Clinical Significance of autonomic nervous System

1. CLINICAL SIGNIFICANCE OF
AUTONOMIC NERVOUS SYSTEM
Presented by
Dr. Ashish Chowdhury
Phase B,Neurology,BSMMU

2. Classification Of Clinical Autonomic
Disorders
1. Autonomic disorders with brain
involvement:
A. Associated with multisystem degeneration
B. Unassociated with multisystem
degeneration(Focal CNS disorders)
2. Autonomic disorders with spinal cord
involvement
3. Autonomic neuropathies
A. Acute/Subacute autonomic neuropathies
B. Chronic peripheral autonomic neuropathies

3. Autonomic disorders with brain involvement
• Multiple System atrophy
• Parkinson′s disease
• Diffuse lewy body disease
• Progressive supranuclear palsy
• Cortico basal degeneration
• Wernicke korsakoff syndrome
• Frontal cortex lesion
• Autonomic Seizure
• Horner syndrome
• Posterior fossa tumors
• Brainstem encephalitis
• Neuroleptic malignant syndrome

4. Autonomic disorders with spinal cord involvement
• Traumatic quadriplegia
• Syringomyelia
• SCD
• MS,NMO
• ALS
• Spinal cord tumors
• Stiff person Syndrome

5. Autonomic neuropathies
• DM
• GBS
• Botulism
• Porphyria
• Drugs and Toxin
• Amyloidosis
• Familial dysautonomia
• Postural orthostatic tachycardia syndrome

6. Clinical features of autonomic
neuropathies
1. Cardiovascular:
• Postural hypotension
• Resting tachycardia
• Fixed Heart rate
2. GIT:
• Dysphagia
• Gastroparesis
• Nocturnal diarrhoea
• Fecal incontinence
• Constipation

7. 3.Genitourinary :
• Difficulty in micturition, urinary incontinence,
recurrent infections due to atonic bladder
• Erectile dysfunction and retrograde
ejaculation
4. Pupillary :
• Decreased pupil size
• Resistance to mydriatics
• Delayed or absent reflexs to light

8. 5. Sudomotor :
• Nocturnal sweats without hypoglycaemia
• Gustatory sweating
• Anhidrosis; fissures in the feet
6.Vasomotor :
• Feet feel cold, due to loss of skin vasomotor responses
• Dependent oedema, due to loss of vasomotor tone and
increased vascular permeability
• Bullous formation

9. Clinical test of autonomic Function
1. Tests of Cardiovascular autonomic functions:
Non Invasive Bed Side test
• Blood pressure response to standing or vertical
tilt or head up tilt test (Fall ≤ 20/10 mmHg)
• Heart rate response to standing and 30:15
R-R ratio (ECG)( ≥ 1.04)
• Heart rate variation with respiration( ≥ 15 b/m)
• Isometric exercise-(↑DBP 15mmHg)
• Valsalva ratio ≥ 1.4
• Plasma noradrenaline level- (rise on tilting)
• Plasma vasopressin level- (rise with induced
hypotension)

10. Invasive Test:
• Valsalva maneuver
• Baroreflex sensitivity
• Infusion of pressor drugs – (↑BP,↓HR)
2.Test for thermoregulatory functions:
• Sympathetic skin response test
• Quantitative sudomotor axon reflex
test(QSART)
• Thermoregulatory sweat test
• Sweat imprint test

11. 3.Test of vasomotor reaction:
• BP response to sustained hand grip
• BP response to mental stress
• Cold pressor test
• Radiant heating of trunk
• Immersion of hand in hot water
4.Miscellaneous tests:
• Lacrimal function: Schirmer test
• Test of pupillary Innervation
• Bladder function- Cystometrogram
• Penile erection- Nocturnal penile tumescence
• GIT motility- Barrium swallow/ manometry

