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Year : 2020  |  Volume : 58  |  Issue : 4  |  Page : 304-306

Gliomatosis cerebri - A rare cause of bilateral blindness

1 Department of Ophthalmology, Pondicherry Institute of Medical Sciences, Puducherry, India
2 Department of Neurology, Pondicherry Institute of Medical Sciences, Puducherry, India

Date of Submission22-Jun-2020
Date of Decision04-Aug-2020
Date of Acceptance21-Sep-2020
Date of Web Publication16-Dec-2020

Correspondence Address:
Dr. P Rajarajeswary
109, Jaya Vilas, Cuddalore Road, Mudaliarpet, Puducherry - 605 004
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_76_20

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We describe a rare case of gliomatosis cerebri (GC) with bilateral blindness. A 45-year-male presented with gradual painless loss of vision both eyes (BE), with generalised tonic–clonic seizures. On examination, the patient denied light perception BE, with sluggishly reacting pupil. Fundus examination revealed papilledema and absent foveal reflex. Magnetic resonance imaging showed features of diffuse infiltrating glial tumor suggesting GC. Visual impairment can occur rarely due to optic nerve involvement, however bilateral cases has not been reported. Early recognition of GC may allow preservation of vision through focal radiotherapy to the optic nerve.

Keywords: Glioblastoma, gliomatosis cerebri, neuroimaging, radiotherapy

How to cite this article:
Sekar P, Rajarajeswary P, Prasanth HR, Ishwarya T S, Srinivasan R, Kannan R. Gliomatosis cerebri - A rare cause of bilateral blindness. TNOA J Ophthalmic Sci Res 2020;58:304-6

How to cite this URL:
Sekar P, Rajarajeswary P, Prasanth HR, Ishwarya T S, Srinivasan R, Kannan R. Gliomatosis cerebri - A rare cause of bilateral blindness. TNOA J Ophthalmic Sci Res [serial online] 2020 [cited 2021 Apr 23];58:304-6. Available from: https://www.tnoajosr.com/text.asp?2020/58/4/304/303658

  Introduction Top

Gliomatosis cerebri (GC) is recognized as a specific entity among neuroepithelial tumors of uncertain origin.[1] GC usually involves at least two lobes of the brain and histopathological evaluation reveals a diffuse infiltrative low-grade astrocytoma.[2] Magnetic resonance imaging (MRI) shows a diffuse infiltrative process, particularly on T2 images, and the diagnosis is made by a stereotactic or open biopsy. Surgical treatment of GC is limited due to the extensive diffuse infiltrative process. Currently, there is no effective treatment for GC. Treatment generally includes anticonvulsants and steroids. Radiation therapy in GC causes temporary improvement or stabilization of symptoms in the majority of cases.[3] Despite treatment, the prognosis is very poor with mostly a median survival ranging between 6 and 39 months.[2] Visual impairment can rarely occur due to optic nerve involvement but bilateral blindness has not been reported.

  Case Report Top

A 45-year-old male presented with complaints of gradual painless loss of vision in both eyes (BE). History of generalized tonic–clonic seizures involving right upper limb and lower limb lasting for few minutes. History of post ictal drowsiness present. History of headache on and off present without history of nausea and vomiting. Rest of the systemic examination was within normal limits.

On ocular examination, the patient denied light perception in BE. Pupils were 3 mm round sluggishly reacting to both direct and indirect light. There was no relative afferent pupillary defect (RAPD). Rest of the anterior segment was unremarkable in BE. On dilated fundus examination BE showed blurred disc margins, veins appeared tortuous and arteries appeared attentuated. Rest of the fundus was within normal limits in BE. Differential diagnosis of optic neuritis secondary to demyelinating disorder or nonarteritic ischemic optic neuropathy was made. Patient was started on anticonvulsants (Levipil 500 mg) thrice a day along with carbamazepine (Tegretol 400 mg) thrice a day, oral steroids (Dexa 4 mg) twice a day. MRI brain showed diffuse infiltrating glial tumor suggestive of GC. Ill defined, poorly demarcated confluent T2 and fluid attenuated inversion recovery hyperintensities involving bilateral periventricular white matter, bilateral basal ganglia, bilateral insular cortex, bilateral thalami, bilateral median temporal lobes, bilateral centrum semiovale, midbrain and splenium of corpus callosum and bilateral cingulated gyri [Figure 1], [Figure 2]. These areas show subtle hypointense signal on T1-weighted images and did not show diffusion restriction. Loss of gray white matter interface is seen in many regions. The rest of the cerebral parenchyma is normal. No mid line shift. Both cerebral hemispheres are normal. Biopsy was advised to confirm the diagnosis and to follow the patient for definitive surgery/radiotherapy/adjuvant chemotherapy. The patient declined treatment in view of the poor prognosis to life.
Figure 1: Magnetic resonance imaging fluid attenuated inversion recovery showing periventricular infiltrations

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Figure 2: T1-weighted magnetic resonance imaging brain showing hypointense areas

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  Discussion Top

GC is characterized by a diffuse infiltration of neoplastic glial cells with preservation of neuronal architecture. In the latest WHO classification, it is graded as a subgroup of neuroepithelial tumors of uncertain origin with involvement of at least two lobes without a cellular, centrally necrotic center.[4] In 2016, GC was eliminated as a distinct pathological entity based on two factors which includes overlap of discrete molecular alterations with other high-grade gliomas, and the absence of distinct molecular or histologic features compared to other malignant gliomas.[5] Clinical presentation includes a variety of neurological symptoms, including headache, cranial nerve involvement, vomiting, meningismus, and decreased mental status. Raised intracranial pressure is the most constant sign. The most common neuro-ophthalmic signs reported are papilledema and cranial sixth nerve palsy. Other abnormalities include third and fourth nerve palsies, nystagmus and vision loss. Double vision is the most common visual complaint, primarily due to sixth nerve palsy.

