ABSTRACT
In thermally activated delayed fluorescence (TADF)-based organic light-emitting diodes (OLEDs), acceleration of reverse intersystem crossing (RISC) and suppression of intersystem crossing (ISC) are demanded to shorten a lifetime of triplet excitons. As a system realizing RISC faster than ISC, inverted singlet-triplet excited states (iST) with a negative energy difference (ΔEST) between the lowest excited singlet and the lowest triplet states have been gathering much attention recently. Here, we have focused on an asymmetric hexa-azaphenalene (A6AP) core to obtain a new insight into iST. Based on A6AP, we have newly designed A6AP-Cz with the calculated ΔEST of -44â meV. The experimental studies of a synthesized A6AP-Cz revealed that the lifetime of delayed fluorescence (τDF) was only 54â ns, which was the shortest among all organic materials. The rate constant of RISC (kRISC=1.9×107â s-1) was greater than that of ISC (kISC=1.0×107â s-1). The negative ΔEST of A6AP-Cz was experimentally confirmed from 1) the kRISC and kISC (-45â meV) and 2) the temperature-dependent τDF. 3) The onsets of fluorescence and phosphorescence spectra at 77â K also supported the evidence of negative ΔEST (-73â meV). This study demonstrated the potential of A6AP as an iST core for the first time.
ABSTRACT
Idiopathic normal pressure hydrocephalus in elderly people is considered a form of glymphopathy caused by malfunction of the waste clearance pathway, called the glymphatic system. Tau is a representative waste material similar to amyloid-ß. During neurodegeneration, lipocalin-type prostaglandin D synthase (L-PGDS), a major cerebrospinal fluid (CSF) protein, is reported to act as a chaperone that prevents the neurotoxic aggregation of amyloid-ß. L-PGDS is also a CSF biomarker in idiopathic normal pressure hydrocephalus and significantly correlates with tau concentration, age, and age-related brain white matter changes detected by magnetic resonance imaging. To investigate this glymphopathy, we aimed to analyze white matter changes and contributing factors in vivo and their interactions ex vivo. Cerebrospinal tap tests were performed in 60 patients referred for symptomatic ventriculomegaly. Patients were evaluated using an idiopathic normal pressure hydrocephalus grading scale, mini-mental state examination, frontal assessment battery, and timed up-and-go test. The typical morphological features of high convexity tightness and ventriculomegaly were measured using the callosal angle and Evans index, and parenchymal white matter properties were evaluated with diffusion tensor imaging followed by tract-based spatial statistics. Levels of CSF biomarkers, including tau, amyloid-ß, and L-PGDS, were determined by ELISA, and their interaction, and localization were determined using immunoprecipitation and immunohistochemical analyses. Tract-based spatial statistics for fractional anisotropy revealed clusters that positively correlated with mini-mental state examination, frontal assessment battery, and callosal angle, and clusters that negatively correlated with age, disease duration, idiopathic normal pressure hydrocephalus grading scale, Evans index, and L-PGDS. Other parameters also indicated clusters that correlated with symptoms, microstructural white matter changes, and L-PGDS. Tau co-precipitated with L-PGDS, and colocalization was confirmed in postmortem specimens of neurodegenerative disease obtained from the human Brain Bank. Our study supports the diagnostic value of L-PGDS as a surrogate marker for white matter integrity in idiopathic normal pressure hydrocephalus. These results increase our understanding of the molecular players in the glymphatic system. Moreover, this study indicates the potential utility of enhancing endogenous protective factors to maintain brain homeostasis.
