Oral applause sign in progressive supranuclear palsy.

The applause sign (AS) is a recognized phenomenon observed in progressive supranuclear palsy (PSP) and other neurological conditions where individuals produce over three claps following a request to clap only thrice after a demonstration. In this study, we introduced a novel linguistic phenomenon termed the oral applause sign (OAS) associated with the AS. The OAS is characterized by increased repetition counts of Japanese repetitive onomatopoeic words, such as uttering "pata-pata-pata" instead of the expected "pata-pata.

The utility of customised tissue probability maps and templates for patients with idiopathic normal pressure hydrocephalus: a computational anatomy toolbox (CAT12) study.

Background: Disproportionately enlarged subarachnoid space hydrocephalus (DESH) is one of the neuroradiological characteristics of idiopathic normal pressure hydrocephalus (iNPH), which makes statistical analyses of brain images difficult. This study aimed to develop and validate methods of accurate brain segmentation and spatial normalisation in patients with DESH by using the Computational Anatomy Toolbox (CAT12).

Methods: Two hundred ninety-eight iNPH patients with DESH and 25 healthy controls (HCs) who underwent cranial MRI were enrolled in this study.

Levodopa-resistant parkinsonism developing after ventriculoperitoneal shunting for obstructive hydrocephalus and improving after endoscopic third ventriculostomy, with specific consideration of brainstem morphology: illustrative case.

Background: Parkinsonism has been reported in patients with obstructive hydrocephalus (OH) following ventriculoperitoneal shunting (VPS). While levodopa works well, some cases are drug resistant. A few case series have reported that endoscopic third ventriculostomy (ETV) is beneficial, though its mechanism remains unclear.

Long-lasting pure topographical disorientation due to heading disorientation following left retrosplenial infarction: A report of two cases.

Topographical disorientation is linked to lesions in the right hemisphere and typically resolves within a few months post-stroke. Persistent topographical disorientation is uncommon and frequently accompanied by impairments in visual memory, complicating the analysis of the underlying mechanisms. Herein, we report two cases of sustained pure topographical disorientation following cerebral hemorrhages in the left retrosplenial region.

A decade with anomic primary progressive aphasia.

Some patients with primary progressive aphasia (PPA) demonstrate only anomia. The lack of longitudinal observations of anomic PPA precluded us from determining whether progressive anomic aphasia was simply an early stage of semantic or logopenic variants, or a relatively independent variant. Herein, we report the 10-year clinical course of a patient with PPA who presented with pure anomic aphasia for 9 years.

The grasp reflex in patients with idiopathic normal pressure hydrocephalus.

Objective: To investigate the prevalence and intensity of grasp reflexes and to examine changes in these reflexes after shunt surgery in patients with idiopathic normal pressure hydrocephalus (iNPH).

Methods: We enrolled 147 patients with probable iNPH. A standard procedure was used to determine the presence of grasp reflexes, and the intensity of these reflexes was assessed using a four-category classification.

[Preoperative Predictors of Outcomes of Cerebrospinal Fluid Shunt Surgery in Patients with Idiopathic Normal Pressure Hydrocephalus].

The disproportionately enlarged subarachnoid space hydrocephalus (DESH) findings on cranial images are useful to predict cerebrospinal fluid (CSF) shunt responsiveness to some extent in patients with idiopathic normal-pressure hydrocephalus (iNPH). However, recent studies show that patients with iNPH often have concomitant Alzheimer's or Lewy body disease regardless of DESH findings, which may be associated with poor outcomes of CSF shunt surgery. Moreover, long-term outcomes after CSF shunt surgery in patients with iNPH, which is one of the most important issues to be addressed for effective treatment of iNPH, remain unknown.

Interhemispheric asymmetrical change in gray matter volume in patients with unilateral hippocampal sclerosis.

Objectives: To clarify the interhemispheric asymmetrical change in gray matter volume (GMV) in unilateral hippocampal sclerosis (HS), we compared changes in GMV relative to normal subjects between the HS and contralateral or non-HS sides.

