Case Reports in Neurology最新文献

{"title":"Delayed Remote Intracerebral Hemorrhage following Coil Embolization Using a Simple Technique for Ruptured Anterior Communicating Artery Aneurysm: A Case Report of Subarachnoid Hemorrhage.","authors":"Akihiro Okada, Hiroaki Minami, Kanae Nakasho, Yasunori Yoshida, Atsushi Matsumoto, Hiroaki Matsumoto, Yusuke Tomogane, Atsushi Masuda, Ikuya Yamaura, Yasuhisa Yoshida","doi":"10.1159/000553347","DOIUrl":"10.1159/000553347","url":null,"abstract":"<p><strong>Introduction: </strong>Delayed remote intracerebral hemorrhage (rICH) is a well-established complication following flow diverter placement for intracranial aneurysms, with a reported incidence of 5.7%-8.5%. Although rare, similar events have also been reported after coil embolization, predominantly in cases in which adjunctive techniques are used. We report a case of delayed rICH after coil embolization performed with a simple technique in a patient at high risk of thromboembolism.</p><p><strong>Case presentation: </strong>A 62-year-old female presented with subarachnoid hemorrhage due to a ruptured anterior communicating artery aneurysm. The patient also had suspected lung cancer and a smoking history. Emergency coil embolization was performed using a simple technique under systemic heparinization and dual antiplatelet therapy. Immediately after the procedure, computed tomography showed no abnormalities; however, a follow-up computed tomography scan performed the next day revealed intracerebral hemorrhage compressing the corpus callosum, remote from the treated aneurysm. No bleeding source was identified angiographically. Elevated D-dimer levels were noted postoperatively.</p><p><strong>Conclusion: </strong>Hemorrhagic transformation of microembolic infarctions is considered a possible mechanism of the bleeding. In this case, multiple risk factors (including subarachnoid hemorrhage, malignancy, smoking, and intensive antithrombotic therapy) may have contributed. Although antithrombotic therapy is essential to prevent thromboembolic complications, clinicians should be aware of the rare risk of delayed rICH even when using a simple technique.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"455-460"},"PeriodicalIF":0.4,"publicationDate":"2026-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13466463/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148720095","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":"Hemifacial Microsomia with Extensive Ipsilateral White Matter Hyperintensity: A Case Report.","authors":"Erin Elizabeth Finn, Eoin P Flanagan, John J Chen, Ahmed Z Obeidat, James P Klaas","doi":"10.1159/000553415","DOIUrl":"10.1159/000553415","url":null,"abstract":"<p><strong>Introduction: </strong>Hemifacial microsomia with asymptomatic ipsilateral cerebral white matter changes has been reported once previously. We present a case of hemifacial microsomia with asymptomatic ipsilateral cerebral white matter changes, which was complicated by unrelated comorbid optic neuropathy and cerebrospinal fluid (CSF) pleocytosis.</p><p><strong>Case presentation: </strong>A 54-year-old woman presented with left monocular inferior altitudinal visual field loss and optic disk edema from an optic neuropathy. Her neurologic examination was otherwise normal. MRI brain revealed left cerebral hemisphere white matter nonenhancing T2-weighted signal prolongation concerning for an inflammatory etiology. CSF analysis displayed mild lymphocytic pleocytosis, although protein, infectious studies, cytology, autoimmune/paraneoplastic panel, and other inflammatory markers were normal. Genetic diseases were ruled out. Steroid course did not yield improvement. Over subsequent months, the disk edema resolved leaving optic nerve pallor with a persistent inferior altitudinal defect. Further history revealed congenital left hemifacial microglossia and craniofacial microsomia for which she had reconstructive surgeries. The most likely etiology of her white matter changes was attributed to hemifacial microsomia with extensive ipsilateral white matter hyperintensity, with vision loss secondary to unrelated non-arteritic anterior ischemic optic neuropathy.</p><p><strong>Conclusion: </strong>In patients with craniofacial developmental abnormalities and no other evidence of neurodegenerative or inflammatory disorders, congenital etiologies should be considered in the differential for ipsilateral central nervous system abnormalities. In an era of increased access to both advanced imaging techniques and elective facial reconstructive surgeries, recognition of this association may prevent unnecessary diagnostic testing and reduce the financial and emotional burden for patients with incidentally discovered imaging abnormalities.