ABSTRACT
PURPOSE: We aimed to investigate the diagnostic feasibility of an adjusted diffusion-weighted imaging (DWI) lexicon using multiple b values to assess breast lesions according to DWI-based breast imaging reporting and data system (BI-RADS). METHODS: This Institutional Review Board (IRB)-approved prospective study included 127 patients with suspected breast cancer. Breast MRI was performed using a 3T scanner. Breast DW images were acquired using five b-values of 0, 200, 800, 1000, and 1500 s/mm2 (5b-value DWI) on 3T MRI. Two readers independently assessed lesion characteristics and normal breast tissue using DWI alone (5b-value DWI and 2b-value DWI with b = 0 and 800 s/mm2) according to DWI-based BI-RADS and in combination with the standard dynamic contrast-enhanced images (combined MRI). Interobserver and intermethod agreements were assessed using kappa statistics. The specificity and sensitivity of lesion classification were evaluated. RESULTS: Ninety-five breast lesions (39 malignant and 56 benign) were evaluated. Interobserver agreement for lesion assessment on 5b-value DWI was very good (k ≥ 0.82) for DWI-based BI-RADS categories, lesion type, and mass characteristics; good (k = 0.75) in breast composition; and moderate (k ≥ 0.44) in background parenchymal signal (BPS) and non-mass distribution. Intermethod agreement between assessments performed using either 5b-value DWI or combined MRI was good-to-moderate (k = 0.52-0.67) for lesion type; moderate (k = 0.49-0.59) for DWI-based BI-RADS category and mass characteristics; and fair (k = 0.25-0.40) for mass shape, BPS, and breast composition. The sensitivity and positive predictive values (PPVs) for 5b-value DWI were 79.5%, 84.6% and 60.8%, 61.1% for each reader, respectively; 74.4%, 74.4% and 63.0%, 61.7% for 2b-value DWI; and 97.4%, 97.4% and 73.1%, 76.0% for combined MRI. The specificity and negative predictive values (NPVs) were 64.3%, 62.5% and 81.8%, 85.4% for 5b-value DWI; 69.6%, 67.9% and 79.6%, 79.2% for 2b-value DWI; and 75.0%, 78.6% and 97.7%, 97.8% for combined MRI. CONCLUSION: Good observer agreement was observed in the 5b-value DWI. The 5b-value DWI based on multiple b-values might have the potential to complement the 2b-value DWI; however, their diagnostic performance tended to be inferior to that of combined MRI for the characterization of breast tumors.
ABSTRACT
PURPOSE: Early detection of pancreatic ductal adenocarcinoma (PDAC) may improve the prognosis. We evaluated novel imaging findings that may contribute to early detection. METHODS: This single-center, retrospective study enrolled 37 patients with a localized main pancreatic duct (MPD) stricture and no obvious pancreatic mass. All patients underwent endoscopic retrograde cholangiopancreatography and brush sampling with cytology and serial pancreatic juice aspiration cytologic examination via endoscopic naso-pancreatic drainage. Patients with cytology-confirmed malignancy underwent surgical resection. The remaining patients were followed by contrast-enhanced computed tomography (CECT), magnetic resonance imaging (MRI), and endoscopic retrograde cholangiopancreatography. RESULTS: Twenty patients had confirmed malignancy (cancer group) and 17 did not (non-cancer group). Age, MPD stricture location, and PDAC risk factors were similar, but the sex predominance and symptom rate differed between the two groups. In the cancer group, 17 patients were diagnosed by cytology and three by clinical symptoms. CECT, MRI, and endoscopic ultrasonography (EUS) revealed no solid tumors in either group. CECT revealed no significant differences between groups. Diffusion-weighted MRI revealed significant differences in the signal intensity between groups. EUS detected indistinct and small hypoechoic areas in 70% and 41.2% of patients in the cancer and non-cancer groups, respectively. In the cancer group, 11 were diagnosed with cancer at the first indication, and nine were diagnosed at follow-up; the prognosis did not differ between these two subgroups.ss CONCLUSIONS: High signal intensity in diffusion-weighted MRI may be useful for detecting early-stage PDAC and may be an indication for surgical resection even without pathologic confirmation. CLINICAL TRIAL REGISTRATION: The study was a registered at the University Hospital Medical Information Network (UMIN000039623).
