Craniopharyngioma (CP) and Rathke's cleft cyst (RCC) are both suprasellar lesions. They are sometimes difficult to distinguish due to their similar findings. We report a case of papillary craniopharyngioma (pCP) with the clinical findings suggesting RCC. A 42-year-old female with intellectual disability presented to our hospital with severe visual dysfunction. Preoperative images revealed a suprasellar cystic lesion without calcification. We performed transsphenoidal surgery. Since the cyst had condensed-milk-like content suggesting RCC, we performed cyst fenestration and wash without removal of the cyst wall. Thereafter, we found fish-egg-like structures on the cyst wall. The histopathological analysis revealed that they had papillary structures surrounded by hyperplastic squamous epithelium with parakeratosis. Immunostaining for BRAF V600E was positive, leading to the diagnosis of pCP. After the surgery, her visual function improved and follow-up Magnetic resonance imaging at 18 months postoperatively showed no apparent recurrence. The presence of condensed-milk-like content suggests a likelihood of RCC indicating that aggressive resection may not be necessary. In contrast, the existence of fish-egg-like structures suggests pCP and requires careful follow-up.
{"title":"A Case of Papillary Craniopharyngioma Mimicking Rathke's Cleft Cyst.","authors":"Hideyuki Harada, Kazuhito Takeuchi, Yuichi Nagata, Naoko Inoshita, Eiji Ito, Eriko Okumura, Tatsuma Kondo, Yoshiki Sato, Ryuta Saito","doi":"10.2176/jns-nmc.2024-0057","DOIUrl":"10.2176/jns-nmc.2024-0057","url":null,"abstract":"<p><p>Craniopharyngioma (CP) and Rathke's cleft cyst (RCC) are both suprasellar lesions. They are sometimes difficult to distinguish due to their similar findings. We report a case of papillary craniopharyngioma (pCP) with the clinical findings suggesting RCC. A 42-year-old female with intellectual disability presented to our hospital with severe visual dysfunction. Preoperative images revealed a suprasellar cystic lesion without calcification. We performed transsphenoidal surgery. Since the cyst had condensed-milk-like content suggesting RCC, we performed cyst fenestration and wash without removal of the cyst wall. Thereafter, we found fish-egg-like structures on the cyst wall. The histopathological analysis revealed that they had papillary structures surrounded by hyperplastic squamous epithelium with parakeratosis. Immunostaining for BRAF V600E was positive, leading to the diagnosis of pCP. After the surgery, her visual function improved and follow-up Magnetic resonance imaging at 18 months postoperatively showed no apparent recurrence. The presence of condensed-milk-like content suggests a likelihood of RCC indicating that aggressive resection may not be necessary. In contrast, the existence of fish-egg-like structures suggests pCP and requires careful follow-up.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"191-194"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11345118/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142057780","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Spinal dural arteriovenous fistulas (SDAVFs) are rare vascular malformations that can occur anywhere in the spine. Most SDAVFs lead to slow aggressive myelopathy due to venous congestion at a level adjacent to the shunt point. However, rare cases of localized brainstem edema without spinal cord lesions have been reported. In this study, we present a case of a lower cervical SDAVF that showed localized congestive edema of the medulla in the absence of an edematous change in the cervical spinal cord. The patient was a 57-year-old woman who experienced vertigo and vomiting without myelopathy that did not improve with conservative treatment. Magnetic resonance imaging (MRI) revealed high signal intensity in the left medulla on T2-weighted imaging (T2WI), while angiography revealed an SDAVF at the right C8 segmental level supplied by the right thyrocervical trunk. She underwent surgical interruption of the draining vein, which led to a rapid improvement in her symptoms. A subsequent follow-up MRI confirmed resolution of both the medullary edema and the dilated draining vein. SDAVFs may cause vertigo and vomiting, which are brainstem symptoms. Early diagnosis and surgical intervention are crucial for successful treatment outcomes.
