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Why Can't This Patient Look Up? Bilateral Vertical Gaze Palsy
PM R XXX (2015) 1-3
www.pmrjournal.org
Case Presentation
Why Can’t This Patient Look Up? Bilateral Vertical Gaze Palsy Krishna Surapaneni, MD, Lisa Ferguson, OTR/L, Eric L. Altschuler, MD, PhD
Abstract We report a case of a patient with profound bilateral vertical gaze palsy resulting from a unilateral thalamic lesiondone of only a handful of such cases ever reported. The patient had significant symptomatic vertical diplopia that was disabling functionally and vocationally. We discuss pathways whereby a unilateral lesion can cause bilateral gaze palsy. We also report on the successful functional and vocational rehabilitation of the patient’s vertical diplopia. To our knowledge, this is the first report of successful rehabilitation of a patient with bilateral vertical diplopia due to a central lesion.
Introduction Bilateral vertical supranuclear gaze palsy is usually due to a variety of bilateral lesions involving the midbrain, with the palsy resulting most commonly from ischemia, adjacent compression from a mass lesion, or neurodegenerative causes. Rarely, paralysis of upward gaze can manifest in the setting of a unilateral thalamic lesion. We present the case of a patient with profound bilateral vertical gaze palsy from a unilateral thalamic infarction, without midbrain involvement. The patient’s vertical diplopia resulted in symptoms that were functionally and vocationally disabling. We discuss the successful rehabilitation of the patient, which is the first such rehabilitation for bilateral vertical diplopia to be reported, along with pathways whereby a unilateral lesion may cause a bilateral vertical gaze palsy. Case Presentation A 48-year-old man presented to the emergency department with sudden onset of dizziness, nausea, vomiting, headache, blurry vision, and vertical diplopia. His medical, family, and social histories were negative. Examination revealed that the patient had mild leftsided weakness and cerebellar signs and mild left facial droop. Although he had normal visual acuity and cognition, he had a profound bilateral upward gaze palsy that was slightly worse on the left (see Video). A
subsequent magnetic resonance imaging (MRI) scan demonstrated an acute infarct in the right medial thalamus without midbrain involvement (Figure 1), along with additional small acute infarcts in the left occipital lobe, cerebellum, and right pons. The clinical and imaging findings were thought to be a result of small acute embolic infarcts from underlying atrial fibrillation. The patient underwent anticoagulation and was transferred to the acute rehabilitation unit. Assessment by therapists revealed that the patient’s bilateral gaze palsy affected all of his daily activities. He reported persistent vertigo and diplopia and frequently lost his balance. The patient required minimal assistance for ambulation with a rolling walker and was at a minimum assistance level for transfers and activities of daily living. In addition to the typical stroke rehabilitation interventions, occupational and physical therapies focused on compensatory visual training, in light of the patient’s occupation that required reading and writing. All therapists and nursing staff implemented a daily eye patching schedule, alternating eyes every 2 hours with a 1-hour break. The schedule was posted on the wall in the patient’s room to promote compliance and carryover with the patient’s family. Additionally, the therapists introduced vertical analogues of various vestibular-ocular reflex exercises for gaze stabilization. The therapists observed torsional nystagmus during visual exercises and documented the recovery time for stabilization, which decreased by the end of the
1934-1482/$ - see front matter ª 2015 by the American Academy of Physical Medicine and Rehabilitation http://dx.doi.org/10.1016/j.pmrj.2015.04.011
2
Bilateral Vertical Gaze Palsy
strategies, including blocking and anchoring cues. Additionally, various therapeutic interventions were implemented for visual tracking, scanning, visual saccades, and gaze stabilization. These interventions included visual mazes, word searches, and saccades within functional activities such as retrieving grocery items in a pantry or clothes in a closet. Dynamic balance activities that incorporated the patient’s interests included mini golf, simulated baseball, and use of “Wii” and “Wii Fit” video game consoles. At the conclusion of the patient’s 2½-week inpatient rehabilitation stay, his balance improved and his level of vertigo and diplopia decreased. The patient was able to read and write functional information without vertigo. He implemented compensatory strategies to safely navigate crowded environments and to complete his daily activities. Upon discharge home, the patient improved to the modified independent level (eg, using a straight cane at times for ambulation) from the supervision level for ambulation and activities of daily living. He was able to return to work. Discussion
Figure 1. Diffusion-weighted imaging (A) and axial T2-weighted imaging (B) demonstrate restricted diffusion (with corresponding low apparent diffusion coefficient values, not shown) consistent with an acute infarct in the right medial thalamus with mild local mass effect.