Subject(s)
Carcinoma, Pancreatic Ductal , Pancreatic Neoplasms , Carcinoma, Pancreatic Ductal/diagnostic imaging , Carcinoma, Pancreatic Ductal/surgery , Humans , Magnetic Resonance Imaging , Pancreas , Pancreatic Neoplasms/diagnostic imaging , Retrospective StudiesABSTRACT
Tremor associated with encephalitis is usually transient and rarely becomes chronic and refractory. Treatment for such tremor using deep brain stimulation (DBS) has not yet been reported. We report an uncommon case of chronic tremor after encephalitis of unknown etiology and its outcome treated with thalamic DBS. A 47-year-old man presented with a 6-month history of medically refractory tremor after non-infectious and probable autoimmune encephalitis. The patient showed an atypical mixture of resting, postural, kinetic, and intention tremor. The tremor significantly disabled the patient's activities of daily life (ADL). The patient underwent bilateral thalamic DBS surgery. DBS leads were placed to cross the border between the ventralis oralis posterior (Vop) nucleus and ventralis intermedius (Vim) nucleus of the thalamus. Stimulation of both the Vop and Vim using the bipolar contacts controlled the mixed occurrence of tremor. The ADL and performance scores on The Essential Tremor Rating Assessment Scale (TETRAS) improved from 47 to 0 and from 44 to 9, respectively. The therapeutic effects have lasted for 24 months. Administration of combined Vop and Vim DBS may control uncommon tremor of atypical etiology and phenomenology.
ABSTRACT
Morquio syndrome is a relatively rare entity that is often associated with atlantoaxial instability from early childhood due to odontoid dysplasia based on a mucopolysaccharoidal disorder. Here, we present the case of a 55-year-old male patient with Morquio syndrome who developed cervical myelopathy, which is an extremely rare condition in the older population. Myelopathy developed gradually with upper-limb paresthesia and clumsiness of both hands. The patient had a characteristic "gargoyle-like" coarse face with a trunk shortening-type short stature. Imaging of the cervical spine demonstrated several problems, including diminutive structures called platyspondyly with small pedicles and fragile bone quality, hypoplasia of the C1 posterior arch that migrated into the spinal canal, and os odontoideum with atlantoaxial instability. With intraoperative navigation guidance, posterior decompression of C1 followed by occipito-cervico-thoracic spinal fusion was successfully performed in this complicated case. Clinical and radiographic outcomes were both excellent and have been maintained for 2 years postoperatively.
Subject(s)
Cervical Vertebrae/surgery , Joint Instability/surgery , Mucopolysaccharidosis IV/surgery , Spinal Fusion/methods , Cervical Vertebrae/diagnostic imaging , Humans , Joint Instability/etiology , Magnetic Resonance Imaging , Male , Middle Aged , Mucopolysaccharidosis IV/complications , RadiographyABSTRACT
PURPOSE: Microcystic meningioma, a rare meningioma subtype, can present diagnostic difficulty. We aimed to investigate the historadiological properties of microcystic meningioma using conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI) analysis. METHODS: We retrospectively analyzed conventional MRI and DWI results of six microcystic meningioma cases by examining their appearance and determining their apparent diffusion coefficient (ADC) values. The ADC values of the intratumoral components were normalized with ADC values of the cerebrospinal fluid in the lateral ventricle (ADC ratios). As cystic formations are frequently associated with microcystic meningiomas, their MRI characteristics were compared with the imaging data from 11 cystic meningiomas of non-microcystic subtypes. RESULTS: We found that cysts in microcystic meningioma tended to have a reticular appearance on DWI, as they did on gadolinium-enhanced T1-weighted imaging. Additionally, these reticular cysts had significantly lower ADC ratios than microcystic non-reticular and non-microcystic cysts. These DWI characteristics likely reflect the histological properties of microcystic meningioma. CONCLUSION: A reticular appearance on gadolinium-enhanced T1-weighted MRI and DWI, and cyst formation with relatively low ADC values can be diagnostic markers of microcystic meningiomas.
Subject(s)
Cysts/diagnostic imaging , Diffusion Magnetic Resonance Imaging , Meningioma/diagnostic imaging , Aged , Female , Gadolinium , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective StudiesABSTRACT
Dynamic physiological and anatomical changes for delivery may adversely induce various specific non-obstetric complications during pregnancy and puerperal period. These complications can be fatal to both the mother and the fetus, thus a precise and early diagnosis ensued by an early treatment is essential. Along with ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) have assumed an increasing role in the diagnosis. This article aims to discuss the pathophysiology of these complications, the indications for CT and MRI, and the imaging findings.
