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Bilateral Horizontal Gaze Palsy with Unilateral Peripheral Facial Paralysis Caused by Pontine Tegmentum Infarction
Case Report
Bilateral Horizontal Gaze Palsy with Unilateral Peripheral Facial Paralysis Caused by Pontine Tegmentum Infarction Andre C. Felicio, MD, Denis B. Bichuetti, MD, Luis F. Marin, MD, William A. C. dos Santos, MD, and Clecio Godeiro-Junior, MD
Clinical features of pontine infarction depend on the topography of vascular lesion and most remarkably sometimes the same topographic region can lead to different clinical syndromes (e.g., dorsal pontine tegmentum). In this report we describe an elderly patient with acute dorsal pontine infarction leading to a unique syndrome of bilateral horizontal gaze palsy and unilateral peripheral facial paralysis. We propose that this syndrome could be included as a part of a continuum that involves one-and-a-half syndrome, eight-and-a-half syndrome, and other variants of pontine tegmentum infarction. Key Words: One-and-a-half syndrome—eight-and-a-half syndrome—bilateral horizontal gaze palsy—peripheral facial paralysis—pontine infarction. Ó 2009 by National Stroke Association
Tegmental pontine infarctions are unusual and may lead to a range of neuro-ophthalmologic findings such as gaze palsies, diplopia, internuclear ophthalmoplegia, nystagmus, and abducens palsy.1 Herein, we present a unique case of acute pontine infarction leading to a combination of bilateral horizontal gaze palsy and unilateral peripheral facial palsy. In addition, we also discuss the relationship between this case and the one-and-a-half and eight-and-a-half syndromes. A 73-year-old white man was admitted with a 15-minute history of sudden-onset slurred speech and right facial weakness. He was on glucoformin (850 mg twice a day) and aspirin (200 mg/day) for diabetes and did not smoke cigarettes or drink alcohol. Family history was unremarkable. At the emergency department his From the Neurology Division, Hospital e Maternidade Sa˜o Camilo, Pompe´ia, Sao Paulo, Brazil. Received September 2, 2008; revision received October 17, 2008; accepted October 24, 2008. Address correspondence to Andre C. Felicio, MD, Bosque da Saude 834/193, Postal Code 04142-081, Sao Paulo, SP, Brazil. E-mail: [email protected]. 1052-3057/$—see front matter Ó 2009 by National Stroke Association doi:10.1016/j.jstrokecerebrovasdis.2008.10.008
244
heart rate was 100 beats/min, blood pressure was 178 3 96 mm Hg, and glucose was 250 mg/dL. The patient was alert, fully oriented, and collaborative. Pupils were symmetric (2 mm) and reactive to light. No ocular bobbing was found. There was a bilateral horizontal gaze palsy with preserved vertical saccades (Fig 1) associated to right peripheral facial paralysis. The oculocephalic reflexes were absent bilaterally. Despite his attentiveness and orientation, speech was compromised with a moderate dysarthria without aphasia. He scored 7 on the National Institutes of Health Stroke Scale. Brain computed tomography was performed and no abnormalities were found. The patient was excluded from venous thrombolytic recombinant tissue plasminogen activator because of recent leg bypass vascular surgery. Brain magnetic resonance imaging showed no alteration at 1.5 T with T1-, T2-, fluid-attenuated inversion recovery, and echocardiographic-gradient-weighted images. Diffusion-weighted image showed a small but irregular restricted diffusion area on the midline pontine tegmentum with a correspondent hypointense lesion on the apparent diffusion coefficient map (Fig 2, A and B). Magnetic resonance angiography showed vascular irregularities on large vessels and a significant stenosis on the middle part of the left posterior cerebral artery (Fig 2, C). The patient was
Journal of Stroke and Cerebrovascular Diseases, Vol. 18, No. 3 (May-June), 2009: pp 244-246
PONTINE TEGMENTUM INFARCTION
245
Figure 2. Brain magnetic resonance imaging on diffusion-weighted sequence (A) showing hyperintense lesion at dorsal pontine tegmentum with correspondent hyposignal on attenuated diffusion coefficient map (B). Note significant irregularities of arteries from posterior circulation and stenosis of left posterior cerebral artery. Stenosis, which is obviously not related to patient’s infarction, shows diffuse disease of cerebral arteries (C).
