Clinical Neurology最新文献

{"title":"[A case of Parkinson's disease with marked head drop after administration of donepezil hydrochloride].","authors":"Keisuke Hara, Eito Miura, Yoshitaka Umeda, Maiko Umeda, Mutsuo Oyake, Nobuya Fujita","doi":"10.5692/clinicalneurol.cn-001983","DOIUrl":"10.5692/clinicalneurol.cn-001983","url":null,"abstract":"<p><p>A 76-year-old woman with a 2-year history of Parkinson's disease presented with dropped head, which had developed rapidly after she had been prescribed donepezil hydrochloride (DNP) at 3 ‍mg/day. After one month of medication, the extent of the head drop reached 90°. Examination revealed hypertrophy of the left sternocleidomastoid muscle, but no weakness of the extensor muscles in the cervical region. Surface electromyography demonstrated co-‍contraction of the sternocleidomastoid and splenius capitus muscles during head flexion and extension. DNP was withdrawn, resulting in immediate amelioration of the head drop, and complete resolution was achieved after two months. Although head drop is often seen in patients with Parkinson's disease, few previous reports have documented DNP as a causative factor. If patients with Parkinson's disease develop head drop, it is important to investigate any history of DNP medication.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"664-667"},"PeriodicalIF":0.0,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056862","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}

{"title":"[A borderline case between multifocal motor neuropathy and motor chronic inflammatory demyelinating polyneuropathy revealed by the magnetic fatigue test].","authors":"Ryo Fukunaga, Nobuhiro Ogawa, Toshika Hata, Hiroyuki Yabata, Isamu Yamakawa, Makoto Urushitani","doi":"10.5692/clinicalneurol.cn-001974","DOIUrl":"10.5692/clinicalneurol.cn-001974","url":null,"abstract":"<p><p>A 26-year-old woman presented with a seven-month history of weakness in her left upper limb, progressing to difficulty lifting her arms within a few weeks. Her symptoms progressed with fluctuations. For the past three months, she has been unable to stand due to weakness in her proximal lower limbs. Nerve conduction studies did not show any definite conduction block or abnormal sensory conduction, but motor conduction studies showed a slight prolongation of the terminal latency and a decrease in the frequency of the F-wave. A magnetic fatigue test indicated a proximal conduction block. Her symptoms were rapidly resolved with intravenous immunoglobulin treatment, leading to a diagnosis of chronic immune-mediated neuropathy, met both criteria for multifocal motor neuropathy (MMN) and motor chronic inflammatory demyelinating polyneuropathy (CIDP). Our case highlights the utility of the magnetic fatigue test in detecting conduction blocks and its role in differentiating between MMN and motor CIDP.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"642-647"},"PeriodicalIF":0.0,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056858","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}

{"title":"[Clinical and neuropsychological features and changes to regional cerebral blood flow in ‍patients with Parkinson's disease dementia].","authors":"Yasuko Kuroha, Tetsuya Takahashi, Yuki Arai, Mihoko Yoshino, Kensaku Kasuga, Arika Hasegawa, Nae Matsubara, Ryoko Koike, Takeshi Ikeuchi","doi":"10.5692/clinicalneurol.cn-001968","DOIUrl":"10.5692/clinicalneurol.cn-001968","url":null,"abstract":"<p><p>This study aimed to clarify associations of clinical and neuropsychological features and change in regional cerebral blood flow (rCBF) on ¹²³I-IMP-SPECT in patients with Parkinson's disease (PD) who developed dementia. Sixty-one PD patients (mean age, 65.9 ± 8.6 years; mean disease duration, 11.0 ± 11.0 years) were recruited and followed-up for two years. Clinical and neuropsychological characteristics, and rCBF from SPECT were compared between PD patients who developed dementia (PDD+) and those who remained undemented (PDD-). Thirty-eight PD patients (62.3%) were diagnosed with PD-MCI at baseline. During follow-up, 22 PD patients (36%) developed dementia (PDD+). Univariate logistic regression models showed that Hoehn and Yahr scale 4 (odds ratio [OR] 5.85; 95% confidence interval [CI] 1.35-30.75]), visual hallucination (OR 5.95; 95%CI 1.67-25.4]), and PD-MCI (OR 6.47; 95%CI 1.57-39.63]) represented a significant risk factor for PDD+. Among neuropsychological parameters, WAIS (Wechsler Adult Intelligence Scale)-III block design (OR 6.55; 95%CI 1.66-29.84), letter number sequencing (OR 7.01; 95%CI 1.65-36.64), digit-symbol coding (OR 3.90; 95%CI 1.13-14.2), Wechsler Memory Scale, revised (WMS-R) visual paired associates II (delayed recall) (OR 4.68; 95%CI 1.36-17.36), Logical memory I (immediate recall) (OR 8.30; 95%CI 1.37-90.89), Logical memory II (delayed recall) (OR 6.61; 95%CI 1.35-44.33), Visual reproduction I (immediate recall) (OR 7.67; 95%CI 2.11-31.40), and Visual reproduction II (delayed recall) (OR 5.64; 95%CI 1.62-21.47) were significant risk factors. Decreased rCBF assessed using the general linear model (two-sample t-test) by SPM8 was observed in the left precuneus (0, -66, 16), right cuneus (6, -76, 30), and left angular gyrus (-46, -74, 32) in PDD+ compared with PDD- patients. Collectively, we have here shown that clinical and neuropsychological characteristics as well as changes to rCBF in PD patients who converted to PDD+. These features should be carefully monitored to detect the development of dementia in PD patients.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"623-631"},"PeriodicalIF":0.0,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142093932","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}