ABSTRACT
AIM: To assess the use of magnetic resonance imaging (MRI) for prenatal differentiation between complete hydatidiform mole with a twin live fetus (CHMTF) and placental mesenchymal dysplasia (PMD). METHODS: Three CHMTF cases and three PMD cases, from two institutions over a 6-year period, were retrospectively included in this study. Clinical findings including age, pregnancy history, serum hCG level, ultrasonography findings, complications of the mother, outcome of the fetus, and results of chromosomal study of fetus, amniotic fluid and lesion, if possible, were noted. MRI findings were evaluated by two radiologists with respect to the location of the disease (intra- or extra-fetal sac), the presence of multicystic component, and presence of intra- or extra-lesional hemorrhage. RESULTS: In all six cases, the diseases were recognized as multicystic lesions by ultrasonography and MRI. In two of three CHMTF cases, patients continued with the pregnancy, which resulted in spontaneous abortion. In one case of CHMTF, the patient underwent artificial abortion, after which the mole progressed into an invasive mole with lung metastases. All three PMD patients had live births, and two of the three babies had fetal growth restriction. By MRI, CHMTF was located within an extra-fetal sac accompanied by intra- and/or extra-lesional hemorrhage, while PMD was located within the placenta in the fetal sac without hemorrhage. CONCLUSION: MRI could provide important information about the prenatal differential diagnosis of CHMTF and PMD, based on the pathophysiology and characteristics of the diseases.
Subject(s)
Epithelial-Mesenchymal Transition , Hydatidiform Mole/diagnosis , Placenta Diseases/diagnosis , Precancerous Conditions/diagnosis , Pregnancy Complications, Neoplastic/diagnosis , Pregnancy, Twin , Adult , Diagnosis, Differential , Female , Humans , Hydatidiform Mole/physiopathology , Japan , Magnetic Resonance Imaging , Placenta Diseases/physiopathology , Precancerous Conditions/physiopathology , Pregnancy , Pregnancy Complications, Neoplastic/physiopathology , Pregnancy Outcome , Prenatal Diagnosis , Retrospective StudiesABSTRACT
This study investigated the production of ethanol from unutilized branches pruned from pear trees by steam explosion pretreatment. Steam pressures of 25, 35, and 45 atm were applied for 5 min, followed by enzymatic saccharification of the extracted residues with cellulase (Cellic CTec2). High glucose recoveries, of 93.3, 99.7, and 87.1%, of the total sugar derived from the cellulose were obtained from water- and methanol-extracted residues after steam explosion at 25, 35, and 45 tm, respectively. These values corresponded to 34.9, 34.3, and 27.1 g of glucose per 100 g of dry steam-exploded branches. Simultaneous saccharification and fermentation experiments were done on water-extracted residues and water- and methanol-extracted residues by Kluyveromyces marxianus NBRC 1777. An overall highest theoretical ethanol yield of 76% of the total sugar derived from cellulose was achieved when 100 g/L of water- and methanol-washed residues from 35 atm-exploded pear branches was used as substrate.
Subject(s)
Carbohydrate Metabolism , Ethanol/metabolism , Fermentation , Pyrus/chemistry , Steam , Trees/chemistry , Biofuels/microbiology , Cellulase/metabolism , Hydrolysis , Kluyveromyces/metabolismABSTRACT
The feasibility of detecting axillary lymph node (LN) metastases with diffusion-weighted MR imaging was retrospectively evaluated. The relative ADC (with b values of 0 and 1000 s/mm(2)) between LNs in each axillary space was calculated (n=75). The area, the long and short diameter of the metastatic LNs were compared to those of non-metastatic LNs. The relative ADC value of metastatic LNs was significantly lower than that of non-metastatic LNs (P=.00). The long and short diameter LN diagnostic performance was superior to that of mean ADC and relative ADC (AUC: 0.84, 0.80 versus 0.64, 0.03), suggesting usefulness of diameter over ADC.
