Case Report /Olgu Sunumu 197

Midbrain Infarction Presenting with Weber’s Syndrome and Central Facial Palsy: A Case Report Orta Beyin ‹nfarkt›na Ba¤l› Geliflen Weber Sendromu ve Santral Fasyal Parezi: Olgu Sunumu

Demet ‹LHAN ALGIN, Figen TAfiER*, Sayime AYDIN**, Elif AKSAKALLI*** Dumlup›nar University Faculty of Medicine, Department of , Kütahya *Dumlup›nar University Faculty of Medicine, Department of Anatomy, Kütahya **Dumlup›nar University Faculty of Medicine, Department of Ophthalmology, Kütahya ***Dumlup›nar University Faculty of Medicine, Department of Physical therapy and Rehabilitation, Kütahya, Turkey

ABSTRACT ÖZET Weber's syndrome is a distinctive brainstem disease characterized by ipsilateral Weber sendromu ipsilateral 3. sinir parezisi ve kontrlateral hemipleji ile karak- 3rd palsy with contralateral hemiplegia and is due to an intrinsic or terize özellikli bir beyinsap› hastal›¤›d›r ve ventral orta beyindeki intrinsik veya extrinsic lesion in the ventral midbrain. To date, there is limited literature ekstrinsik lezyona ba¤l› olarak geliflir. Bugüne kadar, santral fasyal parezi ile concerning Weber's syndrome associated with central facial palsy, but none iliflkili Weber sendromu konusunda s›n›rl› say›da literatür mevcuttur, ancak hiç- was demonstrated with comprehensive explanation. We report a rare case biri kapsaml› bir aç›klama ile sunulmam›flt›r. Mesensefalonun ventromedial presented with Weber’s syndrome and central facial palsy caused by infarction krural bölgesinin infarkt›na ba¤l› geliflen Weber sendromu ve santral fasyal pa- of ventromedial crural region of the mesencephalon. rezi ile baflvuran nadir bir olgudan bahsedilmektedir. The patient was a 68-year-old woman who developed central type facial palsy on Olgu, sa¤ santral fasyal parezi, sol 3. kranial sinir parezisi, sa¤ hemiparezi, sa¤ right side, and complete left 3rd nerve palsy, right and paresthesia üst ve alt ekstremitede derin duyu bozuklu¤u ile birlikte parestezi geliflen 68 ya- with deep sensory disturbance of right upper and lower extremities. fl›nda bir kad›n hasta idi. A T2 weighted cranial MRI showed an acute infarct in the left ventromedial T2 a¤›rl›kl› kranial MRG’de mezensefalonun sol ventromedial krural bölgesinde crural region of the mesencephalon and this lesion was presumed to involve both akut infarkt görüldü ve bu lezyonun kortikospinal ve kortikobulbar traktuslar› bir- the corticospinal and corticobulbar tracts. This report demonstrates an likte etkiledi¤i düflünüldü. Bu sunumda orta beyinin üst k›sm›ndaki infarkta ba¤l› extremely rare case of crossed hemiplegia with oculomotor and okulomotor ve fasyal parezi ile beraber çapraz hemipleji geliflen nadir bir olgu palsy due to an infarct in the upper part of the midbrain as documented by the MRG bulgular›yla beraber tart›fl›lm›flt›r. Hastan›n alt› ay sonra tamamen iyileflmifl MRI scan. The other interesting feature to note in our report is that the patient olmas› da ayr›ca belirtilmesi gereken ilginç bir özelliktir ki bu da hastalar›n bir completely recovered six months later. This indicates that some of these patients k›sm›n›n iyi bir prognoza sahip olabilece¤ini göstermektedir. (Nöropsikiyatri may have a good prognosis. (Archives of Neuropsychiatry 2009; 46: 197-9) Arflivi 2009; 46: 197-9) Key words: Weber’s syndrome; facial nerve; ; corticobulbar Anahtar kelimeler: Weber sendromu, fasyal sinir, okulomotor sinir, kortikobul- tract; midbrain infarct ber traktus, mezensefalon infarkt›

Case Our patient noticed weakness of the right arm and leg and diplopia on waking in the morning. She had been diabetic and A 68-year-old woman was admitted to the Dumlupinar hypertensive for the past 20 years. Her medications included University Hospital because of central type facial palsy on right gliclazide, metformin and insulin. On admission she was alert side, right hemiparesis, and paresthesia with deep sensory disturbance of right upper and lower extremities (Figure 1A). She and had a blood pressure of 155/75 mm/Hg with no arrhythmia. had ptosis, midriasis and lateral-inferior deviation of the left eye Neurological examination revealed a conscious individual with due to (Figure 1B). normal higher cortical functions.

