Presumed PTCS and optic atrophy

2. RE: Presumed PTCS and optic atrophy

Recommend

Eric Singman

Posted 03-26-2025 05:43

Fascinating case.

MRV available?
MRI orbits updated?
Optic nerve damage sounds like NA AION
Sheath fenestration considered?

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Original Message

Original Message:
Sent: 3/25/2025 11:04:00 PM
From: Enrique Rivera
Subject: Presumed PTCS and optic atrophy

Dear colleagues,

I would appreciate your help regarding future management of the following patient.

12 year old boy with history of presumed pseudotumor cerebri syndrome presenting with right optic nerve atrophy.

Pertinent history:

May 2016 - 4 year-old boy developed headaches and blurred vision. He was found to have bilateral optic nerve swelling by a pediatric neurologist. Pediatric neurology notes also document pansinusitis and otitis media associated with the reported symptoms. LP was done and ICP was 23 cmH2O without CSF abnormalities. This was interpreted and increased ICP and the patient was treated with IV Solumedrol and Diamox. He was discharged from the hospital on Diamox. Subsequently, he was seen by a pediatric ophthalmologist who confirmed bilateral ODE (right more than left). Also present were a right CN6 palsy that resolved during follow-up and a right CN4 palsy that required surgery. Lab work-up was unrevealing. Diamox was discontinued after 1 year of treatment and resolution of ODE.

October 2017 - Asymptomatic patient was found to have ODE on a routine examination by his pediatric ophthalmologist. Swelling was worse than during presentation, but lumbar puncture showed an ICP of 13 cmH2O. Despite a normal ICP, Diamox was re-started and slowly tapered over the next 2 years.

2021-2023 - Normal eye exams.

In June 2024 the patient presented to his pediatric ophthalmologist for a routine evaluation. He had no symptoms at the time. A slight drop in vision to 20/25 was noted in the right eye. His color vision was intact, he had normal stereo and a trace APD that could be reversed with a 0.3 LU NDF. OCT of the nerve revealed a drastically depressed RNFL and loss in the GCC with a hint of altitudinal damage (see attached images) on the right eye. Other than scattered misses on HVF, the patient had no visual field defects. Neuroimaging and lab testing, including anti-MOG and anti-AQP4, were unremarkable. ICP was 32.5 cmH2O with a normal CSF panel. No cytologic abnormalities were found in the CSF.

The patient is currently on Diamox.

Given the information presented:

- How should I manage this patient with chronic, asymptomatic ICP elevation and no findings on exam to help gauge when the ICP is elevated? Should he continue chronic use of Diamox? Would a shunt be the best long-term option?

- Is the nerve atrophy a sign of ongoing disease or the result of past damage from previous episodes of ODE? His lack of symptoms and afferent exam do not suggest an acute optic neuropathy.

I welcome additional comments or suggestions regarding this case.

Thanks,

Enrique J. Rivera, M.D.

Caguas, Puerto Rico

3. RE: Presumed PTCS and optic atrophy

Recommend

Scott Forman

Posted 03-26-2025 08:41

He originally presented with pansinusitis. Therefore you need to obtain MRV to look for cerebral venous thrombosis as a cause for his persistent disk swelling.
Scott Forman, MD

Original Message

Original Message:
Sent: 3/25/2025 11:04:00 PM
From: Enrique Rivera
Subject: Presumed PTCS and optic atrophy

Dear colleagues,

I would appreciate your help regarding future management of the following patient.

12 year old boy with history of presumed pseudotumor cerebri syndrome presenting with right optic nerve atrophy.

Pertinent history:

May 2016 - 4 year-old boy developed headaches and blurred vision. He was found to have bilateral optic nerve swelling by a pediatric neurologist. Pediatric neurology notes also document pansinusitis and otitis media associated with the reported symptoms. LP was done and ICP was 23 cmH2O without CSF abnormalities. This was interpreted and increased ICP and the patient was treated with IV Solumedrol and Diamox. He was discharged from the hospital on Diamox. Subsequently, he was seen by a pediatric ophthalmologist who confirmed bilateral ODE (right more than left). Also present were a right CN6 palsy that resolved during follow-up and a right CN4 palsy that required surgery. Lab work-up was unrevealing. Diamox was discontinued after 1 year of treatment and resolution of ODE.

