Y. Mizoi, Kei Ikeda, M. Sonoo, Y. Hatanaka, Toshimasa Yamamoto
A 56‐year‐old man admitted with coronavirus disease 2019 (COVID‐19) became severely ill, required ventilator support and intensive care unit management. After ventilator weaning, he was found to have quadriparesis. Guillain–Barré syndrome (GBS) was suspected and administration of high‐dose intravenous immunoglobulin (IVIg) was considered. However, markedly prolonged compound muscle action potential (CMAP) duration was observed, leading to a diagnosis of critical illness myopathy (CIM). Therefore, IVIg was not given at all, and supportive care was continued. A good functional recovery was obtained. Prolonged CMAP duration is a characteristic finding to CIM. CIM following severe COVID‐19 infection is probably common, although the diagnostic value of prolonged CMAP duration is not widely recognized. This characteristic finding deserves more attention because it contributes to early differentiation between CIM and GBS and the use of IVIg in patients with COVID‐19 may cause thrombotic complications and worsen the prognosis.
{"title":"Markedly prolonged compound muscle action potential duration in a patient with coronavirus disease 2019 related critical illness myopathy","authors":"Y. Mizoi, Kei Ikeda, M. Sonoo, Y. Hatanaka, Toshimasa Yamamoto","doi":"10.1111/ncn3.12703","DOIUrl":"https://doi.org/10.1111/ncn3.12703","url":null,"abstract":"A 56‐year‐old man admitted with coronavirus disease 2019 (COVID‐19) became severely ill, required ventilator support and intensive care unit management. After ventilator weaning, he was found to have quadriparesis. Guillain–Barré syndrome (GBS) was suspected and administration of high‐dose intravenous immunoglobulin (IVIg) was considered. However, markedly prolonged compound muscle action potential (CMAP) duration was observed, leading to a diagnosis of critical illness myopathy (CIM). Therefore, IVIg was not given at all, and supportive care was continued. A good functional recovery was obtained. Prolonged CMAP duration is a characteristic finding to CIM. CIM following severe COVID‐19 infection is probably common, although the diagnostic value of prolonged CMAP duration is not widely recognized. This characteristic finding deserves more attention because it contributes to early differentiation between CIM and GBS and the use of IVIg in patients with COVID‐19 may cause thrombotic complications and worsen the prognosis.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"167 - 169"},"PeriodicalIF":0.4,"publicationDate":"2023-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46892505","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}
S. Toi, M. Hosoya, K. Ishizuka, T. Hoshino, K. Kitagawa
Long‐time monitoring of electrocardiogram with an implantable loop recorder is useful for detection of paroxysmal atrial fibrillation (PAF) in ESUS patients. However, usefulness of noninvasive 7‐day Holter monitoring has not been established. Furthermore, the incidence of other arrhythmias such as sinus pause (SP) remains unknown.
{"title":"Detection of atrial fibrillation and sinus pause in embolic stroke of undetermined sources by chest strap‐style 7‐day Holter monitoring","authors":"S. Toi, M. Hosoya, K. Ishizuka, T. Hoshino, K. Kitagawa","doi":"10.1111/ncn3.12704","DOIUrl":"https://doi.org/10.1111/ncn3.12704","url":null,"abstract":"Long‐time monitoring of electrocardiogram with an implantable loop recorder is useful for detection of paroxysmal atrial fibrillation (PAF) in ESUS patients. However, usefulness of noninvasive 7‐day Holter monitoring has not been established. Furthermore, the incidence of other arrhythmias such as sinus pause (SP) remains unknown.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"146 - 151"},"PeriodicalIF":0.4,"publicationDate":"2023-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49655839","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}
Yuko Kataoka, Masataka Nakamura, T. Kunieda, S. Kaneko, I. Mizuta, T. Mizuno, Yakushiji Yusuke
We report a 50‐year‐old man with no history of stroke or migraine, who developed progressive gait ataxia and dementia. MRI showed diffuse leukoencephalopathy, multiple lacunar lesions, and cerebellar atrophy. Ischemic lesions were absent in the cerebellum. On SPECT, crossed cerebellar diaschisis (CCD) was seen. Genetic testing confirmed the diagnosis of cerebral autosomal‐dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with a heterozygous NOTCH3 mutation p.Arg182Cys. His cerebellar dysfunction may have been caused by CCD due to interruption of cerebral‐cerebellar connections. Our case showed that CCD could be important for cerebellar function in CADASIL.