{"title":"Lower Cervical Dural Arteriovenous Fistula with a \"Skip Lesion\" in the Brainstem: A Case Report.","authors":"Yuki Yasuoka, Takafumi Mitsuhara, Shinya Nabika, Naohiko Ohbayashi, Asahi Saito, Nobutaka Horie","doi":"10.2176/jns-nmc.2024-0030","DOIUrl":"10.2176/jns-nmc.2024-0030","url":null,"abstract":"<p><p>Spinal dural arteriovenous fistulas (SDAVFs) are rare vascular malformations that can occur anywhere in the spine. Most SDAVFs lead to slow aggressive myelopathy due to venous congestion at a level adjacent to the shunt point. However, rare cases of localized brainstem edema without spinal cord lesions have been reported. In this study, we present a case of a lower cervical SDAVF that showed localized congestive edema of the medulla in the absence of an edematous change in the cervical spinal cord. The patient was a 57-year-old woman who experienced vertigo and vomiting without myelopathy that did not improve with conservative treatment. Magnetic resonance imaging (MRI) revealed high signal intensity in the left medulla on T2-weighted imaging (T2WI), while angiography revealed an SDAVF at the right C8 segmental level supplied by the right thyrocervical trunk. She underwent surgical interruption of the draining vein, which led to a rapid improvement in her symptoms. A subsequent follow-up MRI confirmed resolution of both the medullary edema and the dilated draining vein. SDAVFs may cause vertigo and vomiting, which are brainstem symptoms. Early diagnosis and surgical intervention are crucial for successful treatment outcomes.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"175-179"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11223889/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141536271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-13eCollection Date: 2024-01-01DOI: 10.2176/jns-nmc.2023-0192
Masahiro Uchimura, Yuichi Anno, Haruo Takigawa, Manabu Yoshida, Kentaro Hayashi
Chronic encapsulated intracerebral hematoma is a rare type of intracerebral hemorrhage. Reportedly, it is associated with vascular malformations, including arteriovenous malformations, cavernous hemangiomas, microaneurysms, and venous malformations. Recently, an association between chronic encapsulated intracerebral hematoma and stereotactic radiosurgery for arteriovenous malformations has been reported. In general, as the hematoma enlarges, symptoms progress slowly. In this report, we present a case of a 50-year-old woman who had undergone clivus chordoma resection and carbon ion therapy for the clivus respectively 27 and 20 years before developing chronic encapsulated intracerebral hematoma with rapidly progressing disturbance of consciousness. She was referred to our hospital because of difficulty walking due to left hemiparesis. Head computed tomography and magnetic resonance imaging showed a cystic lesion in the right temporal lobe with perifocal edema. On the second day of hospitalization, the patient's consciousness worsened. We suspected a malignant glioma and performed an emergency craniotomy; however, the pathological diagnosis was chronic encapsulated intracerebral hematoma. After the rehabilitation therapy, the patient became ambulatory and was discharged. To the date of reporting, the patient remained recurrence-free. Chronic encapsulated intracerebral hematoma may be due to invasive craniotomy or carbon ion therapy. It usually progresses slowly; however, in some cases, such as this one, it may cause rapid deterioration of consciousness.
{"title":"Chronic Encapsulated Intracerebral Hematoma after Carbon Ion Therapy for Chordoma Mimicking Malignant Glioma: A Case Report.","authors":"Masahiro Uchimura, Yuichi Anno, Haruo Takigawa, Manabu Yoshida, Kentaro Hayashi","doi":"10.2176/jns-nmc.2023-0192","DOIUrl":"10.2176/jns-nmc.2023-0192","url":null,"abstract":"<p><p>Chronic encapsulated intracerebral hematoma is a rare type of intracerebral hemorrhage. Reportedly, it is associated with vascular malformations, including arteriovenous malformations, cavernous hemangiomas, microaneurysms, and venous malformations. Recently, an association between chronic encapsulated intracerebral hematoma and stereotactic radiosurgery for arteriovenous malformations has been reported. In general, as the hematoma enlarges, symptoms progress slowly. In this report, we present a case of a 50-year-old woman who had undergone clivus chordoma resection and carbon ion therapy for the clivus respectively 27 and 20 years before developing chronic encapsulated intracerebral hematoma with rapidly progressing disturbance of consciousness. She was referred to our hospital because of difficulty walking due to left hemiparesis. Head computed tomography and magnetic resonance imaging showed a cystic lesion in the right temporal lobe with perifocal edema. On the second day of hospitalization, the patient's consciousness worsened. We suspected a malignant glioma and performed an emergency craniotomy; however, the pathological diagnosis was chronic encapsulated intracerebral hematoma. After the rehabilitation therapy, the patient became ambulatory and was discharged. To the date of reporting, the patient remained recurrence-free. Chronic encapsulated intracerebral hematoma may be due to invasive craniotomy or carbon ion therapy. It usually progresses slowly; however, in some cases, such as this one, it may cause rapid deterioration of consciousness.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"163-168"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11223888/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141536345","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
We report a male patient with a ruptured persistent primitive trigeminal artery variant aneurysm that resulted in a fistula with the cavernous sinus. He presented with left conjunctival hyperemia and exophthalmos. Cerebral angiography revealed a left direct carotid-cavernous fistula; however, a balloon occlusion test determined that the source was actually a ruptured aneurysm located on the trunk of a persistent primitive trigeminal artery. Endovascular trapping of the persistent primitive trigeminal artery was performed, which resulted in fistula occlusion and symptom resolution.
