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The patient is a 25 year old man with a long history of headaches due to hydrocephalus.
He carries the following diagnoses:
2. Aqueduct stenosis
3. Low grade glioma involving the quadrageminal plate
The patient first came to neurological attention at the age of 12 when he presented with headache and malaise. Hydrocephalus and aqueduct stenosis was diagnosed and a ventriculoperitoneal shunt was placed.
Unfortunately there was little documented history until 1987. Since then the patient has had about 20 shunt revisions. Shunt failure is preceded by increased intracranial pressure manifested as headache and/or seizures. The seizures have only occurred in the setting of increased intracranial pressure. There is no history of tonic, clonic movements or aura. During the seizure he can talk sensibly, but when it is over he will lose consciousness and have amnesia for the period. His last seizure was prior to his last shunt revision in July 1991.
Prior to shunt revision the patient has days to weeks of headaches which are bilateral, frontal or occipital and severe, aggravated by lying flat or coughing and associated with vomiting and drowsiness.
Patient was born in the US but spent several of his earliest years in Peurto Rico.
No family history of neurologic disease
In Jan 1994 he was seen in the Neurology Clinic for re-evaluation of his seizures and presented to Dr. Raymond Adams.
At that time he was receiving Tegretol 200 mg 5 tablets a day.
His mental status was normal with no memory impairment
Pupils 3 mm reactive to light but unresponsive to near
The right pupil constricted only slightly and the left eye not at all.
Fundus examination normal.
Supranuclear upgaze (saccades and pursuit) mild, approximately 20% short of full upgaze.
Complete paralysis of convergence
Horizontal gaze evoked rotary nystagmus to right and left
Normal vertical downgaze
Normal vertical oculocephalic reflex
Normal deviation of the eyes up under closed lids (Bell’s phenomenon)
The remainder of the neurological exam was normal
Tendon reflexes symmetric 1+, flexor plantar responses
No ataxia, normal gait.
An Electroencephalogram showed left greater than right slowing (including delta rhythm) with the patient awake.
An EEG recorded in 1982 showed right greater than left slowing during normal sleep.
Brain MRI 1/15/94:
A pre and post Gadolinium study was performed and compared with a prior head CT dated 3/5/93.
The study showed a right ventriculostomy tube in place. The ventricles normal in size.
A large mass was present involving the quadrageminal plate. The mass was iso-intense to brain on T1 weighted and hyperintense on T2 weighted images, and did not enhance significantly with Gadolinium.
Impression: Low-grade glioma
No interval change in size over 1 year.
Dermatology noted a 1 x 1 hyperpigmented lesion on his left palm which was unremarkable under Wood’s lamp.
The patient had multiple small freckles on his back, none in the axilla or other important locations, and none larger than 1 cm., ruling out neurofibromatosis.
Plan for the patient:
As the patient’s last seizure was in 1991, the plan was to keep him on Tegretol for a 5 year seizure – free interval and at that time consider stopping the medication..
MRI every 2 years
Low grade midbrain gliomas are known to be indolent and have a good prognosis.
A hamartoma was an unlikely alternative diagnosis.
This patient with a low grade glioma of the quadrageminal plate had the clinical triad of Parinaud’s syndrome:
1. Supranuclear upgaze palsy
2. Paralysis of convergence and
3. Light/near dissociation of the pupils
The video tape shows:
1. Paralysis of convergence even with the patient attempting to look at his own finger at near. A slight impairment of full upgaze.
He had no:
1. Convergence retraction nystagmus
2. Lid retraction – Collier’s syndrome
3. Supranuclear paresis of downgaze
Parinaud’s syndrome also called Koeber-Salus-Elschig Syndrome, pretectal syndrome, or Sylvian aqueduct syndrome, is due to a lesion of the posterior commissure and nucleus of the posterior commissure.
A unilateral midbrain lesion can also create the same oculomotor syndrome by interrupting both projections through the posterior commissure.
Disturbances of vergence eye movements in the dorsal midbrain syndrome include:
• Convergence retraction nystagmus
• Paralysis of convergence
• Spasm of convergence
• Paralysis of divergence
• “A” or “V” – pattern exotropia
• Pseudo-abducens palsy
Pseudo-abducens palsy, according to Leigh and Zee, may reflect excessive convergence tone. During horizontal saccades, the abducting eye may move more slowly than its adducting fellow.
Hydrocephalus may produce the pretectal syndrome by enlarging the aqueduct and third ventricle or the suprapineal recess, and so stretching or compressing the posterior commissure.
The patient’s MR images can be viewed at the end of the video tape.
1. Baloh RW, Furman JM, Yee RD. Dorsal midbrain syndrome: clinical and oculographic findings. Neurology 1985;35:54-60. http://www.ncbi.nlm.nih.gov/pubmed/3966002
2. Corbett JJ. Neuro-ohthalmological complications of hydrocephalus and shunting procedures. Semin Neurol 1986; 6:111-123. http://www.ncbi.nlm.nih.gov/pubmed/3332415
3. Daroff RB, Hoyt WF. Supranuclear disorders of ocular control systems in man: Clinical, anatomical and physiological correlations-1969. In Bach-y-Rita P. Collins CC, Hyde JE (eds). the Control of Eye Movements. Academic Press, New York 1971;175-235.
4. Frohman LP, Kupersmith MJ. Reversible vertical ocular deviations associated with raised intracranial pressure. J Clin Neuro-ophthalmol 1985;5:158-163. http://www.ncbi.nlm.nih.gov/pubmed/2934416
5. Leigh, JR. Zee, DS. Diagnosis and Central Disorders of Ocular Motility. Chp 12, 598-718. In: The Neurology of Eye Movements, 4th Edition, Oxford University Press, New York 2006.
6. Osher RH, Corbett JJ, Schatz NJ, Savino PJ, Orr LS. Neuro-ophthalmological complications of enlargement of the third ventricle. Br J Ophthalmol 1978;62:536-542. http://www.ncbi.nlm.nih.gov/pubmed/687551
7. Pullicino P, Lincoff N. Truax BT. Abnormal vergence with upper brainstem infarcts: pseudoabducens palsy. Neurology 2000;55:352-358. http://www.ncbi.nlm.nih.gov/pubmed/10932266