<prompt> All are Congenital Myopathies, Except:Central-core MyopathyNemaline MyopathyZ band MyopathyCentro-nuclear Myopathy  <response>: Answer is C Z band Myopathy: Z band myopathy is not classified as a congenital myopathy. Nemaline myopathy, Central-core myopathy and Centro-nuclear Myotubular myopathy are all examples of congenital mvopathie.s Congenital Myopathies These are a group of rare disorders distinguished from muscular dystrophies by the presence of specific histochemical and structural abnormalities in muscle. Onset in early life primarily disorders of infancy childhood, but may present in adulthood Non progressive or slowly progressive course Proximal or generalized muscle weakness Hipotonia Disease and Inheritance Gene and Locus Clinical Findings logic Findings Central core disease; Ryanodine receptor-1 RYRI gene; Early-onset hypotonia and non Cytoplasmic cores are lightly Autosomal-dominant 19q 13.1 progressive weakness; associated skeletal deformities; may develop malignant hypehermia eosinophilic and distinct from surrounding sarcoplasm; Found only in type I fibers, which usually predominate, best seen on NADH stain Nemaline myopathy; Autosomal-dominant Weakness, hypotonia and Aggregates of subsarcolemmal Autosoma-dominant or N EMI -- Tropomyosin 3 delayed motor development in spindle shaped paicles Autosomal-recessive TPM3 gene; childhood; may also be seen in nemaline rods; occur Autosomal-recessive NEM2 -- nebulin NEB gene; 2q22 adults; usually nonprogressive; involves proximal limb muscles most predominantly in type 1 fibers; derived from Z-band material a-actinin and best seen on Autosomal-dominant or recessive -- skeletal muscle actin, a chain severely; skeletal abnormalities may be present modified Gomori stain ACTA I gene; 1q42.1 Myotubular myopathy X-linked-myotubularin MTM1 X-linked form presents in Abundance of centrally located Centronuclear; gene; Xq28 infancy with prominent nuclei involving the makority of X-linked MTMI, Autosomal recessive, or Autosomal-dominant hypotonia and poor prognosis; autosomal forms have limn weakness and are slowly progressive; autosomal recessive form is intermediate in severity and prognosis muscle fibers; central nuclei are usually confined to type I fibers, which are small in diameter, but can occur in both fiber types er uncommon types include minicore myopathy, fingerprint body myopathy and sarcotubular myopathy. 