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Benedikt and “plus–minus lid” syndromes arising from posterior cerebral artery branch occlusion
Journal of the Neurological Sciences 228 (2005) 105 – 107 www.elsevier.com/locate/jns
Short communication
Benedikt and bplus–minus lidQ syndromes arising from posterior cerebral artery branch occlusion Gqlden Akdala,*, KqrYad Kutluka, Sqleyman Menb, Erdem Yakaa a
Departments of Neurology, Faculty of Medicine, Dokuz Eylu¨l University, 35340, I˙nciralti, I˙zmir, Turkey Departments of Radiology, Faculty of Medicine, Dokuz Eylu¨l University, 35340, I˙nciralti, I˙zmir, Turkey
b
Received 18 May 2004; received in revised form 14 September 2004; accepted 15 September 2004
Abstract A 53-year-old man was admitted with diplopia, right ptosis, left lid retraction, mild left sided weakness and involuntary movements. Neurological examination revealed plus–minus lid and Benedikt’s syndromes together. Magnetic resonance imaging (MRI) showed two distinct mesencephalic infarctions in territories of paramedian and pedincular perforating arteries. Magnetic resonance angiography (MRA) showed severe stenosis of proximal segment of the right posterior cerebral artery (PCA). Rare clinical presentations such as Benedikt and plus–minus syndromes can be seen together and be due to stenosis of the posterior cerebral artery with specific regional infarctions. D 2004 Elsevier B.V. All rights reserved. Keywords: Benedikt; Plus–minus lid syndromes; Occlusion
1. Introduction Mesencephalic infarctions, Benedikt and bplus–minus lidQ syndromes arising from posterior cerebral artery (PCA) branch occlusion present most commonly as part of ischemic lesions in the posterior cerebral circulation; isolated mesencephalic infarctions are rare [1,2]. Moreover, symptomatic stenosis of the posterior cerebral artery (PCA) causing midbrain infarctions has rarely been reported. Mesencephalic lesions cause various neuroophthalmologic syndromes especially nuclear oculomotor and fasicular syndromes with or without movement disorders and motor deficits [3–5]. Unilateral lid retraction with contralateral ptosis (bplus–minus lidQ syndrome) is a very rare neuroopthtalmologic presentation occurring with mesencephalic lesions [6,7]. Using advanced imaging technique, it is now possible to correlate lesion location with carefully documented clinical * Corresponding author. Tel.: +90 232 412 40 59; fax: +90 232 277 7721. E-mail address: [email protected] (G. Akdal). 0022-510X/$ - see front matter D 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2004.09.029
findings. The case presented here has unique clinical features of Benedikt and plus–minus lid syndromes together; which was not previously documented, and the detailed neuroradiological evaluations revealed the occlusions of penetrating arteries secondary to the stenosis of PCA.
2. Case report A 53-year-old man, was admitted 10 days after onset of diplopia, left-sided weakness and involuntary movements on his left arm. He had a history of uncontrolled hypertension for 5 years. Neurological examination revealed ptosis; mydriasis; and limited elevation, depression and adduction in the right eye; as well as marked retraction of the left eyelid (Fig. 1). There was no change of retraction when the ptotic eyelid was raised showing that the retraction was not the result of compensation for ptotis. These findings were interpreted as fascicular third nerve palsy and plus– minus syndrome. There was mild left hemiparesis, and rubral tremor was observed in the left arm. Rubral tremor, which is a characteristic component of Benedikt syndrome,
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G. Akdal et al. / Journal of the Neurological Sciences 228 (2005) 105–107
Fig. 1. Retraction of the left eye, and mild ptosis of the right eye (a), left gaze (b), right gaze (c), upward gaze (d), downward gaze (e). Adduction, elevation and depression of the right eye are limited.
Fig. 2. Axial T2-weighted image (turbo spin echo, TR: 2200 ms, TE: 120 ms, 512512 matrix) through the cerebral peduncles shows two adjacent hyperintense lesions in the right cerebral peduncle secondary to occlusions of PMA and PPA.
