Publications by authors named "Harushi Mori"

125 Publications

Cerebrovascular diseases in two patients with entire NSD1 deletion.

Hum Genome Var 2021 May 24;8(1):20. Epub 2021 May 24.

Department of human genetics, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan.

We describe two patients with NSD1 deletion, who presented with early-onset, or recurrent cerebrovascular diseases (CVDs). A 39-year-old female showed developmental delay and abnormal gait in infancy, and developed slowly-progressive intellectual disability and movement disorders. Brain imaging suggested recurrent parenchymal hemorrhages. A 6-year-old male had tremor as a neonate and brain imaging revealed subdural hematoma and brain contusion. This report suggests possible involvement of CVDs associated with NSD1 deletion.
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http://dx.doi.org/10.1038/s41439-021-00151-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8144564PMC
May 2021

Successful control of portal hypertension-related complications after two embolization procedures for multiple and large spontaneous portosystemic shunts in a patient with liver cirrhosis.

Clin J Gastroenterol 2021 Aug 16;14(4):1227-1232. Epub 2021 May 16.

Division of Gastroenterology, Department of Medicine, Jichi Medical University, Shimotsuke, Tochigi, 329-0498, Japan.

Liver cirrhosis is frequently complicated by spontaneous portosystemic shunt (SPSS) due to portal hypertension. Shunt embolization is considered when symptoms related to SPSSs are refractory to endoscopic and/or medical therapies. However, little information is available on the treatment of patients with multiple and large SPSS. We report a successfully managed case in which patient with such SPSS received two embolization procedures within 6 months. A 57-year-old man with alcoholic liver cirrhosis was transferred to our hospital due to a ruptured gastric varix. CT examination showed gastrorenal and splenorenal shunts of 8 mm and 11 mm in diameter, respectively. In addition, multiple hepatocellular carcinomas (HCCs) were noted. First, balloon-occluded retrograde transvenous obliteration (BRTO) was performed for the gastrorenal shunt, resulting in the disappearance of the varix, followed by transcatheter arterial chemoembolization (TACE) for HCCs. However, the hepatic encephalopathy worsened after the BRTO and TACE, and the splenorenal shunt enlarged to 18 mm in diameter. Although the shunt was tortuous and had another drainage vein, we completed the embolization for the shunt using metallic coils without any events. The patient's hepatic encephalopathy and hepatic function were ameliorated after embolization for the splenorenal shunt, and the patient was free from hepatic encephalopathy.
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http://dx.doi.org/10.1007/s12328-021-01441-3DOI Listing
August 2021

Localization of the central sulcus using the distinctive high signal intensity of the paracentral lobule on T1-weighted images.

Neuroradiology 2021 May 6. Epub 2021 May 6.

Department of Radiology, The University of Tokyo, 1-2-1, Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.

Purpose: The central sulcus is an important landmark in the brain. This study aimed to investigate the distinctive signal of the paracentral lobule (PL) on T1-weighted images (T1WIs; the white PL sign) and evaluate its usefulness as a new method of identifying the central sulcus.

Methods: T1WIs of the brain of 96 participants (age, 58.9 ± 17.9 years; range, 8-87 years) scanned at 3-T MR system were retrospectively reviewed. First, we qualitatively analyzed the signal of the cortex of the PL by comparing it with that of the ipsilateral superior frontal gyrus on a 4-point grading score. Second, we compared the cortical signal intensity and gray/white-matter contrast between the PL and superior frontal gyrus. Third, we evaluated the usefulness of the PL signal for identifying the central sulcus.

Results: The PL cortex was either mildly hyperintense (grade 2) or definitely hyperintense (grade 3) in comparison with that of superior frontal cortex in all participants. The signal intensity of the PL cortex was significantly higher than that of the superior frontal cortex (p < 0.001), whereas the gray/white-matter contrast of the PL was weaker than that of the superior frontal gyrus (p < 0.001). The central sulci were identified with 94.3% accuracy (181/192) using the new method.

Conclusion: The white PL sign may be helpful in identifying the central sulcus, and this approach can be recognized as a new method for identification of the central sulcus.
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http://dx.doi.org/10.1007/s00234-021-02729-4DOI Listing
May 2021

Paracentral homonymous hemianopic scotoma caused by anterior choroidal artery infarction.

QJM 2021 Feb 10. Epub 2021 Feb 10.

Department of Neurology, The University of Tokyo Hospital, Japan, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.

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http://dx.doi.org/10.1093/qjmed/hcab031DOI Listing
February 2021

Three-Year Longitudinal Motor Function and Disability Level of Acute Flaccid Myelitis.

Pediatr Neurol 2021 03 3;116:14-19. Epub 2020 Dec 3.

Infectious Disease Surveillance Center, National Institute of Infectious Diseases, Tokyo, Japan. Electronic address:

Background: We summarize the long-term motor outcome and disability level in a cluster of pediatric patients with acute flaccid myelitis (AFM) associated with the enterovirus D68 outbreak in 2015.

Methods: This is a nationwide follow-up questionnaire analysis study. Clinical data including the motor function (manual muscle strength test) and other neurological symptoms were collected at the acute (nadir), recovery (six months), and chronic (three years) stages. We use the Barthel index, which measures 10 variables describing activity of daily living and mobility to assess the disability level.

