Akinori Futaura

• Showa University

Rationale Anti-mitochondrial antibodies (AMA) M2-positive myositis can lead to severe respiratory failure. Traditional immunotherapies sometimes fail to address respiratory failure. Herein, this CARE-compliant case report described a patient with AMA-M2-positive myositis who recovered from ventilation with tracheostomy owing to immunotherapy-resist...

Autoimmune encephalitis is a rapidly progressive inflammatory brain disease. Gamma-aminobutyric acid type B (GABAB) receptor autoimmune encephalitis is a rare subtype characterized by distinct clinical features. Diagnosis can be especially challenging when typical limbic symptoms and neuroimaging findings are absent. This case report underscores th...

Background Differences in the extent of cerebral white matter lesions (WML) and regional cerebral blood flow (rCBF) in early-stage cognitive impairment (ESCI) contribute to the prognosis of cognitive decline; however, it is unclear precisely how WML and rCBF affect cognitive decline in ESCI. Objective We examined the association between WML, rCBF,...

Background In Japan, only two medications of immediate-release levodopa with distinct ratios of decarboxylase inhibitor (DCI), namely levodopa/benserazide 100/25 mg and levodopa/carbidopa 100/10 mg, are available for the treatment of Parkinson's disease (PD). The relationship between the difference in the DCI to levodopa ratio and the development o...

Background Idiopathic normal pressure hydrocephalus (iNPH) is often misdiagnosed as Alzheimer’s disease (AD) due to overlapping pathophysiology and similar imaging characteristics, including ventricular enlargement and increased white matter lesions (WMLs). Objective To compare the extent and distribution of WMLs directly between iNPH and AD and e...

Marcel Proust had interactions with a lot of neurologists through treatments of his asthma. His great work, "In Search of Lost Time", is one of the highest masterpieces in French literature, about which numerous reviews have been published from various angles as well as literature. This work is also important for neurology, as the origin of Proust'...

Alzheimer's disease (AD) is a disorder in which individuals experience a difficulty in maintaining event memory for when, where, who, and what. However, verbal deficiency, one of the other symptoms of AD, may prevent a precise diagnosis of event memory because existing tests are based on verbal instructions by the tester and verbal response from pa...

The cover image is based on the Research Article Stopwatch training improves cognitive functions in patients with Parkinson's disease by Motoyasu Honma et al., https://doi.org/10.1002/jnr.24812

There is an urgent need to establish blood biomarkers for Alzheimer's disease (AD). Although it has been speculated that brain-derived neurotrophic factor (BDNF) is associated with AD, whether it can be used as a blood biomarker has yet to be determined. We used serum, cerebrospinal fluid (CSF), and medial temporal lobe atrophy from patients with A...

Dementia and cognitive impairment are considered to be one of the biggest social and medical problems. While there is a definite relationship between vitamin B and cognitive decline, this has yet to be fully assessed with regard to sex differences. Thus, the present study investigated the relationship of vitamin B1 or vitamin B12 with dementia in a...

Parkinson's disease (PD) impairs various cognitive functions, including time perception. Dysfunctional time perception in PD is poorly understood, and no study has investigated the rehabilitation of time perception in patients with PD. We aimed to induce the recovery of time perception in PD patients and investigated the potential relationship betw...

Background Toxic amyloid-β protein (Aβ) conformers play an important role in the progression of Alzheimer’s disease (AD). The ratio of toxic conformer to total Aβ42 in cerebrospinal fluid (CSF) was significantly high in AD and mild cognitive impairment (MCI) due to AD using an enzyme-linked immunosorbent assay kit with a 24B3 antibody. Objective W...

We compared ‘CIScore’ determined by quantitative single photon emission computed tomography studies of the cingulate island sign to cerebrospinal fluid (CSF) biomarkers in Lewy body disease (LBD) and Alzheimer’s disease (AD) to assess its usefulness and pathological background. Among the 16 each age-matched LBD and AD patients, the CIScore differed...

