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The evolving spectrum of complex inherited neuropathies.
Rossor, Alexander M; Haddad, Saif; Reilly, Mary M.
Afiliación
  • Rossor AM; Centre for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square institute of Neurology and National Hospital for Neurology and Neurosurgery.
  • Haddad S; Department of Neurology, Guys and St Thomas' Hospitals NHS Foundation Trust, UK.
  • Reilly MM; Centre for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square institute of Neurology and National Hospital for Neurology and Neurosurgery.
Curr Opin Neurol ; 37(5): 427-444, 2024 Oct 01.
Article en En | MEDLINE | ID: mdl-39083076
ABSTRACT
PURPOSE OF REVIEW Inherited peripheral neuropathies can be divided into those diseases in which peripheral neuropathy is the sole or main feature of the disease (Charcot-Marie-Tooth disease) and those in which peripheral neuropathy is just one feature of a more complex syndrome. In recent years there has been a substantial expansion in the number of genes associated with complex neuropathy syndromes. RECENT

FINDINGS:

This review will focus on emerging themes in this group of diseases, namely the increasing number of diseases due to repeat expansions; the emergence of both recessive and dominant negative alleles in the same gene producing a common phenotype and diseases in which there is selective loss of the allele from haematopoietic stem cells making genetic diagnosis on blood derived DNA problematic.

SUMMARY:

In this review we provide a practical approach to investigating and diagnosing patients with peripheral neuropathy as part of a complex syndrome and provide an updated table of the genes associated with this group of diseases.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades del Sistema Nervioso Periférico Límite: Humans Idioma: En Revista: Curr Opin Neurol Asunto de la revista: NEUROLOGIA Año: 2024 Tipo del documento: Article Pais de publicación: Reino Unido

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades del Sistema Nervioso Periférico Límite: Humans Idioma: En Revista: Curr Opin Neurol Asunto de la revista: NEUROLOGIA Año: 2024 Tipo del documento: Article Pais de publicación: Reino Unido