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.

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.

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.

Re-growth of a posterior inferior cerebellar artery aneurysm after resection of the associated posterior fossa arteriovenous malformation.

Arteriovenous malformation (AVM)-related aneurysms have been described in the literature. Their behavior varies based on their location in relation to and the activity of the shunting through the index AVM. The intuitive expectation supported by numerous reports is that these aneurysms should regress if the AVM is excluded from the circulation. We describe a case of 46-year-old man who presented with a posterior fossa AVM with an aneurysm on the posterior inferior cerebellar artery feeding the AVM. The nidus of the AVM was successfully excluded by glue embolization, with initial regression of the PICA aneurysm on serial imaging. Five years after the endovascular treatment, the aneurysm showed significant re-growth necessitating endovascular coiling. This case presents the re-growth of an AVM-related aneurysm and emphasizes the importance of long-term follow-up of such aneurysms even if the AVM is completely excluded.

A hidden giant: Wallenberg syndrome and aortal wall thickening as an atypical presentation of a giant cell arteritis.

We report a case of a 73-year-old woman with a brainstem stroke presenting as Wallenberg syndrome. By transoesophageal echocardiography and combined 18F-fluordeoxyglucose positron emission and CT (18F-FDG PET/CT), the diagnosis of large artery vasculitis owing to giant cell arteritis was confirmed. In the absence of classical clinical signs, the examination of the large extracranial vessels by ultrasound and 18F-FDG PET/CT played the key role in detecting a widespread vasculitis.

[Spontaneous occlusion of PICA-involved dissecting aneurysm with development of a collateral channel from the posterior meningeal artery].

A 53-year-old man suffered severe headache, which continued for three days. No abnormality was shown on CT scan, and a dissecting aneurysm of the right vertebral artery was suspected on MRI. Cerebral angiography revealed a dissection aneurysm of the right vertebral artery involved with the posterior inferior cerebellar artery (PICA) as pearl and string sign. The patient was conservatively managed under careful blood pressure control, and was followed by serial MRI. He presented with Wallenberg syndrome three weeks later. Second angiography revealed the occlusion of the PICA-involved dissecting aneurysm and the lateral medullary segment of the PICA supplied by a newly arising vessel from the right posterior meningeal artery (PMA). For the conservative treatment of a vertebral dissection aneurysm involved with PICA presenting with only pain, observation of the course by MRI was effective, and the PMA could develop as the collateral channel to the PICA territory.

[Wallenberg syndrome and neurocysticercosis: about one case in Ouagadougou, Burkina Faso]. / Syndrome de Wallenberg et neurocysticercose: a propos d'un cas a Ouagadougou, Burkina Faso.

Neurocysticercosis (NCC) frequently appears by seizures following parenchymatous location of encysted worms of Taenia solium. We report a case of NCC revealed by a Wallenberg's syndrome. A man of 44 years old, without any cardiovascular risk factor was admitted at the neurology department of Yalgado-Ouédraogo hospital in Ouagadougou for an abrupt onset of vertigos, recurrent falls on the right side, hiccough, and deglutition weakness. The clinical examination found a blood pressure at 130 mmHg/80 mmHg, a CMI at 24, a Wallenberg's syndrome. The CT scan showed a laterobulbar lacunar infarct with punctiform calcifications of cerebellum, third ventricle, frontal, right temporal, occipital and left parietal lobes. The CSF showed a raise of cells number at 23 lymphocytic elements, proteins rose to 1.5 g/l, glucose and chloride were normal. Cysticercosis blood and CSF serologies were positive. The blood cells count showed only an eosinophilia and the blood sedimentation rate reached 119 mm in the first hour. The accepted diagnosis was: laterobulbar lacunar infarct following a NCC angiitis. The course of the disease under albendazole at 15 mg/kg during two weeks combined with a short steroid therapy by prednisolone at 1 mg/kg during 5 days was favourable. NCC should be more and more regarded as a cerebrovascular risk factor in endemic area, especially in young people.

Injury of an aberrant vertebral artery during a routine corpectomy: a case report and literature review.

CASE REPORT: A case report of a 58-year-old man who sustained a laceration of his left vertebral artery during a routine corpectomy for cervical myelopathy is reported. OBJECTIVE: To report iatrogenic injury of a tortuous vertebral artery during anterior cervical spine surgery and discuss appropriate diagnosis and treatment options for this complication. SETTING: UMass Memorial Medical Center, Worcester, MA, USA. BACKGROUND DATA: Vertebral artery anomalies, although rare, are typically present with degenerative processes and great care must be taken to avoid damage during a corpectomy. Cross-sectional imaging coupled with intraoperative angiography is helpful for the urgent evaluation of the injury site and identification of the contralateral vertebral artery's status. METHODS: This is a single case of a patient sustaining a laceration of the left vertebral artery during surgery, which resulted in a lateral medullary stroke. RESULTS: After the left vertebral artery laceration, hemostasis was achieved. With the intent to better visualize and possibly embolize or stent the injury, an angiographic study was carried out. The angiogram revealed a laceration of the left vertebral artery within the vertebral foramina at vertebral body level C6, but intact distal flow. The patient underwent angiographic embolization and a subsequent magnetic resonance imaging (MRI) revealed a left lateral medullary stroke consistent with the lack of flow through the left vertebral artery from C6 to the basilar artery. CONCLUSION: If a tortuous vertebral artery is suspected, then meticulous review of preoperative cross-sectional imaging should be implemented along with angiographic examination. If anomalies are detected and the standard procedure cannot be safely carried out, then alterations, such as preoperative stent placement, need to be considered.