{"title":"Cancer-associated neuromyelitis optica spectrum disorder: a case report with literature review.","authors":"Naoko Makishi, Keiko Miyazato, Yasuharu Tokuda, Tetsuya Inafuku","doi":"10.5692/clinicalneurol.cn-001943","DOIUrl":"10.5692/clinicalneurol.cn-001943","url":null,"abstract":"<p><p>Neuromyelitis optica spectrum disorders (NMOSD) is one of autoimmune inflammatory diseases and is characterized by area postrema syndrome, brainstem syndrome, optic neuritis, and/or myelitis. Typical myelitis is longitudinally extended transverse myelitis (LETM) which extends over three vertebral bodies. Several previous case reports have suggested association between cancer and NMOSD. A 50-year-old woman had breast cancer and underwent mastectomy and, 10 months later, she had developed acutely progressive dysbasia. Spine MRI showed LETM in 13 vertebrae length and blood test revealed positive anti-aquaporin 4 (anti-AQP4) antibody based on enzyme-linked immunosorbent assay with index of over 40. She was treated by intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin, followed by oral prednisolone. The condition had mostly recovered after the treatment. A small population of NMOSD has the aspect of paraneoplastic neurological syndrome. The age of onset in patients with cancer-associated NMOSD tends to be higher than that in individuals with NMOSD due to any causes of NMOSD.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"564-571"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789399","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}

{"title":"[Herpes simplex encephalitis complicated with cerebral salt wasting syndrome: a case study].","authors":"Fumiya Suzuki, Sunao Takahashi, Ayako Oniki, Shoichiro Ishihara, Hirofumi Yamagishi, Hiroyuki Tomimitsu","doi":"10.5692/clinicalneurol.cn-001966","DOIUrl":"10.5692/clinicalneurol.cn-001966","url":null,"abstract":"<p><p>A 78-year-old man was admitted to the hospital with a 4-day history of fever and confusion. Physical examination revealed oral dryness and decreased skin turgor. Blood tests showed hyponatremia (121.5 ‍mEq/l), and cerebrospinal fluid examination revealed positivity for herpes simplex virus 1 (HSV-1) via polymerase chain reaction. He was diagnosed with herpes simplex encephalitis and initiated acyclovir treatment. The hyponatremia was diagnosed as cerebral salt wasting syndrome (CSWS) and treated with hypertonic saline infusion and fludrocortisone. The cerebrospinal fluid HSV-1 DNA became negative, and the serum sodium levels normalized. Hyponatremia complicated with encephalitis is often caused by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), whereas CSWS is rare, mostly observed in tuberculous meningitis. Differentiating between the SIADH and CSWS is important as they require distinct therapeutic strategies.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"579-582"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789357","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}