Subject(s)
Breast Neoplasms/diagnosis , Breast Neoplasms/secondary , Diagnostic Imaging , Diffusion Magnetic Resonance Imaging/methods , Lymph Nodes/pathology , Adult , Aged , Axilla , Female , Humans , Lymphatic Metastasis/diagnosis , Middle Aged , Reproducibility of Results , Retrospective StudiesABSTRACT
Massive subchorionic thrombosis is a rare condition, defined as a large thrombus confined to the subchorionic space. It is associated with poor perinatal prognosis. However, prenatal diagnosis by ultrasonography is often difficult. We report a case of massive subchorionic thrombosis developing dermatomyositis after the delivery, followed by magnetic resonance imaging. Moreover, we review other 4 cases assessed with magnetic resonance imaging. Magnetic resonance imaging is very useful for confirmation of diagnosis and follow-up in combination with ultrasonography.
Subject(s)
Hematoma/diagnosis , Magnetic Resonance Imaging/methods , Placenta Diseases/diagnosis , Prenatal Diagnosis/methods , Thrombosis/diagnosis , Adult , Cesarean Section , Chorion/diagnostic imaging , Chorion/pathology , Dermatomyositis/complications , Female , Follow-Up Studies , Hematoma/complications , Humans , Pregnancy , Severity of Illness Index , Thrombosis/complications , Ultrasonography, Doppler, Color/methods , Ultrasonography, Prenatal/methodsABSTRACT
PURPOSE: To evaluate the potential of apparent diffusion coefficients (ADCs) obtained at quantitative diffusion-weighted magnetic resonance (MR) imaging of the breast as a biomarker of low-grade ductal carcinoma in situ (DCIS). MATERIALS AND METHODS: This retrospective study was approved by an institutional review board, and the requirement to obtain informed consent was waived. Twenty-two women (age range, 36-75 years; mean age, 56.4 years) with pure DCIS (seven with low-grade DCIS, five with intermediate-grade DCIS, and seven with high-grade DCIS) and three with microinvasion underwent breast MR imaging at 1.5 T between January 2008 and November 2010. MR examinations included contrast material-enhanced (gadoteridol) T1-weighted imaging and diffusion-weighted MR imaging with b values of 0 and 1000 sec/mm(2). ADC maps were generated. The distributions of the ADCs in regions of interest covering the lesions were compared among the three grades by using linear mixed-model analysis, and the discriminatory power of the lesion minimum ADC was determined with receiver operating characteristic analysis. RESULTS: The mean ADC was 1.42 × 10(-3) mm(2)/sec (95% confidence interval [CI]: 1.31 × 10(-3) mm(2)/sec, 1.54 × 10(-3) mm(2)/sec) for low-grade DCIS, 1.23 × 10(-3) mm(2)/sec (95% CI: 1.10 × 10(-3) mm(2)/sec, 1.36 × 10(-3) mm(2)/sec) for intermediate-grade DCIS, 1.19 × 10(-3) mm(2)/sec (95% CI: 1.08 × 10(-3) mm(2)/sec, 1.30 × 10(-3) mm(2)/sec) for high-grade DCIS, and 2.06 × 10(-3) mm(2)/sec (95% CI: 1.94 × 10(-3) mm(2)/sec, 2.18 × 10(-3) mm(2)/sec) for normal breast tissue. The mean ADCs for high- and intermediate-grade DCIS were significantly lower than that for low-grade DCIS (P < .01 and P = .03, respectively), and the mean ADC for low-grade DCIS was significantly lower than that for normal tissue (P < .001). The lesion minimum ADC for low-grade DCIS was also significantly higher than that for high- and intermediate-grade DCIS (P < .01). A threshold of 1.30 × 10(-3) mm(2)/sec for the minimum ADC in the diagnosis of low-grade DCIS had a specificity of 100% (12 of 12 patients; 95% CI: 73.5%, 100%) and a positive predictive value of 100% (four of four patients; 95% CI: 39.8%, 100%). CONCLUSION: These preliminary results suggest that quantitative diffusion-weighted MR imaging could be used to identify patients with low-grade DCIS with very high specificity. If the results of this study are confirmed, this approach could potentially spare those patients from invasive approaches such as mastectomy or axillary lymph node excision.
