ALSFRS-R
Revised Amyotrophic Lateral Sclerosis Functional Rating Scale
改訂版ALS機能評価尺度（ALSFRS-R）
1. Biological & Technical Mechanism
The ALSFRS-R evaluates 12 functional activities across four physiological domains: bulbar function (speech, salivation, swallowing), fine motor tasks (handwriting, cutting food, dressing), gross motor tasks (turning in bed, walking, climbing stairs), and respiratory function (dyspnea, orthopnea, respiratory insufficiency). Each item is scored from 4 (normal) to 0 (complete loss), yielding a total score of 0–48. On average, untreated ALS patients decline by ~1.0 point per month.
ALSFRS-Rは、筋萎縮性側索硬化症（ALS）の臨床試験および自然歴コホート（PRO-ACT, ENCALS等）において最も広く使用されている48点満点の機能評価スケールです。①球機能（言語・唾液・嚥下）、②微細運動（書字・食事動作・着衣）、③粗大運動（寝返り・歩行・階段昇降）、④呼吸機能（呼吸困難・起坐呼吸・人工呼吸器使用）の4ドメイン12項目を各0〜4点で評価し、月あたりのスコア低下率（ΔALSFRS-R / month）が治験の主要評価項目として用いられます。
2. Open Science & Clinical Trial Significance
Combining baseline ALSFRS-R progression slope with plasma NfL and ENCALS survival prediction models enables precision stratification in ALS trials.
ENCALS生存予測モデルや創薬治験において、ベースラインのALSFRS-R変化率（進行速度）と血漿NfL値を組み合わせることで、急速進行群と緩徐進行群を層別化し、検出力を高める設計が標準となっています。
Related Neuroscience Terms & Standards
Nuclear RNA/DNA-binding protein whose cytoplasmic mislocalization, phosphorylation, and loss of nuclear splicing function drive >95% of ALS cases and ~50% of FTD.
Antioxidant enzyme gene; gain-of-toxicity mutations in SOD1 account for ~15-20% of familial ALS and are targeted by the approved antisense oligonucleotide Tofersen.
Hexanucleotide repeat expansion (GGGGCC) in C9orf72 is the most common genetic cause of both familial ALS and familial frontotemporal dementia (FTD).
Axonal cytoskeletal protein released into CSF and blood upon neuroaxonal injury; a cross-disease biomarker of neurodegeneration rate in ALS, MS, AD, and FTD.
Open Datasets & Research Publications Related to ALSFRS-R
Spontaneous slow waves index multiple dimensions of recovery in Disorders of Consciousness
Whether and how residual awareness is expressed in patients with Disorders of Consciousness (DoC) depends on the severity of brain damage and the transient functional state of the cortex at assessment. Classically, DoC are associated with electroencephalographic (EEG) slowing, marked by high-amplitu...
Immediate cortical responses to transcranial magnetic stimulation of the human motor leg area
Background: Immediate transcranial evoked potentials (iTEPs) are ultra-early electroencephalographic responses elicited by single-pulse transcranial magnetic stimulation (TMS) of the primary motor cortical hand area (M1-HAND). Their characteristic latency, waveform, and sensitivity to short-interval...
Not All Entropy Is Equal: Why Representation Matters for Biological Interpretation in Dementia EEG
ObjectiveTo examine what a dementia-associated electroencephalography (EEG) entropy difference permits us to infer about brain dynamics by testing its dependence on permutation-entropy (PE) representation. MethodsFour PE settings represented identical alpha-band data from 1,177 CAUEEG recordings. N...
Assessing respiratory rates and signs of air hunger using the PneumoRator sensor.
ObjectivesTo investigate whether the PneumoRator wearable sensor could accurately assess Respiratory Rate (RR) and signs of air hunger (SAH) compared with clinician-observed assessment in adults with and without breathing pattern disorder (BrPD). DesignA pilot cross-sectional observational study. ...
KCNQ2/3 Regulates Efferent Mediated Slow Excitation of Vestibular Afferents in Mammals
Investigating motor neuron excitability in amyotrophic lateral sclerosis (ALS), this research reveals how KCNQ potassium channel subunits regulate efferent-mediated slow excitation. These electrophysiological findings uncover critical protective mechanisms against motor neuron degeneration.
Evidence-Gated Plasticity and Context-Dependent Consolidation for Multi-Goal Learning in Spiking Neural Networks
Background / Introduction: Biologically inspired spiking neural networks offer a mechanistic framework for adaptive behavior, yet learning multiple goals remains challenging when synaptic updates for different targets interfere. We investigated whether multi-timescale plasticity and context-specific...
Disclaimer: This encyclopedia entry is intended for academic neuroscience research and data science reference only, and does not constitute medical advice.