NMC case report journal最新文献

{"title":"Spontaneous Extracranial Internal Carotid Artery Dissection Solely Presenting with Sudden Onset Pulsatile Tinnitus.","authors":"Tomohisa Okada, Hiroaki Hamada, Shunichi Tanaka, Masaaki Yamamoto, Shingo Fujio, Ryosuke Hanaya, Kazunori Arita","doi":"10.2176/jns-nmc.2025-0089","DOIUrl":"10.2176/jns-nmc.2025-0089","url":null,"abstract":"<p><p>Spontaneous internal carotid artery dissection is a rare condition that generally affects middle-aged individuals. Most cases of reported spontaneous internal carotid artery dissections are unilateral, but approximately 10%-20% involve bilateral internal carotid arteries. Spontaneous internal carotid artery dissection generally presents with neck pain, ischemic cerebral symptoms, and/or Horner's syndrome. Pulsatile tinnitus is present in around 5%-10% of spontaneous internal carotid artery dissection cases. However, this is generally accompanied by other major internal carotid artery dissection symptoms. Herein, we report a case of bilateral spontaneous internal carotid artery dissection that presented with unilateral pulsatile tinnitus of sudden onset as an isolated symptom. An otherwise healthy 50-year-old man visited a neurosurgical clinic complaining of right temporal pulsatile tinnitus that had begun 1 day prior. In addition to tinnitus, a thorough symptom interview revealed mild heavy-headedness. Magnetic resonance angiography showed bilateral extracranial internal carotid artery dissections and severe stenosis of the true lumen of the right internal carotid artery. No cerebral ischemic lesions were observed. Administration of 5 mg/day amlodipine and 100 mg/day aspirin was initiated. Following the 9th day after onset, the pulsatile tinnitus gradually improved. Around the 40th day after onset, the pulsatile tinnitus disappeared, and magnetic resonance angiography showed an improvement of the right internal carotid artery stenosis. Magnetic resonance angiographies at 5 and 9 months after onset revealed complete disappearance of the false lumen and slight stenosis of the right internal carotid artery at the entrance of the carotid canal. This case suggests that the sudden onset of isolated unilateral pulsatile tinnitus can be a clue to the diagnosis of spontaneous internal carotid artery dissection.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"349-354"},"PeriodicalIF":0.0,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12444144/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145116141","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}

{"title":"Pediatric Triptan-induced Reversible Cerebral Vasoconstriction Syndrome with Both Hemorrhagic and Ischemic Stroke: Case Report and Literature Review.","authors":"Yotaro Okazaki, Satoshi Hori, Ryosuke Takagi, Taishi Nakamura, Makoto Ohtake, Hidetaka Onodera, Takashi Kawasaki, Katsumi Sakata, Tetsuya Yamamoto","doi":"10.2176/jns-nmc.2025-0023","DOIUrl":"10.2176/jns-nmc.2025-0023","url":null,"abstract":"<p><p>Reversible cerebral vasoconstriction syndrome is characterized by severe headaches and diffuse, segmental constriction of cerebral arteries. We report a rare pediatric case of triptan-induced reversible cerebral vasoconstriction syndrome, complicated by both subarachnoid hemorrhage and cerebral infarction. A 10-year-old boy presented with persistent severe headaches initially suspected to be migraines, for which he was prescribed rizatriptan. Five days after starting rizatriptan, he developed impaired consciousness (Japan Coma Scale 10) and partial right-sided hemiparesis, prompting emergency hospitalization. A head computed tomography scan revealed subarachnoid hemorrhage in the left frontal cortical region. Cerebral angiography showed stenotic changes in the internal carotid artery, middle cerebral artery, anterior cerebral artery, and posterior cerebral artery. Suspecting triptan-induced reversible cerebral vasoconstriction syndrome, rizatriptan was discontinued, and verapamil treatment was initiated. Subsequent magnetic resonance imaging and magnetic resonance angiography revealed cerebral infarction in the right cerebellum, left middle cerebral artery territory, and right occipital lobe, with persistent stenotic changes from earlier findings. Edaravone was added to the treatment regimen. The patient's condition gradually improved, and follow-up magnetic resonance angiography showed almost complete resolution of the prior stenotic changes. His partial right-sided hemiparesis nearly resolved, and he was discharged home. Triptans should be used with caution, as they may precipitate reversible cerebral vasoconstriction syndrome or exacerbate cerebral vasoconstriction. Careful monitoring and vascular imaging are essential for patients presenting with symptoms following triptan administration.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"303-308"},"PeriodicalIF":0.0,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12391675/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144985153","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}