Subject(s)
Breast Neoplasms/diagnosis , Carcinoma, Intraductal, Noninfiltrating/diagnosis , Diffusion Magnetic Resonance Imaging/methods , Adult , Aged , Biomarkers , Breast Neoplasms/pathology , Carcinoma, Intraductal, Noninfiltrating/pathology , Contrast Media , Female , Gadolinium , Heterocyclic Compounds , Humans , Image Interpretation, Computer-Assisted , Linear Models , Middle Aged , Organometallic Compounds , ROC Curve , Reproducibility of Results , Retrospective StudiesABSTRACT
BACKGROUND: Preoperative diagnosis of lymph node metastasis is important in determining the optimal therapy for rectal cancer. It has been shown that diffusion-weighted magnetic resonance imaging (DWI) is a useful tool for detecting malignant tumors. METHODS: One hundred twenty-nine consecutive patients with rectal cancer were examined with DWI + conventional (T1-weighted and T2-weighted) MRI and computed tomography (CT). All 129 patients underwent rectal resection with total mesorectal excision. Findings on DWI + conventional MRI and CT were compared with those from histopathologic examinations. RESULTS: Fifty-nine (46%) patients had metastatic lymph nodes on histopathologic examinations. Two hundred twenty (18%) of 1,250 lymph nodes were pathologically positive for tumor metastasis. The overall patient-based sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of DWI + conventional MRI were 93, 81, 81, 93, and 87%, respectively. Corresponding values of CT were 73, 79, 74, 77, and 76%, respectively. The overall node-based sensitivity, specificity, PPV, NPV, and accuracy of DWI + conventional MRI were 97, 81, 52, 99, and 84%, respectively. Corresponding values of CT were 86, 80, 48, 96, and 81%, respectively. CONCLUSION: DWI + conventional MRI is effective for the detection of lymph node metastasis and useful for selection of the optimal therapy for rectal cancer.
Subject(s)
Adenocarcinoma/diagnosis , Adenocarcinoma/secondary , Diffusion Magnetic Resonance Imaging/methods , Lymph Nodes/pathology , Neoadjuvant Therapy , Neoplasm Recurrence, Local/mortality , Rectal Neoplasms/diagnosis , Adenocarcinoma/mortality , Adenocarcinoma/therapy , Aged , Aged, 80 and over , Biopsy, Needle , Cohort Studies , Colectomy/adverse effects , Colectomy/methods , Colectomy/mortality , Disease-Free Survival , Female , Humans , Japan , Lymphatic Metastasis , Male , Middle Aged , Neoplasm Recurrence, Local/pathology , Neoplasm Staging , Predictive Value of Tests , Preoperative Care/methods , Prognosis , Rectal Neoplasms/mortality , Rectal Neoplasms/therapy , Risk Assessment , Survival Rate , Tomography, X-Ray Computed/methodsABSTRACT
OBJECT: The prognosis of recurrent glioblastoma multiforme (GBM) remains unsatisfactory. The authors conducted a Phase II study of ifosfamide, carboplatin, and etoposide (ICE) for a first recurrence of GBM to determine whether it prolonged a patient's good-quality life. METHODS: This trial was an open-label, single-center Phase II study. Forty-two patients with a first GBM relapse after surgery followed by standard radiotherapy (60 Gy) and first-line temozolomide- or nimustine-based chemotherapy were eligible to participate. The primary end point was progression-free survival at 6 months after the ICE treatment (PFS-6), and secondary end points were response rate, toxicity, and overall survival. Chemotherapy consisted of ifosfamide (1000 mg/m(2) on Days 1, 2, and 3), carboplatin (110 mg/m(2) on Day 1), etoposide (100 mg/m(2) on Days 1, 2, and 3), every 6 weeks. RESULTS: Progression-free survival at 6 months after ICE treatment was 35% (95% CI 22-50%). The median duration of PFS was 17 weeks (95% CI 10-24 weeks). The response rate was 25% (95% CI 9-34%). Adverse events were generally mild and consisted mainly of alopecia. CONCLUSIONS: This regimen was well tolerated and has some activity and could be one of the options for patients with recurrent GBM.
