The clinical variability of Wallenberg's syndrome

The dorso-lateral medullary syndrome (Wallenberg's syndrome) is produced by infarction of a wedge of lateral medulla posterior to the inferior olivary nucleus and is usually caused by vertebral artery occlusion. Ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion is rather rare and the anatomical structure responsible is still uncertain. Here we describe two patients presenting with ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion. In one the stroke affected the dorso-lateral aspect of the medulla, in the other more lateral aspects of the medulla were involved. Our data suggest that ipsilateral axial lateropulsion may be caused by lesions of different topography involving either the vestibular nuclei, the cerebellar peduncle or the spinocerebellar tracts.     

Rubral lateropulsion due to vertebral artery dissection in a patient with Klippel-Feil syndrome

BACKGROUND: Neurologic deficits in patients with Klippel-Feil syndrome usually are attributed to direct compression of neuronal structures or hypoperfusion secondary to compression of the vertebral arteries by bony abnormalities. OBJECTIVE: To describe a 38-year-old woman with known Klippel-Feil syndrome who developed lateropulsion. RESULTS: The results of magnetic resonance imaging were consistent with rubrothalamic stroke. The cerebral angiogram confirmed vertebral artery dissection at the level of her previously observed bony abnormality. CONCLUSIONS: Hypermobility adjacent to fused vertebrae subjects the vertebral artery to increased shear forces. Thus, Klippel-Feil syndrome may be a predisposing factor for vertebral artery dissection. Moreover, to our knowledge, this case represents the second known case of rubral lateropulsion.     

Lateral gaze disturbance in a case of Wallenberg's syndrome

A 63-year-old Caucasian man was admitted for Wallenberg's syndrome following a left vertebral artery thrombosis. In addition to the classical symptoms, an axial lateropulsion to the left and ocular motor disorders (vertical diplopia, tonic deviation of the gaze to the left, skew deviation and horizonto-rotatory nystagmus) were present. These clinical signs are unusual, but in common Wallenberg's syndrome, neurophysiological tests often reveal slight abnormalities of oculomotor function: impairment of jerks, skew deviation, lateral deviation of the gaze in darkness. Interruption of cerebellar pathways is thought to be the cause of these symptoms. Their existence does not seem to change the outcome of these cases.     

Axial lateropulsion as a sole manifestation of lateral medullary infarction: A clinical variant related to rostral–dorsolateral lesion

Abstract

A 63-year-old woman presented with an isolated axial lateropulsion as a sole manifestation of lateral medullary infarction. She had no vertigo, nystagmus, dysphagia, hiccup, facial/hemisensory loss, Horner syndrome, and limb ataxia. Brain MRI showed a small infarct selectively involving the most dorsolateral portion of the rostral medulla. This patient illustrates that lateral medullary infarction may present as an isolated lateropulsion. The possible mechanism of an isolated lateropulsion is described.     

双侧椎动脉/基底动脉重度狭窄或闭塞的急性后循环梗死患者临床特点及预后分析

目的总结分析双侧椎动脉/基底动脉重度狭窄或闭塞的急性后循环梗死患者的临床特点和预后。方法收集急性后循环梗死且伴有双侧椎动脉/基底动脉重度狭窄或闭塞的住院患者资料,分析其血管危险因素、起始症状、治疗及病情演变情况,并随访90 d预后,以mRS2分定义为预后不良。结果共纳入28例患者,最常见的起始症状为孤立性头晕或眩晕(57.1%)。53.6%的患者出现病情加重,加重≥2次9例(32.1%),预后不良15例(53.6%)。预后不良组高血压3级、病情加重、加重≥2次比例明显高于预后良好组(P0.05)。病情加重≥2次组高血压3级、高脂血症、孤立性头晕或眩晕起病、预后不良和死亡比例明显高于加重2次组(P0.05)。基底动脉不显影组死亡率(75%)明显高于显影组(4.2%)(P0.05)。结论双侧椎动脉/基底动脉重度狭窄或闭塞的急性后循环梗死患者多以孤立性头晕或眩晕起病;孤立性头晕或眩晕起病和高血压3级患者病情容易加重;病情反复加重患者预后不良比例高,死亡率高;基底动脉不显影患者死亡率高。     

Axial lateropulsion as a sole manifestation of lateral medullary infarction: a clinical variant related to rostral-dorsolateral lesion

A 63-year-old woman presented with an isolated axial lateropulsion as a sole manifestation of lateral medullary infarction. She had no vertigo, nystagmus, dysphagia, hiccup, facial/hemisensory loss, Horner syndrome, and limb ataxia. Brain MRI showed a small infarct selectively involving the most dorsolateral portion of the rostral medulla. This patient illustrates that lateral medullary infarction may present as an isolated lateropulsion. The possible mechanism of an isolated lateropulsion is described.     

