ANS Disorders 2

Question 1

MSA: stands for? aka? first sign?

Answer 1

multipe system atrophy, shy drager syndrome. impotence sometimes first sign.

Question 2

MSA: two other signs? defective what?

Answer 2

orthostatic hypothension. hoarseness due to vocal cord paralysis. defective baroreceptor modulation of BP.

Question 3

MSA: degeneration of? results in? loss of what neurons?

Answer 3

degeneration of many CNS areas, alterations in motor + sensory function esp. balance. loss of pregang symp neurons in IML cell column. loss of pregang parasymp neurons in onuf’s nucleus (sacral) + vagal nuclei

Question 4

MSA: lose what to NTs? antibodies?

Answer 4

loss of DA + NA centrally. potentially autoimmune: antibodies in CSF of MSA pts react specifically with locus coeruleus

Question 5

MSA: prognosis? 3 complications?

Answer 5

poor: 8.5 years. aspiration, apnea, dysrhythmias

Question 6

autonomic disorders in SCI: what?

Answer 6

cervical + high thoracic transection sympathetic outflow, and sacral parasymp outflow separated from central control

Question 7

SCI: 7 things that happen in acute phase? how long does it last?

Answer 7

spinal hypoexcitability. spinal shock. paralysis. loss of spinal autonomic reflexes. atony of bladder + bowel. dilation of blood vessels. hypotension. 2 days - 2 weeks.

Question 8

SCI: chronic phase: effects depend on? main effect?

Answer 8

level of lesion. high lesion = lowest blood pressure. postural hypotension.

Question 9

chronic phase SCI: what NT changes? what compensates?

Answer 9

decrease plasma NA = cutaneous vasodilation, hypotherimia. increase renin, aldosterone, vasopressin to help maintain BP

Question 10

SCI: bladder?

Answer 10

becomes neurogenic: descending inhibition normally impairs bladder reflexes

Question 11

SCI: stimuli/irritation below lesion causes? why?

Answer 11

autonomic dysreflexia: extreme hypertension due to contraction of resistance + capacitance vessels, because of uncontrolled spinal reflex below level of lesion

Question 12

autonomic dysreflexia: what happens below/above lesion? cause?

Answer 12

sweating + dilation of cutaneous vessels above level of lesion. bradycardia baroreceptor reflex preserved above lesion - increased alpha receptor sensitivity following decreased sympathetic tone

Question 13

familial dysautonomia: aka? what?

Answer 13

riley day syndrome. inherited developmental abnormality of sensory sympathetic and some parasympathetic neurons

Question 14

familial dysautonomia: what chromosome? what population? age?

Answer 14

chromosome 9. confined to ashkenazi jewish population. neurological function deteriorates with age.

Question 15

familial dysautonomia: 3 abnormal things?

Answer 15

abnormal histamine test/no axon reflex. decrease response to pain/temp. orthostatic hypotension without compensatory tachycardia.

Question 16

familial dysautonomia: absence of? what instead? disease progression?

Answer 16

absence of adrenergic fibers. autonomic nerves might secrete DA instead of NA. disease can’t be arrested.

Question 17

familial dysautonomia: emotional crisis?

Answer 17

leads to autonomic crisis: vomiting, excessive sweating, erythematous blotching, hypertension, tachycardia

Question 18

Hirshsprung’s disease: what? prevelance?

Answer 18

congenital megacolon, aganglionosis, no ENS, hypertrophy of afferent + efferent fibers to and from gut. 1/5 000 live births.

Question 19

Hirshsprung’s disease: differential diagnosis? ENS development?

Answer 19

always considered when diagnosing obstructive bowel disease in infants. ENS develops as vagal neural crest cells populate gut, often a transition zone in gut below which no ENS cells are found

Question 20

Hirshsprung’s disease: lose what receptors (2)?

Answer 20

RET tyrosine kinase receptor for GDNF, endothelin receptor