|Year : 2018 | Volume
| Issue : 11 | Page : 29-31
Claude syndrome: A report of two cases and review of literature
S Sheetal, M Madhusudanan, Reji Thomas, P Byju
Department of Neurology, Pushpagiri Institute of Medical Sciences and Research Centre, Thiruvalla, Kerala, India
|Date of Web Publication||3-Jul-2018|
‘Sajan’, TC 1072/4, Pazhaya Road, Medical College P. O., Trivandrum, Kerala - 695 011, Karnataka
Source of Support: None, Conflict of Interest: None
Claude syndrome refers to the association of unilateral oculomotor and/or trochlear palsy of midbrain origin with contralateral ataxia. Detailed description of this syndrome is rare, partly due to the rarity of its occurrence. This is a report of two patients who presented to us with features suggestive of Claude syndrome.
Keywords: Benedikt syndrome, Claude syndrome, midbrain, Weber's syndrome
|How to cite this article:|
Sheetal S, Madhusudanan M, Thomas R, Byju P. Claude syndrome: A report of two cases and review of literature. N Niger J Clin Res 2018;7:29-31
| Introduction|| |
Detailed studies on the infarction of the midbrain are sparse. Several eponymic oculomotor fascicular syndromes have been described with infarctions of the midbrain. The ones described include Claude syndrome, Nothnagel syndrome, Weber syndrome More Details and Benedikt syndrome. Claude syndrome is caused by a lesion of the ventromedial midbrain, resulting in the combination of an ipsilateral oculomotor palsy and contralateral ataxia. It was first described by Henri Claude in 1912. Involvement of the cranial nerve III nucleus and/or nerve fibers leads to oculomotor nerve palsy. Insult to the red nucleus, brachium conjunctivum, or fibers of the superior cerebellar peduncle results in incoordination and cerebellar hemiataxia. There have been few studies describing the magnetic resonance imaging appearance of lesions associated with this syndrome. We hereby describe the clinical features and MRI appearance two patients with midbrain infarction, with features suggestive of Claude syndrome.
| Case Reports|| |
An 87-year-old male presented to us with complaints of unsteadiness of gait and double vision of 4 days duration. This was noted on waking up in the morning. He noted marked unsteadiness on trying to walk with tendency to fall to right side. Drooping of left eye with binocular diplopia and horizontal separation of images, more on looking toward the right side were noticed. The symptoms showed no further progression after onset. There was no history of any facial deviation, numbness over face, nasal regurgitation, dysphagia, or dysarthria. It was not associated with any limb weakness or bowel bladder symptoms. He gave no history of fever or other systemic symptoms. He was diagnosed to have hypertension 6 years back but was not on any medications for the same. There was no history of diabetes or other systemic illness.
On examination, he was conscious and oriented. He had a pulse of 80/min, which was regular, and a blood pressure of 150/90 mmHg. Higher mental functions were normal. There was ptosis, with impaired adduction of the left eye. His pupils were bilaterally normal sized and reacting well to light. His accommodation reflex and other extraocular movements were normal. Other cranial nerves were normal. Motor system examination revealed normal power and deep tendon reflexes. Plantar was B/L flexor. Sensory examination was normal. He had clumsiness of hand movements on the right side, with finger-nose incoordination, dysdiadokokinesia, and impaired heel to knee test.
Magnetic resonance imaging (MRI) brain showed focal lesion in the left paramedian midbrain with T2 and fluid-attenuated inversion recovery (FLAIR) [Figure 1] hyperintensity and diffusion restriction [Figure 2], suggesting acute infarct. MR angiogram, carotid Doppler, and cardiac evaluation including 24-h Holter evaluation were normal.
|Figure 1: Diffusion-weighted imaging images of the brain showing left paramedian midbrain infarct|
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|Figure 2: Apparent diffusion coefficient images of the brain showing low apparent diffusion coefficient values in left paramedian midbrain suggestive of acute infarct|
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The clinical features indicated a diagnosis of Claude syndrome secondary to acute stroke. He was started on aspirin 150 mg, atorvastatin 40 mg, and other supportive measures including physiotherapy and gait training.
