NMC case report journal最新文献

Dural arteriovenous fistula can present with cerebral venous sinus thrombosis and retrograde leptomeningeal venous drainage. Given the associated intracranial hemorrhage risk, immediate retrograde leptomeningeal venous drainage obliteration is desirable. Herein, we report a case of dural arteriovenous fistula with acute cerebral venous sinus thrombosis and intracranial hemorrhage that was successfully managed using sinus thrombectomy alone. A 76-year-old woman was admitted to our hospital with a sudden headache. Radiological assessments showed a subarachnoid hemorrhage in the left cerebellopontine cistern, Borden type III (Cognard type III) dural arteriovenous fistula with cerebral venous sinus thrombosis in the left transverse-sigmoid sinus, and retrograde leptomeningeal venous drainage in the superficial middle cerebral vein, the vein of Labbé, and the petrosal vein. To prevent re-bleeding, we performed an acute thrombectomy of the transverse-sigmoid sinus, aiming to obliterate retrograde leptomeningeal venous drainage. Following the procedure, the dural arteriovenous fistula was downgraded to Borden type I (Cognard type IIa). In this case, a simple thrombectomy alone prevented the re-bleeding of a dural arteriovenous fistula without direct fistula access. Understanding the relative contributions of the fistula and cerebral venous sinus thrombosis to retrograde leptomeningeal venous drainage in transverse-sigmoid sinus dural arteriovenous fistula is essential for refining the treatment strategy. Sinus thrombectomy is a tentative treatment, and this strategy is the emergency procedure to reduce re-bleeding risk. However, it may be a viable approach to prevent re-bleeding by improving venous outflow in dural arteriovenous fistula cases complicated by acute cerebral venous sinus thrombosis and intracranial hemorrhage.

Cerebral embolism following endoscopic injection sclerotherapy using N-butyl-2 cyanoacrylate for esophagogastric varices is a rare but serious complication. This condition occurs due to the migration of N-butyl-2 cyanoacrylate into the cerebral circulation, which has traditionally been attributed to paradoxical embolism via a patent foramen ovale. However, recent reports have suggested the possibility of alternative embolic pathways. We report the case of a 76-year-old woman who presented with acute gastric variceal bleeding and underwent endoscopic injection sclerotherapy using N-butyl-2 cyanoacrylate and Lipiodol. Shortly after the procedure, the patient developed global aphasia and right hemiparesis. Head computed tomography revealed scattered ultra-hyperdense materials and magnetic resonance imaging confirmed left middle cerebral artery M2 occlusion. Mechanical thrombectomy was performed, achieving successful recanalization and neurological improvement. Imaging findings and histopathological analysis of the retrieved thrombus strongly supported the diagnosis of N-butyl-2 cyanoacrylate embolism. Retrospective evaluation of preoperative contrast-enhanced computed tomography identified a portopulmonary venous anastomosis, a right-to-left shunt connecting gastric varices to the pulmonary veins. This anastomosis is a collateral pathway associated with portal hypertension. This case suggests that even in the absence of a patent foramen ovale, portopulmonary venous anastomosis can serve as a route for N-butyl-2 cyanoacrylate migration into the cerebral circulation. To the best of our knowledge, this is the first reported case of mechanical thrombectomy for N-butyl-2 cyanoacrylate cerebral embolism following endoscopic injection sclerotherapy. This case underscores the importance of early detection and risk assessment of such right-to-left shunting and highlights mechanical thrombectomy as a viable intervention for cases where thrombolysis is contraindicated.

Primary histiocytic sarcoma of the central nervous system is an extremely rare malignancy, with only 43 cases reported to date. It often presents diagnostic challenges due to its rarity and similarities with other central nervous system tumors, particularly glioblastoma. We report a case of primary central nervous system histiocytic sarcoma in a 72-year-old woman, one of the older patients among the reported cases. The patient presented with gait disturbance and left hemiplegia. Initial imaging and intraoperative findings suggested a high-grade glioma. However, comprehensive pathological examination, including immunohistochemistry, electron microscopy, and genetic analyses, led to the diagnosis of histiocytic sarcoma. Notably, the tumor exhibited CDKN2A homozygous deletion and a unique methylation profile distinct from gliomas. Despite treatment with surgery, radiation, and chemotherapy, the patient experienced meningeal dissemination 8 months post-surgery and died 11 months after diagnosis. This case highlights the importance of comprehensive diagnostic evaluation in challenging central nervous system tumors and provides valuable insights into the molecular characteristics of central nervous system histiocytic sarcoma, particularly regarding CDKN2A deletion and methylation patterns. Awareness of this rare entity is crucial for accurate diagnosis and appropriate management of central nervous system tumors.

