Carotid Endarterectomy With Shunt-A Stepwise Surgical Technique Demonstration for Trainees: 2-Dimensional Operative Video.

Carotid endarterectomy (CEA) is a common cerebrovascular surgery and is an effective treatment option for patients with carotid stenosis.1-4 Although the routine or selective use of a shunt is still debatable,5 the CEA technique is generally well-established. We believe that bloodless and "shallow and wide" operative fields make CEA safe and successful. Maintaining a bloodless operative field is highly crucial to prevent postoperative cervical hematoma and damage to anatomic structures, such as the hypoglossal and vagus nerves, because it facilitates their identification. In the cases of CEA, antiplatelet medications are usually continued, and systemic heparinization is performed intraoperatively; therefore, further meticulous hemostasis should be performed than in other neurological surgery cases. The 2-step pull-up technique, which involves pulling up loose connective tissue surrounding the carotid arteries in addition to the carotid sheath, makes the operative field "shallow and wide." This technique allows the internal carotid artery to move from deep to superficial, making CEA feasible, particularly when placing a shunt. This video illustrates the CEA technique used for symptomatic mild carotid stenosis in a 66-year-old man with vulnerable plaques. Evolving carotid artery stenting should facilitate the improvement of the operative technique to increase the safety and accordingly train young surgeons. This video is intended to increase familiarity with CEA because carotid artery stenting decreases CEA indications for carotid stenosis and diminishes proficiency in managing CEA. The patient consented to the publication of his image. Patient consent was obtained to perform the surgery and publish the surgical video.

A pilot study of half-value layer measurements using a semiconductor dosimeter for intraoral radiography.

Purpose: This pilot study was conducted to evaluate half-value layer (HVL) measurements obtained using a semiconductor dosimeter for intraoral radiography. Materials and Methods: This study included 8 aluminum plates, 4 of which were low-purity (less than 99.9%) and 4 high-purity (greater than 99.9%). Intraoral radiography was performed using an intraoral X-ray unit in accordance with the dental protocol at the authors' affiliated hospital: tube voltage, 60 kVp and 70 kVp; tube current, 7 mA; and exposure time, 0.10 s. The accuracy of HVL measurements for intraoral radiography was assessed using a semiconductor dosimeter. A simple regression analysis was performed to compare the aluminum plate thickness and HVL in relation to the tube voltage (60 kVp and 70 kVp) and aluminum purity (low and high). Results: For the low-purity aluminum plates, the HVL at 60 kVp (Y) and 70 kVp (Y) was significantly correlated with the thickness of the aluminum plate (X), with Y = 1.708 + 0.415X (r=0.999, P<0.05) and Y = 1.980 + 0.484X (r=0.999, P<0.05), respectively. Similarly, for the high-purity aluminum plates, the HVL at 60 kVp (Y) and 70 kVp (Y) was significantly correlated with the plate thickness (X), with Y = 1.696 + 0.454X (r=0.999, P<0.05) and Y = 1.968 + 0.515X (r=0.998, P<0.05), respectively. Conclusion: This pilot study examined the relationship between aluminum plate thickness and HVL measurements using a semiconductor dosimeter for intraoral radiography. Semiconductor dosimeters may prove useful in HVL measurement for purposes such as quality assurance in dental X-ray imaging.

Accuracy of non-contact semiconductor X-ray analyzer for quality assurance in intraoral radiography: a comparison with ionization chamber dosimeter.

