Topographic Consideration on the Occurrence of Ipsilesional Facial Paresis in Lateral Medullary Infarction.

INTRODUCTION: The purpose of this study was to identify course of the corticobulbar tract and factors associated with the occurrence of facial paresis (FP) in lateral medullary infarction (LMI). METHODS: Patients diagnosed with LMI who were admitted to tertiary hospital were retrospectively investigated and divided into two groups based on the presence of FP. FP was defined as grade 2 or more by the House-Brackmann scale. Differences between the two groups were analyzed with respect to anatomical location of the lesions, demographic data (age, sex), risk factors (diabetes, hypertension, smoking, prior stroke, atrial fibrillation, and other cardiac risk factors for stroke), large vessel involvement on magnetic resonance angiography, other symptoms and signs (sensory symptoms, gait ataxia, limb ataxia, dizziness, Horner syndrome, hoarseness, dysphagia, dysarthria, nystagmus, nausea/vomiting, headache, neck pain, diplopia, and hiccup). RESULTS: Among 44 LMI patients, 15 patients (34%) had FP, and all of them had ipsilesional central-type FP. The FP group tended to involve upper (p < 0.0001) and relative ventral (p = 0.019) part of the lateral medulla. Horizontally large lesion was also related to the presence of FP (p = 0.044). Dysphagia (p = 0.001), dysarthria (p = 0.003), and hiccups (p = 0.034) were more likely to be accompanied by FP. Otherwise, there were no significant differences. CONCLUSION: The results of present study indicate that the corticobulbar fibers innervating the lower face decussate at the upper level of the medulla and ascend through the dorsolateral medulla, where the concentration of the fibers is densest near the nucleus ambiguus.

Sinus arrest following acute lateral medullary infarction.

Lateral medullary syndrome (LMS) is an ischemic stroke of the medulla oblongata that involves the territory of the posterior inferior cerebellar artery. LMS is often missed as the cause of autonomic dysregulation in patients with recent brain stem stroke. Due to the location of the nucleus tractus solitarius (NTS), the dorsal vagal nucleus, and the nucleus ambiguous in the lateral medulla oblongata, patients with LMS occasionally have autonomic dysregulation-associated clinical manifestations. We report a case of LMS-associated autonomic dysregulation. The case presented by recurrent syncope, requiring permanent pacemaker placement. This case shows the importance of recognizing LMS as a potential cause of life-threatening arrhythmias, heart block, and symptomatic bradycardia. Extended cardiac monitoring should be considered for patients with medullary strokes.

Severe Unilateral Proprioceptive Loss in Medullary- Rostral Spinal Cord Infarction. A Posterior Spinal Artery Syndrome.

We draw attention to a unique presentation, severe unilateral loss of limb proprioception, in patients with medullary and rostral spinal cord infarction. Two patients developed acute severe proprioceptive loss in the limbs ipsilateral to infarcts that involved the caudal medulla and rostral spinal cord. They also had symptoms and signs often found in lateral medullary infarction. The proprioceptive loss is attributable to injury to the gracile and cuneate nuclei and/or their projections to the medial lemniscus. The infarct territory is supplied by the posterior spinal branches of the vertebral artery near its penetration into the posterior fossa. The presence of severe ipsilateral proprioceptive loss in a patient with features of lateral medullary infarction indicates involvement of the rostral spinal cord.

Clinical and Radiological Characteristics Associated with Respiratory Failure in Unilateral Lateral Medullary Infarction.

BACKGROUND: The prognosis for unilateral lateral medullary infarction (ULMI) is generally good but may be aggravated by respiratory failure with fatal outcome. Respiratory failure has been reported in patients with severe bulbar dysfunction and large rostral medullary lesions, but its associated factors have not been systematically studied. We aimed to assess clinical and radiological characteristics associated with respiratory failure in patients with pure acute ULMI. MATERIALS AND METHODS: Seventy-one patients (median age 55 years, 59 males) with MRI-confirmed acute pure ULMI were studied retrospectively. Clinical characteristics were assessed and bulbar symptoms were scored using a scale developed for this study. MRI lesions were classified into 4 groups based on their vertical extent (localized/extensive) and the involvement of the open and/or closed medulla. Clinical characteristics, bulbar scores and MRI lesion characteristics were compared between patients with and without respiratory failure. RESULTS: Respiratory failure occurred in 8(11%) patients. All patients with respiratory failure were male (p = 0.336), had extensive lesions involving the open medulla (p = 0.061), progression of bulbar symptoms (p=0.002) and aspiration pneumonia (p < 0.001). Peak bulbar score (OR, 7.9 [95% CI, 2.3-160.0]; p < 0.001) and older age (OR, 1.2 [95%CI, 1.0-1.6]; p=0.006) were independently associated with respiratory failure. CONCLUSIONS: Extensive damage involving the open/rostral medulla, clinically presenting with severe bulbar dysfunction, in conjunction with factors such as aspiration pneumonia and older age appears to be crucial for the development of respiratory failure in pure ULMI. Further prospective studies are needed to identify other potential risk factors, pathophysiology, and effective preventive measures for respiratory failure in these patients.