Isolated body lateropulsion caused by a lesion of the rostral vermis

A 66-year-old man presented with axial lateropulsion, exhibiting sudden falling as the sole clinical sign of cerebellar infarction in the territory of the medial branch of the superior cerebellar artery (mSCA). A brain MRI showed acute infarction selectively involving the centralis of the rostral vermis. This patient illustrates that cerebellar infarction in the territory of the mSCA can present as an isolated body lateropulsion. The possible mechanism of an isolated body lateropulsion is considered.     

A Case of Wallenberg's Syndrome Presenting with Spontaneous Thrombosis of a Vertebral Artery Aneurysm

Complete spontaneous thrombosis of intracranial aneurysms is uncommon. Although this type of thrombosis is largely asymptomatic, in rare cases it can be accompanied by parent artery occlusion and ischemic stroke. There are limited reports of complete thrombosis of an unruptured aneurysm of the internal carotid artery and middle cerebral artery. Furthermore, there are no reports of occlusion of the vertebral artery caused by thrombosis of an aneurysm. The mechanisms of spontaneous thrombosis are not established. However, aneurysm morphology, arteriosclerosis, and stagnation of aneurysm flow have been suggested. Herein, we present a novel case of Wallenberg's syndrome caused by a fusiform aneurysm in which complete thrombosis of the proximal vertebral artery occurred. We discuss the mechanisms of thrombosis caused by an unruptured aneurysm, which may be useful for managing such patients who present with transient ischemic attacks.     

Otolith manifestations in Wallenberg syndrome

Central vestibular pathways issuing from the otolith may be involved in Wallenberg syndrome, resulting in specific symptoms. These "otolith" symptoms are less well known than vestibular symptoms issuing from the canal. We report 15 patients with Wallenberg syndrome who had one or more clinical signs suggestive of otolith pathway involvement. Clinical examination looked for: Eleven patients presented ipsilesional skew deviation; ipsilesional tilt of subjective visual vertical was found in 8 patients; a room tilt illusion was described in 4 patients in either the frontal or the sagittal plane: 9 patients presented axial lateropulsion toward the lesioned side; 8 patients presented ipsilesional ocular lateropulsion, 6 of them in association with axial lateropulsion. Finally, 3 patients presented positional nystagmus evoked by head tilt in the roll or the pitch plane. Pathophysiology of these symptoms and evidence for an otolith pathway involvement are discussed.     

Atypical Wallenberg's syndrome due to spontaneous vertebral arterial dissection: case report]

We report here a case of atypical Wallenberg's syndrome due to spontaneous vertebral artery (VA) dissection. A 52-year-old woman was admitted to our department because of a sudden onset of left orbital pain. Emergency CT scan disclosed no evidence of intracranial hemorrhage. Neurological examination at the time of the current admission, showed dysphagia, left soft palate palsy, hoarseness, left Horner syndrome, hypalgesia with thermohypesthesia on the right side of her face, however, hypalgesia with thermohypesthesia on the right side of her body. The diagnosis of atypical Wallenberg's syndrome was based on the above findings. MR images disclosed the infarcted lesion at the left lateral medulla depicted as high-intensity on T2-weighted & FLAIR images. We carried out conservative treatment with antiplatelet & hemodilution therapies and the blood pressure control. Left vertebral angiograms obtained 18 days after the onset, showed the segmental severe stenosis of the VA between the ramification of the posterior inferior cerebellar artery (PICA) and the union of the VAs. In the venous phase, retention of contrast medium in the VA and the PICA was observed. The flow rate of the parent artery was decreased. We strongly suspected that her initial symptom of left orbital pain was due to dissection of the VA itself. Three-dimensional CT angiograms obtained 30 days after the onset, demonstrated the defect of the left VA between the ramification of the left PICA and the union of the VAs. Left vertebral angiograms obtained 36 days after the onset, showed the occlusion of the VA between the ramification of the PICA and the union of the VAs. The neurological findings gradually improved and the patient was discharged. Follow up left vertebral angiograms obtained 4 months & 16 months after the onset, revealed almost no changes of left VA occlusion.     