Fig. 3. (a) TOF MRA of circle of Willis (flip angle: 258, TR: 35 ms, TE: 22 ms, 512512 matrix, slice thickness: 1.2 mm). The P1 segment of the right posterior cerebral artery is diffusely narrowed with superimposed stenotic segments (arrow). No signal is seen distal to P1 segment. (b) Source image of the MR angiography. Intraluminal signal, although lower than the other arteries, is seen within the right posterior cerebral artery (arrows). The angiographic reformatted image in the panel a was produced with the maximum intensity projection algorithm. Because the signal intensity within the right P2 and its distal part is below the threshold level used in this algorithm, this artery is not visualized in the angiograpic image and thus may be misinterpreted as occluded.
G. Akdal et al. / Journal of the Neurological Sciences 228 (2005) 105–107
is a combination of rest, postural and kinetic tremor and proximal involvement is more prominent. Magnetic resonance imaging (MRI) showed two foci of increased signal intensity in the right cerebral peduncle on T2- and diffusion-weighted images (Fig. 2). These findings indicated recent subacute infarcts with petechial hemorrhagic transformation. Time of flight (TOF) magnetic resonance angiography (MRA) revealed a very thin and irregular proximal segment of the right posterior cerebral artery (Fig. 3a). Although the artery distal to this segment was not seen on the angiographic images, it was visualized with relatively low signal on the angiography source images, which suggested slow flow (Fig. 3b). There was no carotid stenosis on MRA. Electrocardiography showed normal sinus rhythm. Other routine haematological and metabolic investigations were normal, and the ischemic stroke was categorized as secondary to small vessel occlusion. Antihypertensive therapy (perindopril 4 mg/day) and acetyl salicylic acid (300 mg/day) was started. He had mild disability at discharge, but was independent for his daily living activities.
3. Discussion The patient described here, presented two simultaneous mesencephalic syndromes, Benedikt’s and plus–minus lid syndromes, caused by isolated mesencephalic infarctions secondary to occlusions of multiple small branches originating from the distal basilar artery and the proximal PCA, paramedian (PMA) and pedincular perforating (PPA) arteries. MRI showed two distinct mesencephalic infarctions in the territories of PMA and PPA; the locations of which were consistent with the clinical findings of our patient. Severe stenosis of the P1 segment of the right posterior cerebral artery, shown on MRA, was thought to be responsible for the brain stem infarctions. Ischemic events in PCA territory are usually caused by occlusion of the proximal segment and result in visual or sensorial symptoms [1]. The stenosis of PCA as a cause of isolated mesencephalic lesions has rarely been reported. Duncan and Weindling [4] presented a case with brain stem symptoms secondary to proximal PCA stenosis. The clinical presentation was Benedikt syndrome and MRI showed a midbrain infarction with the involvement of red nucleus, cerebral peduncle, and oculomotor fascicles. The authors underlined that their case was unique because only brain stem symptoms were present. In addition to many similarities with Duncan and Weindling’s case, our patient presented an unusual mesencephalic syndrome bplus–minus lidQ. This rare condition is characterized by ptosis on one side and primary eyelid retraction on the other [6,7].
107
Unilateral paramedian lesions, usually infarctions, dorsal and rostral to the red nucleus in the area of the nucleus of posterior comissure, extending ventrocaudally to the fascicle of the oculomotor nerve on the ptotic side cause this condition [8]. The paramedian midbrain infarction due to PMA occlusion correlated with the neuroophthalmologic findings in our patient. This lesion also affected nucleus ruber and caused Benedikt syndrome. The second mesencephalic infarction within the PPA territory explains the mild pyramidal findings in our patient, since PPA supplies blood to the corticospinal tract in this area. Previous case reports of Benedikt and plus–minus lid syndromes did not provide information on the specific vascular etiology. The stenosis of PCA in our patient could be demonstrated noninvasively by MRA. The initial nonvisualization of the P2 segment has been interpreted as pseudo-occlusion, since there was neither imaging nor clinical findings of total occlusion of PCA. Moreover, source MRA images showed intravascular signal distal to the P1 segment. It is a well-known mechanism that basilar branch infarcts may occur due to basiler artery disease, as well as deep middle cerebral artery (MCA) infarcts due to MCA disease. However, the same mechanism concerning PCA has not been clearly demonstrated. The detailed neuroradiological evaluation of our patient has provided a better correlation between the clinical presentation and anatomic locations; and established the evidence that small brain stem infarctions could arise from focal disease of posterior cerebral artery.