Subject(s)
Antineoplastic Agents/therapeutic use , Antineoplastic Combined Chemotherapy Protocols , Brain Neoplasms/drug therapy , Carboplatin/therapeutic use , Etoposide/therapeutic use , Glioblastoma/drug therapy , Ifosfamide/therapeutic use , Neoplasm Recurrence, Local/drug therapy , Adult , Aged , Antineoplastic Agents/adverse effects , Carboplatin/administration & dosage , Carboplatin/adverse effects , Disease Progression , Etoposide/administration & dosage , Etoposide/adverse effects , Female , Glioblastoma/therapy , Humans , Ifosfamide/administration & dosage , Ifosfamide/adverse effects , Kaplan-Meier Estimate , Male , Middle Aged , Quality of Life , Time Factors , Treatment Outcome , Young AdultABSTRACT
We report on a preterm Japanese male baby with Leigh syndrome, i.e., intrauterine growth restriction, central apnea, and feeding difficulty. These signs improved at 41 weeks of corrected age. At that time, brain magnetic resonance imaging revealed increased signal in diffusion-weighted imaging in the parietal white matter, bilaterally and symmetrically not respecting vascular territory or boundaries. However, clinical improvement deterred us from further investigation. About 3 months later, he manifested frequent ictal apnea with myoclonic seizures and deterioration of consciousness to semicoma. Subsequent diffusion-weighted imaging revealed increased signal in the bilateral symmetric thalamus, internal segments of the globus pallidus, substantia nigra, and pontine tegmentum. Laboratory investigation indicated remarkable elevation of lactate levels in cerebrospinal fluid. The diagnosis was of Leigh syndrome. We think this is the first reported case of Leigh encephalopathy with transient abnormality of diffusion-weighted imaging of the white matter before apparent clinical onset. Leigh syndrome should be included in the differential diagnosis of abnormality of diffusion-weighted imaging in white matter without apparent clinical signs.
Subject(s)
Brain/pathology , Leigh Disease/pathology , Apnea/cerebrospinal fluid , Apnea/diagnosis , Apnea/pathology , Child, Preschool , Consciousness Disorders/cerebrospinal fluid , Consciousness Disorders/diagnosis , Consciousness Disorders/pathology , DNA Mutational Analysis , Diagnosis, Differential , Diffusion Magnetic Resonance Imaging , Disease Progression , Epilepsies, Myoclonic/cerebrospinal fluid , Epilepsies, Myoclonic/diagnosis , Epilepsies, Myoclonic/pathology , Humans , Infant , Leigh Disease/cerebrospinal fluid , Leigh Disease/diagnosis , Male , ParentsABSTRACT
OBJECT: Accurate localization of the subthalamic nucleus (STN) is important for proper placement of the electrodes in deep brain stimulation (DBS) surgery for patients with advanced Parkinson disease. The authors evaluated the accuracy of our modified composite targeting method and the value of using high-field MR imaging for targeting the STN. METHODS: Thirteen patients with advanced Parkinson disease underwent bilateral STN DBS based on 3-T MR imaging, and 13 patients underwent surgery based on 1.5-T MR imaging. By sequentially referring to the postmammillary commissure, the red nucleus, the mammillothalamic tract, and the STN, the modified composite targeting method determined the stereotactic coordinates for targeting the STN. The accuracy and efficacy of the composite targeting method and 3-T MR imaging were evaluated by using the intraoperative microelectrode recording, the postoperative imaging study, and the postoperative clinical improvement. RESULTS: The landmark structures for targeting the STN were visualized clearly with 3-T MR imaging. The mean (+/- SD) path length through the STN of the central track was 4.9 +/- 1.1 mm in the 3-T group and 3.1 +/- 2.0 mm in the 1.5-T group (p < 0.001). Twenty-one (81%) of 26 electrodes were placed in the central track in the 3-T group, whereas 8 (31%) of 26 electrodes were placed in the central track in the 1.5-T group (p = 0.006). The rest of the electrodes were placed in the noncentral optimum track for alleviating parkinsonian motor symptoms. The mean Unified Parkinson's Disease Rating Scale motor part score during off period was reduced by 53% in the 3-T group and by 41% in the 1.5-T group (p = 0.14). The mean reductions of levodopa equivalent daily doses were 48.6% in the 3-T group and 43.7% in the 1.5-T group (p = 0.61). CONCLUSIONS: The use of the modified composite targeting method referring to the multiple landmarks with 3-T MR imaging offers reliable and clinically effective target for STN DBS surgery.
