High Intensity Lesions Research Articles

Neuronal Intranuclear Inclusion Disease Presented with Acute Aphasia

Neuronal intranuclear inclusion disease (NIID) is a rare disorder with slowly progressive neurodegeneration. Major clinical presentation is dementia, but some patients can have episodic symptoms. This is a rare case of NIID presented with acute aphasia. A 62-years-old male visited emergency room presenting with aphasia. On brain magnetic resonance imaging, a U-shaped diffusion high signal intensity lesion in bilateral frontal corticomedullary junction was observed. A skin biopsy showed intranuclear cytoplasmic inclusion bodies, so he was diagnosed as NIID.

Unusual cause of urinary retention in an infant

Introduction: Gastrointestinal tract duplication cysts constitute a rare group of malformation. Patients with presacral mass present with a diagnostic challenge due to the less obvious presentation. Case Report: We report a 7-month-old boy, who presented with constipation and acute intermittent urinary retention. On per-rectal examination, a palpable mass was felt posteriorly with a narrow base, raising a query of pre-sacral/pelvic mass. Ultrasound pelvis revealed a well-defined, rounded oval-shaped cystic mass noted in the center of the lower pelvis, posteriorly. Computed tomography (CT) scan abdomen confirmed a cystic pelvis mass anterior to the rectum causing mass effect. Magnetic resonance imaging (MRI) of the pelvis was done to define its relationship to the sacral spine. A high-intensity lesion was seen on T2 imaging with no spinal involvement. Hence surgical exploration was done, and per operative diagnosis of rectal duplication was confirmed. Postoperatively child was discharged on the fourth post-operative day and doing well on follow-up. Conclusion: We emphasize a complete examination, including per rectal examination in any child presenting with acute urinary retention to exclude pre-sacral masses.

Malassezia restricta as an unexpected cause of infectious osteomyelitis diagnosed by metagenomic sequencing: a case report and literature review

BackgroundMalassezia restricta, a lipophilic and lipodependent yeast belonging to the basidiomycetes group, is an opportunistic fungal pathogen associated with various skin diseases, including seborrheic dermatitis and dandruff. Typically, Malassezia infection in neonates manifests as fungemia or hematogenous dissemination to the bone or lungs. However, vertebral osteomyelitis caused by these fungi is rarely reported owing to non-specific clinical presentations and laboratory/imaging findings. The Pathogen Metagenomics Sequencing (PMseq) technique enables direct high-throughput sequencing of infected specimens, facilitating the rapid and accurate detection of all microorganisms in clinical samples through comprehensive reports.Case presentationA 52-year-old male was admitted to our hospital on July 20, 2022 with a 3-month history of ambulatory difficulties and localized low back pain. Magnetic Resonance Imaging (MRI) examination of the spinal column revealed irregular bone destruction affecting the L2, L3, and L5 vertebral bodies. Additionally, low T1 and high T2 intensity lesions were observed at the intervertebral discs between L3 and L5. The presumptive diagnosis of tuberculous spondylitis was made based on the imaging findings, despite negative results in all mycobacterium tests. However, the patient exhibited no improvement after receiving regular anti-tuberculosis treatment for 3 months. Subsequent MRI revealed an expansive abnormal signal within the vertebral body, leading to progressive bone destruction. The absence of spinal tuberculosis or other infective microorganisms was confirmed through culture from blood and pathological tissue from the L4 vertebral body. Subsequently, PMseq was performed on the specimens, revealing M. restricta as the predominant pathogen with the highest relative abundance value. The pathological examination revealed the presence of fungal mycelium in the L4 vertebral body, with positive findings on periodic Schiff-methenamine and periodic acid-Schiff staining. The anti-tuberculosis treatment was discontinued, and an antifungal combination of fluconazole and voriconazole was administered. All symptoms were resolved after 7 consecutive months of treatment, and the patient was able to ambulate autonomously. Vertebral lesions were reduced on MRI during the 13-month follow-up.ConclusionsM. restricta is not a commonly recognized pathogen associated with infectious vertebral osteomyelitis. However, PMseq can aid in diagnosis, timely treatment, and decision making for some non-specific infectious diseases.

Ruptured Vertebral Artery Dissection Treated with Endovascular Intervention

A retrospective study of cases of endovascular treatment of dissection of the vertebral artery with subarachnoid hemorrhage was conducted. Data were 11 cases of vertebral artery dissecting aneurysm (VADA) among 291 consecutive subarachnoid hemorrhage patients who underwent clipping or endovascular treatment at Ota Memorial Hospital. Classified into 4 patterns based on the location of the dissection and posterior inferior cerebellar artery (PICA): pre-PICA, post-PICA, involved PICA, and non-PICA. And one of the cases had bilateral vertebral artery dissection, and computational fluid dynamics analysis was included in the study. Ruptured VADA occurred in 11 of the 291 patients (3.8%). Endovascular treatment was performed in 8 of these 11 patients. Postoperative diffusion-weighted imaging detected no high-intensity lesions and no postoperative ischemic complications or rebleeding occurred in any patient. In a case of bilateral VADA, computational fluid dynamics analysis of very low or high wall shear stress at the dissection, low aneurysm formation indicator, and high oscillatory shear index may be considered rupture factors. Treatment strategies for each branching pattern of PICA can prevent rupture and avoid ischemic complications. And prediction of the rupture side is important in patients with bilateral dissection to consider the appropriate treatment and timing.

