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Critical Illness Myopathy

critical illness myopathy
Skeletal muscle biopsy demonstrating changes of critical illness myopathy.  (Figure 1) Hematoxylin and eosin (H&E)-stained section prepared from snap frozen skeletal muscle shows atrophic muscle with a subset of atrophic pale staining type 2 muscle fibers (fiber typing stains not shown), and relatively rare scattered mild nonspecific myopathic changes are seen through the specimen.  (Figure 2) Muscle fibers have a blue tinctorial quality change on staining with Modified Gomori Trichrome (MGT).  (Figure 3) Ultrastructural examination by electron microscopy (EM) shows loss of the myosin thick filaments and preservation of thin filaments and Z-lines.  Original Magnifications:  A. Frozen H&E, 100x; B....

Hereditary Amyloid Neuropathy

TTR gene
Elderly female with amyloid neuropathy of the sural nerve. Cross-sections of the left sural nerve prepared from formalin-fixed paraffin-embedded (FFPE) tissue stained with Congo red show dense salmon-orange material (i.e. congophilic) predominantly around endoneurial blood vessels (Figure 1), as well as a few small accumulations within nerve fascicles, diagnostic of amyloid deposition.  The material shows apple-green birefringence with compensated polarized light microscopy (not shown), and also shows an intense bright red glow (Figure 2) against a dull background under UV light fluorescence microscopy with a Texas red excitation filter (i.e. rhodamine optics).  Co-localization to the same nerve fascicle, original magnifications: ...

Peripheral Nerve Myelinating Cells

Schwann Cell
Figure 1 shows a myelin sheath (arrow) with orderly myelin wrapping. No abnormality of myelin periodicity is seen (i.e. no widely spaced or uncompact myelin is observed). No morphologic evidence to suggest a paraproteinopathy is identified. Original magnification:  Electron microscopy, 20K.    A 55-year-old female patient presents with lower extremity weakness and numbness in her feet. Her medication list includes insulin. EMG/NCS showed electrodiagnostic evidence of denervation-type change.  A biopsy of the sural nerve was performed.  Neuropathologic review showed moderate to marked axonopathic changes characterized by relatively diffuse axonal loss, consistent with diabetic polyneuropathy.  After reviewing Figure 1, which cell...

Immuno-Profile of WHO Grade I Meningiomas

Hematoxylin and eosin (H&E)-stained sections with EMA+, SSTR2+, STAT6-
Immunoprofile of Meningioma, WHO grade I, Fibrous Variant.  Hematoxylin and eosin (H&E)-stained sections show a dural-based spindle cell lesion arranged in broad sweeping fascicles without atypical features, higher grade growth patterns, or tumor necrosis (Figure 1), and at higher power there is an exceedingly rare mitotic figure, far less than 1 mitotic figure per 10 high-power fields (Figure 2).  Immunohistochemical (IHC) work-up demonstrated strong diffuse membranous staining with epithelial membrane antigen (EMA+) (Figure 3), and positive immunoreactivity for somatostatin receptor 2A (SSTR2+) (Figure 4).  Original magnifications: A. H&E, 100x; B. H&E, 400x; C. EMA, 100x; and D. SSTR2, 200x.  ...

Ganglioglioma, BRAF V600E-Mutated

BRAF V600E
Young adult female with a ganglioglioma (GG) in the right temporal lobe. Figure 1 is an MRI Brain image in the T1 sequence post-contrast in the coronal plane, which demonstrates a ~4 cm multicystic mass in the medial right temporal lobe with a solid mural nodular component with heterogeneous enhancement of the solid portions and cystic rim. Calcifications are noted along with the septal and wall components (not well demonstrated in the image shown).  Figure 2 is a hematoxylin and eosin (H&E)-stained section of formalin-fixed paraffin-embedded (FFPE) tissue demonstrating a low-grade glioneuronal tumor containing a dysplastic ganglion cell with a...

Ganglioglioma, WHO grade I

Ganglioglioma tumor MRI scan
  Young adult female with headache and a cystic brain tumor in the right temporal lobe.  Figure 1 is an MRI Brain image in the T1 sequence post-contrast in the axial plane, which demonstrates a ~4 cm multicystic mass in the medial right temporal lobe with a solid mural nodular component with heterogeneous enhancement of the solid portions and cystic rim.  Calcifications are noted along with the septal and wall components (not well demonstrated in the image shown).  Figure 3 is a hematoxylin and eosin (H&E)-stained section of formalin-fixed paraffin-embedded (FFPE) tissue demonstrating a low-grade glioneuronal tumor containing dysplastic ganglion...

Lobulated Muscle Fibers with Wedge-shaped Accumulations of Mitochondria

Wedge-shaped Accumulations of Mitochondria
Figure 1 and 2: Lobulated Muscle Fibers with Wedge-shaped Accumulations of Mitochondria. Frozen sections of skeletal muscle stained with NADH-TR preparation (A) show a few scatted type 1 lobulated muscle fibers (black arrows) with coarse internal architecture staining with intensely dark staining wedge-shaped or triangular-shaped accumulations along the periphery of the muscle fiber. A few targetoid muscle fibers are also seen.  Ultrastructural examination confirms that these small accumulations are composed of mitochondria with one such aggregate demonstrated (black arrow).  Original magnifications: A. Frozen Section NADH-TR, 100x; B. Electron microscopy, 1500x.   Considering the clinical vignette from the prior week: An...

4th of July Muscle Fibers

4th of July Muscle Fibers
Neuro Notes is celebrating Old Glory this Independence Day with Red, White, and Blue Muscle Fibers!  Happy 4th of July. Patriotic Red, White, and Blue Muscle Fibers in Celebration of Independence Day, Happy 4th of July! Snap frozen skeletal muscle stained with routine hematoxylin and eosin (H&E), Periodic acid-Schiff with diastase digestion (PASD), and reduced nicotinamide adenine dinucleotide-tetrazolium reductase (NADH-TR), each at 200x original magnification.

Lobulated Muscle Fibers

Lobulated Muscle Fibers
Figure 1: Hereditary myopathy with lobulated muscle fibers in an elderly male presenting with myalgias. The photomicrograph shows snap frozen skeletal muscle stained with the NADH-TR preparation demonstrating variably sized variably well-developed muscle fibers with irregular basement membrane contours and myofibrillar disorganization due to small triangular or wedge-shaped subsarcoplasmic areas of intensely dark staining character, consistent with small accumulations of mitochondria (also known as so-called “lobulated muscle fibers”).  Some of the lobulated (trabeculated) muscle fibers are in small groups or scattered as single individual myofibers throughout the specimen.  Original magnification: A. NADH-TR, 100.   Considering the clinical vignette from last...

Acquired Etiologies of Myopathy with Lobulated Muscle Fibers

Myopathy with Lobulated Muscle Fibers
An elderly male patient presents with a chief complaint of “dull achy pain.” The patient relates that the pain is predominantly in his thighs and hip girdle region, most intense in his left lateral thigh. He says the pain has ongoing and progressive for several months but has significantly worsened over the last few days.  The patient had been recently admitted for “rhabdomyolysis” thought due to his statin medication, which was discontinued; however, no muscle biopsy was performed at that time and his muscle enzymes showed CPK 934.  Other past medical history includes diabetes mellitus, hyperlipidemia, hypertension, hypercalcemia, and vitamin...