Optic nerve involvement has been reported but is uncommon. Bilateral total visual loss has not reported to the best of our knowledge. This case had extensive tumor spread as seen on imaging studies and this may account for bilateral affection of the visual pathways well. Early recognition of optic nerve involvement and prompt chemotherapy may arrest the progress of this invasive tumor and thereby salvage vision.

GC is best detected with MRI. MRI with T2-weighted images is considered the gold standard imaging technique for the diagnosis of GC. Typical finding includes diffuse infiltration of the cortex with an enlargement of the cortical sulci and poor demarcation of the gray and white matter.[6] Cerebrospinal fluid analysis is often nonspecific. Meningeal biopsy is necessary to establish a diagnosis. Because of the diffuse nature of GC, surgery is not usually advised. Large field radiotherapy carries severe risk of toxicity. Initial chemotherapy is useful for some patients with GC. Temozolomide, an alkylating chemotherapeutic drug, is well tolerated and valuable alternative to vincristine, especially for those with slow-growing, low-grade GC.[7] Response rates to chemotherapy are poor with a median survival of <3 years.

In a study done to evaluate the outcome with radiotherapy in GC patients by Perkins et al. they concluded that increased radiotherapy volume, extensive surgery, administration of chemotherapy neither improved overall survival nor the time to disease progression in a median follow-up of 12.8 months.[8]

In a retrospective study done on Review of twenty two cases by Vates et al. regarding the utility of treatment they concluded that biopsies are useful not only for diagnosis but also for prediction of the length of survival.[9]

In another study done by Kong et al., in 45 patients retrospectively to study the impact of adjuvant chemotherapy for GC they concluded that adjuvant chemotherapy following radiotherapy could prolong the survival in patients with GC.[10]

  Conclusion Top

GC is a rare tumor of the central nervous system. Transformation into glioblastoma may occur a few years later. MRI with T2-weighted images is considered the gold standard imaging technique for the diagnosis of GC. Despite treatment, the prognosis is very poor. Early recognition of GC may allow preservation of vision through focal radiotherapy to the optic nerve. Furthermore, direct visualization of the tumor on funduscopy provides a potential opportunity to follow progression of disease and treatment response as an adjunct to neuroimaging.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kleihues P, Cavenee WK. Pathology and Genetics of Tumours of the Nervous System. IARC Press,Lyon,2000.  Back to cited text no. 1
Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen Z. Presentation patterns and outcome of gliomatosis cerebri. Oncol Lett 2012;3:209-13.  Back to cited text no. 2
Horst E, Micke O, Romppainen ML, Pyhtinen J, Paulus W, Schäfer U, et al. Radiation therapy approach in gliomatosis cerebri-Case reports and literature review. Acta Oncol 2000;39:747-51.  Back to cited text no. 3
Kleihues P, Soylemezoglu F, Schäuble B, Scheithauer BW, Burger PC. Histopathology, classification, and grading of gliomas. Glia 1995;15:211-21.  Back to cited text no. 4
Morales La Madrid A, Ranjan S, Warren KE. Gliomatosis cerebri: A consensus summary report from the second international gliomatosis cerebri group meeting, June 22-23, 2017, Bethesda, USA. J Neurooncol 2018;140:1-4.  Back to cited text no. 5
Elshaikh MA, Stevens GH, Peereboom DM, Cohen BH, Prayson RA, Lee SY, et al. Gliomatosis cerebri: Treatment results with radiotherapy alone. Cancer 2002;95:2027-31.  Back to cited text no. 6
Sanson M, Cartalat-Carel S, Taillibert S, Napolitano M, Djafari L, Cougnard J, et al. Initial chemotherapy in gliomatosis cerebri. Neurology 2004;63:270-5.  Back to cited text no. 7
Perkins GH, Schomer DF, Fuller GN, Allen PK, Maor MH. Gliomatosis cerebri: Improved outcome with radiotherapy. Int J Radiat Oncol Biol Phys 2003;56:1137-46.  Back to cited text no. 8
Vates GE, Chang S, Lamborn KR, Prados M, Berger MS. Gliomatosis cerebri: A review of 22 cases. Neurosurgery 2003;53:261-71.  Back to cited text no. 9
Kong DS, Kim ST, Lee JI, Suh YL, Lim DH, Kim WS, et al. Impact of adjuvant chemotherapy for gliomatosis cerebri. BMC Cancer 2010;10:424.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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