{"title":"Possible association of crossed cerebellar diaschisis with cerebellar ataxia in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy","authors":"Yuko Kataoka, Masataka Nakamura, T. Kunieda, S. Kaneko, I. Mizuta, T. Mizuno, Yakushiji Yusuke","doi":"10.1111/ncn3.12702","DOIUrl":"https://doi.org/10.1111/ncn3.12702","url":null,"abstract":"We report a 50‐year‐old man with no history of stroke or migraine, who developed progressive gait ataxia and dementia. MRI showed diffuse leukoencephalopathy, multiple lacunar lesions, and cerebellar atrophy. Ischemic lesions were absent in the cerebellum. On SPECT, crossed cerebellar diaschisis (CCD) was seen. Genetic testing confirmed the diagnosis of cerebral autosomal‐dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with a heterozygous NOTCH3 mutation p.Arg182Cys. His cerebellar dysfunction may have been caused by CCD due to interruption of cerebral‐cerebellar connections. Our case showed that CCD could be important for cerebellar function in CADASIL.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"164 - 166"},"PeriodicalIF":0.4,"publicationDate":"2023-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44120979","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}
T. Sugimoto, K. Kurokawa, H. Naito, T. Kono, E. Nomura, H. Maruyama
Precise diagnosis of amyotrophic lateral sclerosis (ALS) and differential diagnosis between ALS and other diseases are important. For ALS diagnosis, not only needle electromyography but also decremental responses to repetitive nerve stimulation (RNS) and a decreased abductor pollicis brevis (APB)/abductor digiti minimi (ADM) amplitude ratio in nerve conduction studies (NCS) are useful. We should know the incidence of decrement in RNS responses and the decreased amplitude ratio in NCS in ALS patients with upper limb symptoms.
{"title":"Features of repetitive nerve stimulation and nerve conduction studies in patients with amyotrophic lateral sclerosis","authors":"T. Sugimoto, K. Kurokawa, H. Naito, T. Kono, E. Nomura, H. Maruyama","doi":"10.1111/ncn3.12701","DOIUrl":"https://doi.org/10.1111/ncn3.12701","url":null,"abstract":"Precise diagnosis of amyotrophic lateral sclerosis (ALS) and differential diagnosis between ALS and other diseases are important. For ALS diagnosis, not only needle electromyography but also decremental responses to repetitive nerve stimulation (RNS) and a decreased abductor pollicis brevis (APB)/abductor digiti minimi (ADM) amplitude ratio in nerve conduction studies (NCS) are useful. We should know the incidence of decrement in RNS responses and the decreased amplitude ratio in NCS in ALS patients with upper limb symptoms.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"134 - 139"},"PeriodicalIF":0.4,"publicationDate":"2023-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48419119","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}
S. Khadilkar, H. Oza, Pramod Dhonde, Bhagyadhan A. Patel, M. Singla, Riddhi Patel, Rashna S. Dastur, Pradnya Gaitode, Hiral Halani, K. Mansukhani, Mayura Dhonde
Congenital myasthenic syndromes (CMS) are rare inherited heterogeneous disorders of neuromuscular transmission.
先天性肌无力综合征(CMS)是一种罕见的遗传性异质神经肌肉传递疾病。
{"title":"Congenital myasthenic syndromes: A study of 15 cases","authors":"S. Khadilkar, H. Oza, Pramod Dhonde, Bhagyadhan A. Patel, M. Singla, Riddhi Patel, Rashna S. Dastur, Pradnya Gaitode, Hiral Halani, K. Mansukhani, Mayura Dhonde","doi":"10.1111/ncn3.12700","DOIUrl":"https://doi.org/10.1111/ncn3.12700","url":null,"abstract":"Congenital myasthenic syndromes (CMS) are rare inherited heterogeneous disorders of neuromuscular transmission.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"127 - 133"},"PeriodicalIF":0.4,"publicationDate":"2023-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43173490","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}
Yoshio Tsuboi, K. Wada-isoe, Hiroyuki Kondo, Yoshitsugu Kojima, T. Takeshima, K. Iwasaki
Parkinson's disease is common in elderly people and is treated in the neurology department as well as in other departments. Recently, treatment options for Parkinson's disease have expanded; this complicates selection of the best treatment regimen.