OBJECTIVES: The aim of the present study was to evaluate the accuracy of a non-contact semiconductor X-ray analyzer for quality assurance in intraoral radiography, especially a comparison with an ionization chamber dosimeter. METHODS: Intraoral radiography was performed with intraoral X-ray unit using the dental protocol at our hospital: tube voltage, 70 kV; tube current, 7 mA. Accuracy of dose and half-value layer (HVL) measurements was analyzed with a non-contact semiconductor X-ray analyzer and an ionization chamber dosimeter. Stability of the semiconductor sensor, effect of scattered radiation, and comparison of measured HVL between the ionization chamber and the semiconductor sensor were analyzed in this study. RESULTS: The values with the semiconductor sensor were tube voltage: 70.3 ± 0.2 kVp (degree of variability: 0.28%), dose: 454.1 ± 12.3 µGy (degree of variability: 2.7%), and HVL: 1.91 ± 0.02 mmAl (degree of variability: 1.0%). With collimator, the dose with the semiconductor sensor and the ionization chamber decreased by 2.3 µ Gy and 5.2 µ Gy, respectively. The measured HVL of the semiconductor dosimeter was more than that of ionization chamber, and the semiconductor dosimeter was less than ionization chamber in variation of between without and with collimator. CONCLUSION: This study indicated the accuracy of a non-contact semiconductor X-ray analyzer for quality assurance in intraoral radiography, especially a comparison with an ionization chamber dosimeter. The semiconductor sensor can be useful for quality assurance in intraoral radiography.

Midline Suboccipital Unilateral Trans-Cerebellomedullary Fissure Approach for Clipping of Ruptured VA-PICA Aneurysm: Two-Dimensional Operative Video.

The vertebral artery-posterior inferior cerebellar artery (VA-PICA) aneurysm poses a technical challenge for microsurgical clipping due to its anatomical complexity, which requires dissection of lower cranial nerves. Endovascular treatment is regarded as a feasible first-line therapeutic option for VA-PICA aneurysm because it has an acceptable aneurysm occlusion rate and is less invasive. However, microsurgical clipping remains an effective treatment option. We present the case of a 62-year-old man who presented with subarachnoid hemorrhage (SAH) due to a ruptured VA-PICA aneurysm. Neuroradiologic examination revealed a 2-3 mm medially pointing left VA-PICA aneurysm with acute obstructive hydrocephalus due to massive SAH in the posterior cranial fossa. As the patient had acute obstructive hydrocephalus and a relatively small aneurysm, we selected clipping over endovascular treatment. Because the aneurysm was located close to the midline and anterolateral to the medulla oblongata, we approached it from the midline. A midline suboccipital craniotomy, C1 laminectomy, and drilling of the left condylar fossa were performed; a unilateral cerebellomedullary fissure opening was added; and the aneurysm was clipped. Postoperative neuroradiologic examinations revealed complete obliteration of the aneurysm. As shown in this video, unilateral cerebellomedullary fissure opening combined with adequate removal of the condylar fossa provides a wide operative field in the cerebellomedullary cistern while avoiding strong retraction of the cerebellum. We believe that this technique makes VA-PICA aneurysm clipping safe and successful. Patient consent was obtained to perform the surgery and to publish the surgical video (Video 1).

Development of an oral mucosal irritation test using a three-dimensional human buccal oral mucosal model.

The oral mucosa can become irritated by oral care products and lip cosmetics. Therefore, it is important to determine the irritation potential of their ingredients and products during safety evaluations. We developed a method for oral mucosal irritation test using EpiOral, which is a three-dimensional cultured model. Exposure of sodium lauryl sulphate (SLS) to EpiOral showed a dose-dependent decrease in cell viability. Under 120 min exposure conditions, SLS irritation was detected when 60% cell viability was set as a criterion. Evaluation of the irritancy of SLS and four other raw materials used in oral products at three laboratories under the above conditions confirmed good transferability of the test. Focused on the similarity of the oral and eye mucous, 32 chemicals categorised by the UN-GHS eye-irritation classification were evaluated to ensure the reliability of our criteria at these laboratories. The concordance rate between the UN-GHS classification and our test results was 100% for irritants and 60% for non-irritants. The good intra-laboratory reproducibility of our test was confirmed from the evaluation results of negative and positive controls, and the good inter-laboratory reproducibility was confirmed from the results of 32 chemicals. These findings showed that oral mucosal irritation can be evaluated using EpiOral.

Efficacy of combined use of a stent retriever and aspiration catheter in mechanical thrombectomy for acute ischemic stroke.

