Wernicke encephalopathy in a patient with medullary infarctions: a case report.

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.

Medullary infarction with central facial paralysis as the only symptom: a case report.

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.

Posterior Fossa Craniopharyngioma Arising from the Inferior Medullary Velum.

We report the case of a 63-year-old man with a midline posterior fossa tumor and peculiar imaging features where we were unsure of the nature of the lesion preoperatively. Histopathology revealed it to be a craniopharyngioma. It appeared to arise from the inferior medullary velum, a site not described before in the literature. The previous four cases mentioned in literature and speculations on the origins in this uncommon site are discussed.

An analysis of clinical characteristics of rare bilateral medial medullary infarction: An observational study.

This study aimed to characterize the risk factors, etiology, clinical manifestations, anatomical characteristics, stroke mechanisms, imaging features, and prognosis of bilateral medial medullary infarction (BMMI). A retrospective analysis was conducted on 11 patients with BMMI who met the inclusion criteria at the Affiliated Hospital of Xuzhou Medical University from January 2013 to January 2023. The patients' imaging and clinical features were analyzed and summarized. Eleven patients (7 male, 4 female), aged 46 to 62 years, met the inclusion criteria. Common clinical presentations included dysarthria (90.9%), dysphagia (90.9%), quadriplegia (81.8%), and so on. Within 72 hours of onset, 8 cases presented with quadriplegia, 2 cases with hemiplegia, and 1 case without limb paralysis. The main risk factor for BMMI was hypertension, followed by diabetes. "Heart appearance" infarcts occurred in 4 cases (36.4%), while "Y appearance" infarcts occurred in 7 cases (63.6%). Among the patients, 3 had unilateral vertebral artery stenosis or occlusion, 5 had bilateral vertebral artery stenosis or occlusion, 2 had normal vertebral basilar artery, and 1 did not undergo cerebrovascular examination. All patients received standardized treatment for cerebral infarction. The prognosis was poor, with 81.8% of patients having an unfavorable outcome, including 1 death, 9 cases of disability, and only 1 patient achieving self-care ability after recovery. BMMI is more prevalent in males aged 45 to 60 years. The main risk factors are hypertension and diabetes. Atherosclerosis is the primary etiological subtype. The main clinical manifestations are dyskinesia, dizziness, quadriplegia, and dysarthria. The prognosis of BMMI is poor. The specific imaging features of "heart appearance" or "Y appearance" infarcts aid in the diagnosis of BMMI.

Brainstem Chipmunk Sign: A Diagnostic Imaging Clue across All Subtypes of Alexander Disease.

BACKGROUND AND PURPOSE: While classic brain MR imaging features of Alexander disease have been well-documented, lesional patterns can overlap with other leukodystrophies, especially in the early stages of the disease or in milder phenotypes. We aimed to assess the utility of a new neuroimaging sign to help increase the diagnostic specificity of Alexander disease. MATERIALS AND METHODS: A peculiar bilateral symmetric hyperintense signal on T2-weighted images affecting the medulla oblongata was identified in an index patient with type I Alexander disease. Subsequently, 5 observers performed a systematic MR imaging review for this pattern by examining 55 subjects with Alexander disease and 74 subjects with other leukodystrophies. Interobserver agreement was assessed by the κ index. Sensitivity, specificity, and receiver operating characteristic curves were determined. RESULTS: The identified pattern was present in 87% of subjects with Alexander disease and 14% of those without Alexander disease leukodystrophy (P < .001), 3 with vanishing white matter, 4 with adult polyglucosan body disease, and 3 others. It was found equally in both type I and type II Alexander disease (28/32, 88% versus 18/21, 86%; P = .851) and in subjects with unusual disease features (2/2). Sensitivity (87.3%; 95% CI, 76.0%-93.7%), specificity (86.5%; 95% CI, 76.9%-92.5%), and interobserver agreement (κ index = 0.82) were high. CONCLUSIONS: The identified pattern in the medulla oblongata, called the chipmunk sign due to its resemblance to the face of this rodent, is extremely common in subjects with Alexander disease and represents a diagnostic tool that can aid in early diagnosis, especially in subjects with otherwise atypical MR imaging findings and/or clinical features.

A novel perspective for exploring the relationship between cerebral small vessel disease and deep medullary veins with automatic segmentation.

