The document discusses the autonomic nervous system and its disorders. It begins by defining the autonomic nervous system and dividing it into the sympathetic and parasympathetic nervous systems. It then discusses methods of assessing autonomic function, including heart rate variation tests, Valsalva maneuver, quantitative sudomotor axon reflex test, and sympathetic skin response. Next, it covers autonomic disorders like reflex syncope, postural tachycardia syndrome, and functional gastrointestinal disorders. Finally, it discusses autonomic storms and Takotsubo cardiomyopathy, which result from excessive autonomic outflow.

1. D R A M R U T A R A J A M A N Y A
D N B N E U R O L O G Y T R A I N E E
K M C M A N G A L O R E
Autonomic system and its disorders

2.  The term autonomic nervous system, meaning “self-
driven,” refers to an intellectually convenient but
physiologically artificial division of the neuraxis.
 The innervation of each end-organ is highly tailored
to a balance between the primary needs of the organ
itself and the importance of some degree of control
in daily function.

3. I. Divisions
A. Sympathetic
• fight or flight response
B. Parasympathetic
• rest and digestion

4. I. Involuntary Motor System
A. Autonomic vs. Somatic motor
systems
1. Somatic
• voluntary
• direct synapse
• excitatory
2. Autonomic
• involuntary
• disynaptic (preganglion,
postganglion)
• Excitatory and inhibitory III

5. General nerve pathways
A. Sympathetic
• Preganglion cell body – gray matter
• axons move through ventral root of spinal
nerve
• synapse w/ postganglion at sympathetic
chain ganglion
• axons of postganglions exit via Spinal nerve
 Exceptions:some pre do not
synapse at symp chain
 A. Splanchnic nerve
• axons of preganglion exit Splanchnic nerve and
synapse at collateral ganglion
 B. w/post Adrenal gland preganglion synapses directly
w/adrenal

6. B.Parasympathetic
• cell bodies of preganglion –
brainstem (nuclei) and sacral
region of spinal cord
• axons move through cranial
nerves and through spinal
nerves
• synapse w/ postganglion at
ganglia near or in the target

7. IV.Signal transmission
A. Sympathetic
• Preganglion secretes
Acetylcholine (Cholinergic)
• Postganglion – receptor =
Nicotinic
• Postganglion secretes
Norepinephrine (Adrenergic)
B.Parasympathetic
•Preganglion secretes Acetylcholine
(Cholinergic)
•Postganglion – receptor = nicotinic
•Postganglion secretes Acetylcholine
•Target (Smooth muscle, heart,
glands) receptor = muscarinic

12. ASSESSMENT OF AUTONOMIC FUNCTION

13. Heartratevariation
duringrespiration
 The variation of heart rate with respiration is known
as sinus arrhythmia
 Inspiration increases the heart rate Expiration
decreases the heart rate
 This is also called Respiratory Sinus Arrhythmia
(RSA)
 This is an index of vagal control of heart rate

14. SinusArrhythmia
Due to changes in vagal control of heart rate during respiration
Probably due to following mechanisms
- Influence of respiratory centre on the vagal control of heart rate
- Influence of pulmonary stretch receptors
on the vagal control of heart rate

15.  Record maximum and minimum heart rate with
each respiratory cycle
 Average the 3 differences
 - Normal > 15 beats/min
 - Borderline = 11-14 beats/min
 - Abnormal < 10 beats/min
 E:I ratio = longest RR interval (expiration)
shortest RR interval (inspiration)
Normal E:I ratio = 1.2

16. Heartratevariationduring
posturalchange
 Changing posture from supine to standing leads to
an increase in heart rate immediately, usually by 10-
20 beats per minute
 On standing the heart rate increases until it
reaches a maximum at about 15t beat (shortest
R-R interval after standing)
 - after which it slows down to a stable state at
about 30th beat

17.  The ratio of R-R intervals corresponding to the 30th
and 15th heart beat 30:15 ratio
 30:15 ratio = RR interval at 30th
RR interval at 15th beat
 This ratio is a measure of parasympathetic response
 Normal > 1.04 = 1.01-1.04
 Abnormal =<1.00