Address for Correspondence/Yaz›flma Adresi: Dr. Demet ‹lhan Alg›n, Dumlup›nar University Faculty of Medicine, Department of Neurology, Kütahya, Turkey E-mail: [email protected] Received/Gelifl tarihi: 20.05.2009 Accepted/Kabul tarihi: 22.09.2009 © Archives of Neuropsychiatry, Published by Galenos Publishing. All rights reserved. / © Nöropsikiyatri Arflivi Dergisi, Galenos Yay›nc›l›k taraf›ndan bas›lm›flt›r. Her hakk› sakl›d›r. Alg›n et al. Archives of Neuropsychiatry 2009; 46: 197-9 198 Midbrain Infarction Presenting with Weber’s Syndrome and Central Facial Palsy: A Case Report Nöropsikiyatri Arflivi 2009; 46: 197-9

Cranial examination revealed complete left 3rd nerve palsy and Discussion normal ocular fundi. The strength of the frontalis and orbicularis oculi muscles was well preserved, but she had a right lower facial Weber's syndrome was described by the German physician weakness with mild flattening of the right nasolabial fold and Hermann Weber in 1863 (1). The clinical findings of classic paralytic dysarthria. Motor system showed right-sided spastic Weber's syndrome include an ipsilateral oculomotor nerve palsy weakness with grade 4/5 power. Deep tendon reflexes were brisk and a contralateral limb weakness due to a lesion in the on the right side with upgoing plantar response. Initial computerized midbrain (crus cerebri). tomography (CT) scan was normal. Cranial magnetic resonance Most of the muscles of the eye innervates by the oculomotor imaging (MRI) performed 10 days after the onset showed an nerve. The motor nucleus of this nerve is located at the upper infarction in the left ventromedial part of the upper mesencephalon mesencephalic level of brainstem. Nerve nascicles run forward and old multiple chronic lacunar infarctions in the deep white and laterally through the red nuclei and get closer at the matter of the cerebral hemispheres (Figure 1C). inter-peduncular fossa. So nuclei and fascicles of the oculomotor Over the next week her hemiparesis resolved completely and nerve are expanding a relatively wide area within midbrain. her diplopia was getting better. Two weeks later, she was Therefore midbrain lesions generally lead to partial third nerve discharged with normal neurological findings. palsy. It enters the orbit through the superior orbital fissure after come out from the midbrain and branching into upper and lower fibers. While the levator palpebrae superioris and superior rectus muscles were innervated by the upper branch, the medial rectus, the inferior rectus, and the inferior oblique muscles were innervated by the lower branch (2-4).

Figure 1A. Central type facial palsy on right side Figure 1C. T2-weighted cranial magnetic resonance imaging shows an infarction at the left ventromedial part of the upper mesencephalon

Figure 1B. Extraocular movements in 9 cardinal positions Archives of Neuropsychiatry 2009; 46: 197-9 Alg›n et al. Nöropsikiyatri Arflivi 2009; 46: 197-9 Midbrain Infarction Presenting with Weber’s Syndrome and Central Facial Palsy: A Case Report 199