BACKGROUND: The efficacy of combined stent retriever (SR) and aspiration catheter (AC; combined technique: CBT) use for acute ischemic stroke (AIS) is unclear. We investigated the safety and efficacy of single-unit CBT (SCBT)-retrieving the thrombus as a single unit with SR and AC into the guide catheter-compared with single use of either SR or contact aspiration (CA). METHODS: We analysed 763 consecutive patients who underwent mechanical thrombectomy for AIS between January 2013 and January 2020, at six comprehensive stroke centers. Patients were divided into SCBT and single device (SR/CA) groups. The successful recanalization with first pass (SRFP) and other procedural outcomes were compared between groups. RESULTS: Overall, 240 SCBT and 301 SR/CA (SR 128, CA 173) patients were analyzed. SRFP (modified Thrombolysis In Cerebral Infarction (mTICI) ≥2c, 43.3% vs 27.9%, p<0.001; mTICI 3, 35.8% vs 25.5%, p=0.009) and final mTICI ≥2b recanalization (89.1% vs 82.0%, p=0.020) rates were significantly higher, puncture-to-reperfusion time was shorter (median (IQR) 43 (31.5-69) vs 55 (38-82.2) min, p<0.001), and the number of passes were fewer (mean±SD 1.72±0.92 vs 1.99±1.01, p<0.001) in the SCBT group. Procedural complications were similar between the groups. In subgroup analysis, SCBT was more effective in women, cardioembolic stroke patients, and internal carotid artery and M2 occlusions. CONCLUSIONS: SCBT increases the SRFP rate and shortens the puncture-to-reperfusion time without increasing procedural complications.

Effect of Moisture Distribution on Velocity and Waveform of Ultrasonic-Wave Propagation in Mortar.

Considering that the ultrasonic method is applied for the quality evaluation of concrete, this study experimentally and numerically investigates the effect of inhomogeneity caused by changes in the moisture content of concrete on ultrasonic wave propagation. The experimental results demonstrate that the propagation velocity and amplitude of the ultrasonic wave vary for different moisture content distributions in the specimens. In the analytical study, the characteristics obtained experimentally are reproduced by modeling a system in which the moisture content varies between the surface layer and interior of concrete.

Validation of Addenbrooke's cognitive examination III for detecting mild cognitive impairment and dementia in Japan.

BACKGROUND: Early detection of mild cognitive impairment (MCI) and dementia is very important to begin appropriate treatment promptly and to prevent disease exacerbation. We investigated the screening accuracy of the Japanese version of Addenbrooke's Cognitive Examination III (ACE-III) to diagnose MCI and dementia. METHODS: The original ACE-III was translated and adapted to Japanese. It was then administered to a Japanese population. The Hasegawa Dementia Scale-revised (HDS-R) and Mini-mental State Examination (MMSE) were also applied to evaluate cognitive dysfunction. In total, 389 subjects (dementia = 178, MCI = 137, controls = 73) took part in our study. RESULTS: The optimal ACE-III cut-off scores to detect MCI and dementia were 88/89 (sensitivity 0.77, specificity 0.92) and 75/76 (sensitivity 0.82, specificity 0.90), respectively. ACE-III was superior to HDS-R and MMSE in the detection of MCI or dementia. The internal consistency, test-retest reliability, and inter-rater reliability of ACE-III were excellent. CONCLUSIONS: ACE-III is a useful cognitive test to detect MCI and dementia. ACE-III may be widely useful in clinical practice.

[Measurement of the Dose Rate Using Dosimeters in Interventional Radiology and Its Difficulty].