Figure 1. (A) Patient at primary position gaze (P). (B and C) Note normal vertical eye movements. (D and E) Note bilateral horizontal gaze palsy. Arrows indicate direction of gaze shifts. Senile arch is seen around patient’s cornea. Nasoenteral tube is placed. L, Left; R, right.
admitted to the intensive care department for conservative stroke treatment and later discharged from our hospital with a modified Rankin scale score of 2.
Discussion The ‘‘one-and-a-half syndrome’’ was coined by Fisher2 in 1967 and is characterized by an ipsilateral horizontal gaze palsy to one side and a restriction for adduction to the other side (1 1 1⁄2 5 11⁄2 ). Lesion is situated on the
paramedian pontine reticular formation to the side of the restricted adducted eye. When the fibers of the facial nerve fascicle are involved the patient may also develop a peripheral facial palsy. This combination of one-and-ahalf syndrome and ipsilateral facial palsy is known as eight-and-a-half syndrome (11⁄2 1 7 5 81⁄2 ) and was first described by Eggenberger3 in 1998. The main causes for both syndromes are brain stem stroke, multiple sclerosis, brain stem tumors, and arteriovenous malformations.4 In 2001, Utku et al5 documented a case of a patient with bilateral persistent horizontal gaze palsy and bilateral facial palsy caused by pontine infarction. This case was similar to the 2005 report of Bae and Song6 of a 67-yearold diabetic man with one-and-a-half syndrome with bilateral facial nerve palsies. In 2007, Lee et al7 also described a new variant of these syndromes in an elderly patient with not only bilateral facial palsy but also complete loss of vertical saccades and pursuit with bilateral horizontal gaze palsy. Herein, we present another unusual case of pontine infarction. Our patient, similar
A.C. FELICIO ET AL.
246 5
7
to those of Utku et al and Lee et al, also developed bilateral horizontal gaze palsy but peripheral facial paralysis was unilateral in our case and bilateral in their cases. We believe one-and-a-half syndrome, eight-and-a-half syndrome, the case of Bae and Song,6 the case of Utku et al,5 our case, and the case of Lee et al7 are a part of the same continuum involving the pontine tegmentum with vascular lesions situated unilaterally, bilaterally, and with or without the involvement of the facial nerve fascicle. In our case, the exquisite asymmetric anterior extension of the vascular lesion situated at the tegmentum and, therefore, reaching the right facial nerve fascicle is probably responsible for the syndrome we are proposing. In conclusion, alongside one-and-a-half and eight-anda-half syndromes, treating physicians should be also aware of new variants of these disorders such as the one we just described with peculiar involvement of bilateral horizontal gaze.
References 1. Kumral E, Bayulkem G, Evyapan D. Clinical spectrum of pontine infarction: Clinical-MRI correlations. J Neurol 2002;249:1659-1670. 2. Fisher CM. Some neuro-ophthalmological observations. J Neurol Neurosurg Psychiatry 1967;30:383-392. 3. Eggenberger ER. Eight-and-a-half syndrome: One-and-ahalf syndrome plus cranial nerve VII palsy. Neuroophthalmology 1998;18:114-116. 4. Nandhagopal R, Krishnamoorthy SG. Eight-and-a-half syndrome. J Neurol Neurosurg Psychiatry 2006;77:463. 5. Utku U, Celik Y, Balci K. Bilaterally persistent horizontal gaze palsy and facial palsy caused by pontine infarction. J Stroke Cerebrovasc Dis 2001;10:242-243. 6. Bae JS, Song HK. One-and-a-half syndrome with facial diplegia: The 151⁄2 syndrome? J Neuroophthalmol 2005;25:52-53. 7. Lee E, Kim JS, Kim JS, et al. A small dorsal pontine infarction presenting with total gaze palsy including vertical saccades and pursuit. J Clin Neurol 2007;3:208-211.
