A 59-year-old man with hypertension, hyperlipidemia, and type 2 diabetes presented with sudden-onset, bilateral ptosis, ataxia, and confusion. Examination showed bilateral ophthalmoparesis sparing minimal adduction and abduction, poorly reactive and dilated pupils, and global ataxia (video). The oculocephalic reflex revealed normal bilateral abduction. Bell phenomenon and convergence were absent. MRI brain showed bilateral paramedian mesencephalic ischemic stroke (figure). Here in a bilateral variant, clinical presentation and imaging closely follow the description of Henri Claude's first case,1 where pathologic examination revealed a paramedian midbrain stroke involving the superior cerebellar peduncle, red nucleus, and medial longitudinal fasciculus (MLF).1
Video 1
Oculomotor and coordination findings in a patient with bilateral mesencephalic stroke. Bilateral ptosis and reactive blepharospasm are noted when eyes are opened by the examiner. Severely impaired abduction bilaterally and almost absent adduction are present. Ataxic gait is present. Rubral tremor is more pronounced on the left (right not shown), exacerbated by activation.
Figure MRI of the brain
Bilateral paramedian mesencephalic stroke, hyperintense on diffusion-weighted imaging (A.a and B.a, with magnification in A.b and B.b) and hypointense in corresponding areas in the apparent diffusion-coefficient maps (C, D). Lesions involve the third nerves, Edinger-Westphal, and red nuclei, superior cerebellar peduncle, and the reticular activating system, sparing the crura cerebri and tectum.
Third nuclei and MLF lesions likely cause ptosis and vertical and adduction deficits, while injury to fronto-pontine horizontal gaze pathways passing through the midbrain may explain the impaired abduction.2 Eponyms of brainstem syndromes are rarely used, because of overlap among the historical descriptions (table).
Table Historical overview of brainstem syndromes
References
1.
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.
Deleu D, Imam YZ, Mesraoua B, Salem KY. Vertical one-and-a-half syndrome with contralesional pseudo-abducens palsy in a patient with thalamomesencephalic stroke. J Neurol Sci 2012;312:180–183.
The Mystery Case series was initiated by the Neurology® Resident & Fellow Section to develop the clinical reasoning skills of trainees. Residency programs, medical student preceptors, and individuals were invited to use this Mystery Case as an educational tool. Responses were solicited through a group email sent to the American Academy of Neurology Consortium of Neurology Residents and Fellows and through social media.
Forty-six percent of respondents correctly localized the lesion to the paramedian mesencephalic region. Similarly, 48% of respondents correctly identified the nuclei involved to cause the particular constellation of symptoms described. Only 8% of respondents correctly identified large artery disease as the most common cause of mesencephalic stroke.1 Four respondents, including 1 medical student, answered all 3 questions correctly.
This mystery case illustrates Claude syndrome, which is a brainstem stroke syndrome characterized by ptosis, ophthalmoplegia, dysdiadochokinesis, and ataxia first described secondary to damage to the third nerve fascicle, red nucleus, and superior cerebellar peduncle. As the authors point out, historical eponyms for brainstem strokes can be confusing due to overlapping clinical presentations. The similarities among syndromes arise from the close anatomical location of these nuclei within the brainstem. Thus, lesion localization within the brainstem requires interpretation of both clinical symptoms and imaging findings combined with detailed knowledge of cranial nerve pathways.
Ariel M. Lyons-Warren, MD, PhD
Baylor College of Medicine, Texas Children's Hospital, Houston
Reference
1.
Kumral E, Bayulkem G, Akyol A, Yunten N, Sirin H, Sagduyu A. Mesencephalic and associated posterior circulation infarcts. Stroke 2002;33:2224–2231.
From Neurology and Neurosurgery, Division of Stroke and Vascular Neurology (J.L.S.), Department of Neurology (J.W., H.A.), and Department of Vascular Neurology (R.N.), Yale University School of Medicine, New Haven, CT.
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Author Contributions
J. Witsch: clinical care of the patient, study concept, making of figure, editing of video, writing of the manuscript. R. Narula: clinical care of the patient, making of video, revision of the manuscript. H. Amin: clinical care of the patient, revision of the manuscript. J. Schindler: clinical care of the patient, study concept, acquisition of data, revision of the manuscript.
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Cited By
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The Effect of Elastic Eyelid Band Application on Bilateral Severe Ptosis in a Patient With Bilateral Incomplete Claude’s Syndrome: A Case Report, Brain & Neurorehabilitation, 16, 3, (2023).https://doi.org/10.12786/bn.2023.16.e29
Claude Syndrome in Childhood Associated with Probable Neuro-Behcet Disease, Neuropediatrics, 54, 01, (082-087), (2022).https://doi.org/10.1055/s-0042-1759794