Time course of tumorigenesis of a newly developed sporadic hemangioblastoma in an elderly patient: illustrative case.

von Hippel-Lindau disease-associated hemangioblastomas (HBs) account for 20%-30% of all HB cases, with the appearance of new lesions often observed in the natural course of the disease. By comparison, the development of new lesions is rare in patients with sporadic HB. A 65-year-old man underwent clipping for an unruptured aneurysm of the anterior communicating artery. Fourteen years later, follow-up magnetic resonance imaging (MRI) revealed a strongly enhanced mass in the right cerebellar hemisphere, diagnosed as a sporadic HB. A retrospective review of MRI studies obtained over the follow-up period revealed the gradual development of peritumoral edema and vascularization before mass formation. Newly appearing high-intensity T2 lesions in the cerebellum may represent a preliminary stage of tumorigenesis. Careful monitoring of these patients would be indicated, which could provide options for early treatment to improve patient outcomes.

Prevalence and association of high-intensity lesions with degenerative processes in lumbar intervertebral discs

Purpose: This population-based cross-sectional study examined the prevalence of high-intensity zones (HIZ) in the lumbar spine and their relationship to age, gender, disc degeneration, disc displacement (bulging or herniation), and facet joint degeneration.
 Materials and Methods: A retrospective analysis was conducted on MRI studies of 800 patients (4000 discs) who were admitted to the hospital with subacute-chronic low back pain (LBP) and presented with axial pain (musculogenic-discogenic-mechanic) at outpatient clinics. The frequency of speed was determined by considering a total of seven age categories in decades. Correlation analyses were performed between HIZ and other variables.
 Results: The prevalence of HIZ was 30%, highest in the >70 years age group and lowest in the

Acute Hemorrhagic Leukoencephalitis with Concurrent Retinal Vasculitis in an Elderly Japanese Patient: A Case Report.

A 68-year-old Japanese man developed a fever, headache, hiccups, and altered consciousness. Brain magnetic resonance imaging (MRI) revealed a hemorrhagic lesion in the right temporal lobe and multiple high-intensity white matter lesions. A brain biopsy showed pathological findings consistent with acute disseminated encephalomyelitis (ADEM), suggesting a diagnosis of acute hemorrhagic leukoencephalitis (AHLE), an aggressive ADEM variant. The patient also developed myodesopsia and was diagnosed with retinal vasculitis, likely due to a hyperimmune state caused by AHLE. Corticosteroids enabled full recovery. Although AHLE is uncommon in elderly individuals, clinicians should be aware of its occurrence in this patient subgroup and recognize potential retinal manifestations associated with AHLE.

10109-COT-13 LONG-TERM FOLLOW-UP OF FLAIR HIGH LESION IN THE BRAIN

Abstract AIM Incidental FLAIR (Fluid Attenuated Inversion Recovery) high-intensity lesion in the brain can be considered as IDH-mutant low-grade glioma (LGG). Differential diagnosis includes trauma, ischemic changes, demyelinating diseases, and other inflammatory diseases. The biopsy is sometimes high risk for a young patient or an asymptomatic patient with a lesion in the brain stem, motor or language area. Imaging intervals vary among hospitals and the method for regular follow-up for these cases has not been established. METHODS We have studied 26 patients with FLAIR high lesion from 2012 to 2022. In cases of suspected LGG, the biopsy was performed, when necessary, after consultation with the patient and family. Our neurosurgery team protocol was to perform PET-MRI at the first consultation, and if there was negative, we have scheduled MRI examination at the next visit in one month. Then, MRI was performed every 2-3 months for the first year and every 3-6 months thereafter. The clinical features of these FLAIR high lesion and their differentiations from LGG in our cases are examined. RESULTS Biopsy was performed in 5 cases, but no tumor was found. The male-to-female ratio was 7:19, which was more common in female. The median age at onset was 44 years (15-47 years), showing a bimodal distribution with peaks in younger and older individuals. Among 26 cases, 14 cases had been unchanged for more than 5 years. The median maximum lesion diameter was 17 mm (8-47 mm). An annual average increase of LGG has been reported to be 3-5 mm in (Pallud, 2010). There were no cases diagnosed as glioma in cases where no expansion was seen after 5 years or more. CONCLUSION In this study, the possibility of glioma was considered to be low for cases in which no expansion on MRI was seen after 5 years or more.