Bilateral Horizontal Gaze Palsy with Unilateral Peripheral Facial Paralysis Caused by Pontine Tegmentum Infarction Andre C. Felicio, MD, Denis B. Bichuetti, MD, Luis F. Marin, MD, William A. C. dos Santos, MD, and Clecio Godeiro-Junior, MD
Clinical features of pontine infarction depend on the topography of vascular lesion and most remarkably sometimes the same topographic region can lead to different clinical syndromes (e.g., dorsal pontine tegmentum). In this report we describe an elderly patient with acute dorsal pontine infarction leading to a unique syndrome of bilateral horizontal gaze palsy and unilateral peripheral facial paralysis. We propose that this syndrome could be included as a part of a continuum that involves one-and-a-half syndrome, eight-and-a-half syndrome, and other variants of pontine tegmentum infarction. Key Words: One-and-a-half syndrome—eight-and-a-half syndrome—bilateral horizontal gaze palsy—peripheral facial paralysis—pontine infarction. Ó 2009 by National Stroke Association
Tegmental pontine infarctions are unusual and may lead to a range of neuro-ophthalmologic findings such as gaze palsies, diplopia, internuclear ophthalmoplegia, nystagmus, and abducens palsy.1 Herein, we present a unique case of acute pontine infarction leading to a combination of bilateral horizontal gaze palsy and unilateral peripheral facial palsy. In addition, we also discuss the relationship between this case and the one-and-a-half and eight-and-a-half syndromes. A 73-year-old white man was admitted with a 15-minute history of sudden-onset slurred speech and right facial weakness. He was on glucoformin (850 mg twice a day) and aspirin (200 mg/day) for diabetes and did not smoke cigarettes or drink alcohol. Family history was unremarkable. At the emergency department his From the Neurology Division, Hospital e Maternidade Sa˜o Camilo, Pompe´ia, Sao Paulo, Brazil. Received September 2, 2008; revision received October 17, 2008; accepted October 24, 2008. Address correspondence to Andre C. Felicio, MD, Bosque da Saude 834/193, Postal Code 04142-081, Sao Paulo, SP, Brazil. E-mail: [email protected]. 1052-3057/$—see front matter Ó 2009 by National Stroke Association doi:10.1016/j.jstrokecerebrovasdis.2008.10.008
244
heart rate was 100 beats/min, blood pressure was 178 3 96 mm Hg, and glucose was 250 mg/dL. The patient was alert, fully oriented, and collaborative. Pupils were symmetric (2 mm) and reactive to light. No ocular bobbing was found. There was a bilateral horizontal gaze palsy with preserved vertical saccades (Fig 1) associated to right peripheral facial paralysis. The oculocephalic reflexes were absent bilaterally. Despite his attentiveness and orientation, speech was compromised with a moderate dysarthria without aphasia. He scored 7 on the National Institutes of Health Stroke Scale. Brain computed tomography was performed and no abnormalities were found. The patient was excluded from venous thrombolytic recombinant tissue plasminogen activator because of recent leg bypass vascular surgery. Brain magnetic resonance imaging showed no alteration at 1.5 T with T1-, T2-, fluid-attenuated inversion recovery, and echocardiographic-gradient-weighted images. Diffusion-weighted image showed a small but irregular restricted diffusion area on the midline pontine tegmentum with a correspondent hypointense lesion on the apparent diffusion coefficient map (Fig 2, A and B). Magnetic resonance angiography showed vascular irregularities on large vessels and a significant stenosis on the middle part of the left posterior cerebral artery (Fig 2, C). The patient was
Journal of Stroke and Cerebrovascular Diseases, Vol. 18, No. 3 (May-June), 2009: pp 244-246
PONTINE TEGMENTUM INFARCTION
245
Figure 2. Brain magnetic resonance imaging on diffusion-weighted sequence (A) showing hyperintense lesion at dorsal pontine tegmentum with correspondent hyposignal on attenuated diffusion coefficient map (B). Note significant irregularities of arteries from posterior circulation and stenosis of left posterior cerebral artery. Stenosis, which is obviously not related to patient’s infarction, shows diffuse disease of cerebral arteries (C).