Opalski Syndrome Treated with Intravenous Recombinant Tissue Type Plasminogen Activator-Case Report and Review of Literature.

A 65-year-old man with a history of Wallenberg syndrome caused by vertebral artery dissection at 62 years old was admitted to our hospital with nausea, vertigo, right facial dysesthesia, right hemiplegia, crossed sensory disturbance (sensory loss and numbness in the right face and left body below the neck), and right limb ataxia. Magnetic resonance imaging (MRI) performed 80 minutes after onset revealed no acute ischemic stroke lesions, but magnetic resonance angiography (MRA) demonstrated complete occlusion of the right vertebral artery. Based on these neurological and MRA findings, atypical lateral medullary infarction was suggested, and intravenous tissue plasminogen activator (IV-tPA) was started 178 minutes after onset. Right hemiplegia improved immediately after IV-tPA administration. MRI performed on hospital day 2 showed an acute ischemic lesion on the right side of the medulla oblongata, resulting in a diagnosis of Opalski syndrome. Opalski syndrome is a rare subtype of Wallenberg syndrome accompanied by hemiplegia of the side ipsilateral to the lesion, and expansion of the stroke lesion to the corticospinal tract below the pyramidal decussation is considered to cause ipsilateral hemiplegia. Based on this case and previous reports, Opalski syndrome should be considered when limb ataxia and crossed sensory deficit are observed among patients with hyperacute-onset hemiplegia, and IV t-PA therapy should be considered even in the absence of neurological findings such as dysphagia, dysarthria, and Horner's signs and radiological evidence of acute ischemic stroke.

Lateral Medullary Syndrome Following Injury of Lateral Vestibulospinal Tract: Diffusion Tensor Imaging Study.

BACKGROUND: Unilateral lesions of vestibular nucleus can cause lateral medullary syndrome. Little is known about injury of medial and lateral vestibulospinal tract (VST) after dorsolateral medullary infarct. We investigated injury of the lateral VST in patients with typical central vestibular disorder using diffusion tensor tractography (DTT). METHODS: Seven patients with lateral medullary syndrome and ten control subjects were recruited. For the medial VST, we determined seed region of interest (ROI) as medial vestibular nuclei of pons and target ROI on posteromedial medulla. For the lateral VST, the seed ROI was placed on lateral vestibular nuclei of pons, and the target ROI on posterolateral medulla. Fractional anisotropy (FA), mean diffusivity (MD), and tract volume were measured. RESULT: Reconstructed lateral VST on both sides had significantly lower FA values in patients than controls (p<0.05). Tract volume of lateral VST in affected side was significantly lower than unaffected side and control group (p<0.05). However, no DTI parameters of the medial VST differed between patients and controls (p>0.05). CONCLUSION: Injury of the lateral VST was demonstrated in patients with lateral vestibular syndrome following dorsolateral medullary infarct. Analysis of the lateral VST using DTT would be helpful in evaluation of patients with lateral medullary syndrome.

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.

Reversible drug-induced antiphospholipid syndrome.

We report an original case of reversible antiphospholipid syndrome (APS) due to minocycline in a young male patient who experienced recurrent strokes while taking minocycline. He started minocycline therapy (50 mg twice daily) at 15 years old for acne. After three years of treatment, the patient experienced a lateral medullary syndrome. He was treated with aspirin while minocycline was continued. Eighteen months later, the patient complained about horizontal binocular diplopia. MRI revealed an infarct of the oculomotor nerve nucleus. Laboratory investigations revealed high titers of anti-beta 2 glycoprotein 1 (antiß2GP1) antibodies of 470 U/ml (normal range <15 U/ml) and antiphosphatidylethanolamine antibodies of 137.4 U/ml (normal range <18 U/ml). Other laboratory tests were normal. Six weeks after discontinuation of minocycline, anti-ß2GP1 antibodies decreased to 335 U/ml and to 36 U/ml at six months and then remained negative for six years. Many drugs have been considered as possibly causing APS but only in a limited number of patients. To our knowledge this is the first case of drug-induced APS with complete disappearance of high titers of anti-ß2GP1 antibodies after minocycline withdrawal. This case also illustrates the need to monitor the levels of antiphospholipid antibodies, even though initial values are high and confirmed after 12 weeks.

Lateral Medullary Infarction with or without Extra-Lateral Medullary Lesions: What Is the Difference?

