“We had both a neuro ophthalmologist and a neuroradiologist who were noticing that more cases of peri-optic neuritis seemed to be occurring at our institution," said the lead author Nicholas Benjamin, MD, a neurology fellow at the University of Pittsburgh.
“We looked into the literature and found very little in pediatric cases specifically," he told Neurology Today At the Meetings. “We decided to search for all the cases [occurring between January 2017 and March 2024] we could identify within our center."
To find potential cases of pediatric peri-optic neuritis, Dr. Benjamin and colleagues searched the ICD-10 codes for diagnoses including perineuritis, optic neuritis (ON), optic papillitis, retrobulbar neuritis, other ON and unspecified ON. For all cases, they reviewed documented interpretations of MRI brain/orbit imaging by radiology. They then reviewed imaging with a neuroradiologist to confirm the diagnosis.
“We began by looking at all patients with peri-neuritis on imaging and then went backwards to look at treatment they received," Dr. Benjamin said. “Many of these cases also demonstrated optic neuritis as well on imaging concurrently, so they were dual treated for these, as the primary teams were more often treating the optic neuritis picture, as they are more familiar with this etiology. As a result, a number of our reviewed cases did not mention peri-neuritis on imaging reads, but upon our look, clearly had peri-neuritis present."
Dr. Benjamin and colleagues ultimately identified 20 cases, nine of whom were females, with a median age at diagnosis of nine years.
Seventy five percent of the patients presented with changes in vision, headaches (45 percent), or painful eye movements (40 percent).
The most common diagnoses after review of imaging were idiopathic ON (in 35 percent), myelin oligodendrocyte glycoprotein (MOG)+ ON (in 35 percent), and inflammatory P-ON without ON (in 10 percent).
None of the patients was diagnosed with either multiple sclerosis of NMO. “I found that a little surprising and unexpected," Dr. Benjamin said. Seventeen of the 20 patients were tested for myelin oligodendrocyte glycoprotein antibody disease (MOGAD), seven of whom (41 percent) were positive.
Initial treatment for 18 of the patients was intravenous steroids, followed by oral steroids.
“Adults with isolated peri-optic neuritis can sometimes get away with receiving only oral steroids," Dr. Benjamin said. “Our findings suggest that kids seem to do well when they receive IV steroids followed by an oral course." However, many cases in this study had concurrent ON and perineuritis present at the same time, and received treatment for both of these conditions.
Only two of the patients required therapeutic escalation to plasmapheresis or the immunosuppressant drug rituximab.
Four patients experienced documented relapses. At follow-up, ranging from three to 10 months, all 16 patients with available data had stable or improved visual acuity.
“This is just a case series, “but it shows that IV steroids followed by oral steroids may be a good regimen for the pediatric population," Dr. Benjamin said.
Stronger evidence for the best treatment options would require a randomized trial, but doing so would be unlikely for a single institution due to the condition's rarity, Dr. Benjamin said.
“It could be done, but it would likely take multiple institutions collaborating," he said.
A neuroimmunologist familiar with the study said she was glad to see its focus on pediatric cases but hopes that larger studies will eventually be available.
“It's a small case series from a single site," said Jennifer Olson Graves, MD, PhD, professor of neurosciences and division chief of neuroimmunology at the University of California, San Diego, as well as director of the Rady Children's Pediatric MS Center in San Diego.
“Consistent with the adult literature, there were no MS cases with perineuritis," she said. “I teach people that if you see peri-optic neuritis, it's never MS."
She was surprised that only seven of the 17 cases tested for MOGAD were positive, and that only one-third of the cases were positive for MOG.
“We need to think of this as a frequency series in a tertiary care hospital, not a population-based study," Dr. Graves said. “It may be referral bias that most of their cases weren't MOG. I would be curious to know whether the ones who had to escalate were MOG positive or not. I would expect the MOG cases to be responsive to steroids."
Dr. Graves said that in her experience, “kids with MOGAD have about a 50 percent chance of relapse. For the idiopathic cases, it's hard to predict. It's possible that it would be a one-time event and never recur."
Disclosures: Dr. Benjamin had no disclosures.
https://journals.lww.com/neurotodayonline/blog/NeurologyTodayConferenceReportersAANAnnualMeeting/pages/post.aspx?PostID=213
Children with Peri-Optic Neuritis Have Favorable Visual Outcomes After Steroid Treatment: A Singe-Center Pediatric Case Series
Nicholas Benjamin1, Srikala Narayanan2, Catalina Cleves1, Levi Shelton1
1Children's Hospital of Pittsburgh of UPMC, 2Texas Children’s Hospital
Objective:
To determine baseline characteristics and outcomes in pediatric patients presenting with peri-optic neuritis (P-ON).
Background:
P-ON is a rare inflammatory disorder leading to inflammation of the nerve sheath. Most literature addresses adults, with little information on the pediatric population.
Design/Methods:
We conducted a single-center retrospective study of patients presenting to UPMC Children’s Hospital from 01/2017 – 03/2024. We used ICD-10 codes (optic neuritis (ON), optic papillitis, retrobulbar neuritis, other ON, unspecified ON) to identify potential patients with P-ON. For identified patients, we reviewed documented interpretations of MRI brain/orbit imaging by radiology. For patients with interpretations suspicious for P-ON, we reviewed their imaging with a neuroradiologist to confirm the diagnosis. For those patients, we collected baseline characteristics and outcomes.
Results:
We identified 20 cases of P-ON. The median age at diagnosis was 9 years, and 9 patients (45%) were female. Most patients presented with vision changes (75%), headaches (45%), and painful eye movements (40%). Initial ophthalmologic findings included disc edema (55%) and disc pallor (30%). The most common diagnoses (all with confirmed P-ON on imaging) were idiopathic ON (35%), MOG+ ON (35%), and inflammatory P-ON without ON (10%). No patients were diagnosed with MS or NMO. Of the 17 patients tested for MOGAD, 7 (41%) were positive. Eighteen (90%) patients initially received intravenous (IV) steroids, followed by oral steroids. Two (10%) patients had escalation of therapies to plasmapheresis or Rituximab. Four (20%) patients had documented relapses. Steroid therapy produced stable or improved visual acuity at follow-up (ranging from 3-10 months) in all patients (100%) with data present (n=16).
Conclusions:
MOG+ status was common among patients with P-ON, along with ON cases. Regarding visual outcomes, all patients either had stable or improved visual acuity at follow-up, showing that the regimen of IV to oral steroids may be a good treatment option in the pediatric population.
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