Article Text

PSEUDOTUMOR CEREBRI IN PREPUBERTAL CHILDREN: WHAT SHOULD WE DO?
  1. J Galhardo1,
  2. M Costa1,
  3. B Pereira1,
  4. C Moreira1,
  5. S Guimarães2,
  6. P Beleza3,
  7. C Ferreira3,
  8. A Gonçalves1
  1. 1Pediatrics Department, Hospital De São Marcos, Braga, Portugal
  2. 2Ophthalmology Department, Hospital De São Marcos, Braga, Portugal
  3. 3Neurology Department, Hospital De São Marcos, Braga, Portugal

Abstract

Background Pseudotumor cerebri (PC) is characterized by intracranial hypertension (IH) without any evident underlying neurological disease. The majority of cases occur in childbearing age obese women. On rare occasions, PC affects prepubertal children and is associated with different patient features, greater clinical spectrum and special epidemiological context.

Case Reports A 5 and a 6-year-old boy, with a previous upper respiratory tract infection, were brought to the ER with severe headaches and vomiting on awakening, followed by sixth pair paresia and horizontal diplopia. One also presented unilateral facial nerve palsy and torticollis. Fundus examination revealed bilateral papilledema. Blood pressure, visual acuity, field and color vision were all normal. None had ataxia or abnormal tendon reflexes. Cerebrospinal fluid opening pressures were 24 cm H2O and 50 cm H2O. Computed tomography scan and magnetic resonance venography only detected IH signs. There was no history of recent vaccination, head trauma, drugs or toxics. Infectious, endocrine, thrombotic and immune possible causes were excluded. Both improved after acetazolamide with or without prednisolone. Treatment and follow-up are discussed.

Discussion Although earlier thought as a paediatric benign condition, it is now clear that vision impairment can be permanent. By contrast, recent data revealed a 40% 5-year recurrence. By this way, an interdisciplinary management and a close long-term ophthalmologic surveillance are of primordial importance in patients with PC.

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