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Year : 2019  |  Volume : 57  |  Issue : 4  |  Page : 323-325

Supranuclear vertical gaze palsy with midbrain cavernoma: A rare case of Parinaud's syndrome

Department of Ophthalmology, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth, Puducherry, India

Date of Submission30-Jul-2019
Date of Acceptance24-Aug-2019
Date of Web Publication26-Dec-2019

Correspondence Address:
Dr. Muthukrishnan Vallinayagam
Department of Ophthalmology, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth, Puducherry
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_67_19

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Parinaud's syndrome or dorsal midbrain syndrome is a supranuclear vertical gaze palsy, characterized by lid retraction, convergence-retraction nystagmus, light-near dissociation, and spastic-paretic accommodation. It is caused by midbrain or pineal gland tumors, stroke, obstructive hydrocephalus, thalamic hemorrhage, encephalitis, demyelination, brain abscess, and trauma. We report a case of Parinaud's syndrome in a young patient secondary to cavernoma of the midbrain, a rare vascular malformation. The major manifestations included diplopia, skew deviation, restriction of upgaze, light-near dissociation, and convergence-retraction nystagmus. In this case, the visual symptoms were the premonitory presentations instigating medical attention. Parinaud's syndrome was promptly recognized and was referred to a neurosurgeon. An expeditious neuroimaging and prompt neurosurgical intervention contributed to a favorable outcome. Midbrain tumors presenting with vertical gaze palsies have been well documented in the literature. This case highlights the role of an ophthalmologist in the early diagnosis of Parinaud's syndrome which can be instrumental in influencing further management.

Keywords: Convergence-retraction nystagmus, light-near dissociation, vertical gaze palsy

How to cite this article:
Vallinayagam M, Anudeep K, Sajeeth K. Supranuclear vertical gaze palsy with midbrain cavernoma: A rare case of Parinaud's syndrome. TNOA J Ophthalmic Sci Res 2019;57:323-5

How to cite this URL:
Vallinayagam M, Anudeep K, Sajeeth K. Supranuclear vertical gaze palsy with midbrain cavernoma: A rare case of Parinaud's syndrome. TNOA J Ophthalmic Sci Res [serial online] 2019 [cited 2021 Oct 20];57:323-5. Available from: https://www.tnoajosr.com/text.asp?2019/57/4/323/273977

  Introduction Top

Parinaud's syndrome is a supranuclear paralysis of vertical gaze, resulting from damage to the mesencephalic tectum.[1] It consists of abnormalities of ocular movement and pupil such as vertical gaze palsies, skew deviation, convergence-retraction nystagmus, light-near dissociation, spastic-paretic accommodation, and pathological lid retraction.[2] It is due to direct or compressive injury to the dorsal midbrain, resulting from pineal, midbrain, or third ventricle tumors, cerebrovascular disorders, aqueductal stenosis, multiple sclerosis, arteriovenous malformations, encephalitis, obstructive hydrocephalus, and uncal herniation.[3],[4] Here, we report an extremely unusual case of midbrain cavernoma manifesting as Parinaud's syndrome.

  Case Report Top

A 23yearold female patient presented with diplopia and headache for two days. There was no history of fever, nausea, vomiting, drowsiness, seizures, or focal neurological deficits. There were no systemic features of myasthenia gravis or hyperthyroidism. There was no history of trauma. She had no systemic comorbidities such as diabetes mellitus or hypertension.

The visual acuity was 20/20 in both eyes. Hirschberg test revealed a mild vertical misalignment of eyes, suggesting skew deviation [Figure 1]. The direct and consensual light reflexes were sluggish in both eyes [Figure 2]. Light-near dissociation was present, exemplified by brisk constriction of the pupils to a near target. There was restriction of upgaze, and other extraocular movements were normal [Figure 3]. There was binocular diplopia for distance and near. Convergence-retraction nystagmus was observed on attempted upgaze [Figure 4]. Lid retraction was absent. Fundus examination showed bilaterally normal optic disc.
Figure 1: Primary gaze with skew deviation

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Figure 2: Sluggish pupillary reactions to light

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Figure 3: Upgaze restriction with normal dextroversion and levoversion

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Figure 4: Convergence and retraction on attempted upgaze

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A provisional diagnosis of Parinaud's syndrome was made. Magnetic resonance imaging (MRI) revealed a well-defined heterogeneous lesion with surrounding T2 hypointense rim, measuring 2.2 cm × 2.3 cm with its epicenter in the dorsal midbrain, extending into the posterior part of third ventricle, which shows focal areas of diffusion restriction. There was minimal mass effect on the cerebral aqueduct. The lesion demonstrated “blooming.” The lesion predominantly appeared hyperintense on T1-weighted images [Figure 5].
Figure 5: (a) Sagittal section showing heterogeneously T1-weighted hyperintense lesion noted in the midbrain. (b) FLAIR axial sections of brain showing ill-defined hyperintense lesion noted in midbrain with perilesional edema extending up to the thalamus on the right side

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The patient was immediately referred to a neurosurgeon. She subsequently underwent posterior occipital craniotomy with surgical removal of cavernoma. Resolution of clinical features was observed in 4 weeks. The patient is being followed up for residual tumor or recurrence.