Dissection of the posterior inferior cerebellar artery in a young adult with cerebellar infarct.

The posterior inferior cerebellar artery (PICA) is frequently involved in dissection of the vertebral artery (VA); however, isolated PICA dissection has rarely been reported. A 37-year-old man experienced acute and progressive drowsiness, vertigo, occipital headache, vomiting, and ataxia. There was no precedent trauma or chiropractic manipulation. Neurologically, he had dysmetria of the left extremities. His NIHSS score was 3. Brain magnetic resonance imaging showed an acute cerebellar infarct in the left PICA territory. Magnetic resonance angiography showed a faint signal adjacent to the junction of the left VA and PICA, suggesting a vascular shadow. Catheter angiography showed focal stenosis with a post-stenotic fusiform aneurysmal dilatation of the left proximal PICA that was highly suggestive of dissection with pseudoaneurysm formation. He was treated with clopidogrel and was free of neurological symptoms 3 months after the stroke event. Isolated PICA dissection may be considered in patients with PICA territory infarct or subarachnoid hemorrhage. Treatment depends on the manifestations; ruptured dissecting aneurysms are often treated with surgery or embolization, and infarcts are usually treated with antithrombotic agents.

Spontaneous artery dissection in a patient with Human Immunodeficiency Virus (HIV) infection

INTRODUÇÃO: A relação entre AVC e infecção pelo vírus da imunodeficiência humana (HIV) pode ser atribuída em alguns casos a uma vasculopatia subjacente, assim como ocorre nas dissecções arteriais cervicais espontâneas. RELATO DO CASO: Relatamos o caso de um paciente com infecção pelo HIV que desenvolveu uma síndrome de Wallemberg devido a dissecção da artéria vertebral. Os exames laboratoriais revelaram aumento da homocisteina sérica e proteína C reativa. CONCLUSÃO: Este é o primeiro caso na literatura descrevendo a associação entre dissecção arterial e infecção pelo HIV. Sugerimos que o diagnóstico de dissecção arterial deve ser lembrado como um possível mecanismo de AVC isquêmico em pacientes com infecção pelo HIV.

Brain stem ischemia from intracranial dural arteriovenous fistula: case report.

BACKGROUND: Intracranial dural arteriovenous fistulas (AVFs) with spinal perimedullary venous drainage are rarely reported, but most of the patients initially have presented with myelopathy or subarachnoid hemorrhage. This is the first report of the intracranial dural AVF patient who presented with brain stem infarction. CASE DESCRIPTION: A 38-year-old woman experienced nausea and vomiting with an acute onset, followed by vertigo. Magnetic resonance imaging showed ischemic lesion in the medulla oblongata, and she was then sent to our hospital. On admission, she had nystagmus, swallowing difficulties, Homer syndrome, and right hemiparesis and hemisensory disturbance. Cerebral angiography revealed dural AVF draining into spinal perimedullary veins at the left transverse-sigmoid sinus. The patient was treated by transvenous embolization under local anesthesia. A microcatheter proceeded to the left sigmoid sinus via the internal jugular vein, and embolization of the sinus was performed using coils without complications. The patient's swallowing difficulties improved over a few days after the embolization, and one month later, there remained only a slight mild hemiparesis and hemisensory disturbance. Six months after the onset, there was no ischemic lesion in the brain stem on magnetic resonance imaging. CONCLUSIONS: In this case, we showed the possibility of brain stem infarction, caused by the intracranial dural AVF.

[Wallenberg's syndrome as the presenting symptom of a cavernous angioma]. / Síndrome de Wallenberg como forma de presentación de un angioma cavernoso.