18. Valsalva Maneuver
 The Valsalva maneuver consists of respiratory strain
which increases intrathoracic and intraabdominal
pressures and alters hemodynamic and cardiac
functions
 Valsalva maneuver evaluates
 - 1. sympathetic adrenergic functions using the
blood pressure responses
 - 2. cardiovagal (parasympathetic)
 functions using the heart rate responses

19. Protocol of Valsalva Ratio
The patient is supine or with head slightly elevated to about 30°.
• Most labs have the patient strain against 40 mmHg applied for 15 s by blowing into a mouthpiece
attached to a sphygmomanometer.
• The system should have a slow leak to ensure the patient strains continuously
Following cessation of the Valsalva strain, the patient relaxes and breathes at a normal comfortable rate.
The ECG is monitored during the strain and 30-45 s following its release.
The maximal heart rate of phase II actually occurs about 1 s following cessation of the strain
The minimal heart rate occurs about 15-20 s after releasing the strain.
The ratio of the maximal-to-minimal heart rate is determined as a simple ratio. •
After a brief rest, the maneuver is repeated until three ratios are determined.

20.  4 phases
 - Phase I
 - Phase II
 - Phase III
 - Phase IV

21. PhaseI-Onsetofstraining
 Transient increase in BP which lasts for a few
seconds
 - HR does not change much

22. PhaseII-Phaseofstraining
 Early part - drop in BP lasting for about 4 seconds
Latter part - BP returns to normal

23. Mechanism
 Early part
 - venous return decreases with compression of veins by increased
intrathoracic pressure central venous pressure decreases BP decreases
 • Latter part
 - drop in BP in early part will stimulate baroreceptor reflex increased
sympathetic activity increased peripheral resistance increased BP ( returns to
normal )
 • Heart rate increase steadily throughout this phase due to vagal withdrawal in
early part & sympathetic activation in latter part

24. Phase III - Release of straining
 Transient decrease in BP lasting for a few seconds
 • Little change in heart rate
 Mechanical displacement of blood into pulmonary
vascular bed, which was under increased intrathoracic
pressure BP decreases

25. Phase IV - further release of strain
 BP slowly increases and heart rate
proportionally decreases
 • BP overshoots
 • Occurs 15-20 s after release of strain and lasts
for about a minute or more
 Due to increase in venous return, stroke volume
and cardiac output increases
♦ Phase I Decrease in BP
♦ Phase II Decrease in BP, Tachycardia
♦ Phase III Decrease in BP
♦ Phase IV Overshoot of BP, Bradycardia

26. ValsalvaRatio
 Measure of the change of heart rate that takes place during a brief
period of forced expiration against a closed glottis
 Ratio of longest R-R interval during phase IV (within 20 beats of
ending maneuver) to the shortest R-R interval during phase II
 Average the ratio from 3 attempts
 Values
 • more than 1.21 normal
 • less than 1.20 abnormal

27. absent indulation of heart rate in a 37-year-old diabetic patient. during strain,
there is no increase of heart rate; after release of the strain. there is no reflex:
brad/cardia

28. Coldpressortest
 Submerge the hand in ice cold water
This increases
 - systolic pressure by about 20 mmHg
 - diastolic pressure by 10 mmHg

29. Thermoregulatory Sweat Test
 The TST is a sensitive semiquantitative test of
sweating
 After a color indicator (quinizarin powder or
povidone-iodine) is applied to the skin,
 the environmental temperature is increased until
an adequate core temperature rise is attained
(usually a 2°C rise in core temperature or a core
temperature of 38.5°C, whichever is less)
 the presence of sweating causes a change in the
indicator

30. TST
 Estimating the percent of anterior surface anhidrosis
quantitates the results, and the sweat rates may be measured
Some characteristic patterns of anhidrosis include
(1) the peripheral pattern of distal anhidrosis, seen in distal small-fiber
neuropathy and length-dependent axonal neuropathy;
(2) the central patterns of distal sparing or segmental involvement,
generally seen in MSA or PD; and
(3) a sudotomal pattern suggesting involvement at the root or ganglion
level, seen in disorders involving nerve roots or specific ganglia, such as
diabetes, Sjögren disease, and pure autonomic failure.
The TST pattern is therefore helpful in distinguishing between
postganglionic, preganglionic, and central lesions.