A 48-year-old male, chronic smoker, hypertensive, and diabetic, presented to us with complaints of unsteadiness of gait and polyopia of 1-day duration, with horizontal and vertical separation of images. He had a tendency to sway toward the right on walking. There was no history of any other cranial nerve symptoms or limb weakness.
On examination, he was conscious, well oriented. His pulse rate was 80/m and regular. Blood pressure was 130/80 mmHg. He had impaired adduction and partial ptosis of the left eye, indicating a left oculomotor palsy. He was also noted to have impaired infraduction on adduction of the right eye, indicating a right superior oblique palsy. Other extraocular movements were normal. Pupils were equal and reacting normally, bilaterally. Motor power was normal in all four limbs, deep tendon reflexes were normal bilaterally, and plantar was bilaterally flexor. Cerebellar signs were positive on the right side.
MRI brain showed focal lesion in the left paramedian midbrain showing diffusion restriction [Figure 3] and [Figure 4] suggesting acute infarct. However, there was no abnormality on T2 or FLAIR sequences. MR angiogram, carotid Doppler, and cardiac evaluation including 24-h Holter evaluation were normal.
|Figure 3: Diffusion-weighted imaging images of the brain showing hyperintensity in the left paramedian midbrain|
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|Figure 4: Apparent diffusion coefficient images of the brain showing low apparent diffusion coefficient values in left paramedian midbrain suggestive of acute infarct|
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In view of the left-sided oculomotor and trochlear nuclear involvement, with right-sided cerebellar signs and the MRI brain abnormalities, a diagnosis of Claude syndrome was made. He was started on aspirin 150 mg, atorvastatin 40 mg, and other supportive measures including physiotherapy and gait training.
| Discussion|| |
The midbrain syndromes of Claude, Nothnagel, and Benedikt have been subjected to controversies with regard to their involvement of various structures and clinical presentations. The association of unilateral oculomotor palsy of midbrain origin with contralateral ataxia is referred to as Nothnagel syndrome as well as Claude syndrome by various authors. In the initial description by Nothnagel, he described bilateral ophthalmoparesis with pupillary involvement and ipsilateral gait ataxia in a patient with hydrocephalus and sarcoma involving all four colliculi. Although Nothnagel's descriptions were mainly for quadrigeminal neoplasms, the syndrome has been described with infarct and other lesions by many authors. However, Nothnagel syndrome has been often described as bilateral/unilateral oculomotor palsy with ipsilateral/contralateral ataxia. The syndrome described by French psychiatrist and neurologist Henry Claude in 1912 included ipsilateral complete ophthalmoplegia with contralateral ataxia. In his report, there was pupillary involvement in the form of a dilated and fixed pupil and impaired convergence of both eyes. His patient had a paramedian mesencephalic infarction with involvement of superior cerebellar peduncle, medial half of red nucleus, and medial longitudinal fasciculus and oculomotor nerve fascicles. There was no clear mention about cause of superior oblique palsy. Later, Claude and Levi Valenci in their modified description included sensory impairment and trochlear nerve involvement. Another related midbrain syndrome Benedikt syndrome however has rather clear-cut manifestations with unilateral oculomotor palsy, contralateral hemiparesis, tremor, and involuntary movements. The site of involvement in this syndrome is ipsilateral oculomotor nerve fascicle, red nucleus, substantia nigra, and cerebral peduncle.,,
Our first patient had strictly unilateral oculomotor palsy and contralateral ataxia without involvement of the ipsilateral fourth nerve, opposite eye, or of sensory system. The pupil was also spared unlike the original description of the syndromes of Claude and Nothnagel. Our case is unique, in that it was a limited paramedian mesencephalic infarct involving superior cerebellar peduncle and third nerve fascicle. Our second patient had features of ipsilateral oculomotor palsy and contralateral ataxia, along with trochlear palsy as described by Claude and Valenci. However, sensory system was also not involved.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]