Research & Literature

Defining neurodegeneration on Guam by targeted genomic sequencing.

Steele JC, Guella I, Szu-Tu C, et al.

Annals of neurology 2015; (77(3)):458-68 doi:10.1002/ana.24346.

Clinical varieties and epidemiological aspects of amyotrophic lateral sclerosis in the Caribbean island of Guadeloupe: A new focus of ALS associated with Parkinsonism.

Lannuzel A, Mecharles S, Tressières B, et al.

Amyotrophic lateral sclerosis & frontotemporal degeneration 2015; (16(3-4)):216-23 doi:10.3109/21678421.2014.992026.

A unique retinal epitheliopathy is associated with amyotrophic lateral sclerosis/Parkinsonism-Dementia complex of Guam.

Steele JC, Wresch R, Hanlon SD, et al.

Movement disorders : official journal of the Movement Disorder Society 2015; (30(9)):1271-5 doi:10.1002/mds.26264.

The frontotemporal dementia-motor neuron disease continuum.

Burrell JR, Halliday GM, Kril JJ, et al.

Lancet (London, England) 2016; (388(10047)):919-31.

Seeking environmental causes of neurodegenerative disease and envisioning primary prevention.

Spencer PS, Palmer VS, Kisby GE

Neurotoxicology 2016; (56()):269-283 doi:10.1016/j.neuro.2016.03.017.

Care management in amyotrophic lateral sclerosis.

Soriani MH, Desnuelle C

Revue neurologique 2017; (173(5)):288-299 doi:10.1016/j.neurol.2017.03.031.

A critical review of the postulated role of the non-essential amino acid, β-N-methylamino-L-alanine, in neurodegenerative disease in humans.

Chernoff N, Hill DJ, Diggs DL, et al.

Journal of toxicology and environmental health. Part B, Critical reviews 2017; (20(4)):1-47 doi:10.1080/10937404.2017.1297592.

Supportive care needs of patients with amyotrophic lateral sclerosis/motor neuron disease and their caregivers: A scoping review.

Oh J, Kim JA

Journal of clinical nursing 2017; (26(23-24)):4129-4152 doi:10.1111/jocn.13945.

'Hummingbird' Sign in a Patient with Guam Parkinsonism-Dementia Complex.

Yeo T, Tan LC

Journal of movement disorders 2017; (10(3)):145-148 doi:10.14802/jmd.17025.

A migration case of Kii amyotrophic lateral sclerosis/parkinsonism dementia complex with the shortest stay in the endemic area and the longest incubation to develop the disease.

Tsunoda K, Yamashita T, Shimada H, et al.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2017; (46()):64-67 doi:10.1016/j.jocn.2017.08.057.

Motor learning in people with Parkinson's disease: Implications for fall prevention across the disease spectrum.

Paul SS, Dibble LE, Peterson DS

Gait & posture 2018; (61()):311-319 doi:10.1016/j.gaitpost.2018.01.026.

ERLIN1 mutations cause teenage-onset slowly progressive ALS in a large Turkish pedigree.

Tunca C, Akçimen F, Coşkun C, et al.

European journal of human genetics : EJHG 2018; (26(5)):745-748 doi:10.1038/s41431-018-0107-5.

Dysfunction of Protein Quality Control in Parkinsonism-Dementia Complex of Guam.

Verheijen BM, Oyanagi K, van Leeuwen FW

Frontiers in neurology 2018; (9()):173 doi:10.3389/fneur.2018.00173.

Circumstances and Signs of Approaching Death in Patients With Amyotrophic Lateral Sclerosis Undergoing Noninvasive Ventilation in Home Care Settings.

Ushikubo M

The Journal of neuroscience nursing : journal of the American Association of Neuroscience Nurses 2018; (50(3)):182-186 doi:10.1097/JNN.0000000000000362.

Cerebrovascular inflammation is associated with tau pathology in Guam parkinsonism dementia.

Majerova P, Garruto RM, Kovac A

Journal of neural transmission (Vienna, Austria : 1996) 2018; (125(7)):1013-1025 doi:10.1007/s00702-018-1883-3.

