{"title":"[A case of primary central nervous system post-transplant lymphoproliferative disease 14 years after living donor liver transplantation].","authors":"Hiroaki Otsuka, Tomoaki Shima, Koichi Yoshida, Hirokazu Kurohama, Akira Tsujino","doi":"10.5692/clinicalneurol.cn-001991","DOIUrl":"10.5692/clinicalneurol.cn-001991","url":null,"abstract":"<p><p>The patient was a 51-year-old man who had undergone living donor liver transplantation for type B cirrhosis at the age of 37 years, and had a history of immunosuppressive drug use. He had developed focal seizures starting from his right upper limb, and MRI showed a lesion in the subcortical white matter of his left parietal lobe. Sensory disturbance and paralysis progressed in his right upper and lower limbs, and his brain lesion rapidly enlarged. A brain biopsy revealed diffuse large B-cell lymphoma, and Epstein-Barr virus-encoded small RNA in situ hybridization was positive. The patient was diagnosed with primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD) with no lesions in other organs. There are few reports of PCNS-PTLD cases after living donor liver transplantation in Japan. Although rare, it is nevertheless important to consider this disease in patients receiving immunosuppressive drugs after organ transplantation who develop brain lesions, regardless of which organ was transplanted.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"794-801"},"PeriodicalIF":0.0,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142476976","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}
Clinical NeurologyPub Date : 2024-11-22Epub Date: 2024-10-12DOI: 10.5692/clinicalneurol.cn-002020
Yasutake Tada, Hiroyasu Kaya, Keisuke Shima
{"title":"[An adult case of adenovirus type 3 infection presenting as clinically mild encephalitis/encephalopathy with a reversible splenial lesion and increased IL-6 levels in the cerebrospinal fluid].","authors":"Yasutake Tada, Hiroyasu Kaya, Keisuke Shima","doi":"10.5692/clinicalneurol.cn-002020","DOIUrl":"10.5692/clinicalneurol.cn-002020","url":null,"abstract":"<p><p>A 42-year-old Japanese man with a history hepatitis C who had undergone bone marrow transplantation for Burkitt lymphoma. He visited our hospital after developing a fever and sore throat. A computed tomography scan of the chest revealed pneumonia, and the patient was admitted to our hospital. After admission, he experienced a transient alteration of consciousness. Increased IL-6 levels in the cerebrospinal fluid and brain magnetic resonance imaging revealed clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). He received steroid pulse therapy and was discharged on the 14th hospital day. A neutralization test of paired serum revealed more than 4-fold increase in the adenovirus type 3 antibody titer, and a diagnosis of adenovirus-induced pneumonia was made. MERS was suspected to be involved in the pathology of encephalitis or encephalopathy following the adenovirus type 3 infection.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"807-812"},"PeriodicalIF":0.0,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142476978","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}
Clinical NeurologyPub Date : 2024-11-22Epub Date: 2024-10-12DOI: 10.5692/clinicalneurol.cn-001959
Haruna Akanuma, Suguru Kadowaki
{"title":"Meningeal carcinomatosis causing paroxysmal and reversible right midbrain symptoms: a case report.","authors":"Haruna Akanuma, Suguru Kadowaki","doi":"10.5692/clinicalneurol.cn-001959","DOIUrl":"10.5692/clinicalneurol.cn-001959","url":null,"abstract":"<p><p>Meningeal carcinomatosis is known to cause a variety of symptoms. Here, we report a case of meningeal carcinomatosis due to lung cancer in which the patient developed short, frequently recurrent localized symptoms originating from the right midbrain. We considered a diagnosis of meningeal carcinomatosis based on a similar reported case. The underlying mechanisms of the symptoms are unknown, but we suspect that epileptic seizures of brainstem origin or hemiplegic migraine-like symptoms with brainstem symptoms are possible causes. While meningeal carcinomatosis can be challenging to diagnose, the characteristic symptoms in the present case may aid in its diagnosis in future.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"818-821"},"PeriodicalIF":0.0,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142476983","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}
Clinical NeurologyPub Date : 2024-11-22Epub Date: 2024-10-12DOI: 10.5692/clinicalneurol.cn-001988
Yoshinari Nagakane, Eijirou Tanaka, Takehiro Yamada, Masashi Hamanaka, Jun Fujinami, Shinji Ashida, Yuta Kojima, Keiko Maezono-Kandori, Shiori Ogura, Yasumasa Yamamoto
