Vertical gaze palsy
Jason J S Barton MD PhD, author. Dr. Barton of the University of British Columbia in Vancouver has no relevant financial relationships to disclose.
James Goodwin MD, editor. Dr. Goodwin of the University of Illinois at Chicago has no relevant financial relationships to disclose.
Publication dates
Originally released July 19, 2001; last updated February 27, 2012; expires February 27, 2015
• Vertical gaze palsies are due to damage to pre-motor structures in the midbrain, namely the rostral interstitial nucleus of the medial longitudinal fasciculus and the interstitial nuclear of Cajal.
• Vertical gaze palsies can involve upgaze, downgaze, or both.
• Parkinsonian conditions with vertical gaze palsies are due most often to tauopathies, such as progressive supranuclear palsy and corticobasal degeneration.
• Several genetic defects can cause cerebellar ataxia with vertical gaze palsies, chief of which is Niemann Pick type C disease.
The term “gaze palsy” is best restricted to deficits in conjugate eye movements that affect both eyes. Thus, strictly unilateral problems such as palsies of cranial nerves III, IV, or VI are not gaze palsies, even though they do affect gaze. Likewise, impairments in vergence control, such as convergence or divergence insufficiency, are not gaze palsies, as they do not involve conjugate eye movements.
A fundamental distinction is between vertical and horizontal gaze palsies. Most gaze palsies affect 1 direction in 1 plane of eye movement only, reflecting the separation of the prenuclear control systems for vertical and horizontal eye movement. Reduction of eye movements in all planes is best termed “generalized ophthalmoparesis.” These reductions are most commonly myopathic, occurring with mitochondrial disorders (chronic progressive external ophthalmoplegia, Kearns-Sayre syndrome, MELAS) or muscular dystrophies (myotonic dystrophy, oculopharyngeal dystrophy, congenital fibrosis), among others.
The term “gaze palsy” requires further elaboration. There are many different types of conjugate eye movements, including saccades, pursuit, optokinetic, and vestibulo-ocular responses. The anatomic systems that control these diverge and converge at various levels, and it is possible for some lesions to impair some eye movement systems and spare others. Hence, a left saccadic palsy is a selective gaze palsy affecting only leftward saccades but not leftward pursuit or vestibulo-ocular response. A palsy affecting all types of eye movements should be designated as a nonselective gaze palsy. Most vertical gaze palsies are selective in nature.
In contrast, the terms “partial” or “complete” when applied to gaze palsy indicate whether some motion across midline in the paretic direction is present.
Vertical palsies usually appear selective, affecting primarily saccades. However, though clinical testing often shows sparing of pursuit and vestibulo-ocular range, quantitative testing of eye movements shows that this selectivity is relative and not absolute (Sharpe and Kim 2002). Pursuit gain and vestibulo-ocular reflexes are impaired in many patients, though dissociable. Upgaze palsy is most frequent, combined upgaze and downgaze palsy is next in frequency, and pure downgaze palsy the most unusual (Bogousslavsky et al 1988). Because these are due to lesions of rostral midbrain nuclei, associated signs include pupillary or ocular motor signs of partial nuclear or fascicular third palsies (Castaigne et al 1981; Beversdorff et al 1995), including rarely a wall-eyed bilateral internuclear ophthalmoplegia (Sierra-Hidalgo et al 2010), impaired convergence, and skew deviation (Ranalli and Sharpe 1988; Steinke et al 1992). Somnolence or even a transient fluctuating coma at onset reflects damage to the reticular activating system (Castaigne et al 1981; Bogousslavsky et al 1988; Beversdorff et al 1995). Behavioral disturbances from thalamic damage include hemineglect, amnestic syndromes (Bogousslavsky et al 1988; Beversdorff et al 1995), akinetic mutism, or subcortical demented states with apathy and slowness of thought (Guberman and Stuss 1983).
Upgaze palsy.
