January 16, 2016
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Patient experiences ‘flowery’ vision, personality changes

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An 82-year-old white female presented to the eye clinic with complaints of “flowery” spots in her vision that occurred up to seven times per week. These images persisted for a few seconds at a time. They had been ongoing for approximately 2 months.

The patient had been previously diagnosed with dry age-related macular degeneration and was taking Age-Related Eye Disease Study 2 (AREDS 2) formulation vitamins. Her medical history was extensive and included hypothyroidism, anxiety, hypertension, colon polyps, hyperlipidemia and smoking. She was considered a glaucoma suspect based on previously discovered small areas of thinning on her optic nerve optical coherence tomography in the right eye and slightly larger than average cup-to-disc ratios in both eyes. A multivitamin, calcium and vitamin D were taken as maintenance therapy. She was taking pantoprazole for gastroesophageal reflux disease and levothyroxine for hypothyroidism.

Visual testing revealed best corrected visual acuities of 20/30-1 OD and 20/40-2 OS with no improvement on pinhole. Pupils and extraocular muscle movements were normal, and cover test showed orthophoria at both distance and near. The Amsler grid showed no distortion or scotoma when viewed with the right eye but a small area of temporal distortion with the left eye. An OCT of both optic nerves revealed superior nerve fiber layer depression of the right eye. This finding was consistent with previously identified nerve defects. The OCT of the left optic nerve confirmed a normal nerve fiber layer.

Paige Nash

An automated visual field 24-2 test of each eye showed severe general depression in both eyes. Visual field testing was repeated the same day and showed similar results. These results did not correlate with the level of visual acuity or the optic nerve OCT. It was suspected that these visual fields were unreliable.

A fundus photo of the right eye showed mottling and dry AMD changes.

Images: Nash P

Dilated fundus exam showed no dramatic changes in the health of either eye. Both cup-to-disc ratios were evaluated as 0.5 horizontal by 0.6 vertical. Macular pigment changes consistent with dry AMD included retinal pigment epithelium dropout and mottling. The periphery was intact with no holes, tears or detachments. No structural findings seemed to be able to explain the new visual disturbances.

Over the course of the exam, the patient’s daughter revealed that her mother had become combative and that her personality had changed. This information in combination with the inconsistency between subjective and objective findings in testing prompted us to order a magnetic resonance imaging (MRI) scan of the patient’s brain.

A fundus photo of the left eye showed mottling and dry AMD changes.

Our patient’s MRI revealed a mass in the right hemicranium, impinging on the frontal lobe. Radiology measured the mass to be 4.1 cm x 3.6 cm x 4.2 cm in size. It was tentatively diagnosed as a meningioma. Surgery was scheduled to remove the tumor.

Meningiomas are the most common benign brain tumor although a small percentage hold the potential for metastasis. Linsler and colleagues reported that the overall rate of occurrence is 7.8 per 100,000 per year, accounting for 13% to 26% of all intracranial tumors. Only 25% of meningiomas are thought to be symptomatic; therefore, the reported incidence is lower than the actual rate of occurrence, according to Ferri. Up to 2.8% of females may have subclinical meningiomas based on autopsy and imaging reports. Females are twice as likely as males to develop a meningioma; however, men are slightly more likely to develop an atypical or metastatic form of meningioma (Linsler et al. and Wiemels et al.). Racial predilection suggests a slightly higher incidence in black patient populations over Caucasians or Hispanics.

Meningiomas arise from the cells that make up the covering of the brain and spinal cord. They can occur anywhere in the brain. Most commonly meningiomas form in the skull, with only 10% located in the spinal cord (Park and Ferri). The location and size of the tumor will dictate the clinical symptoms or lack thereof.

Etiology

An OCT scan of both optic nerves showed a small area of nerve fiber layer thinning in the right eye, which had been detected at a previous visit.

An OCT scan of both optic nerves showed a small area of nerve fiber layer thinning in the right eye, which had been detected at a previous visit.