Results: Clinical data of 33 patients with AFM (13 females, 20 males; median age = 4.1 years) were available. Among patients with tetraplegia or triplegia, paraplegia, and monoplegia at the acute stage, two of seven, four of thirteen, and two of thirteen exhibited complete recovery without paralysis; of those five of seven, eight of thirteen, and two of thirteen showed improvement with lesser limb involvement at the chronic stage, respectively. Nine patients (27%) demonstrated improvement at the recovery-to-chronic period. All six patients with positive isolation of enterovirus D68 from biological samples at the acute stage showed persistent motor deficits. Other neurological findings had better prognosis than motor weakness. Better Barthel index score at the chronic stage was observed (P < 0.001; median difference [95% confidence interval], 53 [40 to 63]), implying an improved disability level even in patients with persistent motor deficits.

Conclusions: AFM has a high rate of persistent motor deficits showing one- to two-limb paralysis. Disability level of patients with AFM, however, generally improved at the three-year time point.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.11.019DOI Listing
March 2021

Recognizing Radiation-induced Changes in the Central Nervous System: Where to Look and What to Look For.

Radiographics 2021 Jan-Feb;41(1):224-248. Epub 2020 Nov 20.

From the Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo, Tokyo 113-8655, Japan (M.K., J.S., T.F., H.M., O.A.); and Department of Radiology, School of Medicine, International University of Health and Welfare, Chiba, Japan (M.A.).

Radiation therapy (RT) continues to play a central role as an effective therapeutic modality for a variety of tumors and vascular malformations in the central nervous system. Although the planning and delivery techniques of RT have evolved substantially during the past few decades, the structures surrounding the target lesion are inevitably exposed to radiation. A wide variety of radiation-induced changes may be observed at posttreatment imaging, which may be confusing when interpreting images. Histopathologically, radiation can have deleterious effects on the vascular endothelial cells as well as on neuroglial cells and their precursors. In addition, radiation induces oxidative stress and inflammation, leading to a cycle of further cellular toxic effects and tissue damage. On the basis of the time of expression, radiation-induced injury can be divided into three phases: acute, early delayed, and late delayed. Acute and early delayed injuries are usually transient and reversible, whereas late delayed injuries are generally irreversible. The authors provide a comprehensive review of the timeline and expected imaging appearances after RT, including the characteristic imaging features after RT with concomitant chemotherapy. Specific topics discussed are imaging features that help distinguish expected posttreatment changes from recurrent disease, followed by a discussion on the role of advanced imaging techniques. Knowledge of the RT plan, the amount of normal structures included, the location of the target lesion, and the amount of time elapsed since RT is highly important at follow-up imaging, and the reporting radiologist should be able to recognize the characteristic imaging features after RT and differentiate these findings from tumor recurrence. RSNA, 2020.
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http://dx.doi.org/10.1148/rg.2021200064DOI Listing
November 2020

Reply to: "Letter: Two cases of persistent falcine and occipital sinuses".

Brain Dev 2021 01 13;43(1):175. Epub 2020 Oct 13.

Department of Pediatric Neurology, Miyagi Children's Hospital, 4-3-17 Ochiai, Aoba-ku, Sendai-shi, Miyagi, 989-3126, Japan; Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.

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http://dx.doi.org/10.1016/j.braindev.2020.09.008DOI Listing
January 2021

Clinical efficacy of haematopoietic stem cell transplantation for adult adrenoleukodystrophy.

Brain Commun 2020 14;2(1):fcz048. Epub 2020 Jan 14.

Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo 113-8655, Japan.

Accumulated experience supports the efficacy of allogenic haematopoietic stem cell transplantation in arresting the progression of childhood-onset cerebral form of adrenoleukodystrophy in early stages. For adulthood-onset cerebral form of adrenoleukodystrophy, however, there have been only a few reports on haematopoietic stem cell transplantation and the clinical efficacy and safety of that for adulthood-onset cerebral form of adrenoleukodystrophy remain to be established. To evaluate the clinical efficacy and safety of haematopoietic stem cell transplantation, we conducted haematopoietic stem cell transplantation on 12 patients with adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy in a single-institution-based prospective study. Through careful prospective follow-up of 45 male adrenoleukodystrophy patients, we aimed to enrol patients with adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy at early stages. Indications for haematopoietic stem cell transplantation included cerebral form of adrenoleukodystrophy or cerebello-brainstem form of adrenoleukodystrophy with Loes scores up to 13, the presence of progressively enlarging white matter lesions and/or lesions with gadolinium enhancement on brain MRI. Clinical outcomes of haematopoietic stem cell transplantation were evaluated by the survival rate as well as by serial evaluation of clinical rating scale scores and neurological and MRI findings. Clinical courses of eight patients who did not undergo haematopoietic stem cell transplantation were also evaluated for comparison of the survival rate. All the patients who underwent haematopoietic stem cell transplantation survived to date with a median follow-up period of 28.6 months (4.2-125.3 months) without fatality. Neurological findings attributable to cerebral/cerebellar/brainstem lesions became stable or partially improved in all the patients. Gadolinium-enhanced brain lesions disappeared or became obscure within 3.5 months and the white matter lesions of MRI became stable or small. The median Loes scores before haematopoietic stem cell transplantation and at the last follow-up visit were 6.0 and 5.25, respectively. Of the eight patients who did not undergo haematopoietic stem cell transplantation, six patients died 69.1 months (median period; range 16.0-104.1 months) after the onset of the cerebral/cerebellar/brainstem lesions, confirming that the survival probability was significantly higher in patients with haematopoietic stem cell transplantation compared with that in patients without haematopoietic stem cell transplantation ( = 0.0089). The present study showed that haematopoietic stem cell transplantation was conducted safely and arrested the inflammatory demyelination in all the patients with adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy when haematopoietic stem cell transplantation was conducted in the early stages. Further studies are warranted to optimize the procedures of haematopoietic stem cell transplantation for adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy.
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http://dx.doi.org/10.1093/braincomms/fcz048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7425345PMC
January 2020

Evaluation of peripheral bronchiole visualization using model-based iterative reconstruction in quarter-detector computed tomography.

