NMC case report journal最新文献

Normal pituitary glands are usually found to be present in the periphery of tumors. Normal pituitary gland showing an intratumoral septal structure has not been sufficiently discussed. A 73-year-old man presented with a visual field disorder. Endocrinologically, his basal luteinizing hormone and follicle-stimulating hormone levels were elevated. Gadolinium-enhanced T1-weighted magnetic resonance imaging revealed a sellar tumor. A well-enhanced normal pituitary gland was found with a septum-like shape slightly to the right of the tumor in the coronal section, and the tumor seemed to have grown independently on the cavernous sinus and sellar sides. Endoscopic transsphenoidal surgery was performed. After removal of the main tumor on the left side, the normal pituitary gland wall was confirmed to be intact. We observed a crack-like hole in the septum-like structure of the pituitary gland. We removed the tumor on the right side using this hole in the septum-like normal pituitary gland. This case was thought to have arisen because the adenoma perforated the normal pituitary gland during its development and then progressed outward, which is different from a double pituitary adenoma. In such cases, we should locate the hole in the septum-like structure of the normal pituitary gland and remove the tumor laterally progressing from this hole during endoscopic surgery.

Cavernous sinus dural arteriovenous fistulas are generally considered to have a benign clinical course, as spontaneous thrombosis of the fistula or draining veins can result in regression of the lesion. However, we encountered a rare case in which this hemodynamic change led to rapid neurological deterioration, necessitating microsurgical intervention. A 72-year-old woman presenting with chemosis and exophthalmos was diagnosed with a cavernous sinus dural arteriovenous fistula. Cerebral angiography revealed 2 venous drainage routes: the superior ophthalmic vein anteriorly and the superior petrosal sinus towards the petrosal vein posteriorly. Approximately 2 weeks after diagnosis, while awaiting elective endovascular treatment, the patient developed spontaneous thrombosis of the superior ophthalmic vein. This redirected shunt flow exclusively into the petrosal vein, leading to venous congestion in the posterior fossa. Consequently, the patient experienced rapid neurological deterioration accompanied by brain edema in the cerebellum and brainstem. Furthermore, the loss of transvenous access rendered curative endovascular treatment unfeasible. The patient was therefore treated with urgent microsurgical interruption of the petrosal vein via a retrosigmoid approach. This resulted in complete neurological recovery and angiographic obliteration of the cavernous sinus dural arteriovenous fistulas. This case highlights the importance of close observation while waiting for the intervention for cavernous sinus dural arteriovenous fistulas with cortical venous reflux, given the risk of spontaneous hemodynamic change. It also underscores the role of microsurgical intervention as a salvage option when endovascular treatment becomes unfeasible.

Trans-sellar encephalocele is a rare subtype of basal encephalocele characterized by herniation of neural tissue through a defect in the sellar floor, extending into the sphenoid sinus or nasopharynx. Due to its deep anatomical location and proximity to critical neurovascular and endocrine structures, trans-sellar encephalocele presents significant diagnostic and surgical challenges. We report two adult cases of symptomatic trans-sellar encephalocele with progressive visual impairment, both successfully treated via endoscopic endonasal surgery. In addition, to contextualize these cases, we conducted a comprehensive literature review of 47 previously reported symptomatic trans-sellar encephalocele that was surgically treated in the neurosurgical field over the past quarter century, resulting in a total 49 patients (33 males, 16 females). Clinical characteristics, imaging findings, surgical approaches, and outcomes were analyzed. Of the 49 patients, the common symptoms included nasal obstruction (59.2%), endocrine dysfunction (46.9%), cerebrospinal fluid leakage (30.6%), and visual impairment (24.5%). The majority (69.4%) were diagnosed at age six or younger. Surgery was performed primarily via transnasal approaches (49.4%). Outcomes were available in 44 cases; 81.8% experienced symptom resolution or improvement. In our two cases, postoperative imaging confirmed the reduction of the trans-sellar encephalocele and cessation of cerebrospinal fluid inflow. Visual symptoms improved without recurrence or complications. Early diagnosis and tailored surgical intervention are essential for optimizing outcomes in trans-sellar encephalocele. The endoscopic endonasal approach enables safe and effective reduction of the trans-sellar encephalocele while preserving critical structures. Modifying of cerebrospinal fluid dynamics appears to play a key role in halting symptom progression. Long-term follow-up and further studies are warranted to establish standardized management strategies.