patient’s stay. The patient was not taking medicine for vertigo. Reading and writing were particularly challenging for the patient, and therapists introduced compensatory
Classically, bilateral supranuclear vertical gaze palsy is caused by a variety of bilateral midbrain lesions from neurodegenerative causes such as progressive supranuclear palsy, a dorsal midbrain (Parinaud) syndrome from compressive mass lesions, direct midbrain injury from ischemia or demyelination, or metabolic conditions such as Niemann-Pick type C disease. The patient did not have signs of midbrain ischemia, such as a third nerve palsy, and no midbrain infarct was identified on an MRI scan. In this case, the bilateral gaze palsy was most likely due to a unilateral acute right medial thalamic infarct. Only a handful of such cases have been previously reported [1-3], and the pathway from a unilateral lesion mediating a bilateral vertical gaze palsy is not fully understood. The main structures involved in vertical gaze are the rostral interstitial nucleus of the medial longitudinal fasciculus, the interstitial nucleus of Cajal (INC), and the posterior commissure [4]. Lesions involving these structures are well known to produce vertical gaze palsies. Each rostral interstitial nucleus of the medial longitudinal fasciculus communicates with bilateral motor neurons (ipsilateral, and contralateral via the posterior commissure) for the elevator muscles (superior rectus and inferior oblique), although only ipsilaterally to motor neurons for the depressor muscles (inferior rectus and superior oblique) [5]. The INC serves as the neural integrator of vertical gaze holding, and a unilateral injury to the INC can cause impaired vertical and torsional gaze holding, ocular tilt reaction, and a torsional nystagmus. The supranuclear pathways for vertical gaze are not well understood. Tracts from the frontal and occipital cortex descend in the internal capsule through the subthalamic region and terminate
K. Surapaneni et al. / PM R XXX (2015) 1-3
in the pretectal region for the control of vertical ocular movements. Pathways from the frontal and supplementary eye fields traverse the thalamus, and the internal medullary lamina of the thalamus has reciprocal connections to these regions [6,7]. In this case, the patient’s medial thalamic infarct involved the medial dorsal nucleus, which sends projections to the frontal eye field in the motor cortex [6]. The pathway by which a unilateral thalamic lesion causes a bilateral vertical gaze palsy is unclear; the possibility that frontobulbar fibers in the medial thalamus potentially decussate in the medial thalamus has been suggested [1,3]. Damage to these fibers from the unilateral thalamic infarct is a potential explanation for the bilateral vertical gaze palsies. Additionally, the patient’s torsional nystagmus at presentation that subsequently improved with therapy suggested injury to the INC. The INC receives projections from the frontal eye field either directly or indirectly via a basal ganglia pathway, and interruption of this supranuclear pathway at the level of the thalamic infarct is a potential explanation for the patient’s torsional nystagmus. Dynamic balance training and visual scanning and tracking exercises were helpful in ameliorating the patient’s balance and gaze palsy deficits. Education and training regarding the patient’s deficits that we provided for his family was well received. The approach we followed in rehabilitation of these problems is similar to that used in a trial of patients with progressive supranuclear palsy [8]. The approach in that trial followed a paradigm used in a case study [9] interestingly from our institution of rehabilitation for a patient with progressive supranuclear palsy treated some decades ago. These studies showed some benefits that were, unfortunately, obviated by disease progression. Vertical diplopia was the patient’s most significant symptom. The diplopia may have been due to slight asymmetry in the side-to-side gaze deficit. We did not find any prior discussion of rehabilitation of centrally mediated bilateral vertical gaze palsy.