Foramen magnum decompression is an established surgical method for the treatment of Chiari type 1 malformation with syringomyelia. However, in some cases, neurologic symptoms that improve only after foramen magnum decompression may deteriorate again, and it has been suspected that increased instability of the craniocervical junction may be a factor. We report a case of Chiari type 1 malformation accompanied by basilar invagination and syringomyelia in which atlantoaxial facet joint distraction and fixation was performed as a revision surgery for deteriorating neurologic symptoms after foramen magnum decompression. The patient was a 16-year-old boy with chief complaints of gait disturbance and repeated falls. He was diagnosed with Chiari type 1 malformation accompanied by basilar invagination and syringomyelia. The clivo-axial angle was narrow at 105.8°. Initially, only foramen magnum decompression was performed, and the symptoms were relieved after the procedure but re-exacerbated within 2 weeks. The condition was speculated to have worsened instability at the atlantoaxial segment and ventral medullary compression; then, atlantoaxial facet joint distraction and fixation was performed secondarily, and symptoms improved. There is no clear surgical standard for performing foramen magnum decompression, atlantoaxial facet joint distraction and fixation, or a combination of both for Chiari type 1 malformation. Foramen magnum decompression provides horizontal decompression at the craniocervical junction, and atlantoaxial facet joint distraction and fixation achieves vertical indirect decompression of the ventral medulla and stabilization of the atlantoaxial segment in case with basilar invagination. Our experience suggests that combined foramen magnum decompression and atlantoaxial facet joint distraction and fixation may be particularly beneficial in Chiari type 1 malformation cases with basilar invagination and narrow clivo-axial angle.

Neurolymphomatosis is a rare lymphoma infiltrating cranial nerves, peripheral nerves, and spinal nerve roots. We present a unique case of neurolymphomatosis involving the central nervous system, initially resembling polyneuropathy in Sjögren's syndrome. A 79-year-old woman experienced dry eye symptoms for 2 years before presenting with left facial nerve palsy and lumbar pain to her previous physician. Brain magnetic resonance imaging revealed high signal intensity on diffusion-weighted imaging solely in the left peripheral facial nerve and lower cranial nerves. Initially diagnosed with Sjögren's syndrome-associated polyneuritis, she underwent treatment with high-dose steroid pulse therapy and intravenous immunoglobulin. Despite treatment, her symptoms progressed, leading to referral to our hospital with multiple peripheral nerve palsies and severe lumbar pain. Subsequent brain magnetic resonance imaging demonstrated gadolinium enhancement in the bilateral caudate nucleus heads and the left facial and lower cranial nerves. Stereotactic brain tumor biopsy-confirmed diffuse large B-cell lymphoma upon pathological analysis. Treatment with rituximab, high-dose methotrexate significantly improved her symptoms, with no recurrence observed for 12 months. These findings underscore the diagnosis of neurolymphomatosis involving the central nervous system, initially masquerading as Sjögren's syndrome. Neurolymphomatosis should be considered in cases of refractory Sjögren's syndrome presenting with multiple nerve palsies.

Methotrexate-related lymphoproliferative disorder is a rare but serious complication of methotrexate treatment. Recurrent methotrexate-related lymphoproliferative disorder in the central nervous system is uncommon. An 86-year-old woman, who had been on methotrexate for 3 years to treat rheumatoid arthritis, was admitted with left hemiplegia and sensory disturbance. Brain magnetic resonance imaging revealed a right basal ganglia lesion that was hyperintense on diffusion-weighted imaging and showed patchy enhancement. Methotrexate-related lymphoproliferative disorder was suspected, and methotrexate was discontinued, which led to improvement in her symptoms and partial remission. Twenty-eight months after methotrexate cessation, the patient presented with reduced activity and left hemiparesis. Brain magnetic resonance imaging showed homogeneously enhancing lesions in the right frontal lobe, basal ganglia, and brainstem. A craniotomy for biopsy of the right frontal lobe lesion confirmed the diagnosis of methotrexate-related lymphoproliferative disorder. Whole-brain radiation (40 Gy in 20 fractions) was administered without chemotherapy due to her advanced age and poor performance status. Her symptoms improved, and she was transferred to a rehabilitation hospital. Methotrexate-related lymphoproliferative disorder of the central nervous system can recur spontaneously, even without resumption of methotrexate, making long-term follow-up necessary after methotrexate cessation.