12. Valsalva Maneuver
• Response assesses the integrity of the baroreflex
Control of HR ( parasympathetic) and BP
(adrenergic).
• Procedure :
Supine
Exhale against closed glottis
Or Into a manometer exp. Pressure of 40 mmHg
For 10-15 seconds
Measuring changes in HR and beat to beat BP
Four phases, Phase І & ІІІ mechanical

16. Sympathetic skin response
• Sympathetic skin response(SSR)is a momentary change
of the electrical potential of the skin generated by sweat
gland .It originates by activation of reflex arc with
different kinds of internal or externally applied arousal
stimuli.
• Important electrophysiological autonomic test that can
be performed in the EMG Laboratory.
• Reflex Arc :
Afferent pathway : Somatosensory myelinated fibers
Central processing : Mescenphalic reticular formation,
Posterior thalamus & Cortical structures.
Efferent pathway : Preganglionic myelinated and
postganglionic nonmyelinated axons and the
neuroglandular junction

17. SYMPATHETIC SKIN RESPONSE :CONTD…

18. SYMPATHETIC SKIN RESPONSE :CONTD…
• Response shape is most often either biphasic or triphasic in
the hands, and biphasic in the feet; it is seldom monophasic.
 The amplitude of hand waveform larger and shorter latencies
than feet.
 Generally absent response are considered to be abnormal.
 The amplitude of the SSR is age dependent. SSR is normally
present in both hands and feet under the age of 60 years,but
in subjects older than 60 years it is found in only 50% of feet
and in 73% of hands

19. SYMPATHETIC SKIN RESPONSE :CONTD…
• Most Common type of Stimulus is an electrical shock
(10-30mA) delivered at a peripheral nerve, most
commonly median nerve.
• Normal mean onset latency and amplitude of 1.5 ± 0.1
sec and 3.1 ± 1.8 mV for the hands, and 2.05 ± 0.10 sec
and 1.4 ± 0.8 mV for the feet.

20. Orthostatic Hypotension
• Reduction of SBP at least 20mmHg or DBP at least 10mmHg
after 3min of Standing or Head up tilt on a tilt table, is a
manifestation of sympathetic Vasoconstrictor failure.
• Causes:
1.CNS autonomic failure:
• MSA
• PD
• Lewy body dementia
• Pure Autonomic Failure
• Spinal cord trauma, infraction
• Centrally Acting Anti hypertensive drugs and other drugs

21. Causes of Orthostatic Hypotension
2. Autonomic peripheral neuropathies:
• DM
• Hereditary amyloidosis
• Primary amyloidosis
• Hereditary Sensory and autonomic neuropathies
• Idiopathic immune mediated autonomic neuropathy
• Autoimmune autonomic ganglionopathy
• Sjogren′s syndrome
• Paraneoplastic autonomic neuropathy
3. Postprandial hypotension
4. Drug induced
5. Volume depletion

23. Symptoms of orthostatic intolerance
• Lightheadedness (dizziness)
• Weakness or tiredness
• Cognitive difficulty (thinking/concentrating)
• Blurred Vision
• Tremulousness
• Vertigo
• Pallor
• Anxiety
• Palpitations
• Clammy Feelings
• Nausea

24. Management of Orthostatic
Hypotension
• Treatment of specific Cause.
• Removal of Drugs or Conditions that aggravate
the autonomic symptoms.
• Patient educations
• High Salt diet :(10-20g/d)
• High Fluid intake: 2L/d
• Elevate Head of bed 10cm.
• Maintain postural Stimuli
• Learn physical Counter maneuvers
• Long leg elastic stocking
• Correct Anemia

25. Management of Orthostatic
Hypotension Cont…..
Pharmacological treatment:
• Fludrocortisone
• Midodrine
• Droxidopa
• Pyridostigmine
• NSAID
• Combination of Atomoxetine and yohimbine
• Desmopressin
• Erythropoetin

