Objective: Sporadic amyotrophic lateral sclerosis (sALS) is a fatal neurodegenerative
disorder affecting upper and lower motor neurons. In view of the heterogeneous …

Primary lateral sclerosis (PLS), previously undescribed in children, is characterized by a
spastic motor deterioration and pathologic demonstration of corticospinal tract degeneration …

Upper and lower motor neuron pathologies are critical to the autopsy diagnosis of
amyotrophic lateral sclerosis (ALS). Further investigation is needed to determine how the …

Among the motor neuron diseases, three share the clinical features of prominent upper
motor neuron signs–amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS) and …

We examined the phosphorylated 43-kDa TAR DNA-binding protein (pTDP-43) inclusions
as well as neuronal loss in full-length spinal cords and five selected regions of the central …

Objective The pallidonigroluysian (PNL) system, the primary component of corticosubcortical
circuits, is generally spared in amyotrophic lateral sclerosis (ALS). We evaluated the …

Objective To report a kindred with an association between hereditary primary lateral
sclerosis (PLS) and progressive nonfluent aphasia. Patients and methods Six members from …

To determine the significance of TAR DNA binding protein 43 kDa (TDP-43) pathology in
amyotrophic lateral sclerosis (ALS), we examined the whole brains and spinal cords of 57 …

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the
corticomotorneuronal network responsible for voluntary movement. There are well …

Motor neuron disease is a degenerative condition involving both upper motor neurons
(UMN) and lower motor neurons (LMN). While amyotrophic lateral sclerosis (ALS) is an …