Cerebral Polyopia (Cerebral Diplopia)

From EyeWiki


Disease

Cerebral Polyopia, also known as Cerebral Diplopia, is an extremely rare visual phenomenon characterized by the persistence of two or more duplicated images even after the stimulus is removed. These images uniquely present as ordered rows or columns of the patient’s visual field [1]. Cerebral polyopia can occur both monocular bilaterally or binocularly, which means that the duplicated images can be seen in either eye while the other eye is closed (monocular bilaterally) as well as when both eyes are open (binocularly). This unique presentation allows clinicians to differentiate this phenomenon from other visual disturbances such as ocular diplopia and palinopsia.

The presentation of the duplicated image(s) varies between patients. When only two images are observed, the original and the duplicate, the phenomenon is labeled as cerebral diplopia, a subtype of cerebral polyopia. Additional differences between patients include but are not limited to the following [2][3][4]:

·      Number of duplicated images seen, ranging from one to hundreds

·      Distance from the target stimulus to the duplicated image(s)

·      Similarity of the duplicated image(s) to the original

·      Intensity, color, or size of the duplicated image(s)

·      Amount of time that the duplicated image(s) remains in the visual field

·     Onset delay of the duplicated image(s)

Disease History

Cerebral polyopia was first reported in 1908 by Dr. Giovanni Mingazzini, who described the disease in relation to an occipital lobe injury [5]. In 1945, Dr. Morris B. Bender’s work further described polyopia through the documentation of four individuals with the disease [2]. Since then, reports of cerebral polyopia have remained limited. The extreme rarity of the disease, the similarities to other more common visual disturbances, and the variations in the presentation have hindered the ability to properly understand the etiology of the disease as well as find appropriate treatments [6].

Etiology

The mechanisms underlying the disease are uncertain and continue to be investigated [6][7][8]. However, most instances of polyopia are associated with a lesion in the occipital lobe, more specifically the visual association cortex, or following occipital lobe epilepsy [4][9][10]. Cerebral polyopia may result from a variety of cerebral injuries including but not limited to trauma, ischemic stroke, migraine, encephalitis, seizures, tumors, and multiple sclerosis [4][9].

Many theories have been postulated the underlying mechanisms. The initial theory proposed by Bender explains the duplicated images as a result of the instability of fixation during small, involuntary eye movements. This leads to the creation of new retinal and cortical regions that code for a false macula which is the underlying cause of the duplicated images [2].

However, evidence from more recent case studies did not support this theory as cases where the polyopic images did not correspond with the degree of involuntary eye movement. Another possible pathophysiological mechanism proposed by Cornblath suggests that reorganization of the receptive fields of neurons near the damaged area of the visual cortex could lead to cerebral polyopia [6].

Kesserwani proposed an alternative theory suggesting the underlying mechanisms regarding cerebral polyopia resemble a hologram [11]. The theory is modeled after a case study of a 70-year-old woman with a cerebral infarct of the striate cortex, V1, and visual association cortex V2 who displays symptoms of cerebral polyopia. By providing a predictive mathematical and experimental foundation using Fourier transforms, Kesserwani provided a template for how the holographic or holonomic brain theory can explain the duplicated images seen in cerebral polyopia [11]. Isherwood proposed that normal-appearing white matter that is microstructurally abnormal may be the cause of this phenomenon [12].

Differential Diagnosis

Visual Trailing Palinopsia is a condition that is often confused with polyopia. Visual trailing palinopsia occurs when an object that is seen in motion leaves copies behind in the visual field, thus creating a “visual echo” with multiple images appearing. Cerebral polyopia can be distinguished from visual trailing palinopsia by the effect of movement on the duplicated images. In visual trailing, the palinoptic images are left in the wake of a moving object, while in polyopia, the polyopic images move with the original object [1]. In addition, the original object may be removed but the patient will continue to see the stationary image in cerebral polyopia. Kataoka proposes that the two diseases may be related pathophysiologically but acknowledges that they are two distinct phenomena [7].

Diplopia and polyopia can occur distinctively from cerebral polyopia when cerebral function is not involved. Diplopia is a relatively common occurrence and can be caused by the misalignment of the eyes, such as in strabismus [6][7][8][13]. Monocular diplopia has a lower prevalence than binocular diplopia and occurs when two images are seen with one eye closed [6]. This may occur when light is not reflected to the back of the retina appropriately, seen in refractive errors or cataracts [13]. Cerebral polyopia specifically occurs following cerebral damage and is significantly rarer than the aforementioned phenomenon [6].

