Tirabrutinib, an inhibitor of Bruton's tyrosine kinase-a hub gene for primary central nervous system lymphoma proliferation-is a novel therapeutic agent for relapsed and refractory primary central nervous system lymphoma. We report a case of severe Aspergillus encephalitis following tirabrutinib administration. A man in his 50s suddenly presented with left sided hemiparesis, and neuroimaging suggested a primary central nervous system lymphoma in the right parietal lobe. The results of the tissue biopsy revealed a diagnosis of CD20-positive diffuse large B-cell lymphoma. Despite treatment with high-dose methotrexate combined with rituximab, neurological symptoms rapidly deteriorated. We suspected that the growth of the tumor led to an impending brain herniation, so we planned radiation therapy. However, the patient contracted coronavirus disease 2019. Therefore, we decided to administer tirabrutinib, which can be administered orally. The tumor shrank dramatically, and improvement in neurological symptoms was observed. However, due to onset of liver dysfunction, tirabrutinib was discontinued. Immediately thereafter, the primary central nervous system lymphoma began to progress again, necessitating the re-administration of tirabrutinib. Concurrently, a cerebral abscess developed in the basal ganglia. Subsequently, a fatal subcortical hemorrhage occurred. Brain tissue obtained during hematoma evacuation led to a histological diagnosis of Aspergillus cerebral vasculitis. We started treatment with voriconazole first, and then added amphotericin B, but multiple cerebral abscesses developed and progressed to encephalitis, resulting in death. Burton's tyrosine kinase is a gene crucial for the general antifungal immunity, and when using Burton's tyrosine kinase inhibitors, physician should always be mindful of preventing, detecting early, and addressing fungal infections to avoid fatal adverse events like in this case.
Pleomorphic xanthoastrocytoma is a rare brain tumor that frequently harbors the oncogenic BRAF V600E mutation. Approximately 28.6%-47% of high-grade pleomorphic xanthoastrocytomas are associated with TERT promoter mutation and leptomeningeal dissemination, for which no established treatment exists and the prognosis remains poor. Combination therapy with BRAF and MEK inhibitors has demonstrated efficacy in BRAF V600E-mutant brain tumors. We report a case of a 22-year-old man with a right temporal lobe tumor initially diagnosed as World Health Organization grade 2 pleomorphic xanthoastrocytoma after gross total resection. Two years later, the tumor recurred and underwent malignant transformation to World Health Organization grade 3 pleomorphic xanthoastrocytoma. At the third resection, pathological and genomic analyses confirmed BRAF V600E mutation together with TERT promoter mutation. Following chemoradiotherapy, spinal leptomeningeal dissemination developed. After spinal irradiation, dabrafenib plus trametinib was initiated, resulting in partial radiological response and symptomatic improvement. Although regrowth occurred 10 months after initiation of targeted therapy, the patient remains alive at the time of writing. Here, we report a case of recurrent anaplastic BRAF V600E-mutant pleomorphic xanthoastrocytoma with leptomeningeal dissemination that showed a transient but clinically meaningful response to combined BRAF-MEK inhibition and spinal radiation therapy. In addition, this case raises the possibility of an association between TERT promoter mutation and leptomeningeal dissemination, although further studies are required to clarify this relationship.
Vertebral compression fractures (VCF) after radiation therapy (RT) are uncommon, particularly in the mid- and lower cervical spine. Surgical management of symptomatic cases can be challenging. Here, we report the case of a 63-year-old woman with radiculomyelopathy and C7 VCF. The patient presented with neck pain and bilateral hand numbness without osteoporosis. Four years previously, she had been cured of esophageal cancer through endoscopic surgery and RT without chemotherapy. Imaging revealed a pathological C7 vertebral body fracture with kyphotic deformity. Symptomatic VCF was diagnosed. Because of concerns regarding complications with an anterior approach, posterior-only decompression and fixation were performed. Intraoperative biopsy at C7 vertebra confirmed a diagnosis of avascular necrosis without metastasis. Fixation was performed by inserting lateral mass screw at C5-, 6 and pedicle screw at T1-, 2, connected by a rod. A multipoint screw was used to enhance rostral stability. The symptoms were alleviated postoperatively, and the patient remained well at 2-year follow-up.
