1.3ÿÿÿÿÿÿCASE 11^Q@¡$€¡IØ$4! ž¬˜~1<¢¢˜z¢˜Ì¡›lXPW¾››lXPW¾›lXPW¾¡xlXzXVËdØ$šf0¡¡MƒðÜÑ¡ 1qô¢`þÞÒÆ¡g"(¡¸ÿÃÇB(¡˜ JEp****g¥peLfveLPhPgPSvÿÿÿÿ¡xÿÿÿÿ¡@*K4u¡p5\Œ¡˜þ¡¨ eLgÄ¡4¾H5\Œfvfh¡PgbPgÄ¡°fv ñfgÄÿýÐPPSvPhPgº¹“Ä7¹“Ä7ºeiðgXag`˜ç58 year-old male with 3-4 year history of leg muscle atrophy and progressive weakness. Patient has an uncle with a muscular dystrophy. CPK=175. EMG findings are suggestive of an inflammatory myopathy. Scroll Down For Diagnosis4 Diagnosis: Inclusion Body Myositis (IBM). the following is adapted from: http://www.genetics.gla.ac.uk/neil/ibmdx.html Clinical features of IBM The weakness is of long duration (> 6 months) Age of onset > 30 years old Muscle weakness * Must affect proximal and distal muscles of arms and legs and * Patient must exhibit at least one of the following features: 1. Finger flexor weakness 2. Wrist flexor > wrist extensor weakness 3. Quadriceps muscle weakness ( = or < grade 4 MRC) The symptoms are usually unresponsive to steroid therapy Laboratory features Serum creatine kinase < 12 times normal Muscle biopsy 1. Inflammatory myopathy characterized by mononuclear cell invasion of nonnecrotic muscle fibers 2. Vacuolated muscle fibers 3. Either * Intracellular amyloid deposits (must use fluorescent method of identification before excluding the presence of amyloid) or * 15-18-nm tubulofilaments by electron microscopy or multiple SMI-31 positive cytoplasmic filamentous inclusions (corresponding to the tubulofilaments seen on EM) Iomega Tools°õìÂBDºCASE 11.html¾¹“Ä!TEXTMOSSCASE 11ºèÂMIomega Tools:Muscle & Nerve:Neuromuscular-Pathology HTML:CASE 11:CASE 11.html ¨¨afpm9Yu•žMR5James MandellÕs Office ComputerIomega ToolsJim Mandell.iomegaDriverSCSI_0.4 v6.0.4.Iomega Tools l,ö¡ð°õìÂº¾ÿÿÿÿÿÿIomega Tools°õìÂBDºS00-24173-IBM-H/ELOWparaf.jpg¼¹“¾÷JPEGPxraCASE 11ºèÂ^Iomega Tools:Muscle & Nerve:Neuromuscular-Pathology HTML:CASE 11:S00-24173-IBM-H/ELOWparaf.jpg ¨¨afpm9Yu•žMR5James MandellÕs Office ComputerIomega ToolsJim Mandell.iomegaDriverSCSI_0.4 v6.0.4.Iomega Tools l,ö¡ð°õìÂº¼ÿÿÿÿÿÿÐH&E low power of paraffin section. Note multifocal endomysial (within the muscle fascicle) lymphocytic infiltrates, involving non-necrotic fibers. What is the differential diagnosis with this image alone?|o¤¹“¾ø¹“Ä"}€Iomega Tools°õìÂBDºS00-24173-IBM-H/E40x.jpg»¹“¾¶JPEGPxraCASE 11ºèÂYIomega Tools:Muscle & Nerve:Neuromuscular-Pathology HTML:CASE 11:S00-24173-IBM-H/E40x.jpg ¨¨afpm9Yu•žMR5James MandellÕs Office ComputerIomega ToolsJim Mandell.iomegaDriverSCSI_0.4 v6.0.4.Iomega Tools l,ö¡ð°õìÂº»ÿÿÿÿÿÿ„H&E frozen section, high power. The myofiber in center has several rimmed vacuoles (holes lined by a crusty blue basophilic rim). Ä{¹“¾·¹“Ä#}€Iomega Tools°õìÂBDºS00-24173-IBM-SMI31 40x.jpg½¹“¿2JPEGPxraCASE 11ºèÂ\Iomega Tools:Muscle & Nerve:Neuromuscular-Pathology HTML:CASE 11:S00-24173-IBM-SMI31 40x.jpg ¨¨afpm9Yu•žMR5James MandellÕs Office ComputerIomega ToolsJim Mandell.iomegaDriverSCSI_0.4 v6.0.4.Iomega Tools l,ö¡ð°õìÂº½ÿÿÿÿÿÿtImmunohistochemistry with antibody SMI-31, phosphorylated neurofilaments Note dense positive cytoplasmic inclusion.ôÁ ‚¹“¿2¹“Ä$}€