Figure 1. (A) Patient at primary position gaze (P). (B and C) Note normal vertical eye movements. (D and E) Note bilateral horizontal gaze palsy. Arrows indicate direction of gaze shifts. Senile arch is seen around patient’s cornea. Nasoenteral tube is placed. L, Left; R, right.
admitted to the intensive care department for conservative stroke treatment and later discharged from our hospital with a modified Rankin scale score of 2.
Discussion The ‘‘one-and-a-half syndrome’’ was coined by Fisher2 in 1967 and is characterized by an ipsilateral horizontal gaze palsy to one side and a restriction for adduction to the other side (1 1 1⁄2 5 11⁄2 ). Lesion is situated on the
paramedian pontine reticular formation to the side of the restricted adducted eye. When the fibers of the facial nerve fascicle are involved the patient may also develop a peripheral facial palsy. This combination of one-and-ahalf syndrome and ipsilateral facial palsy is known as eight-and-a-half syndrome (11⁄2 1 7 5 81⁄2 ) and was first described by Eggenberger3 in 1998. The main causes for both syndromes are brain stem stroke, multiple sclerosis, brain stem tumors, and arteriovenous malformations.4 In 2001, Utku et al5 documented a case of a patient with bilateral persistent horizontal gaze palsy and bilateral facial palsy caused by pontine infarction. This case was similar to the 2005 report of Bae and Song6 of a 67-yearold diabetic man with one-and-a-half syndrome with bilateral facial nerve palsies. In 2007, Lee et al7 also described a new variant of these syndromes in an elderly patient with not only bilateral facial palsy but also complete loss of vertical saccades and pursuit with bilateral horizontal gaze palsy. Herein, we present another unusual case of pontine infarction. Our patient, similar
A.C. FELICIO ET AL.
246 5
7
to those of Utku et al and Lee et al, also developed bilateral horizontal gaze palsy but peripheral facial paralysis was unilateral in our case and bilateral in their cases. We believe one-and-a-half syndrome, eight-and-a-half syndrome, the case of Bae and Song,6 the case of Utku et al,5 our case, and the case of Lee et al7 are a part of the same continuum involving the pontine tegmentum with vascular lesions situated unilaterally, bilaterally, and with or without the involvement of the facial nerve fascicle. In our case, the exquisite asymmetric anterior extension of the vascular lesion situated at the tegmentum and, therefore, reaching the right facial nerve fascicle is probably responsible for the syndrome we are proposing. In conclusion, alongside one-and-a-half and eight-anda-half syndromes, treating physicians should be also aware of new variants of these disorders such as the one we just described with peculiar involvement of bilateral horizontal gaze.
References 1. Kumral E, Bayulkem G, Evyapan D. Clinical spectrum of pontine infarction: Clinical-MRI correlations. J Neurol 2002;249:1659-1670. 2. Fisher CM. Some neuro-ophthalmological observations. J Neurol Neurosurg Psychiatry 1967;30:383-392. 3. Eggenberger ER. Eight-and-a-half syndrome: One-and-ahalf syndrome plus cranial nerve VII palsy. Neuroophthalmology 1998;18:114-116. 4. Nandhagopal R, Krishnamoorthy SG. Eight-and-a-half syndrome. J Neurol Neurosurg Psychiatry 2006;77:463. 5. Utku U, Celik Y, Balci K. Bilaterally persistent horizontal gaze palsy and facial palsy caused by pontine infarction. J Stroke Cerebrovasc Dis 2001;10:242-243. 6. Bae JS, Song HK. One-and-a-half syndrome with facial diplegia: The 151⁄2 syndrome? J Neuroophthalmol 2005;25:52-53. 7. Lee E, Kim JS, Kim JS, et al. A small dorsal pontine infarction presenting with total gaze palsy including vertical saccades and pursuit. J Clin Neurol 2007;3:208-211.