BACKGROUND: This study aimed to establish an intelligent segmentation algorithm to count the number of deep medullary veins (DMVs) and analyze the relationship between DMVs and imaging markers of cerebral small vessel disease (CSVD). METHODS: DMVs on magnetic resonance imaging (MRI) of patients with CSVD were counted by intelligent segmentation and manual counting. The dice coefficient and intraclass correlation coefficient (ICC) were used to evaluate their consistency and correlation. Structural MR images were used to assess imaging markers and total burden of CSVD. A multivariate linear regression model was used to evaluate the correlation between the number of DMVs counted by intelligent segmentation and imaging markers of CSVD, including white matter hyperintensities of the presumed vascular origin, lacune, perivascular spaces, cerebral microbleeds, and total CSVD burden. RESULTS: A total of 305 patients with CSVD were enrolled. An intelligent segmentation algorithm was established to calculate the number of DMVs, and it was validated and tested. The number of DMVs counted intelligently significantly correlated with the manual counting method (r = 0.761, P< 0.001). The number of smart-counted DMVs negatively correlated with the imaging markers and total burden of CSVD (P< 0.001), and the correlation remained after adjusting for age and hypertension (P< 0.05). CONCLUSIONS: The proposed intelligent segmentation algorithm, which was established to count DMVs, can provide objective and quantitative imaging information for the follow-up of patients with CSVD. DMVs are involved in CSVD pathogenesis and a likely new imaging marker for CSVD.

Lateral medullary vascular compression manifesting as paroxysmal hypertension.

Neurovascular compression of the rostral ventrolateral medulla (RVLM) has been described as a possible cause of refractory essential hypertension. We present the case of a patient affected by episodes of severe paroxysmal hypertension, some episodes associated with vago-glossopharyngeal neuralgia. Classical secondary forms of hypertension were excluded. Imaging revealed a neurovascular conflict between the posterior inferior cerebellar artery (PICA) and the ventrolateral medulla at the level of the root entry zone of the ninth and tenth cranial nerves (CN IX-X REZ). A MVD of a conflict between the PICA and the RVLM and adjacent CN IX-X REZ was performed, resulting in reduction of the frequency and severity of the episodes. Brain MRI should be performed in cases of paroxysmal hypertension. MVD can be considered in selected patients.

Deep medullary vein damage correlates with small vessel disease in small vessel occlusion acute ischemic stroke.

OBJECTIVES: We aim to investigate whether cerebral small vessel disease (cSVD) imaging markers correlate with deep medullary vein (DMV) damage in small vessel occlusion acute ischemic stroke (SVO-AIS) patients. METHODS: The DMV was divided into six segments according to the regional anatomy. The total DMV score (0-18) was calculated based on segmental continuity and visibility. The damage of DMV was grouped according to the quartiles of the total DMV score. Neuroimaging biomarkers of cSVD including white matter hyperintensity (WMH), cerebral microbleed (CMB), perivascular space (PVS), and lacune were identified. The cSVD score were further analyzed. RESULTS: We included 229 SVO-AIS patients, the mean age was 63.7 ± 23.1 years, the median NIHSS score was 3 (IQR, 2-6). In the severe DMV burden group (the 4th quartile), the NIHSS score grade (6 (3-9)) was significantly higher than other groups (p < 0.01). The grade scores for basal ganglia PVS (BG-PVS) were positively correlated with the degree of DMV (R = 0.67, p < 0.01), rather than centrum semivole PVS (CS-PVS) (R = 0.17, p = 0.1). In multivariate analysis, high CMB burden (adjusted odds ratio [aOR], 25.38; 95% confidence interval [CI], 1.87-345.23) was associated with severe DMV scores. In addition, BG-PVS was related to severe DMV burden in a dose-dependent manner: when BG-PVS score was 3 and 4, the aORs of severe DMV burden were 18.5 and 12.19, respectively. CONCLUSION: The DMV impairment was associated with the severity of cSVD, which suggests that DMV burden may be used for risk stratification in SVO-AIS patients. CLINICAL RELEVANCE STATEMENT: The DMV damage score, based on the association between small vessel disease and the deep medullary veins impairment, is a potential new imaging biomarker for the prognosis of small vessel occlusion acute ischemic stroke, with clinical management implications. KEY POINTS: • The damage to the deep medullary vein may be one mechanism of cerebral small vessel disease. • Severe burden of the basal ganglia perivascular space and cerebral microbleed is closely associated with significant impairment to the deep medullary vein. • The deep medullary vein damage score may reflect a risk of added vascular damage in small vessel occlusion acute ischemic stroke patients.