{"title":"[Social maturity scores in adults with Down syndrome. Characteristics at the onset of Alzheimer's disease and changes by age group].","authors":"Takashi Kasai, Makiko Shinomoto, Fukiko Kitani-Morii, Takuma Ohmichi, Yuzo Fujino, Masaki Kondo, Naoto Terada, Harutsugu Tatebe, Takahiko Tokuda, Toshiki Mizuno","doi":"10.5692/clinicalneurol.cn-001976","DOIUrl":"10.5692/clinicalneurol.cn-001976","url":null,"abstract":"<p><p>Age-specific characteristics and annual changes of social maturity in adults with Down syndrome (DS) were retrospectively investigated. Forty-six individuals aged 15-58 years were enrolled. Social age (SA) in all domains and subdomains was assessed using the revised S-M social maturity test. Thirty-four cases were evaluated for SA changes over time at 1 year. The SA of adult DS tended to be lower in those under 20 and over 42 years of age. The SA of adults with DS was lowest in the Socialization domain, and this trend was generally common across all age groups. The annual decline of SA was more prominent in the DS-AD group than in the non-DS-AD group. Annual decline of SA in the communication domain had the same discriminative power as that in the whole domains in the discrimination of DS-AD from non-DS-AD. These results are expected to contribute to the development of clinical diagnostic methods for DS-AD in the future.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"550-556"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141761545","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}

{"title":"[Insular lobe epilepsy. Part 1: semiology].","authors":"Koichi Hagiwara","doi":"10.5692/clinicalneurol.cn-001930-1","DOIUrl":"10.5692/clinicalneurol.cn-001930-1","url":null,"abstract":"<p><p>The insula is often referred to as \"the fifth lobe\" of the brain, and its accessibility used to be very limited due to the deep location under the opercula as well as the sylvian vasculature. It was not until the availability of modern stereo-electroencephalography (SEEG) technique that the intracranial electrodes could be safely and chronically implanted within the insula, thereby enabling anatomo-electro-clinical correlations in seizures of this deep origin. Since the first report of SEEG-recorded insular seizures in late 1990s, the knowledge of insular lobe epilepsy (ILE) has rapidly expanded. Being on the frontline for the diagnosis and management of epilepsy, neurologists should have a precise understanding of ILE to differentiate it from epilepsies of other lobes or non-epileptic conditions. Owing to the multimodal nature and rich anatomo-functional connections of the insula, ILE has a wide range of clinical presentations. The following symptoms should heighten the suspicion of ILE: somatosensory symptoms involving a large/bilateral cutaneous territory or taking on thermal/painful character, and cervico-laryngeal discomfort. The latter ranges from slight dyspnea to a strong sensation of strangulation (laryngeal constriction). Other symptoms include epigastric discomfort/nausea, hypersalivation, auditory, vestibular, gustatory, and aphasic symptoms. However, most of these insulo-opercular symptoms can easily be masked by those of extra-insular seizure propagation. Indeed, sleep-related hyperkinetic (hypermotor) epilepsy (SHE) is a common clinical presentation of ILE, which shows predominant hyperkinetic and/or tonic-dystonic features that are often indistinguishable from those of fronto-mesial seizures. Subtle objective signs, such as constrictive throat noise (i.e., laryngeal constriction) or aversive behavior (e.g., facial grimacing suggesting pain), are often the sole clue in diagnosing insular SHE. Insular-origin seizures should also be considered in temporal-like seizures without frank anatomo-electro-clinical correlations. All in all, ILE is not the epilepsy of an isolated island but rather of a crucial hub involved in the multifaceted roles of the brain.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"527-539"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789358","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}

{"title":"[A case of a young woman with bilateral medial medullary infarcts caused by varicella-zoster virus vasculopathy without skin rash].","authors":"Daisuke Kuzume, Shou Ohturu, Takeshi Yosida, Yuko Morimoto, Masahiro Yamasaki, Naohisa Hosomi","doi":"10.5692/clinicalneurol.cn-001973","DOIUrl":"10.5692/clinicalneurol.cn-001973","url":null,"abstract":"<p><p>The patient, a 36-year-old female, had no previous history of shingles. She was admitted to the hospital due to nausea and lightheadedness. Upon admission, she was diagnosed with bilateral medial medullary infarcts. She received treatment with intravenous edaravone and argatroban, as well as antiplatelet therapy with aspirin and clopidogrel. However, her dysphagia, dysarthria, and paraplegia worsened. Due to changes in the lesion of the basilar artery on brain ‍MRA, we suspected the possibility of basilar artery dissection, and discontinued antiplatelet therapy. Subsequent imaging studies suggested vasculitis. After examining the cerebrospinal fluid, we diagnosed varicella-zoster virus (VZV) vasculopathy. Based on this diagnosis, we administered steroid pulse therapy for three days, started intravenous acyclovir, and resumed antithrombotic therapy with clopidogrel. Prednisone was administered for five days. Biochemical tests revealed an elevated D-dimer level. Due to the presence of lower extremity venous thrombus, clopidogrel was replaced with apixaban. The acyclovir infusion was discontinued due to observed acyclovir-induced neutropenia. These treatments improved neurological symptoms, circumflex thickening of the basilar artery, and contrast effects in the same area. On the 70th day, the patient was transferred to the hospital for rehabilitation. It is important to consider VZV angiopathy as a potential cause of juvenile cerebral infarction accompanying progressive basilar artery stenosis, regardless of the presence or absence of a skin rash.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"583-588"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141761543","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}