BACKGROUND: Lateral medullary infarction (LMI) is not an uncommon disease. Although lesions are usually restricted to the lateral medullary area, some patients have additional infarcts in other parts of the brain. The clinical features and prognosis of isolated LMI (pure LMI, LMIpr) have been investigated. However, it remains unclear whether clinical characteristics, prognosis and factors associated with prognosis differ between patients with LMIpr and those with additional lesions (LMI plus, LMIpl). METHODS: Patients with LMI identified by MRI were enrolled. The demographic and clinical characteristics, in-hospital outcome (intensive care unit [ICU] admission, pneumonia and modified Rankin scale [mRS] at discharge), and long-term residual symptoms (vertigo/dizziness, sensory disturbances, dysphagia) and outcomes (occurrence of stroke, acute coronary syndrome [ACS], death, and mRS at follow-up) were compared between LMIpr and LMIpl patients. Factors associated with poor functional outcome (mRS 2-6) at the follow-up were analyzed. RESULTS: Among 248 LMI patients, 161 (64.9%) had LMIpr and 87 (35.1%) had LMIpl. During admission, patients with LMIpl more frequently experienced ICU care, pneumonia and had a higher discharge mRS (3 vs. 2; p < 0.001) than LMIpr patients. The occurrence of stroke, ACS, frequency of death and functional outcome was not different during follow-up. However, residual neurologic symptoms such as dizziness (p = 0.002), dysphagia (p = 0.04) and sensory symptoms (p < 0.001) were more frequent in LMIpr than in LMIpl patients. In LMIpr patients, the rostral location of LMI was associated with poor functional outcome (p = 0.041), whereas in LMIpl patients, the presence of medial posterior-inferior cerebellar artery lesion was associated with good functional outcome (p = 0.030). CONCLUSION: Although the short-term outcome is poorer in LMIpl than LMIpr patients, long-term residual symptoms are more common in LMIpr patients. The location of the LMI and extra-medullary lesion affects the long-term functional outcome of LMIpr and LMIpl patients respectively.

False-negative diagnostic imaging of Wallenberg's syndrome by diffuse-weighted imaging: a case report and literature review.

Here, we report a case of a 64-year-old female with acute-onset vertigo, nausea, and vomiting. In an emergency imaging examination, the results of computed tomography (CT) and diffusion weighted imaging (DWI) were negative. However, on 1 day post-hospital admission, a small acute infarct in the posterolateral aspect of the left medulla was detected by DWI. Extra attention should be payed to the false-negative imaging results to avoid diagnosis and treatment delay.

Posterior Meningeal Artery Origin Patterns among 300 Cases and Their Clinical Importance.

BACKGROUND AND PURPOSE: The posterior meningeal artery (PMA) is known as a dura mater-nourishing vessel. We encountered a patient with Wallenberg syndrome during transarterial embolization of the PMA associated with the dural arteriovenous fistula (DAVF). METHODS: After development of Wallenberg syndrome in the patient, we assessed origins of the PMA patterns in 300 cases and divided them into 3 types. CASE PRESENTATION: A 63-year-old man was incidentally diagnosed as having transverse-sigmoid sinus DAVF with a cortical venous reflux. During the transarterial embolization, the patient complained of vertigo and numbness of the right extremities. Postoperatively, the patient exhibited Wallenberg syndrome. Diffusion-weighted magnetic resonance imaging showed a high-intensity area on the lateral side of the right medulla. CONCLUSIONS: While performing arterial embolization of the PMA that directly originates from the intracranial vertebral artery, the possibility of deficient brainstem nourishment must be considered.

[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.

Dermatomal Sensory Manifestations in Opalski Syndrome.

A 31-year-old Japanese woman presented with sudden-onset unstable gait followed by nuchal pain. A neurological examination revealed right-sided limb weakness and decreased pain and thermal sensation on the left side below the level of the L1 dermatome. A lower lateral medullary infarction with ipsilateral hemiplegia, known as Opalski syndrome, caused by spontaneous vertebral artery dissection was diagnosed by magnetic resonance imaging. The spinothalamic tract in the medulla oblongata has a topographic arrangement of sensory fibers, and the dermatomal sensory deficit in this case can be explained in relation to that. This is the first reported case of Opalski syndrome with dermatomal sensory manifestations. Opalski syndrome could be a differential diagnosis for dermatomal sensory manifestations.

High-Resolution Magnetic Resonance Imaging Evidence for Intracranial Vessel Wall Inflammation Following Endovascular Thrombectomy.

Intracranial high-resolution vessel wall magnetic resonance imaging evidence of vessel wall inflammation is present following stent retriever manipulation but absent following aspiration thrombectomy. This is presented in a case of rotatory vertebral artery compression causing multiple posterior circulation infarctions requiring multiple separate aspiration and stent retriever thrombectomies.

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.

Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with lateral medullary syndrome: case report and literature review.

BACKGROUND: Only one case of syndrome of inappropriate secretion of antidiuretic hormone with lateral medullary syndrome has been reported so far. We report a case of lateral medullary syndrome showing syndrome of inappropriate secretion of antidiuretic hormone and analyze the pathomechanism underlying its clinical features. CASE PRESENTATION: A 67-year-old man was admitted to our hospital for dizziness, dysarthria, and dysphagia. He was diagnosed with lateral medullary syndrome based on the neurological examination and brain magnetic resonance imaging. Horner syndrome was absent. Asymptomatic hyponatremia appeared 9 days after admission and the patient was diagnosed with syndrome of inappropriate secretion of antidiuretic hormone. Fluid restriction and intravenous furosemide injection improved the hyponatremia. CONCLUSION: Lateral medullary syndrome could be associated with syndrome of inappropriate secretion of antidiuretic hormone.