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.

Wegener's Granulomatosis Presenting as Wallenberg Syndrome: A Case Report.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia. In physical examination she had a saddled nose, horner syndrome, soft palate paralysis, crossed hypoesthesia of face, and limbs and hemi-ataxia. Brain magnetic imaging revealed a left lateral medullary infarction and sinusitis confirmed by paranasal Sinus CT scans. Chest CT showed a cavitary mass. Laboratory findings were remarkable for anemia, elevated erythrocyte sedimentation rate, and C-reactive protein. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA)/anti-proteinase 3 was positive. Diagnosis of GPA was established and treatment was started. During 6-month follow-up improvement was satisfying and no relapses occurred. Medullary infarct is reported in few GPA patients, especially at presentation. Definite diagnosis is based on tissue biopsy. Although in context of extra CNS involvement and positive ANCA diagnosis can be made confidently. Treatment of choice in CNS involvement is not clear, corticosteroids and immunosuppressives seem effective. CNS involvement, especially stroke may present GPA or accompany extra CNS symptoms. Prompt diagnosis and treatment is essential.

Fatal Progression of Gorham-Stout Disease with Skull Base Osteomyelitis and Lateral Medullary Syndrome.

Gorham-Stout disease (GSD) is a rare condition in which spontaneous, progressive resorption of bone occurs. There are no previous reports of patients with fatal progression of GSD with skull base osteomyelitis (SBO) and lateral medullary syndrome (LMS). We present the case of a 27-year-old man diagnosed with GSD with involvement of the maxillofacial bones and skull base. The patient developed SBO; LMS resulted from progressive osteolysis, and the patient died of associated brainstem stroke. Careful follow-up with special emphasis on the early detection of intracranial complications is critical in patients presenting with progressive GSD with involvement of the skull base.

[Lateral medullary stroke with extracranial course of the postero- inferior cerebellar artery]. / Accidente cerebrovascular bulbar lateral con trayecto extracraneal de la arteria cerebelosa póstero-inferior.

We present two cases of lateral medullary stroke in subjects with extracranial trajectory of the postero-inferior cerebellar artery. Case 1: a 21-year-old male who presented ataxia and right dysmetria after cervical trauma in a rugby match. Case 2: 56-year-old woman, who started with vertigo and left hemiparesis after intense physical effort. In both cases, the angiographic studies showed an extracranial trajectory of the posterior inferior cerebellar artery. This vessel rarely originates below the foramen magnum, in close relationship with the first three cervical vertebrae and the atlanto-axial joint. At this level, it is exposed to mechanical damage causing dissection, such as direct trauma, abrupt cervical manipulation or prolonged cephalic extension. Therefore, this association should be considered in patients with stroke of the lateral region of the bulb and extracranial trajectory of the posterior-inferior cerebellar artery.

Accidente cerebrovascular bulbar lateral con trayecto extracraneal de la arteria cerebelosa póstero-inferior / Lateral medullary stroke with extracranial course of the postero- inferior cerebellar artery

Presentamos dos casos de accidente cerebrovascular en sujetos con trayecto extracraneal de la arteria cerebeloso póstero-inferior. Caso 1: varón de 21 años, quien presentó ataxia y dismetría derecha luego de un traumatismo cervical en un partido de rugby. Caso 2: mujer de 56 años, quien inició con vértigo y hemiparesia izquierda luego de esfuerzo físico intenso. En ambos casos, los estudios angiográficos mostraron un trayecto extracraneal de la arteria cerebelosa póstero-inferior. Este vaso raramente se origina por debajo del foramen magno, en relación cercana con las primeras tres vértebras cervicales y la articulación atlanto-axial. En este nivel, está expuesta a daño mecánico causante de disección, como por ejemplo trauma directo, manipulación cervical abrupta o extensión cefálica prolongada. Por lo tanto, en pacientes con accidente cerebrovascular de región lateral de bulbo y trayecto extracraneal de la arteria cerebelosa póstero-inferior se debería considerar esta asociación.