{"title":"Selective Transarterial Embolization for a Ruptured Persistent Trigeminal Artery Variant Aneurysm.","authors":"Ryo Fujisawa, Yayoi Yoshimura, Hiroto Kawano, Keiichi Tsuji, Atsushi Tsuji, Takuya Nakazawa, Haruka Miyata, Masanori Gomi, Kazuhiko Nozaki, Kazumichi Yoshida","doi":"10.2176/jns-nmc.2024-0018","DOIUrl":"10.2176/jns-nmc.2024-0018","url":null,"abstract":"<p><p>We report a male patient with a ruptured persistent primitive trigeminal artery variant aneurysm that resulted in a fistula with the cavernous sinus. He presented with left conjunctival hyperemia and exophthalmos. Cerebral angiography revealed a left direct carotid-cavernous fistula; however, a balloon occlusion test determined that the source was actually a ruptured aneurysm located on the trunk of a persistent primitive trigeminal artery. Endovascular trapping of the persistent primitive trigeminal artery was performed, which resulted in fistula occlusion and symptom resolution.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"169-174"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11224002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141556437","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Craniosynostosis (CS) can develop in the fetal period, but it is difficult to diagnose prenatally. In this case, a 3-month-old female baby developed extensive subgaleal hematoma and severe anemia after vacuum-assisted delivery. Her computed tomography showed bilateral lambdoid and sagittal synostosis (BLSS) with a depressed fracture of the right parietal bone. She was referred to our hospital for treatment of the CS. At 4 months of age, she underwent bilateral lambda and sagittal suturectomy and foramen magnum decompression. CS may result in trauma at delivery, because CS disturbs fetal head molding during delivery and disrupts passage through the birth canal. In particular, the risk of severe peripartum trauma is thought to increase in cases of CS with multiple suture fusions, such as those observed in BLSS, due to the strong inhibition of this process of passage through the birth canal. Therefore, if the delivery is abnormally prolonged or if the infant has a massive subgaleal hematoma, it is important to perform evaluations for CS after birth.
{"title":"A Case of Bilateral Lambdoid and Sagittal Synostosis Diagnosed with Skull Fracture after Vacuum-assisted Delivery: A Case Report and Literature Review.","authors":"Natsuki Nakamura, Atsuko Harada, Kazushige Maeno, Shigeo Kyutoku, Koichi Ueda","doi":"10.2176/jns-nmc.2023-0279","DOIUrl":"10.2176/jns-nmc.2023-0279","url":null,"abstract":"<p><p>Craniosynostosis (CS) can develop in the fetal period, but it is difficult to diagnose prenatally. In this case, a 3-month-old female baby developed extensive subgaleal hematoma and severe anemia after vacuum-assisted delivery. Her computed tomography showed bilateral lambdoid and sagittal synostosis (BLSS) with a depressed fracture of the right parietal bone. She was referred to our hospital for treatment of the CS. At 4 months of age, she underwent bilateral lambda and sagittal suturectomy and foramen magnum decompression. CS may result in trauma at delivery, because CS disturbs fetal head molding during delivery and disrupts passage through the birth canal. In particular, the risk of severe peripartum trauma is thought to increase in cases of CS with multiple suture fusions, such as those observed in BLSS, due to the strong inhibition of this process of passage through the birth canal. Therefore, if the delivery is abnormally prolonged or if the infant has a massive subgaleal hematoma, it is important to perform evaluations for CS after birth.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"157-161"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11224651/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141556436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Neoplastic cerebral aneurysms (NCAs) are rare. This study reported a case of an NCA secondary to a poorly differentiated carcinoma of the parotid gland. An 84-year-old Japanese woman undergoing treatment for parotid gland cancer was admitted to our hospital with headache and progressive loss of consciousness. Based on computed tomography (CT) and CT angiography (CTA), a diagnosis of subarachnoid hemorrhage due to rupture of a left posterior inferior cerebellar artery aneurysm was made, and emergency aneurysmectomy was performed. Pathological examination of the resected aneurysm showed an NCA secondary to parotid carcinoma. After the aneurysmectomy, her condition stabilized; however, 33 days later, the patient developed an intracerebral hemorrhage, and a new aneurysm was confirmed in the right middle cerebral artery. To the best of our knowledge, there have been no previous reports on cases of NCAs secondary to parotid carcinoma. The pathology and clinical course strongly suggest that NCAs derived from malignant tumors may have an aggressive course.