PLoS One 2020 18;15(9):e0239459. Epub 2020 Sep 18.

Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

This study aimed to evaluate the visualization of peripheral bronchioles in normal lungs via quarter-detector computed tomography (QDCT). Visualization of bronchioles within 10 mm from the pleura is considered a sign of bronchiectasis. However, it is not known peripheral bronchioles how close to the pleura in normal lungs can be tracked using QDCT. This study included 228 parts in 76 lungs from 38 consecutive patients who underwent QDCT. Reconstruction was performed with different thicknesses, increments, and matrix sizes: 0.5-mm thickness and increment with 512 and 1024 matrixes (Group5 and Group10, respectively) and 0.25-mm thickness and increment with 1024 matrix (Group10Thin). The distance between the most peripheral bronchiole visible and the pleura was determined in the three groups. The distance between the peripheral bronchial duct ends and the nearest pleural surface were significantly shorter in the order of Group10Thin, Group10, and Group5, and the mean distances from the pleura in Group10Thin and Group10 were shorter than 10 mm. These findings suggest the visualization of peripheral bronchioles in QDCT was better with a 1024 axial matrix than with a 512 matrix, and with a 0.25-mm slice thickness/increment than with a 0.5-mm slice thickness/increment. Our study also indicates bronchioles within 10 mm of the pleura do not necessarily indicate pathology.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0239459PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7500691PMC
November 2020

Two cases of persistent falcine and occipital sinuses.

Brain Dev 2021 Jan 3;43(1):170-173. Epub 2020 Aug 3.

Department of Pediatric Neurology, Miyagi Children's Hospital, 4-3-17 Ochiai, Aoba-ku, Sendai-shi, Miyagi 989-3126, Japan; Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.

Background: The coexistence of falcine and occipital sinuses is rare and its natural course has not been reported.

Case Reports: Two patients with persistent falcine and occipital sinuses are described. Both patients had straight sinuses. In one, both the transverse and sigmoid sinuses were hypoplastic and the patient had an acquired Chiari I malformation. The other patient had no other venous anomalies and had a normal posterior cranial fossa.

Conclusion: The coexistence of falcine and occipital sinuses can lead to an acquired Chiari I malformation. These cases suggest the importance of checking other venous and brain anomalies in this situation.
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http://dx.doi.org/10.1016/j.braindev.2020.07.011DOI Listing
January 2021

IVIG in childhood primary angiitis of the central nervous system: A case report.

Brain Dev 2020 Oct 1;42(9):675-679. Epub 2020 Jul 1.

Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, Japan.

Aggressive immunosuppressive therapies have been proposed to treat primary angiitis of the central nervous system (PACNS). Here, we report the first successfully stabilized case of childhood, small-vessel PACNS with intravenous immunoglobulin (IVIG) therapy. A 12-year-old boy was admitted to our hospital complaining of recurrent headaches and upper-left homonymous quadrantanopia, since the age of 11 years. Brain computed tomography scans revealed fine calcification in the right temporal and occipital lobes. Brain magnetic resonance imaging scans revealed white matter lesions, with gadolinium enhancement, which waxed, waned, and migrated for 1 year, without immunomodulatory therapies. A cerebrospinal fluid study showed pleocytosis (12 cells per µl). No clinical or serological findings suggested systemic inflammation or vasculitis. Brain angiography was unremarkable. Brain biopsy revealed thickened and hyalinized small vessels, with intramural infiltration of inflammatory cells, which confirmed the diagnosis of small-vessel PACNS. Because the patient developed surgical site infection following biopsy, the administration of monthly IVIG (2 g/kg) was prescribed, instead of immunosuppressive agents. After IVIG therapy, the patient remained stable, except for a single episode of mild radiological exacerbation at 16 months, which occurred when the IVIG interval was expanded. Oral prednisone was added and gradually tapered. At 50 months, his intellectual abilities and motor functions were normal, although he showed residual upper-left homonymous quadrantanopia and post-exercise headache. A temporary headache, associated with the immunoglobulin infusion, was resolved by slowing the infusion rate. PACNS should be treated aggressively to improve prognosis. However, when immunosuppressants are contraindicated, IVIG may be an alternative therapeutic option.
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http://dx.doi.org/10.1016/j.braindev.2020.06.007DOI Listing
October 2020

Altered Structural Brain Networks Related to Adrenergic/Muscarinic Receptor Autoantibodies in Chronic Fatigue Syndrome.

J Neuroimaging 2020 11 1;30(6):822-827. Epub 2020 Jul 1.

Department of Radiology, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.

Background And Purpose: Recent studies suggest that the autoantibodies against adrenergic/muscarinic receptors might be one of the causes and potential markers of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). The purpose of this study was to investigate the structural network changes related to autoantibody titers against adrenergic/muscarinic receptors in ME/CFS by performing a single-subject gray matter similarity-based structural network analysis.

