Case #3 (2-14-2004)

[Andrew_Doan]

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CC: Gradual, painless loss of vision in both eyes.

HPI: 53 y.o. AA woman with gradual, painless loss of vision OS 2 months prior to exam. ?My vision went black, but it came back over one month in the left eye. I also have a history of goiter.? One month later, she experienced gradual, painless loss of vision OD to no light perception (NLP).

She denies HA, jaw pain, scalp tenderness, but reports 50 lbs weight loss, fatigue, and severe dry eyes.

PMH: significant only for goiter which has been stable for several years. No other medical problems. No previous ocular history.

EXAM:

Vision: NLP OD, 20/40 OS
Pupils: 6 mm in dark, 6 mm in light OD; 6 mm in dark, 3 mm in light OS; >3.0 Log Unit (very big) RAPD OD.
IOP: 18 mmHg OD, 20 mmHg OS
EOM: full motility OU.

Anterior segment exam unremarkable.
DFE: normal macula, vessels, and periphery OU. Optic nerves were normal with 0.4 C/D ratio OU without evidence of pallor or edema.

ECHO of the orbits were normal without evidence of thyroid ophthalmopathy.

Goldmann Visual Field of the left eye

MRI: Coronal image of the head, T1 weighted, post-contrast, with fat suppression

MRI: Axial image of the orbits/head, T1 weighted, post-contrast, with fat suppression

Feel free to discuss the following:

What tests should you order (I'll post labs when asked for them)?

What's the differential diagnosis?

What's the diagnosis?

What is the treatment of choice, surgically and/or medically?

________________________________
Requested Labs & Studies

ESR = 75 mm/hr
CRP = 3.5 mg/dl (normal < 0.5 mg/dl)
ACE = 62
CBC wnl
ALT = 79 U/L
AST = 74 U/L
Alkaline Phosphatase = 189 U/L

Chest X-Ray

 

[Kalel]

Well, even though I am getting killed in these optho cases by not knowing half of the vocab, participating is fun so I will continue to do so. 🙂

I think that the most likely diagnosis is either sarcoidosis or optic neuritis. I'm certainly not very good at reading MRI's, but I believe that the assymetry in the scans overlies the optic nerve and not things like the ocular muscles (graves, goiter may have been red herring?), and I don't think that you would see this type of lesion with some sort of vascular complication. I don't know what type of paraneoplastic syndrome could cause the patient's symptoms either. Given her significant weight loss, I might suspect that the most likely diagnosis is an unusal presentation of sarcoidosis, but the fact that the lesion changed eyes strongly suggests MS/optic neuritis too, so I'm not certain which one it is. In order to differentiate the two, I would like the following test:

1. CT of chest: Most patients with with high stage sarcoid (don't remember exactly how the staging goes, sorry) will have hilar lymphadenopathy which would contribute to the diagnosis.
2. ACE levels: should be elevated in most patients with sarcoid
3. Lumbar puncture: look for things like oligoclonal bands, glucose, protein, myelin basic protein, IgG levels, ACE levels, cell count and differential
4. TSH: Just in case, to evaluate goiter
5. CMP, ESR, CBC, coag studies, Ca just to be thorough.
6. HLA typing to help dx MS

I might bolus her with steroids since MS/optic neuritis is on my differential.

 

[Redhawk]

1. Optic neuritis - Although the most common form of ON in adults is retrobulbar, one would think that some level of atrophy would be present, at least OS. You could check evoked visual potentials.

2. Arteritic AION (GCA) typically presents in patients over the age of 65. It also presents with ACUTE visual loss with papillitis and disk hemorrhages. She is much younger and had normal DFE OU as well as lacked several of the other sx that can accompany GCA such as HA, jaw pain, scalp tenderness. I think it would also be unusual for a patient to go from "black" vision to 20/40 as she did OS if it really were GCA. Fatigue and wt loss can be seen but are obviously not specific.

3. Non-arteritic AION (arteriosclerotic or embolic) typically presents in late 40's to early 60's, so she does fit in this age group. It would be important to check for vascular risk factors such as HTN, DM, hyperlipidemia, vasculitis. This, however, also tends to present acutely with papillitis and hemorrhages. Can also occur following cataract surgery or coronary intervention.

4. Paraneoplastic syndrome - That would be interesting. I don't think I've come across that causing visual loss yet. Anyone know something in this area that can cause such sx?

5. Thyroid orbitopathy can cause compressive optic neuropathy, but he had normal orbital echos, and apparently no proptosis or restrictive myopathy combined with normal DFE OU.