{"title":"A Case of Multiple Intracranial Hemorrhages due to a Ruptured Neoplastic Cerebral Aneurysm Secondary to Metastasis of Poorly Differentiated Parotid Gland Carcinoma.","authors":"Naoki Irizato, Syogo Fukuya, Hidekazu Nakata, Tomofumi Takenaka, Akihiro Tateishi, Kazumi Yamamoto, Akatsuki Wakayama, Masaaki Taniguchi","doi":"10.2176/jns-nmc.2023-0250","DOIUrl":"10.2176/jns-nmc.2023-0250","url":null,"abstract":"<p><p>Neoplastic cerebral aneurysms (NCAs) are rare. This study reported a case of an NCA secondary to a poorly differentiated carcinoma of the parotid gland. An 84-year-old Japanese woman undergoing treatment for parotid gland cancer was admitted to our hospital with headache and progressive loss of consciousness. Based on computed tomography (CT) and CT angiography (CTA), a diagnosis of subarachnoid hemorrhage due to rupture of a left posterior inferior cerebellar artery aneurysm was made, and emergency aneurysmectomy was performed. Pathological examination of the resected aneurysm showed an NCA secondary to parotid carcinoma. After the aneurysmectomy, her condition stabilized; however, 33 days later, the patient developed an intracerebral hemorrhage, and a new aneurysm was confirmed in the right middle cerebral artery. To the best of our knowledge, there have been no previous reports on cases of NCAs secondary to parotid carcinoma. The pathology and clinical course strongly suggest that NCAs derived from malignant tumors may have an aggressive course.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"181-186"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11223887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141536344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-28eCollection Date: 2024-01-01DOI: 10.2176/jns-nmc.2023-0301
Yu Naruse, Mio Endo, Dai Uzuki, Kiyoshi Saito
Suboccipital decompressive craniectomy with or without resection of necrosis is the preferred treatment for space-occupying cerebellar infarctions with neurological deterioration due to brainstem compression and obstructive hydrocephalus. We herein present our experience with treating space-occupying cerebellar infarctions successfully using endoscopic necrosectomy. A total of 27 patients were admitted to our hospital due to cerebellar infarctions between April 2021 and November 2023. Four patients required surgical interventions due to a drop in consciousness level or compression of the fourth ventricle and brainstem with acute hydrocephalus confirmed by a computed tomography (CT) scan. Three patients were performed endoscopic necrosectomy through a burr hole in a supine-lateral position. Removing most of the necrotic tissue was possible, resulting in early decompression of the fourth ventricle and brainstem. Endoscopic necrosectomy is less invasive than suboccipital decompressive craniectomy. An endoscopic necrosectomy can be performed for patients with unstable health conditions in a supine-lateral position. Therefore, endoscopic necrosectomy might be an effective method for treating patients with space-occupying cerebellar infarctions and poor general condition, although an objective evaluation of the extent and degree of removal is needed.