T1-T2 Mismatch Sign as a Predictor of Ipsilateral Ischemic Change After Carotid Artery Stenting

Magnetic resonance (MR)-plaque imaging reflects the characteristics of carotid plaque. We evaluated the relationship between MR-plaque images and ischemic change after carotid artery stenting (CAS). MR-plaque images were acquired from patients with carotid artery stenosis before CAS treatment. We calculated the relative signal intensity of plaque components compared with that of the sternocleidomastoid muscle and evaluated the presence/absence of T1-T2 mismatch and match sign. We then assessed the appearance of new ischemic lesions after CAS on diffusion-weighted imaging (DWI). Factors associated with the appearance of a high-intensity lesion on DWI were retrospectively analyzed. A total of 64 patients with carotid artery stenoses treated with CAS were included in this study. In univariate analysis, T1-T2 mismatch sign was associated with the appearance of high-intensity lesions on DWI after CAS (odds ratio [OR], 12.00; 95% confidence interval [CI], 3.593-40.072; P < 0.0001), whereas T1-T2 match sign and high intensity on T2-weighted imaging were negatively associated (OR, 0.061, 95% CI, 0.007-0.502, P= 0.009 and OR, 0.085; 95% CI, 0.022-0.334, P= 0.0004, respectively). In multivariate logistic regression analysis, T1-T2 mismatch sign was independently associated with the appearance of a high-intensity lesion on DWI after CAS (OR, 16.695; 95% CI, 1.324-210.52; P= 0.0295). T1-T2 mismatch sign on MR-plaque imaging is significantly associated with the appearance of new ischemic lesions after CAS. T1-T2 mismatch sign may be useful in considering treatment strategies for carotid artery stenosis.

Osmotic demyelination syndrome with transient diabetes insipidus in postpartum female: a case report.

The authors present a case of 29-year-old female who presented with chief complaint of altered sensorium and quadriparesis. Two days prior to onset of symptoms, she underwent caesarean section, was kept on nil per oral and free fluid restriction, after which she had confusion, altered sensorium, and weakness in all four limbs. Sodium level was 170mEq/l. Urine osmolality and plasma osmolality was 150 and 410mOsm/kg of water, respectively. MRI showed high signal intensity lesion in pons suggestive of demyelination. She was diagnosed ODS with transient DI and quadriparesis, in postpartum period due to further rise in sodium after free fluid restriction and nil per oral. She was treated with desmopressin, 5% dextrose and 0.9% normal saline, her quadriparesis recovered and desmopressin was tapered and stopped over 45 days and discharged at stable state. ODS can rarely be associated with hypernatremia in postpartum female presenting as quadriparesis and altered sensorium. Clinicians should be familiar of ODS with hypernatremia with transient DI in postpartum period, which is reversible and can be managed by desmopressin and fluid replacement.

Fahr disease presenting with multiple cerebral calcifications

Fahr disease, also known as idiopathic basal ganglion calcification, is a rare and genetically heterogeneous neurological disorder. Herein, we report a rare case of Fahr disease presenting with multiple cerebral calcifications. A 63-year-old woman presented with dizziness to a local clinic. Brain computed tomography performed on admission revealed multiple high-intensity lesions in both basal ganglia, both cerebellar hemispheres, and the left frontal lobe. In contrast, brain magnetic resonance imaging (MRI) showed no prominent lesions on T2- and T1-weighted images, and there was no contrast enhancement after gadolinium injection. However, multiple dark signals were detected on gradient echo MRI. The location and radiological appearance of the lesion resembled those of a physiological intracranial calcification, except for asymmetric calcification in both cerebellar hemispheres and the left frontal subcortical white matter. The patient was diagnosed with basal ganglion calcification with multiple cerebral cavernous malformations or, less likely, brain tumors. Through a careful radiological and clinical review, the calcifications were diagnosed as Fahr disease. Follow-up was planned. It is important to consider the presence of multiple intracranial calcifications in the basal ganglia, cerebellum, or deep subcortical white matter as a warning sign for Fahr disease.

多発顔面汚染創と後弓反張から破傷風との鑑別を要したMarchiafava–Bignami diseaseの1例(A case of Marchiafava–Bignami disease required differential diagnosis with tetanus due to facial contaminated wounds and opisthotonos)