A case of vertebral dissecting aneurysm manifesting as subarachnoid hemorrhage following nuchal pain]

We report a case of subarachnoid hemorrhage (SAH) from vertebral dissecting aneurysm 4 days after first nuchal pain. The patient was a 46-year-old man with a sudden onset of nuchal pain. There were no obvious abnormalities detected on MR images in another hospital. Four days later, however, he was admitted to our hospital because of severe re-attack of nuchal pain. CT demonstrated moderate SAH and cerebral angiograms revealed right vertebral dissecting aneurysm. Proximal occlusion of the vertebral artery including its aneurysmal dilatation was performed using detachable coils. We strongly suspected that his initial symptom of nuchal pain was due to dissection of the vertebral artery itself, since the aneurysmal dilatation accompanied by intramural hematoma had been observed retrospectively in the initial MR imaging. The incidence of the vertebral dissecting aneurysm presenting with nuchal pain alone due to dissection is reported to be 7% in the literature. The prognosis of non-hemorrhagic vertebral dissecting aneurysm followed by delayed SAH is considered to be fatal. Therefore, careful investigations for differential diagnosis should be taken into account since the diagnostic possibility exists that non-hemorrhagic vertebral dissecting aneurysm would be manifested by a symptom of headache/nuchal pain alone.     

Infarction in the territory of the medial branch of the posterior inferior cerebellar artery.

We report 10 cases of cerebellar infarction in the territory of the medial branch of the posterior inferior cerebellar artery (mPICA). Axial sections on MRI through the middle of the medulla and the cerebellum showed the infarction as a triangular area with a dorsal base and a ventral apex directed towards the fourth ventricle. The infarct also involved the lateral and dorsal medulla when the mPICA supplied all or part of these regions. Three clinical patterns were observed: 1) pseudolabyrinthine signs with or without dysmetria and ataxia when the medulla was spared; marked axial lateropulsion was present in most cases; 2) complete or incomplete Wallenberg's syndrome, when the medulla was involved; 3) silent infarction. These syndromes are precisely those previously attributed to PICA occlusion without distinction of the branch involved. No alteration of consciousness was recorded and spontaneous recovery was the rule. Cerebellar infarction in the distribution of the mPICA can be regarded as a benign condition with a good prognosis.     

Rotational vertebral artery occlusion in a child with multiple strokes: a case-based update

Introduction  

Rotational vertebral artery occlusion (RVAO), sometimes known as “Bow hunter syndrome,” is an important and diagnostically challenging cause of posterior circulation stroke in children. It is caused by impingement of osseous and/or ligamentous structures on the vertebral artery.     

以双耳听力下降为首发症状的脑梗死临床观察

目的观察以双耳听力下降为首发症状的脑梗死患者,探讨其病灶分布特点及血管形态异常之间的关系。方法对3例以双耳听力下降为首发症状的脑梗死患者行头MR(包括T1WI、T2WI、DWI、MRA)、TCD、BAEP检查。结果 3例患者均为后循环多灶性梗死,最常累及的部位是小脑、脑桥,均有双侧椎动脉或基底动脉起始段严重狭窄或闭塞,双侧内听动脉均受累。结论以双耳听力下降为首发症状的脑梗死均符合多灶性后循环缺血的特点,血管形态异常是其重要病理基础和致病因素,预后差。     

A case of isolated nodulus infarction presenting as a vestibular neuritis

We reported a patient with cerebellar infarction who presented with purely isolated vertigo, ipsilesional spontaneous nystagmus, and contralesional axial lateropulsion without usual symptoms or signs of cerebellar dysfunction. An MRI of the brain showed a small left cerebellar infarct selectively involving the nodulus. A pure vestibular syndrome in our patient may be explained by ipsilateral involvement of nodulo-vestibular inhibitory projection to vestibular nucleus. Clinicians should be aware of the possibility of a nodulus infarction in patient with acute vestibular syndrome, even if the pattern of nystagmus and lateropulsion is typical of a vestibular neuritis.     

