Case Reports in Neurology最新文献

{"title":"Flank Pain as a First Symptom of a Diffuse Midline Glioma.","authors":"Burc Bassa,&nbsp;Achim Battmann,&nbsp;Eva Maria Craemer,&nbsp;Uta Meyding-Lamadé","doi":"10.1159/000528311","DOIUrl":"https://doi.org/10.1159/000528311","url":null,"abstract":"<p><p>Diffuse midline gliomas are a new entity in the WHO Classification of Tumors of the Central Nervous System, corresponding to grade 4 gliomas. The diagnostic pathognomonic feature is the presence of a H3K27M mutation. Although mainly seen in children, cases in adults have also been reported. The symptoms are highly variable and usually dependent on the location and extent of spinal cord compression.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"1-5"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0a/a0/crn-2023-0015-0001-528311.PMC9893990.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10662350","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":"Hypoglycemic Encephalopathy Manifesting with Cortical Hemichorea-Hemiballismus Syndrome: A Case Report.","authors":"Francesco Pasini,&nbsp;Aristotelis Karantzoulis,&nbsp;Gaia Fanella,&nbsp;Francesco Brovelli,&nbsp;Davide Iacobucci,&nbsp;Vittoria Aprea,&nbsp;Benedetta Storti,&nbsp;Francesco Santangelo,&nbsp;Francesco Canonico,&nbsp;Paolo Remida,&nbsp;Carlo Ferrarese,&nbsp;Laura Brighina","doi":"10.1159/000528880","DOIUrl":"https://doi.org/10.1159/000528880","url":null,"abstract":"<p><p>Hyper-/hypoglycemic states are rare but well-established causes of hyperkinetic movements, including chorea and ballismus, usually associated with brain lesions in the basal ganglia. We report a case of hemichorea-hemiballismus (HCHB) syndrome that developed after a severe hypoglycemic episode in a 71-year-old man with poorly controlled type 2 diabetes mellitus. Uncommonly, brain MRI showed contralateral cortical-subcortical T2 and T2-FLAIR-hyperintense frontoparietal lesions, with cingulate gyrus involved, while the basal ganglia were unaffected. In patients with hypoglycemic encephalopathy associated with cortical lesions, the long-term prognosis is usually poor. Nevertheless, in our patient, the dyskinesias and the cerebral lesions progressively regressed by achieving good glycemic control. After four and 12 months, the patient's neurological examination was normal. To our knowledge, this is the first evidence of hypoglycemic etiology of cortical HCHB syndrome, supporting recent theories that cortical circuitries may independently contribute to the pathogenesis of chorea and ballismus. This is also the first report of cingulate gyrus involvement in hypoglycemic encephalopathy. Finally, this case may indicate that a subset of patients with cortical lesions due to hypoglycemia could present a good clinical outcome, likely depending on the size of the lesions and the duration and severity of the hypoglycemic episode.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"24-30"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/88/83/crn-2023-0015-0001-528880.PMC9906039.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10679759","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":"Unilateral Wing-Beating Tremor in Neuronal Intranuclear Inclusion Disease.","authors":"Atsuhiko Sugiyama,&nbsp;Kazuho Kojima,&nbsp;Shigeki Hirano,&nbsp;Jun Sone,&nbsp;Satoshi Kuwabara","doi":"10.1159/000531388","DOIUrl":"https://doi.org/10.1159/000531388","url":null,"abstract":"<p><p>Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease with various neurological manifestations, including tremor. Here, we report a case involving a 68-year-old man with an 8-year history of tremor in his right arm. Subsequently, examination revealed that the patient was suffering from a low-frequency, high-amplitude, and posture-induced proximal arm tremor elicited by sustained arm abduction with flexed elbows (wing-beating tremor), which was partially improved by zonisamide treatment. Abnormal expansion of GGC repeats in the <i>NOTCH2NLC</i> gene confirmed the diagnosis of NIID. This case highlights the fact that unilateral wing-beating tremor can be a manifestation of NIID. Zonisamide may be effective for controlling tremors associated with NIID.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"126-130"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/26/12/crn-2023-0015-0001-531388.PMC10359687.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10240542","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":"Hypertrophic Pachymeningitis with Persistent Intrathecal Inflammation Secondary to Neurosarcoidosis Treated with Intraventricular Chemotherapy: A Case Report.","authors":"Ana Luísa de Almeida Marcelino,&nbsp;Simon Streit,&nbsp;Marie Alice Homeyer,&nbsp;Hans-Christian Bauknecht,&nbsp;Helena Radbruch,&nbsp;Klemens Ruprecht,&nbsp;Harald Prüss","doi":"10.1159/000531229","DOIUrl":"https://doi.org/10.1159/000531229","url":null,"abstract":"<p><p>Hypertrophic pachymeningitis (HP) is a rare immune-mediated disease characterized by thickening of the dura mater with consecutive cranial neuropathy. While HP is usually treated with systemic immunotherapies, response to therapy is variable and may be limited by insufficient drug concentrations in the brain. We report on a 57-year-old patient with HP manifesting with vision and hearing loss who had sustained clinical progression despite various systemic