26. Complex Regional pain Syndrome
• Two types:
A. Types 1: Reflex Sympathetic dystrophy (RSD)
B. Types 2: Causalgia –Specific peripheral nerve
/trunk lesion
Reflex Sympathetic Dystrophy:
 After injury-minor Shoulder/limb trauma,
,MI,stroke,Fractures
 One Limb, Vasomotor & Sudomotor abnormalities
 Allodynia, Hyperpathia,Spontaneous pain
 Unrelated to severity of initial trauma
 Not confined to the distribution of a single
peripheral nerve , Burning or Electrical

27. RSD continue
Divided into 3 clinical phase
Phase 1: within weeks to 3 months
• Pain and swelling in the distal extremity
• Warm, edematous, tender joints
• Increase sweating and hair growth
Phase 2: 3-6 months-Thin, shiny, cool skin
Phase 3: Additional 3-6 months
• Atrophy of skin and subcutaneous tissue
• Flexion contracture

28. Treatment Of CRPS
• Early Mobilization
• Physiotherapy
• Glucocorticoids
• Others: Adrenergic blockers, NSAID, CCB,
Phenytoin, opioids,Calcitonin
• Surgical: Stellate ganglion blockade

29. Horner′s Syndrome
• Horner′s syndrome consists of
(1) Constriction of the pupil (miosis),
(2) Slight drooping of the eyelid ( partial ptosis),
(3) Enophthalmos,
(4) Loss of sweating (anhydrosis).
• All these symptoms result from an interruption
of the sympathetic nerve supply to the head and
neck.

30. Ocular Autonomic Pathway

31. Horner’s syndrome
Classical triad:
• Miosis–(constricted pupil)- due
to unopposed action of iris
constrictor muscle
• Partial ptosis- due to Muller
muscle paralysis
• Loss of hemifacial sweating
(Anhydrosis)

32. Other Features:
 Enophthalmos ( weakness of muller′s
muscle)
Iris heterochromia-(with the affected eye
being hypopigmented)-Congenital horner
syndrome/Children younger than 2 yrs/long
standing
Normal light & Accomodation reflex
Normal eye movement

33. Arise from the hypothalamus (1st
order neuron)
Descend uncrossed through the
brainstem
Terminate in the lateral column
level of C8-T2
Exit the spinal cord at the level of
T1 and enter the cervical
sympathetic chain (2nd order neuron)
Ascending through the
sympathetic chain, it synapse’s
at the superior cervical ganglion

34. • Post ganglionic exit the superior
cervical ganglion and ascend along the
Internal Carotid artery
(3rd order neuron)
• Through short and long ciliary nerve,
innervates dilator pupillae
• Travels along the external carotid
artery to innervate the blood vessels
and sweat glands of the face.

36. 1st 0rder neuron lesion
First order neuron (the descending sympathetic tract
within central nervous system) defect could include:
• loss of sweating of the ipsilateral side of the body ( face, arm,
upper trunk)

37. First order neuron lesions
Lesions in Hypothalamus, Brainstem, High
Cervical cord
• Large Pituitary tumor / Expanding tumor
•Brainstem Stroke (LMS), Tumor( glioma),MS
• Syringobulbia, Syringomyelia
• Neck Trauma: Cervical myelopathy, Traumatic
dislocation of Cervical vertebrae

38. 2nd order neuron lesion
Second order neuron (preganglionic horner)
Loss of sweating limited to ipsilateral face.
Lesion in common carotid artery, loss of sweating
involves the entire side of face.
Lesions:
• Anterior roots C8 ,T1 lesions: Tumor-
neurofibroma
• Lower brachial plexopathy : trauma,cervical rib

39. • Cervical sympathetic chain lesion:
Pancoast tumor
 Aneurysm or dissection of aorta, CCA
Trauma / Surgery
Carotid angiography
Lymphadenopathy-Hodgkin′s disease