References [1] Martin PJ, Chang HM, Caplan LR. Midbrain infarction: associations and aetiologies in the New England Medical Center Posterior Circulation Registry. J Neurol Neurosurg Psychiatry 1998;64:392 – 5. [2] Kumral E, Bayulkem G, Akyol A, Yunten N, Sirin H, Sag˘duyu A. Mesencephalic and associated posterior circulation infarcts. Stroke 2002;33:2224 – 31. [3] Akdal G, Baklan B, ErYahin Y, C ¸ akmakc¸i H, Yurtseven T, I˙diman F. Mesencephalic cavernoma causing reversible nuclear third nerve palsy and obstructive hydrocephalus. Neuro-Ophthalmology 2001; 26:127 – 32. [4] Duncan GW, Weindling SM. Posterior cerebral artery stenosis with midbrain infarction. Stroke 1995;26:900 – 2. [5] Bogousslavsky J, Maeder P, Regli F, Meuli R. Pure midbrain infarction: clinical syndromes, MRI, and etiologic patterns. Neurology 1994;44:2032 – 40. [6] Gaymard B, Lofilte C, Gelot A, de Toffol B. Plus–minus lid syndrome. J Neurol Neurosurg Psychiatry 1992;55:846 – 8. [7] C ¸ elebisoy N, Secil Y, Yqceyar N, Ertekin C. Unilateral lid retraction with contralateral oculomotor paresis. Neuro-Ophthalmology 1999;21:165 – 70. [8] Schmidtke K, Bqttner-Ennever JA. Nervous control of eyelid function. A review of clinical, experimental and pathological data. Brain 1992;115:227 – 47.
Short communication
Benedikt and bplus–minus lidQ syndromes arising from posterior cerebral artery branch occlusion Gqlden Akdala,*, KqrYad Kutluka, Sqleyman Menb, Erdem Yakaa a
Departments of Neurology, Faculty of Medicine, Dokuz Eylu¨l University, 35340, I˙nciralti, I˙zmir, Turkey Departments of Radiology, Faculty of Medicine, Dokuz Eylu¨l University, 35340, I˙nciralti, I˙zmir, Turkey
b
Received 18 May 2004; received in revised form 14 September 2004; accepted 15 September 2004
Abstract A 53-year-old man was admitted with diplopia, right ptosis, left lid retraction, mild left sided weakness and involuntary movements. Neurological examination revealed plus–minus lid and Benedikt’s syndromes together. Magnetic resonance imaging (MRI) showed two distinct mesencephalic infarctions in territories of paramedian and pedincular perforating arteries. Magnetic resonance angiography (MRA) showed severe stenosis of proximal segment of the right posterior cerebral artery (PCA). Rare clinical presentations such as Benedikt and plus–minus syndromes can be seen together and be due to stenosis of the posterior cerebral artery with specific regional infarctions. D 2004 Elsevier B.V. All rights reserved. Keywords: Benedikt; Plus–minus lid syndromes; Occlusion
1. Introduction Mesencephalic infarctions, Benedikt and bplus–minus lidQ syndromes arising from posterior cerebral artery (PCA) branch occlusion present most commonly as part of ischemic lesions in the posterior cerebral circulation; isolated mesencephalic infarctions are rare [1,2]. Moreover, symptomatic stenosis of the posterior cerebral artery (PCA) causing midbrain infarctions has rarely been reported. Mesencephalic lesions cause various neuroophthalmologic syndromes especially nuclear oculomotor and fasicular syndromes with or without movement disorders and motor deficits [3–5]. Unilateral lid retraction with contralateral ptosis (bplus–minus lidQ syndrome) is a very rare neuroopthtalmologic presentation occurring with mesencephalic lesions [6,7]. Using advanced imaging technique, it is now possible to correlate lesion location with carefully documented clinical * Corresponding author. Tel.: +90 232 412 40 59; fax: +90 232 277 7721. E-mail address: [email protected] (G. Akdal). 0022-510X/$ - see front matter D 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2004.09.029
findings. The case presented here has unique clinical features of Benedikt and plus–minus lid syndromes together; which was not previously documented, and the detailed neuroradiological evaluations revealed the occlusions of penetrating arteries secondary to the stenosis of PCA.
