High Intracranial Pressure in Retinitis Pigmentosa (RP)

Purpose: To check intracranial pressure (ICP) in patients who have RP, to see association of Pseudotumor Cerebri (PTC) to RP, and to prevent further visual loss due to secondary optic neuropathy in these patients Methods: Nine patients who were known cases of RP, came to check if there are any new method of treatment for their retinal disease. All of them claiming recently developed visual loss. Optical coherence tomography (OCT) showed micro-papilledema and peri-papillary nerve fiber layer thickening in all of patients. Next step was obtaining MRI of brain which showed no intracranial mass, but mild (milder than Dandy Criteria) evidences of high intracranial pressure. So, patients were referred to neurologist for lumbar puncture to check the intracranial pressure. Also, our routine systemic work up for optic neuropathy, including serum level of vitamins A, D and B12 had been done in all of patients Results: : Four of our nine (45%) cases were male. Seven out of nine (78%) had frequent headaches in their past medical history. Seven (78%) were overweight or obese. In all (100%) of our patients intracranial pressure was significantly higher than normal.Cerebrospinal fluid (CSF) opening pressure ranges were 26 to 49 cm H2O. All cases were treated with oral acetazolamide.Eight (89%) of cases had adequate follow-up and most of these had some visual improvement Conclusion: According to our finding, we suggest that patients with Retinitis Pigmentosa should have work up for high intracranial pressure including OCT of peri-papillary nerve fiber layer (NFL) and macula, looking for any thickening or thinning of NFL and/or ganglion cell layer( GCL). We have established that one major cause of vision loss in RP patients is optic neuropathy secondary to associated PTC, which if undiagnosed may destroy their optic nerves and make them poor candidates for retinal implants