CASE REPORT

Classic Raymond Syndrome Majid Khan1, Sadaf Naveed2, Iqbal Haider1, Mohammad Humayun1 and Abidullah Khan1

ABSTRACT Classic Raymond syndrome presents with palsy on the ipsilateral side with contralateral and facial nerve paralysis. A 60-year gentleman presented with deviation of left angle of mouth and right sided weakness. Examination showed that he had left sided abducens nerve palsy, with contralateral central facial paralysis and paresis. MRI of confirmed left pontine infarct. These findings were consistent with classic Raymond syndrome. Till now, only a few cases have been reported worldwide, this being the first case reported in South Asia. This case confirms that classic Raymond syndrome is different from the common type of Raymond syndrome in terms of sparing of coritcofacial fibers in the latter type.

Key Words: Abducens nerve. Contralateral facial paralysis. Pontine infarct.

INTRODUCTION Raymond syndrome was initially described in 1896 by Dr. Raymond in a 39-year lady who presented with an abducens nerve palsy and contralateral facial paralysis and hemiparesis.1 This case compelled other scientists to study the pathophysiology of this disease process. This unusual case gave realisation that the facial nerve decussation occurs close to abducens nerve in the . Kupers provided pathological evidence and validated the above concept about the facial nerve decussation at Figure 1: T2 weighted axial section Figure 2: T2 weighted coronal the level of facial nerve nuclei in the pons.2 This is a rare of brain showing hyperintense in left Section of area in left side of pons syndrome.3-6 side of pons. showing area in left side of pons. The present case is the first case report from the region anticoagulants and his symptoms were improved after 4 and the fourth one over all. days of treatment.

CASE REPORT DISCUSSION A 60-year known hypertensive gentleman presented In 1896, Dr. Raymond first described a syndrome with with chief complaints of diplopia, right sided deviation of left abducens impairment, right central facial paresis, the angle of mouth, and right sided weakness in both and right hemiparesis, which was given his name.1 upper and lower limbs. On examination, he had left Classic Raymond syndrome is a pontine syndrome abducens nerve paralysis with contralateral facial consisting of ipsilateral abducens nerve palsy, paralysis ( type), contralateral contralateral facial paralysis, and contralateral hemi- hemiplegia with hyper-reflexia and up-going plantar paresis. But clinicians commonly encounter Raymond reflex and sensations were intact bilaterally. Pupils were syndrome without facial involvement.4-6 The only of equal size and normally reacting to light. There were differentiating point between classic and the common no cerebellar signs on the right side and left side could Raymond syndrome is the location of the lesion.3 not be appreciated due to weakness. These findings Classic type involves ipsilateral abducens nerve were consistent with classical Raymond syndrome. palsy, contralateral corticofacial and corticospinal tracts; MRI brain was performed on the second day of while in the common type, corticofacial tracts are presentation, which confirmed ischemic infarct in the left spared.3 This syndrome should be differentiated from mid pons (Figures 1 and 2). The patient was put on Millar Gubbler syndrome, which involves ipsilateral abducens and facial nerve paralysis and contralateral Department of Medicine1 / Radiology2, Khyber Teaching hemiplegia.7,8 Hospital, Peshawar. Classic Raymond syndrome is a very rare condition. A Correspondence: Dr. Majid Khan, Postgraduate Trainee, 55-year woman was reported by Sheth and colleagues Department of Medicine, Khyber Teaching Hospital, Peshawar. from USA in 1996.9 Ogawa et al. reported two cases of E-mail: [email protected] classic Raymond syndrome in 2010 from Japan.6 Received: June 18, 2016; Accepted: January 24, 2017. Another case was also reported from USA in 2012.10

Journal of the College of Physicians and Surgeons Pakistan 2017, Vol. 27 (3): 185-186 185 Majid Khan, Sadaf Naveed, Iqbal Haider, Mohammad Humayun and Abidullah Khan

To the best of authors’ knowledge, this is the first ever the evolution of clinical neuroanatomy. CC Thomas Springfield case reported from South Asia. 1936; 139-41. 5. Ogawa K, Tougou M, Oishi M, Kamei S, Mizutani T. A case of This patient suffered from this syndrome by a hyper- pontine infarction causing alternating hemiplegia with tension induced infarct of the (L) midpons as confirmed ipsilateral abducens nerve palsy and contralateral supranuclear on MRI. The location resulted in the classic clinical facial nerve palsy. Clin Neurol 2008; 48:135-8. manifestations, which improved on anti-hyper-tensive 6. Ogawa K, Suzuki Y, Kamei S. Two patients with abducens and supportive treatment. nerve palsy and crossed hemiplegia (Raymond syndrome). Acta Neurologica Belgica 2010; 110:270-1. REFERENCES 7. Marx JJ, Thomke F. Classical crossed brain stem syndromes: myth or reality?. J Neurol 2009; 6:898-903. 1. Raymond F. Concerning a special type of alternating hemiplegia. In Lecons Sur Les Maladies Nerve Uses. 1894; 365-83. 8. Donaldson D, Rosenberg NL. Infarction of abducens nerve fascicle as cause of isolated sixth nerve palsy related to 2. Kupers HGJM. Corticobulbar onnections to the pons and the hypertension. Neurology 1988; 38:1654. lower brain stem in man: An anatomical study. Brain 1958; 81:364-88. 9. Sheth RD, Riggs JE, Ortiz OA. Raymond syndrome: A validation. Eur Neurol 1996; 36:173-217. 3. Zaorsky NG, Luo JJ. A case of classic Raymond syndrome. 10. Satake M, Kira JI, Yamada T, Kobayashi T. Raymond syndrome Case Rep Neurol Med 2012. 2012:58312. (alternating abducent hemiplegia) caused by a small haematoma 4. Wolfe JK. Raymond's syndrome in the classical brain stem at the medial pontomedullary junction. J Neurol Neurosurg syndromes. Translations of the original papers with notes on Psychiatry 1995; 58:261.

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