blood pressure, decreased breath sounds over lung bases, a systolic murmur over the apex, and significant contractures on elbows and knees. She was coherent, oriented to all spheres, with intact cranial nerves, but with impaired recall. Upper extremity muscle testing was 4/5, while that of the lower extremities were 3/5. There was severe muscle atrophy, no fasciculations or other involuntary movements, no sensory deficits, no abnormal reflexes, and no signs of meningeal irritation. No oral ulcers, joint swelling, nor rash was noted. Work up showed anaemia, leukopenia, positive ANA, and positive anti-dsDNA. Serum electrolytes and urinalysis were normal. Chest radiograph showed interstitial infiltrates, while Mantoux test was negative. Cranial magnetic resonance imaging with contrast showed abnormal white matter intensities at the fronto-parietal, periventricular, and subcortical regions, communicating hydrocephalus, and cortical cerebral and cerebellar atrophy. Cerebrospinal fluid analysis was normal. She showed remarkable improvement with intravenous antibiotics and corticosteroids. Upon discharge, she had good well-being, appetite, cognition, and mood, and was already able to ambulate with assistance. She received cyclophosphamide pulse therapy and underwent physical rehabilitation.

Conclusions Normal-pressure hydrocephalus may be part of the presenting manifestations of paediatric SLE.