{"title":"Differences in Parkinson's disease treatment between neurology and other departments in Japan","authors":"Yoshio Tsuboi, K. Wada-isoe, Hiroyuki Kondo, Yoshitsugu Kojima, T. Takeshima, K. Iwasaki","doi":"10.1111/ncn3.12699","DOIUrl":"https://doi.org/10.1111/ncn3.12699","url":null,"abstract":"Parkinson's disease is common in elderly people and is treated in the neurology department as well as in other departments. Recently, treatment options for Parkinson's disease have expanded; this complicates selection of the best treatment regimen.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"79 - 89"},"PeriodicalIF":0.4,"publicationDate":"2023-01-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45690627","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}
Takumi Ikeda, Takeo Sato, T. Komatsu, Kenichiro Sakai, T. Umehara, S. Omoto, Hidetomo Murakami, Hidetaka Mitsumura, S. Matsushima, Reichi Makishima, Hiroyuki Takahashi, T. Ishibashi, Y. Murayama, Y. Iguchi
A 76‐year‐old man developed delayed leukoencephalopathy 3 weeks after successful mechanical thrombectomy for middle cerebral artery occlusion. Pathological evaluation of the removed thrombus showed cholesterol clefts and fibrin with inflammatory cell infiltration, which were compatible with aortogenic embolism. Delayed leukoencephalopathy following recanalization in ischemic stroke has only been rarely reported, and the etiology in all the previously reported cases was cardioembolism, which is distinct from our case. Through this case, we considered that disruption of the blood–brain barrier caused by thrombus composed of cholesterol crystals and fibrin could be a trigger for delayed leukoencephalopathy. This case should provide better understanding of delayed leukoencephalopathy after acute ischemic stroke.
{"title":"Aortogenic emboli might play an important role in the development of delayed leukoencephalopathy after mechanical thrombectomy","authors":"Takumi Ikeda, Takeo Sato, T. Komatsu, Kenichiro Sakai, T. Umehara, S. Omoto, Hidetomo Murakami, Hidetaka Mitsumura, S. Matsushima, Reichi Makishima, Hiroyuki Takahashi, T. Ishibashi, Y. Murayama, Y. Iguchi","doi":"10.1111/ncn3.12698","DOIUrl":"https://doi.org/10.1111/ncn3.12698","url":null,"abstract":"A 76‐year‐old man developed delayed leukoencephalopathy 3 weeks after successful mechanical thrombectomy for middle cerebral artery occlusion. Pathological evaluation of the removed thrombus showed cholesterol clefts and fibrin with inflammatory cell infiltration, which were compatible with aortogenic embolism. Delayed leukoencephalopathy following recanalization in ischemic stroke has only been rarely reported, and the etiology in all the previously reported cases was cardioembolism, which is distinct from our case. Through this case, we considered that disruption of the blood–brain barrier caused by thrombus composed of cholesterol crystals and fibrin could be a trigger for delayed leukoencephalopathy. This case should provide better understanding of delayed leukoencephalopathy after acute ischemic stroke.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"103 - 106"},"PeriodicalIF":0.4,"publicationDate":"2023-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46959571","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}
Haruka Ishibashi, Katsuya Kobayashi, Maya Tojima, S. Neshige, T. Hitomi, H. Ishiura, S. Tsuji, H. Maruyama, Ryosuke Takahashi, A. Ikeda
The clinical diagnostic criteria for benign adult familial myoclonus epilepsy (BAFME) originally included (1) cortical tremor and infrequent generalized seizures, (2) autosomal dominant inheritance, (3) lack of cognitive decline and other neurological symptoms, (4) electrophysiological findings of cortical reflex myoclonus, and (5) lack of clear clinical progression (BAFME criteria‐1). It was revised such that (1) included partial seizures, and (3) and (5) may develop among middle‐aged patients (Revised criteria‐2). The Japanese Ministry of Health, Labor and Welfare proposed their criteria, which included the EEG and MRI findings (MHLW criteria‐3). Recently, high‐frequency oscillations, superimposed on the giant somatosensory evoked potential P25 component (P25‐HFOs), have been found useful as a biomarker for BAFME diagnosis.