The trigone of the lateral ventricle is deep and surrounded by eloquent gyri and subcortical fibers. Resection of intraventricular trigone tumors has therefore been challenging, and the optimal surgical approach to the trigone of the lateral ventricle remains controversial. Three patients with large intraventricular trigone meningioma (≥4 cm in diameter) underwent surgical excision using a combined transtemporal and high-parietal approach at Osaka City University Hospital between July 2016 and January 2021. Clinical and imaging studies, as well as surgical complications, were retrospectively reviewed based on medical records from our institution. We also reviewed 153 patients with intraventricular trigone meningioma from 11 reports in the literature and assessed pre- and postoperative symptoms. Gross total resection of the tumor was achieved in all cases. None of the patients showed deterioration of neurological symptoms at 3 months after tumor resection, although one patient experienced transient language dysfunction several weeks after surgery. No cases showed recurrence or required additional therapy. According to our literature review, postoperative visual field defects are more likely to persist than postoperative language dysfunction at 3 months postoperatively. In conclusion, combining the transtemporal and high-parietal approaches appears to be useful for treating large intraventricular trigone meningioma. Postoperative language dysfunction after resection of intraventricular trigone meningioma may tend to resolve more rapidly than postoperative visual field defect.

Low back pain during pregnancy is common. However, lumbar disc herniation in pregnancy is rare, and when severe, it can cause lower extremity pain that resists conservative treatment or lead to cauda equina syndrome. In this report, we describe a case of severe lower back pain that did not improve with conservative treatment, necessitating surgery at 12 weeks of gestation. Additionally, we reviewed the literature. A 35-year-old woman, 7 weeks pregnant, presented to our outpatient clinic with right lower extremity pain. Magnetic resonance imaging revealed a herniated disc at the right L4/5. Acetaminophen, a pregnancy-safe analgesic was prescribed. Despite initiating treatment, the pain persisted, and the patient was hospitalized for bed rest. After one month of conservative treatment with no relief, a repeat magnetic resonance imaging showed an enlarged herniated disc. After consulting with the patient and her family, surgery was performed at Kobe University Hospital, which offers comprehensive neurosurgery, obstetrics and gynecology, and anesthesiology services. At 12 weeks of gestation, the patient underwent a microscopic discectomy in the prone position under general anesthesia. Fluoroscopy for surgical level confirmation was minimized. The surgery lasted approximately 1 hour, and the right lower extremity pain was alleviated. Five months later, the patient delivered a healthy child. We report a case of surgery for a herniated disc during pregnancy, along with a review of the literature.

Neurofibromatosis 2-related schwannomatosis is a rare genetic disorder marked by the development of multiple central nervous system tumors and, occasionally, non-hydrocephalic intracranial hypertension. This report details two cases of papilledema in patients with neurofibromatosis 2-related schwannomatosis, characterized by normal ventricular morphology on Magnetic resonance imaging and refractory to conservative treatment. Both patients-a 23-year-old and a 31-year-old female-demonstrated complete resolution of papilledema and restoration of visual function following ventriculoperitoneal shunt placement. These cases underscore the critical role of early ventriculoperitoneal surgical intervention in mitigating vision loss and emphasize the necessity for rigorous clinical surveillance in this unique patient population.

The monitoring of intraoperative optic nerve protection using visual evoked potential has been increasingly used in neurosurgical procedures. Although visual evoked potential is a valuable tool, its application is often hindered by anesthetic limitations and challenges related to measurement and recording environments. This case study indicates the successful use of visual evoked potential monitoring during long-term anesthesia maintenance and in artifact-prone environments for the treatment of a ruptured cerebral arteriovenous malformation. We report the case of a woman in her 30s who underwent surgical treatment for a ruptured cerebral arteriovenous malformation in the right occipital lobe, adjacent to the optic radiation. Visual evoked potential monitoring was essential during both transarterial embolization and subsequent microsurgical resection. During transarterial embolization, the implementation of improved measurement environments, localized light stimulation, and optimized recording conditions facilitated stable visual evoked potential monitoring despite the high levels of environmental noise in the angiography suite. Post-embolization, deep sedation was required to mitigate the risk of postoperative bleeding, causing an 18-hr duration of anesthesia before microsurgical resection. Despite the prolonged anesthesia, visual evoked potential monitoring was successfully maintained by carefully managing anesthesia depth. This case shows that visual evoked potential monitoring can be reliable and reproducible during extended periods of anesthesia and in challenging, artifact-rich environments. These findings underscore the feasibility of using visual evoked potential in complex neurosurgical cases, even under less-than-ideal conditions.