{"title":"Endoscopic Cerebellar Necrosectomy for Space-occupying Cerebellar Infarction: A Case Report.","authors":"Yu Naruse, Mio Endo, Dai Uzuki, Kiyoshi Saito","doi":"10.2176/jns-nmc.2023-0301","DOIUrl":"10.2176/jns-nmc.2023-0301","url":null,"abstract":"<p><p>Suboccipital decompressive craniectomy with or without resection of necrosis is the preferred treatment for space-occupying cerebellar infarctions with neurological deterioration due to brainstem compression and obstructive hydrocephalus. We herein present our experience with treating space-occupying cerebellar infarctions successfully using endoscopic necrosectomy. A total of 27 patients were admitted to our hospital due to cerebellar infarctions between April 2021 and November 2023. Four patients required surgical interventions due to a drop in consciousness level or compression of the fourth ventricle and brainstem with acute hydrocephalus confirmed by a computed tomography (CT) scan. Three patients were performed endoscopic necrosectomy through a burr hole in a supine-lateral position. Removing most of the necrotic tissue was possible, resulting in early decompression of the fourth ventricle and brainstem. Endoscopic necrosectomy is less invasive than suboccipital decompressive craniectomy. An endoscopic necrosectomy can be performed for patients with unstable health conditions in a supine-lateral position. Therefore, endoscopic necrosectomy might be an effective method for treating patients with space-occupying cerebellar infarctions and poor general condition, although an objective evaluation of the extent and degree of removal is needed.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"141-144"},"PeriodicalIF":0.0,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11190657/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141444019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Superficial siderosis (SS) of the central nervous system is a rare disorder that is caused by chronic or recurrent hemorrhage in the subarachnoid space via a dural defect at the spinal level. The most common clinical features of SS include slow-progressive sensorineural deafness, cerebellar symptoms, and pyramidal tract signs. Considering that SS can present with broad clinical manifestations, for precise diagnosis, this disease must be understood. Anti-Ro/SSA antibodies are commonly detected in patients with Sjögren's syndrome and are utilized as markers for autoimmune diseases. In this report, we present a unique pathological condition in which SS coincided with a positive anti-Ro/SSA antibody test result. During the diagnosis of gait disturbance, an elevation in anti-Ro/SSA antibody was detected, and steroid pulse therapy was initiated as the initial treatment for autoimmune diseases. Head magnetic resonance imaging (MRI) revealed extensive hypointensity as a dark band that surrounded the intracranial basal structures and cerebellar hemispheres. Spinal MRI indicated ventral longitudinal intraspinal fluid collection extending from C7 to T5 as well as a defect in the ventral T2-3 dura mater. Intraoperative visualization revealed that the intradural venous plexus was the source of bleeding that caused the SS. To our knowledge, this report is the first to discuss the presence of anti-Ro/SSA antibodies in patients with SS. The role of anti-Ro/SSA antibodies in the pathophysiology of SS remains unclear; therefore, to confirm a possible association, further research and accumulation of cases are required.
中枢神经系统表层蛛网膜下腔出血(SS)是一种罕见的疾病,由脊柱硬膜缺损导致的蛛网膜下腔慢性或复发性出血引起。SS 最常见的临床特征包括缓慢进展的感音神经性耳聋、小脑症状和锥体束征。考虑到 SS 可有广泛的临床表现,要准确诊断这种疾病,必须对其有所了解。抗 Ro/SSA 抗体通常在斯约格伦综合征患者中检测到,并被用作自身免疫性疾病的标志物。在本报告中,我们介绍了一种独特的病理情况,即斯氏综合征与抗Ro/SSA抗体阳性检测结果同时出现。在诊断步态障碍期间,检测到抗 Ro/SSA 抗体升高,并启动了类固醇脉冲疗法作为自身免疫性疾病的初始治疗。头部磁共振成像(MRI)显示,颅内基底结构和小脑半球周围有广泛的低密度暗带。脊柱磁共振成像显示,腹侧纵向椎管内积液从C7延伸至T5,腹侧T2-3硬脑膜也有缺损。术中观察发现,硬膜内静脉丛是导致 SS 的出血源。据我们所知,该报告首次讨论了SS患者体内存在抗Ro/SSA抗体的问题。抗Ro/SSA抗体在SS病理生理学中的作用仍不清楚;因此,要证实可能的关联性,还需要进一步的研究和病例的积累。