6. Sarcoid can can present as "neurosarcoidosis" and uncommonly cause a retrobulbar optic neuropathy. It might also explain the presence of keratoconjuctivitis sicca secondary to inflitration of the lacrimal glands. She does lack several of the other possible presenting ocular sx of sarcoid such as anterior uveitis or "candle wax drippings" of the retina. There appears to be "something" affecting the prechiasmal optic nerves bilaterally, perhaps this could be granulomatous tissue? A biopsy would be needed to confirm such a diagnosis but could be supported by elevated ACE levels or hypercalciuria. Biopsy would be highest yield from the lung (can also be done from conjuctival granulomas but lower yield). A chest xray should be done first to check for the presence of hilar lymphadenopathy. Then a CT might be done.

Labs:
Maybe consider ESR, CRP, CBC (platelets) for GCA but I think this would only be to "CYA"
Check thyroid levels just to rule out
ACE, chem panel, CXR, biopsy

 

[Andrew_Doan]

I just want to make two points.

1. GCA should be considered in individuals 55 or above. Although rare, I've seen a few cases in their early 50's. Also, GCA is extremely rare in African Americans, particularly in a patient without jaw claudication, temple HA, and scalp tenderness.

2. Paraneoplastic syndromes can cause a retrobulbar optic neuritis.

http://www.ncbi.nlm.nih.gov/entrez/...ve&db=PubMed&list_uids=12838519&dopt=Abstract

Good job! Keep the discussion coming.

 

[TomOD]

We know there is an APD.

Optic neuritis (retrobulbar): I'm thinking pain typically-not present in this patient.

Arteritic ION: No temperal artery tenderness, optic nerves signs but does have rapid weight loss.

Non-arteritic ION: Again, no visible optic nerve findings. I'd expect something here.........at least some pallor. Right age group.

Sarcoid: Expect pain, photophobia. No uveitis or signs of past events. No retinal findings.

Thyroid: No proptosis, no EOM restrictions. Optic-nerve compression can cause vision loss and APD. Can develop with minimal exophthalmos?

I'd need help with the MRI's but it appears that "something" is going on around the nerves.

My diagnosis: Beat's the hell out of me.

This is a case for University Ophthalmology😀

My guess would be sarcoid http://www.ncbi.nlm.nih.gov/entrez/...ve&db=PubMed&list_uids=14504590&dopt=Abstract

followed by neoplastic syndrome and then Thyroid (but I don't think Andrew would give such a soft ball as Thyroid with a history of Goiter 😉

 

[Andrew_Doan]

Labs

ESR = 75 mm/hr
CRP = 3.5 mg/dl (normal < 0.5 mg/dl)
ACE = 62
CBC wnl
ALT = 79 U/L
AST = 74 U/L
Alkaline Phosphatase = 189 U/L

Chest X-Ray

What diagnostic study and/or procedure should be performed?

What is the treatment?

 

[Andrew_Doan]

Although more common in African Americans, sarcoidosis can be found in Caucasians. In Iowa, we see numerous new cases yearly, and sarcoid must be on the differential of unilateral or bilateral vision loss associated with systemic manifestations, e.g. weight loss, fatigue, breathing problems, fevers, and dry eyes. Sarcoid can manifest in the lacrimal and parotid glands; thus, patients may complain of severe dry eyes or dry mouth.

An elevated ACE and liver enzymes in this patient suggest lung and liver disease. The chest x-ray showed a single, large nodule in the left upper lobe. Without a CT scan and biopsy, the differential diagnosis should include lung tumor at this point. A CT scan was performed which showed perihilar lymphadenopathy as well as the large left upper lobe nodule. Bronchoscopy was performed, and aspirate showed numerous non-necrotizing granulomas, which supports the diagnosis of sarcoidosis. In addition, the absence of uveitis or retinal disease does not rule-out sarcoid.

Sarcoid is a multi-systemic disease that may affect the: skin, brain, eyes, lungs, spleen, thyroid, and liver. It is a vision threatening and deadly disease, and sarcoid is responsive to aggressive immunosuppression therapy with high dose steroids.

This patient was admitted to the neurology service for neuro-sarcoidosis and started on IV steroids. Her vision in the right eye did not improve, but the left eye improved from 20/40 to 20/20 in two days. Her visual field also improved:

GVF before treatment

GVF 2 days after Steroids IV

Differential Diagnosis
- Atypical optic neuritis
- lymphoma
- paraneoplastic syndrome
- vasculitis