31. Quantitative Sudomotor Axon Reflex Test
 The physiological basis of the QSART is elicitation of an
axon reflex mediated by the postganglionic sympathetic
sudomotor axon
 Acetylcholine (ACh) activates the axon terminal.
 The impulse travels antidromically, reaches a branch-
point, then travels orthodromically to release ACh from
the nerve terminal.
 ACh traverses the neuroglandular junction and binds to
M3 muscarinic receptors on eccrine sweat glands to
evoke the sweat response.
 The QSART specifically evaluates the functional status of
postganglionic sympathetic axons.

32. QSART
 Current is applied to one compartment of a
multicompartmental sweat cell,
 the sweat response is recorded from a second
compartment with a sudorometer.
 The multicompartmental sweat cells are attached to
sites on the upper and lower limbs.
 This distribution permits the detection of dysfunction
localizable to one specific peripheral nerve territory or
of a length-dependent autonomic neuropathy.
 An absent response indicates a lesion of the
postganglionic axon.

33. Sympathetic skin response (SSR)
 The SSR requires integrity of hypothalamic, brainstem,
and spinal circuits, as well as postganglionic
sympathetic neurons.
 It is performed by applying a strong electrical stimulus
to the median nerve while a long time course (≈2
seconds) recording of skin potential is made in the
contralateral palm and sole.
 The potential change linked to the median nerve
stimulus is generated in the skin by the activation of
sweat glands.
 Following a latency period, the typical response
includes a negative (upward) deflection followed by a
positive (downward) correction over several hundred
milliseconds
 The SSR is usually recorded as present or absent in the
hand and in the foot.

37. FUNCTIONAL AUTONOMIC DISORDERS

38. Reflex Syncope
 transient loss of consciousness due to loss of brain
perfusion as a protective reflex occurs at least once in
50% of healthy young adults, usually as an emotional
faint with a well-recognized precipitating stimulus.
 Syncope is defined as a transient loss of
consciousness secondary to a transient global
cerebral hypoperfusion which is of rapid onset, short
duration, and spontaneous complete recovery
 The loss of consciousness is also short, usually less than 20
seconds, and recovery is very fast.

39.  The guidelines divide syncope in 4 groups:
(1) vaso-vagal: produced by either orthostatic stress or
emotionally mediated (fear, blood phobia, etc.);
(2) situational: can be triggered by exercise,
defecation, post-prandial, visceral pain cough, etc.;
(3) carotid sinus syncope and
(4) atypical without an apparent trigger or with an
atypical presentation

40. Tilt-table testing
 Subject patients to head-up tilt at angles of 60 to 80
degrees ,
induce either syncope or intense presyncope with
reproduction of presenting symptoms.
Passive tilt tests simply use upright tilt for up to 45
minutes to induce vasovagal syncope (sensitivity ≈ 40%,
specificity ≈ 90%).
Provocative tilt tests use a combination of orthostatic
stress and drugs such as isoproterenol, nitroglycerin, or
adenosine to provoke syncope with a slightly higher
sensitivity but reduced specificity.