In equipment used for interventional radiology (IVR), automatic exposure control (AEC) is incorporated to obtain the X-ray output suitable for the treatment of targeted lesions. For the AEC, users select a region as the signal sensing region (measuring field, MF) in the flat panel detector; MFs with various sizes and shapes were pre-defined and prepared in the system. The aim of this study was to examine the change of measured dose rate with the selection of MFs, the type of dosimeters (the ionization chamber dosimeter and the semiconductor dosimeter), and the dosimeter placement relative to the direction of X-ray tube (from cathode to anode). The IVR equipment was Allura Xper FD20/10 (Philips Medical Systems), and six kinds of built-in MFs were used. It was found that dose rate measured by the ionization chamber dosimeter showed a variation of -2 mGy/min with the MFs and the ionization chamber dosimeter placement. The dose rate measured by the semiconductor dosimeter showed more variation than the ionization chamber dosimeter. The change of dose rate with the dosimeter placement would be caused by the MF overlapping the dosimeter which would affect the AEC (the X-ray output). Also, the change of dose rate with the dosimeter placement was considered to be related to the heel effect of the X-ray beam. When performing dose rate measurements, we should notice that the selection of MFs, the type of dosimeters, and the dosimeter placement would affect the measured values.

Intracranial tuberculoma in non-immunosuppressive state.

The rise in the incidence of tuberculosis is generally related to human immunodeficiency virus infection. However, intracranial tuberculoma, a complication of tuberculosis considered to be a critical disease, can develop even in the absence of immunosuppressive state. Here, we describe 2 cases of intracranial tuberculoma occurring in patients with no evidence of immunosuppressive state or past history of tuberculosis. In Case 1, lesions were observed in the right lateral ventricle, with histological examination revealing granulomatous lesions. In Case 2, scattered lesions were observed in the cranium and the lung fields. In both cases, the QuantiFERON Test (QFT) was positive, and improvements were observed in the symptoms following administration of antituberculous drugs. Intracranial tuberculoma cannot be considered rare, and needs to be included in the differential diagnosis of intracranial lesions. Diagnosis can be tricky since this disease can develop in a patient in a non-immunosuppressive state or without a past history of tuberculosis. The QFT is an effective test to enable the diagnosis of tuberculomas in atypical patients.

Abuse of people with cognitive impairment by family caregivers in Japan (a cross-sectional study).

Caregiving is often stressful in an aging society. Our research questions were two. First, In Japan, how often does abusive behavior by the caregivers of elders with clinically mild cognitive dysfunction (mild cognitive impairment and clinically mild dementia) occur? Second, what risk factors affect the abusive behavior? We studied 123 Japanese caregivers and care recipients who had been referred to the Memory Clinic at Okayama University Hospital. We used the Japanese version of the modified Conflict Tactics Scale (m-CTS) to measure abusive behaviors. We estimate the prevalence of abusive behavior meeting threshold (m-CTS score of 2 or higher) for abuse case on the modified m-CTS. The prevalence of abusive behavior was 15.4%. Stepwise multiple regression analysis revealed that the sex of caregivers and scores on the Neuropsychiatric Inventory (NPI), Zarit Caregiver Burden scale, and Addenbrooke's Cognitive Examination had significant effects on the m-CTS scores. We demonstrated that in Japan, caregivers of the elderly with even clinically mild cognitive dysfunction exhibit abusive behavior toward them. The severity of the disease might reflect the prevalence of and factors that affect the abusive conflict score.

Cilostazol reduces periprocedural hemodynamic depression in carotid artery stenting.

