Mycophenolate mofetil-induced pseudotumor cerebri in a boy with autoimmune lymphoproliferative disease

Childs Nerv Syst. 2011 May;27(5):853-5. doi: 10.1007/s00381-011-1402-4. Epub 2011 Feb 9.

Abstract

Introduction: Pseudotumor cerebri (PTC) is a syndrome characterized with increased intracranial pressure, normal cerebrospinal fluid content (CSF), and a normal brain on imaging studies. In this case report, PTC has been linked to mycophenolate mofetil (MMF) that has been used for autoimmune lymphoproliferative syndrome (ALPS).

Case report: A 5-year-old boy, who was using MMF for 4 months because of the ALPS, suffered from occipital headache and vomiting with no other symptom. The initial physical examination was normal expect bilateral papilledema. The patient underwent a lumbar puncture which showed elevated opening pressure (590 mmH₂O) but no laboratory abnormalities of the CSF. A diagnosis of PTC was established. MMF was stopped, and the child was started on an acetazolamide treatment for 2 weeks. His symptoms and complaints recovered after this treatment.

Discussion: According to our knowledge, we report the first case of MMF-induced PTC in a boy with ALPS. This case illustrates that despite the rarity of MMF-induced PTC, the physicians should be aware of this possibility. Furthermore, in the setting of new-onset headaches or visual changes, early ophthalmologic examination for papilledema is recommended for early diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Acetazolamide / therapeutic use
  • Autoimmune Lymphoproliferative Syndrome / drug therapy*
  • Autoimmune Lymphoproliferative Syndrome / physiopathology
  • Carbonic Anhydrase Inhibitors / therapeutic use
  • Child, Preschool
  • Humans
  • Immunosuppressive Agents / adverse effects*
  • Male
  • Mycophenolic Acid / adverse effects
  • Mycophenolic Acid / analogs & derivatives*
  • Pseudotumor Cerebri / chemically induced*
  • Pseudotumor Cerebri / therapy
  • Spinal Puncture

Substances

  • Carbonic Anhydrase Inhibitors
  • Immunosuppressive Agents
  • Mycophenolic Acid
  • Acetazolamide