重度锁骨下动脉盗血综合征的侧支代偿及临床特点

目的探讨重度锁骨下动脉盗血综合征(SSS)的侧支代偿及临床特点。方法回顾性分析12例经全脑数字减影血管造影(DSA)证实为重度SSS患者的临床资料。结果 12例SSS患者主要临床表现为发作性头晕、视物旋转、跌倒等,活动同侧上肢后症状加重;DSA示左侧锁骨下动脉重度狭窄或闭塞10例,双侧锁骨下动脉重度狭窄2例;侧支代偿途径椎动脉-椎动脉-锁骨下动脉10例,颈内动脉-后交通动脉-大脑后动脉/基底动脉8例,颈外动脉-枕动脉下支-椎动脉肌支动脉-椎动脉-锁骨下动脉9例。8例患者行锁骨下动脉支架成形术后临床症状完全消失;2例患者因锁骨下动脉闭塞及对侧椎动脉中度狭窄而接受对侧椎动脉支架成形术,术后症状减轻;随访3个月～2.5年,症状无复发。结论重度SSS临床表现主要为发作性头晕,侧支代偿途径主要为椎动脉-椎动脉-锁骨下动脉,支架成形术可纠正血流动脉动力学紊乱,缓解临床症状。     

Vertebral artery stump syndrome

After occlusion of flow in an artery, further ischaemic episodes are not expected due to lack of a flow conduit to carry the embolus. In the carotid stump syndrome, ongoing ischaemic events may continue due to collateral flow via the external carotid artery. We report two patients presenting with posterior circulation strokes after documented vertebral artery occlusion, due to a vertebral stump syndrome. Their presentation, the pathophysiology of cervico-vertebral anastomoses and management are described.     

Correlation of anatomy and function in medulla oblongata infarction

Background:  A presentation of all aspects of the dorsolateral medulla oblongata syndrome is clinically very rare to find. In most cases patients present with fragmentary symptoms, e.g. ipsilateral axial lateropulsion, nystagmus, dysarthria, dysphagia or hemiataxia. However, the clinical presentation and lesion anatomy at the level of the medulla oblongata is still unsatisfactory. The aim of this study was to correlate the functional deficit with structural MRI-data.
Methods:  We included thirteen patients (eight male, five female, mean age 65.5) with medulla oblongata infarction with clinically predominant ipsilateral axial lateropulsion and correlated clinical with structural deficits.
Results:  Magnetic resonance imaging lesion mapping demonstrated ipsilateral axial lateropulsion to result from lesions of the spinocerebellar tract, the inferior cerebellar peduncle or the inferior vestibular nucleus. Nystagmus was associated with lesions of the inferior vestibular nucleus, dissociated sensory loss with the spinothalamic tract and hemiataxia with the spinocerebellar tract.
Conclusions:  Correlating dysfunction and lesion anatomy is a promising approach to enhance our knowledge on medulla oblongata topography.     

Combination of infarctions in the posterior inferior cerebellar artery and anterior spinal artery territories

After an episode of vasodilator-induced systemic hypotension, a 75-year-old man developed ocular lateropulsion to the right, left-side-dominant quadriparesis, loss of superficial sensation below C4 dermatome level, and anuresis. Magnetic resonance imaging (MRI) showed infarcts in the right cerebellar hemisphere (posterior inferior cerebellar artery territory) and the upper cervical cord (anterior spinal artery territory); the combination of posterior inferior cerebellar artery (PICA) and anterior spinal artery (ASA) infarcts has not been reported previously. Angiography revealed severe stenosis in the bilateral vertebral arteries. Hemodynamic hypoperfusion of the stenotic vertebral arteries may cause this unusual combination.     

Infarcts presenting with a combination of medial medullary and posterior inferior cerebellar artery syndromes

Cerebellar and medial medullary infarctions are well-known vertebrobasilar stroke syndromes. However, their development in a patient with distal vertebral artery occlusion has not been previously reported. A 49-year-old man with longstanding hypertension suddenly developed vertigo, right-sided Horner syndrome, and left-sided weakness. An MRI of the brain showed acute infarcts in the right inferior cerebellum (posterior inferior cerebellar artery territory) and the right upper medial medulla (direct penetrating branches of vertebral artery). Magnetic resonance angiogram showed occlusion of the distal vertebral artery on the right side. Atherothrombotic occlusion of the distal vertebral artery may cause this unusual combination of vertebrobasilar stroke.