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. Author manuscript; available in PMC: 2013 Sep 22.
Published in final edited form as: Amyotroph Lateral Scler Frontotemporal Degener. 2013 May;14(0 1):5–18. doi: 10.3109/21678421.2013.778548

Table I.

ALS classifications.

Phenotype Molecular Pathology Genetics
Typical ALS
Bulbar/pseudobulbar ALS Limb onset variants:

  • Typical limb onset

  • Flail arm or bibrachial ALS

  • Flail leg

  • Polyneuritic variant

  • Hemiplegic ALS (Mill’s variant)

ALS with associated FTD or impairment of higher cortical function
Primary lateral sclerosis
Progressive muscular atrophy		 TDP-43 proteinopathy (ubiquitinated pathology)

  • Non- C9ORF72 related

  • C9ORF72 related

SOD1 proteinopathy
FUS proteinopathy (basophilic inclusion body disease (BIBD))		 Sporadic ALS
Familial ALS, incl.:

  • C9ORF72

  • FUS

  • OPTN

  • SOD1

  • TARDBP

  • UBQLN

  • VCP