October 2017 - Asymptomatic patient was found to have ODE on a routine examination by his pediatric ophthalmologist. Swelling was worse than during presentation, but lumbar puncture showed an ICP of 13 cmH2O. Despite a normal ICP, Diamox was re-started and slowly tapered over the next 2 years.

2021-2023 - Normal eye exams.

In June 2024 the patient presented to his pediatric ophthalmologist for a routine evaluation. He had no symptoms at the time. A slight drop in vision to 20/25 was noted in the right eye. His color vision was intact, he had normal stereo and a trace APD that could be reversed with a 0.3 LU NDF. OCT of the nerve revealed a drastically depressed RNFL and loss in the GCC with a hint of altitudinal damage (see attached images) on the right eye. Other than scattered misses on HVF, the patient had no visual field defects. Neuroimaging and lab testing, including anti-MOG and anti-AQP4, were unremarkable. ICP was 32.5 cmH2O with a normal CSF panel. No cytologic abnormalities were found in the CSF.

The patient is currently on Diamox.

Given the information presented:

- How should I manage this patient with chronic, asymptomatic ICP elevation and no findings on exam to help gauge when the ICP is elevated? Should he continue chronic use of Diamox? Would a shunt be the best long-term option?

- Is the nerve atrophy a sign of ongoing disease or the result of past damage from previous episodes of ODE? His lack of symptoms and afferent exam do not suggest an acute optic neuropathy.

I welcome additional comments or suggestions regarding this case.

Thanks,

Enrique J. Rivera, M.D.

Caguas, Puerto Rico

7. RE: Presumed PTCS and optic atrophy

Recommend

Mays El-Dairi

Posted 03-26-2025 18:00

can you please share a single line scan through the macula superiorly? The macular GCL OD seems thinner superiorly, yet the RNFL is diffusely thin which doesn't match...

If there is no active disc edema and the child has no symptoms, I wouldn't treat but I would watch carefully

Mays

Original Message

Original Message:
Sent: 03-25-2025 23:03
From: Enrique Rivera
Subject: Presumed PTCS and optic atrophy

Dear colleagues,

I would appreciate your help regarding future management of the following patient.

12 year old boy with history of presumed pseudotumor cerebri syndrome presenting with right optic nerve atrophy.

Pertinent history:

May 2016 - 4 year-old boy developed headaches and blurred vision. He was found to have bilateral optic nerve swelling by a pediatric neurologist. Pediatric neurology notes also document pansinusitis and otitis media associated with the reported symptoms. LP was done and ICP was 23 cmH2O without CSF abnormalities. This was interpreted and increased ICP and the patient was treated with IV Solumedrol and Diamox. He was discharged from the hospital on Diamox. Subsequently, he was seen by a pediatric ophthalmologist who confirmed bilateral ODE (right more than left). Also present were a right CN6 palsy that resolved during follow-up and a right CN4 palsy that required surgery. Lab work-up was unrevealing. Diamox was discontinued after 1 year of treatment and resolution of ODE.

October 2017 - Asymptomatic patient was found to have ODE on a routine examination by his pediatric ophthalmologist. Swelling was worse than during presentation, but lumbar puncture showed an ICP of 13 cmH2O. Despite a normal ICP, Diamox was re-started and slowly tapered over the next 2 years.

2021-2023 - Normal eye exams.