Analysis has found that these tumors develop from clonal growth descending from a single mutated cell. Often, one chromosome is mutated to cause the development and growth of a grade 1 benign tumor. A deletion of chromosome 22 has been found to be characteristic of meningiomas. Atypical and more aggressive tumors are more likely to be the result of multiple genetic mutations in the original cell.

The grading of meningiomas is based on the cell differentiation and potential for metastasis. Most meningiomas (78% to 81%) are classified as grade 1 or benign. These tumors are slow-growing and have distinct borders. Grade 2 are called atypical meningiomas and make up 15% to 20% of all meningiomas. The cells that make up a grade 2 tumor are not considered malignant or benign but have potential for malignancy. These tumors are also more likely to recur after treatment and tend to grow more rapidly. Grade 3 meningiomas are known as malignant or anaplastic. These represent a small subset of meningiomas that are aggressive and invade adjacent brain tissue. Only 1% to 4% of meningiomas are grade 3 (Johns Hopkins Medicine).

Risk factors for the development of meningioma are not as well understood as for other intracranial neoplasms. Exposure to high doses of ionizing radiation is the most consistently reported and investigated risk factor. Low doses may also increase risk, but the latency of the disease is 30 to 40 years and often other risk factors over that time period can confound the determination of the underlying epidemiology in cases of low-dose exposure.

Approximately half of patients with neurofibromatosis type 2 (NF2) will develop an intracranial meningioma (Ferri). NF2 is an autosomal dominant disorder associated with multiple intracranial tumors.

An automated visual field 24-2 of the right eye showed apparent overall severe depression. The field was run twice with similar results.

An automated visual field 24-2 of the right eye showed apparent overall severe depression. The field was run twice with similar results.

Risk factors

Hormones have been documented as a potential risk in the development of meningiomas. This is based on the expression of hormone receptors in the tumors themselves. These hormone receptors are found in tumors in both males and females. An increase in the size of meningiomas has been reported during pregnancy, and the incidence seems to be additionally skewed toward females during the prime childbearing years. Use of exogenous hormone therapy has been anecdotally linked to an increased risk of meningioma after menopause, but further study is needed in this area to draw any conclusions.

A positive history of head trauma has been studied as another prospective risk factor. Small sample size has hindered the ability to draw any inferences in this area, and results of these studies have not been consistent. Finally, cell phone use has been a recent area of interest regarding the risk of meningioma. Due to the latency period of exposure to ionizing radiation, it is not unreasonable to expect that results from studies regarding cell phone use will not produce useable data for decades. Little evidence is currently available to link cell phone use and meningioma incidence. This area does merit future study due to the short time since the initiation of cell phone use, and widespread exposure to this is a possible risk factor.

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Symptoms

Potential symptoms of intracranial meningioma cover a broad spectrum of onsets and severities. Seizures may be present in up to 40% of patients, according to Ferri. Subtle visual changes such as decreased vision, visual field defects and double vision have been reported. Weakness of the extremities presenting in specific patterns may differentiate spinal from cranial meningiomas.

As mentioned previously, it is believed that only 25% of meningiomas are symptomatic. Those tumors that involve the frontal lobe do not often cause notable neurologic defects. This leads many patients to psychiatrists for treatment of new-onset depression and personality changes. These particular tumors are often left undiagnosed or misdiagnosed as mental illness and early-onset dementia.

Indications for imaging

Anxiety, social withdrawal, increased thoughts of death and forgetfulness for daily activities can all be part of the presenting clinical picture for a patient with a meningioma causing frontal lobe compression. It would be irrational to suggest that all patients with these symptoms need to be sent for imaging. However, there are a few features that can aid in the diagnosis of these cases.

Any initial onset of depression after the age of 50, slowly progressing psychological changes and a lack of improvement after treatment with selective serotonin reuptake inhibitors (i.e., fluoxetine, paroxetine, sertraline) or other typical antidepressants warrants further consideration of the possibility of an organic cause for the depression.

Case history gathered from the patients themselves may not be sufficient to detect some of the changes listed. This kind of investigation often requires the input of those who have known the patient well for a long period of time.

An automated visual field 24-2 of the left eye showed apparent overall severe depression.