{"title":"[Five-year outcomes in patients with ischemic stroke or transient ischemic attack after widespread use of direct oral anticoagulants].","authors":"Yoshinari Nagakane, Eijirou Tanaka, Takehiro Yamada, Masashi Hamanaka, Jun Fujinami, Shinji Ashida, Yuta Kojima, Keiko Maezono-Kandori, Shiori Ogura, Yasumasa Yamamoto","doi":"10.5692/clinicalneurol.cn-001988","DOIUrl":"10.5692/clinicalneurol.cn-001988","url":null,"abstract":"<p><p>The long-term outcomes of patients with stroke or transient ischemic attack (TIA) after widespread use of direct oral anticoagulants (DOACs) were investigated. Patients with ischemic stroke or TIA admitted between April 2014 and September 2015 were prospectively enrolled and followed for up to 5 years after the index stroke or TIA. Primary outcome measures were any cause of death and stroke recurrence. A total of 555 consecutive patients (323 men; mean age, 75 years; ischemic stroke, n = 520; TIA, n = 35) were analyzed. The follow-up rate was 93%, and the mean follow-up period was 48 ± 20 months. DOACs accounted for 52% of anticoagulants at discharge. During follow-up, 162 patients died, for cumulative mortality rates of 30% (particularly, 53% in cardioembolism) at 5 years. Recurrent stroke occurred in 90 patients, with cumulative risks of stroke recurrence of 19% at 5 years. The 5-year mortality rate remain even after widespread use of DOACs, and further treatment approaches are warranted.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"781-788"},"PeriodicalIF":0.0,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142476979","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}
Clinical NeurologyPub Date : 2024-11-22Epub Date: 2024-10-19DOI: 10.5692/clinicalneurol.cn-001951
Yoko Fujii, Mutsumi Okura, Yaeko Kashiwai, Mitsutaka Taniguchi, Motoharu Ohi
{"title":"[A case of propriospinal myoclonus at sleep onset in which video-polysomnography with additional surface electromyogram was useful for diagnosis].","authors":"Yoko Fujii, Mutsumi Okura, Yaeko Kashiwai, Mitsutaka Taniguchi, Motoharu Ohi","doi":"10.5692/clinicalneurol.cn-001951","DOIUrl":"10.5692/clinicalneurol.cn-001951","url":null,"abstract":"<p><p>Propriospinal myoclonus at sleep onset (PSM-S) is a sudden myoclonic jerk that occurs during the transition from wakefulness to sleep. It is a sleep-related movement disorder that causes difficulty falling asleep due to involuntary movements that spread caudally and rostrally through the propriospinal tract. Diagnosis requires observation of movements and polysomnography (PSG), and there are few reports. An 80-year-old man was referred to our center for insomnia due to abdominal movements at sleep onset. During the EEG test, we observed the caudal and rostral propagation of movements emanating from the abdomen. Attended video-PSG with additional surface electromyography revealed that myoclonic jerks occurred during the transition from wake to stage N1 and disappeared during sleep stage N2. EMG activity originated from the rectus abdominis muscle, followed by rostral and caudal propagation. Here, we report a case demonstrating that PSG with additional surface electromyography is important and useful for the diagnosis of PMS at sleep onset.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"813-817"},"PeriodicalIF":0.0,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142476977","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 T1 radiculopathy caused by intervertebral disc herniation with cervical angina].","authors":"Kazuki Sogawa, Tetsuo Ando, Yasuo Kanamori, Syunsuke Koga, Masahiro Sonoo, Toshio Fukutake","doi":"10.5692/clinicalneurol.cn-001989","DOIUrl":"10.5692/clinicalneurol.cn-001989","url":null,"abstract":"<p><p>A 65-year-old man presented to the emergency department with the complaints of left anterior chest and back pain, numbness in the left medial arm, and weakness in the left hand grip. Myocardial infarction was suspected, but later ruled out, and the patient was subsequently referred to our department. Among the intrinsic muscles, the left abductor pollicis brevis was the most severely weakened, and there was a sensory disturbance in the left T1 region and left Horner's sign. An MRI T<sub>2</sub>-weighted image of the cervical spine showed a herniated disc on the left lateral side at the T1/2 level, suggesting compression of the T1 nerve root. Cervical angina is a rare, angina-like anterior chest pain due to cervical spine diseases. It is often reported in patients with C6 and C7 radiculopathy. The most severe weakness in the abductor pollicis brevis muscle in T1 radiculopathy is important to distinguish it from C8 radiculopathy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"725-729"},"PeriodicalIF":0.0,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142355938","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":"[Proposals for the early diagnosis of late-onset hereditary ATTR amyloidosis in nonendemic areas in Japan].","authors":"Keiko Maruyama Saladini, Haruki Koike, Mitsuharu Ueda, Yoshiki Sekijima, Yukio Ando","doi":"10.5692/clinicalneurol.cn-002002","DOIUrl":"10.5692/clinicalneurol.cn-002002","url":null,"abstract":"<p><p>Late-onset hereditary ATTR (ATTRv) amyloidosis in nonendemic areas takes long periods of time to diagnose in many cases because the clinical symptoms are varied and nonspecific with the family history often unidentifiable. In recent years, disease-modifying therapies have been available for ATTRv amyloidosis, and early diagnosis is increasingly needed. The diagnosis of ATTRv amyloidosis usually requires histological confirmation of the amyloid deposition, although the amyloid detection rate largely depends on the experience, knowledge, and skill of the physician who performs the biopsy. It is important to consider ATTRv amyloidosis as a differential disease in idiopathic polyneuropathy. If ATTRv amyloidosis is strongly suspected, it is acceptable to perform TTR genetic testing prior to histological examination after a thorough differential diagnosis has been made.