Third Nerve Palsy;
Fixed Dilated Pupil;
Wray, Shirley H.
Shirley H. Wray, MD, PhD, FRCP, Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Fascicular Third Nerve Palsy;
Fixed Dilated Pupil;
Contralateral Limb Ataxia;
Unilateral Third Nerve Palsy;
Fascicular Oculomotor (Third) Nerve Palsy;
Third Nerve Dysfunction
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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.
Right third nerve palsy involving the pupil
Ataxia of the left hand on finger-nose test
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.
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:
• 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.
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.
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.
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.
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