Material And Methods: Forty-five patients with unilateral HS and 30 healthy subjects were enrolled. We quantified changes in GMV in the patients with HS as compared to GMV in the normal subjects by introducing the Z-score (Z-GMV) in each region or region of interest in unilateral HS.

Auditory phonological identification impairment in primary progressive aphasia.

Objective: To examine the audiological characteristics and neuroanatomical regions associated with auditory phonological identification impairment in primary progressive aphasia (PPA).

Methods: Twenty-seven patients with PPA [13 non-fluent/agrammatic variant PPA (nfvPPA), three logopenic variant PPA (lvPPA), seven semantic variant PPA (svPPA), and four mixed type PPA] were included in the study. Neuropsychological, language, audiological, and neuroradiological examinations were also performed.

Ventriculoperitoneal Shunt Failure 3-year after Shunt Surgery Caused by Migration of Detached Ventricular Catheter into the Cranium: A Case Study of Idiopathic Normal-pressure Hydrocephalus.

Idiopathic normal-pressure hydrocephalus (iNPH) is a neurological disorder that typically presents with gait disturbance, cognitive impairment, and urinary incontinence. Although most patients respond to cerebrospinal-fluid shunting, some do not react well because of shunt failure. A 77-year-old female with iNPH underwent ventriculoperitoneal shunt implantation, and her gait impairment, cognitive dysfunction, and urge urinary incontinence improved.

Olfactory Dysfunction, an Often Neglected Symptom of Hydrocephalus: Experience from a Case of Late-Onset Idiopathic Aqueductal Stenosis.

Disturbance of smell is often accompanied with common neurodegenerative diseases such as Parkinson's and Alzheimer's diseases. In addition, patients with head trauma, intracranial tumors, and hydrocephalus can also develop olfactory dysfunction, and some of which can improve with treatment of the underlying disease. In clinical practice, few patients complain of smell disturbances, thus olfactory dysfunction is often overshadowed by visible motor symptoms.

Dyslexia and dysgraphia of primary progressive aphasia in Chinese: A systematic review.

Introduction: Currently, little is known about Chinese-speaking primary progressive aphasia (PPA) patients compared to patients who speak Indo-European languages. We examined the demographics and clinical manifestations, particularly reading and writing characteristics, of Chinese patients with PPA over the last two decades to establish a comprehensive profile and improve diagnosis and care.

Methods: We reviewed the demographic features, clinical manifestations, and radiological features of Chinese-speaking PPA patients from 56 articles published since 1994.

Mirror writing and cortical hypometabolism in Parkinson's disease.

Mirror writing (MW) is the production of individual letters, words, or word strings in the reverse direction. Parkinson's disease (PD) is a progressive neurodegenerative disorder, and high MW rates have been reported in patients with PD. Thus, the present study sought to identify the factors that cause MW in patients with PD.

Buccofacial apraxia in primary progressive aphasia.

Buccofacial apraxia (BFA) is associated with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) as well as with the severity of apraxia of speech (AOS), a core symptom of nfvPPA. However, an association with agrammatism has not been established. The aim of this study was to examine the association between BFA and agrammatism in nfvPPA and to determine differences in atrophic regions in primary progressive aphasia (PPA) with and without BFA.

Risk factors for unfavourable outcomes after shunt surgery in patients with idiopathic normal-pressure hydrocephalus.

A number of vascular risk factors (VRFs) have been reported to be associated with idiopathic normal-pressure hydrocephalus (iNPH), but it remains unclear whether these VRFs are related to patient outcomes after shunt surgery. Therefore, we investigated the risk factors for unfavourable outcomes after shunt surgery in iNPH patients using two samples from Tohoku University Hospital and from a multicentre prospective trial of lumboperitoneal (LP) shunt surgery for patients with iNPH (SINPHONI-2). We enrolled 158 iNPH patients.

Case Report: Semantic Variant Primary Progressive Aphasia With Impaired Verbal Word Discrimination.

Some patients with primary progressive aphasia (PPA) present with various types of hearing deficits. Research on the auditory function and speech sounds in PPA, including temporal, phonemic, and prosodic processing, revealed impairment in some of these auditory processes. Many patients with PPA who present with impaired word recognition subsequently developed non-fluent variant PPA.