Marchiafava–Bignami disease（MBD）は，長期間のアルコール摂取や低栄養を原因に意識障害を引き起こす一方で，稀な疾患であるため意識障害の原因疾患として鑑別に挙がらず，正確なMBDの診断に至らない可能性がある。症例は大酒家の56歳男性。受診3日前に1mの高さより転落し，その後体動困難となり前医を受診した。前医では顔面および後頭部に多発する汚染創と後弓反張を認め，破傷風と診断され加療目的で当院へ転院搬送された。当院来院時，破傷風は否定的であったが，前医での経過から確実に除外することも困難であり，低侵襲な治療は必要と判断した。一方で長期のアルコール摂取歴からアルコールや栄養障害に伴う脳症を第一に疑い，ビタミンB1を中心とするビタミン補充療法を開始した。第2病日に，飲酒歴と臨床症状，前医の頭部MRI画像における脳梁膨大部高信号からMBDと診断した。ビタミン補充療法で症状は改善し，第13病日に前医へ転院となった。MBDは早期のビタミン補充療法で神経症状改善の可能性があるため，救急医はMBDの病態を理解し，長期間のアルコール摂取歴のある意識障害患者では，MBDを想起し鑑別を行うことが重要である。 Marchiafava–Bignami disease (MBD) is a rare, severe, and usually fatal neurological disorder associated with chronic alcoholism. An accurate diagnosis of MBD is difficult. A 56–year–old man with a history of long–term alcohol use disorder originally visited a doctor because he had fallen from a height of 1m three days prior, injuring himself and subsequently experiencing difficulty moving. His doctor identified contaminated wounds and opisthotonos. He was diagnosed with tetanus and transferred to our hospital for intensive tetanus–specific care; treatment was initiated despite scant symptoms. Given his history, alcoholic encephalopathy was also considered, and vitamin supplementation therapy was initiated. On day 2, he was diagnosed with MBD based on symptoms and the presence of a high–intensity lesion in the splenium of the corpus callosum on diffusion–weighted and fluid–attenuated inversion recovery imaging. His symptoms improved with vitamin supplementation therapy. On day 13, he returned to the original hospital. This case emphasizes the need to consider MBD when patients with impaired consciousness have a history of long–term alcohol use disorder. Marchiafava–Bignami disease（MBD）は，長期間のアルコール摂取や低栄養を原因として脳梁に脱髄壊死を来す疾患であり，急性期には意識レベル低下を含む精神状態の変化や歩行障害，構音障害を呈する 1。MBDは稀な疾患であるため意識障害の原因疾患として鑑別に挙がらず，正確なMBDの診断に至らない可能性がある。今回我々は，受傷から数日以上経過した多発する汚染創と後弓反張から破傷風と診断され当院へ搬送となったが，長期間のアルコール摂取歴と頭部MRI所見からMBDと診断された症例を経験したので報告する。 なお，本報告は介入のない1例の症例報告であり，倫理委員会の承諾を必要としない。また本報告にかかわる症例はすべて個人情報保護法に基づいて匿名化が行われており，報告に際しては患者本人より同意を取得している。 患 者：56歳，男性，身長173cm，体重53kg，BMI 17.7 既往歴：高血圧症 内服薬：アムロジピン5mg/日 喫煙歴：15本/日，36年間 飲酒歴：焼酎 500mL/日，36年間 現病歴：患者は焼酎を500mL/日ほど飲酒していたが，受診3か月前に失業し，その後から飲酒量が1,000mL/日に増加した。受診2か月前からふらつきが出現し，頻回に転倒するようになった。受診1か月前には｢着物を着た猫が見える｣などの幻覚症状が出現し，それを追いかけるようになった。受診3日前に1mの高さの窓から地面に頭部から転落し，受診2日前から呂律が回らなくなった。その後ふらつきが悪化し体動困難となったため前医を受診した。