Latest Research in Hereditary Neurometabolic Disorders

119 research papers · 2026 median publication year

Top Research Topics in Hereditary Neurometabolic Disorders

Highest-Cited Papers

  1. Population-specific genetic variants and related health outcomes in a cohort of Inuit children in Nunavut
  2. Neuroradiologic Findings in Patients With S CN2A Disease
  3. Cramps as the initial presentation of CANVAS/RFC1-related disorder
  4. Duodenal fluid analysis of 13 patients with progressive familial intrahepatic cholestasis type 2 from a single institution
  5. Clinical and genotypic characterization of SETD5-related disorders using Human Phenotype Ontology-based profiling
  6. CIDP in Association With CMT : The Importance of Repeated History Taking and Clinical Examination in the Monitoring of Genetic Neuropathy
  7. Expert Delphi consensus on the diagnosis and management of Hypophosphatasia in the Gulf Cooperation Council region
  8. Adult X-linked hypophosphatemia: multisystem morbidity and no evidence of genotype–phenotype correlation – insights from a Brazilian cohort
  9. Involvement of Reduced ENPP1 Function in the Pathogenesis of Ossification of the Posterior Longitudinal Ligament
  10. The Multifaceted Role of the FLNC Gene in Hereditary Cardiomyopathies and Prognosis
  11. Expanding the genotypic and phenotypic spectrum of PGAP1 deficiency: clinical and functional insights from 15 patients
  12. Novel genetic variants and atypical phenotypes in pediatric progressive familial intrahepatic cholestasis
  13. Nav1.5 Beyond Genetics: Loss of Cardiac Sodium-Channel Function in Brugada Syndrome
  14. The Future of Genetic Therapy for Inherited Cardiomyopathy
  15. The changing landscape of Fabry disease: Impact of the inclusion of the GLA-gene in broader NGS or WES based panels on the phenotypic spectrum
  16. Sociodemographic and Clinical Profile of Adult Males With Duchenne Muscular Dystrophy
  17. Report on the 7th Ottawa International Conference on Neuromuscular Disease & Biology – September 11‐13, 2025, Ottawa, Canada
  18. Ketone metabolism defects in childhood: a spectrum of overlapping presentations and clinical features
  19. Burosumab as bridging therapy in autosomal dominant hypophosphatemic rickets with femoral fracture nonunion and iron deficiency
  20. Progressive dilated cardiomyopathy in a patient with previously unrecognized Klinefelter syndrome: a case report

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L2 Region - - 2026 Sep Q3

Hereditary Neurometabolic Disorders

119 papers

Top Topics (10)

Hereditary Neurological Disorders7
Muscle Physiology and Disorders6
Lysosomal Storage Disorders Research5
Genomics and Rare Diseases4
Alkaline Phosphatase Research Studies4
Parathyroid Disorders and Treatments4
Metabolism and Genetic Disorders4
Glycogen Storage Diseases and Myoclonus4
Genetic Neurodegenerative Diseases3
Cardiomyopathy and Myosin Studies3

Top Publications (20)

1.Population-specific genetic variants and related health outcomes in a cohort of Inuit children in Nunavut2.Neuroradiologic Findings in Patients With S CN2A Disease3.Cramps as the initial presentation of CANVAS/RFC1-related disorder4.Duodenal fluid analysis of 13 patients with progressive familial intrahepatic cholestasis type 2 from a single institution5.Clinical and genotypic characterization of SETD5-related disorders using Human Phenotype Ontology-based profiling6.CIDP in Association With CMT : The Importance of Repeated History Taking and Clinical Examination in the Monitoring of Genetic Neuropathy7.Expert Delphi consensus on the diagnosis and management of Hypophosphatasia in the Gulf Cooperation Council region8.Adult X-linked hypophosphatemia: multisystem morbidity and no evidence of genotype–phenotype correlation – insights from a Brazilian cohort9.Involvement of Reduced ENPP1 Function in the Pathogenesis of Ossification of the Posterior Longitudinal Ligament10.The Multifaceted Role of the FLNC Gene in Hereditary Cardiomyopathies and Prognosis11.Expanding the genotypic and phenotypic spectrum of PGAP1 deficiency: clinical and functional insights from 15 patients12.Novel genetic variants and atypical phenotypes in pediatric progressive familial intrahepatic cholestasis13.Nav1.5 Beyond Genetics: Loss of Cardiac Sodium-Channel Function in Brugada Syndrome14.The Future of Genetic Therapy for Inherited Cardiomyopathy15.The changing landscape of Fabry disease: Impact of the inclusion of the GLA-gene in broader NGS or WES based panels on the phenotypic spectrum16.Sociodemographic and Clinical Profile of Adult Males With Duchenne Muscular Dystrophy17.Report on the 7th Ottawa International Conference on Neuromuscular Disease & Biology – September 11‐13, 2025, Ottawa, Canada18.Ketone metabolism defects in childhood: a spectrum of overlapping presentations and clinical features19.Burosumab as bridging therapy in autosomal dominant hypophosphatemic rickets with femoral fracture nonunion and iron deficiency20.Progressive dilated cardiomyopathy in a patient with previously unrecognized Klinefelter syndrome: a case report

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