Intracranial dermoid cysts are rare congenital lesions of ectodermal origin. Those arising in the Sylvian fissure are particularly rare, and rupture in this location has only infrequently been reported. A 54-year-old woman presented with a persistent headache after minor head trauma. Magnetic resonance imaging demonstrated a well-circumscribed 30-mm lesion in the left Sylvian fissure with heterogeneous hyperintensity on T1-weighted images. Multiple disseminated hyperintense droplets within the basal cisterns suggested cyst rupture. The patient underwent microsurgical resection via a trans-Sylvian approach. Intraoperatively, the cyst wall was partially deficient and adherent to the surrounding vessels. Owing to dense calcification of the wall and firm adhesion to adjacent neurovascular structures, the capsule was left in place, and the cyst contents were evacuated and curetted, followed by thorough irrigation. Histopathological examination confirmed a dermoid cyst. The postoperative course was uneventful, and no recurrence has been observed during 9 months of follow-up. Ruptured dermoid cysts in the Sylvian fissure are exceedingly rare. Recognition of disseminated lipid droplets is crucial for diagnosis, and safe resection with preservation of critical neurovascular structures is essential for favorable outcomes.
Aneurysms associated with arteriovenous malformations occur in up to 20% of cases, but distal anterior inferior cerebellar artery aneurysms associated with arteriovenous malformations are extremely rare. We report a case of a ruptured distal anterior inferior cerebellar artery aneurysm associated with a cerebellar arteriovenous malformation in a 71-year-old man presenting with subarachnoid hemorrhage. Digital subtraction angiography revealed a cerebellar arteriovenous malformation supplied mainly by the right anterior inferior cerebellar artery and a fusiform aneurysm located at the meatal loop within the internal auditory canal. The aneurysm was considered the most likely rupture point based on the hemorrhage distribution and angiographic findings. Endovascular treatment was attempted to prevent re-rupture. Due to challenging distal catheterization and unattainable complete coil embolization of the aneurysm, the aneurysm and a short proximal segment of the parent artery were occluded. Postoperatively, the patient developed a mild hearing loss without brainstem infarction. Subsequent arteriovenous malformation resection via lateral suboccipital craniotomy led to complete obliteration. Parent artery occlusion reduced arteriovenous malformation flow and served as preoperative embolization to facilitate safe surgical removal. This case highlights the importance of individualized treatment strategies for rare distal anterior inferior cerebellar artery aneurysms associated with arteriovenous malformations and suggests that parent artery occlusion with subsequent staged arteriovenous malformation resection can be an effective approach.
The trigone of the lateral ventricle is surrounded by important white matter fibers, making it difficult to approach. White matter fibers, such as the superior longitudinal fasciculus, optic radiation, arcuate fasciculus, and inferior fronto-occipital fasciculus, can be clearly depicted by tractography. We propose the safest approach to a trigonal tumor of the lateral ventricle via the superior parietal lobe using tractography. Case: A 49-year-old female presented to our hospital with a chronic headache. Magnetic resonance imaging revealed a meningioma with partial hydrocephalus in the trigone of the left lateral ventricle. After total gross resection, the patient exhibited no cranial nerve, visual, sensorimotor, or language deficits. However, there was a mild acalculia right after surgery, from which the patient made an excellent recovery with no neurological deficits at 3 months after surgery. The endoport-assisted superior parietal lobe trans-sulcus approach using tractography is the safest approach to the trigonal lesion, as it protects the most important white matter fibers around the lateral ventricular trigone.
Inflammatory pseudotumor involving the cranial nerves is extremely rare, and delayed diagnosis may cause irreversible neurological deficits. To our knowledge, this represents the third reported case of an inflammatory pseudotumor involving the cavernous sinus with extension along the oculomotor nerve. A 56-year-old woman presented with a visual field defect in her left eye, and her condition was initially managed conservatively because of her mild clinical presentation. Initial magnetic resonance imaging revealed a homogeneously enhanced dumbbell-shaped mass lesion extending from the left cavernous sinus through the superior orbital fissure into the intraconal orbital space. At 3-year follow-up, the patient's condition worsened during routine outpatient visits, with left visual acuity decreasing to light perception. Surgical biopsy revealed a fibrous mass involving the cisternal portion of left oculomotor nerve and extending into the cavernous sinus. Histopathological examination and laboratory findings confirmed the diagnosis of an immunoglobulin G4-related inflammatory pseudotumor. Oral corticosteroid therapy caused marked radiological regression of the lesion; however, visual impairment was not alleviated. This case highlights that delayed histopathological confirmation may produce irreversible neurological deficits despite a favorable radiological response to corticosteroid therapy.
