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.

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.

Formation of a Large Fusiform Aneurysm near a Medullary Infarction Caused by Dissection of the Posterior Inferior Cerebellar Artery.

Infarction of the posterior inferior cerebellar artery (PICA) can lead to ischemic stroke in the lateral medullary oblongata. PICA dissection can also elicit an ischemic event in this region, but its detection on radiological images is difficult because of the small diameter of the vessel. We report a case of Wallenberg syndrome due to PICA dissection in a 48-year-old man, which was difficult to diagnose on first admission. He reported sudden onset of sensory disturbance on the right side of his face, ataxic gait, and headache. Brain magnetic resonance imaging (MRI) revealed a fresh cerebral infarct in the right lateral medulla oblongata. Magnetic resonance angiography (MRA) performed at the time of his admission showed no cerebral vessel abnormalities. An MRI study 18 months after the event revealed a fusiform aneurysm on the lateral medullary segment of the PICA, which was extremely close to the cerebral infarct. We concluded that the infarct was due to PICA dissection because of the sudden onset of symptoms and because the infarcted territory of the occluded penetrating branch of the dissecting aneurysm was consistent with Wallenberg syndrome. The aneurysm was trapped and an occipital artery-PICA bypass was placed. At the latest follow-up, 1 year after the procedure, he had no neurological symptoms. Imaging findings at the time of his first admission indicated that the PICA was intact. However, 18 months later, MRI revealed enlargement of an aneurysm at the site of the dissection. A cerebral infarct with headache may indicate PICA dissection.

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.

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.

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.

Radiological correlates of pseudobulbar affect: Corticobulbar and cerebellar components in primary lateral sclerosis.

INTRODUCTION: Pseudobulbar affect (PBA) is a distressing symptom of a multitude of neurological conditions affecting patients with a rage of neuroinflammatory, neurovascular and neurodegenerative conditions. It manifests in disproportionate emotional responses to minimal or no contextual stimulus. It has considerable quality of life implications and treatment can be challenging. METHODS: A prospective multimodal neuroimaging study was conducted to explore the neuroanatomical underpinnings of PBA in patients with primary lateral sclerosis (PLS). All participants underwent whole genome sequencing and screening for C9orf72 hexanucleotide repeat expansions, a comprehensive neurological assessment, neuropsychological screening (ECAS, HADS, FrSBe) and PBA was evaluated by the emotional lability questionnaire. Structural, diffusivity and functional MRI data were systematically evaluated in whole-brain (WB) data-driven and region of interest (ROI) hypothesis-driven analyses. In ROI analyses, functional and structural corticobulbar connectivity and cerebello-medullary connectivity alterations were evaluated separately. RESULTS: Our data-driven whole-brain analyses revealed associations between PBA and white matter degeneration in descending corticobulbar as well as in commissural tracts. In our hypothesis-driven analyses, PBA was associated with increased right corticobulbar tract RD (p = 0.006) and decreased FA (p = 0.026). The left-hemispheric corticobulbar tract, as well as functional connectivity, showed similar tendencies. While uncorrected p-maps revealed both voxelwise and ROI trends for associations between PBA and cerebellar measures, these did not reach significance to unequivocally support the "cerebellar hypothesis". CONCLUSIONS: Our data confirm associations between cortex-brainstem disconnection and the clinical severity of PBA. While our findings may be disease-specific, they are consistent with the classical cortico-medullary model of pseudobulbar affect.

Medullary branches of the vertebral artery: microsurgical anatomy and clinical significance.

BACKGROUND: Since the medullary arteries are of a great neurologic and neurosurgical significance, the aim was to perform a detailed microanatomic study of these vessels, as well as of the medullary infarctions in a group of patients. METHODS: The arteries of 26 halves of the brain stem were injected with India ink and gelatin, microdissected and measured with an ocular micrometer. Neurologic and magnetic resonance imaging (MRI) examinations were performed in 11 patients. RESULTS: The perforating medullary arteries, averaging 6.7 in number and 0.26 mm in diameter, most often originated from the anterior spinal artery (ASA), and rarely from the vertebral (VA) (38.5%) and the basilar artery (BA) (11.6%). They supplied the medial medullary region. The anterolateral arteries, 4.8 in number and 0.2 mm in size, most often arose from the ASA and PerfAs, and nourished the anterolateral region. The lateral arteries, 2.2 in number and 0.31 mm in diameter, usually originated from the VA and the posterior inferior cerebellar artery (PICA). They supplied the lateral medullary region. The dorsal arteries, which mainly arose from the PICA and the posterior spinal artery (PSA), nourished the dorsal region, including the roof of the 4th ventricle. The anastomotic channels, averaging 0.3 mm in size, were noted in 42.3%. Among the medullary infarctions, the lateral ones were most frequently present (72.8%). CONCLUSION: The obtained anatomic data, which can explain the medullary infarctions symptomatology, are also important in order to avoid damage to the medullary arteries during neurosurgical and neuroradiologic interventions.

