A rare neuro-ophthalmological presentation of Nine syndrome: clinical radiological-anatomical correlation
DOI:
https://doi.org/10.18203/2320-6012.ijrms20261720Keywords:
Nine syndrome, One-and-a-half syndrome, Pontine infarct, Internuclear ophthalmoplegia, Facial palsy, Basilar artery aneurysm, Brainstem strokeAbstract
Nine syndrome is a very rare neuro-ophthalmological entity resulting from combined involvement of the abducens nucleus or paramedian pontine reticular formation (PPRF), the medial longitudinal fasciculus (MLF), the facial colliculus, and the corticospinal tract. This constellation produces the combination of horizontal gaze palsy, internuclear ophthalmoplegia, ipsilateral facial palsy, and contralateral hemiparesis/ hemianesthesia/ hemiataxia together constitute Nine syndrome (7th nerve+1 and 1/2 syndrome+1/2 hemiparesis/ hemianesthesia/ hemiataxia). Our objective was to present a case of nine syndrome providing a comprehensive clinical examination, detailed radiological evaluation and anatomical correlation to explain the neurological findings. This case highlights the importance of recognizing characteristic neuro-anatomical correlations in pontine stroke syndromes. We report a case of a 57-year-old male who presented with sudden-onset left hemiplegia, dysarthria, and binocular diplopia. Neurological examination revealed right horizontal conjugate gaze palsy, right internuclear ophthalmoplegia, right lower motor neuron facial palsy, and left hemiplegia-clinically consistent with nine syndrome. MRI brain revealed an acute right paramedian pontine infarct with basilar artery aneurysm. The patient was managed with antiplatelet therapy, high-intensity statin, glycemic control, and supportive care. Clinical improvement was noted over the following days. This case highlights the importance of recognizing combined cranial nerve and long-tract signs in localizing pontine lesions. Nine syndrome, though rare, should be considered in patients presenting with horizontal gaze palsies and contralateral hemiparesis and aggressive vascular risk-factor management is critical for preventing recurrence.
References
Freeman W, Ammerman H, Stanley M. Syndromes of the pontile tegmentum: Foville’s syndrome: report of three cases. Arch Neurol Psychiatry. 1943;50;(4):462-71.
Fisher CM. Some neuro-ophthalmological observations. J Neurol Neurosurg Psychiatry. 1967;30(5):383-92.
Eggenberger E. Eight-and-a-half syndrome. J Neuroophthalmol. 1998;18:114-6.
Rosini F, Pretegiani E, Guideri F, Alfonso C, Alessandra R. Eight and a half syndrome with hemiparesis and hemihypesthesia: the nine syndrome. J Stroke Cerebrovasc Dis. 2013;22(8):e637-8.
Tiwari N, Sharma S, Madhusudan BK, Mathad VU. Nine Syndrome: A rare neuroopthalmological syndrome associated with HIV vasculopathy. Indian J Neurosurg. 2019;09(01):1-3.
Ng CY, Yong GY, Ngai JJ, Zainal-Abidin ZB. A rare diagnosis of nine syndrome: Clinical, imaging findings, and literature review. Taiwan J Ophthalmol. 2023;14(3):431-6.
Kwiatkowska M, Rzepliński R, Ciszek B. Anatomy of the pontine arteries and perforators of the basilar artery in humans. J Anat. 2023;243(6):997-1006.
Keane JR. Internuclear ophthalmoplegia: Unusual causes in 114 of 410 patients. Arch Neurol. 2005;62:714-7.