  Discussion Top

Parinaud's syndrome, also known as the Sylvian aqueduct or pretectal syndrome, results from damage to the pretectum, posterior commissure, and superior colliculus.[3] This condition presents with supranuclear vertical gaze palsy, affecting either upgaze alone or both upgaze and downgaze. The other concomitant ocular features include impaired pupillary light reactions, light-near dissociation, Collier's lid retraction sign, and skew deviation. A classical contemporary sign is convergence-retraction nystagmus.[5]

A literature review of 40 consecutive cases of Parinaud's syndrome documented that the most common presenting complaints were diplopia (67.5%) and blurring of vision (25%), followed in the descending order of incidence by visual field defect, ataxia, and squint. The predominant clinical signs were vertical gaze palsy (100%), convergence-retraction nystagmus (87.5%), and light-near dissociation (65%).[6] The major manifestations in this patient were diplopia, restriction of upgaze, light-near dissociation, and convergence-retraction nystagmus. The presence of diplopia in this patient is presumably due to the phenomenon of skew deviation.

Vertical gaze palsy (upgaze or downgaze) is an aftermath of lesions involving bilateral rostral interstitial nucleus of medial longitudinal fasciculus or unilateral posterior commissure. Convergence-retraction nystagmus is caused by a lesion in the dorsal mesencephalon.[7],[8] The patient discussed here presented with both of these cardinal features. A relatively good prognosis was presumably consequential to an early diagnosis in this case.

The major etiological factors implicated in Parinaud syndrome are pineal gland and midbrain tumors, stroke involving collicular branches of posterior choroidal artery, and obstructive hydrocephalus below the Sylvian aqueduct. Thalamic hemorrhage is more common than ischemic stroke.[3],[6] Encephalitis, demyelination, brain abscess, trauma, posterior tentorial herniation, and Wernicke's syndrome are unusual causes.[3],[4] The lesion in this case was a midbrain cavernoma, an unusual brain tumor which has rarely been reported as a cause of Parinaud's syndrome.[6]

Cavernoma represents 10% of intracranial vascular malformations, which commonly occur in the supratentorial space. When located in the brainstem, these cavernous malformations exhibit a predilection for pons and midbrain.[9]

Cavernomas that have previously bled are usually detectable on MRI due to the prominent signal intensity of hemorrhagic products. The intact lesions are almost invisible on imaging. These tumors may subsequently develop dystrophic calcifications that are picked up on computed tomographic scan. Contrast enhancement is inconsistent and often nonspecific. In cases of ruptured cavernoma, the presence of multiple coexisting cavernomas offers corroborative evidence for the diagnosis.[10] In this case, MRI unveiled a well-defined heterogeneous lesion with hypointense rim in the dorsal midbrain.

Midbrain cavernoma could manifest as Parinaud's syndrome, as portrayed in this report. Parinaud's syndrome must be considered in a patient presenting with vertical gaze palsy of sudden onset. A convergence-retraction nystagmus is highly suggestive. A vigilant approach and prompt neuroimaging targeted at the midbrain discloses the underlying lesion in Parinaud's syndrome.

  Conclusion Top

Cavernoma of the midbrain is an unaccustomed cause of Parinaud's syndrome. This further supports current literature relating the syndrome with midbrain tumors. This case highlights the role of ophthalmologists in the early diagnosis of Parinaud's syndrome. Early clinical recognition is indispensable for successful neurosurgical management. The importance of urgent neuroimaging cannot be overemphasized.

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

Hironishi M, Koshimura I, Nishi K, Komiya T, Mizuno Y. Pretectal syndrome caused by a plaque of multiple sclerosis. Rinsho Shinkeigaku 1994;34:236-40.  Back to cited text no. 1
Azar-Kia B, Palacios E, Churchill R. Aqueductal stenosis and Parinaud's syndrome. IMJ Ill Med J 1975;148:532-3.  Back to cited text no. 2
Keane JR. The pretectal syndrome: 206 patients. Neurology 1990;40:684-90.  Back to cited text no. 3
Walsh FB, Hoyt WF, Miller NR, editors. Walsh and Hoyt's Clinical Neuro-Ophthalmology: the Essentials. 2nd ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams and Wilkins; 2008. p. 539.  Back to cited text no. 4
Ahmad K, Ansari S, Dhungel K, Gupta M, Rauniyar R, Mishra N. Pineal germinoma presenting with Parinaud's syndrome. Health Renaissance 2014;11:276-8.  Back to cited text no. 5
Shields M, Sinkar S, Chan W, Crompton J. Parinaud syndrome: A 25-year (1991-2016) review of 40 consecutive adult cases. Acta Ophthalmol 2017;95:e792-3.  Back to cited text no. 6
Thurtell MJ, Leigh RJ, Halmagyi GM. Monocular ophthalmoplegia and partial supranuclear vertical gaze palsy due to unilateral paramedian rostral midbrain infarction. J Neurol 2009;256:664-6.  Back to cited text no. 7
Clark JM, Albers GW. Vertical gaze palsies from medial thalamic infarctions without midbrain involvement. Stroke 1995;26:1467-70.  Back to cited text no. 8
Li ST, Zhong J. Surgery for mesencephalic cavernoma: Case report. Surg Neurol 2007;67:413-7.  Back to cited text no. 9
Tong KA, Ashwal S, Obenaus A, Nickerson JP, Kido D, Haacke EM, et al. Susceptibility-weighted MR imaging: A review of clinical applications in children. AJNR Am J Neuroradiol 2008;29:9-17.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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