We present two cases of lateral medullary stroke in subjects with extracranial trajectory of the postero-inferior cerebellar artery. Case 1: a 21-year-old male who presented ataxia and right dysmetria after cervical trauma in a rugby match. Case 2: 56-year-old woman, who started with vertigo and left hemiparesis after intense physical effort. In both cases, the angiographic studies showed an extracranial trajectory of the posterior inferior cerebellar artery. This vessel rarely originates below the foramen magnum, in close relationship with the first three cervical vertebrae and the atlanto-axial joint. At this level, it is exposed to mechanical damage causing dissection, such as direct trauma, abrupt cervical manipulation or prolonged cephalic extension. Therefore, this association should be considered in patients with stroke of the lateral region of the bulb and extracranial trajectory of the posterior-inferior cerebellar artery.

Vertebral Artery Occlusion Causing Facial Colliculus and Opalski Stroke Syndromes Simultaneously.

Brainstem stroke syndromes refer to a group of vascular disorders that occur secondary to occlusion of small perforating arteries of the posterior circulation. Although such syndromes result in a characteristic clinical picture, they are challenging to localize. In practice, such syndromes are rarely seen in their pure form, and the clinical consequences of a given vascular lesion are probably less predictable than for arteries in the anterior circulation. If 2 or more brainstem strokes happen simultaneously, localization becomes much harder. In this article, we present a case with 2 embolic strokes that occurred simultaneously because of left vertebral artery occlusion. The first in the right facial colliculus causing right sixth and seventh cranial nerves' palsy. The second happened in the left lateral medulla extending below the pyramidal decussation causing a classic left lateral medullary syndrome with ipsilateral hemiparesis, which constitutes a left Opalski stroke syndrome. To our knowledge, this is the first reported case of these 2 brainstem stroke syndromes happening simultaneously.

Lateral medullary infarction with similar features of Brown Sequard syndrome caused by vertebrobasilar dysplasia and Klippel-Feil syndrome: A case report.

RATIONALE: Patients with Klippel-Feil syndrome (KFS) are always anomaly associated with vertebrobasilar dysplasia. That may present commonly as infarction of brainstem, medulla, and cerebellum. In this article, we reported a rare case of lateral medullary infarction (LMI) with similar features of Brown Sequard syndrome caused by vertebrobasilar dysplasia and KFS, and the 2 rare conditions that are causally related. The case is being reported because of its unusual and rare presentation. PATIENT CONCERNS: A 38-year-old female presented with acute unsteadiness, along with a tendency to lean to the left side while walking or sitting, and paresthesia in the right lower limb and trunk, at 2 days before admission. She had no history of hypertension and diabetes, but had a 20 years history of neck pain and dizziness, which was related to head movement. DIAGNOSES: Brown Sequard syndrome and a lesion of the left thoracic spinal cord were suspected initially. KFS was confirmed by the cervical magnetic resonance imaging (MRI) and computed tomographic angiography (CTA) results. Transcranial Doppler (TCD) results confirmed that there was a causal link between LMI and KFS. INTERVENTIONS: The patient rejected the operation of stabilization of the cervical spine with fusion at appropriate levels. OUTCOMES: No recurrence of stroke, but neck pain and dizziness remained after 6 months of discharge. LESSONS: For such patients, the conventional treatment of cerebral infarction might be ineffective, but stabilization of the cervical spine with fusion at appropriate levels can successfully prevent further episodes of syncope and stroke.

Progressive Bilateral Vertebral Artery Dissection in a Case of Osteogenesis Imperfecta.

A 32-year-old woman with osteogenesis imperfecta (OI) was admitted to the hospital because of a right-sided occipital headache and facial paresthesia. She was diagnosed with lateral medullary syndrome due to right vertebral artery (VA) dissection. She was treated conservatively without antithrombotic therapy. She developed subarachnoid hemorrhage because of contralateral VA dissection 18 days later. This clinical course may reflect the underlying weakness of the vessel wall in OI. In patients with OI, occlusion of a unilateral VA could cause dissection and subsequent rupture of the contralateral VA. Early surgical treatment for lesions of the VA is required in such cases.

