Automatic classification of AD pathology in FTD phenotypes using natural speech.

Introduction: Screening for Alzheimer's disease neuropathologic change (ADNC) in individuals with atypical presentations is challenging but essential for clinical management. We trained automatic speech-based classifiers to distinguish frontotemporal dementia (FTD) patients with ADNC from those with frontotemporal lobar degeneration (FTLD).

Methods: We trained automatic classifiers with 99 speech features from 1 minute speech samples of 179 participants (ADNC = 36, FTLD = 60, healthy controls [HC] = 89).

Changes in Digital Speech Measures in Asymptomatic Carriers of Pathogenic Variants Associated With Frontotemporal Degeneration.

Background And Objectives: Clinical trials developing therapeutics for frontotemporal degeneration (FTD) focus on pathogenic variant carriers at preclinical stages. Objective, quantitative clinical assessment tools are needed to track stability and delayed disease onset. Natural speech can serve as an accessible, cost-effective assessment tool.

Digital markers of motor speech impairments in spontaneous speech of patients with ALS-FTD spectrum disorders.

Objective: To evaluate automated digital speech measures, derived from spontaneous speech (picture descriptions), in assessing bulbar motor impairments in patients with ALS-FTD spectrum disorders (ALS-FTSD).

Methods: Automated vowel algorithms were employed to extract two vowel acoustic measures: vowel space area (VSA), and mean second formant slope (F2 slope). Vowel measures were compared between ALS with and without clinical bulbar symptoms (ALS + bulbar (n = 49, ALSFRS-r bulbar subscore: x¯ = 9.

Comparison of category and letter fluency tasks through automated analysis.

Introduction: Category and letter fluency tasks are commonly used neuropsychological tasks to evaluate lexical retrieval.

Methods: This study used validated automated methods, which allow for more expansive investigation, to analyze speech production of both category ("Animal") and letter ("F") fluency tasks produced by healthy participants ( = 36) on an online platform. Recordings were transcribed and analyzed through automated pipelines, which utilized natural language processing and automatic acoustic processing tools.

Apraxia of Speech in the Spontaneous Speech of Nonfluent/Agrammatic Primary Progressive Aphasia.

Background: Apraxia of speech (AOS) is a core feature of nonfluent/agrammatic primary progressive aphasia (naPPA), but its precise characteristics and the prevalence of AOS features in spontaneous speech are debated.

Objective: To assess the frequency of features of AOS in the spontaneous, connected speech of individuals with naPPA and to evaluate whether these features are associated with an underlying motor disorder such as corticobasal syndrome or progressive supranuclear palsy.

Methods: We examined features of AOS in 30 patients with naPPA using a picture description task.

Digital markers of motor speech impairments in natural speech of patients with ALS-FTD spectrum disorders.

Background And Objectives: Patients with ALS-FTD spectrum disorders (ALS-FTSD) have mixed motor and cognitive impairments and require valid and quantitative assessment tools to support diagnosis and tracking of bulbar motor disease. This study aimed to validate a novel automated digital speech tool that analyzes vowel acoustics from natural, connected speech as a marker for impaired articulation due to bulbar motor disease in ALS-FTSD.

Methods: We used an automatic algorithm called Forced Alignment Vowel Extraction (FAVE) to detect spoken vowels and extract vowel acoustics from 1 minute audio-recorded picture descriptions.

Prosodic characteristics of prepausal words produced by patients with neurodegenerative disease.

Prosody of patients with neurodegenerative disease is often impaired. We investigated changes to two prosodic cues in patients: the pitch contour and the duration of prepausal words. We analyzed recordings of picture descriptions produced by patients with neurodegenerative conditions that included either cognitive (n=223), motor (n=68), or mixed cognitive and motor impairments (n=109), and by healthy controls (n=28; HC).

CSF Biomarkers of Alzheimer Disease in Patients With Concomitant α-Synuclein Pathology.

Background And Objectives: CSF biomarkers β-amyloid 1-42 (Aβ), phosphorylated tau 181 (p-tau), total tau (t-tau), and neurogranin (Ng) can diagnose Alzheimer disease (AD) in life. However, it is unknown whether CSF concentrations, and thus their accuracies, are affected by concomitant pathologies common in AD, such as α-synuclein (αSyn). Our primary goal was to test whether biomarkers in patients with AD are altered by concomitant αSyn.

Quantifying articulatory impairments in neurodegenerative motor diseases: A scoping review and meta-analysis of interpretable acoustic features.