Respiratory Failure in Amyotrophic Lateral Sclerosis.

Niedermeyer S, Murn M, Choi PJ

Chest 2019; (155(2)):401-408 doi:10.1016/j.chest.2018.06.035.

The analysis of underivatized β-Methylamino-L-alanine (BMAA), BAMA, AEG & 2,4-DAB in Pteropus mariannus mariannus specimens using HILIC-LC-MS/MS.

Foss AJ, Chernoff N, Aubel MT

Toxicon : official journal of the International Society on Toxinology 2018; (152()):150-159 doi:10.1016/j.toxicon.2018.07.028.

Parkinsonian traits in amyotrophic lateral sclerosis (ALS): a prospective population-based study.

Calvo A, Chiò A, Pagani M, et al.

Journal of neurology 2019; (266(7)):1633-1642 doi:10.1007/s00415-019-09305-0.

Advance care planning in progressive neurological diseases: lessons from ALS.

Seeber AA, Pols AJ, Hijdra A, et al.

BMC palliative care 2019; (18(1)):50 doi:10.1186/s12904-019-0433-6.

Hypothesis: Etiologic and Molecular Mechanistic Leads for Sporadic Neurodegenerative Diseases Based on Experience With Western Pacific ALS/PDC.

Spencer PS

Frontiers in neurology 2019; (10()):754 doi:10.3389/fneur.2019.00754.

Clinical usefulness of scales for evaluating cognitive impairment in patients with amyotrophic lateral sclerosis.

Nagashima K, Makioka K, Fujita Y, et al.

Journal of the neurological sciences 2019; (405()):116414 doi:10.1016/j.jns.2019.07.032.

Activation of the Unfolded Protein Response and Proteostasis Disturbance in Parkinsonism-Dementia of Guam.

Verheijen BM, Lussier C, Müller-Hübers C, et al.

Journal of neuropathology and experimental neurology 2020; (79(1)):34-45 doi:10.1093/jnen/nlz110.

Top Ten Tips Palliative Care Clinicians Should Know About Amyotrophic Lateral Sclerosis.

Everett EA, Pedowitz E, Maiser S, et al.

Journal of palliative medicine 2020; (23(6)):842-847 doi:10.1089/jpm.2020.0046.

Emerging Drugs for the Treatment of Amyotrophic Lateral Sclerosis: A Focus on Recent Phase 2 Trials.

Barp A, Gerardi F, Lizio A, et al.

Expert opinion on emerging drugs 2020; (25(2)):145-164 doi:10.1080/14728214.2020.1769067.

Amyotrophic lateral sclerosis: a clinical review.

Masrori P, Van Damme P

European journal of neurology 2020; (27(10)):1918-1929 doi:10.1111/ene.14393.

Etiology of Retinal and Cerebellar Pathology in Western Pacific Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex.

Spencer PS

Eye and brain 2020; (12()):97-104 doi:10.2147/EB.S260823.

Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases.

Quinn C, Elman L

Continuum (Minneapolis, Minn.) 2020; (26(5)):1323-1347 doi:10.1212/CON.0000000000000911.

Western Pacific ALS-PDC: Evidence implicating cycad genotoxins.

Spencer PS, Palmer VS, Kisby GE

Journal of the neurological sciences 2020; (419()):117185 doi:10.1016/j.jns.2020.117185.

Kampō medicine and Muro disease (Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex): Postscript and Historical Footnote.

Spencer PS, Palmer VS, Kihira T, et al.

eNeurologicalSci 2021; (22()):100308 doi:10.1016/j.ensci.2020.100308.

Multidisciplinary care in Amyotrophic Lateral Sclerosis: a systematic review and meta-analysis.

de Almeida FEO, do Carmo Santana AK, de Carvalho FO

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2021; (42(3)):911-923 doi:10.1007/s10072-020-05011-2.

Diagnostics of Amyotrophic Lateral Sclerosis: Up to Date.

Štětkářová I, Ehler E

Diagnostics (Basel, Switzerland) 2021; (11(2)) doi:10.3390/diagnostics11020231.