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"442-447"},"PeriodicalIF":0.4,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13456671/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148705506","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":"Elderly-Onset Moyamoya Disease Presenting with Hemichorea Associated with Basal Ganglia Hyperperfusion: Symptomatic Improvement after Revascularization Surgery.","authors":"Sho Takata, Tomoya Kamide, Takeshi Kidoguchi, Taishi Tsutsui, Kouichi Misaki, Mitsutoshi Nakada","doi":"10.1159/000552937","DOIUrl":"10.1159/000552937","url":null,"abstract":"<p><strong>Introduction: </strong>Chorea has been reported in pediatric moyamoya disease, which is commonly associated with ischemia of the basal ganglia. However, elderly patients are rare, and the underlying mechanisms remain unclear.</p><p><strong>Case presentation: </strong>A 69-year-old woman presented with a 6-month history of involuntary hemichorea affecting her left upper and lower limbs. Magnetic resonance imaging showed no acute infarction but demonstrated right middle cerebral artery (MCA) occlusion. Digital subtraction angiography revealed moyamoya collateralization. The ¹²³I-iodoamphetamine single-photon emission computed tomography scan revealed increased perfusion of basal ganglia and impaired cerebrovascular reserve in the right MCA territory. The patient underwent right superficial temporal artery-MCA bypass combined with encephalo-duro-myo-synangiosis. Postoperatively, basal ganglia hyperperfusion persisted, but cerebrovascular reserve improved. Her hemichorea markedly subsided, and she was discharged without neurological deficits. This is one of the oldest reported cases of moyamoya syndrome presenting with hemichorea.</p><p><strong>Conclusion: </strong>Our findings suggest that chorea associated with moyamoya disease may result not only from decreased, but also from imbalanced basal ganglia perfusion. Revascularization surgery may help restore this balance and alleviate symptoms, even in elderly patients.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"430-435"},"PeriodicalIF":0.4,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13446892/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148683485","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":"Morning-Predominant Dysphagia in Myasthenia Gravis: A Report of Two Cases with Tongue Pressure Measurement.","authors":"Yoshihiro Watanabe, Masako Fujiu-Kurachi, Toshiaki Tamura, Masashi Ishizaki, Koki Maruyama, Kota Imai, Yoichi Sato, Kenshi Terajima","doi":"10.1159/000552889","DOIUrl":"10.1159/000552889","url":null,"abstract":"<p><strong>Introduction: </strong>Myasthenia gravis (MG) is an autoimmune disorder characterized by fatigable muscle weakness. While symptoms typically worsen from afternoon to evening, morning-predominant dysphagia has not been well characterized, and standardized methods to assess diurnal changes and fatigability in dysphagia have not been established. We report two cases of MG presenting with an atypical diurnal pattern of morning-predominant dysphagia, in which maximum tongue pressure (MTP) measurement was useful for objective assessment.</p><p><strong>Case presentations: </strong>Case 1 was an 80-year-old woman without thymoma (anti-acetylcholine receptor [AChR] antibody 18.8 nmol/L, Myasthenia Gravis Foundation of America [MGFA] class IIIb, Myasthenia Gravis Activities of Daily Living [MG-ADL] score 10, percent vital capacity [%VC] 87.8%, Dysphagia Severity Scale [DSS] 3). Case 2 was a 75-year-old woman with thymoma (anti-AChR antibody 42.3 nmol/L, MGFA class IIIa, MG-ADL score 8, %VC 84.8%, DSS 7), who developed dysphagia after thymectomy (DSS 4). MTP demonstrated marked diurnal variation in both cases, with lower values in the morning compared with the afternoon (Case 1: 5.8 vs. 14.9 kPa; Case 2: 8.5 vs. 20.6 kPa). Early morning administration of acetylcholinesterase inhibitors and modification of breakfast texture effectively alleviated symptoms. With immunotherapy and swallowing rehabilitation, swallowing function improved in both cases (Case 1: DSS 6; Case 2: DSS 7).