In June 2024 the patient presented to his pediatric ophthalmologist for a routine evaluation. He had no symptoms at the time. A slight drop in vision to 20/25 was noted in the right eye. His color vision was intact, he had normal stereo and a trace APD that could be reversed with a 0.3 LU NDF. OCT of the nerve revealed a drastically depressed RNFL and loss in the GCC with a hint of altitudinal damage (see attached images) on the right eye. Other than scattered misses on HVF, the patient had no visual field defects. Neuroimaging and lab testing, including anti-MOG and anti-AQP4, were unremarkable. ICP was 32.5 cmH2O with a normal CSF panel. No cytologic abnormalities were found in the CSF.

The patient is currently on Diamox.

Given the information presented:

- How should I manage this patient with chronic, asymptomatic ICP elevation and no findings on exam to help gauge when the ICP is elevated? Should he continue chronic use of Diamox? Would a shunt be the best long-term option?

- Is the nerve atrophy a sign of ongoing disease or the result of past damage from previous episodes of ODE? His lack of symptoms and afferent exam do not suggest an acute optic neuropathy.

I welcome additional comments or suggestions regarding this case.

Thanks,

Enrique J. Rivera, M.D.

Caguas, Puerto Rico

8. RE: Presumed PTCS and optic atrophy

Recommend

Enrique Rivera

Posted 03-28-2025 04:23

I want to thank those that have commented both publicly and privately.

To answer some of the questions that have been posted:

MRI brain and orbit and MRV head were done during the initial presentation in 2016. These were not repeated during the recurrence in 2017, but were done again with the latest recurrence in 2024. No abnormalities have been observed in these scans, except for volume loss in the right optic nerve during his latest scan.
Sheath fenestration has not been considered mainly because of his excellent visual function.
I don't have the OCT machine readily available because the scans were done in the office of the pediatric ophthalmologist. However, I do agree that the GCL looks thinner superiorly and this is what raises the possibility of some ischemic insult.

Thanks,

Enrique J. Rivera

Original Message

Original Message:
Sent: 03-25-2025 23:03
From: Enrique Rivera
Subject: Presumed PTCS and optic atrophy

Dear colleagues,

I would appreciate your help regarding future management of the following patient.

12 year old boy with history of presumed pseudotumor cerebri syndrome presenting with right optic nerve atrophy.

Pertinent history:

May 2016 - 4 year-old boy developed headaches and blurred vision. He was found to have bilateral optic nerve swelling by a pediatric neurologist. Pediatric neurology notes also document pansinusitis and otitis media associated with the reported symptoms. LP was done and ICP was 23 cmH2O without CSF abnormalities. This was interpreted and increased ICP and the patient was treated with IV Solumedrol and Diamox. He was discharged from the hospital on Diamox. Subsequently, he was seen by a pediatric ophthalmologist who confirmed bilateral ODE (right more than left). Also present were a right CN6 palsy that resolved during follow-up and a right CN4 palsy that required surgery. Lab work-up was unrevealing. Diamox was discontinued after 1 year of treatment and resolution of ODE.

October 2017 - Asymptomatic patient was found to have ODE on a routine examination by his pediatric ophthalmologist. Swelling was worse than during presentation, but lumbar puncture showed an ICP of 13 cmH2O. Despite a normal ICP, Diamox was re-started and slowly tapered over the next 2 years.

2021-2023 - Normal eye exams.

In June 2024 the patient presented to his pediatric ophthalmologist for a routine evaluation. He had no symptoms at the time. A slight drop in vision to 20/25 was noted in the right eye. His color vision was intact, he had normal stereo and a trace APD that could be reversed with a 0.3 LU NDF. OCT of the nerve revealed a drastically depressed RNFL and loss in the GCC with a hint of altitudinal damage (see attached images) on the right eye. Other than scattered misses on HVF, the patient had no visual field defects. Neuroimaging and lab testing, including anti-MOG and anti-AQP4, were unremarkable. ICP was 32.5 cmH2O with a normal CSF panel. No cytologic abnormalities were found in the CSF.

The patient is currently on Diamox.

Given the information presented:

- How should I manage this patient with chronic, asymptomatic ICP elevation and no findings on exam to help gauge when the ICP is elevated? Should he continue chronic use of Diamox? Would a shunt be the best long-term option?

