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Table of Contents    
LETTER TO EDITOR
Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1082-1083

Ocular “Three” Syndrome: A Paraventricular Ocular Syndrome


1 Department of Neurology and Critical Care Neurology, Aster Medcity, Kothad, Kochi, Kerala, India
2 Department of Neurosurgery, Aster Medcity, Kothad, Kochi, Kerala, India

Date of Submission11-Feb-2020
Date of Decision08-Aug-2021
Date of Acceptance07-Jul-2020
Date of Web Publication2-Sep-2021

Correspondence Address:
Boby Varkey Maramattom
Department of Neurology and Critical Care Neurology, Aster Medcity, Kothad, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.325306

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How to cite this article:
Maramattom BV, Haridas N, Nair AP. Ocular “Three” Syndrome: A Paraventricular Ocular Syndrome. Neurol India 2021;69:1082-3

How to cite this URL:
Maramattom BV, Haridas N, Nair AP. Ocular “Three” Syndrome: A Paraventricular Ocular Syndrome. Neurol India [serial online] 2021 [cited 2021 Oct 23];69:1082-3. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1082/325306




Sir,

A 67-year-old man developed acute occipital headache, and CT brain showed a cerebellar parenchymal bleed with IVth ventricular extension. The modified Graeb score [mGS] was 5.[1] For obstructive hydrocephalus, an external ventricular drain [EVD] was placed. The next morning, the mGS was 9. On examination, he was fully conscious, had bilateral ptosis, comitant skew deviation, upbeat nystagmus, and complete loss of bilateral horizontal ocular movements, and impaired upgaze and convergence. Only the downgaze and pupil reflexes were preserved [Figure 1].
Figure 1: Axial FLAIR MRI sections through caudal medulla [a], pons [b], and midbrain [c] showing the distortion of tectum and periventricular CSF seepage. Blue arrow shows the asymmetric left pontine edema near the left abducens nucleus

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MRI showed upward herniation of the cerebellum with buckling of the posterior commissure [PC], midbrain, and pontine edema. However, by the next morning, his ocular symptoms improved and repeat CT showed mild resolution of intraventricular blood and cerebellar herniation. The EVD was removed on day five. Only a partial bilateral horizontal gaze paresis, upgaze paresis, and skew deviation remained.

The eponymous numeric neuroophthalmological brainstem syndromes include the 1½, 8½, 9, 13 ½, 15 ½, and 16 ½ syndromes.[2] Our patient's ocular movement disorder involved bilateral horizontal gaze [1 + 1] with upgaze palsy [1]. We name it the “Three syndrome”.

All the components of the “Three” syndrome can be explained by a “paraventricular” pathology as most of the centers involved in conjugate gaze lie along the “paraventricular” pontomesencephalic region [Figure 2]. These include the primary horizontal gaze center, the parapontine reticular formation [PPRF] and the decussating upgaze fibers in the PC. As bilateral discrete midbrain lesions are required to produce a downgaze palsy, this was spared in our patient. The IVth ventricular expansion caused pontine tegmental edema involving bilateral PPRF with a bilateral horizontal gaze palsy whereas the upward herniation of the cerebellum buckled the posterior commissure [PC] [Figure 3].
Figure 2: Sagittal FLAIR [a] and comparative control T2 weighted MRI [b] images showing midbrain swelling and edema [blue arrow], posterior commissure buckling [orange arrow], and pontine paraventricular edema [green arrow]

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Figure 3: Diagram and MRI overlay the demonstrating regions of interest

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We did a contemplate intraventricular tissue plasminogen activator [tPA] or posterior fossa decompression; however, our patient recovered spontaneously.[3]

In conclusion, the “paraventricular” pontomesencephalic pathology involved the upgaze pathways and bilateral horizontal gaze centers resulting in the ocular “three” syndrome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Morgan TC, Dawson J, Spengler D, Lees KR, Aldrich C, Mishra NK, et al. CLEAR and VISTA investigators. The modified graeb score: An enhanced tool for intraventricular hemorrhage measurement and prediction of functional outcome. Stroke 2013;44;635-41.  Back to cited text no. 1
    
2.
Eggenberger E. Eight-and-a-half syndrome: One-and-a-half syndrome plus cranial nerve VII palsy. J Neuroophthalmol 1998;18:114-6.  Back to cited text no. 2
    
3.
Luong CQ, Nguyen AD, Nguyen CV, Mai TD, Nguyen TA, Do SN, et al. Effectiveness of combined external ventricular drainage with intraventricular fibrinolysis for the treatment of intraventricular haemorrhage with acute obstructive hydrocephalus. Cerebrovasc Dis Extra. 2019;9:77-89.  Back to cited text no. 3
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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