1. Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum
- Author
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John R. Hodges, Smriti Agarwal, Margaret C. Zoing, Matthew C. Kiernan, Thanuja Dharmadasa, James Howells, José Manuel Matamala, Rebekah M. Ahmed, Jashelle Caga, Kazumoto Shibuya, Elizabeth Highton-Williamson, Nimeshan Geevasinga, and Steve Vucic
- Subjects
0301 basic medicine ,Male ,medicine.medical_specialty ,Neurology ,medicine.medical_treatment ,Kaplan-Meier Estimate ,Neuropsychological Tests ,03 medical and health sciences ,Disability Evaluation ,0302 clinical medicine ,Cognition ,Internal medicine ,medicine ,Dementia ,Humans ,Amyotrophic lateral sclerosis ,Motor Neuron Disease ,Primary Lateral Sclerosis ,Aged ,business.industry ,Amyotrophic Lateral Sclerosis ,Motor Cortex ,Motor neuron ,Middle Aged ,medicine.disease ,Survival Analysis ,Transcranial Magnetic Stimulation ,Transcranial magnetic stimulation ,030104 developmental biology ,medicine.anatomical_structure ,Frontotemporal Dementia ,Cardiology ,Disease Progression ,Female ,Neurology (clinical) ,business ,030217 neurology & neurosurgery ,Frontotemporal dementia ,Motor cortex - Abstract
To investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis–frontotemporal dementia (ALS–FTD) spectrum of diseases. Comprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n = 27) and ALS–FTD (n = 12). Clinical features, Addenbrooke’s Cognitive Examination (ACE) scores, Motor Neuron Disease Behaviour (Mind-B) scores, motor disability on the ALS functional rating scale (ALSFRS) and survival times were documented. Motor cortex excitability was evaluated using transcranial magnetic stimulation (TMS). Global cognition was impaired in PLS (mean total ACE score 82.5 ± 13.6), similar to ALS–FTD (mean total ACE score 76.3 ± 7.7, p > 0.05) while behavioural impairments were not prominent. TMS revealed that resting motor threshold (RMT) was significantly higher in PLS (75.5 ± 6.2) compared ALS–FTD (50.1 ± 7.2, p
- Published
- 2018