Neuromuscular

CENTRONUCLEAR (MYOTUBULAR) MYOPATHY

Centronuclear myopathy: Infantile

A single central nucleus in many muscle fibers


 
From: T Mozaffar

H&E stain
H&E stain

H&E stain

H&E stain
Toluidine blue stain

Toluidine blue stain

Toluidine blue stain

Toluidine blue stain
Electron microscopy from T Mozaffar

Type I (light) muscle fibers tend to be smaller than type II.
Note clear regions in center of some fibers.


ATPase stain, pH 9.4
ATPase stain, pH 9.4

Abnormal internal architecture.
Small muscle fibers are darker staining
Note clear rim around edge of many smaller fibers.


NADH stain
NADH stain




NADH stain

Centronuclear myopathy: Childhood


H&E stain
H&E stain
Note central nuclei in several smaller muscle fibers.
Central regions in other fibers are basophilic.

NADH stain
ATPase stain, pH 9.4
Abnormal internal architecture.
Some fibers have central dark staining.
Others have coarse internal architecture.
 Type I (light) muscle fibers tend
to be smaller than type II.
Note clear regions in center of
some fibers.

Centronuclear myopathy: Juvenile
H+E
H&E stain 		ATPase, pH 9.4
ATPase stain, pH 9.4
NADH stain
Central nuclei in several smaller
muscle fibers.
 Type I (light) muscle fibers are
generally smaller than type II
(dark) fibers.
Note clear regions in center of
type I fibers.
 Abnormal internal architecture
in smaller (type I) muscle fibers.


Also see: Congenital fiber type size disproportion


Return to Centronuclear myopathy

4/30/2008