[HTML][HTML] Increased cerebral integrity metrics in poliomyelitis survivors: putative adaptation to longstanding lower motor neuron degeneration

SLH Shing, J Lope, MC McKenna, RH Chipika… - Journal of the …, 2021 - Elsevier
Background Post-polio syndrome (PPS) has been traditionally considered a slowly
progressive condition that affects poliomyelitis survivors decades after their initial infection …

Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment

Y Hu, L Wei, A Li, T Liu, Y Jiang, C Xie… - Frontiers in …, 2023 - frontiersin.org
Objective Spinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by
the degeneration of motor neurons in the spinal cord. It remains uncertain whether the …

Spinal muscular atrophy-like phenotype in a mouse model of acid ceramidase deficiency

MS Nagree, J Rybova, A Kleynerman… - Communications …, 2023 - nature.com
Mutations in ASAH1 have been linked to two allegedly distinct disorders: Farber disease
(FD) and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME). We …

Promises and pitfalls of imaging-based biomarkers in motor neuron diseases

EL Tan, P Bede, PF Pradat - Current Opinion in Neurology, 2023 - journals.lww.com
Although we acknowledge the academic achievements of large descriptive studies, an
unmet priority of neuroimaging in MND is the development of robust diagnostic, prognostic …

Cervical spondylotic amyotrophy: a systematic review

W Luo, Y Li, Q Xu, R Gu, J Zhao - European Spine Journal, 2019 - Springer
Purpose Cervical spondylotic amyotrophy (CSA) is characterized by upper limb muscle
weakness and atrophy, without sensory deficits. The pathophysiology of CSA has been …

Functional abnormalities of cerebellum and motor cortex in spinal muscular atrophy mice

A Tharaneetharan, M Cole, B Norman, NC Romero… - Neuroscience, 2021 - Elsevier
Spinal muscular atrophy (SMA) is a devastating genetic neuromuscular disease. Diffuse
neuropathology has been reported in SMA patients and mouse models, however, functional …

[HTML][HTML] Cerebellar remodelling decades after spinal cord insult: neuroplasticity in poliomyelitis survivors

SLH Shing, A Murad, J Lope, O Hardiman… - Journal of Integrative …, 2022 - imrpress.com
Background: The cerebellum integrates a multitude of motor and cognitive processes
through ample spinal and supratentorial projections. Despite emerging evidence of adaptive …

[HTML][HTML] Altered supraspinal motor networks in survivors of poliomyelitis: A cortico-muscular coherence study

A Coffey, S Bista, A Fasano, T Buxo, M Mitchell… - Clinical …, 2021 - Elsevier
Objective Poliomyelitis results in changes to the anterior horn cell. The full extent of cortical
network changes in the motor physiology of polio survivors has not been established. Our …

Structural brain and spinal cord damage in symptomatic and pre-symptomatic VAPB-related ALS

TB Leoni, TJR Rezende, TM Peluzzo… - Journal of the …, 2022 - Elsevier
Introduction The VAPB gene is associated with fALS (fALS 8). This disease presents a
variable phenotype and no study sought to characterize its neuroanatomical abnormalities …

Brain magnetic resonance imaging (MRI) in spinal muscular atrophy: a scoping review

N Mugisha, A Oliveira-Carneiro… - Journal of …, 2023 - content.iospress.com
Background: 5q Spinal Muscular Atrophy (SMA) is a prototypical lower motor neuron
disorder. However, the characteristic early motor impairment raises the question on the …