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"708-713"},"PeriodicalIF":0.0,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142355940","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 hypertrophic pachymeningitis as a systemic autoimmune/inflammatory disorder (SAID) associated with chronic myelomonocytic leukemia].","authors":"Tatsuya Sato, Tomoyuki Inoue, Satoshi Kubo, Kota Sato, Takahiro Himeno, Yuka Terasawa","doi":"10.5692/clinicalneurol.cn-002007","DOIUrl":"10.5692/clinicalneurol.cn-002007","url":null,"abstract":"<p><p>A 67-year-old woman with past medical history of chronic myelomonocytic leukemia (CMML) presented with a chief complaint of headache, diplopia, and hearing impairment in the right ear. Examination revealed impaired ocular movement in the left eye and sensorineural hearing loss in the right ear. Cerebrospinal fluid analysis showed increased cell count and protein, and MRI showed contrast enhancement of hypertrophic dura mater. Since there were no other abnormalities which would have been a cause of hypertrophic pachymeningitis, it was considered as systemic autoimmune/inflammatory disorder (SAID) associated with CMML. Treatment with steroid, cyclophosphamide, and methotrexate led to improvement of the symptoms. SAIDs develop in up to 25% of patients with myelodysplastic syndromes (MDS) or CMML, which may be the only symptoms of MDS/CMML. As a phenotype of SAIDs, systemic vasculitis, connective tissue diseases, and neutrophilic diseases are frequently reported; however, isolated involvement of central nerve system is rarely reported. To our knowledge, this is the first report of hypertrophic pachymeningitis as SAID associated with CMML. To clarify the pathogenesis of neurologic involvement of SAIDs, accumulation of cases is necessary.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"742-745"},"PeriodicalIF":0.0,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308725","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":"[Amaurosis fugax in Marfan syndrome with myxomatous degeneration].","authors":"Takeo Sato, Mari Satake, Hiroki Iitsuka, Masahiro Mimori, Tadashi Umehara, Chisen Takeuchi, Yasuyuki Iguchi","doi":"10.5692/clinicalneurol.cn-001987","DOIUrl":"10.5692/clinicalneurol.cn-001987","url":null,"abstract":"<p><p>A 28-year-old male presented with a sudden, transient visual impairment in the right eye. Brain MRI revealed no obvious ischemic lesions, and he was diagnosed with amaurosis fugax. Physical examination revealed characteristic features of Marfan syndrome, along with a family history of tall stature and valvular heart disease. Further investigation revealed: 1) an ascending aortic aneurysm, 2) a bicuspid aortic valve with fenestration and regurgitation, and 3) a mitral valve prolapse with regurgitation. Genetic testing identified a heterozygous variant c.6905G>A of FBN1, confirming the diagnosis of Marfan syndrome. Bentall operation and mitral valve replacement were performed, resulting in a favorable outcome. Pathological examination showed extensive myxomatous degeneration with thickening of the aortic and mitral valves, suggesting a potential source of embolism.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"719-724"},"PeriodicalIF":0.0,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308728","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":"[Acute internal carotid artery occlusion following administration of Andexanet alfa for the reversal of direct factor Xa inhibitors in patients with cerebral hemorrhage].","authors":"Sota Uemura, Junji Takasugi, Nobuyuki Ohara, Masamori Koyanagi, Tsuyoshi Ohta, Michi Kawamoto","doi":"10.5692/clinicalneurol.cn-001997","DOIUrl":"10.5692/clinicalneurol.cn-001997","url":null,"abstract":"<p><p>An 87-year-old woman receiving aspirin and apixaban with a history of large artery atherosclerotic stroke, and pulmonary embolism presented to the hospital for aphasia and right hemiplegia. A head CT scan showed 18-ml hematoma in the left thalamus. Low-dose Andexanet alfa was administered 84 minutes after the onset of stroke, and 10 hours and 24 minutes after the last dose of apixaban. Three hours later after admission, she had flaccid hemiplegia and became comatose. CT and CT angiography revealed occlusion of left internal carotid artery (ICA) and no evidence of hematoma expansion. Although repetitive mechanical thrombectomy resulted in recanalization (modified TICI 2b), carotid ultrasound revealed the occlusion of left ICA on next day. On day 7, she died of brain herniation following extensive cerebral infarction. It has been reported that some patients did experience thrombotic events after administration of Andexanet alfa. Our case illustrates that even large vessel occlusion might occur after intravenous injection of Andexanet alfa. Thus, careful follow-up, including cerebrovascular imaging, is required immediately after administration of Andexanet alfa.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"730-735"},"PeriodicalIF":0.0,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308727","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}