Research & Literature

Clinical and genetic heterogeneity in hereditary spastic paraplegias: from SPG1 to SPG72 and still counting.

Klebe S, Stevanin G, Depienne C

Revue neurologique 2015; (171(6-7)):505-30.

Primary Lateral Sclerosis.

Statland JM, Barohn RJ, Dimachkie MM, et al.

Neurologic clinics 2015; (33(4)):749-60.

Primary Lateral Sclerosis and Early Upper Motor Neuron Disease: Characteristics of a Cross-Sectional Population.

Fournier CN, Murphy A, Loci L, et al.

Journal of clinical neuromuscular disease 2016; (17(3)):99-105 doi:10.1097/CND.0000000000000102.

Safety and efficacy of botulinum toxin A for the treatment of spasticity in amyotrophic lateral sclerosis: results of a pilot study.

Vázquez-Costa JF, Máñez I, Alabajos A, et al.

Journal of neurology 2016; (263(10)):1954-60 doi:10.1007/s00415-016-8223-z.

Best Practices for Intrathecal Baclofen Therapy: Patient Selection.

Saulino M, Ivanhoe CB, McGuire JR, et al.

Neuromodulation : journal of the International Neuromodulation Society 2016; (19(6)):607-15 doi:10.1111/ner.12447.

Efficacy and safety of oral baclofen in the management of spasticity: A rationale for intrathecal baclofen.

Ertzgaard P, Campo C, Calabrese A

Journal of rehabilitation medicine 2017; (49(3)):193-203 doi:10.2340/16501977-2211.

Demographics and clinical characteristics of primary lateral sclerosis: case series and a review of literature.

Ramanathan RS, Rana S

Neurodegenerative disease management 2018; (8(1)):17-23 doi:10.2217/nmt-2017-0051.

Atypical Initial Presentation of Painful Muscle Cramps in a Patient with Amyotrophic Lateral Sclerosis: A Case Report and Brief Review of the Literature.

Kuzel AR, Lodhi MU, Syed IA, Rahim M

Cureus 2017; (9(11)):e1837 doi:10.7759/cureus.1837.

Respiratory muscle testing in amyotrophic lateral sclerosis: a practical approach.

Sferrazza Papa GF, Pellegrino GM, Shaikh H, et al.

Minerva medica 2018; (109(6 Suppl 1)):11-19 doi:10.23736/S0026-4806.18.05920-7.

Primary lateral sclerosis: a distinct entity or part of the ALS spectrum?

Finegan E, Chipika RH, Shing SLH, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2019; (20(3-4)):133-145 doi:10.1080/21678421.2018.1550518.

A neuropsychological and behavioral study of PLS.

de Vries BS, Spreij LA, Rustemeijer LMM, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2019; (20(5-6)):376-384 doi:10.1080/21678421.2019.1620284.

Primary lateral sclerosis (PLS) functional rating scale: PLS-specific clinimetric scale.

Mitsumoto H, Chiuzan C, Gilmore M, et al.

Muscle & nerve 2020; (61(2)):163-172 doi:10.1002/mus.26765.

Primary lateral sclerosis: consensus diagnostic criteria.

Turner MR, Barohn RJ, Corcia P, et al.

Journal of neurology, neurosurgery, and psychiatry 2020; (91(4)):373-377 doi:10.1136/jnnp-2019-322541.

Primary lateral sclerosis: diagnosis and management.

Turner MR, Talbot K

Practical neurology 2020; (20(4)):262-269 doi:10.1136/practneurol-2019-002300.

TDP-43 pathology in primary lateral sclerosis.

Mackenzie IRA, Briemberg H

Amyotrophic lateral sclerosis & frontotemporal degeneration 2020; (21(sup1)):52-58 doi:10.1080/21678421.2020.1790607.

Evolving diagnostic criteria in primary lateral sclerosis: The clinical and radiological basis of "probable PLS".

Finegan E, Li Hi Shing S, Siah WF, et al.

Journal of the neurological sciences 2020; (417()):117052 doi:10.1016/j.jns.2020.117052.

The NEALS primary lateral sclerosis registry.

Paganoni S, De Marchi F, Chan J, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2020; (21(sup1)):74-81 doi:10.1080/21678421.2020.1804591.

Rehabilitation in primary lateral sclerosis mimicking parkinsonism: A case report.

Moggio L, Petraroli A, Marotta N, et al.

NeuroRehabilitation 2020; (47(4)):381-386 doi:10.3233/NRE-201527.

Utility of intrathecal baclofen pump in primary lateral sclerosis.

Zubair AS, Raymond M, Patwa HS

Journal of the neurological sciences 2021; (420()):117227 doi:10.1016/j.jns.2020.117227.

A case of vertebral artery compression syndrome mimicking primary lateral sclerosis.

Cai H, Zhang B, Huang B, et al.

