Delavelle, J.

Landis, T.

Lüscher, C.

Mégevand, P.

Pilly, B.

Safran, A.

Tajouri, N.

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LETTER TO THE EDITORSSixth cranial nerve palsy and contralateral hemiparesis(Raymond’s syndrome) sparing the faceP. Me´gevand Æ B. Pilly Æ J. Delavelle Æ N. Tajouri ÆA. B. Safran Æ T. Landis Æ C. Lu¨scherReceived: 4 August 2008 / Revised: 6 January 2009 / Accepted: 28 January 2009 / Published online: 1 March 2009 Springer-Verlag 2009The combination of VIth cranial (abducens) nerve palsyand contralateral hemiplegia is termed Raymond’s syn-drome [1–3]. Whether this rarely reported syndrome [4]necessarily includes central facial paralysis is debated [5].Case reportA 74-year old man presented with horizontal diplopia ofsudden onset, worsened by directing gaze to the left. Hispast medical history included arterial hypertension treatedwith an angiotensin conversion enzyme inhibitor, a 60-pack-per-year smoking history and moderate alcoholconsumption.Neurological examination showed moderate left VIthnerve paresis and slight paresis of the right arm and leg.Both spontaneous and voluntary facial movements werenormal. Deep tendon reflexes were normal and symmetric.Cutaneous plantar response was flexor on both sides. Therewas no ataxia or sensory deficit. Gait was unremarkable.Laboratory examinations were normal except forslightly raised liver enzymes. Magnetic resonance imaging,performed 2 days after the onset of symptoms, showed alesion in the left caudal ventral paramedian pons (Fig. 1a,b). Magnetic resonance angiography as well as extra- andintracranial ultrasound with Doppler examination did notreveal any hemodynamically significant arterial stenosis.Heart rhythm remained regular during 24-h monitoring inthe stroke unit.The patient was not handicapped by the slight hemipa-resis; diplopia resolved partially over several days.Acetylsalicylic acid was introduced.DiscussionThe French neurologist Fulgence Raymond (born 1844,died 1910) described the clinical picture that bears hisname in 1895 [1–3]. Landry previously reported a similarpatient with a hemisensory deficit in addition to hemiplegia[2, 6]. In both Raymond’s and Landry’s patients, thehemiplegia included central facial palsy. The lesion(probable syphilis in Raymond’s patient, stroke in morerecent reports) lies in the medial ventral caudal pons. Othereponymous syndromes of the caudal pons include Millard-Gubler’s syndrome (peripheral facial palsy plus contralat-eral hemiplegia) and Foville’s syndrome (peripheral facialpalsy and horizontal gaze palsy plus contralateral hemi-plegia), both caused by lesions extending more laterallyand dorsally [2, 3].A patient with VIth nerve paresis and contralateralhemiparesis sparing the face, caused by a lesion in theP. Me´gevand (&)  T. Landis  C. Lu¨scherDepartment of Neurology, Geneva University Hospitals andUniversity of Geneva, Rue Micheli-du-Crest 24,1211 Geneva 14, Switzerlande-mail: pierre.megevand@medecine.unige.chB. Pilly  N. Tajouri  A. B. SafranOphthalmology Clinics, Clinical Neuroscience Department,Geneva University Hospitals and University of Geneva,Rue Micheli-du-Crest 24, 1211 Geneva 14, SwitzerlandJ. DelavelleRadiology Department, Geneva University Hospitals andUniversity of Geneva, Rue Micheli-du-Crest 24,1211 Geneva 14, SwitzerlandP. Me´gevand  C. Lu¨scherBasic Neuroscience Department, Faculty of Medicine,University of Geneva, Rue Michel-Servet 1,1211 Geneva 4, Switzerland123J Neurol (2009) 256:1017–1018DOI 10.1007/s00415-009-5041-6pontomedullary junction, was recently diagnosed withRaymond’s syndrome [7]. Others suggested using theeponym only when central facial palsy is present [5]. Thiscontroversy likely reflects uncertainty about the anatomicalcourse of corticofacial fibers in the brainstem. Corticofacialfibers can take one of several routes through the pons,including an ‘‘aberrant bundle’’ located close to the mediallemniscus in the pontine tegmentum [8]. This anatomicalvariability might reflect the phylogenetically recentappearance of the corticospinal and corticobulbar tracts [8].Corticofacial fibers traveling dorsally in the aberrant bun-dle would not be involved by a ventral lesion of the caudalpons, producing Raymond’s syndrome sparing the face(Fig. 1c, d). Alternatively, a very small pontine lesion mayonly partially damage the corticospinal and corticobulbartracts. Regardless of the presence or absence of centralfacial palsy, the combination of VIth nerve palsy andcontralateral hemiplegia should direct the clinician’sattention towards the medial ventral caudal pons.Acknowledgements P.M. is supported by grant #323600-111505from the Swiss National Science Foundation.References1. Raymond F (1896) In: Ricklin E, Souques A (eds) Lec¸ons sur lesmaladies du syste`me nerveux, recueillies et publie´es par MM.Doin, Paris2. Wolf JK (1971) The classical brain stem syndromes. Translationsof the original papers with notes on the evolution of clinicalneuroanatomy. Thomas, Springfield3. Silverman IE, Liu GT, Volpe NJ, Galetta SL (1995) The crossedparalyses. The original brain-stem syndromes of Millard-Gubler,Foville, Weber, and Raymond-Cestan. Arch Neurol 52:635–6384. Hommel M, Besson G (1993) Clinical features of lacunar andsmall deep infarcts at specific anatomical sites. Adv Neurol62:161–1795. Sheth RD, Riggs JE, Ortiz OA (1996) Raymond syndrome: avalidation. Eur Neurol 36:173–1746. Landry M (1858) Bull Soc Anat Paris 33:406–4107. Satake M, Kira J, Yamada T, Kobayashi T (1995) Raymondsyndrome (alternating abducent hemiplegia) caused by a smallhaematoma at the medial pontomedullary junction. J NeurolNeurosurg Psychiatr 58:2618. Urban PP, Wicht S, Vucorevic G, Fitzek S, Marx J, Tho¨mke F,Mika-Gru¨ttner A, Fitzek C, Stoeter P, Hopf HC (2001) The courseof corticofacial projections in the human brainstem. Brain124:1866–1876Fig. 1 Axial diffusion-weighted (a) and coronalFLAIR brainstem MRI (b)showing an ischemic lesion inthe left caudal ventralparamedian pons (arrows).When corticofacial fibers(dashed arrow) travel with thecorticospinal tract in the ventralpons, a lesion in the medialcaudal ventral pons (circle)damages the corticospinal,corticofacial and VIth cranialnerve fibers together (c). Ifcorticofacial fibers travel in anaberrant bundle in the pontinetegmentum, the medial caudalventral pontine lesion damagesonly corticospinal fibers and theVIth cranial nerve, sparing thecorticofacial projection (d).CST: corticospinal tract; VInucl: abducens nucleus; VIInucl: facial motor nucleus1018 J Neurol (2009) 256:1017–1018123

Sixth cranial nerve palsy and contralateral hemiparesis (Raymond's syndrome) sparing the face

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Sixth cranial nerve palsy and contralateral hemiparesis (Raymond's syndrome) sparing the face

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