Methods: We prospectively examined 89 consecutive right-handed ME/CFS patients who underwent both brain MRI including 3D T1-wighted images and a blood analysis of autoantibodies titers against β1 adrenergic receptor (β1 AdR-Ab), β2 AdR-Ab, M3 acetylcholine receptor (M3 AchR-Ab), and M4 AchR-Ab. Single-subject gray matter similarity-based structural networks were extracted from segmented gray matter images for each patient. We calculated local network properties (betweenness centrality, clustering coefficient, and characteristic path length) and global network properties (normalized path length λ, normalized clustering coefficient γ, and small-world network value δ). We investigated the correlations between the autoantibody titers and regional gray matter/white matter volumes, the local network properties, and the global network properties.

Results: Betweenness centrality showed a significant positive correlation with β1-AdR-Ab in the right dorsolateral prefrontal cortex. The characteristic path length showed a significant negative correlation with β2-AdR-Ab in the right precentral gyrus. There were no significant correlations between the antibody titers and the regional gray matter/white matter volumes, and the global network properties.

Conclusions: Our findings suggest that β1 AdR-Ab and β2 AdR-Ab are potential markers of ME/CFS.
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http://dx.doi.org/10.1111/jon.12751DOI Listing
November 2020

Resection and Reconstruction of Giant Abdominoscrotal Arteriovenous Malformation.

Plast Reconstr Surg Glob Open 2020 Mar 27;8(3):e2725. Epub 2020 Mar 27.

Department of Radiology, University of Tokyo, Tokyo, Japan.

Genital arteriovenous malformations are rare and present unique surgical challenges in preserving urogenital function, abdominal wall integrity, and lower limb perfusion. A 32-year-old man with a giant abdominoscrotal arteriovenous malformation presented with recurrent heavy bleeding. Due to the high risk of rebleeding and fatal hemorrhage, surgery with curative intent was proposed and the patient was counseled on the risks of ischemia to the lower limb, testes, and penis. Preoperative embolization of the feeding vessels was performed. Three days later, surgical excision of the mass with the affected scrotum, left rectus muscle, sheath, and overlying abdominal skin followed. The testes were dissected from the malformation and preserved along with the right internal pudendal artery. The left thigh skin was advanced to the scrotal remnants and a neoscrotum created. The resulting large abdominal wall defect was reconstructed in layers with a pedicled anterolateral thigh flap, including innervated vastus lateralis muscle, to prevent herniation. Recovery was uneventful, and a 4-year follow-up revealed no significant clinical or radiological recurrence with recovery of flap sensation, retained erectile function, and no herniation. We report this case due to rarity of giant abdominoscrotal arteriovenous malformations and present preoperative embolization, surgical resection, and functional anterolateral thigh flap reconstruction as a valuable treatment option of this life-threatening illness.
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http://dx.doi.org/10.1097/GOX.0000000000002725DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253270PMC
March 2020

Computed tomography findings of early-stage TAFRO syndrome and associated adrenal abnormalities.

Eur Radiol 2020 Oct 21;30(10):5588-5598. Epub 2020 May 21.

Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.

Objectives: To compare CT findings of early (within 3 weeks post-onset)- and later (within 1 month before or after diagnostic criteria were satisfied, and later than 3 weeks post-onset) stage thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome.

Methods: Between 2014 and 2019, 13 patients with TAFRO syndrome (8 men and 5 women; mean age, 54.9 years) from nine hospitals were enrolled. The number of the following CT findings (CT factors) was recorded: the presence of anasarca, organomegaly, adrenal ischaemia, anterior mediastinal lesion, bony lesion, and lymphadenopathy. Records of adrenal disorders (adrenomegaly, ischaemia, and haemorrhage) throughout the disease course were also collected. Differences in CT factors at each stage were statistically compared between remission and deceased groups.

Results: Para-aortic oedema and mild lymphadenopathy were observed in all patients, whereas pleural effusion, ascites, and subcutaneous oedema were found in 5/13, 7/13, and 7/13 cases, respectively, at the early stage. CT factors at the early stage were significantly higher in the deceased than in the remission group (mean, 11 vs 6.5; p = 0.04), while they were nonsignificant at the later stage. Adrenal disorders were present in 7/13 cases throughout the course including 6 of adrenomegaly and 4 of ischaemia at the early stage.

Conclusions: Para-aortic oedema and mild lymphadenopathy are most common at the early stage. Anasarca, organomegaly, lymphadenopathy, and adrenal disorders on early-stage CT are useful for unfavourable prognosis prediction. Moreover, adrenal disorders are frequent even at the early stage and are useful for early diagnosis of TAFRO syndrome.

Key Points: • CT findings facilitate early diagnosis and prognosis prediction in TAFRO syndrome. • Adrenal disorders are frequently observed in TAFRO syndrome. • Adrenal disorders are useful for differential diagnosis of TAFRO syndrome.
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http://dx.doi.org/10.1007/s00330-020-06919-1DOI Listing
October 2020

Accelerated acquisition of carotid MR angiography using 3D gradient-echo imaging with two-point Dixon.

Neuroradiology 2020 Oct 18;62(10):1345-1349. Epub 2020 May 18.

Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.

This pilot study tests the feasibility of rapid carotid MR angiography using the liver acquisition with volume acceleration-flex technique (LAVA MRA). Seven healthy volunteers and 21 consecutive patients suspected of carotid stenosis underwent LAVA and conventional time-of-flight (cTOF) MRAs. Artery-to-fat and artery-to-muscle signal intensity ratios were manually measured. LAVA MRA exhibited a significantly larger artery-to-fat signal intensity ratio compared with cTOF MRA in all slices (P < 0.001) and exhibited a larger (P < 0.001) or equivalent (P = 1.0) artery-to-muscle signal intensity ratio in the extracranial carotid arteries. The image quality of the cervical carotid bifurcation and the signal change on each MRA were visually assessed and compared among the MRAs. There was no significant difference between the two MRAs in visual assessment. LAVA MRA can provide visualization similar to cTOF MRA in the evaluation of the cervical carotid bifurcation while reducing scan time by one-fifth.
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http://dx.doi.org/10.1007/s00234-020-02452-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7479001PMC
October 2020

Acute Flaccid Myelitis With Neuroradiological Finding of Brachial Plexus Swelling.

