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General features Infantile Childhood Juvenile Necklace fibers Abnormal fiber types Punctate, Small fibers |
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![]() 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 |
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![]() ATPase stain, pH 9.4 |
![]() ATPase stain, pH 9.4 |
![]() NADH stain |
![]() NADH stain |
![]() ![]() ![]() NADH stain |
Centronuclear myopathy: Childhood![]() H&E stain |
![]() H&E stain |
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Central nuclei in
several smaller muscle fibers. Central regions in other fibers are basophilic. |
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![]() NADH stain |
![]() ATPase stain, pH 9.4 |
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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. Clear regions in center of type 1 fibers. |
Centronuclear myopathy: Juvenile with Necklace fibers |
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![]() H&E stain |
![]() ATPase stain, pH 9.4 |
![]() NADH stain |
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Central nuclei in several smaller muscle fibers. |
Type I (light) muscle fibers are generally smaller than type II (dark) fibers. Clear regions in center of type I fibers. |
Abnormal internal architecture in smaller (type I) muscle fibers. |
![]() H&E stain |
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Bimodal distribution of muscle fiber sizes Central nuclei, especially in smaller fibers Clefts in center of other muscle fibers ![]() H&E stain |
![]() Gomori trichrome stain |
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Central nuclei, especially in smaller fibers Clefts in center of other muscle fibers Rings (Necklaces) in some muscle fibers ![]() Gomori trichrome stain |
![]() NADH stain Necklace fibers |
![]() ATPase pH 9.4 stain |
Most small muscle fibers are type 1![]() ATPase pH 4.3 stain |
![]() Esterase stain |
Centronuclear myopathy: Juvenile with many Central nuclei & Type 2C fibers |
![]() H&E stain |
Many muscle fibers have single central nuclei![]() H&E stain |
![]() VvG stain |
![]() NADH stain Abnormal internal architecture around central nuclei |
![]() ATPase pH 9.4 stain |
![]() ATPase pH 4.3 stain Many abnormal, intermediate-staining (type 2C), muscle fibers |
Centronuclear myopathy: Juvenile with Small muscle fibers & Punctate central NADH stain |
![]() H&E stain |
Many muscle fibers have single central nuclei![]() H&E stain stain |
![]() NADH stain Punctate central staining in many muscle fibers |
ATPase pH 9.4 stain |
Type 1 muscle fibers are smaller than typ2 2
ATPase pH 4.3 stain |