The skull base is an intricate interface between the cranium and the face. Both intracranial and extracranial pathologies can occur at this location. Although the tumor origin is clear in most cases, certain extracranial tumors may mimic primary intracranial pathologies. A 48-year-old woman with a history of resection of a right tentorial meningioma 17 years earlier presented with worsening right facial palsy. No obvious enlargement of the parotid gland was noted. Imaging revealed a residual tentorial meningioma and a homogenously enhancing mass extending alongside the right facial nerve. The patient underwent several biopsies, and the diagnosis of adenoid cystic carcinoma arising from the parotid gland was made. She subsequently underwent carbon-ion radiation therapy and remained stable for 28 months after surgery. The representative case reiterates the importance of evaluating both intracranial and extracranial pathologies when a clinician encounters skull base tumors, especially those with perineural spreading. The tendency of meningiomas to involve the trigeminal nerve while sparing the facial nerve may provide a clue for the differential diagnosis.

A 72-year-old woman with a history of breast cancer presented with left oculomotor nerve palsy. Magnetic resonance imaging revealed a progressive mass lesion in the cavernous sinus. Initially, Tolosa-Hunt syndrome and metastatic brain tumor from breast cancer were suspected; however, radiological differentiation proved challenging. Pathological examination confirmed the diagnosis of primary intracranial Ewing sarcoma. The tumor exhibited progressive growth, and Gamma Knife radiosurgery was performed. After treatment, tumor shrinkage and symptomatic improvement were observed. Ewing sarcoma typically occurs in children and young adults; however, the safety and efficacy of chemotherapy in older populations remain largely unstudied. In this older patient, the rare location of the tumor within the cavernous sinus posed challenges to surgical resection. Chemotherapy was administered at a reduced dose of 50%, with limited side effects. After 7 cycles of chemotherapy, tumor showed further shrinkage, and no recurrence was observed. This case demonstrates that, even in rare tumors with unestablished chemotherapy protocols for older patients, satisfactory outcomes can be achieved with accurate pathological diagnosis and a multidisciplinary treatment approach.

Dynamic assessment by computed tomography angiography or digital subtraction angiography is important for the diagnosis of vascular eagle syndrome, although these require the use of contrast media. Magnetic resonance angiography with neck flexion and extension, named dynamic magnetic resonance angiography, can dynamically evaluate the relationship between the internal carotid artery and the styloid process with no use of contrast media. A 74-year-old male with chronic kidney disease was rushed to our hospital due to recurrent infarction in the left cerebral hemisphere. Magnetic resonance angiography in the neutral neck position showed no abnormality in the left internal carotid artery, but computed tomography revealed bilateral elongated styloid process. Dynamic magnetic resonance angiography was performed to show that the left internal carotid artery was compressed by the styloid process during neck flexion but not during neck extension. Carotid ultrasound revealed a thrombus in the high cervical portion of the left internal carotid artery. Styloidectomy was performed, and cerebral infarction has not recurred since then. Dynamic magnetic resonance angiography may be a useful diagnostic modality without using contrast media for vascular eagle syndrome.

Moyamoya disease is a progressive steno-occlusive cerebrovascular disorder that may be complicated by intracranial aneurysms, particularly, in the posterior circulation, which are at higher risk of rupture than those in the general population. Clazosentan, a selective endothelin A receptor antagonist, has been approved in Japan for the prevention of cerebral vasospasm after aneurysmal subarachnoid hemorrhage. However, cerebral hemodynamics after subarachnoid hemorrhage and safety of clazosentan in patients with moyamoya disease remain unknown. We report the case of a 46-year-old man with bilateral moyamoya disease who presented with severe subarachnoid hemorrhage caused by a ruptured saccular aneurysm in the right P2 segment of the posterior cerebral artery. Coil embolization was successfully performed, and clazosentan (10 mg/hr), along with cilostazol, was administered to prevent vasospasm. Fluid balance was carefully managed, and no clazosentan-related adverse events were observed. Follow-up radiological examinations showed no evidence of cerebral vasospasm or ischemic lesions. The patient made a favorable recovery and was discharged with a modified Rankin scale score of 1. Four months later, revascularization surgery was performed to reduce hemodynamic stress and the risk of hemorrhage from choroidal collaterals. The aneurysm remained occluded, the choroidal channels regressed, and no recurrent strokes occurred during 1 year of follow-up. To the best of our knowledge, this is the first report of successful clazosentan therapy for aneurysmal subarachnoid hemorrhage in a patient with moyamoya disease. Careful perioperative management allowed the safe use of clazosentan without complications. Further studies are needed to evaluate its broader safety and efficacy in this population.