</p><p><strong>Conclusion: </strong>Morning-predominant dysphagia may represent an under-recognized pattern in MG. MTP provides a simple and objective method to quantify diurnal variation in swallowing function and may assist in optimizing medication timing and dietary management, potentially reducing the risk of aspiration and improving clinical outcomes.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"422-429"},"PeriodicalIF":0.4,"publicationDate":"2026-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13441280/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677249","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":"Downbeat Nystagmus and Saccadic Oscillations in Spinocerebellar Ataxia Type 27B: A 14-Month Follow-Up Case Report.","authors":"Pavol Skacik, Monika Turcanova Koprusakova, Stefan Sivak, Milan Grofik, Egon Kurca","doi":"10.1159/000552966","DOIUrl":"10.1159/000552966","url":null,"abstract":"<p><strong>Introduction: </strong>Spinocerebellar ataxia type 27B (SCA27B) is a late-onset autosomal dominant cerebellar disorder associated with oculomotor abnormalities, particularly downbeat nystagmus (DBN) and saccadic oscillations such as square-wave jerks (SWJ). Although these features are common, their longitudinal evolution and response to aminopyridine therapy remain insufficiently characterized.</p><p><strong>Case presentation: </strong>We report a 67-year-old male with genetically confirmed SCA27B. At baseline, he presented with cerebellar ataxia (SARA score 11) and frequent SWJ, while DBN was detectable only during head-shaking. After 12 months, mild clinical progression was observed (SARA score 12) with the emergence of spontaneous DBN, which intensified during provocative maneuvers. Treatment with 4-aminopyridine (20 mg/day) resulted in clinical improvement after 2 months, reflected by a reduced SARA score (8) and improved gait, stance, and speech. However, video-oculography showed only minimal changes in DBN and no significant reduction in SWJ frequency.</p><p><strong>Conclusion: </strong>This case highlights that DBN in SCA27B may initially be detectable only under provocative conditions before becoming spontaneous. Despite clinical improvement with 4-aminopyridine, objective oculomotor parameters remained largely unchanged, suggesting a possible dissociation between clinical and oculomotor outcomes. Larger longitudinal studies are needed to clarify these relationships.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"436-441"},"PeriodicalIF":0.4,"publicationDate":"2026-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13450987/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148697092","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":"Efgartigimod for Anti-n-Methyl-d-Aspartate Shanxi Provincial People's Hospital, Taiyuan, China Receptor Encephalitis: A Case Report and Literature Review.","authors":"Zexing Li, Xiaolei Zhang","doi":"10.1159/000552699","DOIUrl":"10.1159/000552699","url":null,"abstract":"<p><strong>Introduction: </strong>Neonatal Fc receptor (FcRn) antagonists can improve autoimmune diseases such as myasthenia gravis by rapidly and selectively reducing serum IgG levels, and they hold potential for broader applications in other autoimmune disorders.</p><p><strong>Case presentation: </strong>We report a 32-year-old female admitted with psychiatric and behavioral abnormalities, who tested positive for anti-NMDA receptor antibodies indicative of autoimmune encephalitis. Despite first-line therapy with corticosteroids and intravenous immunoglobulin, her cognitive dysfunction progressively worsened, ultimately rendering her unable to communicate. Following four administrations of efgartigimod, the patient exhibited rapid and marked clinical improvement.</p><p><strong>Conclusion: </strong>This review highlights the potential of FcRn antagonists as an additional therapeutic option in clinical practice.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"533-540"},"PeriodicalIF":0.4,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13588615/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149007670","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 Nutritional Axonal Neuropathy in the Setting of Semaglutide-Associated Gastrointestinal Intolerance: A Case Report.","authors":"Chloe J Cohan, Liam Townley, Erik Ortega","doi":"10.1159/000552961","DOIUrl":"10.1159/000552961","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of the study was to report a unique case of severe sensorimotor polyneuropathy due to acute nutritional axonal neuropathy (ANAN) associated with thiamine and other vitamin deficiencies in the setting of semaglutide-related malnutrition, emphasizing early recognition and intervention to prevent permanent neurologic injury.