{"title":"A Case of Superficial Siderosis with Elevated Anti-Ro/SSA Antibody.","authors":"Shoji Watanabe, Maulidina Amalia Putri, Hitoshi Yamahata, Ryosuke Hanaya","doi":"10.2176/jns-nmc.2023-0214","DOIUrl":"10.2176/jns-nmc.2023-0214","url":null,"abstract":"<p><p>Superficial siderosis (SS) of the central nervous system is a rare disorder that is caused by chronic or recurrent hemorrhage in the subarachnoid space via a dural defect at the spinal level. The most common clinical features of SS include slow-progressive sensorineural deafness, cerebellar symptoms, and pyramidal tract signs. Considering that SS can present with broad clinical manifestations, for precise diagnosis, this disease must be understood. Anti-Ro/SSA antibodies are commonly detected in patients with Sjögren's syndrome and are utilized as markers for autoimmune diseases. In this report, we present a unique pathological condition in which SS coincided with a positive anti-Ro/SSA antibody test result. During the diagnosis of gait disturbance, an elevation in anti-Ro/SSA antibody was detected, and steroid pulse therapy was initiated as the initial treatment for autoimmune diseases. Head magnetic resonance imaging (MRI) revealed extensive hypointensity as a dark band that surrounded the intracranial basal structures and cerebellar hemispheres. Spinal MRI indicated ventral longitudinal intraspinal fluid collection extending from C7 to T5 as well as a defect in the ventral T2-3 dura mater. Intraoperative visualization revealed that the intradural venous plexus was the source of bleeding that caused the SS. To our knowledge, this report is the first to discuss the presence of anti-Ro/SSA antibodies in patients with SS. The role of anti-Ro/SSA antibodies in the pathophysiology of SS remains unclear; therefore, to confirm a possible association, further research and accumulation of cases are required.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"151-155"},"PeriodicalIF":0.0,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11190658/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141444018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
We present a case of pontine infarction caused by subclavian steal phenomenon (SSP) due to subclavian artery stenosis (SAS) and an arteriovenous shunt in the forearm in a 74-year-old man with hemodialysis and stenting for SAS with improvement of SSP. He developed dysarthria during dialysis. He was admitted to our hospital and diagnosed with a pontine infarction. As the basilar artery appeared to be occluded on magnetic resonance angiography, an emergency diagnostic angiography was performed. Aortagram showed severe stenosis of the left subclavian artery. Right vertebral artery (VA) angiogram revealed retrograde arterial blood flow from the right VA to the left VA via the VA union, which suggested SSP. In addition, the steal was augmented by an ipsilateral hemodialysis arteriovenous shunt. Percutaneous subclavian artery stenting was performed 12 days later, and there was no recurrence of symptoms in the follow-up period. To our knowledge, this study is the first to report a patient with SSP who developed a pontine infarction due to SAS and an arteriovenous shunt during hemodialysis and who underwent subclavian artery stenting and had a good outcome.
我们报告了一例因锁骨下动脉狭窄(SAS)和前臂动静脉分流而导致的锁骨下窃血现象(SSP)引起的桥脑梗死病例,患者是一名 74 岁的男性,曾接受血液透析和 SAS 支架治疗,SSP 有所改善。他在透析期间出现构音障碍。他被送入我院并被诊断为桥脑梗死。由于磁共振血管造影显示基底动脉闭塞,因此对他进行了紧急血管造影诊断。大动脉造影显示左锁骨下动脉严重狭窄。右侧椎动脉(VA)血管造影显示动脉血流从右侧 VA 经 VA 结合部逆流至左侧 VA,这表明存在 SSP。此外,同侧血液透析动静脉分流也加重了盗血。12 天后进行了经皮锁骨下动脉支架植入术,随访期间症状没有复发。据我们所知,本研究首次报道了一名在血液透析期间因 SAS 和动静脉分流而发生桥脑梗死的 SSP 患者,该患者接受了锁骨下动脉支架植入术,并取得了良好的疗效。
{"title":"A Case of Pontine Infarction due to Subclavian Steal Phenomenon Enhanced by an Arteriovenous Shunt for Hemodialysis.","authors":"Motohide Takahara, Tomoaki Murakami, Shingo Toyota, Shuki Okuhara, Kazuhiro Touhara, Yuhei Hoshikuma, Takamune Achiha, Shuhei Yamada, Maki Kobayashi, Haruhiko Kishima","doi":"10.2176/jns-nmc.2024-0004","DOIUrl":"10.2176/jns-nmc.2024-0004","url":null,"abstract":"<p><p>We present a case of pontine infarction caused by subclavian steal phenomenon (SSP) due to subclavian artery stenosis (SAS) and an arteriovenous shunt in the forearm in a 74-year-old man with hemodialysis and stenting for SAS with improvement of SSP. He developed dysarthria during dialysis. He was admitted to our hospital and diagnosed with a pontine infarction. As the basilar artery appeared to be occluded on magnetic resonance angiography, an emergency diagnostic angiography was performed. Aortagram showed severe stenosis of the left subclavian artery. Right vertebral artery (VA) angiogram revealed retrograde arterial blood flow from the right VA to the left VA via the VA union, which suggested SSP. In addition, the steal was augmented by an ipsilateral hemodialysis arteriovenous shunt. Percutaneous subclavian artery stenting was performed 12 days later, and there was no recurrence of symptoms in the follow-up period. To our knowledge, this study is the first to report a patient with SSP who developed a pontine infarction due to SAS and an arteriovenous shunt during hemodialysis and who underwent subclavian artery stenting and had a good outcome.