身体所見では全身不潔と異臭が目立ち，鼻根部と頭部に受傷時期不明な骨組織まで露出する汚染した挫滅創を数か所認めた。診察後に撮影したCTでは明らかな骨折や出血はなく，続けて撮像を行ったMRIでは拡散強調画像とfluid attenuated inversion recovery（FLAIR）画像で脳梁部に高信号（Fig. 1a, b）を，apparent diffusion coefficient（ADC）画像で脳梁部に低信号（Fig. 1c）を認めたが，この時点では臨床所見との関連性は不明とされた。MRI撮像後に5分間継続する四肢を伸展し，頸部や背部を反り返らせる痙攣を認めた。痙攣の様式から後弓反張と判断し，フェニトイン750mg静注によって痙攣は消失した。顔面および頭部に多発する汚染創と後弓反張から破傷風と診断され，加療目的に同日当院へ転院搬送された。 Brain MRI on day 1. DWI (a) and FLAIR (b) showed a high intensity lesion in the splenium of the corpus callosum on MRI. ADC(c) showed a low intensity lesion in the splenium of the corpus callosum on MRI. DWI: diffusion–weighted imaging, FLAIR: fluid–attenuated inversion recovery, ADC: apparent diffusion coefficient 来院後経過：当院到着時はJapan coma scale（JCS）3，痙攣や不随意運動，錐体外路症状を認めなかった。バイタルサインは血圧126/65mmHg，心拍数98/分・整，腋窩温37.3度，SpO2 96%（大気下）であった。全身不潔で異臭があり，鼻根部と頭部に多発性の骨組織まで到達する汚染創を認めた（Fig. 2a, b）。開口障害を認めなかった。また光や疼痛刺激による痙攣誘発を認めなかった。来院時検査所見をTable 1に示す。動脈血液ガス分析では代償困難な代謝性アシドーシスや低血糖を認めなかった。血液生化学検査では，軽度の腎機能障害と貧血，炎症反応の上昇を認めた。髄液検査では細胞数の増加と糖の低下は見られず，髄膜炎は否定的であった。 Injuries to the face and head. Open wounds reaching the periosteum were found at the base of the nose (a) and back of the head (b). 前医で後弓反張を一度認めたが，来院後に開口障害，筋強剛・痙攣，自律神経障害に伴う血圧変動を認めなかった。また軽度の意識障害を認めるものの，その他のバイタルサインは安定しており，積極的に破傷風を疑う所見に乏しかったが，前医での経過から確実に破傷風を除外することも困難であった。よって侵襲性の低い破傷風に対する治療は必要と判断し，鼻根部および頭部の汚染創は救急外来でデブリドマンを行い，その後は連日創部洗浄を行い，感染コントロールを図る方針とした。また集中治療室で暗室管理を行い，マグネシウム製剤，抗破傷風ヒト免疫グロブリンおよびセフトリアキソン 2g/日の投与を開始したが，この時点での経口気管挿管による確実な気道確保は必要ないと判断した。一方で長期間の飲酒歴からアルコールや栄養障害による脳症を第一に疑い，ビタミンB1 200mg/日を中心とするビタミン補充療法を開始した。 第2病日も開口障害，筋強剛・痙攣，自律神経障害に伴う血圧変動を認めず，破傷風は否定的であった。一方でビタミン補充療法や輸液療法を中心とした全身管理の開始後から意識レベルは改善を認め，JCS 2となった。長期間のアルコール摂取歴と臨床症状，前医で撮像を行った頭部MRI拡散強調画像およびFLAIR画像での脳梁膨大部高信号（Fig. 1a, b）からMBDと診断した。また同日よりリハビリテーションを開始し，栄養はリフィーディング症候群発症の危険因子である5日以上の経口摂取不良とアルコール依存症があったため，glutamine–fiber–oligosaccharideで経腸栄養を開始し，投与カロリーを漸増する方針とした。第3病日の精神学的評価では，アルコール依存傾向はあるが離脱症状はなく，後弓反張はアルコール離脱によるものではなく，MBDに伴う症状と考えられた。その後も意識障害の悪化や筋強剛・痙攣を認めなかった。第10病日に意識レベルはJCS 1となり，汚染創は感染コントロールが得られたため縫合閉鎖を行った。第11病日までに経腸栄養を目標カロリーである34kcal/kg/日（合計1,800kcal/日）に漸増することができ，経過中にリフィーディング症候群の所見は見られなかった。第13病日には意識は清明で，リハビリテーションにより端座位まで可能となった。また経口摂取も来院時は不可能であったが，意識レベルの改善に伴いゼリー摂取可能なまでに改善を認めた。同日リハビリテーション継続目的に前医へ転院となった。 MBDは1903年にMarchiafavaとBignamiが3例の剖検所見を初めて報告した疾患であり 2，1981年から2012年1月までの約31年間で153例が報告されている 1。長期間のアルコール摂取や低栄養が原因と考えられており，臨床症状として初期には本症例のように意識レベル低下を含む精神状態の変化や歩行障害，構音障害を来すことが多い 1。