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Imaging and clinical data indicate considerable disease burden in ‘probable’ PLS: Patients with UMN symptoms for 2–4 years

2020; Elsevier BV; Volume: 32; Linguagem: Inglês

10.1016/j.dib.2020.106247

2352-3409

Eoin Finegan, We Fong Siah, Stacey Li Hi Shing, Rangariroyashe H. Chipika, Kai‐Ming Chang, Mary Clare McKenna, Mark A. Doherty, Jennifer C. Hengeveld, Alice Vajda, Colette Donaghy, Siobhán Hutchinson, Russel McLaughlin, Orla Hardiman, Peter Bede,

Alzheimer's disease research and treatments

Primary lateral sclerosis (PLS) is an adult-onset upper motor neuron disease manifesting in progressive spasticity and gradually resulting in considerably motor disability. In the absence of early disease-specific diagnostic indicators, the majority of patients with PLS face a circuitous diagnostic journey. Until the recent publication of consensus diagnostic criteria, 4-year symptom duration was required to establish the diagnosis. The new diagnostic criteria introduced the category of 'probable PLS' for patients with a symptom duration of 2–4 years. "Evolving diagnostic criteria in primary lateral sclerosis: The clinical and radiological basis of "probable PLS" [1]. This dataset provides radiological metrics in a cohort of 'probable PLS' patients, 'definite PLS' patients and age-matched healthy controls. Region-of-interest radiological data include diffusivity metrics in the corticospinal tracts and corpus callosum as well as mean cortical thickness values in the pre- and para-central gyri in each hemisphere. Our data indicate considerable grey matter and relatively limited white matter involvement in 'probable PLS' which supports the rationale for this diagnostic category as a clinically useful entity. The introduction of this diagnostic category will likely facilitate the timely recruitment of PLS patients into research studies and pharmacological trials before widespread neurodegenerative change ensues.