Significance of CK-elevation in noncompaction with regard to cardiac and neuromuscular disease

Neuromuscular disorders and non compaction: How much is the strength of the association and how can it be suspected?

“…In the same report the authors described a neurological involvement in more than 50% of patients [1].…”

“…In the last report the authors reported a significant increase of the CK plasmatic level in patients with non compaction, and in particular an association between CK elevation and neuromuscular disorders [1]. It could be interesting to know if CK elevation was the MB, BB or MM isoform.…”

“…In many reports Finsterer and Stöllberger reported a strong association between non compaction of the left ventricle and neuromuscular disorders [1][2][3][4][5].…”

“…Specific aetiology of non compaction of left ventricle remains poorly understood, and the major genetic determinants are unknown [4].A genetic heterogeneity had been described, and it was present in specific neuromuscular disorders, but none of the genetic variants showed a strong association with the morphological aspect [1][2][3][4][5][6][7][8][9][10].…”

“…A correlation between neuromuscular disorders and cardiac non compaction is present, even if, until today, genetic involvement has not been identified clearly [1][2][3][4][5][6][7][8][9][10]. However, in our opinion, an estimated incidence of 50% of neuromuscular disorders in this population of patients could be too exaggerated [5][6][7][8][9][10].…”