INTRODUCTION: Cavernous angiomas account for 5-13% of all vascular malformations. In 75% of cases they are situated in the posterior fossa and up to 30% are associated with abnormal venous drainage. The main complication is haemorrhage; the presence of a neurological focus without radiological evidence of bleeding is very rare. CASE REPORT: We report the case of a 54-year-old male with cardiovascular risk factors who presented symptoms that progressively deteriorated over a 72-hour period involving the left lower cranial nerves, sensory impairment and coordination disorder, compatible with Wallenberg's syndrome. Two computerised axial tomography scans of the brain were normal and so a tentative diagnosis of ischemic stroke in progression was proposed. Five days later, magnetic resonance imaging (MR) revealed the presence of a venous angioma and associated abnormal venous drainage. CONCLUSIONS: Cavernous angiomas present a dynamic balance between intracavernous bleeding and thrombosis, with very slow venous blood flow. Upsetting this balance leads to an increase in the intracavernous pressure and involvement of the surrounding tissue, with no radiological expression of bleeding. In these cases MR scanning helps to distinguish between a vascular malformation with reduced blood flow and a clinical picture of ischemic stroke of an arterial origin.

Fibromuscular dysplasia of the vertebral artery presenting with lateral medullary syndrome: a case report.

We have recently encountered a rare case of fibromuscular dysplasia (FMD) of the vertebral artery (VA) presenting as lateral medullary syndrome. A 39-year-old male was admitted to our hospital due to vertigo, dysarthria and numbness of the left face and the right limbs. A magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of brain revealed lateral medullary infarction in the territory of the left posterior inferior cerebellar artery (PICA). The angiography of the VA revealed tubular stenosis of the left extracranial VA and a focal vascular kinking as well as web in the right extracranial VA, confirming the diagnosis of FMD. We present this rare case to emphasize that FMD could be one of the risk factors causing lateral medullary syndrome in young people.

[A case of embolic infarction originating from extracranial vertebral artery stenosis by cervical spondylosis at C5/6: its pathogenesis and surgical treatment].

We report a case of right brainstem and cerebellar infarction induced by head rotation. The infarction was due to an artery to artery embolism originating from extracranial vertebral artery stenosis resulting from C5-6 cervical spondylosis. A-64-year-old man with cervical spondylosis experienced swallowing difficulty and vertigo while eating dinner. Neurological examination at admission indicated lateral medullary syndrome (Wallenberg syndrome). MRI (FLAIR) revealed multiple high-intensity signal areas in the right lateral medulla and the cerebellar hemisphere in the territory of the posterior inferior cerebellar artery (PICA). Right vertebral angiogram demonstrated marked stenosis in the neutral position and occlusive changes with head rotation to the right (30 degrees) at C5-6. Rotation induced substantially delayed filling of the right vertebral artery. To remedy this, we resected the osteophyte, fused the uncovertebral joint and connective tissue adhering tightly to the right vertebral artery at C5-6 level, and unroofed the transverse foramens at C5 and C6. Post-operative angiogram confirmed the disappearance of marked stenosis and improved antegrade filling of the right vertebral artery. In the present report, the pathogenesis and surgical treatment of this particular case are discussed.

[A dissecting aneurysm of the posterior inferior cerebellar artery was reduced spontaneously during conservative therapy: case report].

We report here a case of a patient with a dissecting aneurysm of the anterior medullary segment of the posterior inferior cerebellar artery (PICA) which presented with Wallenberg's syndrome. A 32-year-male presented with an unusual case of Wallenberg's syndrome due to a dissecting aneurysm of the PICA manifesting as a sensation of heaviness in the occipital region and vertigo. The occipital symptoms persisted and vertigo and vomiting developed after 6 days. Numbness developed on the left side of the patient's face, and hyperalgesia on the right side of the body. The diagnosis of Wallenberg's syndrome was based on the above findings. MRI revealed infarction of the lateral aspect of the medulla oblongata and MR angiography revealed dilatation in the proximal portion of the left PICA. Digital subtraction angiography revealed that the left vertebral artery was essentially normal, but there was a spindle-shaped dilatation in the proximal portion of the left PICA. We carried out conservative therapy at the patient's request and 3D-CTA revealed that the dissecting aneurysm was markedly reduced in size seven months after the onset. Dissecting aneurysms of the intracranial posterior circulation have been shown to be less uncommon than previously thought. However, those involving the PICA without involvement of the vertebral artery at all are extremely rare. The natural history of the dissecting PICA aneurysm was unknown, and the indication for surgical treatment of such aneurysms remains controversial. Management options are conservative treatment, open surgical treatment including wrapping, trapping, and resection with reconstruction, but almost all of the patients underwent radical treatment to prevent rupture of the aneurysm. However we had no knowledge of the risk of rupture of a PICA dissecting aneurysm presenting with ischemic symptoms. We have reviewed the well-documented 15 cases of dissecting aneurysms of the PICA reported in the literature and we discuss the management of the dissecting PICA aneurysm presenting with ischemic symptoms.

[Lateral medullary infraction due to left atrial myxoma--a case report]. / Sluzak lewego przedsionka przyczyna zespolu bocznego opuszki--opis przypadku.

A case of 53 year old women with Wallenberg syndrome due to atrial myxoma is reported. She was operated. Atrial myxoma on 7 x 8 cm was removed. After few months she can walk independently, she needs only a little help in everyday activities.