Pediatr Neurol 2020 08 13;109:85-88. Epub 2020 Apr 13.

Department of Pediatric Neurology, Fukuoka Children's Hospital, Fukuoka, Japan.

Background: Acute flaccid myelitis is a recently defined clinically distinct syndrome of polio-like acute flaccid paralysis. Acute flaccid myelitis cases show characteristic neuroradiological features of longitudinal spinal cord lesions with predominant gray matter involvement. Current evidence suggests injury to the anterior horn neurons as the underlying mechanism.

Methods: We describe three patients with acute flaccid myelitis who developed flaccid upper limb weakness with diminished deep tendon reflexes after prodromal fever. Spinal magnetic resonance imaging (MRI) (axial and sagittal T1- and T2-weighted sequences) and brachial plexus MRI (coronal short tau inversion recovery sequence) at the acute stage were performed.

Results: Spinal MRI showed extensive longitudinal lesion in the spinal cord with predominant gray matter involvement. We were able to demonstrate concurrent swelling and hyperintensity in the brachial plexus in all the three patients at the acute stage.

Conclusion: The coexisting signal intensities suggest an extension of acute flaccid myelitis pathology to the brachial plexus, highlighting the possible peripheral nerve involvement in acute flaccid myelitis.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.04.004DOI Listing
August 2020

Differentiation between ovarian metastasis from colorectal carcinoma and primary ovarian carcinoma: Evaluation of tumour markers and "mille-feuille sign" on computed tomography/magnetic resonance imaging.

Eur J Radiol 2020 Mar 9;124:108823. Epub 2020 Jan 9.

Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan. Electronic address:

Purpose: The purpose of this retrospective study was to evaluate the usefulness of serum tumour markers and morphological characteristics in CT/MRI to differentiate between ovarian metastases from colorectal carcinomas (OMCRC) and primary ovarian carcinomas (POC).

Method: Preoperative radiological images of 41 OMCRCs from 27 patients (mean age ± SD: 52.2 ± 10.7 years) and 46 POCs from 36 patients (52.1 ± 12.7 years) were included. Three blinded gynecological radiologists classified tumour morphology into 'mille-feuille sign', 'solid and cystic', 'multicystic without nodules', and 'multicystic with nodules' groups and analysed using Fisher's exact test. Serum carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), and carbohydrate antigen 19-9 levels were compared by Wilcoxon rank-sum test.

Results: 'Mille-feuille sign' indicated OMCRC (OMCRC: 8/41, POC: 1/46, specificity = 0.98, p = 0.011) and had excellent interobserver agreement (Fleiss's kappa value = 0.96). 'Solid and cystic' indicated POC (18/41 vs 41/45, p < 0.001) and 'multicystic without nodules' indicated OMCRC (8/41 vs 2/46, p = 0.041). There was no significant difference in 'multicystic with nodules'. CA125 levels were higher in POCs (292.5 U/mL vs. 41.0 U/mL, p = 0.003). CEA levels were higher in OMCRCs (24.5 ng/mL vs 2 ng/mL, p < 0.001). CEA (< 6.3 ng/mL) AND (CA125 (≥87.0 U/mL) OR 'solid and cystic') indicated POC with high accuracy (3/41 vs 44/46, accuracy = 0.94, p < 0.001).

Conclusions: Our new method with morphological classification and tumour markers were useful for differentiating the two tumours. In particular, the 'mille-feuille sign' frequently indicated OMCRC with high specificity and excellent interobserver agreement.
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http://dx.doi.org/10.1016/j.ejrad.2020.108823DOI Listing
March 2020

Multinodular and vacuolating neuronal tumor (MVNT): A presumably incidental and asymptomatic case in an intractable epilepsy patient.

Clin Neurophysiol Pract 2019 16;4:164-167. Epub 2019 Jul 16.

Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.

Introduction: Multinodular and vacuolating neuronal tumor (MVNT) had been initially described as an epilepsy-related brain tumor, but recent studies demonstrated it could be found incidentally in non-epilepsy patients.

Case Report: A 33-year-old woman with intractable post-encephalitis epilepsy presented a cluster of multinodular T2 hyperintensity in the left temporal lobe, which was very similar to the characteristics of MVNT. Long-term video electroencephalogram demonstrated that the habitual seizures were originated from bilateral temporal area and the interictal epileptic discharges were seen multifocally, although the lesions with MVNT appearance were localized in the left temporal lobe. It was presumed that the epilepsy in this patient was due to encephalitis in the past, and the link between the lesions and the epilepsy in this patient seemed weak.

Conclusion: Although MVNT had been considered as an epilepsy-related brain tumor, we suggest it is not necessarily preferable to perform surgical resection of MVNT even on patients with epilepsy, unless epileptic foci are highly related to MVNT.
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http://dx.doi.org/10.1016/j.cnp.2019.05.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6921157PMC
July 2019

Association of volume of self-directed versus assigned interpretive work with diagnostic performance of radiologists: an observational study.

BMJ Open 2019 12 17;9(12):e033390. Epub 2019 Dec 17.

Radiology, The University of Tokyo, Tokyo, Japan.