MBDの診断には頭部MRIが有用であり，拡散強調像とFLAIR画像で脳梁に左右対称性に高信号を呈することが特徴である。ADC画像ではADC値が上昇する場合も，低下する場合もあるとされている 3。これは長期間のアルコール摂取や低栄養によるビタミンB1や微量元素不足による血管壁障害を背景に，ピルビン酸の蓄積に伴う局所アシドーシスや血液脳関門の破綻を引き起こし，脳梁の浮腫誘発と同部位の脱髄壊死，血管漏出性出血を来すためと報告されている 4。病変は脳梁内の中間層に限局し，上下層は障害を免れる傾向を認める（sandwich sign）が，なぜ病変が中間層に限局するかは不明である 5。一方で脳梁高信号はMBD以外にも，ウイルス性脳炎 / 脳症，てんかん発作，高Na血症，低血糖でも来しうるため 6，血液検査や髄液検査を行い他の疾患を鑑別する必要がある。MBDはしばしば脳梁外病変を合併することが知られており，脳梁病変と同様に左右対称性であることが多い。脳梁外病変の中でも皮質病変は，認知症などの予後を決定する重要因子と考えられている 7。Heinrichらは，神経放射線学的所見と臨床症状および予後との関連性について検討しており，急性期の意識障害が強く，画像所見では脳梁全体に病変が及び，予後不良なA群と，急性期の意識障害がないか軽度で，脳梁の障害が部分的な予後良好なB群に分類している 8。MBDに対する特異的な治療法はないが，他のアルコール性脳症と同様に早期のビタミンB1を中心とするビタミン補充療法が神経症状の改善に有効である。またビタミン補充療法の一つであるチアミン大量療法は，1,500mg/日のチアミンを3～5日間，その後250mg/日のチアミンを3～5日間または神経症状の改善が得られるまで投与を行う方法で，Wernicke脳症に対する治療として脳神経領域で行われており 9，MBDでも有効である可能性が報告されている 10。一方でビタミン補充療法に反応せず，長期にわたって見当識障害，健忘，失行といった神経症状が継続した報告もあり 3，治療開始後も長期の経過観察が必要と考えられる。本症例は長期間のアルコール摂取歴と，MRI拡散強調画像およびFRAIR画像でMBDに特徴的な脳梁膨大部の高信号を認めた。一方で血液検査･髄液検査で脳炎 / 髄膜炎，電解質異常，代謝異常などの脳梁膨大部に高信号を来す他の疾患を否定できたため，MBDと診断した。来院時JCS 3と意識障害が軽度であり，MRI画像で脳梁高信号が部分的であったため，本症例はB群に分類されると考えられた。実際，入院後直ちにビタミン補充療法を開始し，意識障害は改善が得られ経過は良好であった。本症例ではビタミンB1 200mg/日と全身管理で意識レベルは改善を認めたが，意識レベルの改善が得られなければチアミン大量療法の実施も検討に値する状況であったと考えられる。 一方で初診時に疑われた破傷風は，破傷風菌が産生する神経毒素により運動ニューロンの抑制シグナル伝達が阻害され，全身の筋緊張亢進を来し，重篤化すると自律神経障害を引き起こす疾患である 11。開口障害など初期症状から全身性痙攣が始まるまでの時間をonset timeといい，これが48時間以内である場合，予後が不良といわれている 12。本症例は来院時に意識障害を認め，開口障害，筋強剛・痙攣，自律神経障害に伴う血圧変動など破傷風に特徴的な臨床症状が乏しかった。また呂律障害を初期症状として，後弓反張を来し破傷風第3期まで進行したとすれば，本症例のように破傷風の治療開始直後から経時的に症状が改善するような経過をたどるとは考え難く，破傷風は否定的と判断できる。 後弓反張は四肢を伸展し頸部や体幹を反り返らせる痙攣様式と定義されており 11，破傷風以外では解離性障害や髄膜炎でも来すことが報告されている 13, 14。本症例では精神疾患の既往はなく，髄液検査でも髄膜炎を示唆する所見は見られず，いずれも否定的と判断した。一方でMBDの報告例の中には四肢対称性に筋強直を示した症例があり 15，本症例でも前医で四肢を伸展し頸部や体幹を反り返らせる痙攣を認めていた。これらの痙攣様式は後弓反張の定義と矛盾せず，MBDでも後弓反張を呈しうると考えられた。本症例ではフェニトインで速やかに鎮痙が得られたが，MBDの痙攣に対する特異的な治療法の報告はなく，てんかん重積状態に準じて抗けいれん薬を使用することが有効である可能性がある。 本症例は外傷歴と後弓反張から破傷風として紹介されたが，長期間のアルコール摂取歴と頭部MRI画像から後にMBDと診断された。MBDは稀な疾患であるため，意識障害の原因疾患として鑑別に挙がらず，正確なMBDの診断に至らない可能性がある。意識障害の原因として長期間のアルコール摂取歴は重要であり，意識障害を伴う場合には，飲酒歴を聴取しMBDなどのアルコール関連疾患の可能性を検討する必要がある。また長期間のアルコール摂取歴と精神状態の変化や歩行障害などの特徴的な臨床症状から，MBDなどのアルコール関連脳症が疑われる患者では，早急にビタミン補充療法を開始し，診断のため全身状態安定後に頭部MRI検査を実施する必要がある。 外傷歴と後弓反張から初診医で破傷風と診断されたものの，長期間のアルコール摂取歴とMRI所見よりMBDと判明した症例を経験した。MBDは早期に適切なビタミン補充療法がなされれば神経症状の改善の可能性があるため，救急医はMBDの病態を理解し，長期間のアルコール摂取歴がある意識障害患者では本疾患を想起し鑑別を行うことが重要である。 本稿のすべての著者には規定された利益相反はない。

