Axonal ion channel dysfunction in c9orf72 familial amyotrophic lateral sclerosis

N Geevasinga, P Menon, J Howells… - JAMA …, 2015 - jamanetwork.com
Importance Abnormalities of axonal excitability characterized by upregulation of persistent
sodium (Na+) conductances and reduced potassium (K+) currents have been reported in …

[HTML][HTML] Assessment of motor units in neuromuscular disease

RD Henderson, PA McCombe - Neurotherapeutics, 2017 - Elsevier
The motor unit comprises the anterior horn cell, its axon, and the muscle fibers that it
innervates. Although the true number of motor units is unknown, the number of motor units …

Fatigue and activity dependent changes in axonal excitability in amyotrophic lateral sclerosis

S Vucic, AV Krishnan, MC Kiernan - Journal of Neurology …, 2007 - jnnp.bmj.com
Background: While patients with amyotrophic lateral sclerosis (ALS) may complain of
fatigue, the underlying mechanisms appear complex, with dysfunction of central and …

Motor responses evoked by transcranial magnetic stimulation and peripheral nerve stimulation in the ulnar innervation in amyotrophic lateral sclerosis: the effect of …

M de Carvalho, A Turkman, M Swash - Journal of the neurological sciences, 2003 - Elsevier
We studied the upper (UMN) and lower motor neuron (LMN) innervations of 159 hands from
81 patients with amyotrophic lateral sclerosis (ALS). Eleven patients with various chronic …

The neurophysiological index in ALS

M Swash, M de Carvalho - Amyotrophic Lateral Sclerosis and Other …, 2004 - Taylor & Francis
The Neurophysiological Index (NI) consists of a mathematical derivation of three
standardised neurophysiological measurements. Since these measurements are part of …

Neurophysiological index as a biomarker for ALS progression: validity of mixed effects models

BC Cheah, S Vucic, AV Krishnan… - Amyotrophic Lateral …, 2011 - Taylor & Francis
Our objective was to evaluate the neurophysiological index (NI) as a biomarker for
amyotrophic lateral sclerosis (ALS) and to assess the validity of linear mixed effects models …

Does caffeine modify corticomotor excitability?

V Cerqueira, A De Mendonça, A Minez, AR Dias… - Neurophysiologie …, 2006 - Elsevier
AIMS: To test the influence of caffeine on the lower and upper motor neuron excitability.
METHODS: In Experiment A, 18 healthy subjects received 200 mg of caffeine or placebo, in …

Amyotrophic lateral sclerosis diagnostic index: Toward a personalized diagnosis of ALS

N Geevasinga, J Howells, P Menon, M van den Bos… - Neurology, 2019 - AAN Enterprises
Objective The aim of the study was to assess the utility of a novel amyotrophic lateral
sclerosis (ALS) diagnostic index (ALSDI). Methods A prospective multicenter study was …

Changes of the phrenic nerve motor response in amyotrophic lateral sclerosis: longitudinal study

S Pinto, R Geraldes, N Vaz, A Pinto… - Clinical neurophysiology, 2009 - Elsevier
OBJECTIVE: Phrenic nerve motor amplitude (Diaphr Ampl) is predictive of hypoventilation in
amyotrophic lateral sclerosis (ALS). We aimed to evaluate its change over disease course …

[HTML][HTML] Coexistence of neuronal intranuclear inclusion disease and amyotrophic lateral sclerosis: an autopsy case

A Sugiyama, T Takeda, M Koide, H Yokota, H Mukai… - BMC neurology, 2021 - Springer
Background Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative
disease. Pathologically, it is characterized by eosinophilic hyaline intranuclear inclusions in …