The International journal of neuroscience 2022; (132(12)):1178-1181 doi:10.1080/00207454.2020.1867853.

Neurophysiological features of primary lateral sclerosis.

de Carvalho M, Kiernan MC, Pullman SL, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2020; (21(sup1)):11-17 doi:10.1080/21678421.2020.1837174.

Neuroimaging in primary lateral sclerosis.

Pioro EP, Turner MR, Bede P

Amyotrophic lateral sclerosis & frontotemporal degeneration 2020; (21(sup1)):18-27 doi:10.1080/21678421.2020.1837176.

Measuring disease progression in primary lateral sclerosis.

Gilmore M, Elman L, Babu S, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2020; (21(sup1)):59-66 doi:10.1080/21678421.2020.1837179.

Clinical care and therapeutic trials in PLS.

Floeter MK, Warden D, Lange D, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2020; (21(sup1)):67-73 doi:10.1080/21678421.2020.1837180.

Natural History of "Pure" Primary Lateral Sclerosis.

Hassan A, Mittal SO, Hu WT, et al.

Neurology 2021; (96(17)):e2231-e2238 doi:10.1212/WNL.0000000000011771.

Estimation of the prevalence and incidence of motor neuron diseases in two Spanish regions: Catalonia and Valencia.

Barceló MA, Povedano M, Vázquez-Costa JF, et al.

Scientific reports 2021; (11(1)):6207 doi:10.1038/s41598-021-85395-z.

Effect of one-year dextromethorphan/quinidine treatment on management of respiratory impairment in amyotrophic lateral sclerosis.

Sancho J, Ferrer S, Burés E, et al.

Respiratory medicine 2021; (186()):106536 doi:10.1016/j.rmed.2021.106536.

The Upper Motor Neuron-Improved Knowledge from ALS and Related Clinical Disorders.

Menon P, Vucic S

Brain sciences 2021; (11(8)) doi:10.3390/brainsci11080958.

European expert consensus on improving patient selection for the management of disabling spasticity with intrathecal baclofen and/or botulinum toxin type A.

Biering-Soerensen B, Stevenson V, Bensmail D, et al.

Journal of rehabilitation medicine 2022; (54()):jrm00241 doi:10.2340/16501977-2877.

A Case Report on Robot-Aided Gait Training in Primary Lateral Sclerosis Rehabilitation: Rationale, Feasibility and Potential Effectiveness of a Novel Rehabilitation Approach.

Portaro S, Ciatto L, Raciti L, et al.

Innovations in clinical neuroscience 2021; (18(4-6)):15-19.

Clinical and genetic spectra of 1550 index patients with hereditary spastic paraplegia.

Méreaux JL, Banneau G, Papin M, et al.

Brain : a journal of neurology 2022; (145(3)):1029-1037 doi:10.1093/brain/awab386.

Does limited EMG denervation in early primary lateral sclerosis predict amyotrophic lateral sclerosis?

Hassan A, Mittal SO, Hu WT, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2022; (23(7-8)):554-561 doi:10.1080/21678421.2022.2039714.

Iron-sensitive MR imaging of the primary motor cortex to differentiate hereditary spastic paraplegia from other motor neuron diseases.

Cosottini M, Donatelli G, Ricca I, et al.

European radiology 2022; (32(12)):8058-8064 doi:10.1007/s00330-022-08865-6.

Pathological laughter and crying in neurological disorders: recognition and treatment.

Husbands E, Talbot K

Practical neurology 2022; (22(6)):486-490 doi:10.1136/pn-2021-003301.

Structural magnetic resonance imaging findings and histopathological correlations in motor neuron diseases-A systematic review and meta-analysis.

Zejlon C, Nakhostin D, Winklhofer S, et al.

Frontiers in neurology 2022; (13()):947347 doi:10.3389/fneur.2022.947347.

Progressive motor neuron syndromes with single CNS lesions and CSF oligoclonal bands: never forget solitary sclerosis!

Giacopuzzi Grigoli E, Cinnante C, Doneddu PE, et al.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2022; (43(12)):6951-6954 doi:10.1007/s10072-022-06407-y.

Motor Band Sign in Primary Lateral Sclerosis: A Case Report Proposing the Need for an Imaging Biomarker.

Valaparla VL, Lobaina M, Patel C, Patel AV

Cureus 2023; (15(3)):e36121 doi:10.7759/cureus.36121.

Genetic characterization of primary lateral sclerosis.

de Boer EMJ, de Vries BS, Pennings M, et al.

Journal of neurology 2023; (270(8)):3970-3980 doi:10.1007/s00415-023-11746-7.

Guideline "Motor neuron diseases" of the German Society of Neurology (Deutsche Gesellschaft für Neurologie).

Petri S, Grehl T, Grosskreutz J, et al.

Neurological research and practice 2023; (5(1)):25 doi:10.1186/s42466-023-00251-x.

