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Essential thrombocythemia (ET) is a myeloproliferative malignancy caused by the excessive proliferation of megakaryocytes in the bone marrow, resulting in the overproduction of peripheral platelets. ET can lead to thrombotic events, such as ischemic stroke (IS), though it is a rare cause of IS. Bilateral medial medullary infarction (BMMI), also known as "Y appearance" infarction due to its distinctive imaging morphology, is a rare clinical subtype of IS which typically has a poor prognosis and a high mortality rate. Herein, we report the case of a 43-year-old male with a history of ET. The patient's platelet count was poorly controlled, and he did not receive regular treatment. After developing symptoms such as dizziness, dysphagia, choking on water, slurred speech, blurred vision, and bilateral limb numbness. Head magnetic resonance imaging revealed a "Y appearance" infarction in the bilateral medial medulla. After admission, the patient was administered intravenous antiplatelet therapy with tirofiban. However, when he was switched to oral aspirin after three days, he experienced decreased muscle strength and worsening symptoms. Therefore, tirofiban was continued for 14 days. Upon discharge, the patient experienced residual limb numbness. His National Institutes of Health Stroke Scale score was 1, Modified Rankin Scale score was 0, and platelet count had decreased to the normal range. During the 9-month follow-up period after discharge, the patient still had only mild limb numbness. Our report presents a special case of "Y appearance" infarction due to ET. Owing to fluctuations in the patient's condition, he received long-term high-dose tirofiban, which ultimately led to a significant improvement in his symptoms.
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The anatomical structure of the medulla oblongata is complex, its nerve fibers are dense, and its blood vessels are complex. Clinical manifestations of ischemic damage to the medulla oblongata are therefore relatively diverse, and include vertigo, dysphagia, and dysarthria. Although facial paralysis may also occur, medullary infarction with facial paralysis as the first and only symptom is rare. Herein, we report a case of medullary infarction with ipsilateral central facial paralysis as the only symptom.
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Paralisia Facial , Bulbo , Humanos , Paralisia Facial/diagnóstico , Paralisia Facial/etiologia , Paralisia Facial/patologia , Bulbo/patologia , Bulbo/diagnóstico por imagem , Bulbo/irrigação sanguínea , Masculino , Infartos do Tronco Encefálico/complicações , Infartos do Tronco Encefálico/diagnóstico por imagem , Infartos do Tronco Encefálico/patologia , Infartos do Tronco Encefálico/diagnóstico , Pessoa de Meia-Idade , Imageamento por Ressonância Magnética , FemininoRESUMO
Acute respiratory failure (ARF) due to lateral medullary infarction (LMI) can be a fatal condition, although its details remain unclarified. To clarify clinical aspects of ARF due to LMI, we reviewed eight applicable cases treated in a tertiary hospital. Of them, we focused on the detailed clinical course of one case. We clarified that even patients with a serious clinical course may have no apparent magnetic resonance imaging abnormalities in respiratory control centers at the initial examination. We should be mindful that respiratory monitoring is necessary even without infarction of regions responsible for respiratory control at the initial presentation.
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Wernicke encephalopathy (WE) is an acute life-threatening neurological condition caused by thiamine (vitamin B1) deficiency. Patients with WE often present with a triad of symptoms consisting of ophthalmoplegia, gait ataxia, and mental confusion. If WE is not treated in a timely manner, it can lead to serious complications such as confusion, coma, or death. Although alcohol abuse is the most commonly reported cause of WE, nonalcoholic causes-although rare-do exist. Herein, we present the case of a nonalcoholic woman with medullary infarctions who presented with intractable vomiting. Her clinical state subsequently progressed to include ophthalmoplegia and gait ataxia. A diagnosis of WE was suspected based on her clinical presentation; this was confirmed by brain magnetic resonance imaging (MRI) and the finding of decreased serum thiamine levels. Brain magnetic resonance imaging demonstrated the complete resolution of abnormal hyperintensities during a follow-up visit, 6 months after treatment.