Background To investigate whether the number of cerebral microbleeds (CMB) could be a useful indicator to predict glymphatic system dysfunction in Alzheimer’s disease (AD) patients, by comparing the degree of cerebral spinal fluid (CSF) and interstitial fluid (ISF) stasis. Method Forty probable AD patients were included, with those exhibiting two...

Numbsense is a phenomenon, wherein patients can correctly respond to somatosensory stimuli at a higher rate than expected by chance, but cannot perceive the same stimuli consciously. Previously, numbsense has been reported in tactile localization of stimuli on the patient’s own body. Here, we describe a patient with numbsense that involved touched...

An adult female complained of enlargement of right eyes in other people. Diffusion-weighted imaging detected an abnormal high-intensity area in the region from the splenium of the corpus callosum to the major forceps on the right side. The patient reported that right eyes appeared larger in size, which suggested prosopometamorphopsia. Adichotic lis...

Objective: To investigate whether the number of cerebral microbleeds (CMB) could be a useful indicator to predict glymphatic system dysfunction in Alzheimer’s disease (AD) patients, by comparing the degree of cerebral spinal fluid (CSF) and interstitial fluid (ISF) stasis. Methods: Forty probable AD patients were included, with those exhibiting two...

Background and purpose: Chorea-acanthocytosis is clinically difficult to distinguish from Huntington's disease because these disorders have similar symptoms and MR imaging findings. We evaluated the usefulness of single-case voxel-based morphometry (VBM) analysis for differentiating the two diseases as well as VBM analysis. Materials and methods:...

Autism spectrum disorder (ASD) presents certain hallmark features associated with cognitive and social functions, however, the ability to estimate self-generated distance and duration in individuals with ASD are unclear. We compared the performance of 20 ASD individuals with 20 typical developments (TDs) with respect to two tasks: (1) the drawing o...

The thyroid hormones have been reported to be associated with cognitive decline and Alzheimer’s disease. The relationship between thyroid function within the normal range and cerebral blood flow in Alzheimer’s disease patients has been shown in a recent study. Mild cognitive impairment is often the first stage of Alzheimer’s disease; thus, early di...

Patient characteristics and all their relevant data. (XLSX)

The issue of whether serum lipid marker values are cognitively and neurologically significant for elderly individuals attending a memory clinic has been controversial. We investigated the associations of serum lipid markers with the memory function and cortical structure in 52 patients aged ≥75 years who had attended our memory clinic based on thei...

Parkinson's disease (PD) is associated with various cognitive impairments. However, the nature of cognitive modification in patients with PD remains to be elucidated. In the present study, we examined whether patients with PD could correct and maintain subjective time duration and line length estimation. After training sessions, in which participan...

Group comparisons in the delayed reproduction task. (PDF)

Differences in the length estimation training task between the last trial in the training session and the trials in the retest session. (PDF)

A representative trial of length estimation training without cues in a patient with Parkinson’s disease. (MP4)

Background The relationship between dementia and time perception impairment is unknown. Aim This study aims to explore subjective perception of the passage of time in patients with Alzheimer’s disease (AD). Methods We conducted semi-structured interviews with 11 AD patients. Grounded theory, a qualitative research methodology, was used for data a...

Musical hallucination is one of the most complex forms of auditory hallucinations, where subjects perceive complex sound in the form of music, in the absence of an acoustic stimulus. It has been reported in patients with diseases such as psychiatric disorders, organic brain diseases, and epilepsy. However, the most common of these are idiopathic mu...

Diagonistic apraxia is a corpus callosal disconnection syndrome. Callosal lesions in Neuromyelitis optica spectrum disorder (NMOSD) have been reported, but callosal disconnection syndrome are rare. A 48-year-old woman was treated for fever and a cough before hospitalization. Her fever abated immediately, but she had balance problems in walking and...