Deep brain stimulation lead fracture may necessitate intracranial lead replacement. If deep brain stimulation was clinically effective before the fracture, reimplantation along the original trajectory to the same target is preferable; however, detailed technical descriptions remain limited. We present a 70-year-old woman with Parkinson disease who underwent subthalamic nucleus-deep brain stimulation. Two months after surgery, partial impedance abnormalities were detected on the left lead. At 12 months, all contacts demonstrated abnormal impedance, accompanied by worsening motor symptoms. Fourteen months after the initial surgery, we attempted to place a new lead by manually advancing it through the scar tissue that had formed along the original lead tract. Stereotactic reimplantation using a Leksell frame was prepared as a contingency if this scar-guided approach proved unsuccessful. After reopening the prior incision, the original lead was removed without resistance, and the cortical entry point was clearly identified. The preinserted stylet was removed from a new deep brain stimulation lead, which was then gently advanced through the original entry point along the pre-existing tract without reinsertion of the stylet. The lead progressed smoothly, and C-arm fluoroscopy confirmed that the tip corresponded to the prior position. Intraoperative test stimulation improved symptoms without adverse effects. Postoperative computed tomography confirmed that the reimplanted lead was positioned in an almost identical location to the prior lead. Stimulation was resumed with clinical benefit, and lead function remained normal at 6 months. Scar-guided reinsertion may represent a practical alternative; however, stereotactic backup should remain readily available, as adequate tract formation cannot be reliably predicted preoperatively.
Cervical calcification of the ligamentum flavum is a relatively rare disease that causes myelopathy due to calcification within the ligament. We recently encountered a patient with rapid progression of cervical calcification of the ligamentum flavum after anterior fixation surgery, resulting in spinal cord symptoms. An 81-year-old woman had a history of anterior decompression and fixation surgery (Williams-Isu method) at C3-4 and C5-6 at the age of 58 years, with no sequelae. Eighteen months preoperatively, the patient developed walking difficulties, arm pain, and weakness in both arms. Magnetic resonance imaging showed C4-5 anterior spinal cord compression, and C4-5 anterior decompression and fixation surgery were performed. Postoperatively, the patient's condition improved, and she was discharged from the hospital, able to walk unaided. However, 17 months postoperatively, the patient rapidly developed right arm pain, walking difficulties, and urinary and rectal incontinence. There was a rapid progression within 18 months in cervical calcification of the ligamentum flavum at the C6-7 level on magnetic resonance imaging and severe compression of the spinal cord; therefore, urgent laminectomy was performed. Postoperatively, the symptoms improved, and there was no recurrence for 3 years. In addition to the previously performed C3-4 and C5-6 fusion procedures, the addition of a C4-5 fusion resulted in a longer fusion spanning from C3 to C6, and it is thought that mechanical stress was concentrated on the adjacent intervertebral space of C6-7. Although the cause of cervical calcification of the ligamentum flavum has not been determined, this case strongly suggests that mechanical stimulation may exacerbate the condition.
Endodermal cysts typically occur in intradural regions of the ventral spine or posterior cranial fossa. However, they are rarely found in supratentorial regions, especially within the frontal bone. Here, we report a rare case of an intraosseous endodermal cyst in the frontal convexity and discuss its potential pathogenesis in this unusual location. A 54-year-old woman presented with a gradually enlarging, soft, elastic mass in the left frontal region. Neurological abnormalities were not observed. Computed tomography revealed a cystic lesion within the left frontal bone, associated with a bulging contour. The cyst contents appeared hyperintense on T1-weighted magnetic resonance and showed a hypointense component on T2-weighted magnetic resonance imaging. The cyst wall was not enhanced with gadolinium. The cyst and its mucinous content were completely excised from the frontal bone. Intraoperatively, the cyst wall was completely separated from the frontal sinus by a thin bony structure. Histopathological examination revealed that the cyst wall consisted of inflammatory granulation tissue with a ciliated columnar epithelium, consistent with a diagnosis of endodermal cyst. Although the pathogenesis of supratentorial endodermal cysts remains unclear, the location of the cyst in the frontal convexity, as opposed to the anterior cranial base, may suggest a distinct pathogenesis for each endodermal cyst in terms of embryological aspects.
