Eur Neurol 2001;45:54–56 Sensory Ataxia in Claude’s Syndrome Tomás Segura, Pedro Villanueva, José M. Puentes, Manuel D. Zorita Neurology Service, Hospital General de Albacete, Spain In 1912, French neurologist Henri Claude described a midbrain syndrome of hemiataxia with contralateral ocular ptosis and ophthalmoplegia that spared abduction [1]. He located the lesion in the red nucleus and the superior cerebellar tract at the paramedian mesencephalon, without involvement of the substantia nigra and the cerebral peduncles [2]. This syndrome has rarely been described in the literature. 54 Short Reports Case Report A 72-year-old male patient with a long history of arterial hypertension was admitted due to sudden gait instability and diplopia. On admission, there was complete left ptosis and severely impaired motility of the left eye on upgaze, downgaze and adduction with impaired abduction. The left pupil was ovoid but not dilated, and both pupils had normal photomotor responses. No additional cranial nerve abnormalities or pyramidal signs were detected. The patient suffered considerable gait instability and right limb ataxia that was worse when his eyes were shut. He complained of slight right hemihypoesthesia, and there was decreased vibration and position sense on the right side, while pain and temperature perception was normal. The patient’s blood pressure remained normal during the period of hospitalization. Blood analyses were normal except for mild hyperglycemia (233 mg/dl). The cranial computed tomography practised on admission was normal, but an MRI of the brain performed 48 h later showed a left mesencephalic tegmento-basal infarction (fig. 1). Duplex scanning of the supra-aortic trunks showed minor (! 30%) bilateral atheromatosis and normal blood flow in both vertebral arteries. Gait instability and limb ataxia were almost completely resolved 10 days after admission, but the vertical gaze palsy had only slightly regressed and the ptosis remained unchanged. Discussion We report a patient with a crossed midbrain syndrome corresponding to the definition of Claude’s syndrome (ipsilateral fascicular third nerve palsy with contralateral hemiataxia). We think that this case has some peculiarities. Firstly, the patient has a well-delimited midbrain ischemic lesion (fig. 1) which is unusual, as mesencephalic infarcts normally extend rostrally towards the thalamus [3]. This infarction was probably due to the occlusion of a perforate artery of the proximal posterior cerebral artery (small-vessel disease). Secondly, both sensory and cerebellar ataxia are present, leading us to hypothesize that not only was the superior cerebellar peduncle affected, but the medial lemniscus also. Claude’s syndrome has not been given a definitive clinicopathological description in the neuroophthalmologic textbooks nor in other medical literature, and certain discrepancies between one description and another exist [4]. Given that the medial lemniscal tract is close to the red nucleus and the Fig. 1. Axial (right) and coronal (left) T2- weighted images showing an isolated left paramedian tegmento-basal area of hyperintensity in the rostral mesencephalon due to ischemic infarction. Fig. 2. Schematic representation of the different anatomic structures in the mesencephalon at the level of the superior colliculus. ML = Medial lemniscus; RN = red nucleus; SN = substantia nigra; A = aqueduct; CSC = crossed superior cerebellar tract; TNF = third nerve fascicle; TNN = third nerve nucleus. Short Reports 55 superior cerebellar peduncle in midbrain (fig. 2) it is not unreasonable to speculate that this tract may, at least in some cases, be involved in Claude’s syndrome, and that the disturbance of deep sensation contributes to ataxia in these patients. Acknowledgments The authors would like to express their thanks to Mr. Andrew Hughes for his valuable help with the translation of the manuscript. References 1 Claude H: Syndrome pédonculaire de la région du noyau rouge. Rev Neurol (Paris) 1912;1:311–313. 2 Claude H: Ramollissement du noyau rouge. Rev Neurol (Paris) 1912;2: 49–51. 3 Bogousslavsky J, Regli F: Intra-axial involvement of the common oculomotor nerve in mesencephalic infarctions. Rev Neurol (Paris) 1984;140:263– 270. 4 Liu GT, Crenner CW, Logigian EL, Charness ME, Samuels MA: Midbrain syndromes of Benedikt, Claude, and Nothnagel: Setting the record straight. Neurology 1992;42:1820–1822. Dr. Tomás Segura Servicio de Neurologı́a, Hospital General de Albacete C/Hermanos Falcó, s/n, E–02006 Albacete (Spain) Tel. +34 967 597100, Fax +34 967 243952, E-Mail tseguram@nexo.es 56 Short Reports Copyright: S. Karger AG, Basel 2001. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.