Objective: To determine whether Parkinson disease (PD) affects cross-modal function of vision and olfaction because it is known that PD impairs various cognitive functions, including olfaction. Methods: We conducted behavioral experiments to identify the influence of PD on cross-modal function by contrasting patient performance with age-matched...

Figure S1 Patient's fixed time schedule.

Figure S3 Weekly timetable.

Video S2 Patient showed mistakes in estimating the passage of time and disorientation in present time.

Figure S4 A hypothetical functional mechanism of topographical orientation in medial parietal lobes.

Video S1 Patient interview about mental time impairment and daily life.

Figure S2 A sketch of the rooms layout.

Video S3 Patient narrated the layout of his house as he walked around it.

The Wechsler Memory Scale-Revised (WMS-R) is one of the internationally well-known batteries for memory assessment in a general memory clinic setting. Several factor structures of the WMS-R for patients aged under 74 have been proposed. However, little is known about the factor structure of the WMS-R for patients aged over 75 years and its neurolog...

Lesions of the medio-parietal lobes are linked with topographical and temporal disorientation and of interest to understanding mental time. We examined a 39-year-old man who worked as a driving instructor before cerebral hemorrhage, and followed his case for eight years including neuropsychological testing and brain imaging. The patient had mild an...

In daily life, we sometimes select temporal cues of one sort while suppressing others. This study investigated the mechanism of suppression by examining a split-brain patient’s perception of target intervals while ignoring distractor intervals. A patient with agenesis of corpus callosum and five age- and sex-matched control subjects participated in...

Background: Selegiline enhances the patient’s endogenous dopamine by inhibiting dopamine metabolism. The efficacy of selegiline monotherapy for drug-naïve Parkinson’s disease (PD) patients may depend on the degree of dopaminergic neuronal degeneration. ¹²³I-Ioflupane single photon emission computed tomography (SPECT) and ¹²³I-meta-iodobenzylguanidi...

To produce coordinated manual actions within specific space and time, their relationship must be properly dealt with in a sensorimotor system. This study examined how such a coordination system might be impaired in normal aging and in Parkinson's disease (PD). Using a tablet device, young participants, elderly participants, and patients with PD wer...

The nerve center responsible for controlling our circadian rhythm is located in a cluster of cells known as the suprachiasmatic nucleus in the hypothalamus. Various physiological functions such as sleep, arousal, blood pressure, body temperature, and hormone secretion are regulated in a 24-hour rhythm by this circuit. Somatic cells of other organs...

Leber's hereditary optic neuropathy(LHON), a mitochondrial disease, is clinically characterized by bilateral subacute loss of central vision because of optic nerve involvement. LHON is usually maternally inherited; however, sporadic cases may occur. Three mitochondrial DNA (mtDNA)mutations(the most frequent being G11778A, G3460A, and T14484C)accoun...

Patients with Parkinson’s disease (PD) often underestimate time intervals, however it remains unclear why they underestimate rather than overestimate them. The current study examined time underestimation and counting in patients with PD, in relation to dopamine transporter (DaT) located on presynaptic nerve endings in the striatum. Nineteen non-dem...

Confabulation is a false story that amnesic patients make up unintentionally, by recollecting irrelevant memories instead of relevant. Disconnection of a brain network, including the diencephalon, basal forebrain, orbitofrontal, cortex and medial temporal lobe, could be the cause of this neuropsychological symptom. Considering confabulation from th...

Background: Dopaminergic drugs, the gold standard for motor symptoms, are known to affect cognitive function in Parkinson’s disease (PD) patients. Objective: We compared the effects of dopaminergic treatment on motor and cognitive function in drug-naïve patients. Methods: Dopaminergic medication (levodopa, dopamine agonist, selegiline) was given to...