A 50-year-old male who had undergone ventriculoperitoneal shunt for hydrocephalus following acoustic neurinoma surgery 18 months earlier presented with progressive fatigue and loss of appetite. Three months prior to visiting our hospital, he fell off his bicycle and sustained a skin laceration just above the tube on the right clavicle. He exhibited tenderness in the right upper quadrant but no other neurological symptoms. A contrast-enhanced abdominal computed tomography scan revealed a large well-defined lesion in the right lobe of the liver, with the shunt tube having migrated into the lesion. Ultrasound-guided percutaneous drainage was performed, resulting in a diagnosis of liver abscess. Culture of the drainage fluid confirmed the presence of methicillin-resistant Staphylococcus aureus, and vancomycin therapy was initiated. Later, an increased cell count, predominantly neutrophils, was observed in his cerebrospinal fluid. We then removed the entire shunt system and administered linezolid, after which the cerebrospinal fluid cell count normalized within almost one month. Liver abscesses as a complication of ventriculoperitoneal shunt are rare, with only 11 cases reported in the previous literature. This is possibly the first case associated with traumatic skin injury occurring long after ventriculoperitoneal shunt placement. With proper management of the shunt tube and administration of the appropriate antibiotic treatment, a favorable prognosis can be expected.
Neurogenic pulmonary edema is a rare but severe complication of acute central nervous system injury, such as subarachnoid hemorrhage, typically presenting with immediate onset. This report describes an uncommon case of delayed-onset neurogenic pulmonary edema following subarachnoid hemorrhage. A 68-year-old man with subarachnoid hemorrhage, caused by a ruptured left posterior communicating artery aneurysm, developed tachypnea and hypoxemia with bilateral pulmonary infiltrates 96 hours after symptom onset. Echocardiography was normal, ruling out cardiogenic causes, and neurogenic pulmonary edema was diagnosed. The patient improved rapidly with fluid restriction and diuretic therapy, and subsequently underwent successful aneurysm coil embolization. Neurogenic pulmonary edema is thought to result from an excessive sympathetic activation leading to pulmonary vasoconstriction, increased capillary pressure, and endothelial injury. Delayed cases may involve secondary sympathetic surges due to intracranial pressure fluctuations, vasospasm, or inflammatory injury. This case highlights the need for clinicians to consider delayed neurogenic pulmonary edema when respiratory deterioration occurs several days after subarachnoid hemorrhage to ensure timely diagnosis and life-saving management.
Isolated middle cerebral artery dissection is a rare cause of acute ischemic stroke, accounting for approximately 2.4% of anterior circulation events. Due to its rarity, no optimal management strategy has been established, and evidence regarding the safety and efficacy of endovascular therapy remains limited. We report a 26-year-old male who presented with sudden-onset headache, vomiting, right hemiparesis, and aphasia. Magnetic resonance imaging revealed multiple faint diffusion-restricted lesions in the left hemisphere (DWI-ASPECTS 7), and magnetic resonance angiography demonstrated occlusion of the left middle cerebral artery (M2 segment). Emergency mechanical thrombectomy using a combined stent retriever and aspiration technique achieved complete recanalization (Thrombolysis in Cerebral Infarction 3), although significant residual stenosis persisted. Balloon angioplasty restored satisfactory luminal patency without stent placement. Postoperative contrast-enhanced vessel wall imaging showed mural thickening and marked enhancement of the left M2 segment, confirming arterial dissection. The patient recovered well, and no infarct progression or re-occlusion occurred during the 6-month follow-up. The present case suggests that endovascular therapy employing mechanical thrombectomy as the first-line strategy may be one of the therapeutic options for acute middle cerebral artery occlusion secondary to arterial dissection. The procedural strategy and device selection should be individualized to minimize vessel injury. Furthermore, given the potential for dynamic vascular changes, long-term radiological surveillance remains essential.
Calvarial hemangiomas are rare benign vascular tumors originating from the skull bones, most commonly observed in the frontal and parietal bones of the cranium. Although most cases are asymptomatic, they can present with headache, cosmetic deformity, or, rarely, neurological deficit. The lesions are usually solitary; multicentric involvement and dural invasion are exceedingly rare phenomena. Here, we report a 22-year-old male patient who presented with frontal headache and a palpable scalp swelling. Imaging revealed two separate expansile lytic lesions in the right and left frontal bones with no connection between them. Surgical resection was planned. Intraoperatively, the lesion on the left side demonstrated findings highly suggestive of dural invasion, and both lesions were completely resected. This case highlights the rarity of the combination of multicentric involvement with dural invasion in the literature.
