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.

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.

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).

Successful microvascular decompression surgery for dolichoectatic vertebral artery compression of medulla oblongata in a patient with hypersomnia disorder.

INTRODUCTION: Hypersomnia is a condition in which a person has trouble staying awake during the day. There are several potential causes of it, including sleep apnea and sleep disorders. CASE PRESENTATION: A 43 year old male was referred to our practice with complaints of hypersomnia, snoring, slurred speech and sleep apnea for more diagnostic and therapeutic interventions. His brain MRI was significant for a vascular loop compression on medulla oblongata. The patient underwent microvascular decompression surgery subsequently and showed improvement in all of his symptoms. CONCLUSION: One of the rare causes of sleep apnea is medulla oblongata compression by a vascular loop. Vertebrobasilar dolichoectasia may cause this phenomenon probably and should be reviewed in imaging examinations more precisely. Microvascular decompression by using a synthetic Teflon patch may be helpful in management of these patients.

A taxonomy for brainstem cavernous malformations: subtypes of medullary lesions.

OBJECTIVE: Medullary cavernous malformations are the least common of the brainstem cavernous malformations (BSCMs), accounting for only 14% of lesions in the authors' surgical experience. In this article, a novel taxonomy for these lesions is proposed based on clinical presentation and anatomical location. METHODS: The taxonomy system was applied to a large 2-surgeon experience over a 30-year period (1990-2019). Of 601 patients who underwent microsurgical resection of BSCMs, 551 were identified who had the clinical and radiological information needed for inclusion. These 551 patients were classified by lesion location: midbrain (151 [27%]), pons (323 [59%]), and medulla (77 [14%]). Medullary lesions were subtyped on the basis of their predominant surface presentation. Neurological outcomes were assessed according to the modified Rankin Scale (mRS), with an mRS score ≤ 2 defined as favorable. RESULTS: Five distinct subtypes were defined for the 77 medullary BSCMs: pyramidal (3 [3.9%]), olivary (35 [46%]), cuneate (24 [31%]), gracile (5 [6.5%]), and trigonal (10 [13%]). Pyramidal lesions are located in the anterior medulla and were associated with hemiparesis and hypoglossal nerve palsy. Olivary lesions are found in the anterolateral medulla and were associated with ataxia. Cuneate lesions are located in the posterolateral medulla and were associated with ipsilateral upper-extremity sensory deficits. Gracile lesions are located outside the fourth ventricle in the posteroinferior medulla and were associated with ipsilateral lower-extremity sensory deficits. Trigonal lesions in the ventricular floor were associated with nausea, vomiting, and diplopia. A single surgical approach was preferred (> 90% of cases) for each medullary subtype: the far lateral approach for pyramidal and olivary lesions, the suboccipital-telovelar approach for cuneate lesions, the suboccipital-transcisterna magna approach for gracile lesions, and the suboccipital-transventricular approach for trigonal lesions. Of these 77 patients for whom follow-up data were available (n = 73), 63 (86%) had favorable outcomes and 67 (92%) had unchanged or improved functional status. CONCLUSIONS: This study confirms that the constellation of neurological signs and symptoms associated with a hemorrhagic medullary BSCM subtype is useful for defining the BSCM clinically according to a neurologically recognizable syndrome at the bedside. The proposed taxonomical classifications may be used to guide the selection of surgical approaches, which may enhance the consistency of clinical communications and help improve patient outcomes.

Heart in the brain: classical imaging sign in bilateral medial medullary infarction.

Bilateral medial medullary infarction is a rare type of posterior fossa stroke due to occlusion of blood supply to the anteromedial aspect of the medulla. It can be diagnosed by its classical imaging appearance - the "heart sign".

Application of postmortem MRI for identification of medulla oblongata contusion as a cause of death: a case report.

Whiplash injury is common in traffic accidents, and severe whiplash is characterized by cervical spinal cord injuries with cervical dislocation or fracture, that can be diagnosed by postmortem computed tomography (PMCT), postmortem magnetic resonance (PMMR), or conventional autopsy. However, for cervical spinal cord injury without fracture and dislocation, PMMR can be more informative because it provides higher resolution of soft tissues. We report the case of a 29-year-old male who died immediately following a traffic accident, in which the vehicle hit an obstacle at a high speed, causing deformation of the bumper and severe damage of the vehicle body. PMCT indicated no significant injuries or diseases related to death, but PMMR showed patchy abnormal signals in the medulla oblongata, and the lower edge of the cerebellar tonsil was herniated out of the foramen magnum. The subsequent pathological and histological results confirmed that death was caused by medulla oblongata contusion combined with cerebellar tonsillar herniation. Our description of this case of a rare but fatal whiplash injury in which there was no fracture or dislocation provides a better understanding of the potentially fatal consequences of cervical spinal cord whiplash injury without fracture or dislocation and of the underlying lethal mechanisms. Compared with PMCT, PMMR provides important diagnostic information in forensic practice for the identification of soft tissue injuries, and is therefore an important imaging modality for diagnosis of whiplash injury when there is no fracture or dislocation.

Mixed Germ Cell Tumor with a Yolk Sac Tumor Component in the Medulla Oblongata of a 50-year-old Patient: A Case Report and Literature Review.

Yolk sac tumor (YST) is a rare primary brain tumor that occurs almost exclusively in patients under 30 years old. Intracranial germ cell tumors are most frequently located in the pineal and suprasellar region. Medulla oblongata YSTs are particularly rare. Extragonadal YSTs may be difficult to diagnose because of their characteristics, such as the rarity and variety of growth patterns. Furthermore, they are known to have a very poor prognosis. We herein report a case of YST of the medulla oblongata in a 50-year-old woman. She was followed up for 18 months without any tumor recurrence.

A case of craniocervical junction pial arteriovenous fistula causing postoperative medullary and spinal cord edema.

OBJECTIVES: Pial arteriovenous fistulas (pAVFs) are direct connections between the pial artery and vein without an intervening nidus. We report a rare case of craniocervical junction (CCJ) pAVF causing medullary and spinal cord edema resulting from surgical removal of the varix with remnant shunt after coil embolization. CASE DESCRIPTION: A 16-year-old man presented with subarachnoid hemorrhage. Digital subtraction angiography revealed a CCJ pAVF with multiple fistulas at the 2 varices (varix A and varix B), which was fed by the bilateral lateral spinal arteries and anterior spinal artery (ASA), and drained into the median posterior vermian vein with varix (varix C) and anterior spinal vein (ASV). Varices A and B were embolized using coils, but the shunts remained in varix C. Then, varix C was surgically removed. After this operation, medullary and spinal cord edema occurred. Digital subtraction angiography showed the ASV drainage responsible for edema. Finally, surgical removal of varices A and B was performed. However, arteriovenous shunts, supplied by the ASA and drained into the ASV via the intrinsic vein, were found in the medulla oblongata and coagulated, resulting in disappearance of edema. CONCLUSIONS: Edema was probably caused by concentration of drainage from the arteriovenous shunt in the medulla oblongata into the ASV by surgical removal of varix C acting as another draining route. High flow AVF can induce angiogenesis and secondary arteriovenous shunt. Precise analysis of the angioarchitecture is important to treat such cases without complications.