Supranuclear Paralysis of Upgaze

Supranuclear Paralysis of Upgaze Convergence Retraction Nystagmus Eyelid Retraction - Collier's Sign Normal Downgaze Esotropia Sixth Nerve PalsyHeadache and unsteadinessThis young child presented with headache and unsteadiness. He was found to have obstructive hydrocephalus, aqueduct stenosis and a medulloblastoma. The constellation of clinical eye signs localized to the Dorsal Midbrain and included: • Supranuclear paralysis of upgaze (saccadic and pursuit movements) • Convergence retraction nystagmus on attempted upgaze • Lid retraction - Collier's sign • Pupillary abnormalities (light/near dissociated) In addition he had: • Esotropia of the right eye due to • A right sixth nerve palsy Neurological examination: Slight enlargement of head circumference Trunkal ataxia Diminished motor strength throughout Hyperreflexia and extensor plantar responses Brain CT showed: Obstructive hydrocephalus Aqueduct stenosis An infiltrating brainstem neoplasm consistent with a medulloblastoma Treatment: Ventriculoperitoneal Shunt Diagnosis: Medulloblastoma Obstructive hydrocephalus Aqueduct stenosisThis infant was filmed in the Intensive Care Unit shortly after placement of a ventriculoperitoneal shunt for treatment of obstructive hydrocephalus. Dorsal midbrain eye signs illustrated are: • Supranuclear paralysis of upgaze (saccadic and pursuit movements) • Convergence retraction nystagmus on attempted upgaze • Lid retraction - Collier's sign • Normal downgaze • Pupillary abnormalities (light/near dissociated) In addition he had: • Esotropia of the right eye due to • A right sixth nerve palsy Dorsal Midbrain (Pretectal) Syndrome in childhood: Other causes include: Congenital aqueduct stenosis leading to hydrocephalus Niemann-Pick disease (Type C) Gaucher's disease Tay-Sach's disease Kernicterus Pretectal pseudobobbing: Another disorder of saccades called pretectal pseudobobbing is reported in patients with acute obstructive hydrocephalus. The disorder consists of non-rhythmic, rapid movements which carry the eyes down and medially, (i.e. adducting movements) followed by a slow return toward the midline. Each movement may be preceded by a blink. The horizontal component may be a variant of convergence nystagmus. Convergence Retraction Nystagmus: Convergence retraction nystagmus has been regarded as a saccadic disorder consisting of asynchronous, opposed saccades whenever upward quick phases are stimulated. However, other studies indicate that, at least in some patients, convergence-retraction nystagmus is a primary disorder of the vergence system. Sixth Nerve Palsy in Children: Impaired abduction of one eye, sometimes transient, maybe the first sign of a tumor in the posterior fossa. Children however seldom complain of double vision and a head turn to the involved side is the most prominent finding. Co-existence of a sixth nerve palsy with cerebellar signs is usually indicative of an astrocytoma or ependymoma, or a medulloblastoma as in this child. Less commonly a supratentorial mass lesion may present with a sixth nerve palsy as a false localizing sign, in a child with raised intrarcranial pressure and papilledema. Pseudo-Abducens Palsy: The dorsal midbrain syndrome also results in a disturbance of vergence eye movements. In some patients convergence is paralyzed whilst in others it is excessive and causes convergent spasm. During horizontal saccades, the abducting eye may move more slowly than its adducting fellow. This finding has been called pseudo-abducens palsy and may reflect excess of convergence tone. Alternatively, abduction weakness with midbrain lesions that cause sustained esotropia may be due to effects on ocular motor internuclear neurons that project to the pons, since experimental inactivation of these cells causes paresis of abduction.The CT study is unavailable in this case.Hydrocephalus with aqueduct stenosis leads to dilation of the third ventricle and aqueduct or enlargement of the suprapineal recess with pressure on the posterior commissure. Three neural structures in the midbrain reticular formation are involved in the generation of vertical eye movements: 1. The posterior commissure (PC) 2. The interstitial nucleus of Cajal (INC) 3. The rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) Lesions of the posterior commissure are traditionally equated with a syndrome of loss of upward gaze and associated findings, and is generally known by a variety of names: Dorsal midbrain syndrome Pretectal syndrome Parinaud's syndrome Koeber-Salus-Elschig syndrome Sylvian aqueduct syndrome The clinical features of the dorsal midbrain (pretectal) syndrome are due to lesions of the nucleus of the posterior commissure (nPC), and axons projecting to the INC which may be important for the control of vertical gaze and eyelid movements. Cells in the nPC project through the posterior commissure and may contact the riMLF, INC, and M-group of neurons, which relays to the central caudal subdivision of the ocular motor nucleus and to coordinate vertical eye and lid movements. 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