Primary Lateral Sclerosis

Abstract

: Our experience supports the observation that PLS progresses more slowly than other forms of acquired motor neuron disease, particularly amyotrophic lateral sclerosis. Follow-up data suggest that patients with active denervation changes develop greater disability.

The Stripe of Primary Lateral Sclerosis

“…However, from the existing pathological data, PLS tends to affect the precentral gyrus or primary motor cortex and may show a decreased number of Betz cells. 1 Comparatively, PLS appears to involve the primary motor cortex more so than pure ALS. 10 Neuroimaging studies have confirmed selective vulnerability of the primary motor cortex.…”

“…Clinical presentation and disease course are characterized by slowly progressive symptoms ranging from bulbar dysfunction, spastic dysarthria, dysphagia, and emotional lability to corticospinal tract findings such as limb weakness with spasticity. 1 Pure PLS represents a more benign neurodegenerative condition thought to affect only upper motor neurons and is characterized with a better prognosis than amyotrophic lateral sclerosis (ALS). 2 For the clinician, PLS is a challenging diagnosis because it can mimic several disorders such as Parkinson disease, multiple systems atrophy, corticobasal syndrome, 3 neurofilament inclusion body disease, 4 and hereditary spastic paraplegia.…”

Neuroimaging of motor neuron diseases

“…In addition, fluid-attenuated inversion recovery (FLAIR) images were slightly more sensitive in detecting CST signal alterations than T1w or T2w images (see Grosskreutz and colleagues for a review) [Grosskreutz et al 2008]. Also for the diagnosis of primary lateral sclerosis (PLS), MRI signs are described, that is, abnormalities (atrophy) in the precentral gyrus region and CST hyperintensities [Singer et al 2007]. In ALS and PLS, changes in the corpus callosum were described at individual and group level (see below).…”

Amyotrophic lateral sclerosis: Clinical heterogeneity and approaches to classification

“…Формы с преимущественным поражением верхнего мотонейрона. ПБС характеризуется изолированным поражением только верхнего мотонейрона в течение как минимум 4 лет после начала болезни [33,34]. Дан-ному варианту в отечественной классификации соот-ветствует высокая форма БАС.…”