Agent | Mode of action | Dose | Common side effects | Comments |
---|---|---|---|---|
Acetazolamide | ▸ Cabrbonic anhydrase inhibitor. ▸ May reduce CSF production by reducing sodium ion transport from the choroid plexus cells | ▸ Prepubertal children: start at 25 mg/kg/d in 2–3 divided doses and increase by 25 mg/kg/d to a maximum of 100 mg/kg/day. ▸ Adolescents: start at 1 g/day in 2–3 divided doses and if needed increase by 250 mg/d till a maximum of 4 g/day.12 | ▸ Metallic taste, tingling sensation in the mouth, paraesthesia and nausea. ▸ Routine urea and electrolytes and blood gases are not recommended unless high doses are used. | ▸ Used as firstline treatment. ▸ Success rate quoted in paediatric case reports ranged between 47% and 67%.12 |
Topiramate | Weak carbonic anhydrase inhibitor effect and analgesic | Start at 25 mg at night and increase by 25 mg weekly. Maximum dose is typically 100–150 mg.28 | Word finding difficulties, psychomotor slowing, impaired concentration anorexia, weight loss and sedation. | ▸ In an open-label study in adults, topiramate was as effective as acetazolamide with the added benefit of reducing weight.28 ▸ May work better as an ‘add-on’ to acetazolmaide |
Furosemide | Probably by diuresis and reduction of sodium transport into the brain | 1–2 mg/kg/day with or without acetazolamide.12 | Electrolyte disturbances, postural hypotension | ▸ Usually reserved as second-line therapy due to risks of electrolytes disturbance on prolonged use. |
Bumetanide | Diuretic, Inhibition of Na+-K+-2Cl− co-transport within choroid plexus | 0.25 mg daily29 | As for furosemide | Used as monotherapy in a single paediatric case report29 |
Steroids | Unclear | ▸ Prednisolone: 1–2 mg/kg/day. ▸ Dexamethasone: 2 mg qds or 0.1–0.75 mg/kg/day in 4 divided doses11 | Weight gain, immunosuppression, endocrine disturbances | ▸ Used as a short-term measure (with acetazolamide) in patients presenting with severe visual failure while awaiting surgical intervention.30 ▸ Chronic use not advisable. |
CSF, cerebrospinal fluid.