ArticleJournal of the neurological sciences2014
ALS-Plus syndrome: non-pyramidal features in a large ALS cohort.
Article in Journal of the neurological sciences, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Motor neuron disease of paraneoplastic origin: a rare but treatable condition.Journal of neurology · 2018Pooled it
- Cognitive Impairment in Chinese Patients with Sporadic Amyotrophic Lateral Sclerosis.PloS one · 2015Trial
- Systemic brain-body metabolic coupling patterns in amyotrophic lateral sclerosis: a whole-body [European journal of nuclear medicine and molecular imaging · 2026Article
- Amyotrophic lateral sclerosis -plus patient with an intermediate-lengthFrontiers in genetics · 2026Article
- Non-motor symptoms in patients with amyotrophic lateral sclerosis: current state and future directions.Journal of neurology · 2024Review
- Misfolded alpha-synuclein in amyotrophic lateral sclerosis: Implications for diagnosis and treatment.European journal of neurology · 2024Article
- Hyperhidrosis as the initial symptom in FUS mutation-associated amyotrophic lateral sclerosis: a case report and comprehensive literature review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Review
- Dysautonomia in Amyotrophic Lateral Sclerosis.International journal of molecular sciences · 2023Review
- ALS-plus related clinical and genetic study from China.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Article
- Sensory Involvement in Amyotrophic Lateral Sclerosis.International journal of molecular sciences · 2022Review
- Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration.Neural regeneration research · 2022Review
- Article
- Primary progressive aphasia and motor neuron disease: A review.Frontiers in aging neuroscience · 2022Review
- Phenotype of VCP Mutations in Chinese Amyotrophic Lateral Sclerosis Patients.Frontiers in neurology · 2022Article
- Pathophysiological Underpinnings of Extra-Motor Neurodegeneration in Amyotrophic Lateral Sclerosis: New Insights From Biomarker Studies.Frontiers in neurology · 2021Review
- Shape Analysis of the Subcortical Nuclei in Amyotrophic Lateral Sclerosis without Cognitive Impairment.Journal of clinical neurology (Seoul, Korea) · 2020Article
- The path to biomarker-based diagnostic criteria for the spectrum of neurodegenerative diseases.Expert review of molecular diagnostics · 2020Review
- The Peripheral Nervous System in Amyotrophic Lateral Sclerosis: Opportunities for Translational Research.Frontiers in neuroscience · 2019Review
- Precision Medicine for Frontotemporal Dementia.Frontiers in psychiatry · 2019Review
- Article
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11 authors.
Funding
Abstract
objectiveAutopsy studies show widespread pathology in amyotrophic lateral sclerosis (ALS), but clinical surveys of multisystem disease in ALS are rare. We investigated ALS-Plus syndrome, an understudied group of patients with clinical features extending beyond pyramidal and neuromuscular systems with or without cognitive/behavioral deficits.
methodsIn a large, consecutively-ascertained cohort of 550 patients with ALS, we documented atypical clinical manifestations. Genetic screening for C9orf72 hexanucleotide expansions was performed in 343 patients, and SOD1, TARDBP, and VCP were tested in the subgroup of patients with a family history of ALS. Gray matter and white matter imaging was available in a subgroup of 30 patients.
resultsSeventy-five (13.6%) patients were identified with ALS-Plus syndrome. We found disorders of ocular motility, cerebellar, extrapyramidal and autonomic functioning. Relative to those without ALS-Plus, cognitive impairment (8.0% vs 2.9%, p=0.029), bulbar-onset (49.3% vs 23.2%, p<0.001), and pathogenic mutations (20.0% vs 8.4%, p=0.015) were more than twice as common in ALS-Plus. Survival was significantly shorter in ALS-Plus (29.66 months vs 42.50 months, p=0.02), regardless of bulbar-onset or mutation status. Imaging revealed significantly greater cerebellar and cerebral disease in ALS-Plus compared to those without ALS-Plus.
conclusionsALS-Plus syndrome is not uncommon, and the presence of these atypical features is consistent with neuropathological observations that ALS is a multisystem disorder. ALS-Plus syndrome is associated with increased risk for poor survival and the presence of a pathogenic mutation.
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