| Identifier |
Claudes_Syndrome |
| Title |
Claudes Syndrome |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital, Boston, Massachusetts |
| Subject |
Ptosis; Fascicular Third Nerve Palsy; Fixed Dilated Pupil; Contralateral Limb Ataxia; Claude Syndrome; Midbrain Infarct; Oculomotor Nerve; Unilateral Third Nerve Palsy; Subnuclear; Fascicular Oculomotor (Third) Nerve Palsy; Third Nerve Dysfunction |
| History |
The patient is a 76 year old woman who woke one morning unable to see out of her right eye because of ptosis. She came to the Massachusetts General Hospital emergency room and was admitted. Neurological Examination: Right third nerve palsy involving the pupil Ataxia of the left hand on finger-nose test Diagnosis: Claude's Syndrome - a lesion of the third nerve fascicle in the midbrain with involvement of the superior cerebellar peduncle. Fascicular third nerve palsies are rare. Lesions of the third nerve fascicle can present as an isolated complete or incomplete third nerve palsy, in association with other neurologic signs due to involvement of adjacent midbrain structures. Four syndromes are recognized, and verified pathologically. The commonest cause is ischemia from embolic or thrombotic occlusion of perforating branches off the basilar artery. The Midbrain Syndromes are: 1. Weber's syndrome: ipsilateral third nerve palsy, plus contralateral hemiparesis including the lower face and tongue due to involvement of the cerebral peduncle. 2. Benedikt's syndrome: Ipsilateral third nerve palsy, plus contralateral tremor due to involvement of the red nucleus. 3. Nothnagel's syndrome: Ipsilateral third nerve palsy, plus ipsilateral cerebellar ataxia due to involvement of the superior cerebellar peduncle. 4. Claude's syndrome: Ipsilateral third nerve palsy (often partial) plus contralateral ataxia, asynergy, and dysdiadochokinesis due to involvement of the red nucleus and superior cerebellar peduncle. |
| Anatomy |
Claude's syndrome is a well-known midbrain syndrome characterized by ipsilateral oculomotor nerve palsy and contralateral cerebellar ataxia. This syndrome is very rare; only a few cases have been reported since Claude's original description in 1912. The condition occurs by simultaneous involvement of the cerebellar efferent fibers to the thalamus and the oculomotor nerve fascicles. Claude's case had a midbrain infarction that involved the medial half of the red nucleus and the region of the decussation of the superior cerebellar peduncle. Although major neuro-ophthalmology textbooks have described that Claude's syndrome may be attributed to a lesion of the red nucleus, the exact localization of this syndrome is still speculative. Seo et al examined six patients with Claude's syndrome who clearly showed lesions on MRI. All patients had lesions in the midbrain below the red nucleus. The findings in their patients suggested that the lesion responsible for Claude's syndrome was the superior cerebellar peduncle, below the red nucleus. To validate this finding, Seo also reviewed the literature for previous individual case reports. The findings from the literature search strongly suggested that Claude's syndrome occurs because of a lesion of the superior cerebellar peduncle just below and medial to the red nucleus. Midbrain infarction and a partial oculomotor nerve palsy was common. |
| Pathology |
Ischemia is the commonest cause due to occlusion or perforating branches from the basilar artery or medial interpeduncular branches of the posterior cerebral artery. Other causes include hemorrhage, infiltration, inflammation, compression, trauma and demyelination. |
| Disease/Diagnosis |
Fascicular third nerve palsy; Contralateral limb ataxia; Midbrain infart; Claude's syndrome |
| Clinical |
This elderly lady with Claude's syndrome - a right ipsilateral fascicular third nerve palsy with contralateral limb ataxia had on examination of the right eye: • Ptosis • Exotropia (lateral deviation of the eye out) • Paresis of all muscles innervated by the third nerve • Dilated fixed pupil • Cranial nerves 4 and 6 normal • No facial palsy • Ataxia on finger-nose test with the left hand Small midbrain lesions may selectively involve the fascicles of the oculomotor nerve causing paresis of one or more of the extraocular muscles with no associated neurological deficits. Recognition of the pattern of involvement enables precise localization of third nerve palsies. |