Purpose: Neurodegenerative motor diseases (NMDs) have devastating effects on the lives of patients and their loved ones, in part due to the impact of neurologic abnormalities on speech, which significantly limits functional communication. Clinical speech researchers have thus spent decades investigating speech features in populations suffering from NMDs. Features of impaired articulatory function are of particular interest given their detrimental impact on intelligibility, their ability to encode a variety of distinct movement disorders, and their potential as diagnostic indicators of neurodegenerative diseases.

Natural speech markers of Alzheimer's disease co-pathology in Lewy body dementias.

Introduction: An estimated 50% of patients with Lewy body dementias (LBD), including Parkinson's disease dementia (PDD) and Dementia with Lewy bodies (DLB), have co-occurring Alzheimer's disease (AD) that is associated with worse prognosis. This study tests an automated analysis of natural speech as an inexpensive, non-invasive screening tool for AD co-pathology in biologically-confirmed cohorts of LBD patients with AD co-pathology (SYN + AD) and without (SYN-AD).

Methods: We analyzed lexical-semantic and acoustic features of picture descriptions using automated methods in 22 SYN + AD and 38 SYN-AD patients stratified using AD CSF biomarkers or autopsy diagnosis.

Lexical and Acoustic Speech Features Relating to Alzheimer Disease Pathology.

Background And Objectives: We compared digital speech and language features of patients with amnestic Alzheimer disease (aAD) or logopenic variant primary progressive aphasia (lvPPA) in a biologically confirmed cohort and related these features to neuropsychiatric test scores and CSF analytes.

Methods: We included patients with aAD or lvPPA with CSF (phosphorylated tau ([p-tau]/β-amyloid [Aβ] ≥0.09, and total tau/Aβ ≥0.

Psychometric Properties of Rapid Word-Based Rate Measures in the Assessment of Bulbar Amyotrophic Lateral Sclerosis: Comparisons With Syllable-Based Rate Tasks.

Purpose Rapid maximum performance repetition tasks have increasingly demonstrated their utility as clinimetric markers supporting diagnosis and monitoring of bulbar disease in amyotrophic lateral sclerosis (ALS). A recently developed protocol uses novel real-word repetitions instead of traditional nonword/syllable sequences in hopes of improving sensitivity to motor speech impairments by adding a phonological target constraint that would activate a greater expanse of the motor speech neuroanatomy. This study established the psychometric properties of this novel clinimetric protocol in its assessment of bulbar ALS and compared performance to traditional syllable sequence dysdiadochokinetic (DDK) tasks.

Image-Guided Biopsy for Relapsed Neuroblastoma: Focus on Safety, Adequacy for Genetic Sequencing, and Correlation of Tumor Cell Percent With Quantitative Lesion MIBG Uptake.

Purpose: Many novel therapies for relapsed and refractory neuroblastoma require tumor tissue for genomic sequencing. We analyze our experience with image-guided biopsy in these patients, focusing on safety, yield, adequacy for next-generation sequencing (NGS), and correlation of tumor cell percent (TC%) with quantitative uptake on I-meta-iodobenzylguanidine (MIBG) single-photon emission computed tomography with computed tomography (SPECT/CT).

Materials And Methods: An 11-year retrospective review of image-guided biopsy on 66 patients (30 female), with a median age of 8.

Digital Speech Analysis in Progressive Supranuclear Palsy and Corticobasal Syndromes.

Background: Progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS) as well as non-fluent/agrammatic primary progressive aphasia (naPPA) are often associated with misfolded 4-repeat tau pathology, but the diversity of the associated speech features is poorly understood.

Objective: Investigate the full range of acoustic and lexical properties of speech to test the hypothesis that PSPS-CBS show a subset of speech impairments found in naPPA.

Methods: Acoustic and lexical measures, extracted from natural, digitized semi-structured speech samples using novel, automated methods, were compared in PSPS-CBS (n = 87), naPPA (n = 25), and healthy controls (HC, n = 41).

Translational research in pediatric contrast-enhanced ultrasound.

The role of contrast-enhanced ultrasound (CEUS) imaging is being widely explored by various groups for its use in the pediatric population. Clinical implementation of new diagnostic or therapeutic techniques requires extensive and meticulous preclinical testing and evaluation. The impact of CEUS will be determined in part by the extent to which studies are oriented specifically toward a pediatric population.

Balloon occlusion as an adjunctive technique during sclerotherapy of Puig's classified advanced venous malformations.

Objective: Puig types 2 through 4 venous malformations (VMs) are challenging to treat with sclerotherapy given their robust systemic outflow. Endovenous balloon occlusion offers a means of temporarily occluding systemic venous outflow to allow for more complete sclerotherapy. This study reviews our experience of implementing this technique in patients with Puig advanced (types 2 through 4) VMs.