{"title":"[Multiple dural arteriovenous fistulas showing isolated subcortical white matter T₂ hyperintensity with gadolinium enhancement].","authors":"Hitoshi Hayashida, Katsuhisa Masaki, Hidenori Ogata, Ayumi Harada, Koichi Arimura, Ryo Yamasaki, Noriko Isobe","doi":"10.5692/clinicalneurol.cn-001947","DOIUrl":"10.5692/clinicalneurol.cn-001947","url":null,"abstract":"<p><p>We describe a 44-year-old man with a complaint of atonic seizures of the left upper limb, followed by generalized seizures. Brain MRI showed isolated juxtacortical white matter T₂ hyperintensity with gadolinium (Gd) enhancement of the adjacent cortical gray matter and subcortical white matter in the right frontal convexity. Treatment with levetiracetam was effective for seizure suppression, and he had no other neurological abnormalities. Human leukocyte antigen typing revealed B54 and Cw1, which indicated the possibility of neuro-Sweet disease. However, a general examination, which included vital signs and eye and skin findings, was normal. A cerebrospinal fluid test showed a mild elevation in protein levels without pleocytosis and a normal range of interleukin-6. Electroencephalography showed intermittent slow waves without epileptic discharge in the bilateral temporal lobes. We detected subtle flow voids in the pia mater of the left frontal lobe, which suggested cerebrovascular disease, and specifically, the possibility of dural arteriovenous fistulas. Computed tomography angiography showed abnormally dilated perimedullary veins in the left frontal lobe. Cerebral angiography confirmed the existence of four dural arteriovenous fistulas, which included two retrograde leptomeningeal venous drainages in the right frontal cortical veins supplied by the anterior branch of the right middle meningeal artery. The other dural arteriovenous fistulas were retrograde leptomeningeal venous drainages in the left frontal cortical veins supplied by the anterior and posterior convexity branches of the left middle meningeal artery. The patient underwent successful endovascular embolization of all dural arteriovenous fistulas with Onyx injection. A follow-up MRI showed gradual improvement of the T₂ hyperintensity and Gd enhancement. He remained seizure-free for 2 years following endovascular embolization.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"572-578"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141761544","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}

{"title":"[A case of left posterior cortical atrophy presenting with kana-predominant reading impairment].","authors":"Natsuki Nakatani, Daisuke Ono, Kosei Hirata, Kotaro Yoshioka, Hironobu Endo, Kenjiro Ono, Makoto Higuchi, Takanori Yokota","doi":"10.5692/clinicalneurol.cn-001907","DOIUrl":"10.5692/clinicalneurol.cn-001907","url":null,"abstract":"<p><p>The patient was an 85-year-old man with a one-year history of difficulty reading kana. Neuropsychological evaluation revealed kana (phonogram)-selective reading impairment and kanji (ideogram)-dominant writing impairment. MRI revealed significant cerebral atrophy in the left occipital cortex, leading to the clinical diagnosis of posterior cortical atrophy (PCA). Cerebrospinal fluid amyloid β_1-42 levels were reduced, and amyloid PET showed accumulation in the posterior cingulate cortex, precuneus, and frontal lobe. In contrast, tau PET showed no accumulation in the atrophied brain areas. Episodes of REM sleep behavior disorder and decreased uptake on meta-iodobenzylguanidine (MIBG) myocardial scintigraphy suggested the involvement of Lewy body pathology. PCA with distinct laterality has been rarely reported, and ‍this is the first case to present Kana-selective reading impairment and Kanji-dominant writing impairment with neurodegenerative background.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"557-563"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789356","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}