CLAUDE SYNDROME: A CASE REPORT

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I.K. Chukwuocha, O. Oguntiloye, A. Fagbemi, O. Olalusi, V. Onyenokwe

1. Department of Neurology, University College Hospital, Ibadan, Oyo State, Nigeria.Dr. I.K. Chukwuocha.

Abstract

Introduction: Detailed description of infarction of the midbrain is sparse likely due to the complex arterial supply of this region of the brain. Among the ventral midbrain syndromes reported is Claude syndrome. This syndrome caused majorly by a vascular insult to the ventromedial midbrain, characteristically presents with ipsilateral third cranial nerve palsy and contralateral hemiataxia. It is a rare syndrome and only a few cases have been reported since 1912 when it was first described by Henri Claude.

Case presentation: A 45-year-old male, who developed sudden onset dysarthria, right third cranial nerve palsy and left sided ataxia. An infarct in the right ventromedial midbrain was revealed on magnetic resonance imaging of the
brain.

Conclusion: We describe a case report of a middle-aged man with minimal vascular risk factors (ASCVD = 1.3%) for stroke, who presented with features suggestive of Claude syndrome.

Keywords: Claude syndrome, Midbrain infarction, ASCVD

Introduction

The midbrain also known as the mesencephalon is the division of the brainstem linking the pons and cerebellum with the forebrain and is divided into a ventral part, the tegmentum, and a dorsal part, the tectal or quadrigeminal.¹ Midbrain infarction though rare can be caused by the obstruction of its blood supply and is responsible for about 0.6% of the total patients admitted. Most case are described in individuals with high cardiovascular risk (ASCVD >7.5%)with ischemic stroke.² The midbrain receives its blood supply from the paramedian mesencephalic branches (basilar), peduncular perforating branches (posterior cerebral artery) as well as from the superior cerebellar artery and the choroidal arteries..³ Studies have shown that infarction of the midbrain is 10-fold more likely to be accompanied by ischemia of neighbouring structures⁴ The constellation of signs and symptoms caused by the lesion of these structures give rise to syndromes of both ventral and dorsal midbrain origins. Among the ventral midbrain syndromes is Claude syndrome. This syndrome comes in various forms which include affectation of the fibers from CN III, the rubrodentate fibers, corticospinal tract fibers and corticobulbar fibers.⁵ The manifestation of this syndrome depends on the extent of the lesion to the structures involved and can present as ipsilateral CN III palsy, contralateral ataxia with or without contralateral hemiplegia of lower facial muscles, tongue, shoulder, upper and lower limb along. The blood supply involved is from the posterior cerebral artery.⁶ Detailed description of this syndrome is sparse, with few studies on the imaging findings of the site of lesion and the whole spectrum of clinical manifestation involved. Other ventral midbrain syndromes described are Benedikt, Weber, Nothnagel syndrome and Parinaud syndrome which is a dorsal midbrain syndrome

Benedikt syndrome, also called Paramedian midbrain syndrome is caused by an insult in the tegmentum of the midbrain and cerebellum. It is characterized by the presence of oculomotor nerve palsy and cerebellar ataxia including tremor and involuntary chore athetotic movements.

Weber syndrome presents with ipsilateral oculomotor nerve palsy with contralateral hemiparesis. It occurs due to the involvement of oculomotor fascicles in the interpeduncular cisterns and cerebral peduncle

Nothnagel syndrome is a rare midbrain syndrome that involves the tectum of the midbrain and superior cerebellar peduncles. Classically, the syndrome involves the oculomotor nerve fascicles and superior cerebellar peduncle, leading to ipsilateral CN III palsy and limb ataxia respectively⁷

Parinaud syndrome a dorsal midbrain syndrome is classically described by the triad of impaired upward gaze, convergence retraction nystagmus, and pupillary hyporeflexia.⁸

We herein describe a case of a patient with low ASCVD score who presented with clinical features and neuroimaging findings suggestive of Claude syndrome.