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Imageamento por Ressonância Magnética , Bulbo , Encefalopatia de Wernicke , Humanos , Encefalopatia de Wernicke/diagnóstico , Encefalopatia de Wernicke/etiologia , Encefalopatia de Wernicke/diagnóstico por imagem , Encefalopatia de Wernicke/complicações , Feminino , Bulbo/patologia , Bulbo/diagnóstico por imagem , Bulbo/irrigação sanguínea , Tiamina/uso terapêutico , Tiamina/sangue , Pessoa de Meia-Idade , Infartos do Tronco Encefálico/diagnóstico por imagem , Infartos do Tronco Encefálico/complicaçõesRESUMO
Anterior spinal artery occlusion resulting in bilateral medial medullary infarction (bMMI) and anterior spinal artery syndrome (ASAS) simultaneously has been rarely described. To the best of our knowledge, this is the first report of such occurrence during pregnancy. A 23-year-old preeclamptic parturient at 374/7 weeks underwent an emergent cesarean section after developing gradual neurological deficits. Her symptoms started with a severe occipital headache and progressed to right-hand tingling, left-hand weakness, dyspnea, and elevated blood pressure (165/117 mmHg). Spinal anesthesia was performed by injection of bupivacaine 0.5% with no complications. Twenty minutes into the surgery, after the patient's systolic pressure fell below 85 mmHg, a bolus dose of ephedrine was administered. After a while, the patient presented with sudden respiratory distress and declining consciousness, prompting her immediate intubation. In the intensive care unit, she initially exhibited flaccid quadriplegia, sensory loss, areflexia, upward vertical nystagmus, and some cranial nerve (CN) palsy, including CN 9, 10, and 12, indicative of a medullary-level infarction extending downward. The magnetic resonance imaging (MRI) of the brain revealed a heart-shaped sign in the medulla, suggesting bMMI as a result of anterior spinal artery (ASA) occlusion. During the course of hospitalization, the patient regained the senses of vibration, touch, and proprioception; however, she has remained quadriplegic up to now.
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Background: Opalski syndrome is a rare variant of Wallenberg syndrome (lateral medullary syndrome) that demonstrates concomitant ipsilateral hemiplegia due to infarctions within the lateral medulla and the cervical spinal cord, which also extend to the post-pyramidal decussation to affect the corticospinal tract. Case presentation: A 56-year-old man initially presented with a unilateral headache with right cervical pain. Consequently, he developed symptoms that indicate Wallenberg syndrome: vertigo, dysphonia, dysarthria, right limb ataxia with a tendency to fall, and ptosis, in addition to ipsilateral hemiparesis. The diffusion-weighted imaging MRI (DWI MRI) performed initially demonstrated an acute infarct in the dorsolateral medulla. Accordingly, an axial T2-weighted MRI showed a hyperintense focal region in the same area, even as magnetic resonance angiography (MRA) revealed complete stenosis of the corresponding vertebral artery. Discussion: Opalski syndrome can result from different etiologies such as vascular occlusion or dissection. Although symptoms may vary, patients commonly present with sudden weakness, vertigo, and gait instability. Diagnosis often relies on MRI or DW-MRI. Treatment is tailored depending on individual factors and may involve medications and monitoring. Complications like respiratory failure can occur but are not directly linked to Opalski syndrome. Proper management includes addressing ischemic risk factors and ensuring appropriate nutrition. Conclusion: This case highlights the importance of including Opalski syndrome early in the differential diagnosis of patients with clinically suspected ipsilateral lateral medullary infarction and hyperacute ipsilateral hemiparesis.