{"title":"Iatrogenic Cerebral Amyloid Angiopathy after Cadaveric Dura Mater Transplantation to an Intact Brain Surface: A Case Report.","authors":"Yuto Katsuyama, Yasuhiro Aida, Daisuke Kita, Fumihide Enkaku, Tsuyoshi Hamaguchi","doi":"10.2176/jns-nmc.2025-0042","DOIUrl":"10.2176/jns-nmc.2025-0042","url":null,"abstract":"<p><p>Iatrogenic cerebral amyloid angiopathy is a rare yet clinically relevant condition that may develop decades after cadaveric dura mater transplantation. We present the case of a 48-year-old man who experienced recurrent, bilateral, multilobar intracerebral hemorrhages approximately 40 years after childhood dura mater transplantation. Pathological examination confirmed amyloid β protein deposition around small cerebral blood vessels in the brain parenchyma. Genetic testing excluded mutations associated with hereditary cerebral amyloid angiopathy. According to the patient's operative record, at age 7 years, a left parietal bone tumor and the adjacent dura mater were removed, and a cadaveric dural patch was transplanted onto the intact brain surface. This case suggests that exogenous amyloid β protein can be transmitted through cadaveric dura mater, even when placed on intact brain tissue with preserved glymphatic and dural lymphatic drainage function, manifesting decades after transplantation.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"317-321"},"PeriodicalIF":0.0,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12391669/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144985158","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}

{"title":"Posterior Reversible Encephalopathy Syndrome Arising from Simultaneous Cranioplasty and Ventriculoperitoneal Shunting: A Case Report.","authors":"Kensuke Ikeda, Keisuke Maruyama, Hiroki Kagiwata, Atsushi Yamamichi, Kei Okada, Shoko Fujii, Kuniaki Saito, Hirofumi Nakatomi, Teruyuki Hirano, Motoo Nagane","doi":"10.2176/jns-nmc.2025-0067","DOIUrl":"10.2176/jns-nmc.2025-0067","url":null,"abstract":"<p><p>Posterior reversible encephalopathy syndrome is a well-known condition that causes reversible vasogenic edema, mainly in the occipital lobe. However, no guideline for its diagnosis or treatment has been established to date. While many atypical cases have been reported in recent years, posterior reversible encephalopathy syndrome associated with cranioplasty has not yet been reported. A man in his 50s underwent right decompressive craniectomy for brain swelling secondary to ischemic stroke. He was transferred to another hospital for rehabilitation 9 months later and was again admitted for cranioplasty after 2 months. Because of sustained brain distension, right cranioplasty was performed simultaneously with ventriculoperitoneal shunting. No sudden change in blood pressure occurred during the perioperative period. However, generalized convulsions occurred postoperatively. Magnetic resonance imaging showed signal changes in the right frontal lobe, left basal ganglia, left thalamus, and right occipital lobe. When cranioplasty was performed simultaneously with ventriculoperitoneal shunting for a skull defect complicated by brain distension, intraoperative cerebrospinal fluid drainage and continuous cerebrospinal fluid drainage by shunts may have caused a sudden decrease in intracranial pressure and an increase in cerebral perfusion pressure, possibly leading to the development of posterior reversible encephalopathy syndrome.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"323-329"},"PeriodicalIF":0.0,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12391665/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144985184","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}

