B6.074 Prework: Endocrine Myopathies Flashcards

1
Q

myopathy

A

any abnormal state of striated muscle

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2
Q

source of muscle disorders associated with endocrine abnormalities

A

interactions between the force generating and metabolic functions of skeletal muscle

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3
Q

endocrine abnormalities that can lead to myopathy

A

adrenal dysfunction - Cushing, steroid myopathy
thyroid dysfunction - myxedema coma or thyrotoxic myopathy
parathyroid dysfunction - MEN
pituitary dysfunction
Islands of Langerhans dysfunction - diabetic myopathy from ischemic infarction of the femoral muscles

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4
Q

symptoms of myopathy

A

muscle weakness, pain, cramps, tenderness, and spasms of various degrees

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5
Q

epidemiology of endocrine myopathies

A

increasingly recognized

corticosteroid myopathy is most common

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6
Q

recognition of endocrine myopathies

A

patients frequently complain of fatigue and weakness
usually other multisystem signs and symptoms (myopathy rarely the presenting symptoms)
myopathy as sole manifestation may have delayed diagnosis

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7
Q

diagnosis of endocrine myopathy

A

lack of histologic and electrophysiologic criteria

often muscle atrophy without degeneration

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8
Q

components of the endocrine system

A
hypothalamus
pituitary
thyroid
parathyroid
thymus
adrenals
pancreas
ovaries
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9
Q

hypothalamus

A

regulates hunger, thirst, sleep, and wakefulness plus most of involuntary mechanisms including temperature

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10
Q

pituitary

A

controls all other endocrine glands

influences growth, metabolism, and regeneration

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11
Q

thyroid

A

regulates energy and metabolism

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12
Q

parathyroid

A

secretes hormones necessary for calcium absorption

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13
Q

thymus

A

helps build resistance to disease

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14
Q

adrenals

A

secrete hundreds of compounds including cortisone and adrenaline which helps you react to emergencies
regulates metabolic processes in the cells, water balance, BP, etc

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15
Q

ovaries

A

influences how your blood circulates and determines your mental vigor and sex drive

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16
Q

metabolism

A

conversion of nutrients into energy and building materials to meet your body’s needs

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17
Q

etiologies of hypoadrenalism

A

infection
inflammatory disease
tumor
may follow pituitary failure

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18
Q

findings associated with hypoadrenalism

A

neuro manifestations such as disturbances of behavior and mentation are prominent
myopathy is not likely to be a presenting finding

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19
Q

factors contributing to myopathy in hypoadrenalism

A

circulatory insufficiency
fluid and electrolyte imbalance
impaired carb metabolism
starvation

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20
Q

etiologies of hyperadrenalism

A

pituitary or ectopic overproduction of ACTH
adrenal tumors
exogenous steroid administration

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21
Q

pituitary ACTH hypersecretion

A

Cushing disease

caused by corticotroph microadenoma in 90% of patients

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22
Q

signs of a hormone secreting tumor on physical exam

A

neck flexor weakness
dysphagia
respiratory muscle weakness
muscle stretch reflexes are usually present (even in weak muscles) but may be depressed

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23
Q

Addison disease

A

long term insufficient function of the adrenal cortex leading to underproduction of corticosteroids

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24
Q

ACTH stimulation test

A

evaluation of cortisol after IV injection of ACTH
normal individual should have an increase in cortisol
patient with adrenal insufficiency will have no response or a limited one

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25
Q

causes of Addison disease

A

US - autoimmune destruction of adrenal glands

worldwide - tuberculous adrenalitis

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26
Q

primary adrenal deficiency

A

adrenal gland themselves are destroyed so the patient becomes deficient in cortisol and aldosterone
uncommon

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27
Q

secondary adrenal insufficiency

A

adrenal failure caused by a lack of ACTH stimulation from the pituitary
caused by disease of the pituitary OR commonly by chronic exogenous admin of steroids, which suppress the H-P-A axis
R-A-A system usually maintains aldosterone, so patient is only deficient in cortisol

28
Q

Cushing Syndrome

A

chronic endogenous hyper-cortisolism

chronic, autonomous and excessive secretion of cortisol from the adrenal glands

29
Q

most common cause of Cushing Syndrome

A

70% cause by pituitary tumor producing excess ACTH

30
Q

Cushing comorbidities

A
metabolic syndrome (systemic arterial hypertension, visceral obesity, impairment of glucose metabolism, and dyslipidemia)
MSK disorders (myopathy, osteoporosis, fractures)
neuropsychiatric disorders (impairment of cognitive function, depression, mania)
impairment of repro and sexual function
derm (acne, hirsutism, alopecia)
31
Q

symptoms of hypothyroidism

A
weight gain
neuropathy
fatigue
dry skin
cold intolerance
bradycardia
sleepiness
emotional disturbances
muscle stiffness, weakness, and pain (may be main or only presenting symptoms)
32
Q

physical exam findings of hypothyroidism

A

motor movements have reduced velocity
delayed relaxation of muscle stretch reflexes
median neuropathy at wrist (carpal tunnel)
myoedema or muscle enlargement rarely occur