41.  The use of support stockings
 increased salt intake may help - 2 g of salt in the
morning and 2 g in the early afternoon
 physical countermaneuvers
 and tilt training

42. Syncopal Migraine(basilar migraine )
 headache with migrainous features immediately
prior to or after the syncopal spell
 increased duration of loss of consciousness (up to 15
minutes in this series),
 and longer time to full recovery
 prompt response to anti-migrainous medications
such as verapamil and topiramate

43. Carotid Sinus Hypersensitivity
 defined as an asystole of 3 seconds, a fall in systolic
pressure of 50 mm Hg, or both in response to carotid
artery massage in a patient with otherwise unexplained
dizziness or syncope
 35 to 100 patients per million per year present
 diagnosis is by manual massage of the carotid sinus
 Longitudinal massage should be performed for 5 seconds
 Hypotension, bradycardia, or both may dominate the clinical picture
 Weiss–Baker syndrome
headache, dizziness, vertigo, paresthesias, homonymous
hemianopsia, and hemiplegia occur in the absence of measured blood
pressure or heart rate change

44. Gravity-Induced Loss of Consciousness
 during aerial maneuvers is a special kind of
syncope seen occasionally in aircraft pilots when
the aircraft pulls up quickly after descent, during
which gravitational forces may be greatly increased
 At about 4G, without an antigravity suit or use of
the antigravity straining maneuver, blood flow to
the brain will cease, and the individual loses
consciousness and may then experience jerking
movements of the arms or legs.
 With resolution of acceleration stress,
consciousness recovers over the succeeding 10 to
60 seconds, but confusion may persist for 1 to 2
minutes

45. Postural Tachycardia Syndrome
 defined as an increase of at least 30 bpm on standing
(> 40 bpm in subjects <19 yrs of age) , associated
with symptoms of sympathetic activation
 Orthostatic symptoms include light-headedness,
palpitations, tremulousness, visual changes,
discomfort or throbbing of the head, poor
concentration, tiredness, weakness, and occasionally
fainting
 an elevated plasma norepinephrine concentration of
600 pg/mL or more on standing

47. POTS
 4 : 1 female preponderance
 typically in the 15- to 45-year age group
 250,000 to 500,000 Americans
 propranolol 10 to 20 mg three times daily; increased
dietary salt; fludrocortisone 0.1 mg orally daily;
clonidine 0.05 mg once or twice daily; and
midodrine 5 mg orally twice daily.

48. Functional Gastrointestinal Disorders
 frequent symptom of nausea.
 Hyperalgesia is very common, from increased rectal
sensation to distention of anal balloons to increased
sensitivity and discomfort with normal physiological
functions
 The role of either increased intestinal bacterial in the
foregut (small-bowel bacterial overgrowth) or altered
bacteria is well documented.
 IBS is defined as 3 months of abdominal discomfort
relieved by a bowel movement or associated with a change
in bowel movement frequency or consistency and occurs in
10% to 20% of the general population.

49. AUTONOMIC DISORDERS CHARACTERIZED BY
EXCESSIVE AUTONOMIC OUTFLOW

50. Autonomic Storm and Takotsubo Cardiomyopathy
 autonomic storms result in acute alterations in body
temperature, blood pressure, heart rate, respiratory rate,
sweating, and muscle tone.
 a massive catecholamine surge occurs that can induce
seizures, neurogenic pulmonary edema, and myocardial
injury
 (heightened activity of diencephalic or brainstem
sympathoexcitatory pathways appears to be the major
substrate of these episodes.)

51. Takotsubo “Broken Heart” Syndrome
 It mimics myocardial infarction and is characterized by
chest pain and shortness of breath. It was described
initially in Japan as tako tsubo (octopus trap) syndrome
and in the United States as apical ballooning syndrome
or broken heart syndrome
 Diagnosis is from nonspecific ST–T abnormalities, ST
elevation, or QT prolongation with large negative T
waves, often occurring over days in succession
 Plasma and urinary catecholamines are typically elevated
 Treatment is supportive, and while 95% of patients
experience complete recovery, approximately 10% will
have recurrence over a 4-year period.