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Introduction: This study aims to examine brain activity during different swallowing actions in patients with dysphagia caused by medullary infarction (MI) before and after treatment using blood oxygen level-dependent (BOLD) functional magnetic resonance imaging. Methods: Fifteen patients were enrolled in this study. Brain activation during saliva swallowing and effortful saliva swallowing was observed using BOLD imaging in the acute phase of stroke and after 4 weeks of rehabilitation training. Differences in the activation of brain regions during saliva swallowing before and after treatment, during effortful saliva swallowing before and after treatment, and between the two swallowing actions before and after treatment were compared. Results: In the acute phase of stroke, only the bilateral precentral and left lingual gyrus were partially activated during saliva swallowing, and there was no obvious activation in the insula. Effortful saliva swallowing activated more brain regions than saliva swallowing before treatment, including the bilateral supplementary motor area (SMA), postcentral gyrus, and right insular cortex. The number of brain regions activated during saliva swallowing increased after treatment, including the bilateral precentral gyrus, postcentral gyrus, insula, thalamus, and SMA. Discussion: Cortical activation increases after recovery from dysphagia, and the increased activation of the postcentral gyrus might play a functional compensatory role. Effortful saliva swallowing is a more effective rehabilitation training method for patients with dysphagia caused by MI.
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Bilateral medial medullary infarction (BMMI) is a rare stroke syndrome, which frequently has poor clinical outcomes. Reports on physical therapy for BMMI are few because of its poor prognosis. Therefore, this report aims to present a patient who developed BMMI and underwent well-considered rehabilitation. A 67-year-old man presented to our clinic with an acute onset of vomiting and dizziness. Magnetic resonance imaging (MRI) showed no abnormal signal intensity, and the patient was admitted for peripheral dizziness. On day two, he developed quadriplegia, bulbar palsy, and respiratory impairment, such as prolonged apnea. A second MRI revealed a high-intensity lesion in the bilateral medial medulla oblongata. He was diagnosed with BMMI, and rehabilitation treatment was initiated. On day 16, his sputum volume increased, and he could not expectorate effectively due to decreased coughing ability. Therefore, mechanical insufflation-exsufflation (MI-E) was performed to improve his airway clearance. On day 21, he developed aspiration pneumonia (AP), which became severe and led to acute respiratory failure. Nasal airway intubation and oxygen flow of 5 L/minute were initiated. His respiratory function was not seriously aggravated, and recurrent AP was prevented with the application of respiratory physiotherapy procedures, such as postural drainage, in collaboration with other medical staff, and MI-E. On day 60, the patient was transferred to the recovery phase rehabilitation ward. BMMI tends to worsen swallowing disorders progressively and is associated with a high risk of severe AP. Providing physiotherapy in the acute phase is important to reduce the risk of serious illness.
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Lateral Medullary Syndrome (LMS) poses clinical challenges, often resulting from ischemic events in the medulla oblongata. We present a unique case of LMS in a woman in her 60s with a complex medical history. Alongside neurological deficits, she experienced a transient high-grade atrioventricular block (HgAVB), a rare cardiac manifestation linked to LMS. Given the rarity of simultaneous transient HgAVB with acute lateral medullary infarct, only a handful of case reports documenting similar findings are available in the existing literature. More research and case reporting are needed to better our understanding of this area. The patient's condition, marked by a sudden onset of severe headache and left-sided weakness, revealed an acute infarct in the medulla territory. Notably, her HgAVB spontaneously resolved after 72 hours. This case emphasises the crucial need for extended cardiac surveillance in LMS patients due to their susceptibility to life-threatening arrhythmias. The intricate interplay between the brainstem and cardiovascular system highlights autonomic dysregulation as a potential mechanism for cardiac abnormalities. The report advocates for a holistic approach to managing LMS cases, stressing interdisciplinary collaboration for timely diagnosis and intervention, ultimately improving patient outcomes and reducing the risk of fatal arrhythmias.