Cavitation with low-energy sonication during focused ultrasound thalamotomy for a patient with tremor-dominant Parkinson's disease: a potential risk.

We report a patient with tremor-dominant Parkinson's disease who had a mild cavitation bioeffect during magnetic resonance-guided focused ultrasound thalamotomy. During the aligning phase with low-energy sonication, cavitation caused mild dysarthria and paresthesia, prompting treatment cessation. At the same time, tremor and rigidity improved. MRI revealed extensive high-intensity lesions in the thalamus 1day after the procedure followed by steroid infusion, which resulted in resolution of adverse events. Tremor and rigidity improved 1.5years after the procedure. Although cavitation can relieve tremors and rigidity, it should be carefully monitored due to potential permanent adverse events by unpredictable and unknown behaviors.

Two Cases of Juvenile Myelomonocytic Leukemia and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

BackgroundMyelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an autoimmune demyelinating disorder that often manifests after infections or vaccinations. We report two patients who developed MOGAD out of eight patients with juvenile myelomonocytic leukemia (JMML) that has never been reported. MethodsWe investigated two patients with JMML who developed MOGAD among 127 patients with leukemia from 2012 to 2021. ResultsPatient 1 was treated for JMML and developed fever and impaired consciousness at two years and one month of age. Magnetic resonance imaging revealed high-intensity lesions in the left frontal and left occipital white matter. The serum anti-MOG antibody test was positive, while the test was negative in the stored serum 45 days before the onset of encephalopathy. He had relapse of MOGAD after steroid therapy and plasmapheresis. Patient 2, who was treated for JMML, became apathetic and mute at three years and seven months of age. Magnetic resonance imaging revealed left frontoparietal subcortical high-intensity lesions. Anti-MOG antibody at the onset of encephalopathy was positive, while it was negative in stored serum 57 days before and 47 days after the onset. ConclusionWe treated two patients who developed MOGAD out of eight patients with JMML and none with MOGAD out of 119 patients with acute lymphocytic leukemia, acute myelocytic leukemia, or chronic myelocytic leukemia. The activated autoimmune process via the RAS pathway abnormality may have led to the formation of the anti-MOG antibody and the onset of MOGAD. MOGAD can occur in children with JMML, and abnormalities of the RAS pathway possibly contribute to its onset.

A case of neurosyphilis presenting as sudden onset of limbic encephalitis

A 52-year-old male was carried to hospital by ambulance, because of an abrupt abnormal behavior and impaired consciousness. Soon after the arrival, the patient started a generalized seizure. Although the seizure was stopped by Midazolam, amnesia were observed. With meningeal irritation signs, in addition to the clinical course, the patient was thought to develop limbic encephalitis. The cause of the encephalitis was diagnosed as neurosyphilis because of the positive serum and CSF syphilis reactions, and the patient was treated with penicillin G from the first admission day on. Steroid pulse therapy was also conducted, followed by acyclovir since herpes encephalitis could not be ruled out; the brain MRI showed left-side dominant T2/FLAIR high intensity lesions in the bilateral temporal lobes and left hippocampus. With the treatment progression, the amnestic syndrome improved and the patient returned to work. Although neurosyphilis is a rare cause of acute onset limbic encephalitis, it is important to keep the possibility of this disease in mind in making a treatment plan.

Anti-NMDAR encephalitis with GFAPα IgG: a case report

BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an inflammatory disease of the central nervous system (CNS) in which antibodies within the serum and cerebrospinal fluid (CSF) target NMDA receptors. Glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune disease affecting the central nervous system (CNS). Meningoencephalitis can affect any anatomical region rostrocaudally, from the optic nerve to the spinal cord. The clinical implications of NMDAR antibodies overlapping with other antibodies against glial or neuronal cell surface proteins have not been investigated.Case presentationA 35-year-old male presented with headaches along with amnesia, slurred and awkward speech, psychiatric symptoms, cognitive decline, and insomnia. His medical history revealed ankylosing spondylitis for six months. Ancillary findings included CSF pleocytosis and elevated protein levels. T2-weighted fluid attenuation inversion recovery was used to image high-intensity lesions of the bilateral paraventricular, radiate corona, semioval centre, and right subcortical regions. The CSF was positive for NMDAR and GFAP antibodies through transfected cell-based assays. A diagnosis of anti-GFAP encephalitis was made, although the prominent clinical features were of anti-NMDAR encephalitis.ConclusionsHerein, we describe a case of anti-NMDAR encephalitis with overlapping symptoms of GFAP antibody positivity. Patients with unusual symptoms of anti-NMDAR encephalitis should also be tested for anti-GFAP antibodies. However, because this was a single case study, caution should be exercised when interpreting the observations. Since the patient was diagnosed with autoimmune encephalitis, intravenous methylprednisolone was administered, which yielded a positive outcome.

Muscle magnetic resonance imaging abnormality in neuroleptic malignant syndrome: a case report

BackgroundNeuroleptic malignant syndrome (NMS) is a rare and occasionally fatal undesirable reaction to dopamine antagonists, and its phenotype is diverse owing to causative drugs. Classically, elevation of serum creatine kinase is described in NMS. Some reports have described muscular pathological findings; however, muscle magnetic resonance imaging (MRI) has not been reported previously.Case presentationA 63-year-old woman with a history of schizophrenia presented to our hospital with a high fever, excessive sweating, muscle weakness, and elevated serum creatine kinase levels. Muscle MRI revealed T2 high-intensity lesions in several muscles with gadolinium enhancement, and the pathology of the muscle biopsy showed a very mild presence of muscle fiber necrosis and regeneration with type 2c fibers without inflammation. Her symptoms resolved by treatment with levodopa/carbidopa, dantrolene. Finally, the patient was diagnosed with NMS.ConclusionsThis is the first report of muscle MRI abnormalities in a patient with NMS. Muscle MRI abnormalities in NMS may be associated with non-inflammatory myopathic changes. The cause of creatine kinase elevation cannot be explained by abnormal strong muscle contraction nor inflammation.