Objectives: To understand the sources of variability in diagnostic performance among experienced radiologists.

Design: All prostate MRI examinations performed between 2016 and 2018 were retrospectively reviewed.

Setting: University hospital in Japan.

Participants: Data derived from 334 pathology-proven cases (male, mean age: 70 years; range: 35-90 years) that were interpreted by 10 experienced radiologists were subjected to the analysis.

Primary And Secondary Outcome Measures: Diagnostic performance measures of the radiologists were compared with candidate factors, including interpretive volume of prostate MRIs, volume of self-directed and assigned total annual interpretive work, and years of experience. The potential influence of fatigue was also evaluated by examining the effect of the report's issue time.

Results: There were 186 prostate cancer cases. Performance was based on accuracy, sensitivity and specificity (86%, 85% and 84%, respectively). While performance was not correlated with the volume of prostate MRIs, per se (ρ=-0.15, p=0.69; ρ=-0.01, p=0.99; ρ=-0.33, p=0.36) or the total MRIs assigned for each radiologist (p>0.6) or years of experience (p>0.4), all measures were strongly correlated with voluntary work represented by the interpretive volume of abdominal CTs (r=0.79, p<0.01; r=0.80, p<0.01; r=0.64, p=0.048). The performance did not differ based on the issue time of the report (morning, afternoon and evening) (χ(2)=3.65, p=0.16).

Conclusions: Greater autonomy, represented as enhanced self-directed interpretive work, was most significantly correlated with the performance of prostate MRI interpretation. The lack of a correlation between the performance and assigned volume confirms the complexity of human learning. Together, these findings support the hypothesis that successful promotion of internal drivers could have a pervasive positive impact on improving diagnostic performance.
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http://dx.doi.org/10.1136/bmjopen-2019-033390DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6936980PMC
December 2019

Spinal cord swelling in patients with cervical compression myelopathy.

BMC Musculoskelet Disord 2019 Jun 14;20(1):284. Epub 2019 Jun 14.

Department of Orthopaedic Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan.

Background: Intramedullary hyperintense lesions associated with spinal cord edema on T2-weighted MR images (T2WI) are rare findings in patients with cervical spondylosis and are poorly characterized. We investigated the clinical characteristics of spinal cord edema due to cervical spondylosis (SCECS).

Methods: In total, 214 patients with cervical spondylotic myelopathy who underwent surgery between April 2007 and March 2017 were divided into SCECS and non-SCECS groups with SCECS defined as follows: (1) intramedullary signal intensity (ISI) of the cervical spinal cord in sagittal T2WI extending to more than one vertebral body height; (2) "fuzzy" ISI, recognized as a faint intramedullary change with a largely indistinct and hazy border; and (3) a larger sagittal diameter of the spinal cord segment with ISI just above or below the cord compression area compared with areas of the cervical spine without ISI. Radiographic parameters, demographic characteristics, and the Japanese Orthopedic Association (JOA) surgical outcomes score were compared between the groups.

Results: Seventeen patients (7.9%) were diagnosed with SCECS. These patients were younger than those in the non-SCECS group [median (interquartile range), 64 (20) vs. 69 (15) years, respectively, p = 0.016], and the disease duration from onset to surgery was significantly shorter in the SCECS group than in the non-SCECS group [6 (7) vs. 20 (48) months, respectively]. No significant difference was observed between groups with respect to sex, radiologic findings, or surgical outcomes.

Conclusion: The disease showed an earlier onset and more rapid progression in the patients with SCECS than in those without SCECS.
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http://dx.doi.org/10.1186/s12891-019-2673-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6570955PMC
June 2019

Acute flaccid myelitis associated with enterovirus D68 in a non-epidemic setting.

IDCases 2019 3;17:e00549. Epub 2019 May 3.

Department of Pediatrics, Okayama Red-Cross Hospital, Okayama, Japan.

Acute flaccid myelitis (AFM) is a recently defined clinical disease accompanied by the national outbreak of enterovirus D68 (EV-D68) in the United States during the late summer/fall of 2014; 258 cases of EV-D68 and 59 cases of AFM were reported in Japan during the late summer/fall of 2015. Subsequently, there have been no epidemics of AFM or EV-D68. However, we encountered a patient who had AFM associated with EV-D68 in 2017. This is the first case of AFM caused by EV-D68 after the 2015 epidemic, and the only reported case in 2017. This report indicates that AFM caused by EV-D68 can arise even in non-epidemic situations. If a patient presents with paralysis, AFM caused by EV-D68 should be included in the differential diagnosis, regardless of the absence of an epidemic of EV-D68 infection.
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http://dx.doi.org/10.1016/j.idcr.2019.e00549DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6515126PMC
May 2019

Evaluation of the depiction ability of the microanatomy of the temporal bone in quarter-detector CT: Model-based iterative reconstruction vs hybrid iterative reconstruction.

Medicine (Baltimore) 2019 Jun;98(24):e15991

Department of Radiology, Graduate School of Medicine, University of Tokyo.