Posterior condylar canal dural arteriovenous fistula is rare, and cases of posterior condylar canal dural arteriovenous fistula directly related to trauma have been reported even less frequently. We describe a case of posterior condylar canal dural arteriovenous fistula that presented with tinnitus soon after head trauma and was successfully treated with transarterial embolization. A 60-year-old man presented with right-sided tinnitus that had persisted for 2 weeks after sustaining a right temporal injury. Digital subtraction angiography revealed a posterior condylar canal dural arteriovenous fistula with venous drainage from the posterior condylar vein to the suboccipital cavernous sinus, without cortical venous reflux. The shunt was supplied by a single feeder, the jugular branch of the occipital artery. Transarterial embolization using N-butyl cyanoacrylate and coils achieved complete occlusion of the shunt. The patient's tinnitus resolved immediately after the procedure, and follow-up angiography demonstrated persistent complete occlusion of the fistula, with no evidence of recurrence. Posterior condylar canal dural arteriovenous fistula is a rare lesion that may occur following head trauma. Bone-window three-dimensional angiographic reconstruction is useful for diagnosis, as it clearly delineates the relationship between the skull base canal and the shunt. Transarterial embolization can be a curative treatment option when performed with a thorough understanding of the relevant vascular anatomy and potentially hazardous anastomoses.
Detailed evaluation of language function is important in the management of dominant temporal lobe lesions. Although spoken language is routinely evaluated in patients, disturbances in written language may remain undetected without detailed assessment. The Japanese writing system, consisting of logographic Kanji and phonographic Kana, provides a unique framework for identifying selective impairments of the ventral language stream. We report a 51-year-old right-handed Japanese male who presented with selective agraphia for Kanji following a left posterior temporal subcortical hemorrhage. Upon admission, spontaneous speech was fluent, and auditory comprehension was preserved, with no clinically apparent aphasia. However, comprehensive neuropsychological assessment revealed a profound impairment in Kanji writing. Although standard language scores improved significantly by the chronic phase (Day 87), qualitative assessment revealed persistent deficits in Kanji retrieval, characterized by frequent non-responses and morphologically related errors. Conversely, Kana writing and reading abilities were largely preserved. This case demonstrates that selective impairment of written language (pure agraphia for Kanji) may occur in association with posterior temporal lesions, despite preserved spoken language. Unlike typical cortical lesions that destroy the orthographic representation itself, we propose that this specific subcortical hemorrhage caused a transient disconnection within the ventral language stream, selectively disrupting the semantic access pathway to Kanji. From a functional and clinical perspective, systematic evaluation of writing function is essential for neurosurgeons and clinicians to prevent overlooked disabilities.
Eight years after resection of hypopharyngeal carcinoma with neck dissection and ligation of the left internal jugular vein, a man in his 50s presented with an incidentally discovered isolated left transverse sinus dural arteriovenous fistula. Transvenous embolization was performed to achieve complete obliteration. The patient likely developed venous hypertension due to stenosis of the left transverse sinus, limiting contralateral drainage and the absence of collateral communication between the internal jugular vein and the suboccipital cavernous sinus. A retrospective review of serial imaging revealed progression of venous drainage from the initial development of transverse sinus dural arteriovenous fistula to an eventual isolated sinus configuration. Although dural arteriovenous fistula formation is associated with venous hypertension, this case suggests that anatomic venous variants may predispose individuals to delayed dural arteriovenous fistula formation, with abrupt alterations in venous flow serving as triggers. The serial imaging demonstrates the progressive nature of this pathology. Assessment of venous anatomy in patients undergoing planned occlusion of major venous pathways may help predict long-term complications.
Idiopathic normal pressure hydrocephalus is a disease of unknown cause that occurs in people aged 60 years or older and causes gait disturbance, cognitive decline, and urinary problems. A 63-year-old man with untreated diabetes presented with unsteady gait and amnesia. Head magnetic resonance imaging revealed disproportionately enlarged subarachnoid-space hydrocephalus and brain herniation into the para-superior sagittal sinus arachnoid granulation. Although the patient experienced generalized convulsions immediately before surgery, a lumbo-peritoneal shunt was performed, and symptoms improved. These brain herniations into the arachnoid granulation might accelerate the pathology, and idiopathic normal pressure hydrocephalus with brain herniation into the arachnoid granulation of the lateral lacuna of the superior sagittal sinus is extremely rare and has not been reported previously.
