Journal of Neuro-Ophthalmology, June 2013, Volume 33, Issue 2

16 Syndrome in a Patient With Multiple Sclerosis

We read with interest the report of Connors et al (1) of a case of 16 syndrome with complete horizontal gaze paralysis and facial diplegia caused by a pontine hemorrhage. We describe a patient with multiple sclerosis(MS) who experienced an evolving eye movement disorder, which included internuclear ophthalmoplegia, one-and-a-half syndrome, eight-and-a-half syndrome, fifteen-and-a-half syndrome, and finally 16 syndrome.

REFERENCES 1. Rajagopal R, Bateman A, Van Stavern GP. Visual involvement in corticobasal syndrome. J Neuroophthalmol. 2012;32:338-340. 2. Spector RH, Davidoff RA, Schwartzman RJ. Phenytoin-induced ophthalmoplegia. Neurology. 1976;31:1188-1190. 3. Edis RH, Mastaglia FL. Vertical gaze palsy in barbiturate intoxication. BMJ. 1977;1:144. 4. Mullally WJ. Carbamazepine-induced ophthalmoplegia. Arch Neurol. 1982;39:64. 5. Van der Zwan A. Transient parkinson syndrome and tremor caused by the use of sodium valproate. Ned Tijdschr Geneeskd. 1989;133:1230-1232. 6. Aksay E, Baker R, Seung HS, Tank DW. Anatomy and discharge properties of pre-motor neurons in the goldfish medulla that have eye-position signals during fixations. J. Neurophysiol. 2000;84:1035- 1049. 7. Horn AKE, Helmchen C, Wahle P. GABAergic neurons in the rostral mesencephalon of the macaque monkey that control vertical eye movements. Ann NY Acad Sci. 2003; 1004:19-28. 8. Ohashi T. Neuron group of rostral midbrain associated with selective vertical gaze palsy in alert cats. Hokkaido Igaku Zasshi. 1995;70:141-157. 16 Syndrome in a Patient With Multiple Sclerosis We read with interest the report of Connors et al (1) of a case of 16 syndrome with complete horizon-tal gaze paralysis and facial diplegia caused by a pontine hemorrhage. We describe a patient with multiple sclerosis (MS) who experienced an evolving eye movement disorder, which included internuclear ophthalmoplegia, one-and-a-half syndrome, eight-and-a-half syndrome, fifteen-and-a-half syndrome, and finally 16 syndrome. A 44-year-old man with a history of hypertension, heavy smoking, and a positive family history of cardio-vascular disease complained of double vision upon awakening. Neurological examination revealed bilateral internuclear ophthalmoplegia (INO), which was attrib-uted to a brainstem infarction given the sudden onset and patient's vascular risk factors. Magnetic resonance imaging (MRI) of the brain and the intracranial vessels revealed several periventricular white matter lesions but no brainstem abnormality. Three days later, the patient developed drooping of the right corner of his mouth and complained of drooling. Examination revealed a right lower motor neuron facial nerve paresis in addition to bilateral INO. Repeat MRI revealed a nonenhancing le-sion in the midline of the dorsal pons (Fig. 1). Two days later, he developed a complete right conjugate horizontal gaze palsy which, coupled with his left INO, produced a one-and-a-half syndrome. Concurrently, he also experienced bilateral facial paresis. Evaluation for stroke including extensive hematological tests, Holter cardiac monitoring, and transesophageal echocardiography was normal. Cerebrospinal fluid analysis for IgG, oligoclonal bands, and aquaporin-4 antibodies as well as visual evoked responses were normal. MRI of the cervical spinal cord revealed no lesions. On the 11th day of hospitalization, the patient developed bilateral horizontal gaze palsies with worsening of his facial diplegia. His clinical course in combination with the periventricular white matter lesions was highly suggestive of demyelinating disease. Treatment with methylprednisolone FIG. 1. Axial fluid-attenuated inversion recovery (A) and sagittal T2 (B) MRI show an area of high signal (arrows) in the dorsal pons ventral to the fourth ventricle. Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 202-207 203 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. was begun, and 3 weeks later, he had recovered com-pletely. Several follow-up MRIs were performed. The most recent MRI, 1.5 years after the onset of symptoms, revealed an increase in number and extent of the white matter hyperintensities (Fig. 2). Glatiramer acetate therapy was initiated. On occasion, a one-and-a-half syndrome can be accom-panied by a facial paresis if the fascicle or nucleus of the seventh cranial nerve in the lower part of the dorsal pontine tegmentum is also affected. Eggenberger (2) designated this as eight-and-a-half (1.5 1 7) syndrome. As mentioned by Connors et al (1), variations of this syndrome caused by pathology of the dorsal pontine tegmentum have since been described including a combination of a one-and-a-half syn-drome and a bilateral peripheral facial paresis which Bae and Song (3) designated fifteen-and-a-half (1.5 1 7 1 7) syndrome. Only 3 cases of isolated eight-and-a-half syndrome caused by MS have been described in the literature (4,5). In one of these cases (4), the eight-and-a-half syndrome was, as in our patient, the initial symptom of MS. A 16 syn-drome caused by MS has not been reported previously. Hennie Lee, MD Paul L.M. de Kort, MD, PhD Department of Neurology, St. Elisabeth Ziekenhuis, Tilburg, the Netherlands, [e-mail] The authors report no conflicts of interest. REFERENCES 1. Connors R, Ngan V, Howard J. A case of complete lateral gaze paralysis and facial diplegia: the 16 syndrome. J Neuroophthalmol. 2012. 2. Eggenberger ER. Eight-and-a-half syndrome: one-and-a-half syndrome plus cranial nerve VII palsy. J Neuroophthalmol. 1998;18:114-116. 3. Bae JS, Song HK. One-and-a-half syndrome with facial diplegia: the 15½ syndrome? J Neuroophthalmol. 2005;25:52-53. 4. Andreé C, De Castro AL, Vincent MB, De Mattos JP, Maranhao Filho Pde A, Novis SA. One-and-a- half syndrome: anatomical-clinical considerations apropos of a case. Ag Neuropsiquiatr. 1989;47:365-370. 5. Rufa A, Cerase A, De Santi L, Mandala M, Nuti D, Giorgio A, Annunziata P. Impairment of vertical saccades from an acute pontine lesion in multiple sclerosis. J Neuroophthalmol. 2008;28:305-307. Optic Disc Edema and Optic Nerve Head Drusen We are concerned about the conclusions reported by Sarac et al (1) in their article entitled "Differentia-tion of optic disc edema from optic nerve head drusen with spectral-domain optical coherence tomography" and the application of these conclusions to clinical practice. Sarac et al seek to answer an old and important neuro-ophthalmic question: How can one distinguish between optic disc edema and optic nerve head drusen? Most clinicians have no trouble diagnosing advanced optic disc edema, such as Frisen Stages 3, 4, and 5 (2). Most clinicians have no trouble diagnosing optic nerve head drusen that are visible on ophthalmoscopy ("visible drusen"). Where clini-cians do find themselves in a quandary is when they are asked to distinguish mild cases of optic disc edema (Frisen Stages 0, 1, and 2) from optic nerve head drusen that are not visible on ophthalmoscopy ("buried drusen"). Clinicians frequently look to new tools such as optical coherence tomography (OCT) to help sort out these difficult situations. FIG. 2. Axial fluid-attenuated inversion recovery (A) and sagittal T2 (B) scans reveal several areas of high signal intensity in the periventricular region and corpus callosum. 204 Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 202-207 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.