Case Report Eight and a Half Syndrome with Hemiparesis and Hemihypesthesia: The Nine Syndrome? Francesca Rosini, MD,* Elena Pretegiani, MD,* Francesca Guideri, MD,† Alfonso Cerase, MD,‡ and Alessandra Rufa, MD, PhD* ‘‘Eight-and-a-half’’ syndrome is ‘‘one-and-a-half’’ syndrome (conjugated horizontal gaze palsy and internuclear ophthalmoplegia) plus ipsilateral fascicular cranial nerve seventh palsy. This rare condition, particularly when isolated, is caused by circumscribed lesions of the pontine tegmentum involving the abducens nucleus, the ipsilateral medial longitudinal fasciculus, and the adjacent facial colliculus. Its recognition is therefore of considerable diagnostic value. We report a 71-year-old man who presented with eight and a half syndrome associated with contralateral hemiparesis and hemihypesthesia, in which brain magnetic resonance imaging scans revealed a lacunar pontine infarction also involving the corticospinal tract and medial lemniscus. These features could widen the spectrum of pontine infarctions, configuring a possible ‘‘nine’’ syndrome. Key Words: Acute pontine stroke—neuroimaging—neuro-ophthalmology. Ó 2013 by National Stroke Association Case Report A 71-year-old man presented with nausea and vomiting, horizontal diplopia, left lateropulsion, and left hypoesthesia. His medical history was significant for several vascular risk factors, such as obesity, smoking, hypercholesterolemia and, more recently, obstructive sleep apnea syndrome. In 2008, he was hospitalized for angina pectoris; however, no significant stenosis of coronary arteries were seen upon coronarographic examination. At admission to our department, treatment for benign prostatic From the *Eye Tracking & Visual Application Lab, Department of Neurological, Neurosurgical, and Behavioral Sciences; †Stroke Unit, Department of Neurosciences, University of Siena, Italy; and ‡Neuroimaging and Neurointervention Unit, Azienda Ospedaliera Universitaria Senese, Policlinico ‘‘Santa Maria alle Scotte,’’ Siena, Italy. Received December 30, 2012; accepted January 28, 2013. Address correspondence to Alessandra Rufa, MD, PhD, Department of Neurological, Neurosurgical and Behavioral Sciences, University of Siena, viale Bracci 2, Policlinico Santa Maria alle Scotte, Siena, Italy. E-mail: rufa@unisi.it. 1052-3057/$ - see front matter Ó 2013 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2013.01.018 hypertrophy was the sole therapy taken by the patient, and his National Institutes of Health Stroke Scale score was 3. The neurologic examination revealed right horizontal gaze palsy, internuclear ophtalmoplegia (INO), and right-sided lower motor neuron facial nerve palsy associated with left arm slight hemiparesis and hemihypesthesia. A magnetic resonance imaging (MRI) scan revealed an area of restricted diffusion in the right pons involving the abducens nucleus, adjacent medial longitudinal fasciculus (MLF), and facial colliculus, extending to the ipsilateral medial lemniscus and corticospinal tract (Fig 1). The clinical and MRI characteristics were consistent with an acute pontine ischemia. The immediate treatment with antiaggregating therapy (acetylsalicylic acid 300 mg) and the subsequent physiotherapy led to a sensible improvement of the ocular movement disturbances and left arm hyposthenia. Discussion ‘‘Eight and a half’’ syndrome (EHS)1 is the rare association of ‘‘one and a half’’ syndrome2 (i.e., conjugated horizontal gaze palsy [the ‘‘one’’] and internuclear ophthalmoplegia [the ‘‘half’’]), with ipsilateral fascicular Journal of Stroke and Cerebrovascular Diseases, Vol. 22, No. 8 (November), 2013: pp e637-e638 e637 F. ROSINI ET AL. e638 Figure 1. Diffusion-weighted imaging of the brainstem. Consecutive axial images show areas (arrows) of restricted diffusion in the right medulla oblongata and pons, including the corticospinal tract, medial lemniscus, the abducens nucleus illustration (A), and facial colliculus (B). (C) Schematic of the brainstem, noting the structures involved by the lesion. Abbreviations: CST, corticospinal tract; III, oculomotor nerve nucleus; IV, IV ventricle; LR, lateral rectus; ML, medial lemniscus; MLF, medial longitudinal fascicle; MR, medial rectus; VI, troclear nerve nucleus; VII, facial nerve nucleus. cranial nerve VII palsy. First described in detail by Eggenberger3 in 1998, it is caused by a selective unilateral lesion of pontine tegmentum involving the cranial nerve VI nucleus, the internuclear fibers of the ipsilateral medial longitudinal fasciculus, and the adjacent facial colliculus. Most isolated syndromes are associated with pontine lacunar infarction due of the occlusion of small penetrating arteries; other rarer causes, such as infectious, tumors, demyelination, and vasculitis of the brainstem have also been reported.1,4 In addition to EHS, our patient also complained of contralateral persistent hemiparesis and hemihypesthesia of the left arm, which was still present at the discharge. The concomitant involvement of the corticospinal tract and medial lemniscus was consistent with these features and suggested a direct tissue damage caused by ischemia rather than a transient extension of the peri-infarct edema as recently postulated.5 In fact, in our subject, the hemiparesis and hemihypesthesia were persistent and well documented by neuroimaging— findings that differ from those by Uysal et al.5 In conclusion, the clinical features reported in this patient configure a variant of EHS (the ‘‘nine’’ syndrome), widening the spectrum of clinical presentation of pontine infarctions and allowing their precise anatomic localization. References 1. Skaat A, Huna-Baron R. Eight-and-a-half syndrome: A rare pontine neuro-ophthalmologic syndrome. Arch Neurol 2012;69:934-935. 2. Leigh RJ, Zee DS. The neurology of eye movements. 4th ed. New York: Oxford University Press, 2006. 3. Eggenberger E. Eight-and-a-half syndrome: One-anda-half syndrome plus cranial nerve VII palsy. J Neuroophthalmol 1998;18:114-116. 4. Sarwal A, Garewal M, Sahota S, et al. Eight-and-a-halfsyndrome. J Neuroimaging 2009;19:288-290. 5. Uysal S, Demirtas-Tatlidede A, Selcuk OY, et al. Diffusionweighted imaging in eight-and-a-half syndrome presenting with transient hemiparesis. Clin Neuroradiol 2012. Oct 12 [Epub ahead of print].