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PHOTO ESSAY
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 839-841

One-and-a-half syndrome with crossed hemiparesis, upgaze palsy, and paralytic pontine exotropia - An unusual amalgam


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

Date of Submission08-Oct-2020
Date of Acceptance21-Mar-2021
Date of Web Publication09-Oct-2021

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


DOI: 10.4103/ijo.IJO_3204_20

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  Abstract 


Keywords: Crossed hemiparesis, one-and-a-half syndrome, paralytic pontine exotropia (PPE), upgaze palsy


How to cite this article:
Vallinayagam MK, Sainath D, Sebastian S, Rekha C S. One-and-a-half syndrome with crossed hemiparesis, upgaze palsy, and paralytic pontine exotropia - An unusual amalgam. Indian J Ophthalmol Case Rep 2021;1:839-41

How to cite this URL:
Vallinayagam MK, Sainath D, Sebastian S, Rekha C S. One-and-a-half syndrome with crossed hemiparesis, upgaze palsy, and paralytic pontine exotropia - An unusual amalgam. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 18];1:839-41. Available from: https://www.ijoreports.in/text.asp?2021/1/4/839/327664



“One-and-a-half” syndrome is a concomitance of horizontal gaze palsy and ipsilateral internuclear ophthalmoplegia (INO). It is a dysconjugate gaze palsy with impaired adduction ipsilaterally and nystagmus on abduction contralaterally. A 60-year-old hypertensive patient presented with right hemiparesis and binocular diplopia. Clinical examination unveiled limited adduction in the left eye, nystagmus on abduction in the right eye, left gaze palsy, right-sided facial weakness, right exotropia, upgaze palsy, and defective convergence. Crossed hemiparesis and facial palsy may confound the diagnosis. Contralateral paralytic pontine exotropia (PPE) and upgaze palsy are unusual features. A conglomerate of the above manifestations may pose a diagnostic ambiguity in one-and-a-half syndrome. This report describes one-and-a-half syndrome with contralateral upper motor neuron facial palsy complementing the “crossed” hemiparesis.

A 60-year-old hypertensive lady presented with sudden onset of weakness of the right side of the body with binocular diplopia and outward deviation of the right eye for 24 h.

The right eye demonstrated 30° exotropia with limited adduction while both adduction and abduction were absent in the left eye. The only horizontal movement in the right eye was abduction, associated with ataxic nystagmus on lateral gaze [Figure 1]. A partial limitation of upgaze was noted [Figure 2]. There was right-sided hemiparesis with ipsilateral (right) upper motor neuron type of facial palsy [Figure 3]. Magnetic resonance imaging of the brain in T2-weighted fluid-attenuated inversion recovery sequence shows acute focal non-hemorrhagic infarct as white matter hyperintense lesion in the left caudate nucleus and left central pons due to basilar artery thrombosis [Figure 4]; apparent diffusion coefficient shows the corresponding hypointensity in hemi pons and diffusion-weighted imaging shows diffusion limitation as hyperintensity at the site of infarct [Figure 5].
Figure 1: (a) 30° right exotropia in primary gaze, (b) dextroversion showing adduction limitation in the left eye and horizontal nystagmus in the right eye, (c) levoversion showing adduction limitation in the right eye and abduction restriction in the left eye

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Figure 2: (a) Left internuclear ophthalmoplegia showing limited upgaze palsy, (b) 30° right exotropia in primary gaze, and (c) normal down gaze

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Figure 3: Right-sided facial nerve palsy Upper Motor Neuron (UMN type) demonstrating deviation of angle of mouth to the left and loss of prominence of the right nasolabial fold

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Figure 4: Magnetic resonance imaging T2-weighted fluid-attenuated inversion recovery sequence revealing white matter hyperintense lesion in hemi pons and midbrain indicating an infarct

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Figure 5: (a) Magnetic resonance imaging apparent diffusion coefficient showing corresponding hypointensity in hemi pons suggesting an infarct, (b) magnetic resonance imaging diffusion-weighted imaging showing diffusion limitation as hyperintensity at the site of infarct

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  Discussion Top


“One-and-a-half” syndrome is characterized by a confluence of horizontal gaze palsy and ipsilateral INO, consequential to a lesion involving paramedian pontine reticular formation and medial longitudinal fasciculus, respectively.[1],[2],[3]

Stroke and multiple sclerosis are the prime causes of INO.[1],[2] One-and-a-half syndrome manifested secondary to brain stem ischemic event in this patient.

Exotropia may be encountered in the acute stage of INO which tends to be overlooked, owing to its rapid resolution. “Paralytic pontine exotropia” is contralateral exotropia in one-and-a-half syndrome associated with abducting nystagmus.[4]

The involvement of the rostral interstitial nucleus of medial longitudinal fasciculus and posterior commissure is a plausible explanation for vertical gaze palsy in bilateral INO.[5] Upgaze palsy was a conspicuous feature associated with unilateral one-and-a-half syndrome in this patient.

Defective convergence here favors an anterior lesion in the midbrain. However, this localizing value has fallen into disfavor as evidenced in current literature.[1]

An amalgamation of crossed hemiparesis, paralytic pontine exotropia, vertical gaze palsy, and convergence deficit may confound a case of one-and-a-half syndrome. However, these concurrent clinical signs should be borne in mind to clinch the right diagnosis.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Xue F, Zhang L, Zhang L, Ying Z, Sha O, Ding Y. One-and-a-half syndrome with its spectrum disorders. Quant Imaging Med Surg 2017;7:691-7.  Back to cited text no. 1
    
2.
Johkura K, Kudo Y, Amano Y, Kikyo H, Imazeki R, Amari K, et al. Gaze palsy and exotropia in internuclear ophthalmoplegia. J Neurol Sci 2015;353:158-60.  Back to cited text no. 2
    
3.
Wall M, Wray SH. The one-and-a-half syndrome-A unilateral disorder of the pontine tegmentum: A study of 20 cases and review of the literature. Neurology 1983;33:971-80.  Back to cited text no. 3
    
4.
Feroze KB, Wang J. Internuclear Ophthalmoplegia. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020.  Back to cited text no. 4
    
5.
Wu Y-T, Cafiero-Chin M, Marques C. Wall-eyed bilateral internuclear ophthalmoplegia: Review of pathogenesis, diagnosis, prognosis and management. Clin Exp Optom 2015;98:25-30.  Back to cited text no. 5
    


    Figures

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



 

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