The clinical effects of two different types of antiplatelet drugs, cilostazol and thienopyridine drugs, were compared in patients treated by carotid artery stenting (CAS). Two hundred patients scheduled for CAS were randomized to either cilostazol or a thienopyridine drug (ticlopidine or clopidogrel). The study was conducted in open-label design. Aspirin was also given to all patients. All episodes of periprocedural hemodynamic instability (bradycardia, hypotension) were recorded together with all instances of stroke, cardiac morbidity, and death within 30 days of the procedure. Angiographic follow-up studies were conducted about 6 months after CAS. Finally, 197 patients were enrolled in this study; 97 were treated with cilostazol (cilostazol group) and 100 with a thienopyridine drug (thienopyridine group). In the 30-day follow-up period, the incidence of stroke, cardiac adverse effects, and death was not significantly different between the 2 groups (cilostazol group 7.2%, thienopyridine group 11.0%; p = 0.85). The incidence of intra- and postprocedural bradycardia was significantly lower in the cilostazol group (cilostazol group 18.6% and 2.1%, thienopyridine group 40.0% and 18.0%, respectively; p < 0.01). Although the incidence of intraprocedural hypotension did not significantly differ between the 2 groups, postprocedural hypotension was significantly lower in the cilostazol group (16.5% vs. 34.0%, p < 0.01). In-stent restenosis on follow-up angiograms was lower in the cilostazol group but not significantly (0% vs. 4.4%, p = 0.12). This small open-label study shows that cilostazol may reduce periprocedural bradycardia and hypotension compared with thienopyridine drugs in patients treated by CAS.

Histological examination of expanded polytetrafluoroethylene artificial dura mater at 14 years after craniotomy: case report.

Expanded polytetrafluoroethylene (ePTFE) porous material (GORE(®) PRECLUDE(®) Dura Substitute) does not degenerate or deteriorate in vivo, and is currently used as artificial dura mater. This material does not adhere well to the surrounding tissues, but cerebrospinal fluid leakage along the suture line has been observed in several cases. We describe a case of craniotomy for tumor resection performed 14 years after dural repair with ePTFE sheet. Histological examination of the ePTFE sheet revealed that the sheet was structurally intact, with no evidence of tissue adhesion or cellular infiltration. However, collagen deposition was observed around the suture thread. When the suture thread was removed the collagen was also removed, and the original needle hole appeared again. No significant changes were observed in the features of the ePTFE sheet even 14 years postoperatively. The formation of fibrous tissue around the needle hole was important in preventing cerebrospinal fluid leakage.

Intraprocedural prediction of hemorrhagic cerebral hyperperfusion syndrome after carotid artery stenting.

Hyperperfusion syndrome (HPS) is a rare but severe complication after carotid artery stenting (CAS). Reliable methods for predicting HPS remain to be developed. We aimed to establish a predictive value of hemorrhagic HPS after CAS. Our retrospective study included 136 consecutive patients who had undergone CAS. We determined the cerebral circulation time (CCT) by measuring the interval between the point of maximal opacification of the terminal portion of the internal carotid artery and the cortical vein. We calculated intraprocedural CCT changes (ΔCCT) by subtracting postprocedural CCT values from preprocedural CCT values. The mean ΔCCT was 0.9 ± 0.9 seconds; 3 patients (2.2%) with prolonged ΔCCT (2.7, 5.4, and 5.8 seconds) developed HPS. The cutoff time of 2.7 seconds predicted hemorrhagic HPS retrospectively with 100% sensitivity and 99% specificity. Our findings suggest that post-CAS HPS can be predicted by using the ΔCCT value obtained by intraprocedural digital subtraction angiography. Patients with a ΔCCT >2.7 seconds require careful intensive hemodynamic and neurologic monitoring after CAS.

Validation of the revised Addenbrooke's Cognitive Examination (ACE-R) for detecting mild cognitive impairment and dementia in a Japanese population.

BACKGROUND: Early detection of dementia will be important for implementation of disease-modifying treatments in the near future. We aimed to investigate the diagnostic validity and reliability of the Japanese version of the revised Addenbrooke's Cognitive Examination (ACE-R J) for identifying mild cognitive impairment (MCI) and dementia. METHODS: We translated and adapted the original ACE-R for use with a Japanese population. Standard tests for evaluating cognitive decline and dementing disorders were applied. A total of 242 subjects (controls = 73, MCI = 39, dementia = 130) participated in this study. RESULTS: The optimal cut-off scores of ACE-R J for detecting MCI and dementia were 88/89 (sensitivity 0.87, specificity 0.92) and 82/83 (sensitivity 0.99, specificity 0.99) respectively. ACE-R J was superior to the Mini-Mental State Examination in the detection of MCI (area under the curve (AUC): 0.952 vs. 0.868), while the accuracy of the two instruments did not differ significantly in identifying dementia (AUC: 0.999 vs. 0.993). The inter-rater reliability (ICC = 0.999), test-retest reliability (ICC = 0.883), and internal consistency (Cronbach's α = 0.903) of ACE-R J were excellent. CONCLUSION: ACE-R J proved to be an accurate cognitive instrument for detecting MCI and mild dementia. Further neuropsychological evaluation is required for the differential diagnosis of dementia subtypes.

