Evidence mapPaperPMID 41511639Full record

ArticleJournal of neurology2026

Clinical trajectories and genetic profiles of SOD1-related amyotrophic lateral sclerosis: insights from a single-center cohort in India.

Muddasu Suhasini Keerthipriya, Ananthapadmanabha Kotambail, Madhusudhan Deekshitha, R Mahima, M B Ramyashree, Bhoomika M Rao, Purbasha Biswas, Dipti Baskar, Peneti Balaji, Mugdha Mehta and 11 more

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Article in Journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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0cells of the map it votes in
1citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

21 authors.

Muddasu Suhasini Keerthipriya *Department of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Ananthapadmanabha Kotambail *Department of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Madhusudhan DeekshithaDepartment of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
R MahimaDepartment of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
M B RamyashreeDepartment of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Bhoomika M RaoDepartment of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Purbasha BiswasDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Dipti BaskarDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Peneti BalajiDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Mugdha MehtaDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Olivia GrayVariant Bio Inc, Seattle, USA.
Kaja A WasikVariant Bio Inc, Seattle, USA.
Anne-Katrin EmdeVariant Bio Inc, Seattle, USA.
Kiran PolavarapuChildren's Hospital of Eastern Ontario (CHEO) Research Institute, Ottawa, Canada.
Veeramani Preethish-KumarNeurofoundation, Salem, Tamil Nadu, India.
Pradeep ReddySUNY Upstate Medical University, New York, USA.
Priya Treesa ThomasDepartment of Psychiatric Social Work, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Saraswati NashiDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Gautham ArunachalDepartment of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Seena VengalilDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
Atchayaram NaliniDepartment of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India. atchayaramnalini@yahoo.co.in.ORCID http://orcid.org/0000-0001-9791-3639

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Mutations in the superoxide dismutase 1 (SOD1) gene are a predominant, genetic cause of amyotrophic lateral sclerosis (ALS). Given the marked variability in SOD1 variant prevalence and clinical manifestations across global populations, this study aimed to characterize the genetic and clinical profile of SOD1-associated ALS (SOD1-ALS) in a large cohort of Indian patients. Whole-exome sequencing (WES) was performed for the retrospective cohort, along with comprehensive bioinformatic analyses and interpretation of genetic variants. Data were analyzed using descriptive statistics and Kaplan-Meier survival analysis to assess clinical and survival outcomes. Among 765 individuals who underwent WES, 37 probands (4.8%) from 33 families were identified with SOD1-ALS, representing a substantial 24.2% of familial ALS (fALS) cases. Patients showed a male preponderance (1.64:1) with a mean age at onset of 41.9 ± 13.1 years. Analysis revealed 23 distinct pathogenic/likely pathogenic SOD1 variants, including four novel variants. Remarkably, a high frequency of homozygous variants (6 patients) were observed in the cohort, which were associated with earlier disease onset. Most patients presented with a lower limb onset (67.6%) and a lower motor neuron phenotype. Survival was noted to be prolonged in carriers of H47R, V88M, and I152N variants, while those with juvenile onset showed reduced survival. In conclusion, this study provides the first comprehensive characterization of SOD1-ALS in the Indian population, revealing a distinct genetic profile with a unique spectrum of SOD1 variants and a higher prevalence of homozygous cases. These detailed genotype-phenotype correlations contribute significantly to the genetic etiology of ALS.

Indexed as

Amyotrophic Lateral SclerosisSuperoxide Dismutase-1AdultAgedAge of OnsetCohort StudiesExome SequencingFemaleHumansIndiaMaleMiddle AgedMutationRetrospective StudiesYoung AdultSOD1 protein, humanSuperoxide Dismutase-1Amyotrophic lateral sclerosisExome sequencingFamilial ALSGenetic variantsGenotype–phenotype correlationSOD1

Identifiers

PMID41511639

What Socratic holds

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.