3
Conclusion It is interesting that vertical analogues of vestibularocular reflex exercisesdwhich are typically used for horizontal vestibular problemsdwere useful for this patient’s symptoms and contributed to successful vocational rehabilitation. Further studies of such an approach may be warranted. In the future, smart adaptive optics may also be useful for cases such as this. Video Gallery: To view the online videos, use your smartphone camera QR Reader App to scan and capture this QR Code or visit www. pmrjournal.org to locate this video content. URL: http://www.pmrjournal.org/article/ S1934-1482(15)00216-6/fulltext
References 1. Khan M, Sidiropoulos C, Mitsias P. Unilateral thalamic infarction presenting as vertical gaze palsy: A case report. J Med Case Rep 2011;5:535. 2. Blitshteyn S, Hentschel K, Czervionke LF, Eidelman BH. Transient vertical diplopia and nystagmus associated with acute thalamic infarction. Clin Imaging 2006;30:54-56. 3. Clark JM, Albers GW. Vertical gaze palsies from medial thalamic infarctions without midbrain involvement. Stroke 1995;26: 1467-1470. 4. Wong A. Eye Movement Disorders. New York, NY: Oxford University Press; 2008. 5. Serino J, Martins J, Paris L, Duarte A, Ribeiro I. Parinaud’s syndrome due to an unilateral vascular ischemic lesion. Int Opthalmol 2015; 35:275-279. 6. Bennaroch E. Medical Neurosciences: An Approach to Anatomy, Pathology and Physiology by Symptoms and Levels. 4th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 1999. 7. Gentilini M, De Renzi E, Crisi G. Bilateral paramedian thalamic infarcts: Report of eight cases. J Neurol Neurosurg Pyschiatry 1987; 50:900-909. 8. Zampieri C, Di Fabio RP. Improvement of gaze control after balance and eye movement training in patients with progressive supranuclear palsy: a quasi-randomized controlled trial. Arch Phys Med Rehabil 2009;90:263-270. 9. Izzo KL, DiLorenzo P, Roth A. Rehabilitation in progressive supranuclear palsy: Case report. Arch Phys Med Rehabil 1986;67:473-476.
Disclosure K.S. Department of Radiology, Temple University School of Medicine, Philadelphia, PA Disclosure: nothing to disclose L.F. Department of Physical Medicine and Rehabilitation, Temple University Hospital, Philadelphia, PA Disclosure: nothing to disclose
E.L.A. Department of Physical Medicine and Rehabilitation, Temple University School of Medicine, 3401 N Broad St, Philadelphia, PA 19140. Address correspondence to: E.L.A.; e-mail: [email protected] Disclosure: nothing to disclose Submitted for publication February 26, 2015; accepted April 16, 2015.
www.pmrjournal.org
Case Presentation
Why Can’t This Patient Look Up? Bilateral Vertical Gaze Palsy Krishna Surapaneni, MD, Lisa Ferguson, OTR/L, Eric L. Altschuler, MD, PhD
Abstract We report a case of a patient with profound bilateral vertical gaze palsy resulting from a unilateral thalamic lesiondone of only a handful of such cases ever reported. The patient had significant symptomatic vertical diplopia that was disabling functionally and vocationally. We discuss pathways whereby a unilateral lesion can cause bilateral gaze palsy. We also report on the successful functional and vocational rehabilitation of the patient’s vertical diplopia. To our knowledge, this is the first report of successful rehabilitation of a patient with bilateral vertical diplopia due to a central lesion.
Introduction Bilateral vertical supranuclear gaze palsy is usually due to a variety of bilateral lesions involving the midbrain, with the palsy resulting most commonly from ischemia, adjacent compression from a mass lesion, or neurodegenerative causes. Rarely, paralysis of upward gaze can manifest in the setting of a unilateral thalamic lesion. We present the case of a patient with profound bilateral vertical gaze palsy from a unilateral thalamic infarction, without midbrain involvement. The patient’s vertical diplopia resulted in symptoms that were functionally and vocationally disabling. We discuss the successful rehabilitation of the patient, which is the first such rehabilitation for bilateral vertical diplopia to be reported, along with pathways whereby a unilateral lesion may cause a bilateral vertical gaze palsy. Case Presentation A 48-year-old man presented to the emergency department with sudden onset of dizziness, nausea, vomiting, headache, blurry vision, and vertical diplopia. His medical, family, and social histories were negative. Examination revealed that the patient had mild leftsided weakness and cerebellar signs and mild left facial droop. Although he had normal visual acuity and cognition, he had a profound bilateral upward gaze palsy that was slightly worse on the left (see Video). A