Angiographically confirmed stent over expansion in the internal carotid artery during stenting: incidence, predictors, and outcomes.

INTRODUCTION: Selection of the appropriate diameter of stent is difficult in patients with the size mismatch between the internal carotid artery (ICA) and the common carotid artery (CCA). Although stent overexpansion (SOE) in the ICA after carotid artery stenting (CAS) is suspected of producing restenosis, SOE has not been well established. We discuss its incidence, predictors, and outcomes. METHODS: We retrospectively reviewed follow-up angiographs of 206 CAS-treated arteries in 201 patients who had undergone CAS. SOE was defined as angiographic evidence of an intimal gap between the non-stented normal and the dilated stented ICA at the distal stent edge. We also collected data on the patients' clinical status, comorbidities, and radiological and procedural data. Patients with SOE were further followed up closely by duplex ultrasound scans. RESULTS: SOE was detected in nine of 206 CAS-treated ICAs (4.4%). Univariate analysis revealed a significant association between SOE and open-cell stents, the stent diameter (p < 0.01), pre-procedural stenosis, the ICA diameter, ICA/CCA ratio, and the ICA/stent ratio (p < 0.05). Entering these variables into a logistic regression model, open-cell stents were the only variable that significantly increased the risk for SOE (OR 2.36; 95% CI 0.99-4.60; p < 0.05). During a mean clinical follow-up of 31.1 months (range 24-39 months), none of the patients with SOE developed new neurologic ischemic symptoms, stent-edge stenosis, or in-stent restenosis. CONCLUSION: SOE after CAS was not associated with clinical adverse effects. This study suggests that the diameter of stent should be determined by reference to the CCA diameter without respect to the ICA diameter.

Creutzfeldt-Jakob disease with the M232R mutation in the prion protein gene in two cases showing different disease courses: a clinicopathological study.

We report two autopsy cases of Creutzfeldt-Jakob disease (CJD) with the M232R mutation of the prion protein (PrP) gene that exhibited different clinicopathological features (age at death, 64/54 years; disease duration, 13/26 months). Both cases showed myoclonus, hyperintensity on diffusion-weighted MRI, and increased 14-3-3 protein in the cerebrospinal fluid. The initial sign in each case was memory disturbance and abnormal pharyngeal sensation, respectively. In the first case, the disease progressed rapidly with akinetic mutism developing 6 months after onset, while it occurred 23 months after onset in the second case. Pathologically, both cases had severe neuronal loss with gliosis and spongiform change in the cerebral cortex, basal ganglia, and cerebellum. PrP deposition was the diffuse synaptic type in the first case, but the second case had both diffuse synaptic and perivacuolar types. PrP(sc) immunoblotting revealed a type 1 band pattern in the first case, but both types 1 and 2 in the second case. Based on these findings, together with the results in previous CJD cases with M232R, we noted the possibility that the presence of type 2 PrP(sc) may be associated with both morphological features of PrP deposition and slow disease progression in this genetic prion disease.

Early ceasing of intra-aneurysmal contrast opacification during coil embolization in ruptured aneurysms compared with unruptured aneurysms.