Medical Workup

Determination of all visual disturbances is vital in the diagnosis of cerebral polyopia. Often, cerebral polyopia presents alongside other more common disturbances such as palinopsia [6][7]. A thorough history of the onset of symptoms can help determine if the cause of the polyopia is cerebral. Following this analysis, neuroimaging should be done to detect possible infractions [7]. CT scans are not the most effective at identifying lesions, so ideally PET and MRI may be performed to aid in the diagnosis of cerebral polyopia.

Treatment

There is no known cure for cerebral polyopia, and treatments vary on a case-by-case basis. The underlying cerebral mechanism causing the visual disturbances may help guide treatment plans. A case study on a patient experiencing cerebral polyopia secondary to occipital lobe epilepsy reported positive effects following the administration of valproate sodium and Gabapentin, an anticonvulsant drug [7]. Following treatment, the effects of polyopia were reduced to palinopsia and the severity of visual disturbances were reduced [7]. Further testing and a larger sample size is required to analyze the effectiveness of these drugs on treating cerebral polyopia as well as the generalizability to future patients.

Medical Follow-up

There is no known evidence or case-reports regarding appropriate medical follow-up.

Additional Resources

References

  1. 1.0 1.1 Gersztenkorn D, Lee AG. Palinopsia revamped: a systematic review of the literature. Surv Ophthalmol. 2015 Jan-Feb;60(1):1-35. doi: 10.1016/j.survophthal.2014.06.003.
  2. 2.0 2.1 2.2 Bender MD. Polyopia and monocular diplopia of cerebral origin. Arch Neurol Psychiatry 1945; 54: 323-38
  3. Zakaria A, Lalani I, Belorgey L, Jay Foreman P. Focal occipital seizures with cerebral polyopia. Epileptic Disord. 2006 Dec;8(4):295-7.
  4. 4.0 4.1 4.2 Jones MR, Waggoner R, Hoyt WF. Cerebral polyopia with extrastriate quadrantanopia: report of a case with magnetic resonance documentation of V2/V3 cortical infarction. J Neuroophthalmol. 1999 Mar;19(1):1-6.
  5. Mingazzini, G. (1908). Uber symptome infolge von verletzungen des occipitallapens dureh Geschosse. Neurologishces Zentralblatt, 27, 1112.
  6. 6.0 6.1 6.2 6.3 6.4 6.5 6.6 Cornblath WT, Butter CM, Barnes LL, Hasselbach MM. Spatial characteristics of cerebral polyopia: a case study. Vision Res. 1998 Dec;38(24):3965-78. doi:10.1016/s0042-6989(97)00431-8.
  7. 7.0 7.1 7.2 7.3 7.4 7.5 7.6 Kataoka H, Ueno S. Cerebral polyopia and palinopsia in a patient with occipital lobe epilepsy. Epilepsy Behav. 2009 Apr;14(4):684-6. doi: 10.1016/j.yebeh.2009.01.026.
  8. 8.0 8.1 9. Safran AB, Kline LB, Glaser JS, Daroff RB. Television-induced formed visual hallucinations and cerebral diplopia. Br J Ophthalmol. 1981 Oct;65(10):707-11. doi:10.1136/bjo.65.10.707.
  9. 9.0 9.1 Raieli V, Eliseo GL, La Franca G, La Vecchia M, Puma D, Eliseo M. Cerebral polyopia in migraine: a clinical case. J Headache Pain. 2000 Dec;1(2):127–9. doi:10.1007/PL00012179.
  10. Gottlieb D. The unidirectionality of cerebral polyopia. J Clin Neuroophthalmol. 1992 Dec;12(4):257-62. doi: 10.3109/01658109209058148.
  11. 11.0 11.1 Kesserwani H. An Analytic Dissection of a Case of Cerebral Diplopia: Is the Human Brain a Holographic Device? Cureus. 2020 Sep 7;12(9):e10292. doi: 10.7759/cureus.10292.
  12. Isherwood S, Jewsbury H, Nitkunan A, Ali N. An unusual case of cerebral polyopia. Can J Ophthalmol. 2017 Jun;52(3):e102-e104. doi: 10.1016/j.jcjo.2016.10.016.
  13. 13.0 13.1 Brady CJ. Diplopia - Eye Disorders. https://www.merckmanuals.com/professional/eye-disorders/symptoms-of-ophthalmologic-disorders/diplopia. Published July 2019.