Distal_hereditary_motor_neuropathy

Distal hereditary motor neuronopathies

Distal hereditary motor neuronopathies

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Distal hereditary motor neuronopathies (distal HMN, dHMN), sometimes also called distal hereditary motor neuropathies, are a genetically and clinically heterogeneous group of motor neuron diseases that result from genetic mutations in various genes and are characterized by degeneration and loss of motor neuron cells in the anterior horn of the spinal cord and subsequent muscle atrophy.[citation needed]

Although they can hardly be distinguished from hereditary motor and sensory neuropathies on the clinical level, dHMNs are considered a separate class of disorders.[citation needed]

Another common system of classification groups many of DHMNs under the heading of spinal muscular atrophies.[citation needed]

Classification

In 1993, A. E. Hardnig proposed to classify hereditary motor neuropathies into seven groups based on age at onset, mode of inheritance, and presence of additional features. This initial classification has since been widely adopted and expanded and currently looks as follows:[1][2]

More information Type, OMIM ...

Note: Acronym HMN is also used interchangeably with DHMN.

See also


References

  1. Irobi, J; De Jonghe, P; Timmerman, V (2004). "Molecular genetics of distal hereditary motor neuropathies". Human Molecular Genetics. 13 (suppl 2): R195–R202. doi:10.1093/hmg/ddh226. PMID 15358725.
  2. Scherer, SS (2006). "Finding the causes of inherited neuropathies". Archives of Neurology. 63 (6): 812–6. doi:10.1001/archneur.63.6.812. PMID 16769861.

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