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This 17-year-old elite endurance athlete underwent muscle biopsy to evaluate for recent inability to perform at the level they could six-months ago. In the context of this individual’s clinical history what is the best explanation for the changes seen on light and electron microscopy? A. Inclusion Body Myositis B. Vacuolar Myopathy C. Polymyositis D. Athletic Conditioning #NeuroNotes #neuropath #pathology #neuromuscularpath

Clinical History: - History: 68-year-old man with 1 week of bilateral proximal upper and lower extremity weakness and 1 month of facial, eyebrow, neck, chest, and back rash (initially diagnosed as seborrheic dermatitis). - PMH: HTN, PVD, asthma, hyperlipidemia, GERD, type 1 DM with retinopathy, IgA deficiency, hypothyroidism, former light smoker. - FH: Stroke, diabetes, arthritis, cancer, macular degeneration, heart disease. - Meds: Atorvastatin, insulin, levothyroxine, zolpidem; treated with Solu-Medrol and prednisone. - Physical exam: Mild-moderate proximal weakness, lower extremity edema. - MRI: Diffuse heterogeneous edema of bilateral quadriceps, adductors, and sartorius, suspicious for inflammatory myopathy. - Labs: CK peaked at 1570, trending down to 680; aldolase 20; mildly elevated CRP; ANA, Lyme, and RF negative; HMGCR Ab, MG panel, extended myositis and ENA panels pending. What is the most likely direct mechanism of muscle injury? A. T-cell mediated inflammation of myofibers B. B-cell mediated inflammation of myofibers C. Metabolic myopathy D. Ischemia #NeuroNotes #neuropath #pathology #neuromuscularpath

65-year-old woman with progressive proximal weakness, pain, fasciculations and malaise. Physical exam: decreased proximal strength and decreased reflexes. EMG/NCS: severe demyelinating sensorimotor polyneuropathy. What is the diagnosis? A.) Guillain-Barré syndrome B.) Chronic inflammatory demyelinating polyneuropathy C.) Lepromatous leprosy D.) Acute inflammatory demyelinating polyneuropathy

This 65-year-old patient presented with a two-month history of progressive proximal upper and lower extremity muscle weakness, and photosensitive skin rash involving their cheeks and lower neck. Their past medical history was significant for Rheumatoid arthritis. Home medications included prednisone and leflunomide. Muscle biopsy was performed to evaluate for a myopathy. What is your diagnosis? #NeuroNotes #neuropath #pathology #neuromuscularpath #neurox

This 40-year-old patient underwent sural nerve biopsy to evaluate for a painful mixed large fiber and small fiber axonal neuropathy. The biopsy showed light microscopic features of a chronic active axonopathic process. Neurofilament immunohistochemical stain performed on a FFPE tissue section showed diffuse significant loss of small diameter unmyelinated axons. What are the structures indicated by arrows in Image #1? Once you identify these you will notice multiple other such structures in the image. Image #2 is what normal would look like for comparison. #NeuroNotes #neuropath #pathx #neuromuscular

Which genetic test is required to establish the diagnosis in this 59-year-old with distal muscle weakness, mild myotonia, and persistently elevated CPK values (200-300 U/L)? A.) PCR fragment analysis of CNBP B.) Muscular dystrophy gene panel  C.) PCR fragment analysis of DPMK D.) Myositis specific antibody panel #NeuroNotes #neuropath #pathology #neuromuscularpath #neurotwitter #neurox

Here is the clinical history for a 71 year old man with proximal weakness: - Past medical history: advanced esophageal adenocarcinoma status post radiation and chemo/immunotherapy with immune mediated hepatitis, and moderate alcohol use. - Medications: nivolumab, FOLFOX chemotherapy (fluorouracil, folinic acid, and oxaliplatin), and prednisone. - Physical exam: strength: bilateral mild proximal upper and severe lower extremity weakness; reflexes: silent in bilateral lower extremities; sensation decreased in feet and hands; no mandatory. - Imaging: White matter volume loss and microvascular injury; no definite cord or neural foraminal compression. Lab results: highest CK 20,000 currently trending downward. What agent is primarily responsible based on the reference images? A. Nivolumab B. Fluorouracil C. Oxaliplatin D. Prednisone #NeuroNotes #neuropath #pathology #neuromuscularpath

The 50 y/o patient was admitted for shortness of breath and acute muscle weakness following a viral illness. Laboratory studies showed elevated CPK (1200-1400 range). The patient was treated with steroid prior to muscle biopsy. No myotoxic medications were noted in the patient's home medication list. What is your pathologic diagnosis? #NeuroNotes #neuropath #pathology #neuromuscularpath