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Lateral medullary infarction (LMI), or Wallenberg syndrome, can develop various symptoms, but it is rare that ipsilateral axial lateropulsion (or body lateropulsion, BL) or atonic bladder (AB) are caused by LMI. This report describes a case of LMI with both BL and AB. A 77-year-old man, with a history of hypertension and diabetes, developed acute left BL and anuresis. A neurological exam showed right gaze nystagmus, slight dysarthria and dysphagia, right dysesthesia in the trunk, and ataxia in the left limbs and trunk. Horner's syndrome and paralysis were unremarkable. Brain magnetic resonance imaging revealed hyperintensity in the lateral medulla oblongata. Cystometry revealed AB, although the patient had the urge to urinate. Owing to acute therapy, although trunk ataxia was presented for several months, BL and anuresis were recovered on day 15 and day 35, respectively. Here, we describe the potential mechanisms of BL and AB caused by LMI.
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Bilateral medial medullary infarction (BMMI) is a rare stroke subtype that accounts for less than 1% of acute strokes. Common manifestations of this stroke include quadriparesis, bilateral hypoglossal palsy, bilateral sensory loss, and respiratory failure. We present the case of a 39-year-old male with deafness and mutism who was brought to the emergency department due to acute onset of altered mental status and generalized weakness, further decompensated, and was lately diagnosed with bilateral medial medullary infarction. This case hopes to illustrate a differential diagnosis to be considered and promptly managed when a patient presents with altered mental status and quadriparesis, especially in the acute setting where tissue plasminogen activator (tPA) can still be given.
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Bilateral medial medullary stroke is a very rare condition. It is usually associated with severe motor deficits, dysarthria, sensory symptoms, nystagmus, and respiratory disturbances. The most common etiology is atherosclerotic disease of the vertebral and anterior spinal artery or its branches. We present the case and brain imaging of a 48-year-old man with a bilateral medial medullary stroke and its classic "Heart Sign" in the magnetic resonance imaging. This case highlights the anatomy of the rostral medulla, clinical presentation, etiology, and characteristic radiologic findings of this uncommon type of stroke.
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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.
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Transtornos de Deglutição , Paralisia Facial , Síndrome Medular Lateral , Acidente Vascular Cerebral , Humanos , Paralisia Facial/diagnóstico por imagem , Paralisia Facial/etiologia , Disartria/complicações , Disartria/patologia , Estudos Retrospectivos , Imageamento por Ressonância Magnética/efeitos adversos , Bulbo/diagnóstico por imagem , Infarto , Síndrome Medular Lateral/complicações , Síndrome Medular Lateral/diagnóstico por imagemRESUMO
BACKGROUND AND AIM: Medial medullary infarction (MMI) is a rare type of posterior circulation stroke. We aim to examine the clinical and radiological features, etiology, and prognosis of patients with MMI. METHOD: MMI patients registered consecutively in the stroke databank of the Istanbul Medical Faculty between January 1999 and April 2022 were included in the study. Medullary lesions were rostrocaudally classified as rostral, middle, and caudal, and ventrodorsally as ventral, middle, and dorsal. The etiological classification was performed, and functional outcome was assessed based on the modified Rankin Scale (mRS). Overall survival was estimated using the Kaplan-Meier technique. RESULTS: We examined 48 cases of MMI including 9 with bilateral MMI. There were 34 men (70%), and mean age was 62.9 (± 12.8) years. The median NIHSS score was 7 (IQR; 4.5-10.5). The most common symptom was motor dysfunction. The medullary lesions were located caudally in 4 patients, rostrally in 30, rostromedially in 10, and rostro-medio-caudally in 2 patients. On ventro-dorsal classification; unilateral lesions were found ventrally in 19, ventromedially in 11, and ventro-medio-dorsally in 4 patients. The median follow-up duration was 20 months (interquartile range (IQR); 1-60). According to the third-month mRS, 39% of the patients were considered to have a good prognosis. CONCLUSION: The most common etiology was distal vertebral artery atherosclerosis. More than 50% of the patients could walk unassisted in the long-term follow-up, and stroke recurrence was infrequent. Patients with bilateral MMI had poor outcomes.