A Rare Case of Amyotrophic Lateral Sclerosis With Asymmetrical Phrenic Nerve Lesion Presenting With Acute Respiratory Failure as an Initial Manifestation.

Blanco R, Pichardo J, Abdullah H

Cureus 2021; (13(1)):e12547 doi:10.7759/cureus.12547.

Exploring the experiences of living with Lewy body dementia: An integrative review.

Bentley A, Morgan T, Salifu Y, Walshe C

Journal of advanced nursing 2021; (77(12)):4632-4645 doi:10.1111/jan.14932.

Lytico-bodig in Guam: Historical links between diet and illness during and after Spanish colonization.

Giménez-Roldán S, Steele JC, Palmer VS, Spencer PS

Journal of the history of the neurosciences 2021; (30(4)):335-374 doi:10.1080/0964704X.2021.1885946.

Interventions for preventing falls and fall-related fractures in community-dwelling older adults: A systematic review and network meta-analysis.

Dautzenberg L, Beglinger S, Tsokani S, et al.

Journal of the American Geriatrics Society 2021; (69(10)):2973-2984 doi:10.1111/jgs.17375.

Parkinsonism and motor neuron disorders: Lessons from Western Pacific ALS/PDC.

Spencer PS

Journal of the neurological sciences 2022; (433()):120021 doi:10.1016/j.jns.2021.120021.

An immigrant family with Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex.

Kokubo Y, Morimoto S, Sasaki R, et al.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2022; (43(2)):1423-1425 doi:10.1007/s10072-021-05737-7.

Genotoxic Damage During Brain Development Presages Prototypical Neurodegenerative Disease.

Kisby GE, Spencer PS

Frontiers in neuroscience 2021; (15()):752153 doi:10.3389/fnins.2021.752153.

Embedded Palliative Care for Amyotrophic Lateral Sclerosis: A Pilot Program and Lessons Learned.

Fahrner-Scott K, Zapata C, O'Riordan DL, et al.

Neurology. Clinical practice 2022; (12(1)):68-75 doi:10.1212/CPJ.0000000000001124.

Development of a behavioural framework for dementia care partners' fall risk management.

Zhou Y, Berridge C, Hooyman NR, et al.

BMC geriatrics 2022; (22(1)):975 doi:10.1186/s12877-022-03620-4.

MGMT, a risk factor for both genetic and environmental forms of dementia.

Kisby GE, Oakes H, Beckett D, Spencer PS

Alzheimer's & dementia : the journal of the Alzheimer's Association 2023; (19(5)):2208-2210 doi:10.1002/alz.12900.

Palliative care principles in ALS.

Shoesmith C

Handbook of clinical neurology 2023; (191()):139-155 doi:10.1016/B978-0-12-824535-4.00007-0.

Guam ALS-PDC is a distinct double-prion disorder featuring both tau and Aβ prions.

Condello C, Ayers JI, Dalgard CL, et al.

Proceedings of the National Academy of Sciences of the United States of America 2023; (120(13)):e2220984120 doi:10.1073/pnas.2220984120.

The experiences of patients with amyotrophic lateral sclerosis of their decision-making processes to invasive home mechanical ventilation-A qualitative study.

Thorborg T, Finderup J, Winther DS, et al.

Nursing open 2023; (10(8)):5139-5148 doi:10.1002/nop2.1749.

Combined Tui na and Western medicine treatment improves pulmonary function and quality of life in patients with amyotrophic lateral sclerosis: A case report.

Li B, Liu H, Li C, et al.

Medicine 2023; (102(16)):e33612 doi:10.1097/MD.0000000000033612.

What do people affected by amyotrophic lateral sclerosis want from health communications? Evidence from the ALS Talk Project.

Genuis SK, Luth W, Bubela T, Johnston WS

Muscle & nerve 2023; (68(3)):286-295 doi:10.1002/mus.27935.

Accessing and Receiving Speech-Language Pathology Services at the Multidisciplinary Amyotrophic Lateral Sclerosis Clinic: An Exploratory Qualitative Study of Patient Experiences and Needs.