Little is known regarding differences between model-based iterative reconstruction (MBIR) and hybrid iterative reconstruction (HIR) in temporal bone computed tomography (CT). This study compared the ability to depict microstructures in temporal bone in quarter-detector CT (QDCT) between MBIR and HIR.Sixty-two temporal bones in 31 consecutive adult patients who underwent QDCT were included. Reconstruction was performed with Forward projected model-based Iterative Reconstruction SoluTion (FIRST) BONE mild mode and Adaptive Iterative Dose Reduction 3D (AIDR3D) enhanced mild mode. Imaging quality was graded for 3 microstructures (spiral osseous lamina, tympanic membrane, and singular canal).Spiral osseous lamina was significantly well-delineated in the AIDR3D enhanced group, compared with the FIRST group. In nearly all cases with FIRST, spiral osseous lamina was poorly defined. Although there was no significant difference, depiction of the tympanic membrane and singular canal tended to be better with AIDR3D enhanced mode.Routine reconstruction for preoperative temporal bone CT should be performed with HIR, rather than MBIR.
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http://dx.doi.org/10.1097/MD.0000000000015991DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6587614PMC
June 2019

Effect of bolus tracking region-of-interest position within the descending aorta on luminal enhancement of coronary arteries in coronary computed tomography angiography.

Medicine (Baltimore) 2019 May;98(19):e15538

Department of Radiology, Graduate School of Medicine, University of Tokyo.

To compare coronary artery luminal enhancement in coronary computed tomography angiography (CCTA) between ventral and dorsal region-of-interest (ROI) bolus tracking in the descending aorta.The records of 165 consecutive patients who underwent CCTA with non-helical acquisition from July 2017 to March 2018 were retrospectively examined. We performed 320-row CCTA with bolus tracking [scan triggered at 260 HU in the descending aorta] and 133 patients were finally included. ROI was set in the ventral and dorsal halves of the descending aorta in 68 and 65 patients, respectively.Contrast arrival time was significantly shorter in the dorsal group (ventral: 21.8 ± 0.372 s; dorsal: 20.7 ± 0.369; P = .0295). The mean density of the proximal and distal RCA was significantly higher in the ventral group (proximal: ventral, 428.1 ± 6.95 HU; dorsal, 405.5 ± 7.72 HU, P = .0318; distal: ventral, 418.0 ± 9.29 HU; dorsal, 393.2 ± 9.46 HU, P = .0133).Dorsal bolus tracking ROI in the descending thoracic aorta significantly reduced preparation time and RCA CT values.
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http://dx.doi.org/10.1097/MD.0000000000015538DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6531088PMC
May 2019

Reply to "Poor clinico-radiological correlation: A hallmark of acute flaccid myelitis".

Brain Dev 2019 05 11;41(5):482. Epub 2019 Mar 11.

Department of Radiology, Graduate School and Faculty of Medicine, The University of Tokyo, Tokyo, Japan.

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http://dx.doi.org/10.1016/j.braindev.2019.02.009DOI Listing
May 2019

Serial MRI findings of acute flaccid myelitis during an outbreak of enterovirus D68 infection in Japan.

Brain Dev 2019 May 26;41(5):443-451. Epub 2018 Dec 26.

Infectious Disease Surveillance Center, National Institute of Infectious Diseases, Tokyo, Japan.

Objecive: To clarify the neuroimaging findings of children with acute flaccid myelitis during an outbreak of EV-D68 infection.

Methods: We performed a detailed review of the spinal and cranial MRI results of 54 children with acute flaccid myelitis. We focused on the range of longitudinal lesions, the localization and appearance of lesions within a horizontal section, Gadolinium-enhancement, and changes over time.

Results: All children had longitudinal spinal lesions involving central gray matter. Twenty-six children had lesions spanning the entire spine. Six of them had weakness in all limbs, whereas seven had weakness of only one limb. Thirty-eight children had lesions in both gray and white matter and limb weakness tended to be more severe in these children. During the acute period, spinal lesions showed bilateral ill-defined widespread T2 hyperintensity. During the subacute period, lesions were well defined and confined to the anterior horn. The distribution of limb weakness was correlated with the appearance of lesions during the subacute period. Gadolinium enhancement was performed in 37 children, and enhancement was seen in the cauda equina in 29 children. Enhancement was infrequent within 2 days after onset but was seen in almost all children thereafter. Twenty-two children had brainstem lesions continuous with spinal lesions.

Conclusion: Extensive longitudinal spinal lesions were characteristic in children with acute flaccid myelitis. Lesions were usually bilateral and widespread during the acute period, whereas localization to the anterior horn could become obvious. Although enhancement of the cauda equina was often observed, its appearance was sometimes delayed.
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http://dx.doi.org/10.1016/j.braindev.2018.12.001DOI Listing
May 2019

[Diplopia/Double Vision].

Authors:
Harushi Mori

Brain Nerve 2018 Dec;70(12):1349-1358

Department of Radiology, Graduate School and Faculty of Medicine, the University of Tokyo.

Here, I review the efficacy of radiological methods in the diagnosis of diplopia/double vision. The simplest technique for achieving a successful diagnosis is to capture a wide field-of-view image. In order to start the process of diagnosis, it is fundamental to first study the image findings. Analysis based on the principle of MECE or mutually exclusive collectively exhaustive is performed using four methods: deductive reasoning, fractionation, longitudinal study, and priority setting. The conventional practical procedure to attain a diagnosis is as follows. First, identify the location of the lesion by imaging, which will shorten the list of differential diagnosis. Second, obtain as much information as possible on the characteristics of the lesion in order to determine the pathology. Third, look for any associated findings, such as tortuous vasculature around the brain. Fourth, refer to all the available information; for example, the main complaint, clinical history, previous history, family history, physical findings, physiological findings, laboratory data, previous images, and other modalities. Finally, if still in doubt, one should consult with colleagues and the attending physician. However, because rationality (statistical analyses, such as posterior probability or positive predictive value with positive findings), predicted utility, and emotions play a factor in a person's decision making, it seems impossible to completely avoid oversights and misdiagnosis.
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http://dx.doi.org/10.11477/mf.1416201193DOI Listing
December 2018

Acute flaccid myelitis: an emerging clinical entity.