2. Case report A 53-year-old man, was admitted 10 days after onset of diplopia, left-sided weakness and involuntary movements on his left arm. He had a history of uncontrolled hypertension for 5 years. Neurological examination revealed ptosis; mydriasis; and limited elevation, depression and adduction in the right eye; as well as marked retraction of the left eyelid (Fig. 1). There was no change of retraction when the ptotic eyelid was raised showing that the retraction was not the result of compensation for ptotis. These findings were interpreted as fascicular third nerve palsy and plus– minus syndrome. There was mild left hemiparesis, and rubral tremor was observed in the left arm. Rubral tremor, which is a characteristic component of Benedikt syndrome,
106
G. Akdal et al. / Journal of the Neurological Sciences 228 (2005) 105–107
Fig. 1. Retraction of the left eye, and mild ptosis of the right eye (a), left gaze (b), right gaze (c), upward gaze (d), downward gaze (e). Adduction, elevation and depression of the right eye are limited.
Fig. 2. Axial T2-weighted image (turbo spin echo, TR: 2200 ms, TE: 120 ms, 512512 matrix) through the cerebral peduncles shows two adjacent hyperintense lesions in the right cerebral peduncle secondary to occlusions of PMA and PPA.
Fig. 3. (a) TOF MRA of circle of Willis (flip angle: 258, TR: 35 ms, TE: 22 ms, 512512 matrix, slice thickness: 1.2 mm). The P1 segment of the right posterior cerebral artery is diffusely narrowed with superimposed stenotic segments (arrow). No signal is seen distal to P1 segment. (b) Source image of the MR angiography. Intraluminal signal, although lower than the other arteries, is seen within the right posterior cerebral artery (arrows). The angiographic reformatted image in the panel a was produced with the maximum intensity projection algorithm. Because the signal intensity within the right P2 and its distal part is below the threshold level used in this algorithm, this artery is not visualized in the angiograpic image and thus may be misinterpreted as occluded.
G. Akdal et al. / Journal of the Neurological Sciences 228 (2005) 105–107
is a combination of rest, postural and kinetic tremor and proximal involvement is more prominent. Magnetic resonance imaging (MRI) showed two foci of increased signal intensity in the right cerebral peduncle on T2- and diffusion-weighted images (Fig. 2). These findings indicated recent subacute infarcts with petechial hemorrhagic transformation. Time of flight (TOF) magnetic resonance angiography (MRA) revealed a very thin and irregular proximal segment of the right posterior cerebral artery (Fig. 3a). Although the artery distal to this segment was not seen on the angiographic images, it was visualized with relatively low signal on the angiography source images, which suggested slow flow (Fig. 3b). There was no carotid stenosis on MRA. Electrocardiography showed normal sinus rhythm. Other routine haematological and metabolic investigations were normal, and the ischemic stroke was categorized as secondary to small vessel occlusion. Antihypertensive therapy (perindopril 4 mg/day) and acetyl salicylic acid (300 mg/day) was started. He had mild disability at discharge, but was independent for his daily living activities.
