Thirteen-And-A-Half Syndrome

Clinical Observation Thirteen-And-A-Half Syndrome Daniel S. Allbon, MBChB, PGDipOphthBS, Ben La Hood, MBChB, PGDipOphthBS Abstract: We describe a 50-year-old man who developed eight-and-a-half syndrome associated with an ipsilateral trigeminal nerve palsy because of a post-transplant lymphoproliferative disorder. This case widens the spectrum of eight-and-a-half syndrome to include a thirteen-and-a-half syndrome. Journal of Neuro-Ophthalmology 2016;36:191-192 doi: 10.1097/WNO.0000000000000341 © 2016 by North American Neuro-Ophthalmology Society T he eight-and-a-half syndrome represents a combination of one-and-a-half syndrome and ipsilateral seventh nerve palsy. It localizes the disease process to the pons, commonly as a result of stroke but also from tumor, demyelination, and vasculitis. Our patient had eight-and-a-half syndrome plus ipsilateral fifth nerve palsy. Our case expands the one-and-a-half syndrome. CASE REPORT A 50-year-old man developed horizontal diplopia, right facial numbness and weakness, and ataxia. His medical history included end stage renal failure for which he had received 3 previous renal transplantations. He also had a history of multiple strokes from small vessel disease, recurrent deep vein thromboses, hypertension, gout, and recurrent herpes simplex infections involving the lip. His medications included allopurinol, warfarin, mycophentolate, prednisone, and tacrolimus. The patient was admitted to hospital with a right facial nerve palsy, right trigeminal nerve palsy, and ataxia. On ophthalmic examination visual acuity was hand movements, right eye, and 20/25 left eye. The pupils were isocoric without a relative afferent pupillary defect. There was 3 mm Department of Ophthalmology, Dunedin Hospital, Dunedin, New Zealand. The authors report no conflicts of interest. Address correspondence to Daniel S. Allbon, Department of Ophthalmology, Dunedin Hospital, Private Bag 1921, Dunedin, 9054, New Zealand; E-mail: [e-mail] Allbon and La Hood: J Neuro-Ophthalmol 2016; 36: 191-192 of right lagophthalmos with absent corneal sensation and significant punctate epithelial erosions on the right cornea. There was a complete right horizontal gaze palsy and significant reduction in right eye adduction, with leftbeating nystagmus of the left eye in abduction (Fig. 1). Convergence to near stimuli was intact, and the remaining ocular examination was normal. Initial hematological studies were unremarkable and multiple lumbar punctures negative for herpes simplex virus, herpes zoster virus, cytomegalovirus, and JC virus. Brain magnetic resonance imaging (MRI) demonstrated hyperintensity within the right lateral aspect on the pons, extending into the right cerebellar peduncles along with enhancement around the right trigeminal nerve, and slight enhancement to the right facial vestibulocochlear nuclear complex (Fig. 2). MRI spectroscopy demonstrated the appearance of an aggressive lesion, most suggestive of cerebral lymphoma. Chest computed tomography revealed multiple pulmonary lesions, and a wedge resection of the upper lobe confirmed the diagnosis of a lymphoproliferative disorder. The patient was referred to oncology for further treatment. DISCUSSION One-and-a-half syndrome is comprised of a horizontal gaze palsy in one direction combined with internuclear ophthalmoplegia in the other. This syndrome arises from a unilateral lesion involving either the sixth nerve nucleus and/or the paramedian pontine reticular formation along with the ipsilateral medial longitudinal fasciculus (1,2). Combining one-and-a-half syndrome with ipsilateral facial (seventh) nerve palsy produces eight-and-a-half syndrome (3,4). The affected structures are the same as for one-and-a-half syndrome mentioned above, but includes the ipsilateral facial nerve nucleus. The resulting disorder produces one-and-a-half syndrome and paresis of the ipsilateral facial nerve (3-6). These syndromes occur from a unilateral lesion in the dorsal tegmentum of the pons, most commonly a result of pontine lacunar infarction, but also from demyelinating disorders, infection, tumour, and vasculitis (1,2,4,5,7). 191 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation FIG. 1. The patient has right gaze palsy and right internuclear ophthalmoplegia consistent with one-and-a-half syndrome. The pupils are pharmacologically dilated. FIG. 2. Axial FLAIR (A) and coronal T2 (B) magnetic resonance imaging reveals hyperintensity within the right lateral aspect of the pons extending into the right cerebellar peduncles and right cerebellar hemisphere. Our case is unique in that our patient presented with eight-and-a half syndrome and ipsilateral trigeminal (fifth) nerve palsy. Our patient's reduced visual acuity in the right eye was the result of corneal surface disease due to seventh (neuroparalytic keratitis) and fifth (neurotrophic keratitis) nerve involvement. This report widens the spectrum of eight-and-a-half syndrome to include a thirteen-and-a-half syndrome variant. REFERENCES 1. Fisher CM. Some neuro-ophthalmological observations. J Neurol Neurosurg Psychiatry. 1967;30:383-392. 2. 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;33:971-980. 192 3. Eggenberger E. Eight-and-a-half syndrome: one-and-a-half sydrome plus cranial nerve VII palsy. J Neuroophthalmol. 1998;18:114-116. 4. Sampath Kumar NS, Raju CG, Kiran PR, Kumar TA, Goopal BV, Khassem DB. Eight-and-a-half syndrome: a rare presentation of pontine infarction. J Stroke Cerebrovasc Dis. 2014;23: e389-e391. 5. Kakar P, Brown Z, Banerjee S. Eight-and-a-half syndrome: an unusual presentation of brainstem infarction. Q J Med. 2013;106:273-276. 6. Rosini F, Pretegiani E, Guideri F, Cerase A, Rufa A. Eight-and-ahalf syndrome with hemiparesis and hemihypesthesia; the nine syndrome? J Stroke Cerebrovasc Dis. 2013;22:e637- e638. 7. Felicio AC, Bichuetti DB, Marin LF, dos Santos WA, GodeiroJunior C. Bilateral horizontal gaze palsy with unilateral peripheral facial paralsysi caused by pontine tegmentum infarction. J Stroke Cerebrovasc Dis. 2009;18:244-246. Allbon and La Hood: J Neuro-Ophthalmol 2016; 36: 191-192 Copyright © North American Neuro-Ophthalmology Society. 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