Intracranial cryptococcoma is an uncommon manifestation of central nervous system cryptococcosis. Imaging commonly shows ring-enhancing lesions that resemble malignant tumors radiologically. Early diagnosis and extended antifungal treatment are crucial for decreasing morbidity and enhancing outcomes. Treatment typically involves a minimum of six weeks of induction therapy, followed by 6-18 months of maintenance therapy. Limited information is available on the complete clinical progression in cases where remission is not attained with initial treatment. A 66-year-old immunocompetent man presented with ten ring-enhancing lesions in the brain parenchyma and an infiltrative lesion in the lungs. Complete resection of the largest lesion in the left frontal lobe confirmed a histopathological diagnosis of cryptococcoma. Despite receiving eight weeks of induction therapy with liposomal amphotericin B and oral flucytosine, exceeding standard recommendations, the lesions only partially regressed, failing to achieve complete remission. Fluconazole maintenance therapy was initiated. At five months, lesion recurrence was observed. Induction therapy was resumed for three weeks, followed by a switch back to fluconazole. However, the patient's level of consciousness deteriorated, making oral medication impossible. The patient died 15 months after the initiation of antifungal treatment. Cryptococcoma is a critical diagnostic complication. When chest imaging findings are not characteristic of a typical malignancy, a biopsy is essential before determining the appropriate treatment strategy. When lesions do not completely resolve after induction therapy, careful clinical and radiological follow-up is crucial because the risk of recurrence remains significant.

Most pseudoaneurysms after traumatic vertebral artery injuries are caused by penetrating or blunt injuries without vertebral fractures. Here, we report a cervical spinal cord injury in a patient with a giant vertebral artery pseudoaneurysm within a split cervical vertebral fracture. An 82-year-old man presented with a history of complete paralysis of the left side of his body due to basilar artery occlusion. He fell while riding in a wheelchair and presented with obstructive respiratory failure 2 days before transfer to our hospital. Radiological examination revealed C4-5 split fractures with diffuse idiopathic skeletal hyperostosis and a suspected cervical spinal cord injury. A contrast-enhanced lesion was observed within the fracture cavity of the C5 vertebral body, which was suggestive of a pseudoaneurysm originating from the left vertebral artery. Because of a history of basilar artery occlusion and a right vertebral artery that terminated in the posterior inferior cerebellar artery, we did not perform emergency interventional treatment. After conservative treatment under sedation for 3 days, cerebral angiography revealed the almost complete disappearance of the pseudoaneurysm. The patient was deemed to have undergone fracture reduction, posterior decompression, and fusion (C3-7) without risk of aneurysm rupture. Although his neurological recovery was limited, the pseudoaneurysm disappearance and tendency of the fractured vertebral body to fuse were confirmed. Pseudoaneurysms of the vertebral artery associated with vertebral body fractures are extremely rare. This report suggests that pseudoaneurysms within a split cervical vertebral fracture could spontaneously disappear with conservative treatment, with care to avoid rupture and embolism.

Mycotic cerebral aneurysms secondary to infective endocarditis carry a high risk of fatal rupture. Early identification and appropriate intervention are critical. We present a case in which a punctate hyperdense lesion on noncontrast-enhanced computed tomography appeared several days before aneurysm formation and rupture. A 43-year-old man with infective endocarditis presented with a ruptured mycotic aneurysm in the peripheral left middle cerebral artery. The patient underwent emergency craniotomy. During the postoperative follow-up, 2 new punctate hyperdense lesions were detected on noncontrast-enhanced computed tomography. A total of 3 days later, one of these lesions progressed to aneurysm formation and hemorrhage, requiring repeat surgery. The outcome was favorable, with a modified Rankin scale score of 2. This case highlights that punctate hyperdense lesions on noncontrast-enhanced computed tomography may serve as an early radiological marker of impending mycotic aneurysm formation and suggests the importance of close imaging surveillance to prevent catastrophic rupture.