Observations of multiorgan system involvement associated with plasma cell dyscrasia led to the characterization of POEMS syndrome, with the acronym standing for prominent findings of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Other common signs include optic disc edema, ascites, pleural effusion, and thrombocytosis. Although optic disc edema occurs in one third to one half of patients with POEMS syndrome (1), vision loss is rare.
A 43-year-old man noted sudden painless loss of vision in his left eye following 2–3 days of stuttering visual decline in that eye. Six years earlier, he had been diagnosed with POEMS syndrome on the basis of demyelinating polyneuropathy, hepatosplenomegaly, hypogonadism, hyperprolactinemia, hypothyroidism, vitamin D deficiency, adrenal insufficiency, IgA lambda monoclonal proteinemia, skin darkening, a sclerotic bone lesion in the spine, and extravascular volume overload causing ascites and pleural effusions. He had received a stem cell transplant with clinical improvement for 2 years followed by recurrent symptoms of volume overload. Mild optic disc swelling and high serum vascular endothelial growth factor (VEGF) were found at the time of recurrence and were attributed to POEMS syndrome. He had been chronically managed on 20 mg of dexamethasone weekly. Chemotherapy was planned for treatment of recurrent symptoms and necessitated stopping dexamethasone, 2 weeks prior to vision loss.
Two weeks prior to vision loss, his acuity was 20/40 in each eye with normal color vision, posterior subcapsular cataracts, and bilateral optic disc swelling (Fig. 1). The disc swelling had been present for at least 6 months. Automated visual fields demonstrated general depression in the left eye (mean deviation −5.6 dB, compared with −1.9 dB in the right eye) without focal visual field loss. Because optic disc edema is a common finding in patients with POEMS syndrome, no further testing was performed.
The patient was evaluated 4 days after acute vision loss in the left eye. Visual acuity was 20/40, right eye, and hand motions, left eye. Pupils were equal, round, and reactive to light with a left relative afferent pupillary defect. Color vision was normal on the right, but the patient could not identify any of the color plates with the left eye. The appearance of the optic disc edema was unchanged, and the fundi were otherwise normal.
MRI of the orbits performed 3 days later revealed enlargement and enhancement of the left optic nerve and an adjacent enhancing lesion in the suprasellar cistern (Fig. 2). Lumbar puncture had an opening pressure of 17 cm H2O. Cerebrospinal fluid analysis demonstrated elevated protein of 131 mg/dL (normal, <45 mg/dL), normal glucose, no pleocytosis, and no malignant cells.
Steroid therapy was immediately reinitiated (single dose of 40 mg of dexamethasone followed by 20 mg weekly) and, 2 weeks later, chemotherapy consisting of cyclophosphamide, bortezomib, and dexamethasone was started. Vision in the left eye declined to no light perception and remained 20/40 in the right eye.
One month later, the patient's ascites had decreased and his energy level had increased. Vision in the left eye remained no light perception while the right optic nerve remained mildly swollen, and the left optic nerve became flat without pallor. MRI performed 6 weeks after acute vision loss demonstrated persistent enlargement and enhancement of the left optic nerve, with progressive enlargement of the mass lesion (Fig. 3).
Follow-up imaging 5 months after presentation showed some regression of the mass, and biopsy was deferred. Ten months after presentation, the patient could count fingers with his left eye and MRI showed further reduction in the size of the suprasellar mass.
Screening for and evaluation of vision loss in POEMS syndrome is challenging since optic nerve edema is relatively common in POEMS syndrome occurring in 29% and 55% of patients (1,2). It is a minor criterion for the diagnosis of the syndrome. The etiology of optic nerve edema is not known in most cases. Some cases can be explained on the basis of increased CSF protein or high intracranial pressure (3). Microvascular etiologies have been proposed on the basis of high VEGF levels. Systemic interstitial volume overload may also be a factor in patients associated with cystoid macular edema (4,5). Despite optic nerve edema being common in POEMS syndrome, vision loss is unusual (3). Rare instances of acute visual decline have been attributed to macular edema (4,5) and papilledema related to elevated intracranial pressure due to venous sinus thrombosis (6).
We are unaware of previous reports of an intracranial mass lesion and optic nerve enhancement in POEMS syndrome. The presumed composition of the mass lesion is plasmacytoma. The proposed pathophysiology of vision loss in our patient may be multifactorial. First, there may be compression of the optic nerve similar to reports of POEMS syndrome associated with orbitopathy (7,8). Second, in an analogous fashion to vision loss due to IgG multiple myeloma, tumor infiltration (9,10) and ischemia (9) by IgA osteosclerotic myeloma may have contributed to the optic neuropathy. Finally, nerve conductivity may have been decreased by IgA paraproteins (11).
The authors thank Lisa Birmingham for her assistance with the figures.
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A follow-up MRI 14 months after presentation showed complete regression of the suprasellar mass. The abnormal signal within the optic nerve persisted. Vision remained stable at count fingers with the left eye. This complete radiographic resolution following chemotherapy directed at POEMS syndrome supports the presumed pathology of plasmacytoma. Radiation to the area is planned to prevent regrowth.