Subject(s)
Deep Brain Stimulation , Magnetic Resonance Imaging/methods , Parkinson Disease/therapy , Subthalamic Nucleus/pathology , Adult , Aged , Humans , Microelectrodes , Middle Aged , Parkinson Disease/pathology , Stereotaxic TechniquesABSTRACT
We report a case of spontaneous remission of a massive CNS lesion with eosinophilic infiltrate. This 69-year-old man had eosinophilia without any systemic disorder or laboratory evidence of the most common causes of hypereosinophilia. MRI of the brain suggested an infiltrating neoplasm, but histological examination of a needle biopsy specimen failed to show evidence of a neoplasm. Instead, the tissue demonstrated rarefaction and gliosis with striking perivascular and parenchymal infiltrates of eosinophils. The MRI abnormality and eosinophilia disappeared spontaneously. We speculate that eosinophil-derived neurotoxins might have been the cause of the transient CNS disorder observed in this patient.
Subject(s)
Encephalitis/diagnosis , Encephalitis/etiology , Eosinophilia/complications , Aged , Eosinophilia/pathology , Humans , Magnetic Resonance Imaging , Male , Remission, SpontaneousABSTRACT
Hydroxymethylglutathione (gamma-L-glutamyl-L-cysteinyl-L-serine; hmGSH) occurs in many species belonging to the family Gramineae, but the biosynthetic pathway for hmGSH has not been identified. We found that carboxypeptidase Y (CPY), but not carboxypeptidase A, catalyzed hmGSH synthesis from glutathione and L-serine in vitro at acidic pH. CPY also catalyzed methylglutathione synthesis from glutathione and L-alanine. These findings suggested that a carboxypeptidase-like enzyme may be involved in hmGSH synthesis in vivo.
Subject(s)
Carboxypeptidases/metabolism , Glutathione/analogs & derivatives , Glutathione/biosynthesis , Glutathione/metabolism , Serine/metabolism , Alanine/metabolism , Catalysis , Cathepsin A , Chromatography, High Pressure Liquid , Hydrogen-Ion Concentration , Hydroxylation , Serine/chemistry , Yeasts/enzymologyABSTRACT
BACKGROUND: Until recently, it has been impossible to demonstrate vascular compression at the root entry or exit zone (REZ) of the trigeminal nerve and facial nerve in patients with trigeminal neuralgia (TN) and hemifacial spasm (HFS) preoperatively, although surgical findings have revealed apparent neurovascular compression and its correction has resulted in a good outcome in most cases. Revealing the anatomic correlation between nerves and vessels at the REZ preoperatively would be useful to predict operative findings. METHODS: To assess whether the vascular contact of the nerve at the REZ could be demonstrated preoperatively, high-resolution magnetic resonance tomographic angiography (MRTA) was performed in 21 patients with TN and 39 with HFS. Neuroradiological findings were compared with the operative findings in all patients. Contralateral asymptomatic nerves were evaluated as a control. RESULTS: MRTA correctly identified offending vessels in 14 (67%) of the 21 TN and 34 (87%) of the 39 HFS patients. Failure to identify neurovascular contact was noted in the cases with compression by veins or small arteries, thickened arachnoid, or distal compression. Neurovascular contact was also observed in 15% of the asymptomatic nerves. The deformity of the nerve seemed to be a more important factor for determining operative indication. CONCLUSIONS: MRTA could demonstrate offending vessels in TN and HFS at a high rate and was useful to predict operative findings. MRTA gave supportive evidence of surgical indications in patients with TN and HFS, although attention should be paid to the fact that MRTA did not necessarily detect all of the offending vessels.