40. 3rd order neuron lesion
Postganlionic Horner Syndrome
Lesions occurring after vasomotor and
sudomotor fibers have branched off the
sympathetic chain
Anhidrosis is usually absent or limited to an area
above the ipsilateral medial aspect of forehead
and side of the nose.
Lesions :
• Internal carotid artery:
trauma/dissection/occlusion
• Middle fossa: tumor, granuloma

41. Argyll Robertson Pupil
• Argyll Robertson pupil is characterized by a small
pupil, which is of
1. Fixed size and irregular in shape
2. Does not react to light
3. But react to accommodation.
• Caused by neurosyphilis, diabetes, alcohol or any
midbrain lesion interrupting the fibers that run
from the pretectal nucleus to parasympathetic
nuclei of the oculomotor nerve on both sides.

42. Urinary Bladder Dysfunction
Following Spinal Cord Injuries
• The normal bladder is innervated as follows:
• Sympathetic innervation is from the first and
second lumbar segments of the spinal cord.
• Parasympathetic innervation is from the
second, third, and fourth sacral segments of
the spinal cord.
• Sensory nerve fibers enter the spinal cord at
the above segments.

45. Site of lesion Result
Atonic (lower motor
neuron)
Lesions of sacral segments of
cord (conus medullaris)
Lesions of sacral roots and
nerves, Loss of detrusor
contraction
Difficulty initiating
micturition, Bladde
distension with ove
Hypertonic (upper motor
neuron)
Pyramidal tract lesion in
spinal cord or brainstem
Urgency with urge
incontinence
Bladder sphincter
incoordination
(dyssynergia)
Incomplete bladde
emptying
Cortical Post-central
Pre-central
Frontal
Loss of awareness
bladder fullness, D
initiating micturitio
Inappropriate mictu45

46. • The atonic bladder occurs during the phase of spinal
shock immediately following the injury and may last
from a few days to several weeks.
• The bladder wall muscle is relaxed, the sphincter
vesicae is tightly contracted (loss of inhibition from
higher levels), and the sphincter urethrae is relaxed.
• The bladder becomes greatly distended and finally
overflows.
• Depending on the level of the cord injury, the patient
may or may not be aware that the bladder is full; there
is no voluntary control.

47. • Automatic reflex bladder occurs after the patient
has recovered from spinal shock
• Provided that cord lesion lies above the level of
parasympathetic outflow
• Normally found in infancy
• Since the descending fibres in the spinal cord are
sectioned,there is no voluntary control
• The bladder fills and empties reflexly
• Known as spastic neurogenic bladder

48. • The autonomus bladder is the condition that
occurs if the sacral segment of spinal cord or
cauda equina is destroyed
• No reflex control or voluntary control
• Bladder wall is flaccid,overflows, resulting in
continuous dribbling

49. • Uninhibited bladder is associated with urge
incontinence with sudden uncontrolled
evacuation and having no residual urine
• Results from lesions affecting frontal lobe,
parasagittal meningioma or aneurysm of
anterior communicating artery

50. Treatment of Neurogenic bladder
• Atonic bladder- Intermittent self
catheterization, In-dwelling catheterization.
• Hypertonic bladder-
Anticholinergics : Solifenacin,
Tolterodine,Imipramine
Intermittent self catheterization
• Cortical bladder- Intermittent or In-dwelling
catheterization
50

51. Dysfunction in Erection and
Ejaculation Following Spinal Cord
Injuries
• The erection of the penis or clitoris is controlled
by the parasympathetic nerves that originate
from the second, third, and fourth sacral
segments of the spinal cord.
• Bilateral damage to the reticulospinal tracts in
the spinal cord above the second sacral segment
of the spinal cord will result in loss of erection.

52. • Ejaculation is controlled by sympathetic
nerves that originate in the first and second
lumbar segments of the spinal cord.
• severe bilateral damage to the spinal cord
results in loss of ejaculation.

53. • Sympathectomy is done as a method of
treating arterial disease , Like
Raynaud disease
Intermittent claudication
Hypertension