Olivary body exposure through far lateral and lower retrosigmoid approaches. Comparative analysis of the exposed surface and angle of attack.

OBJECTIVES: Throughout neurosurgical history, the treatment of intrinsic lesions located in the brainstem has been subject of much controversy. The brainstem is the anatomical structure of the central nervous system (CNS) that presents the highest concentration of nuclei and fibers, and its simple manipulation can lead to significant morbidity and mortality. Once one of the safe entry points at the medulla oblongata has been established, we wanted to evaluate the safest approach to the olivary body (the most used safe entry zone on the anterolateral surface of the medulla oblongata). The proposed objective was to evaluate the working channel from the surface of each of the far lateral and retrosigmoid approaches to the olivary body: distances, angles of attack and channel content. MATERIAL AND METHODS: To complete this work, a total of 10 heads injected with red/blue silicone were used. A total of 40 approaches were made in the 10 heads used (20 retrosigmoid and 20 far lateral). After completing the anatomical study and obtaining the data referring to all the approaches performed, it was decided to expand the sample of this research study by using 30 high-definition magnetic resonance imaging of anonymous patients without cranial or cerebral pathology. The reference points used were the same ones defined in the anatomical study. After defining the working channels in each of the approaches, the working distances, angle of attack, exposed surface, and the number of neurovascular structures present in the central trajectory were analyzed. RESULTS: The distances to the cranial and medial region of the olivary body were 52.71 mm (SD 3.59) from the retrosigmoid approach and 27.94 mm (SD 3.99) from the far lateral; to the most basal region of the olivary body, the distances were 49.93 (SD 3.72) from the retrosigmoid approach and 18.1 mm (SD 2.5) from the far lateral. The angle of attack to the caudal region was 19.44° (SD 1.3) for the retrosigmoid approach and 50.97° (SD 8.01) for the far lateral approach; the angle of attack to the cranial region was 20.3° (SD 1.22) for the retrosigmoid and 39.9° (SD 5.12) for the far lateral. Regarding neurovascular structures, the probability of finding an arterial structure is higher for the lateral far, whereas a neural structure will be more likely from a retrosigmoid approach. CONCLUSIONS: As conclusions of this work, we can say that far lateral approach presents more favorable conditions for the microsurgical treatment of intrinsic bulbar and bulbomedullary lesions approached through the caudal half of the olivary body. In those cases of bulbar and pontine-bulbar lesions approached through the cranial half of the olivary body, the retrosigmoid approach can be considered for selected cases.

Attempted Suicide with a Self-Inflicted Penetrating Stab Wound to the Medulla Oblongata Caused by a Pair of Scissors: Case Illustration.

A 78-year-old male patient with a history of psychotic depression was found in the bathroom of his apartment with a pair of scissors driven deeply into the back of his neck. He presented with tetraparesis with residual control over the left lower limb movement. Immediate skull radiograph and brain computed tomography scans revealed the tip of the scissors passing into the foramen magnum. Emergent surgery with midline suboccipital craniectomy and resection of the posterior arch of C1 was performed. The scissors were spontaneously dislocated in the course of surgery. Thereafter, debridement, placement of external ventricular drain and primary closure of the dura and skin were achieved. Post-operatively, the patient was not able to follow any instructions. On the 22nd post-operative day, the patient passed away in palliation. To our knowledge, this is the first reported case of a suicide attempt involving the use of scissors reaching the medulla oblongata.

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.

Substitution of Glu to Lys at Codon 332 on the GFAP Gene Alone Is Causative for Adult-onset Alexander Disease.

A 57-year-old man whose mother had been pathologically diagnosed with Alexander disease (ALXDRD), presented with cerebellar ataxia, pyramidal signs, and mild dysarthria. Brain magnetic resonance imaging revealed typical ALXDRD alterations, such as atrophy of the medulla oblongata (MO) and cervical spinal cord, a reduced sagittal diameter of the MO, and garland-like hyperintensity signals along the lateral ventricular walls. A genetic analysis of GFAP by Sanger sequencing revealed a single heterozygous mutation of Glu to Lys at codon 332 (c.994G>A) in the GFAP gene. Our results newly confirmed that p.E332K alone is the pathogenic causative mutation for adult-onset ALXDRD.