- Is the nerve atrophy a sign of ongoing disease or the result of past damage from previous episodes of ODE? His lack of symptoms and afferent exam do not suggest an acute optic neuropathy.

I welcome additional comments or suggestions regarding this case.

Thanks,

Enrique J. Rivera, M.D.

Caguas, Puerto Rico

9. RE: Presumed PTCS and optic atrophy

Recommend

Deborah Friedman

Posted 03-29-2025 16:00

HI Enrique,

I agree with the suggestions of others...and am wondering whether the diagnosis is correct. The 2017 OCTs show very asymmetric involvement (which can happen in PTCS but is uncommon), his LPs all showed normal opening pressure until 2024 (he may have been sedated for the procedure at age 4 but still in the normal range for a child). Fourth nerve palsy is VERY uncommon with IIH/PTCS.

It sounds like his headaches resolved within a year after the first episode at age 4? You indicated that he was followed by a pediatric ophthalmologist. I know it has been 8 years but optic atrophy should have been present at age 5 if he has been otherwise asymptomatic ever since then. Was optic atrophy every documented in his old records? (Did the "normal eye exams" from 2021-23 mean that there the optic nerves looked healthy?)

Recheck anti MOG? Were antibodies checked in the CSF?

Sorry to pose more questions than answers (but that is pretty common on NANOSNET)

Deb

Original Message

Original Message:
Sent: 3/25/2025 11:04:00 PM
From: Enrique Rivera
Subject: Presumed PTCS and optic atrophy

Dear colleagues,

I would appreciate your help regarding future management of the following patient.

12 year old boy with history of presumed pseudotumor cerebri syndrome presenting with right optic nerve atrophy.

Pertinent history:

May 2016 - 4 year-old boy developed headaches and blurred vision. He was found to have bilateral optic nerve swelling by a pediatric neurologist. Pediatric neurology notes also document pansinusitis and otitis media associated with the reported symptoms. LP was done and ICP was 23 cmH2O without CSF abnormalities. This was interpreted and increased ICP and the patient was treated with IV Solumedrol and Diamox. He was discharged from the hospital on Diamox. Subsequently, he was seen by a pediatric ophthalmologist who confirmed bilateral ODE (right more than left). Also present were a right CN6 palsy that resolved during follow-up and a right CN4 palsy that required surgery. Lab work-up was unrevealing. Diamox was discontinued after 1 year of treatment and resolution of ODE.

October 2017 - Asymptomatic patient was found to have ODE on a routine examination by his pediatric ophthalmologist. Swelling was worse than during presentation, but lumbar puncture showed an ICP of 13 cmH2O. Despite a normal ICP, Diamox was re-started and slowly tapered over the next 2 years.

2021-2023 - Normal eye exams.

In June 2024 the patient presented to his pediatric ophthalmologist for a routine evaluation. He had no symptoms at the time. A slight drop in vision to 20/25 was noted in the right eye. His color vision was intact, he had normal stereo and a trace APD that could be reversed with a 0.3 LU NDF. OCT of the nerve revealed a drastically depressed RNFL and loss in the GCC with a hint of altitudinal damage (see attached images) on the right eye. Other than scattered misses on HVF, the patient had no visual field defects. Neuroimaging and lab testing, including anti-MOG and anti-AQP4, were unremarkable. ICP was 32.5 cmH2O with a normal CSF panel. No cytologic abnormalities were found in the CSF.

The patient is currently on Diamox.

Given the information presented:

- How should I manage this patient with chronic, asymptomatic ICP elevation and no findings on exam to help gauge when the ICP is elevated? Should he continue chronic use of Diamox? Would a shunt be the best long-term option?

- Is the nerve atrophy a sign of ongoing disease or the result of past damage from previous episodes of ODE? His lack of symptoms and afferent exam do not suggest an acute optic neuropathy.

I welcome additional comments or suggestions regarding this case.

Thanks,

Enrique J. Rivera, M.D.

Caguas, Puerto Rico

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