Muscle pathology Flashcards
Thymic hyperplasia
Thymoma
associations of Myasthenia Gravis
Pathology of Myasthenia gravis
autoantibodies attach post synaptic AChRs
Diagnostics for Myasthenia Gravis
AChR antibody test Edrophonium test (rapid improvement after administration of edrophonium
Fluctuating weakness (worse on exertion) vision problems Bulbar muscle weakness Proximal limb weakness Respiratory muscle weakness Normal reflexes
Clinical presentation of Myasthenia Gravis
Neuroendocrine carcinoma of the lung
Thyroid Disease
Vitiligo
Associations of Lambert Eaton Syndrome
Pathology of Lambert Eaton Syndrome
Autoantibodies attack presynaptic calcium channels
Diagnostics for Lambert Eaton Syndrome
antibodies agianst voltage gated calcium channels
Exercise improves strength
Autonomic Dysfunction
Extremity weakness
Reduced or absent reflexes
Clinical Presentation of Lambert Eaton Syndrome
How does C. botulinum cause botulism
neurotoxin blocks the release of ACh from presynaptic nerve terminals by cleaving SNARE protein
canned foods
honey
associations of Botulism
flaccid descending paralysis Mydriasis Dysphagia Dysarthria Xerostomia NVD then constipation
Clinical presentation of Botulism
Major difference between Dermatomyositis and Polymyositis
Dermatomyositis has cutaneous involvement
Pathology of Dermatomyositis
Deposition of CD4+ T cells and MAC complex in the capillary vessels of skeletal muscle
Pathology of Polymyositis
CD8+ T cells attack skeletal muscle antigens, mostly endomysium
Which diagnostic of dermatomyositis yields the worst prognosis?
Anti M2( helicase)
Anti-M2
Anti-Jo1
Anti-P155/P140
Diagnostics suggesting Dermatomyositis
Increased CK, Aldolase, myoglobin, ESR/CRP, Anti-Jo1
Presence of ANA
Diagnostics suggestive of Polymyositis
Morphology of Dermatomyositis
perifascular atrophy of muscle fibers
perimyseal inflammation
Morphology of Polymyositis
Endomysial inflammation with CD8+ T cells
Pathologic difference in Duchenne vs Becker Muscular dystrophy
Duchenne= mutation causing total absence of dystrophin Becker= mutation causing reduced dystrophin
Diagnostic suggestive of Duchenne Muscular Dystrophy
elevated CK in first decade, then drops due to decrease in muscle mass
Differentiate the onset of Duchenne Muscular Dystrophy vs Becker Muscular Dystrophy
Duchenne= normal at birth and appears to slow down with developlment Becker= sx present in late childhood or adulthood
Differentiate the life expectancy of Duchenne Muscular Dystrophy vs Becker Muscular Dystrophy
Duchenne= death before 30 y/o Becker= Normal Life Expectancy
Which muscular dysdtophy presents with cardiac abnormalities
Duchenne
Inheritance of Muscular Dystrophy
X linked
Mode of inheritance of Myotonic Dystrophy
Autosomal Recessive
Pathology of Myotonic Dystrophy
Expansions of CTG triplet repeats at the 3’ noncoding region of the DNPK gene on chromosome 19
which set of fibers undergo atrophy in myotonic dystrophy
type 1
Clinical features of myotonic dystrophy
muscle weakness/ wasting myalgias myotonia cataracts frontal balding testicular atrophy arrhythmias
Pathology of Malignant Hyperthermia
inhales halogens cause RYR1 gene mutations, leading to increased efflux from SR
What causes fibromyalgia
abnormal ascending and descending pain pathways lead to central amplification of pain signals causes widespread musculoskeletal pain without evidence of inflammation or increase in muscle enzymes
what is used to diagnose fibromyalgia
11/18 tender points
pain primarily where muscles and tendons attach to bones HA fatigue morning stiffness unrefreshing sleep cognitive dysfunction paresthesias ANS dysfunction
Clincal findings of fibromyalgia
treatment for fibromyalgias
TCA
SNRI
Northern european descent
Giant Cell arteritis
HLA-DR4
associations of Polymyalgia Rheumatica
Diagnostics used to diagnose Polymyalgia Rheumatic
Elevated inflammatory markers Leukocytosis Normochormic anemia Normal CK Negative Rheumatoid Factor
Fever Weight loss Night sweats Fatigue Malaise Morning stiffness in neck, shoulder, and pelvic girdle subjective weakness
Clinical findings of polymyalgia rheumatica
Toxic myopathies with elevated CK
Statin use
Hypothyroidism
Which toxic myopathy yields rhabdomyolysis, renal failure and myoglobinuria
EtOH
Explain channelopathies
mutations in channel proteins cause decreased or increased excitability
hyper/hypotonia leads to varied potassium levels and periodic paralysis