33
Q

severe muscular association with hypothyroidism

A

rhabdomyolysis

weakness, myalgia, markedly elevated CK and myoglobinuria

34
Q

thyrotoxic myopathy

A

disturbance in the function of muscle fibers from increased mitochondrial activity, accelerated protein degradation and lipid oxidation, and enhanced B adrenergic sensitivity
due to excess thyroid hormone

35
Q

symptoms of hyperthyroidism

A
weight loss
sweating
tremor
muscle wasting
painless weakness
ocular symptoms
myalgia
cramps
36
Q

pathophysiology of hypoparathyroidism

A

deficiency of PTH or inability of hormone to have effect at end receptors

37
Q

symptoms of hypoparathyroidism

A
tetany, with or without carpopedal spasm
muscle pain
cramps
mild weakness
short stature
rounded face
bony abnormalities
neuro symptoms
38
Q

physical exam findings with hypoparathyroidism

A

tetany is common
cataracts may be present
increased intracranial pressure, not constant but possible

39
Q

pathophysiology of hyperparathyroidism

A

over secretion of PTH, frequently from parathyroid adenoma

myopathy results from altered TH level and impaired action of vit D

40
Q

symptoms of hyperparathyroidism

A

painful bones
renal stones
GI groans
pscyh moans (depression, mentation defects, memory loss, mood changes)

41
Q

physical exam findings in hyperparathyroidism

A

myopathy is prominent

symmetric weakness of the proximal limbs and atrophy

42
Q

myopathy due to pituitary disease

A

may be a result of secondary adrenal dysfunction and/or other endocrine disturbances such as thyroid dysfunction

43
Q

symptoms of hypopituitarism

A
amenorrhea
loss of libido
alabaster skin
lethargy
constipation
cold intolerance
44
Q

symptoms of hyperpituitarism

A

infertility
impotence
headaches
mass effects of the tumor

45
Q

etiology of polymyalgia rheumatics and temporal arteritis

A

age related changes in the neuroendocrine system could represent a pathogenic factor in genetically disposed individuals

46
Q

hyper parathyroid myopathy sex

A

female to male ratio 2:1

47
Q

hyperthyroid myopathy sex

A

female to male ratio 1:1

48
Q

Iatrogenic steroid myopathy sex

A

female to male ratio 2:1

49
Q

hypothyroid myopathy sex

A

female to male ratio 5:1

50
Q

Cushing myopathy sex

A

female to male ratio 3:1

though depends on etiology

51
Q

hyper parathyroid myopathy age

A

40-60

52
Q

hyperthyroid myopathy age

A

20-60

53
Q

hypothyroid myopathy age

A

incidence increases after 40

54
Q

Cushing myopathy age

A

20-40

55
Q

history of endocrine myopathy in general

A

proximal > distal
with or without pain, cramps, spasms
symmetric
atrophy may or may not be present

56
Q

CK levels in endocrine myopathy

A

may be normal or elevated

hypothyroidism: usually elevated
hyperthyroidism: usually normal

57
Q

screening for hypothyroidism

A
serum TSH (elevated)
free T3, T4 (low)
58
Q

EMG findings in endocrine myopathy

A

may reveal myopathy, but a normal exam does not rule out
EMG preferentially studies type I units, so disease processes that involving type II units may reveal no abnormalities on EMG (steroid myopathy)

59
Q

EMG findings with myopathic process

A

polyphasic motor unit potentials
shortened duration of motor unit potentials
decreased amplitude of motor unit potentials

60
Q

EMG in hypothyroidism

A

differentiated delayed muscle relaxation from myotonia

61
Q

EMG in hyperthyroidism

A

abnormalities more proximal

myopathic type

62
Q

EMG in hyperparathyroidism

A

myopathic motor unit potentials and increased polyphasic potentials without spontaneous activity
if severe, may have fasciculations and a reduced recruitment pattern with normal nerve conduction velocities

63
Q

muscle biopsy in endocrine myopathy

A

considered mainly to exclude other treatable or congenital muscle diseases
variable findings, rarely specific

64
Q

diagnostic histo features of myopathies

A

absence of neurogenic abnormalities
necrotic muscle fibers
basophilic (regenerating myofibers)
fibrosis of the endomysium

65
Q

treatment of endocrine myopathy

A

correction of underlying endocrine dysfunction (surg or med)

B clockers may improve muscle strength, esp in resp muscles

66
Q

prognosis of endocrine myopathy

A

depends on underlying disease process
often abates with correction of underlying disease
prolonged weakness and partial recovery are common especially in severe cases and in patients with delayed or suboptimal treatment