123 documents found, page 1 of 13
The role of gastrostomy and noninvasive ventilation in primary lateral sclerosis
Oliveira Santos, Miguel; Domingues, Sara; Simão, Sara; Gromicho, Marta; Alves, Inês; Carvalho, MamedeIntroduction/aims: Literature on the role of gastrostomy and noninvasive ventilation (NIV) in primary lateral sclerosis (PLS) is limited. We aim to investigate whether PLS patients develop dysphagia requiring feeding tubes or respiratory failure necessitating NIV. Methods: We conducted a retrospective study of PLS patients with a definite diagnosis followed at our center (1994-2024). Patients with marked dyspha...
Effect of electrode size in phrenic nerve CMAP
Castro, José; Oliveira Santos, Miguel; Carvalho, MamedePhrenic nerve (PN) investigation is a simple and safe non-invasive diagnostic tool widely utilized in assessing respiratory failure in patients with neuromuscular disorders [10]. In amyotrophic lateral sclerosis (ALS), PN amplitude has been shown to correlate with forced vital capacity, serving as a predictor of hypoventilation and survival. In intensive care units (ICU), PNI study can also provide valuable inf...
The F-wave fingerprint of amyotrophic lateral sclerosis
Carvalho, MamedeInitially detailed by Magladery and McDougal, F-waves are late responses resulting from the antidromic activation of motor axons distally, causing a recurrent discharge of spinal motoneurons. These waves are readily elicited and best recorded from small muscles in the hands and feet. Conversely, F-wave responses in proximal muscles often have latencies too close to M-waves, complicating accurate identification.
Subclinical involvement of small hand muscles in early amyotrophic lateral scle...
Carvalho, Mamede; Oliveira Santos, Miguel; Swash, MichaelObjectives: Split-hand phenomenon is common in patients with amyotrophic lateral sclerosis (ALS), but it is unknown if first dorsal interosseous (FDI) and abductor pollicis brevis (ABP) are affected earlier than abductor digiti minimi (ADM). We aimed to address this issue. Methods: One clinically normal hand from ALS patients was investigated, including needle EMG of the FDI, motor amplitude, distal latency, F-...
Segmental motor neuron dysfunction in amyotrophic lateral sclerosis: Insights f...
Castro, José; Oliveira Santos, Miguel; Swash, Michael; Carvalho, MamedeIntroduction/aims: In amyotrophic lateral sclerosis (ALS), the role of spinal interneurons in ALS is underrecognized. We aimed to investigate pre- and post-synaptic modulation of spinal motor neuron excitability by studying the H reflex, to understand spinal interneuron function in ALS. Methods: We evaluated the soleus H reflex, and three different modulation paradigms, to study segmental spinal inhibitory mech...
F-waves responses derived from low-intensity electrical stimulation: a method t...
Lopes, Miguel; Swash, Michael; Carvalho, MamedeObjectives: The "split-hand syndrome" is a common clinical sign in amyotrophic lateral sclerosis (ALS), being characterized by more severe atrophy of the hand muscles on the radial side of the hand compared to the ulnar side. We aimed to investigate possible physiological differences between relevant hand muscles using low-intensity F-wave stimulation to assess spinal motoneuron excitability. Methods: We recrui...
Predicting clinical events characterizing the progression of amyotrophic latera...
Guazzo, Alessandro; Atzeni, Michele; Idi, Elena; Trescato, Isotta; Tavazzi, Erica; Longato, Enrico; Manera, Umberto; Chió, Adriano; Gromicho, MartaBackground: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that results in death within a short time span (3-5 years). One of the major challenges in treating ALS is its highly heterogeneous disease progression and the lack of effective prognostic tools to forecast it. The main aim of this study was, then, to test the feasibility of predicting relevant clinical outcomes that char...
Profiling tofersen as a treatment of superoxide dismutase 1 amyotrophic lateral...
Oliveira Santos, Miguel; Carvalho, MamedeIntroduction: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive motor neuron disorder with a fatal outcome 3-5 years after disease onset due to respiratory complications. Superoxide dismutase 1 (SOD1) mutations are found in about 2% of all patients. Tofersen is a novel oligonucleotide antisense drug specifically developed to treat SOD1-ALS patients. Areas covered: Our review covers and discusses tofe...
Imaging the fasciculating motor unit
Swash, Michael; Carvalho, MamedeFasciculations are a common early feature of amyotrophic lateral sclerosis (ALS), prompting a stream of research reports on their characteristic features based on clinical observation, surface EMG, concentric needle EMG, multipoint surface recordings, ultrasound detection within muscles, and now, MR imaging (Heskamp et al., 2024). This has revealed transient signal voids in pulsed gradient spin echo (PGSE) diff...
Artificial intelligence and statistical methods for stratification and predicti...
Tavazzi, Erica; Longato, Enrico; Vettoretti, Martina; Aidos, Helena; Trescato, Isotta; Roversi, Chiara; Martins, Andreia S.; Castanho, Eduardo N.Background: Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disorder characterised by the progressive loss of motor neurons in the brain and spinal cord. The fact that ALS's disease course is highly heterogeneous, and its determinants not fully known, combined with ALS's relatively low prevalence, renders the successful application of artificial intelligence (AI) techniques particularly arduous...