Effects of Aided Communication on Communicative Participation for People With Amyotrophic Lateral Sclerosis.

Peters B, Wiedrick J, Baylor C

American journal of speech-language pathology 2023; (32(4)):1450-1465 doi:10.1044/2023_AJSLP-22-00346.

Primary lateral sclerosis plus parkinsonism: a case report.

Moturu A, Welch W

BMC neurology 2023; (23(1)):312 doi:10.1186/s12883-023-03360-x.

Non-motor symptoms in motor neuron disease: prevalence, assessment and impact.

Beswick E, Forbes D, Johnson M, et al.

Brain communications 2024; (6(1)):fcad336 doi:10.1093/braincomms/fcad336.

Primary lateral sclerosis: application and validation of the 2020 consensus diagnostic criteria in an expert opinion-based PLS cohort.

Witzel S, Micca V, Müller HP, et al.

Journal of neurology, neurosurgery, and psychiatry 2024; (95(8)):737-747 doi:10.1136/jnnp-2023-333023.

Acceptance and Commitment Therapy plus usual care for improving quality of life in people with motor neuron disease (COMMEND): a multicentre, parallel, randomised controlled trial in the UK.

Gould RL, McDermott CJ, Thompson BJ, et al.

Lancet (London, England) 2024; (403(10442)):2381-2394 doi:10.1016/S0140-6736(24)00533-6.

The Role of Gastrostomy and Noninvasive Ventilation in Primary Lateral Sclerosis.

Oliveira Santos M, Domingues S, Simão S, et al.

Muscle & nerve 2025; (71(3)):450-456 doi:10.1002/mus.28346.

Pseudobulbar affect: clinical associations, social impact and quality of life implications - Lessons from PLS.

Finegan E, Kleinerova J, Hardiman O, et al.

Journal of neurology 2025; (272(4)):266 doi:10.1007/s00415-025-12971-y.

Progression and life expectancy in primary lateral sclerosis.

Lester DG, Thompson AG, Talbot K, Turner MR

Journal of neurology, neurosurgery, and psychiatry 2025; (96(10)):1008-1011 doi:10.1136/jnnp-2025-336037.

Primary Lateral Sclerosis: Implications for Diagnostic Criteria From a Natural History Study in the Netherlands.

de Vries BS, de Boer EMJ, Brugman F, et al.

Neurology 2025; (104(11)):e213461 doi:10.1212/WNL.0000000000213461.

Differentiating upper- and lower motor neuron diseases using automated acoustic analysis.

Truong J, Simmatis L, Pommée T, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2025; (26(7-8)):691-701 doi:10.1080/21678421.2025.2506444.

Toward therapeutic trials in primary lateral sclerosis.

Scirocco E, Allen MD, Giacomelli E, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2025; (26(7-8)):623-630 doi:10.1080/21678421.2025.2527123.

Intrathecal Baclofen to Improve Functional Status in ALS: A Case Report.

Pressler MP, Cooper PS, Carter W, et al.

Pain medicine case reports 2023; (7(4)):201-203.

The Epidemiology of Primary Lateral Sclerosis: Results from a Population-Based Cohort.

Vasta R, Matteoni E, Pellegrino G, et al.

Annals of neurology 2026; (99(3)):606-613 doi:10.1002/ana.78105.

Efficacy and safety of dextromethorphan/quinidine in treating pseudobulbar affect in neurological disorders: A systematic review and dose-classified network meta-analysis.

Muneer MA, Tariq I, Zulfiqar E, et al.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2026; (47(1)):159.

Primary lateral sclerosis in Brazil: phenotypic heterogeneity, non-motor features, and prognostic markers in a 17-year multicentre cohort.

Marques Couto C, Vidal Watkins C, Silva Carvalho R, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2026; 1-10 doi:10.1080/21678421.2026.2652325.

Amyotrophic Lateral Sclerosis: A Review.

Ravits J, Ferrey D, Gundogdu B, et al.

JAMA 2026; (335(22)):1970-1982 doi:10.1001/jama.2026.6385.

Primary Lateral Sclerosis French National Diagnostic and Care Protocol.

Corcia P, Bernard E, de la Cruz E, et al.

Revue neurologique 2026; (182(6)):555-562 doi:10.1016/j.neurol.2026.04.006.

Diagnostic Revision From Primary Lateral Sclerosis to Amyotrophic Lateral Sclerosis: A Cohort Study.

de Boer EMJ, Willemse SW, Veldink JH, et al.

Neurology 2026; (106(12)):e218055 doi:10.1212/WNL.0000000000218055.

Early Cognitive and Behavioral Changes in Primary Lateral Sclerosis: A Population-Based Study.

Calvo A, Moglia C, Canosa A, et al.

European journal of neurology 2026; (33(8)):e70720 doi:10.1111/ene.70720.