{"title":"Co-existence of Primary Glioma and Lymphoma: An Unusual Case Report.","authors":"Mayuko Moritsubo, Takuya Furuta, Yoshihiro Natori, Yoshihiro Ohishi, Yasuo Sugita, Hiroaki Miyoshi","doi":"10.2176/jns-nmc.2024-0307","DOIUrl":"10.2176/jns-nmc.2024-0307","url":null,"abstract":"<p><p>Composite or collision tumors in the central nervous system can significantly impact disease progression and metastasis, potentially affecting treatment efficacy. Studying the mechanisms associated with these tumors can provide neuro-oncologists with insights into tumor diversity, progression, and aid in the development of novel treatments. We encountered an 84-year-old female with memory disturbance who presented with tumors consistent with wild-type isocitrate dehydrogenase high-grade glioma and low-grade B-cell lymphoma at the same site. Magnetic resonance imaging revealed a solid enhanced mass in the right frontal lobe. A pre-operative suspicion of primary central nervous system lymphoma led to a brain biopsy. Histologically, 2 types of lesions were observed; the first consisted of atypical glial cells with diffuse infiltration and mitoses, positive for glial fibrillary acidic protein and negative for anti-isocitrate dehydrogenase 1 (IDH1)-R132H, characterized by partial amplification of <i>PDGFRA</i> and homozygous deletion of <i>CDKN2A</i>. The second type consisted of small atypical lymphoid positive forCD20, showing <i>immunoglobulin heavy chain(IgH)</i> rearrangement, and minimal invasion of vessel walls while filling the perivascular space. Based on these findings, collision neoplasms of high-grade gliomas and marginal zone B-cell lymphomas were suspected. To our knowledge, this is the first reported co-existence of a glioma and intracranial lymphoma.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"309-315"},"PeriodicalIF":0.0,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12391670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144985140","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}

{"title":"Acute Sinus Recanalization for the Urgent Treatment of an Isolated Transverse-sigmoid Sinus Dural Arteriovenous Fistula with Hemorrhagic Presentation: Case Report.","authors":"Mayuko Kunii, Tomofumi Takenaka, Nobuto Hirai, Yuhei Hoshikuma, Shogo Fukuya, Tomoki Kidani, Akihiro Tateishi, Katsumi Matsumoto, Masaaki Taniguchi","doi":"10.2176/jns-nmc.2024-0311","DOIUrl":"10.2176/jns-nmc.2024-0311","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"295-301"},"PeriodicalIF":0.0,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12391668/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144985192","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}

{"title":"Mechanical Thrombectomy for N-butyl-2 Cyanoacrylate Cerebral Embolism via Portopulmonary Venous Anastomosis Following Endoscopic Injection Sclerotherapy: A Case Report.","authors":"Ichiro Maeda, Taichiro Imahori, Shigeru Miyake, Hiroki Goto, Rikuo Nishii, Haruka Enami, Daisuke Yamamoto, Shouichi Nakayasu, Hirotoshi Hamaguchi, Naokazu Miyamoto, Takashi Sasayama","doi":"10.2176/jns-nmc.2025-0076","DOIUrl":"10.2176/jns-nmc.2025-0076","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"289-294"},"PeriodicalIF":0.0,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12279401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144683977","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}

{"title":"Difficulties of Differentiating Primary Histiocytic Sarcoma of the Central Nervous System from Glioblastoma in Older Patients: Methylation Analysis and Review of the Literature.","authors":"Ryota Taomoto, Mikiko Aoki, Toshiyuki Enomoto, Noriko Hirao, Hiroshi Abe, Junji Shibahara, Koichi Ichimura, Kaishi Satomi, Makoto Hamasaki","doi":"10.2176/jns-nmc.2024-0335","DOIUrl":"10.2176/jns-nmc.2024-0335","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"275-281"},"PeriodicalIF":0.0,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12279446/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144692988","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}

{"title":"The Efficacy of Atlantoaxial Facet Joint Distraction and Fixation and Foramen Magnum Decompression for Chiari Type 1 Malformation with Basilar Invagination: A Case Report.","authors":"Kotaro Kohara, Tatsuya Maegawa, Eitaro Okumura, Ryo Hashimoto, Motoo Kubota","doi":"10.2176/jns-nmc.2025-0052","DOIUrl":"10.2176/jns-nmc.2025-0052","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"283-288"},"PeriodicalIF":0.0,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12279402/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144683978","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}

{"title":"A Rare Case of Brain Biopsy-confirmed Neurolymphomatosis Involving the Central Nervous System Mimicking Polyneuropathy as a Complication of Refractory Sjögren's Syndrome.","authors":"Yusuke Okamura, Hiroaki Nagashima, Kazuhiro Tanaka, Norio Chihara, Kenji Sekiguchi, Riki Matsumoto, Takashi Sasayama","doi":"10.2176/jns-nmc.2024-0150","DOIUrl":"10.2176/jns-nmc.2024-0150","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":101331,"journal":{"name":"NMC case report journal","volume":"12 ","pages":"249-254"},"PeriodicalIF":0.0,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12208793/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144546717","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}