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

Data in Brief

Mary Clare McKenna¹  Russel L. McLaughlin²  Peter Bede³  Alice Vajda³  Colette Donaghy³  Jennifer C. Hengeveld³  Mark A. Doherty⁴  Kai Ming Chang⁴  We Fong Siah⁴  Rangariroyashe H. Chipika⁴  Stacey Li Hi Shing⁴  Eoin Finegan⁴  Orla Hardiman⁵  Siobhan Hutchinson⁶ 

[1] Electronics and Computer Science, University of Southampton, Southampton, United Kingdom;Social Care Trust, Belfast, United Kingdom;Complex Trait Genomics Laboratory, Smurfit Institute of Genetics, Trinity College Dublin, Ireland;Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland;Department of Neurology, St James's Hospital, Dublin, Ireland;;Department of Neurology, Western Health &

关键词: Primary lateral sclerosis;    Neuroimaging;    MRI;    Upper motor neuron;   

DOI  :  

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

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