Vertebral artery-posterior inferior cerebellar artery convergence aneurysms treated by endovascular or surgical treatment: Mid- and long-term outcome.

INTRODUCTION: Management of vertebral artery-posterior inferior cerebellar artery convergence (VA-PICA) saccular aneurysms requires a specialized neurovascular team. The objective is to preserve the functional outcome while performing a complete and reliable long-term exclusion of the aneurysm. OBJECTIVE: The aim of our study was to evaluate the outcome of patients with VA-PICA saccular aneurysms after treatment. MATERIALS AND METHODS: This was a retrospective series of 21 consecutive patients with a VA-PICA saccular aneurysm treated between 2000 and 2012 at our institution. Treatment option (endovascular or microsurgical) was decided for each patient following a multidisciplinary discussion. RESULTS: Twenty-one patients were treated for a VA-PICA saccular aneurysm including 16 for a ruptured aneurysm and 5 for an asymptomatic aneurysm. Among all patients, 11 underwent endovascular treatment and 10 had microsurgical treatment. Our results showed a major aneurysm recurrence after endovascular treatment in 3 patients that required a further endovascular treatment in 2 cases. These 3 major recurrences occurred after treatment of a ruptured aneurysm when the initial angiography demonstrated the origin of the PICA at the neck of the aneurysm. After microsurgery, angiography showed a remnant neck in 2 patients including 1 treated by further endovascular procedure. CONCLUSION: VA-PICA aneurysms are rare and require multidisciplinary management. Microsurgical treatment should be discussed when the PICA originates from the aneurysmal neck, particularly in patients with a ruptured small aneurysm, in order to obtain a reliable and long-term exclusion of the aneurysm.

Successful endovascular treatment of a dissecting aneurysm of vertebral artery associated with double origin of the posterior inferior cerebellar artery.

BACKGROUND: Double origin of the posterior inferior cerebellar artery (DOPICA) has been rarely reported in the literature, with a reported incidence of 1.45%. In contrast, a high concurrence rate of DOPICA and vertebral artery dissecting aneurysm has been reported. CLINICAL PRESENTATION: A 61-year old woman presented with vomiting and diplopia with preceding headache. Magnetic resonance imaging (MRI) showed fresh infarction of the left lateral medulla and a vertebral artery dissecting aneurysm of the left vertebral artery. The next day, she exhibited transient loss of consciousness and worsening of headache, and MRI depicted subarachnoid hemorrhage. Four-vessel digital subtraction angiography showed a posterior inferior cerebellar artery (PICA) arising both intracranially and extracranially from the left vertebral artery. Although the dissecting lesion involved the V3 and V4 portion, it did not involve an extracranially originating PICA. Internal trapping of the V3 and V4 portion was chosen as the extracranial channel was expected to supply the PICA territory. This procedure was safely performed. CONCLUSION: Early endovascular intervention should be considered in the treatment of dissecting aneurysm of vertebral artery associated with DOPICA for patients with relatively long lesions even in unruptured cases.

Isolated Posterior Inferior Cerebellar Artery Dissection as a Cause of Ischemic Stroke: Clinical Features and Prognosis.

