NMC case report journal最新文献

Older patients represent a unique and vulnerable subgroup, requiring careful consideration when determining treatment options. Treatment-related leukoencephalopathy is commonly observed in older patients months to years after receiving radiotherapy and/or methotrexate for primary central nervous system lymphoma (PCNSL). Tirabrutinib is an orally available, highly selective, and potent second-generation Bruton's tyrosine kinase inhibitor (BTKi) approved for treating recurrent/refractory PCNSL in Japan. However, limited studies evaluate the imaging findings in patients receiving tirabrutinib. In this study, we reported 2 cases of older patients with PCNSL who did not develop treatment-related neurotoxicity or leukoencephalopathy after long-term administration of tirabrutinib.

Shunt dependence syndrome is a serious long-term complication characterized by symptoms and signs of increased intracranial pressure with normal-sized lateral ventricles after several years of arachnoid cyst-peritoneal shunting. It is easy to misdiagnose and overlook when combined with sinus stenosis, thus delaying treatment. Here, we present a 35-year-old man with an unexplained headache and binocular horizontal diplopia with high intracranial pressure. Based on stenosis of the right transverse sinus and absent filling of the left transverse sinus found by cerebral angiography, we first suspected cerebral venous sinus thrombosis (CVST). However, the treatment of anticoagulation and enhanced dehydration did not work. Twenty years ago, he had a cyst-peritoneal (CP) shunt performed for an asymptomatic arachnoid cyst. Finally, neurosurgeons ruled out CVST by direct retrograde cerebral venography and diagnosed shunt dependence syndrome. All his symptoms resolved well after ventriculoperitoneal (VP) shunt treatment. This case reminds pediatrics and neurology colleagues to consider shunt dependence syndrome first when someone has a history of CP shunting of arachnoid cysts presented with a severe headache, even if imaging shows a thin or occlusive venous sinus. Once high intracranial pressure secondary to shunt dependence is diagnosed, timely reconstruction of the cerebrospinal fluid (CSF) pathway is recommended rather than osmotic therapies because cerebral herniation formation will be life-threatening if ongoing increased intracranial pressure is not relieved.

Ganglioglioma, a glioneuronal neoplasm, typically presents in adolescents' temporal lobes. While pediatric brainstem gangliogliomas (BSGGs) are well documented, adult BSGGs are limited, resulting in a lack of comprehensive understanding of their pathophysiology and prognosis. A 41-year-old woman who presented with dizziness and numbness in her right upper extremity and right face underwent radiological examination. A dorsally exophytic tumor in the medulla oblongata was identified. Moderate uptake of ¹¹C-methionine on positron emission tomography suggests a low-grade glioma. Histopathological evaluation, following a suboccipital craniotomy and biopsy under neuromonitoring, confirmed the tumor as a mix of neoplastic ganglion cells and glial cells, which exhibited immunoreactivity for chromogranin A and OLIG2, respectively. Eosinophilic granular bodies and Rosenthal fibers were also observed. These findings confirmed the diagnosis of a ganglioglioma. The BRAF V600E mutation tested negative by real-time polymerase chain reaction. No postoperative adjuvant treatment was administered, and no progression of the residual tumor was noted 34 months post-surgery. Increased reporting of adult BSGGs, complete with detailed radiological, molecular, and genetic profiles, as well as their clinical course, is essential for clarifying our understanding of this rare entity's oncogenic pathway, optimal management strategy, and prognosis.

We report a case of persistent consciousness disturbance due to non-convulsive status epilepticus (NCSE) following a successful mechanical thrombectomy (MT). A 98-year-old female with atrial fibrillation presented with impaired consciousness and right hemiparesis 6 hrs after her last known well state. Magnetic resonance angiography revealed occlusion of the left internal carotid artery, necessitating MT to achieve complete recanalisation. Following admission, her consciousness showed no signs of improvement, and electroencephalography (EEG) revealed NCSE as the underlying cause. Levetiracetam treatment led to improvement in both clinical symptoms and EEG findings. This case highlights the importance of prompt diagnosis and management of NCSE in patients with ischaemic stroke who have persistent consciousness disturbance even after MT.

Intradural lumbar disc herniation (ILDH) is a very rare condition, with cerebrospinal fluid (CSF) leakage as a postoperative complication. The central canal of the conus medullaris was reported to communicate with the subarachnoid space through a caudal aperture; however, this aperture has never been observed in vivo. Herein, we report a case of L1/2 ILDH with postoperative spinal adhesive arachnoiditis and syringomyelia in which the communication considered to be a caudal aperture was detected. A 67-year-old woman complained of acute lower back pain and right leg pain with muscle weakness. Magnetic resonance imaging (MRI) revealed a large mass in the spinal canal at the L1/2 level. Intraoperatively, no herniated fragments were found in the epidural space. Thus, ILDH was suspected, and an intradural examination revealed tightly adherent herniated fragments in the swollen cauda equina and surrounding neovascularization. Postoperatively, symptoms improved once; however, 3 months later, severe bilateral leg muscle weakness and urinary retention developed. She was diagnosed with postoperative syringomyelia by MRI, and myelography and follow-up computed tomography was performed. Immediately after the administration of nonionic contrast agents, the syrinx of conus and epiconus were delineated through the central canal, and the communication between the central canal and the subarachnoid space could be visualized. Symptoms improved after syringo-subarachnoid shunt and duroplasty. It is likely that when CSF perfusion was impaired because of adhesive arachnoiditis around the conus medullaris, the central canal, which had been obstructed with growth, reopened, and the patient presented with syringomyelia.