subsequent magnetic resonance imaging (MRI) scan demonstrated an acute infarct in the right medial thalamus without midbrain involvement (Figure 1), along with additional small acute infarcts in the left occipital lobe, cerebellum, and right pons. The clinical and imaging findings were thought to be a result of small acute embolic infarcts from underlying atrial fibrillation. The patient underwent anticoagulation and was transferred to the acute rehabilitation unit. Assessment by therapists revealed that the patient’s bilateral gaze palsy affected all of his daily activities. He reported persistent vertigo and diplopia and frequently lost his balance. The patient required minimal assistance for ambulation with a rolling walker and was at a minimum assistance level for transfers and activities of daily living. In addition to the typical stroke rehabilitation interventions, occupational and physical therapies focused on compensatory visual training, in light of the patient’s occupation that required reading and writing. All therapists and nursing staff implemented a daily eye patching schedule, alternating eyes every 2 hours with a 1-hour break. The schedule was posted on the wall in the patient’s room to promote compliance and carryover with the patient’s family. Additionally, the therapists introduced vertical analogues of various vestibular-ocular reflex exercises for gaze stabilization. The therapists observed torsional nystagmus during visual exercises and documented the recovery time for stabilization, which decreased by the end of the
1934-1482/$ - see front matter ª 2015 by the American Academy of Physical Medicine and Rehabilitation http://dx.doi.org/10.1016/j.pmrj.2015.04.011
2
Bilateral Vertical Gaze Palsy
strategies, including blocking and anchoring cues. Additionally, various therapeutic interventions were implemented for visual tracking, scanning, visual saccades, and gaze stabilization. These interventions included visual mazes, word searches, and saccades within functional activities such as retrieving grocery items in a pantry or clothes in a closet. Dynamic balance activities that incorporated the patient’s interests included mini golf, simulated baseball, and use of “Wii” and “Wii Fit” video game consoles. At the conclusion of the patient’s 2½-week inpatient rehabilitation stay, his balance improved and his level of vertigo and diplopia decreased. The patient was able to read and write functional information without vertigo. He implemented compensatory strategies to safely navigate crowded environments and to complete his daily activities. Upon discharge home, the patient improved to the modified independent level (eg, using a straight cane at times for ambulation) from the supervision level for ambulation and activities of daily living. He was able to return to work. Discussion
Figure 1. Diffusion-weighted imaging (A) and axial T2-weighted imaging (B) demonstrate restricted diffusion (with corresponding low apparent diffusion coefficient values, not shown) consistent with an acute infarct in the right medial thalamus with mild local mass effect.
patient’s stay. The patient was not taking medicine for vertigo. Reading and writing were particularly challenging for the patient, and therapists introduced compensatory
Classically, bilateral supranuclear vertical gaze palsy is caused by a variety of bilateral midbrain lesions from neurodegenerative causes such as progressive supranuclear palsy, a dorsal midbrain (Parinaud) syndrome from compressive mass lesions, direct midbrain injury from ischemia or demyelination, or metabolic conditions such as Niemann-Pick type C disease. The patient did not have signs of midbrain ischemia, such as a third nerve palsy, and no midbrain infarct was identified on an MRI scan. In this case, the bilateral gaze palsy was most likely due to a unilateral acute right medial thalamic infarct. Only a handful of such cases have been previously reported [1-3], and the pathway from a unilateral lesion mediating a bilateral vertical gaze palsy is not fully understood. The main structures involved in vertical gaze are the rostral interstitial nucleus of the medial longitudinal fasciculus, the interstitial nucleus of Cajal (INC), and the posterior commissure [4]. Lesions involving these structures are well known to produce vertical gaze palsies. Each rostral interstitial nucleus of the medial longitudinal fasciculus communicates with bilateral motor neurons (ipsilateral, and contralateral via the posterior commissure) for the elevator muscles (superior rectus and inferior oblique), although only ipsilaterally to motor neurons for the depressor muscles (inferior rectus and superior oblique) [5]. The INC serves as the neural integrator of vertical gaze holding, and a unilateral injury to the INC can cause impaired vertical and torsional gaze holding, ocular tilt reaction, and a torsional nystagmus. The supranuclear pathways for vertical gaze are not well understood. Tracts from the frontal and occipital cortex descend in the internal capsule through the subthalamic region and terminate