immunotherapies. Intraventricular chemotherapy with methotrexate, cytarabine, and dexamethasone was initiated. We present clinical, imaging and cerebrospinal fluid (CSF) findings, including cytokine levels before and after intraventricular treatment: rapid decrease of cell count, lactate and profibrotic cytokine levels in the CSF following intraventricular chemotherapy was paralleled by a mild reduction of dura thickness in MRI. The already severely impaired visual acuity and hearing loss did not progress further. Treatment was complicated by exacerbation of previously subtle psychiatric symptoms. Follow-up was terminated after 6 months as the patient suffered from a fatal ischemic stroke. Autopsy revealed neurosarcoidosis as the underlying cause of HP. This case report suggests that intrathecal chemotherapy can reduce the inflammatory milieu in the CNS and should be considered for treatment-refractory HP before irreversible damage of cranial nerves has occurred.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"87-94"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10294282/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10201097","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":"Improved Osteoarthritis during Erenumab Treatment for Migraine: A Case Report.","authors":"Frederik Thal Jantzen,&nbsp;Rune Häckert Christensen,&nbsp;Basit Ali Chaudhry,&nbsp;Faisal Mohammad Amin","doi":"10.1159/000530138","DOIUrl":"https://doi.org/10.1159/000530138","url":null,"abstract":"<p><p>Antibodies blocking the calcitonin gene-related peptide have revolutionized episodic and chronic migraine treatment. However, their applicability to non-cephalic pain conditions, such as osteoarthritis, is yet unknown. Osteoarthritis remains a clinical challenge, associated with high disability and limited treatment options. Like migraine, neuropeptides including calcitonin gene-related peptides are involved in its pathophysiology. We present the first case of a patient: a 73-year-old female with osteoarthritis who received monthly treatment for her chronic migraine with 140 mg subcutaneous erenumab, a monoclonal antibody against the receptor of calcitonin gene-related peptide. Though the migraine was unresponsive, the patient's arthritic symptoms improved drastically during treatment period with erenumab; daily pain decreased from VAS 7 to 2, and walking distance doubled from 1,000 m to 2,000 m. The arthritic symptoms relapsed after discontinuation of erenumab. Erenumab could potentially have beneficial effects on symptoms of osteoarthritis. Future studies investigating these effects are warranted.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"95-99"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6b/f6/crn-2023-0015-0001-530138.PMC10363275.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9867595","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":"An Autopsy Case of Elderly Onset Brainstem Acute Disseminated Encephalomyelitis.","authors":"Yasuyuki Takai,&nbsp;Shinsuke Tobisawa,&nbsp;Asuka Funai,&nbsp;Takashi Komori,&nbsp;Kazushi Takahashi","doi":"10.1159/000529180","DOIUrl":"https://doi.org/10.1159/000529180","url":null,"abstract":"<p><p>Acute disseminated encephalomyelitis (ADEM), which is a disease that causes multifocal inflammatory demyelination of the central nervous system, occurs predominantly in children and young adults. We report an autopsy case of an elderly man with brainstem ADEM that progressed over a period of about 3 months. An 82-year-old man developed disturbance of consciousness, dysphagia, and ataxic gait over a period of about 3 months. He was admitted to another hospital for aspiration pneumonia and recovered but was transferred to our hospital due to prolonged disturbance of consciousness. The patient was able to follow simple commands but had a tendency to somnolence. In addition to meningeal stimulation signs, the patient had left-dominant upper and lower limb ataxia and right-dominant limb spasticity. Brain FLAIR/T2-weighted imaging showed high-intensity lesions from the brainstem to the middle cerebellar peduncle bilaterally, medulla oblongata and upper cervical spinal cord, and T1-weighted imaging revealed contrast-enhanced lesions in the left middle cerebellar peduncle and cervical spinal cord. Although spinal fluid examination revealed elevated proteins, other laboratory tests indicated no evidence of infection, vasculitis, collagen diseases or tumors, and anti-ganglioside, anti-AQP4 and anti-MOG antibodies were negative. After admission, the patient again developed aspiration pneumonia, which progressed to acute respiratory distress syndrome, and he died on the 15th day of hospitalization. Autopsy findings indicated acute and subacute demyelination mainly in the brainstem and cerebellum, and perivascular lymphocyte and macrophage infiltration in the areas of demyelination. A postmortem diagnosis of ADEM was made based on the generally monophasic course of the disease and the absence of regenerating myelinated sheaths. There are very few reports of elderly patients with brainstem ADEM. ADEM should be considered as a differential diagnosis in patients with brainstem encephalitis.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"31-40"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a9/28/crn-2023-0015-0001-529180.PMC9929651.