52. STRUCTURAL AUTONOMIC DISORDERS

53.  PREDOMINANTLY PERIPHERAL AFFERENT
STRUCTURAL AUTONOMIC DISORDERS

54. Familial Dysautonomia
(Riley–Day syndrome )
 Ashkenazi Jewish extraction carrying mutations in
the IB kinase-associated protein gene (IKBKAP)
 FD is part of a group of disorders termed
hereditary sensory and autonomic neuropathies
(HSANs) and is classified as HSAN I
 pathophysiology originates from loss of afferent
nerve function, particularly baroreceptor
information

55.  decreased pain and temperature perception, with relative
preservation of large-fiber sensory function such as proprioception
and touch sensation, labile autonomic responses, hyporeflexia,
alacrima, poor oropharyngeal coordination, and absence of lingual
fungiform papillae serving the taste modality of sweet.
 Histamine injection produces no widespread flare response but a
very circumscribed response, usually measuring less than 2 to 3 cm
in diameter, because of the absence of C-fibers that mediate the
neuroinflammatory response.
 episodes (dysautonomic crises) are usually associated with
agitation, tachycardia, and hypertension. Vasomotor and
cardiovascular perturbations, manifesting as erythematous skin
blotching and hyperhidrosis, occ
 extreme hypertension or profound postural hypotension without
compensatory tachycardia. Supersensitivity to cholinergic and
adrenergic agents is present.

56.  Preventive and supportive strategies
 for maintaining eye moisture,
 fundoplication with gastrostomy to provide nutrition and avoid
risk of aspiration,
 use of central agents such as benzodiazepines, clonidine, or
carbidopa to control vomiting and the dysautonomic crisis,
 and fludrocortisone and midodrine to combat cardiovascular
lability

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centers in the
medulla
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baroreceptors
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nerves
Sympathetic
chain

58. Baroreflex Failure
 Acute baroreflex failure
 display stress-induced systolic blood pressure surges of
more than 300 mm Hg
 hypertensive crisis
 bilateral destruction of baroreflex afferent function
results in concomitant destruction of much efferent
vagal function
 if the baroreflex failure occurs with relative sparing
of the parasympathetic efferent vagal fibers, sleep
or sedation may lead to malignant vagotonia with
severe bradycardia and hypotension and episodes
of sinus arrest

59.  chronic baroreflex failure
 Abnormalities in the vascular baroreceptors, the
glossopharyngeal or vagal nerves, or their brainstem
connections
 Trauma from injury, tumor, radiation, surgical intervention, or
brainstem stroke

60.  Leigh syndrome
 baroreflex failure patient with impaired function of the nucleus
tractus solitarii but no history of radiation, tumor, or trauma
 Groll–Hirschowitz syndrome
 carotid sinus nerve dysfunction, progressive sensory
neuropathy, and duodenal diverticula
 Syndrome of autosomal dominant hypertension and
brachydactyly with loss of baroreflex buffering

61. DD’s of baroreflex failure
 Pheochromocytoma
 panic attack,
 generalized anxiety disorder,
 migraine,
 pure autonomic failure,
 hyperthyroidism,
 alcohol withdrawal, and drug use (e.g.,
amphetamine, cocaine)

62.  The best test is to document normal or excess
excursions of heart rate during normal daily
activities (confirming autonomic control of heart
rate), and then document an absence of bradycardic
response of heart rate to the pressor effect of
phenylephrine or the tachycardic heart rate response
to a depressor agent.
 Alternatively, the observation of wide heart rate and
blood pressure swings in the same direction at the
same time

63. Jordan syndrome
 Malignant vagotonia from selective
baroreflex failure presents as severe
bradycardia and asystole due to surges in
parasympathetic tone.
 episodes of hypotension with a systolic
pressure below 50 mm Hg.
 fatigue and dizziness with possible
progression to frank syncope
 most severe episodes tend to occur
during early-morning sleep
&administration of intravenous
nitroprusside and sublingual
nitroglycerin,
 and periods of asystole longer than 20
seconds may occur
Baroreflex afferents (BA) are
damaged in patients with selective
and nonselective baroreflex failure.
Efferent sympathetic (SNS) and
parasympathetic nerves (PNS) are
intact in selective baroreflex failure.