Moyamoya disease (MMD) is characterized by distinct histopathological changes in intracranial arteries, such as narrowing of the arterial lumen due to thickening of the tunica intima, waving of the internal elastic membranes, and thinning of the tunica media. Ring finger protein 213 (RNF213) is a susceptibility gene for MMD that affects clinical outcomes. However, little is known about its relationship with histopathology. Herein, we present the postmortem histopathological evaluation of 2 MMD cases with and without RNF213 p.R4810K variant. The first patient was a 39-year-old female, with a history of undergoing bilateral indirect revascularization at the age of 10 years, who died from brainstem ischemic stroke. The second patient was a 32-year-old female who was conservatively treated from the age of 14 years but died from intraventricular hemorrhage. Postmortem analysis revealed that the first patient had a homozygous variant, whereas the second patient lacked this variant. Both cases exhibited similar advanced stages of MMD with posterior artery involvement on magnetic resonance angiography. However, compared with the second patient without the RNF213 p.R4810K variant, the first patient with this homozygous variant showed more prominent histopathological changes, such as narrowing of the arterial lumen due to thickening of the tunica intima, waving of the internal elastic membranes, and thinning of the tunica media. Although an accumulation of cases is required to draw a definite conclusion, these cases suggest that RNF213 p.R4810K variant status may affect the vascular histopathology of MMD and its clinical outcome.

Persistent primitive olfactory arteries (PPOAs) are a rare variant of the anterior cerebral artery (ACA). Cerebral aneurysms may arise in the PPOA; most are saccular and on the unilateral PPOA. We report a 66-year-old male with bilateral PPOAs and a fusiform aneurysm on the left side detected at a health check-up. A brain magnetic resonance imaging (MRI) scan revealed a fusiform dilation in the proximal portion of the left ACA on a brain MRI. Good surgical results were obtained by combining trapping-and bonnet bypass surgery. Brain MRI and cerebral angiograms showed bilateral PPOAs and a fusiform aneurysm with the pearl-and-string sign in the proximal portion of the left PPOA. The aneurysm was trapped and a bonnet bypass using a radial artery (RA) graft was placed between the left superficial temporal artery and the distal portion of the left PPOA. The postoperative course was uneventful and 30 months after surgery he had no neurological symptoms; MRA showed no recurrence. In this patient, aneurysmal trapping and an A3-A3 bypass were an option, however, it would have placed an additional load on the right PPOA. Our decision to trap the aneurysm and perform bonnet bypass surgery using an RA graft led to success.

Intracranial cystic lesions such as hemangioblastoma (HB) are commonly found incidentally; however, they can be difficult to diagnose because they require various differential diagnoses. A contrast-enhanced mural nodule on magnetic resonance imaging (MRI) is typical and can be diagnosed preoperatively; however, some small nodules cannot be visualised and only cysts may be seen, complicating preoperative diagnosis. In such cases, thorough observation of the cysts is necessary for a definitive diagnosis. To achieve this, minimally invasive surgery, such as endoscopic keyhole surgery, is required. Herein, we report the case of a man in his 50s who presented with an unstable gait, and experienced dizziness for several months. Preoperative MRI revealed a cystic lesion in the left cerebellar hemisphere, without a mural nodule. Although there was no diagnostic evidence of HB, we suspected that the symptoms were caused by this cystic lesion because of its recent occurrence. Upon detecting a mural nodule, we diagnosed it as a cerebellar HB and completely resected it using an endoscopic keyhole approach. The patient's symptoms alleviated postoperatively. The endoscopic keyhole approach may be useful as a less invasive procedure for diagnosing and removing cystic cerebellar HBs, especially for lesions that are difficult to diagnose using preoperative imaging.

Hemifacial spasm (HFS) is a disorder that causes involuntary movements of the ipsilateral facial muscles because of vascular compression of the facial nerve. Microvascular decompression (MVD), a surgical procedure to detach the culprit vessel from the nerve is believed to be the most effective treatment for HFS. Nevertheless, in the rare case in which the vessel penetrates the nerve, positioning the vessel sufficiently far from the nerve is challenging. In this report, a case of right HFS in a 54-year-old man with an anterior inferior cerebellar artery that had penetrated the nerve fiber cleft between the facial nerve and the nervus intermedius is presented. In the present case, the patient achieved relief from spasm, and postoperative facial hemiplegia was prevented by splitting the cleft slightly, gently moving the vessel, and affixing it to the petrous part of the temporal bone. The anatomic relationship between the facial nerve and the offending vessel must be properly understood, which must be carefully manipulated in such "penetration-type" HFS cases.

Although rare, penetrating cervical vascular injury poses significant challenges with a poor patient prognosis, often attributed to severe hemorrhage and accompanying injuries. We encountered a case of hemorrhagic shock resulting from a penetrating injury to the common carotid artery (CCA), which was successfully managed using a combination of endovascular therapy and direct surgical intervention. A 23-year-old man presented with a self-inflicted stab wound on the left side of his neck from a kitchen knife. This injury resulted in hemorrhagic shock and coma. Initial management included fluid resuscitation and transfusion, with continuous manual compression to control profuse bleeding. Contrast-enhanced neck and brain computed tomography (CT) showed a left CCA injury that was challenging to directly address due to its low anatomical location and continuous bleeding. Neurosurgeons initiated endovascular treatment by placing a guiding balloon catheter proximal to the left CCA for occlusion. Despite this, pulsatile bleeding persisted, prompting the distal placement of a balloon catheter to achieve better bleeding control. Cardiovascular surgeons then performed direct surgery, clearly identifying damage to the anterior and posterior walls of the CCA. The repair involved the use of a self-made cylindrical bovine pericardial patch, which resulted in successful revascularization. Following surgery, the patient regained independent mobility and was discharged. This case emphasizes the importance of a prompt and reliable approach involving endovascular intervention for initial bleeding control followed by precise repair through direct surgery, especially in challenging bleeding scenarios.