The preganglionic parasympathetic fibers of the 3rd nerve oculomotor nerve, was demonstrated in this case (3). which are transported by the nerve to the inferior oblique Terao et al. reported two patients with contralateral central muscle arrive to the ciliary ganglion and from here the facial and hemiparesis of the limbs, resulted from post-ganglionic parasympathetic fibers emerge. The ciliary unilateral ventromedial medullary infarction (14). According to muscle and the muscles of the iris are innervated by these these cases, they described the course of the facial corticobulbar post-ganglionic parasympathetic fibers. The nerve fibers to levator palpebrae muscle and the pupilloconstrictor fibers for tract as consisting of looping fibers that descend at least to the the muscles of the iris are located in a superficial and dorsal medullary level and then decussate. In another study with larger position on the nerve relaying in the ciliary ganglion. Before group (70 patients), they attempted to further clarify the course external ophthalmoplegia develops, a fixed dilated pupil is often and the distribution of the facial (10). But the the first sign of 3rd nerve (oculomotor) compression, and ptosis authors could not coincide with third nerve palsy or Weber’s the second, upon this anatomical characteristic (5). syndrome within these central facial palsy patients. The clinical manifestation of the patients with isolated Finally, this report demonstrates an extremely rare case of mesencephalic infarct was shown up nuclear or fascicular crossed hemiplegia with oculomotor and facial nerve palsy due oculomotor nerve palsy and contralateral motor deficits (6,7). to an infarct in the upper part of the midbrain as documented by In patients with isolated mesencephalic infarct, the clinical picture was dominated by nuclear or fascicular third-nerve palsy the MRI scan. The other interesting feature to note in our report and contralateral motor deficits (6,8). is that the patient completely recovered six month later. This On the other hand the corticobulbar tract (CBT) is commonly indicates that some of these patients may have a good prognosis. used to describe the pathway taken by motor fibers innervating the cranial nerve nuclei, especially trigeminal, facial, hypoglos- References sal motor nuclei, nucleus ambiguus, and spinal accessory nucleus. The oculomotor, trochlear and abducens 1. Silverman IE, Liu GT, Volpe NJ et al. The Crossed Paralyses. The nuclei receive no input from the CBT. The cell bodies of primary Original Brain-Stem Syndromes of Millard-Gubler, Foville, Weber and motor neurons are located in (9). Raymond-Cestan. Arch Neurol 1995; 52:635-8. (Abstract) / (PDF) The motor nucleus of the facial nerve, which supplies the 2. Liu CT, Cremer CW, Logigian EL et al. Midbrain syndrome of Benedict, muscles of the facial expression, is located at the lower pontine Claude, Nothnagel-setting record straight. Neurology 1992 ;42:1820-2. level, dorsolaterally in the caudal (4). The CBT fibers that 3. Umasankar U, Huwez FU. A patient with reversible pupil-sparing connect the motor cortex with the facial nucleus provide Weber’s syndrome . Neurol India 2003; 51:388-9. (Abstract) / (Full Text) / strongly unilateral innervation to the contralateral lower facial (PDF) muscles and bilateral innervation to the upper . 4. Hendelman WJ. Atlas of Functional Neuroanatomy. 2nd edition CRC The facial CBT fibers descend at the ventromedial region of the Pres Taylor&Francis Group. 2006; pp. 126. crus cerebri, near the corticospinal tract (10). 5. Sunderland S. Neurovascular relations and anomalies at the base of The clinical manifestations of midbrain infarcts mostly show the brain. J Neurol Neurosurg Psychiatry 1948; 11:243. (Full Text) / (PDF) the location of the lesion directly, but these features could not 6. Kumral E, Bayulkem G, Akyol A, et al. Mesencephalic and associated posterior circulation infarcts. . 2002; 33:2224-31. (Abstract) / always match with classical syndromes which described (Full Text) / (PDF) previously in the literature, just like our case that we present in 7. Cormier PJ, Long ER, Russell EJ. MR imaging of posterior fossa this report (11). infarctions: vascular territories and clinical correlates. Radiographics Posterior cerebral artery branch disease may be caused by 1992; 12:1079-96. (Abstract) / (PDF) occlusion of the arterial branch with atherothrombotic plaque 8. Johnson MH, Christman CW. Posterior circulation infarction: anatomy, (4). In these cases, hypertension is a major risk factor, as seen in pathophysiology, and clinical correlation. Semin Ultrasound CT MR. our patient (10). 1995; 16:237-52. (Abstract) Kumral et al. presented the topographic and clinical 9. Nolte J. The Human Brain: An Introduction to its Functional Anatomy, distribution of the acute posterior circulation infarcts involving Edition 5th, Elsevier Health Science; pp. 461. mesencephalon. They described four patients with Weber’s 10. Terao S, Miura N, Takeda A et al. Course and distribution of facial syndrome and central facial palsy due to isolated midbrain corticobulbar tract fibres in the lower brain stem. J Neurol Neurosurg infarct around ventromedial crural region within 41 patients with Psychiatry 2000; 69:262-5. (Abstract) / (PDF) mesencephalic infarction. These were the only cases in the 11. Kim JS, Kang JK, Lee SA et al. Isolated or predominant ocular motor literature identical to our patient (6). nevre palsy as a manifestation of brain stem stroke. Stroke. 1993; Some minor infarctions at the midbrain which resulted in 24:581-6. (Abstract) / (Full Text) / (PDF) 12. Kwon JH, Kwon SU, Ahn HS et al. Isolated Superior Rectus Palsy Due localized paralysis like weakness of a single extraocular muscle to Contralateral Midbrain Infarction. Arch Neurol 2003;60:1633-5. (12), isolated contralateral superior rectus palsy (13) have been (Abstract) / (Full Text) / (PDF) demonstrated previously. Some interesting cases, such as 13. Terao S, Takatsu S, Izumi M et al. Central facial weakness due to presence of left oculomotor nerve palsy with normal pupil and medial medullary infarction: the course of facial corticobulbar fibres. right hemiparesis, were described. An ischemic lesion of the J Neurol Neurosurg Psychiatry 1997; 63:391-3. (Abstract) / (Full Text) / lower midbrain, which corresponds to the motor nucleus of the (PDF)