Huynh A, Adams K, Barnett-Tapia C, et al.

Journal of speech, language, and hearing research : JSLHR 2024; (67(10S)):4025-4037 doi:10.1044/2023_JSLHR-23-00087.

[Parkinson-dementia and amyotrophic lateral sclerosis association (complex of Guam). Diagnostic challenge, Mexican patient].

Aguilar-Vázquez CA, Gallardo-González LI, Raymundo-Carrillo AD, et al.

Revista medica del Instituto Mexicano del Seguro Social 2023; (61(5)):677-684 doi:10.5281/zenodo.8316479.

Exploring Advancements in the Treatment of Amyotrophic Lateral Sclerosis: A Comprehensive Review of Current Modalities and Future Prospects.

Hoxhaj P, Hastings N, Kachhadia MP, et al.

Cureus 2023; (15(9)):e45489 doi:10.7759/cureus.45489.

Prevention of Falls in Parkinson's Disease: Guidelines and Gaps.

Camicioli R, Morris ME, Pieruccini-Faria F, et al.

Movement disorders clinical practice 2023; (10(10)):1459-1469 doi:10.1002/mdc3.13860.

Brait-Fahn-Schwartz Disease: A Unique Co-Occurrence of Parkinson's Disease and Amyotrophic Lateral Sclerosis.

Aslam A, Sarmad E, Nawaz A, et al.

Case reports in neurology 2023; (15(1)):207-214 doi:10.1159/000532092.

Tau filaments from amyotrophic lateral sclerosis/parkinsonism-dementia complex adopt the CTE fold.

Qi C, Verheijen BM, Kokubo Y, et al.

Proceedings of the National Academy of Sciences of the United States of America 2023; (120(51)):e2306767120 doi:10.1073/pnas.2306767120.

Brain health: Pathway to primary prevention of neurodegenerative disorders of environmental origin.

Spencer PS, Berntsson SG, Buguet A, et al.

Journal of the neurological sciences 2025; (468()):123340 doi:10.1016/j.jns.2024.123340.

Associations Between End-Stage ALS Care and Specialty Palliative Care: A Hypothesis-Generating Study.

Lero CM, Yang A, Everett E, et al.

Muscle & nerve 2025; (71(4)):632-638 doi:10.1002/mus.28370.

Providing life-sustaining treatments at home for those with Motor Neurone Disease: A qualitative study of bereaved family members' experiences of responsibility.

Wilson E, Palmer J, Kaltsakas G, et al.

Palliative medicine 2025; (39(5)):584-593 doi:10.1177/02692163251327866.

Barriers and strategies affecting the use of assistive devices for falls prevention among people with Parkinson's disease: a qualitative pilot study.

Fernandes JB, Domingos J, Fernandes S, et al.

BMC geriatrics 2025; (25(1)):352 doi:10.1186/s12877-025-06022-4.

[Sporadic form of Guam disease (ALS-parkinsonism-dementia complex)].

Kicherova OA, Doyan YI, Kicherova KP, et al.

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova 2025; (125(7)):135-141 doi:10.17116/jnevro2025125071135.

Fall Prevention Interventions for Cognitively Impaired Older Adults: A Systematic Literature Review and Meta-Analysis.

Choi H, H Tak S, Lee D

Western journal of nursing research 2026; (48(1)):90-101 doi:10.1177/01939459251381979.

SYNE1 Deficiency Manifesting Primarily With Motor Neuron Disease.

Senghor HVF, Rubio RD, Marco C, et al.

Neurology. Genetics 2025; (11(6)):e200306 doi:10.1212/NXG.0000000000200306.

Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) features CTE-like tau seeds in brain and spinal cord.

Saez-Calveras N, Verheijen BM, Morgan N, et al.

bioRxiv : the preprint server for biology 2025; doi:10.64898/2025.12.22.696002.

Nursing a Patient With Amyotrophic Lateral Sclerosis Stage 4B With Epilepsy: A Case Study.

Zhang R, Zhang N, Li B, et al.

Nursing in critical care 2026; (31(4)):e70573 doi:10.1111/nicc.70573.