This is frequent with unilateral lesions at either the thalamomesencephalic junction (Bogousslavsky et al 1986; 1988), or the posterior commissure, or its nucleus(Buttner-Ennever et al 1982). There are often other signs of the pretectal syndrome. A lesion of the periaqueductal grey matter rarely causes this, perhaps by destroying descending outputs from the riMLF (Thames et al 1984). Rarely, it occurs as a transient effect of right frontoparietal lesions, with bilateral ptosis (Averbuch-Heller et al 1996).
This is frequent with unilateral lesions at either the thalamomesencephalic junction (Bogousslavsky et al 1986; 1988), or the posterior commissure, or its nucleus(Buttner-Ennever et al 1982). There are often other signs of the pretectal syndrome. A lesion of the periaqueductal grey matter rarely causes this, perhaps by destroying descending outputs from the riMLF (Thames et al 1984). Rarely, it occurs as a transient effect of right frontoparietal lesions, with bilateral ptosis (Averbuch-Heller et al 1996).
Downgaze palsy. This occurs with bilateral dorsomedial lesions of the rostral intrastitial nucleus of the medial longitudinal fasciculus (Buttner-Ennever et al 1982; Bogousslavsky et al 1988). It is hypothesized that bilateral lesions extending laterally impair upgaze also; therefore, selective downgaze palsy must require a small and specific lesion, accounting for its rarity (Pierrot-Deseilligny et al 1982). Convergence, accommodative responses, and the pupillary near response may all be impaired too (Cogan 1974). The pupillary light response can be affected (Cogan 1974) or preserved (Pierrot-Deseilligny et al 1982). Skew deviation and internuclear ophthalmoplegia can occur (Cogan 1974).
Downgaze is also affected by akinetic movement disorders, most typically progressive supranuclear palsy (Cogan 1974).
Combined up and down gaze palsy. The lesions involve the riMLF or the interstitial nucleus of Cajan, most frequently bilaterally. In the less common unilateral cases the lesion of the ipsilateral riMLF likely also interrupts decussating fibers from the contralateral riMLF. Vertical vestibulo-ocular response frequently appears normal (Buttner-Ennever et al 1982; Page et al 1982; Pierrot-Deseilligny et al 1982; Yamamoto 1989; Bogousslavsky et al 1990), but is sometimes absent (Beversdorff et al 1995) or impaired in 1 direction alone (Guberman and Stuss 1983). Torsional and vertical nystagmus may occur if the interstitial nucleus of Cajal is involved (Ranalli and Sharpe 1988). Bell phenomenon can be absent (Page et al 1982) or inverted (Ranalli and Sharpe 1988).
Pretectal syndrome. This syndrome combines vertical supranuclear palsy, affecting either upgaze alone or both upgaze and downgaze, sparing vestibulo-ocular response range, with a variable number of other signs (Keane 1990). These include light-near pupillary dissociation, with loss of the pupillary light reactions from damage to the pretectum, Collier lid retraction sign, and skew deviation. Horizontal conjugate eye movements are spared but there may be esotropia, exotropia, or convergence insufficiency. An unusual convergence-retraction nystagmus is pathognomonic. Fragmentary pretectal syndrome, with only some of the above features, is common.
Vertical one-and-a-half and other syndromes. Rarely a patient may have a vertical impairment that spares only a single direction in 1 eye. Supranuclear bilateral downgaze paresis affecting all movements combined with monocular elevator palsy occurs with bilateral midbrain infarction (Deleu et al 1989). The opposite, supranuclear bilateral upgaze paresis with monocular depressor palsy, has also been described with unilateral midbrain infarctions (Bogousslavsky and Regli 1984; Miyashita et al 1987; Gulyas et al 2006). A unique case of ipsilateral monocular elevator paresis and contralateral monocular depressor paresis, combined with mild bilateral ptosis, has been reported (Wiest et al 1996). Finally, a patient with supranuclear vertical palsy combined with complete ophthalmoplegia of 1 eye has been described, with the ophthalmoplegia attributed to a combination of oculomotor nerve palsy and pseudoabducens palsy (Thurtell et al 2009).
Vertical congenital ocular motor apraxia is rare (Ro et al 1989; Brown and Willshaw 2003) and has been related to perinatal hypoxia (Hughes et al 1985) or bilateral mesencephalic-diencephalic lesions (Ebner et al 1990).
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