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"145-150"},"PeriodicalIF":0.0,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11190656/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141444017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-17eCollection Date: 2024-01-01DOI: 10.2176/jns-nmc.2023-0136
Kosuke Miwa, Kyongsong Kim, Rinko Kokubo, Hiroyuki Dan, Kenta Koketsu, Yasuo Murai
The flexor digitorum accessorius longus muscle (ALM) can be overlooked as the eliciting factor in patients with tarsal tunnel syndrome (TTS), an entrapment neuropathy of the posterior tibial nerve that elicits sole numbness and pain. Most elicitations are idiopathic, however, mass lesions within the tarsal tunnel can be also implicated. We report an 80-year-old woman whose flexor digitorum ALM led to the onset of bilateral TTS. She had suffered numbness in both soles for 3 years. Magnetic resonance imaging (MRI) of the bilateral tarsal tunnel showed that the posterior tibial nerve was compressed by the arteriovenous complex and in contact with the flexor digitorum ALM. We diagnosed bilateral TTS based on her symptoms and imaging findings, and performed bilateral decompression surgery of the posterior tibial nerve under local anesthesia. The artery on both sides was dislocated for nerve decompression. Because the posterior tibial nerve on the right side was strongly compressed in ankle plantar flexion we excised a portion of the tendon compressing the nerve. Postoperatively her symptoms gradually improved and she reported surgical satisfaction 6 months after the operation. In patients with flexor digitorum ALM-related TTS, the effect of dynamic factors on MRI findings and on surgical treatment decisions must be considered. Intraoperatively, not only the flexor digitorum ALM, but also other potential etiologic factors eliciting TTS must be kept in mind.
{"title":"A Patient with Tarsal Tunnel Syndrome Associated with the Flexor Digitorum Accessorius Longus Muscle.","authors":"Kosuke Miwa, Kyongsong Kim, Rinko Kokubo, Hiroyuki Dan, Kenta Koketsu, Yasuo Murai","doi":"10.2176/jns-nmc.2023-0136","DOIUrl":"10.2176/jns-nmc.2023-0136","url":null,"abstract":"<p><p>The flexor digitorum accessorius longus muscle (ALM) can be overlooked as the eliciting factor in patients with tarsal tunnel syndrome (TTS), an entrapment neuropathy of the posterior tibial nerve that elicits sole numbness and pain. Most elicitations are idiopathic, however, mass lesions within the tarsal tunnel can be also implicated. We report an 80-year-old woman whose flexor digitorum ALM led to the onset of bilateral TTS. She had suffered numbness in both soles for 3 years. Magnetic resonance imaging (MRI) of the bilateral tarsal tunnel showed that the posterior tibial nerve was compressed by the arteriovenous complex and in contact with the flexor digitorum ALM. We diagnosed bilateral TTS based on her symptoms and imaging findings, and performed bilateral decompression surgery of the posterior tibial nerve under local anesthesia. The artery on both sides was dislocated for nerve decompression. Because the posterior tibial nerve on the right side was strongly compressed in ankle plantar flexion we excised a portion of the tendon compressing the nerve. Postoperatively her symptoms gradually improved and she reported surgical satisfaction 6 months after the operation. In patients with flexor digitorum ALM-related TTS, the effect of dynamic factors on MRI findings and on surgical treatment decisions must be considered. Intraoperatively, not only the flexor digitorum ALM, but also other potential etiologic factors eliciting TTS must be kept in mind.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"11 ","pages":"125-129"},"PeriodicalIF":0.0,"publicationDate":"2024-05-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11165262/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141307779","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}