Reduced default mode network connectivity relative to white matter integrity is associated with poor cognitive outcomes in patients with idiopathic normal pressure hydrocephalus.

Background: The aim of this study was to investigate whether default mode network (DMN) connectivity and brain white matter integrity at baseline were associated with severe cognitive impairments at baseline and poor cognitive outcomes after shunt placement in patients with idiopathic normal pressure hydrocephalus (iNPH).

Methods: Twenty consecutive patients with iNPH whose symptoms were followed for 6 months after shunt placement and 10 healthy controls (HCs) were enrolled. DMN connectivity and brain white matter integrity at baseline in the patients with iNPH and HCs were detected by using resting-state functional magnetic resonance imaging (MRI) with independent component analysis and diffusion tensor imaging, respectively, and these MRI indexes were compared between the patients with iNPH and HCs.

Improvement in callosal disconnection syndrome with recovery of callosal connectivity.

Recent advancements in radiological techniques have enabled the observation of the topographic distribution of the human corpus callosum. However, its functional connectivity remains to be elucidated. The symptoms of callosal disconnection syndrome (CDS) can potentially reveal the functional connections between the cerebral hemispheres.

Facial memory ability and self-awareness in patients with temporal lobe epilepsy after anterior temporal lobectomy.

Anterior temporal lobectomy (ATL) is the most common surgical treatment for drug-resistant temporal lobe epilepsy (TLE). Right ATL has been reported to reduce facial memory ability in patients with TLE, as indicated by poor performance on the Warrington Recognition Memory Test for Faces (RMF), which is commonly used to evaluate visual memory in these patients. However, little is known about whether patients with TLE exhibit difficulties in identifying faces in daily life after ATL.

Echolalia in patients with primary progressive aphasia.

Objective: This study aimed to examine echolalia and its related symptoms and brain lesions in primary progressive aphasia (PPA).

Methods: Forty-five patients with PPA were included: 19 nonfluent/agrammatic variant PPA (nfvPPA), 5 semantic variant PPA, 7 logopenic variant PPA, and 14 unclassified PPA patients. We detected echolalia in unstructured conversations.

Neural substrates underlying progressive micrographia in Parkinson's disease.

Introduction: The neural substrates associated with the development of micrographia remain unknown. We aimed to elucidate the neural substrates underlying micrographia in Parkinson's disease (PD) patients.

Methods: Forty PD patients and 20 healthy controls underwent handwriting tests that involved free writing and copying.

A change in brain white matter after shunt surgery in idiopathic normal pressure hydrocephalus: a tract-based spatial statistics study.

Background: The aim of this study was to elucidate changes in cerebral white matter after shunt surgery in idiopathic normal pressure hydrocephalus (INPH) using diffusion tensor imaging (DTI).

Methods: Twenty-eight consecutive INPH patients whose symptoms were followed for 1 year after shunt placement and 10 healthy control (HC) subjects were enrolled. Twenty of the initial 28 INPH patients were shunt-responsive (SR) and the other 8 patients were non-responsive (SNR).

The Pareidolia Test: A Simple Neuropsychological Test Measuring Visual Hallucination-Like Illusions.

Background: Visual hallucinations are a core clinical feature of dementia with Lewy bodies (DLB), and this symptom is important in the differential diagnosis and prediction of treatment response. The pareidolia test is a tool that evokes visual hallucination-like illusions, and these illusions may be a surrogate marker of visual hallucinations in DLB. We created a simplified version of the pareidolia test and examined its validity and reliability to establish the clinical utility of this test.

Callosal Disconnection Syndrome Associated with Relapsing Polychondritis.

Relapsing polychondritis (RP) is a rare inflammatory disorder of the cartilagenous structures, and it sometimes involves the central nervous system. Encephalitis associated with RP causes a wide variety of symptoms according to the affected sites. We herein report the first case of 72-year-old right-handed man who developed acute meningoencephalitis associated with RP involving the corpus callous.