{"title":"Clinical diagnostic criteria of benign adult familial myoclonus epilepsy type 1 are highly concordant with genetic testing","authors":"Haruka Ishibashi, Katsuya Kobayashi, Maya Tojima, S. Neshige, T. Hitomi, H. Ishiura, S. Tsuji, H. Maruyama, Ryosuke Takahashi, A. Ikeda","doi":"10.1111/ncn3.12696","DOIUrl":"https://doi.org/10.1111/ncn3.12696","url":null,"abstract":"The clinical diagnostic criteria for benign adult familial myoclonus epilepsy (BAFME) originally included (1) cortical tremor and infrequent generalized seizures, (2) autosomal dominant inheritance, (3) lack of cognitive decline and other neurological symptoms, (4) electrophysiological findings of cortical reflex myoclonus, and (5) lack of clear clinical progression (BAFME criteria‐1). It was revised such that (1) included partial seizures, and (3) and (5) may develop among middle‐aged patients (Revised criteria‐2). The Japanese Ministry of Health, Labor and Welfare proposed their criteria, which included the EEG and MRI findings (MHLW criteria‐3). Recently, high‐frequency oscillations, superimposed on the giant somatosensory evoked potential P25 component (P25‐HFOs), have been found useful as a biomarker for BAFME diagnosis.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"140 - 145"},"PeriodicalIF":0.4,"publicationDate":"2023-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41344356","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}
Houssem Slimène, K. Moalla, S. Sakka, F. Nouha, M. Damak, C. Mhiri
Mild encephalopathy with reversible splenial lesion (MERS) is a rare clinicoradiological entity characterized by a transient mild encephalopathy and MRI findings of a reversible lesion in the splenium of the corpus callosum. Here, we report an atypical case of MERS in a 22‐year‐old woman who presented with 2‐day history of headaches and confusion associated with fever and who completely recovered within 7 days. Exhaustive biological tests panel revealed positive IgM rickettsial antibody, and the final diagnosis was MERS caused by rickettsial infection. Although viruses are the most common pathogen of MERS, for proper management, bacteria should be considered, as they may also lead to this condition.
{"title":"Rickettsial infection‐related encephalitis/encephalopathy with reversible splenial lesion of the corpus callosum","authors":"Houssem Slimène, K. Moalla, S. Sakka, F. Nouha, M. Damak, C. Mhiri","doi":"10.1111/ncn3.12697","DOIUrl":"https://doi.org/10.1111/ncn3.12697","url":null,"abstract":"Mild encephalopathy with reversible splenial lesion (MERS) is a rare clinicoradiological entity characterized by a transient mild encephalopathy and MRI findings of a reversible lesion in the splenium of the corpus callosum. Here, we report an atypical case of MERS in a 22‐year‐old woman who presented with 2‐day history of headaches and confusion associated with fever and who completely recovered within 7 days. Exhaustive biological tests panel revealed positive IgM rickettsial antibody, and the final diagnosis was MERS caused by rickettsial infection. Although viruses are the most common pathogen of MERS, for proper management, bacteria should be considered, as they may also lead to this condition.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"100 - 102"},"PeriodicalIF":0.4,"publicationDate":"2023-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45264636","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}
We report a female patient who developed Pompe disease at 15 years old and received enzyme replacement therapy (ERT) from 25 years old. After 14 years of treatment, she became pregnant. ERT was suspended during the organogenic period as a precaution and restarted at 15 weeks' gestation. During the pregnancy, there were no complications or respiratory problems, but transient muscle weakness was observed during the third trimester. The patient gave birth by vaginal delivery at 39 weeks' gestation. Eight months postpartum, her muscle weakness had recovered slowly, and the patient was able to stand up without help.
{"title":"Pregnancy during enzyme replacement therapy with alglucosidase alfa over a 14‐year period in late‐onset Pompe disease","authors":"C. Yanagihara, Yuki Hayasaka, Y. Kageyama","doi":"10.1111/ncn3.12695","DOIUrl":"https://doi.org/10.1111/ncn3.12695","url":null,"abstract":"We report a female patient who developed Pompe disease at 15 years old and received enzyme replacement therapy (ERT) from 25 years old. After 14 years of treatment, she became pregnant. ERT was suspended during the organogenic period as a precaution and restarted at 15 weeks' gestation. During the pregnancy, there were no complications or respiratory problems, but transient muscle weakness was observed during the third trimester. The patient gave birth by vaginal delivery at 39 weeks' gestation. Eight months postpartum, her muscle weakness had recovered slowly, and the patient was able to stand up without help.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"97 - 99"},"PeriodicalIF":0.4,"publicationDate":"2023-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41440052","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}
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