Background: Autobiographical memory is a form of episodic memory characterized by a sense of time and consciousness that enables an individual to subjectively re-experience his or her past. As part of this mental re-enactment, the past is recognized relative to the present. Dysfunction of this memory system may lead to confusion regarding the pres...

Background Picture agnosia has become more significant as a symptom in diagnosing neurodegenerative diseases. However, there is no standardized test for picture agnosia. Aim To design a picture test for picture agnosia and better understand related 3‐D visual perception. Methods A total of 10 Alzheimer's disease patients and 10 healthy older adul...

An aging global population is driving the current epidemic of dementia and type 2 diabetes mellitus. Diabetes is a known risk factor for the development of vascular dementia, Alzheimer's disease, and mild cognitive impairment. Good control of diabetes may improve cognitive decline and prevent Alzheimer's disease. Mild cognitive impairment with type...

Background In patients with Parkinson's disease (PD), executive deficits are known to correlate with motor dysfunctions such as gait and postural instability. However executive deficits are sometimes difficult to detect using common frontal assessment batteries. Behavioral Assessment of Dysexecutive Syndrome (BADS) includes 6 subtests to evaluate d...

It is known that the number of patients with dementia is increasing in Japan. Stratified by age, the incidence of epilepsy rises among patients aged ≥ 60 years, demonstrating a high incidence among the elderly. Therefore, an increased incidence of epilepsy among patients with dementia would be expected. However, no study has addressed this question...

Mental time is altered by a number of factors and the underlying neural processing involved is highly complicated. Recent research suggests that mental time in patients with particular neurological diseases is perceptually shorter than in normal individuals. This review introduces mental time dysfunction and a model for processing of mental time in...

Background Studies show that diabetes mellitus is the greatest lifestyle risk factor for dementia. Appropriate management and treatment of type 2 diabetes mellitus may thus prevent onset and progression of MCI to dementia.AimDetection of mild cognitive impairment in patients with type 2 diabetes mellitus (DM-MCI).Methods Mild cognitive impairment (...

A 49-year-old man with mitochondrial disease presented with visual allesthesia, a rare and puzzling phenomenon. He was admitted for treatment because of convulsions. After the convulsions ceased, he exhibited left homonymous hemianopsia. Brain diffusion-weighted magnetic resonance imaging (MRI) showed a high-intensity area in the right occipital lo...

Abstract An 83-year-old man with 3 years symptomatic hearing loss suddenly experienced musical hallucinosis. He heard children's songs, folk songs, military songs, and the Japanese national anthem for seven months every day. He sometime had paroxysmal nausea, dull headaches and depressive mood. On examination he had no psychosis or neurological sym...

Abstract Apraxia is the inability to perform actions or move different parts of the body in the intended manner, despite normal physical capability of movement. Based on his studies, Liepmann divided apraxia into three types: ideational apraxia, ideomotor apraxia, and limb-kinetic apraxia. Clinical findings such as ideomotor apraxia in Broca's area...

Parietal ataxia is a rare condition of hemi- or mono-ataxia caused by contralateral lesions in the parietal lobe. Parietal ataxia is categorized into sensory ataxia and pseudocerebellar ataxia, and is usually reported in single-case studies. Here we report 13 cases of hemi- or mono-ataxia caused by acute brain infarction in the parietal lobe, and d...

AimCorrelations between motor function and frontal-executive function in Parkinson's disease (PD) have been examined previously, but correlations with other cognitive domains remain unknown. We examined the correlation between motor dysfunction and cognitive impairment with regard to their precise domains. Methods Motor and cognitive functions were...

Abstract Mental time is the cognitive conscious perception of past, present, and future. In this review we examine the novel research field of the neuropsychology of mental time in five ways. First, we review the mental time of amnesic patients. Second, we review reports of "Chronognosia". Third, we review confabulation associated with disturbance...