BACKGROUND: The purpose of this study was to clarify the incidence, clinical and radiological features and outcomes of isolated posterior inferior cerebellar artery (PICA) dissection in isolated PICA territory infarctions. METHODS: We retrospectively reviewed consecutive inpatients with ischemic stroke secondary to isolated PICA dissection from our stroke database between January 2004 and December 2013 and reviewed the literature with regard to those patients. RESULTS: Of 167 consecutive patients with an isolated PICA territory infarction, a total of 10 patients (6.0%, 3 women, 48.1 ± 7.1 years) were diagnosed as having an isolated PICA dissection. Patients with PICA dissection were younger (p < 0.001), more commonly experienced headache at onset (p = 0.008), less commonly had hyperlipidemia (p = 0.044) and showed a lower modified Rankin Scale score at discharge (p = 0.002) when compared with patients without arterial dissection. In 6 of these 10 patients, PICA dissections had not been suspected on initial magnetic resonance angiography (MRA) and were confirmed by follow-up MRA or digital subtraction angiography. In the follow-up period (median 1.5 years, interquartile range 0.5-6.3 years), there were no recurrent ischemic or hemorrhagic stroke events. CONCLUSIONS: Isolated PICA dissection as an etiological mechanism in isolated PICA territory infarctions may be more common than was previously recognized to be. The diagnosis of PICA dissection is often difficult and requires close and repeated morphological evaluation. We should carefully identify PICA dissections as a possible cause of PICA territory infarctions.

[Opalski syndrome caused by vertebral artery dissection].

A 52-year-old man developed sudden occipital headache followed by vomiting and vertigo. On admission, he displayed right Horner syndrome with ipsilateral reduced facial sensation to pain and temperature that crossed in the body, affecting the left limbs. In addition, he had right hemiparesis. Diffusion weighted magnetic resonance images showed a high intensity lesion localized in the lower and right lateral medulla oblongata. Magnetic resonance angiography showed severe luminal stenosis in the right vertebral artery and T2-weighted sampling perfection with application optimized contrasts using different flip angle evolution (SPACE) showed arterial wall expansion. T1-weighted SPACE showed subacute intramural hematoma at that point, suggesting arterial dissection. First described in 1946, Opalski syndrome is considered a variant of Wallenberg syndrome with ipsilateral hemiparesis. This motor impairment is considered as a result of extension of the ischemia from the lateral medulla to the upper cervical cord involving corticospinal fibers caudal to pyramidal decussation. This case adds information regarding the anatomy of the pyramidal decussation.

Long-term observation of lateral medullary infarction due to vertebral artery dissection assessed with multimodal neuroimaging.

A 33-year-old man presented with a lateral medullary infarction, vertigo, and nausea. At the time of hospital admission, he had Wallenberg syndrome. Although initial magnetic resonance imaging showed no abnormalities, subsequent diffusion-weighted magnetic resonance imaging showed a high-intensity area in the right lateral medulla oblongata. The right vertebral artery was shown to be dilated on basi-parallel anatomical scanning but to be stenosed on magnetic resonance angiography (MRA). Cerebral angiography 7 days after onset showed the "pearl and string sign" in the right vertebral artery. Follow-up MRA showed gradual improvement of the stenosis in the right vertebral artery. Multiple neuroimaging studies, such as MRA, basi-parallel anatomical scanning, 3-dimensional computed tomographic angiography, and cerebral angiography, should be performed soon after onset in suspected cases of cerebral artery dissection. In addition, serial imaging examinations increase diagnostic accuracy, and the medical history and neurological examination are important.

[Lateral medullary infarction presenting with truncal lateropulsion and decreased pain and temperature sensation below T10 sensory level].

A 45-year-old man noted a tendency to fall to the left side on standing following headache and vomiting. Neurological findings were unremarkable except for truncal lateropulsion to the left side. Brain magnetic resonance imaging (MRI) showed a small infarct in the left inferolateral part of medulla. Clinical and imaging findings suggested vertebral artery dissection as the cause of stroke. On hospital day 6, the patient developed decreased pain and temperature sensation in the right side below T10 sensory level and decreased sweating on the left side of the face with miosis of the left pupil. Brain MRI revealed an expanded lesion of the left inferolateral part of medulla. In our patient, the involvement of the spinocerebellar tract and the lateral spinothalamic tract likely contributed to the development of truncal lateropulsion and sensory disturbance below thoracic levels, respectively. Our case is clinically important in understanding a correlation between clinical symptoms and lateral medullary lesions.