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10756206","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":"Olfactory Dysfunction, an Often Neglected Symptom of Hydrocephalus: Experience from a Case of Late-Onset Idiopathic Aqueductal Stenosis.","authors":"Naoya Yamazaki,&nbsp;Takafumi Hasegawa,&nbsp;Kensuke Ikeda,&nbsp;Ako Miyata,&nbsp;Shin-Ichiro Osawa,&nbsp;Kuniyasu Niizuma,&nbsp;Shigenori Kanno,&nbsp;Teiji Tominaga,&nbsp;Masashi Aoki","doi":"10.1159/000529532","DOIUrl":"https://doi.org/10.1159/000529532","url":null,"abstract":"<p><p>Disturbance of smell is often accompanied with common neurodegenerative diseases such as Parkinson's and Alzheimer's diseases. In addition, patients with head trauma, intracranial tumors, and hydrocephalus can also develop olfactory dysfunction, and some of which can improve with treatment of the underlying disease. In clinical practice, few patients complain of smell disturbances, thus olfactory dysfunction is often overshadowed by visible motor symptoms. Herein, we report a case of late-onset idiopathic aqueductal stenosis, a rare form of adult-onset hydrocephalus in which olfactory dysfunction and gait disturbance was markedly improved after endoscopic ventriculostomy. This case report is expected to make more physicians aware that hydrocephalus can cause olfactory dysfunction and that it can be corrected postoperatively. Furthermore, in addition to motor and neuropsychological function, olfactory function test might be useful for functional assessment before and after surgical treatment of hydrocephalus.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"41-47"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/83/f6/crn-2023-0015-0001-529532.PMC9978917.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9410778","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":"Cerebral Sinus Vein Thrombosis following Sneezing: Case Report.","authors":"Helal Nashef,&nbsp;Salo Haratz,&nbsp;Rom Mendel","doi":"10.1159/000530812","DOIUrl":"https://doi.org/10.1159/000530812","url":null,"abstract":"<p><p>Cerebral sinus vein thrombosis (CVT) is a relatively rare neurovascular entity, usually associated with acquired or genetic hypercoagulable states, and in many cases it remains idiopathic. Trauma is also associated with CVT among patients with major head or neck trauma, including penetrating injuries. However, CVT associated with acceleration trauma has only been described in few cases so far. We present an unusual case of a 19-year-old woman with no past medical history, admitted with an extensive CVT following sneezing. A thorough investigation did not reveal any other potential etiology or risk factor other than estrogen-containing oral contraceptives. The patient was treated with anticoagulation and improved clinically with complete recanalization on follow-up imaging. This case suggests acceleration trauma may be a potential factor of risk for CVT.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"108-112"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9b/a9/crn-2023-0015-0001-530812.PMC10353342.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10221429","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":"Efficacy of Intravenous Immunoglobulins against Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids: A Case Report.","authors":"Takumi Tsuchida,&nbsp;Shigehisa Ura,&nbsp;Ichiro Yabe","doi":"10.1159/000529121","DOIUrl":"https://doi.org/10.1159/000529121","url":null,"abstract":"<p><p>Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly affects the brainstem. Apart from corticosteroids, there are few reported treatment options for CLIPPERS, and there is no standard therapy. A 77-year-old man presented with diplopia that had persisted for 5 months. Dysarthria and numbness of the distal right upper extremity and right lips were also observed. Brain magnetic resonance imaging (MRI) revealed a hyperintense area around the brainstem. Symptoms were relieved immediately following intravenous methylprednisolone (IVMP) administration. However, after gradual tapering of oral prednisolone to 5 mg/day, the symptoms relapsed, and brain imaging revealed that the condition had worsened. Intravenous immunoglobulins (IVIg) were administered for recurrence, with no clinical improvement. After each IVMP treatment, the patient recovered promptly. Based on the patient's symptoms and characteristic MRI findings, exclusion of other diseases, and the significant efficacy of corticosteroids, he was diagnosed with CLIPPERS. There was no recurrence at a maintenance prednisolone dose of 8 mg/day. IVIg had a poor effect on the acute phase of CLIPPERS symptoms. Compared with other immunosuppressants, IVIg is less effective in suppressing the relapse of CLIPPERS.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"15 1","pages":"48-53"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/65/b6/crn-2023-0015-0001-529121.PMC10018422.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9201076","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":"Acknowledgement to Reviewers","authors":"","doi":"10.1159/000528563","DOIUrl":"https://doi.org/10.1159/000528563","url":null,"abstract":"<br />Case Rep Neurol 2022;14:491–493","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"80 4","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138508714","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}