Dev Med Child Neurol 2019 03 12;61(3):290-291. Epub 2018 Nov 12.

Department of Radiology, Graduate School and Faculty of Medicine, The University of Tokyo, Tokyo, Japan.

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http://dx.doi.org/10.1111/dmcn.14107DOI Listing
March 2019

Biallelic COLGALT1 variants are associated with cerebral small vessel disease.

Ann Neurol 2018 12 30;84(6):843-853. Epub 2018 Nov 30.

Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Objective: Approximately 5% of cerebral small vessel diseases are hereditary, which include COL4A1/COL4A2-related disorders. COL4A1/COL4A2 encode type IV collagen α1/2 chains in the basement membranes of cerebral vessels. COL4A1/COL4A2 mutations impair the secretion of collagen to the extracellular matrix, thereby resulting in vessel fragility. The diagnostic yield for COL4A1/COL4A2 variants is around 20 to 30%, suggesting other mutated genes might be associated with this disease. This study aimed to identify novel genes that cause COL4A1/COL4A2-related disorders.

Methods: Whole exome sequencing was performed in 2 families with suspected COL4A1/COL4A2-related disorders. We validated the role of COLGALT1 variants by constructing a 3-dimensional structural model, evaluating collagen β (1-O) galactosyltransferase 1 (ColGalT1) protein expression and ColGalT activity by Western blotting and collagen galactosyltransferase assays, and performing in vitro RNA interference and rescue experiments.

Results: Exome sequencing demonstrated biallelic variants in COLGALT1 encoding ColGalT1, which was involved in the post-translational modification of type IV collagen in 2 unrelated patients: c.452 T > G (p.Leu151Arg) and c.1096delG (p.Glu366Argfs*15) in Patient 1, and c.460G > C (p.Ala154Pro) and c.1129G > C (p.Gly377Arg) in Patient 2. Three-dimensional model analysis suggested that p.Leu151Arg and p.Ala154Pro destabilized protein folding, which impaired enzymatic activity. ColGalT1 protein expression and ColGalT activity in Patient 1 were undetectable. RNA interference studies demonstrated that reduced ColGalT1 altered COL4A1 secretion, and rescue experiments showed that mutant COLGALT1 insufficiently restored COL4A1 production in cells compared with wild type.

Interpretation: Biallelic COLGALT1 variants cause cerebral small vessel abnormalities through a common molecular pathogenesis with COL4A1/COL4A2-related disorders. Ann Neurol 2018;84:843-853.
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http://dx.doi.org/10.1002/ana.25367DOI Listing
December 2018

Improvement in anti--methyl-d-aspartate receptor antibody-mediated temporal lobe epilepsy with amygdala enlargement without immunotherapy.

Epilepsy Behav Case Rep 2018 3;10:96-98. Epub 2018 Aug 3.

Department of cellular neurobiology, Brain research institute, Niigata University, Japan.

Focal neuroinflammation is considered one of the hypotheses for the cause of temporal lobe epilepsy (TLE) with amygdala enlargement (AE). Here, we report a case involving an adult female patient with TLE-AE characterized by late-onset seizures and cognitive impairment. Anti--methyl-d-aspartate receptor (NMDAR) antibodies were detected in her cerebrospinal fluid. However, administration of appropriate anti-seizure drugs (ASD), without immunotherapy, improved TLE-AE associated with NMDAR antibodies. In the present case, two clinically significant observations were made: 1) anti-NMDAR antibody-mediated autoimmune processes may be associated with TLE-AE, and 2) appropriate administration of ASD alone can improve clinical symptoms in mild cases of autoimmune epilepsy.
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http://dx.doi.org/10.1016/j.ebcr.2018.07.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6120426PMC
August 2018

Single-energy metal artifact reduction technique for reducing metallic coil artifacts on post-interventional cerebral CT and CT angiography.

Neuroradiology 2018 Nov 24;60(11):1141-1150. Epub 2018 Aug 24.

Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.

Purpose: To evaluate the effects of the single-energy metal artifact reduction (SEMAR) algorithm on image quality of cerebral CT and CT angiography (CTA) for patients who underwent intracranial aneurysm coiling.

Methods: Twenty patients underwent cerebral CT and CTA using a 320-detector row CT after intracranial aneurysm coiling. Images with and without application of the SEMAR algorithm (SEMAR CT and standard CT images, respectively) were reconstructed for each patient. The images were qualitatively assessed by two independent radiologists in a blinded manner for the depiction of anatomical structures around the coil, delineation of the arteries around the coil, and the depiction of the status of coiled aneurysms. Artifact strength was quantitatively assessed by measuring the standard deviation of attenuation values around the coil.

Results: The strength of artifacts measured in SEMAR CT images was significantly lower than that in standard CT images (25.7 ± 10.2 H.U. vs. 80.4 ± 67.2 H.U., p < 0.01, Student's paired t test). SEMAR CT images were significantly improved compared with standard CT images in the depiction of anatomical structures around the coil (p < 0.01, the sign test), delineation of the arteries around the coil (p < 0.01), and the depiction of the status of coiled aneurysms (p < 0.01).

Conclusion: The SEMAR algorithm significantly reduces metal artifacts from intracranial aneurysm coiling and improves visualization of anatomical structures and arteries around the coil, and depiction of the status of coiled aneurysms on post-interventional cerebral CT.
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http://dx.doi.org/10.1007/s00234-018-2081-6DOI Listing
November 2018
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