An automated visual field 24-2 of the left eye showed apparent overall severe depression.

In the case of our patient, her daughter reported personality changes. Often this is misdiagnosed as rapidly progressing dementia. For our patient, visual disturbances were an essential additional symptom that differentiated her condition and raised suspicion of a downstream cause. After a complete exam of the patient’s ocular structures, it was determined that the visual hallucinations were not originating in the eyes, indicating a need for neuroimaging.

MRI or computed tomography (CT) scans may be used to investigate the location of an intracranial mass. MRI is the preferred imaging technology because it allows radiology to gather more specific information regarding the association of the tumor to other tissues. The appearance of a tumor on imaging is enough to make a tentative diagnosis for treatment, but histological confirmation is the only definitive diagnosis.

Treatment

Most meningiomas are benign and well circumscribed (grade 1). If this is the case and the tumor is in a location in which it is safe to do so, a neurosurgeon can excise the tumor. This is the treatment of choice. The surgery most often performed to remove a meningioma is known as a craniotomy. The surgeon removes a piece of bone from the skull in order to access the tumor and removes as much as possible without damaging surrounding tissue.

Leonid Skorin Jr.

Radiation may also be considered in the treatment plan for meningiomas. This route is generally reserved for those tumors in locations that make surgical excision too difficult or dangerous. Examples of these cases include those in the base of the skull under the brain or a tumor that forms around a carotid artery.

An axial MRI of the brain showed an approximately 4-cm mass in the right hemicranium. The arrow indicates the cystic space at the center of the mass.

In cases of meningioma where the tumor is found incidentally and is causing no symptoms, it may be appropriate to postpone intervention and instead use regular imaging to observe the progression of the tumor. Some tumors remain stable and never require treatment, while others may progress to the point of necessitating treatment later in life.

A coronal MRI of the brain showed a mass in the right hemicranium causing right ventricle compression.

Clinical pearls

Patients presenting with subjective reports of visual hallucinations that are unexplained by organic ocular causes should increase suspicion of an intracranial cause. If a family member or close friend indicates a change in personality or demeanor, frontal lobe involvement must be near the top of the differential list. Brain imaging with an MRI should be ordered for these patients.

If a neoplasm is the cause of these findings, removal may be the only treatment necessary. This can drastically increase the quality of life for patients who may otherwise have been unsuccessfully managed by psychotic medications and antidepressants.

This patient’s management

Our patient’s tumor was removed successfully, and pathology confirmed that it was a meningioma as suspected by radiology. The final diagnosis was grade 2 falcine meningioma. Falcine refers to the origin of the tumor. The falx is the thin layer of tissue that separates the right and left brain hemispheres. This lining is the location of the original mutation that allowed for the clonal replication of cells to form our patient’s tumor.

Approximately 1 month after the tumor was excised, the patient returned to the eye clinic for re-evaluation of the visual field and OCT. Visual acuities at this visit were 20/40-2 OD and 20/50-2 OS. There was an unusual variability during visual acuity testing of the left eye, and pinhole showed no improvement for either eye. Objective tests revealed no appreciable improvement. The visual field results still suggested dramatic overall depression in sensitivity. The optic nerve OCT remained unchanged, and dilated fundus examination showed unchanged dry AMD. Subjectively, the patient reported that the flowery visual disturbances she was experiencing at her original presentation had subsided.

The patient’s daughter explained that her mother was no longer combative and was more agreeable and accepting of health care intervention. She confirmed that her personality was beginning to stabilize back toward baseline. The patient and her daughter informed us that there was a decrease in motor control of the left side of the body immediately following surgery. This recovered over the course of the surgery day and completely resolved by the time she left the hospital.

After surgery, the patient quit smoking with the help of nicotine replacement therapy. She has been placed on short-term anticonvulsant medication during the postoperative period. She will attend rehabilitation sessions to continue working on improving memory and speech cognition. Overall, the patient and her family were happy with the results of the tumor removal. The patient was pleasant and upbeat at her follow-up visit. She will be seen back to repeat the visual field testing in 3 months.

Disclosures: Nash and Skorin report no relevant financial disclosures.