</p><p><strong>Methods: </strong>A 65-year-old woman with type 2 diabetes and obesity presented with profound weakness, cranial nerve deficits, dysphagia, and a 120-pound weight loss after months of semaglutide therapy with persistent nausea and vomiting. Neurologic examination was performed, and cerebrospinal fluid analysis and serum vitamin levels were obtained. Electromyography demonstrated absent sensory responses and mild motor slowing, consistent with severe sensory-predominant polyneuropathy. Serum levels of thiamine, folate, and vitamin B12 were below normal, and supplementation was administered.</p><p><strong>Results: </strong>Within 5 days of thiamine, folate, and vitamin B12 supplementation, the patient showed improvement in cognition and bulbar and limb strength. Oral intake improved over 1 week, and partial functional recovery was achieved.</p><p><strong>Discussion: </strong>This case of ANAN highlights a severe yet potentially reversible neurologic complication of nutritional deficiencies occurring in the setting of GLP-1 receptor agonist-associated gastrointestinal intolerance and malnutrition. Recognizing and addressing nutrient deficiencies in patients with sensorimotor and/or cognitive deficits after persistent gastrointestinal symptoms can improve deficits and prevent long-term or permanent neurologic injury.</p><p><strong>Practical implications: </strong>Clinicians should maintain a high suspicion for thiamine, folate, vitamin B12, and other nutritional deficiencies in patients on GLP-1 receptor agonists presenting with neuropathy, unexplained weakness, cranial nerve deficits, and/or confusion, as prompt supplementation may mitigate neurologic injury.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"402-412"},"PeriodicalIF":0.4,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13432944/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148668252","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":"Successful Recovery from Life-Threatening Ventilatory Failure with Efgartigimod in Seronegative Myasthenia Gravis: Two Case Reports.","authors":"Satoshi Tada, Hayato Yabe","doi":"10.1159/000552505","DOIUrl":"10.1159/000552505","url":null,"abstract":"<p><strong>Introduction: </strong>In Japan, efgartigimod is approved for treating generalized myasthenia gravis in patients who are anti-acetylcholine receptor antibody-positive, anti-muscle-specific kinase antibody-positive, or seronegative. Efgartigimod efficacy has been reported in seropositive patients with myasthenic crisis; however, evidence in seronegative patients with myasthenic crisis remains limited. We report 2 patients with seronegative generalized myasthenia gravis presenting with myasthenic crisis as the initial manifestation, successfully treated with efgartigimod.</p><p><strong>Case presentation: </strong>Case 1 involved an 83-year-old man admitted with acute dyspnea requiring mechanical ventilation for respiratory failure initially attributed to acute exacerbation of heart failure with pleural effusion. Due to poor respiratory recovery, he was referred to neurology on hospital day 14. Clinical features including ventilatory impairment and fatigable limb weakness, together with electrophysiological findings, supported myasthenia gravis diagnosis. Following oral immunosuppressants and intravenous efgartigimod, he was weaned from mechanical ventilation on hospital day 42. Case 2 involved a 75-year-old man with progressive dyspnea and signs of right heart failure requiring mechanical ventilation for respiratory failure. He was referred to neurology on hospital day 2. Electrophysiological testing under sedation suggested myasthenia gravis. After methylprednisolone pulse therapy, subcutaneous efgartigimod was initiated, achieving ventilator liberation on hospital day 40.</p><p><strong>Conclusion: </strong>Efgartigimod was effective in treating myasthenic crisis requiring mechanical ventilation in seronegative generalized myasthenia gravis, regardless of autoantibody status. These cases suggest efgartigimod may serve as an alternative or adjunctive therapeutic option for severe myasthenia gravis exacerbations.