64. Treatment of
baroreflex failure
 reduce the frequency
and magnitude of life-
threatening surges in
blood pressure and
heart rate
 attenuate
symptomatic
hypotensive episodes

65. PREDOMINANTLY PERIPHERAL EFFERENT STRUCTURAL
AUTONOMIC DISORDERS CHARACTERIZED BY IMPAIRED
AUTONOMIC OUTFLOW

66. Pure Autonomic Failure
 synucleinopathy with synuclein found within Lewy bodies confined to
autonomic ganglia, presenting in mid- to late life
 The initial feature in men is impotence,
 but OH usually brings patients to the physician with unsteadiness or
faintness on standing.
 It is worst in the morning and improves as the day progresses.
 Supine hypertension may occur during the night while supine. Meals,
exercise, fever, or environmental heat worsen OH.
 orthostatic pain in the neck, shoulders, or occiput, relieved by lying
down.
 Although a decline in systolic blood pressure of 20 mm Hg and diastolic
blood pressure of 10 mm Hg after at least 1 minute of standing defines
OH, most PAF patients suffer from profound OH with a decrease in
systolic blood pressure of 50 mm Hg and sometimes more than 100
mmHg.
 Convulsive near-syncope may occur.
 Urinary hesitancy, urgency, dribbling, and occasional incontinence may
also occur related to specific bladder dysfunction.

67.  Lewy bodies characterize the pathology of PAF, along
with a loss of cells in the intermediolateral column of
the spinal cord and a loss of catecholamine uptake
and catecholamine fluorescence in sympathetic
postganglionic neurons.
 reduced catecholamine levels
 most common cause of death in these patients is
pulmonary embolus or intercurrent infection

68. Autoimmune Autonomic Ganglionopathy (acute pandysautonomia )
 Severe generalized sympathetic and parasympathetic
autonomic failure unfolds over a few days to a few
weeks.
 Orthostatic hypotension, fixed heart rate, anhidrosis,
dry mouth, dry eyes, sexual dysfunction,
constipation, and impaired pupillary function
,Anorexia, early satiety, postprandial abdominal pain
and vomiting, constipation, or diarrhea

69.  autoimmune pathogenesis is the demonstration of
ganglionic nicotinic acetylcholine receptor (AChR)
antibodies in high titers
 response of this disorder to intravenous globulin and
plasma exchange

70. Autonomic Neuropathy
 involving both autonomic and small unmyelinated
or thinly myelinated sensory nerves
 distal burning pain and changes in color of the
distal limb reflecting poor vasomotor control
 affect erectile function first, may then affect bowel
or bladder function with constipation or urgency,
followed by parasympathetic glandular function
resulting in dry eyes or mouth, and ultimately lead
to OH

71.  Causes- diabetes or metabolic syndrome , Sjögren
syndrome
 , paraneoplastic autonomic neuropathy, postinfectious
causes,
 collagen vascular disorders such as lupus or rheumatoid
arthritis
 Infectious causes include herpes zoster, Lyme disease,
and syphilis.
 infiltrative disorders such as α-galactosidase deficiency
(Fabry disease), porphyria, heavy metal poisoning,
 and other drugs and toxins

72. Drug-Induced Dysautonomia
 mechanisms :
 blood volume depletion-hyperadrenergic orthostatic
hypotension.
 sympathoplegic effects causing impairment in maintenance
of vascular resistance, venous tone, or cardiac output -
hypoadrenergic orthostatic hypotension ;
 and direct vasodilatation, which lowers vascular resistance
or venous tone
 sedatives, hypnotics, antidepressants, diuretics,
antihypertensive drugs, or nitrates , Oncological
agents such as vincristine and cisplatin ,
zonisamide,

73. Dopamine β-Hydroxylase Deficiency
 selective absence of norepinephrine and all its metabolite
 absent sympathetic noradrenergic function but normal
parasympathetic and sympathetic cholinergic functions
 Symptoms in the perinatal period include
 vomiting, dehydration, hypotension, hypothermia, and profound
hypoglycemia requiring repeated hospitalization.
 Exercise capacity is poor.
 By early adulthood,
 profound orthostatic hypotension,
 greatly reduced exercise tolerance, ptosis of the eyelids, and supine
nasal stuffiness.
 Presyncopal symptoms include dizziness, blurred vision, dyspnea,
nuchal discomfort, and chest pain.
 During adult life, some DBH-deficient patients develop renal function
abnormalities, including raised blood urea nitrogen and creatinine
levels.