K. Surapaneni et al. / PM R XXX (2015) 1-3
in the pretectal region for the control of vertical ocular movements. Pathways from the frontal and supplementary eye fields traverse the thalamus, and the internal medullary lamina of the thalamus has reciprocal connections to these regions [6,7]. In this case, the patient’s medial thalamic infarct involved the medial dorsal nucleus, which sends projections to the frontal eye field in the motor cortex [6]. The pathway by which a unilateral thalamic lesion causes a bilateral vertical gaze palsy is unclear; the possibility that frontobulbar fibers in the medial thalamus potentially decussate in the medial thalamus has been suggested [1,3]. Damage to these fibers from the unilateral thalamic infarct is a potential explanation for the bilateral vertical gaze palsies. Additionally, the patient’s torsional nystagmus at presentation that subsequently improved with therapy suggested injury to the INC. The INC receives projections from the frontal eye field either directly or indirectly via a basal ganglia pathway, and interruption of this supranuclear pathway at the level of the thalamic infarct is a potential explanation for the patient’s torsional nystagmus. Dynamic balance training and visual scanning and tracking exercises were helpful in ameliorating the patient’s balance and gaze palsy deficits. Education and training regarding the patient’s deficits that we provided for his family was well received. The approach we followed in rehabilitation of these problems is similar to that used in a trial of patients with progressive supranuclear palsy [8]. The approach in that trial followed a paradigm used in a case study [9] interestingly from our institution of rehabilitation for a patient with progressive supranuclear palsy treated some decades ago. These studies showed some benefits that were, unfortunately, obviated by disease progression. Vertical diplopia was the patient’s most significant symptom. The diplopia may have been due to slight asymmetry in the side-to-side gaze deficit. We did not find any prior discussion of rehabilitation of centrally mediated bilateral vertical gaze palsy.
3
Conclusion It is interesting that vertical analogues of vestibularocular reflex exercisesdwhich are typically used for horizontal vestibular problemsdwere useful for this patient’s symptoms and contributed to successful vocational rehabilitation. Further studies of such an approach may be warranted. In the future, smart adaptive optics may also be useful for cases such as this. Video Gallery: To view the online videos, use your smartphone camera QR Reader App to scan and capture this QR Code or visit www. pmrjournal.org to locate this video content. URL: http://www.pmrjournal.org/article/ S1934-1482(15)00216-6/fulltext
References 1. Khan M, Sidiropoulos C, Mitsias P. Unilateral thalamic infarction presenting as vertical gaze palsy: A case report. J Med Case Rep 2011;5:535. 2. Blitshteyn S, Hentschel K, Czervionke LF, Eidelman BH. Transient vertical diplopia and nystagmus associated with acute thalamic infarction. Clin Imaging 2006;30:54-56. 3. Clark JM, Albers GW. Vertical gaze palsies from medial thalamic infarctions without midbrain involvement. Stroke 1995;26: 1467-1470. 4. Wong A. Eye Movement Disorders. New York, NY: Oxford University Press; 2008. 5. Serino J, Martins J, Paris L, Duarte A, Ribeiro I. Parinaud’s syndrome due to an unilateral vascular ischemic lesion. Int Opthalmol 2015; 35:275-279. 6. Bennaroch E. Medical Neurosciences: An Approach to Anatomy, Pathology and Physiology by Symptoms and Levels. 4th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 1999. 7. Gentilini M, De Renzi E, Crisi G. Bilateral paramedian thalamic infarcts: Report of eight cases. J Neurol Neurosurg Pyschiatry 1987; 50:900-909. 8. Zampieri C, Di Fabio RP. Improvement of gaze control after balance and eye movement training in patients with progressive supranuclear palsy: a quasi-randomized controlled trial. Arch Phys Med Rehabil 2009;90:263-270. 9. Izzo KL, DiLorenzo P, Roth A. Rehabilitation in progressive supranuclear palsy: Case report. Arch Phys Med Rehabil 1986;67:473-476.
Disclosure K.S. Department of Radiology, Temple University School of Medicine, Philadelphia, PA Disclosure: nothing to disclose L.F. Department of Physical Medicine and Rehabilitation, Temple University Hospital, Philadelphia, PA Disclosure: nothing to disclose
E.L.A. Department of Physical Medicine and Rehabilitation, Temple University School of Medicine, 3401 N Broad St, Philadelphia, PA 19140. Address correspondence to: E.L.A.; e-mail: [email protected] Disclosure: nothing to disclose Submitted for publication February 26, 2015; accepted April 16, 2015.