3. Discussion The patient described here, presented two simultaneous mesencephalic syndromes, Benedikt’s and plus–minus lid syndromes, caused by isolated mesencephalic infarctions secondary to occlusions of multiple small branches originating from the distal basilar artery and the proximal PCA, paramedian (PMA) and pedincular perforating (PPA) arteries. MRI showed two distinct mesencephalic infarctions in the territories of PMA and PPA; the locations of which were consistent with the clinical findings of our patient. Severe stenosis of the P1 segment of the right posterior cerebral artery, shown on MRA, was thought to be responsible for the brain stem infarctions. Ischemic events in PCA territory are usually caused by occlusion of the proximal segment and result in visual or sensorial symptoms [1]. The stenosis of PCA as a cause of isolated mesencephalic lesions has rarely been reported. Duncan and Weindling [4] presented a case with brain stem symptoms secondary to proximal PCA stenosis. The clinical presentation was Benedikt syndrome and MRI showed a midbrain infarction with the involvement of red nucleus, cerebral peduncle, and oculomotor fascicles. The authors underlined that their case was unique because only brain stem symptoms were present. In addition to many similarities with Duncan and Weindling’s case, our patient presented an unusual mesencephalic syndrome bplus–minus lidQ. This rare condition is characterized by ptosis on one side and primary eyelid retraction on the other [6,7].
107
Unilateral paramedian lesions, usually infarctions, dorsal and rostral to the red nucleus in the area of the nucleus of posterior comissure, extending ventrocaudally to the fascicle of the oculomotor nerve on the ptotic side cause this condition [8]. The paramedian midbrain infarction due to PMA occlusion correlated with the neuroophthalmologic findings in our patient. This lesion also affected nucleus ruber and caused Benedikt syndrome. The second mesencephalic infarction within the PPA territory explains the mild pyramidal findings in our patient, since PPA supplies blood to the corticospinal tract in this area. Previous case reports of Benedikt and plus–minus lid syndromes did not provide information on the specific vascular etiology. The stenosis of PCA in our patient could be demonstrated noninvasively by MRA. The initial nonvisualization of the P2 segment has been interpreted as pseudo-occlusion, since there was neither imaging nor clinical findings of total occlusion of PCA. Moreover, source MRA images showed intravascular signal distal to the P1 segment. It is a well-known mechanism that basilar branch infarcts may occur due to basiler artery disease, as well as deep middle cerebral artery (MCA) infarcts due to MCA disease. However, the same mechanism concerning PCA has not been clearly demonstrated. The detailed neuroradiological evaluation of our patient has provided a better correlation between the clinical presentation and anatomic locations; and established the evidence that small brain stem infarctions could arise from focal disease of posterior cerebral artery.
References [1] Martin PJ, Chang HM, Caplan LR. Midbrain infarction: associations and aetiologies in the New England Medical Center Posterior Circulation Registry. J Neurol Neurosurg Psychiatry 1998;64:392 – 5. [2] Kumral E, Bayulkem G, Akyol A, Yunten N, Sirin H, Sag˘duyu A. Mesencephalic and associated posterior circulation infarcts. Stroke 2002;33:2224 – 31. [3] Akdal G, Baklan B, ErYahin Y, C ¸ akmakc¸i H, Yurtseven T, I˙diman F. Mesencephalic cavernoma causing reversible nuclear third nerve palsy and obstructive hydrocephalus. Neuro-Ophthalmology 2001; 26:127 – 32. [4] Duncan GW, Weindling SM. Posterior cerebral artery stenosis with midbrain infarction. Stroke 1995;26:900 – 2. [5] Bogousslavsky J, Maeder P, Regli F, Meuli R. Pure midbrain infarction: clinical syndromes, MRI, and etiologic patterns. Neurology 1994;44:2032 – 40. [6] Gaymard B, Lofilte C, Gelot A, de Toffol B. Plus–minus lid syndrome. J Neurol Neurosurg Psychiatry 1992;55:846 – 8. [7] C ¸ elebisoy N, Secil Y, Yqceyar N, Ertekin C. Unilateral lid retraction with contralateral oculomotor paresis. Neuro-Ophthalmology 1999;21:165 – 70. [8] Schmidtke K, Bqttner-Ennever JA. Nervous control of eyelid function. A review of clinical, experimental and pathological data. Brain 1992;115:227 – 47.