74.  Biochemical features
 minimal or undetectable plasma, CSF, and urinary
norepinephrine and epinephrine and a fivefold to tenfold
elevation of plasma dopamine
 lack urinary normetanephrine, metanephrine, and
vanillylmandelic acid.

75. Menkes Kinky Hair Syndrome (Trichopolydystrophy, X-linked Copper
Deficiency)
 DBH deficiency -copper-containing enzyme, and
congenital disorders of impaired copper
metabolism
 stubby, tangled, sparse hair (often white or gray in
color), pudgy cheeks, spasticity, seizures,
hypothermia, retarded growth, and decreased
visual function. Subdural hematoma, jaundice, and
osteoporosis
 Administration of droxidopa (l-threo-3,4-
dihydroxyphenylserine), or LDOPS alleviates OH

76. PREDOMINANTLY CENTRAL STRUCTURAL AUTONOMIC
DISORDERS CHARACTERIZED BY IMPAIRED AUTONOMIC
OUTFLOW

77. Multiple System Atrophy
(Shy-Drager syndrome )
 progressive neurodegenerative disorder
encompassing
 autonomic,
 extrapyramidal,
 cerebellar, and
 pyramidal features
 Pathological hallmark of MSA is neuronal loss and
gliosis within multiple sites in the brain,
intermediolateral columns, and the Onuf nucleus,
with characteristic glial cytoplasmic inclusions
(GCIs) containing α-synuclein and ubiquitin

78. glial cytoplasmic inclusions (GCIs)
 1. Shape: GCIs tend to be irregular in outline, in
contrast to the target-shaped concentric circular
Lewy bodies of PD.
 2. Cellular location: GCIs are in glia, whereas Lewy
bodies are in neurons.
 3. Neuraxis location: GCIs dominate in the basal
ganglia and pons, whereas Lewy bodies occur in
midbrain, cortex, and autonomic ganglia.

79.  The clinical picture is usually dominated by three
major issues:
 a dysautonomia manifested by severe orthostatic hypotension
 and severe (most often lower motor neuron) urinary
dysfunction,
 and their motor system disturbance that results in wheelchair
requirement early in the course of the disorder.

80.  Patients may have multiple other related complaints
impotence, slurred speech, sleep apnea, vivid
nightmares, orthostatic headache, neck pain,
dimming of vision, and leg discomfort. emotional
,periodic gasping respirations
 management continues to be symptomatic
 rifampicin was shown to inhibit aggregation of α-
synuclein in vitro and in this mouse model, leading to a
National Institutes of Health-sponsored trial of
rifampicin in MSA
 intravenous globulin showed guarded promise

81. Parkinson Disease
 the Lewy bodies of PD directly involve ganglia, and
hence postganglionic neurons, with degeneration of
peripheral autonomic fibers
 reduction in axon reflex sweating more frequently in
PD than in MSA
 this peripheral predilection in PD is the basis
for the denervation seen on
metaiodobenzylguanidine (MIBG) scanning of
the heart, diagnostic test to distinguish PD
from MSA

82. Myelopathy
 Following spinal cord injury,
 the initial response is hypoexcitability (spinal shock) with flaccid
paralysis, impaired tendon reflexes, and spinal autonomic
dysfunction presenting as atonic bladder and bowel, vasodilation,
and absent spinal autonomic reflexes. This stage lasts days to weeks,
and then activity below the transected cord returns.
 Chronically, a quite distinct autonomic dysfunction emerges
 Head- Riddoch syndrome
 Severe hypertension may occur during autonomic dysreflexia after
noxious stimulation below the level of the lesion
 Guttmann sign
 The nasal stuffiness from vasodilation in the nasal mucosa