Bilateral medial medullary infarction with intravenous thrombolytic therapy: A case report.

RATIONALE: Bilateral medial medullary infarction is a rare stroke subtype. To investigate its clinical manifestations, etiology, imaging features and thrombolytic effect, We here in reported a patient with acute ischemic stroke in the bilateral medial medullary and reviewed the related literatures. PATIENTS CONCERN: A 64-year-old female was taken to our hospital after 4.5 hours of dizziness in the morning, followed by somnolence and limb weakness. She gradually worsened into a rapidly progressive tetraparesis and slurred speech. DIAGNOSES: Diffusion weighted imaging exhibited a "heart appearance" sign in bilateral medial medulla oblongata, and high-resolution magnetic resonance imaging suggested the left vertebral artery-4 thromboembolism. INTERVENTIONS: Timely intravenous thrombolysis was performed. OUTCOME: After intravenous thrombolysis, the patient's symptoms did not worsen in a short time. Although the symptoms were aggravated in the later stage, they were alleviated after active treatment. LESSONS: Diffusion weighted imaging can assist in the early diagnosis of bilateral medial medullary infarction, which will help in the decision to proceed with intravenous thrombolysis therapy. High-resolution magnetic resonance imaging should be improved as soon as possible, which can provide basis for the next intravascular interventional therapy.

[Varicella-Zoster Myelitis with Medullary Infarction: A Case Report].

We present the case of a 62-year-old woman who was receiving treatment for herpes zoster and experienced paraplegia, and bladder and bowel disturbance. The brain MRI diffusion-weighted image showed an abnormal hyperintense signal and apparent diffusion coefficient decreased in left medulla oblongata. The spinal cord MRI T2-weighted image showed abnormal hyperintense lesions in the left side of cervical spinal cord and thoracic spinal cord. We diagnosed varicella-zoster myelitis with medullary infarction, because varicella-zoster virus DNA was detected in the cerebrospinal fluid by polymerase chain reaction. The patient recovered with early treatment. This case shows the importance of evaluating not only skin lesions, but also distant lesions. (Received 15 November, 2022; Accepted 12 Jaunuary, 2023; Published 1 March, 2023).

Midline Suboccipital Unilateral Trans-Cerebellomedullary Fissure Approach for Clipping of Ruptured VA-PICA Aneurysm: Two-Dimensional Operative Video.

The vertebral artery-posterior inferior cerebellar artery (VA-PICA) aneurysm poses a technical challenge for microsurgical clipping due to its anatomical complexity, which requires dissection of lower cranial nerves. Endovascular treatment is regarded as a feasible first-line therapeutic option for VA-PICA aneurysm because it has an acceptable aneurysm occlusion rate and is less invasive. However, microsurgical clipping remains an effective treatment option. We present the case of a 62-year-old man who presented with subarachnoid hemorrhage (SAH) due to a ruptured VA-PICA aneurysm. Neuroradiologic examination revealed a 2-3 mm medially pointing left VA-PICA aneurysm with acute obstructive hydrocephalus due to massive SAH in the posterior cranial fossa. As the patient had acute obstructive hydrocephalus and a relatively small aneurysm, we selected clipping over endovascular treatment. Because the aneurysm was located close to the midline and anterolateral to the medulla oblongata, we approached it from the midline. A midline suboccipital craniotomy, C1 laminectomy, and drilling of the left condylar fossa were performed; a unilateral cerebellomedullary fissure opening was added; and the aneurysm was clipped. Postoperative neuroradiologic examinations revealed complete obliteration of the aneurysm. As shown in this video, unilateral cerebellomedullary fissure opening combined with adequate removal of the condylar fossa provides a wide operative field in the cerebellomedullary cistern while avoiding strong retraction of the cerebellum. We believe that this technique makes VA-PICA aneurysm clipping safe and successful. Patient consent was obtained to perform the surgery and to publish the surgical video (Video 1).