Differences between spinal cord injury and cervical compressive myelopathy in intramedullary high-intensity lesions on T2-weighted magnetic resonance imaging: A retrospective study.

Increases in aging populations have raised the number of patients with cervical spinal cord injury (SCI) without fractures due to compression of the cervical spinal cord. In such patients, it is necessary to clarify whether SCI or cervical compressive myelopathy (CCM) is the cause of disability after trauma. This study aimed to clarify the differences in magnetic resonance imaging (MRI) features between SCI and CCM.Overall, 60 SCI patients and 60 CCM patients with intramedullary high-intensity lesions on T2-weighted MRI were included in this study. The longitudinal lengths of the intramedullary T2 high-intensity lesions were measured using sagittal MRI sections. Snake-eye appearance on axial sections was assessed as a characteristic finding of CCM. The T2 values of the high-intensity lesions and normal spinal cords at the first thoracic vertebra level were measured, and the contrast ratio was calculated using these values.The longitudinal length of T2 high-intensity lesions was significantly longer in SCI patients than in CCM patients. Snake-eye appearance was found in 26 of the 60 CCM patients, but not in SCI patients. On both the sagittal and axial images, the contrast ratio was significantly higher in the SCI group than in the CCM group. Based on these results, a diagnostic scale was created. This scale made it possible to distinguish between SCI and CCM with approximately 90% accuracy.

Uncommon cause of trigeminal neuritis and central nervous system involvement by herpes labialis: a case report

BackgroundTrigeminal neuropathy is characterized by numbness in the region innervated by the trigeminal nerves, with or without neuropathic weakness in the muscles of mastication. Trigeminal neuritis is a form of trigeminal neuropathy in which the lesion is caused by an inflammation. Herein, we report a patient with trigeminal neuritis due to central nervous system (CNS) involvement of herpes labialis (HL) infection, which was successfully treated with anti-viral and anti-inflammatory agents.Case presentationA young healthy female presented with numbness in the left hemiface for two weeks. She had a preceding typical HL infection on left facial lip one week before the sensory symptom onset. Brain magnetic resonance imaging revealed high signal intensities and asymmetrical thickening with enhancement along the cisternal segment of the left trigeminal nerve. Additionally, brain MR angiography showed multifocal stenoses in the M1 segment of the middle cerebral artery and the cavernous portion of the internal carotid artery. Cerebrospinal fluid (CSF) examination showed mild pleocytosis with normal protein level, glucose ratio, but CSF polymerase chain reaction assay for specific anti-viral antibodies including herpes simplex virus was negative, and CSF culture also did not identify a specific pathogen. The results of serologic testing including tumor markers and autoimmune markers were all unremarkable. A tentative diagnosis of trigeminal neuritis as a complication of HL involving the CNS was made considering the clinical, neuroradiological, and laboratory findings of the patient. Therefore, the patient was treated with intravenous methylprednisolone and acyclovir for 10 days. After the treatments, her sensory disturbance was markedly improved. Brain MRI at the 3-month follow-up also demonstrated improvement of previously identified high signal intensity lesions and multifocal intracerebral artery stenoses.ConclusionHL is usually a self-limiting, benign disease without complications, but rarely presents as trigeminal neuritis due to CNS involvement. Therefore, meticulous evaluation may be necessary if trigeminal neuritis or CNS involving symptoms occur after HL.

Case Report: Paraneoplastic Tumefactive Demyelination Associated With Seminoma

Paraneoplastic tumefactive demyelination (TD) is a rare disorder of the central nervous system that can be challenging to diagnose. Here, we describe a 32-year-old Japanese man with a TD associated with testicular seminoma. He presented with symptoms of right-sided motor and sensory impairment 2 days after vaccination for coronavirus disease 2019 (COVID-19). Brain magnetic resonance imaging (MRI) showed a high-intensity lesion in the left internal capsule. He had a 3-year history of enlargement of the left testicle. Blood examination showed tumor marker elevation and the presence of anti-amphiphysin antibodies. Whole-body computed tomography (CT) revealed mass lesions in the left testicle and enlargement of the retroperitoneal lymph nodes. Radical orchiectomy was performed. As the pathology showed testicular seminoma, chemotherapy was administered. After surgery, his neurological symptoms deteriorated. MRI revealed that the brain lesion had enlarged and progressed to a tumefactive lesion without gadolinium enhancement. The cerebrospinal fluid (CSF) examination was normal without pleocytosis or protein elevation. Steroid pulse therapy was added; however, his symptoms did not improve. A brain stereotactic biopsy was performed and the sample showed demyelinating lesions without malignant cells. As the initial corticosteroid therapy was ineffective, gamma globulin therapy was administered in parallel with chemotherapy, and the clinical symptoms and imaging findings were partially ameliorated. TD seldom appears as a paraneoplastic neurological syndrome. In addition, there are few reports of COVID-19 vaccination-associated demyelinating disease. Clinicians should recognize paraneoplastic TD, and the further accumulation of similar cases is needed.