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"394-401"},"PeriodicalIF":0.4,"publicationDate":"2026-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13423331/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148629410","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":"Carotid Web as a Treatable Etiology of Recurrent Cryptogenic Ischemic Stroke Highlighting Neurointervention: A Case Report and Literature Review.","authors":"Manal Ayesh Alanazi, Taha Khayat, Ibrahim Ismail Ibrahim, Samar Alharbi","doi":"10.1159/000553014","DOIUrl":"10.1159/000553014","url":null,"abstract":"<p><strong>Introduction: </strong>Carotid web is a rare focal variant of intimal fibromuscular dysplasia characterized by a thin, shelf-like projection from the posterior wall of the carotid bulb into the arterial lumen. This abnormality can disturb blood flow and promote thrombus formation, increasing the risk of transient ischemic attacks (TIAs) and ischemic strokes.</p><p><strong>Case presentation: </strong>A 55-year-old male, known case of hypertension, presented with sudden-onset dysarthria and left-sided weakness. Initial imaging revealed no acute infarction, and an underlying cause remained unclear. Despite receiving dual antiplatelet therapy and statin, he experienced a recurrent TIA within a week. Digital subtraction angiography revealed a focal shelf-like intraluminal projection in the right internal carotid artery, consistent with a carotid web. Carotid artery stenting (CAS) was performed successfully without any immediate complications. The patient was then commenced on clopidogrel for 3 months and lifelong aspirin. At the 1-month and 3-month follow-ups, he remained symptom-free and had no recurrence.</p><p><strong>Conclusion: </strong>This case highlights carotid web as a potential but underrecognized cause of recurrent ischemic events, illustrating both the diagnostic challenge of distinguishing it from atherosclerotic plaque with shelf-like configuration and the value of revascularization therapy. Early recognition and timely management by either CAS or carotid endarterectomy can prevent recurrent neurological deficits. Additional studies and case reports are needed to improve understanding of carotid web and to guide the development of evidence-based management strategies.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"413-421"},"PeriodicalIF":0.4,"publicationDate":"2026-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13436940/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148673119","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":"Deep Brain Stimulation Withdrawal Syndrome in Parkinson's Disease: Clinical Features and Outcomes of Two New Cases.","authors":"Emma Ottela, Jaana Rönkä, Valtteri Kaasinen, Juho Joutsa","doi":"10.1159/000552712","DOIUrl":"10.1159/000552712","url":null,"abstract":"<p><strong>Introduction: </strong>Deep brain stimulation (DBS) withdrawal syndrome is a rare but potentially fatal complication in Parkinson's disease (PD), manifesting as akinetic crisis with severe rigidity, fever, autonomic instability, altered mental status, and elevated creatinine kinase (CK) levels. The syndrome is strongly associated with older age, long duration of the disease (>15 years), and the duration of DBS therapy (>5 years), with most reported cases having at least two of these risk factors. However, all available evidence comes from a limited number of case reports.</p><p><strong>Case presentations: </strong>We report two cases of DBS withdrawal syndrome, including a patient without any of the currently acknowledged risk factors. This patient, in their late 50s with young-onset PD, was treated with bilateral subthalamic nucleus DBS with a good response, although requiring high stimulation amplitude (4.1 V bilaterally). The first patient developed an akinetic crisis after battery depletion at a PD duration of 11 years and a DBS duration of 4 years. Implantable pulse generator (IPG) replacement was performed 14 days later, resulting in permanent disability and, eventually, death. The second patient, in their mid-60s with long-standing PD and DBS therapy for 14 years, presented with a severe akinetic crisis and markedly elevated CK. IPG replacement within 2 days led to full recovery.</p><p><strong>Conclusion: </strong>DBS withdrawal syndrome can occur without the typical risk profile, and high stimulation amplitude may represent an additional risk factor. Early recognition and immediate DBS therapy restoration are critical to prevent severe, potentially fatal outcomes.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"18 1","pages":"380-385"},"PeriodicalIF":0.4,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13427027/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148653007","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}