Starting a pediatric contrast ultrasound service: made simple!

The addition of contrast US to an existing pediatric US service requires several preparatory steps. This overview provides a guide to simplify the process. Initially, it is important to communicate to all stakeholders the justifications for pediatric contrast US, including (1) its comparable or better diagnostic results relative to other modalities; (2) its reduction in procedural sedation or anesthesia by avoiding MRI or CT; (3) its reduction or elimination of radiation exposure by not having to perform fluoroscopy or CT; (4) the higher safety profile of US contrast agents (UCA) compared to other contrast agents; (5) the improved exam comfort and ease inherent to US, leading to better patient and family experience, including bedside US exams for children who cannot be transported; (6) the need for another diagnostic option in light of increasing demand by parents and providers; and (7) its status as an approved and reimbursable exam.

Contrast-enhanced ultrasound in pediatric interventional radiology.

There is growing interest in the use of contrast-enhanced ultrasound (CEUS) in diagnostic and interventional radiology. CEUS applications in interventional radiology are performed with intravascular or intracavitary administration of microbubble-based US contrast agents to allow for real-time evaluation of their distribution within the vascular bed or in body cavities, respectively, providing additional information beyond gray-scale US alone. The most common interventional-radiology-related CEUS applications in children have been extrapolated from those in adults, and they include the use of CEUS to guide lesion biopsy and to confirm drain placement in pleural effusions and intra-abdominal fluid collections.

Automated analysis of lexical features in frontotemporal degeneration.

We implemented an automated analysis of lexical aspects of semi-structured speech produced by healthy elderly controls (n = 37) and three patient groups with frontotemporal degeneration (FTD): behavioral variant FTD (n = 74), semantic variant primary progressive aphasia (svPPA, n = 42), and nonfluent/agrammatic PPA (naPPA, n = 22). Based on previous findings, we hypothesized that the three patient groups and controls would differ in the counts of part-of-speech (POS) categories and several lexical measures. With a natural language processing program, we automatically tagged POS categories of all words produced during a picture description task.

Cognitive Profile and Markers of Alzheimer Disease-Type Pathology in Patients With Lewy Body Dementias.

Objective: To determine whether patients with Lewy body dementia (LBD) with likely Alzheimer disease (AD)-type copathology are more impaired on confrontation naming than those without likely AD-type copathology.

Methods: We selected 57 patients with LBD (dementia with Lewy bodies [DLB], n = 38; Parkinson disease dementia [PDD], n = 19) with available AD CSF biomarkers and neuropsychological data. CSF β-amyloid (Aβ), phosphorylated-tau (p-tau), and total-tau (t-tau) concentrations were measured.

Lexical and Acoustic Characteristics of Young and Older Healthy Adults.

Purpose This study examines the effect of age on language use with an automated analysis of digitized speech obtained from semistructured, narrative speech samples. Method We examined the Cookie Theft picture descriptions produced by 37 older and 76 young healthy participants. Using modern natural language processing and automatic speech recognition tools, we automatically annotated part-of-speech categories of all tokens, calculated the number of tense-inflected verbs, mean length of clause, and vocabulary diversity, and we rated nouns and verbs for five lexical features: word frequency, familiarity, concreteness, age of acquisition, and semantic ambiguity.

MRI for Response Assessment of Extensive Lymphatic Malformations in Children Treated With Sirolimus.

Extensive lymphatic malformations (LMs) may cause substantial morbidity. The mammalian target of rapamycin (mTOR) inhibitor sirolimus shows promise for treating vascular anomalies, although response assessment is not standardized. The purpose of this study was to retrospectively characterize changes seen on MRI of children with extensive LMs treated with sirolimus.

Validation of Articulatory Rate and Imprecision Judgments in Speech of Individuals With Amyotrophic Lateral Sclerosis.

Purpose Perceptual judgments of articulatory function are commonly used by speech-language pathologists to evaluate articulatory performance in individuals with amyotrophic lateral sclerosis (ALS). The goal of this study was to evaluate the psychometric properties (e.g.

Can Radiology Technologists be Trained to Measure Leg Length Discrepancies as Accurately as Pediatric Radiologists?

Rationale And Objectives: Leg length discrepancy studies are labor intensive. They are procedurally simple and represent inefficient use of the radiologists' time and expertise. We hypothesized that radiology technologists could be trained to measure leg length discrepancies, and that their performance would be statistically equivalent to that of board-certified, fellowship-trained pediatric radiologists.