BACKGROUND: The difference between coil-embolized ruptured and unruptured aneurysms with respect to intra-aneurysmal thrombus formation remains to be determined. OBJECTIVE: We examined whether there was a difference between ruptured and unruptured coil-embolized aneurysms in the rate and timing of thrombus formation in the aneurysmal sac and discuss the effect of thrombus on the treatment outcome. METHODS: We evaluated 209 aneurysms with an aneurysmal dome smaller than 10 mm and a neck size less than 4 mm. Of these, 91 (43.5%) were ruptured. We assessed intra-aneurysmal thrombus formation by the coil-packing ratio (CPR): the percentage of coil volume occupying the aneurysmal sac. The initial CPR was defined as the CPR at which contrast influx into the sac ceased and the final CPR as that at the end of the procedure. ΔCPR was calculated as the difference between initial and final CPRs. Embolized aneurysms were evaluated on follow-up angiograms. RESULTS: The initial CPR was significantly lower in ruptured aneurysms (P < .01), and there was not a significant difference in the final CPR between ruptured and unruptured aneurysms (P = .05). ΔCPR was significantly higher in ruptured aneurysms (P < .01). The rate of aneurysmal recanalization was significantly higher in ruptured aneurysms (P < .05). The incidence of recanalization was high in ruptured aneurysms with low initial CPR and ΔCPR values. CONCLUSION: In ruptured aneurysms, intra-aneurysmal thrombus formation tends to occur in the earlier stages of coil embolization. In some cases, thrombus formation may inhibit dense coil packing and result in recanalization.

Coexistence of TDP-43 and tau pathology in neurodegeneration with brain iron accumulation type 1 (NBIA-1, formerly Hallervorden-Spatz syndrome).

We report here an autopsy case of sporadic adult-onset Hallervorden-Spatz syndrome, also known as neurodegeneration with brain iron accumulation type 1 (NBIA1), without hereditary burden. A 49-year-old woman died after a 27-year disease course. At the age of 22, she suffered from akinesia, resting tremor, and rigidity. At the age of 28, she was admitted to our hospital because of worsening parkinsonism and dementia. Within several years, she developed akinetic mutism. At the age of 49, she died of bleeding from a tracheostomy. Autopsy revealed a severely atrophic brain weighing 460 g. Histologically, there were iron deposits in the globus pallidus and substantia nigra pars reticulata, and numerous axonal spheroids in the subthalamic nuclei. Neurofibrillary tangles were abundant in the hippocampus, cerebral neocortex, basal ganglia, and brain stem. Neuritic plaques and amyloid deposits were absent. Lewy bodies and Lewy neurites, which are immunolabeled by anti-α-synuclein, were absent. We also observed the presence of TDP-43-positive neuronal perinuclear cytoplasmic inclusions, with variable frequency in the dentate gyrus granular cells, frontal and temporal cortices, and basal ganglia. TDP-43-positive glial cytoplasmic inclusions were also found with variable frequency in the frontal and temporal lobes and basal ganglia. The present case was diagnosed with adult-onset NBIA-1 with typical histological findings in the basal ganglia and brainstem. However, in this case, tau and TDP-43 pathology was exceedingly more abundant than α-synuclein pathology. This case contributes to the increasing evidence for the heterogeneity of NBIA-1.

Validation of Addenbrooke's cognitive examination for detecting early dementia in a Japanese population.

There is a clear need for brief, but sensitive and specific, cognitive screening instruments for dementia. We assessed the diagnostic accuracy of the Japanese version of Addenbrooke's Cognitive Examination (ACE) in identifying early dementia in comparison with the conventional Mini-Mental State Examination (MMSE). Standard tests for evaluating dementia screening tests were applied. A total of 201 subjects (Alzheimer's disease (AD)=65, frontotemporal dementia (FTD)=24, vascular dementia=26, dementia with Lewy bodies=11, mild cognitive impairment (MCI)=13, and controls=62) participated in this study. The reliability of the ACE was very good (alpha coefficient=0.82). In our patient series, the sensitivity for diagnosing dementia with an ACE score of ≤74 was 0.889 with a specificity of 0.987, and the sensitivity of an ACE score of ≤80 was 0.984 with a specificity of 0.867. The Japanese version of the ACE is a very accurate instrument for the detection of early dementia, and should be widely used in clinical practice.