Abstract Higher brain dysfunction is a major problems of epileptic patients. Epilepsy may cause transient epileptic higher brain dysfunctions like transient epileptic amnesia (TEA), or persistent epileptic higher brain dysfunction with or without findings of non-convulsive status epilepticus (NCSE). These are all defined as epilepsy with higher bra...

AimAlthough a previous functional magnetic resonance imaging study showed that the fusiform and parahippocampal gyri respond differentially to faces of the same versus different races, it is not clear whether the cortical regions are involved in perception of race. We examined whether two brain-damaged patients with bilateral fusiform and parahippo...

AimDiagnosis of mild cognitive impairment (MCI) in Parkinson's disease (PD; PD-MCI) can be difficult. We examined whether the Japanese version of the Montreal Cognitive Assessment (MoCA-J) and the Neurobehavioral Cognitive Status Examination (COGNISTAT-J) were suitable to screen PD-MCI. MethodsA total of 50 patients with PD and PD with dementia, to...

Background and AimBroca's area, which comprises the inferior frontal gyrus and its surrounding regions, is associated with language function. Recent functional imaging studies showed that it is also related to processing goal-directed actions.This study aimed to determine the types of action disorders that occur after damage to Broca's area. Method...

Abstract Acquired higher brain dysfunction is for the most part due to cerebral vascular disease, but epilepsy may also be a cause. In this study with five patients, we discuss the advantages of anti-epileptic drugs (AEDs) for persistent higher brain dysfunction. The patients showed chronic amnesia or acute aphasia, with associated symptoms like pe...

AimTo clarify the diagnosis and treatment of epilepsy with higher brain dysfunction (E-HBD). Methods Using neuropsychological testing we identified three cases. ResultsAll three cases showed persistent aphasia, amnesia or attention deficits – possibly connected with epilepsy – and were treated with anti-epileptic drugs (AED). Although the AED prove...

Abstract Epilepsy with higher brain dysfunction (E-HBD) is sometimes associated with dementia. For example, temporal lobe epilepsy may cause amnesia or behavioral abnormality, and the patient resembles an Alzheimer disease or frontotemporal dementia. E-HBD has two types, transient epileptic higher brain dysfunction and persistent. The persistent ty...

Apraxia is a well-known disorder of praxis and is caused mainly by damage to the left parietal lobe. We presented two cases of neurodegenerative disease with a distinct disorder of praxis, predominantly involving left parietal lobe. While both patients could understand what they should do, they were not able to initiate action and often stopped dur...

We report a 27-year-old man who presented with a continuous headache, nausea, and lack of awareness after convulsive attacks. Electroencephalography showed rhythmic theta activity or generalized sharp-and-slow wave complexes that met EEG criteria for NCSE. A Magnetic resonance imaging study showed focal cortical edema in the mesial temporal lobes,...

Posterior cortical atrophy (PCA) is a degenerative disease characterized by progressive visual agnosia with posterior cerebral atrophy. We examine the role of the picture naming test and make a number of suggestions with regard to diagnosing PCA as atypical dementia. We investigated 3 cases of early-stage PCA with 7 control cases of Alzheimer disea...

Prosopagnosia, the inability to recognize faces, has a history going back to Charcot and Hughlings-Jackson, but was first named by Bodamer in 1947. Its anatomical loci are still unclear. However, progressive prosopagnosia is normally linked to right dominant temporal lobe atrophy, and diagnosed as part of frontotemporal lobar degeneration. Here we...

Progressive visual agnosia was discovered in the 20th century following the discovery of classical non-progressive visual agnosia. In contrast to the classical type, which is caused by cerebral vascular disease or traumatic injury, progressive visual agnosia is a symptom of neurological degeneration. The condition of progressive visual loss, includ...

This study aimed to test whether type 1 myotonic dystrophy (DM1) patients who have a lower sensitivity to emotional facial expressions have an abnormal olfactory threshold or recognition level. We measured DM1 patients' performances in an olfactory acuity test and respiratory responses to odor stimuli, and compared their results to those of healthy...