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Acute onset quadriplegia with or without facial sparing is an extremely rare vascular syndrome, and the main focus of attention is on the cervical and upper thoracic spinal cord as the putative site of the damage. Quadriplegia has been occasionally reported in brainstem strokes within well-defined lesion patterns, but these reports have gained little attention so far because of the rarity of this clinical syndrome. The clinical, neuroanatomical and neuroimaging features of ischemic stroke locations associated with quadriplegia have been collected and reviewed in a pragmatical view, which includes a detailed description of the neurological signs associated with the damage of the pyramidal pathways. Two clinical examples have been added to raise practical suggestions in neurovascular practice. Ischemic stroke sites determining quadriplegia have some main well-defined midline locations in the brainstem, involving the pyramidal pathways of both sides in a single synchronous ischemic lesion in the medulla oblongata and in the pons. Several accompanying neurological signs have been described when the ischemic lesion involves tracts and nuclei other than the pyramidal pathways, and they can be useful as localizing clues. In some cases, the typical neuroimaging appearance of the ischemic lesion on Magnetic Resonance Imaging (MRI) has been reported as being a "heart appearance sign". This last sign has been described in midbrain strokes too, but this location is not associated with quadriplegia. The main etiology is atherothrombosis involving the intradural segment of the vertebral artery (VA) and their perforating branches. Two clinical examples of these rare vascular syndromes have been chosen to support a pragmatical discussion about the management of these cases. A midline ischemic stroke in the brainstem is a very rare vascular syndrome, and the acute onset quadriplegia is a distinctive feature of it. The awareness of this cerebrovascular manifestation might help to recognize and treat these patients.
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OBJECTIVE: The aim: This study aims in a prospective hospital-based cohort study to determine clinical and imaging features of medial medullary infarction and report a relevant clinical case in a white European adult. PATIENTS AND METHODS: Materials and methods: We have prospectively enrolled one hundred twenty adult patients with acute posterior circulation stroke. All patients were admitted and enrolled in the study within 6 to 24 hours from the onset of the stroke symptoms. Study subjects were recruited from the hospital's wards and emergency departments from 2011 to 2020. Comprehensive clinical, MRI, ultrasound, and laboratory examinations were performed on all patients. RESULTS: Results: 68 men and 52 women aged 28 to 89 years (average age 60.7 ± 12.1 years) with an acute ischemic posterior circulation stroke were enrolled in the study. Out of these 120 patients, 22 (18.3%) had acute medulla oblongata infarctions. Clinical and imaging features of medial medullary infarction are analyzed and illustrated with a clinical case presentation in a white European adult. CONCLUSION: Conclusions: Specific features of medial medullary infarction were determined, analyzed, described, and illustrated with a clinical case.
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Bulbo , Acidente Vascular Cerebral , Adulto , Masculino , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Estudos Prospectivos , Estudos de Coortes , Bulbo/diagnóstico por imagem , Imageamento por Ressonância Magnética/efeitos adversos , Imageamento por Ressonância Magnética/métodos , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Infarto/complicações , HospitaisRESUMO
We report a bilateral medial medullary infarction in which diffusion-weighted images revealed a unique configuration: a heart appearance sign. If it is early diagnosed, it might predict a poor outcome. An 85-year-old man developed dysarthria and numbness in his four limbs and was transferred to our hospital. Brain MR diffusion-weighted images revealed a high-intensity lesion in the bilateral medial medulla oblongata -- a heart appearance sign -- and we diagnosed a bilateral medial medullary infarction. Although his symptom changed aggressively for the worse, it finally changed for the better without bulbar paralysis, and he was transferred to another hospital for rehabilitation. When the medial medulla oblongata is supplied by the unilateral control of the anterior spinal artery, its occlusion can cause a bilateral medial medullary infarction.