| Presenting Symptom |
Ptosis |
| Ocular Movements |
Ptosis; Third Nerve Palsy; Fixed Dilated Pupil; Subnuclear |
| Neuroimaging |
Neuroimaging studies were not available in this patient. An MRI in another patient with Claude's syndrome shows an infarct in the midbrain. Courtesy Sarah Sheikh, M.D. |
| Etiology |
Midbrain Infarct |
| References |
1. Akdal G, Kutluk K, Men S, Yaka E. Benedikt and ‘plus-minus lid' syndromes arising from posterior cerebral artery branch occlusion. J Neurol Sci 2005;228:105-107. http://www.ncbi.nlm.nih.gov/pubmed/15607218 2. Bogousslavsky J, Maeder P, Regli F, Meuli R. Pure midbrain infarction: clinical syndromes, MRI, and etiologic patterns. Neurology 1994;44:2032-2040. http://www.ncbi.nlm.nih.gov/pubmed/7969955 3. Biller J, Shapiro R, Evans LS, Haag JR. Fine M. Oculomotor nuclear complex infarction. Clinical and radiological correlation. Arch Neurol 1984;41:985-987. http://www.ncbi.nlm.nih.gov/pubmed/6477233 4. Broadley SA, Taylor J, Waddy HM, Thompson PD. The clinical and MRI correlate of ischaemia in the ventromedial midbrain: Claude's syndrome. J Neurol 2001;248:1087-1089. http://www.ncbi.nlm.nih.gov/pubmed/12013587 5. Casto O, Johnson LN, Mamourian AC. Isolated inferior oblique paresis from brain-stem infarction. Perspective on oculomotor fascicular organization in the ventral midbrain tegmentum. Arch Neural 1990;47:235-237. http://www.ncbi.nlm.nih.gov/pubmed/2405823 6. Claude H, Loyez M. Ramollissement du noyau rouge. Rev Neurol (Paris) 1912;24:49-51. 7. Gauntt CD, Kashii S, Nagata I. Monocular elevation paresis caused by an oculomotor fascicular impairment. J Neuro-ophthalmol 1995;15:11-14. http://www.ncbi.nlm.nih.gov/pubmed/7780565 8. Gaymard B, Saudeau D, de Toffol B, Larmande P, Autret A. Two mesencephalic lacunar infarcts presenting as Claude's syndrome and pure motor hemiparesis. Eur Neurol 1991;31:152-155. http://www.ncbi.nlm.nih.gov/pubmed/2044629 9. Hriso E, Masdeu JC, Miller A. Monocular elevation weakness and ptosis: an oculomotor fascicular syndrome? J Clin Neuro-ophthalmol 1991;11:111-113. http://www.ncbi.nlm.nih.gov/pubmed/1832683 10. Iwatsubo T, Iwata M, Inoue K, Mannen T. Magnetic resonance imaging in a case of paramedian mesencephalic infarction. Rev Neurol (Paris) 1987;143:605-607. http://www.ncbi.nlm.nih.gov/pubmed/3671964 11. Ksiazek SM, Slamovitz TL, Rosen CE, Burde RM, Parisi F. Fascicular arrangement in partial oculomotor paresis. Am J Ophthalmol 1994;118:97-103. http://www.ncbi.nlm.nih.gov/pubmed/8023883 12. Leigh JR, Zee DS. Diagnosis of Peripheral Ocular Motor Palsies and Strabismus. Chp 9;385-474. In: The Neurology of Eye Movements, 4th Edition, Oxford University Press, New York 2006. 13. 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. http://www.ncbi.nlm.nih.gov/pubmed/1513475 14. Milandre L, Peretti P, Gouirand R, Khalil R. Paralysis of the common oculomotor nerve and contralateral cerebellar syndrome (Claude's syndrome). 2 cases caused by mesencephalic infarction Rev Neurol (Paris) 1992;225-229. http://www.ncbi.nlm.nih.gov/pubmed/1604140 15. Pratt DV, Orengo-Nania S, Horowitz BL, Oram O. Magnetic resonance imaging findings in a patient with nuclear oculomotor palsy. Arch Ophthalmol 1995;113:141-142. http://www.ncbi.nlm.nih.gov/pubmed/7864742 16. Saeki N, Yamaura A, Sunami K. Bilateral ptosis with pupil sparing because of a discrete midbrain lesion: magnetic resonance imaging evidence of topographic arrangement within the oculomotor nerve. J Neuro-ophthalmol 2000;20:130-134. http://www.ncbi.nlm.nih.gov/pubmed/10870930 17. Seo SW, Heo JH, Lee KY, Shin WC, Chang DI, Kim SM, Heo K.. Localization of Claude's syndrome. Neurology 2001;57:2304-2307. http://www.ncbi.nlm.nih.gov/pubmed/11756616 18. Toyoda K, Saku Y, Ibayashi S, Fushima M. Claude's syndrome associated with sensory loss. Cerebrovasc Dis 1997;7:55-56. |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Relation is Part of |
166-26 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Shirley H. Wray Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s63xmj88 |
| Setname |
ehsl_novel_shw |
| ID |
2174190 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s63xmj88 |
