autonomic nervous system

7. Anatomically (central) :
Anatomically (central) :
1
1-
-telencephalon
telencephalon
2
2-
-diencephalon
diencephalon
3
3-
-brain stem
brain stem
4
4-
-spinal cord
spinal cord
Physiological (nerve
Physiological (nerve
specific) :
specific) :
1
1-
-diffuse autonomic
diffuse autonomic
failure
failure
(
(pandysautonomia
pandysautonomia)
)
2
2-
-pure cholinergic
pure cholinergic
3
3-
-pure adrenergic
pure adrenergic
Topographically (organ
Topographically (organ
specific) :
specific) :
1
1-
-pupil
pupil
2
2-
-facial
facial
3
3-
-limb
limb
4
4-
-GIT
GIT dysmotility
dysmotility
5
5-
-neurogenic
neurogenic bladdar
bladdar
6
6-
-sexual dysfunction
sexual dysfunction
Clinical syndromes involving autonomic nervous system :

8. 1- Anatomical (central - level specific) :
1- Telencephalon 2- Diencephalon 3- brainstem 4- spinal cord

9. 1- Telencephalon :
1- stroke :
- ischemic damage of insula  cardiac arrythmia&
contralateral hyperhydrosis
- ischemia of cingulate & paracentral cortices 
urinary incontinence d.t. uninhibited bladdar
contraction
2- temporolimbic seizures :
- cardiovascular manifestations  syncope , sinus
tachy/brady arrythmia , ventricular tachcardia /
fibrillation , cardiac arrest , sudden death.
- autonomic manifestations  pallor/flushing ,
shivering, sweating, vomiting, respiratory changes.
(Basal Ganglia)

10. 2- Diencephalon :
1- wernicke`s $ :
- DCL + Diturbed oculomotor function + unexplained
hypothermia in alcoholic or malnourished patient
2- Episodic hyperhydrosis with hypothermia
- No apparent cause.
- Occurs as amanifestation of agenesis of corpus
callosum & adjacent forebrain OR during paroxysms of
autonomic hyperactivity.
3- Paroxysmal autonomic hyperactivity :
- Firstly described in pt. with brain tumour in the region
of 3rd ventricle causing hydrocephalus.
- Most cases occur in severe closed head injury with 
ICP or acute hydrocephalus following SAH.
- It was falsly named diencephalic seizures but EEG
showed no ictal activity & no response to antiepileptic
(thalamus,
hypothalamus,
subthalamus )

11. 4- Fatal familial insomnia :
- Autosomal dominant disorder .
- Characterized by severe atrophy of anterioventral and
dorsomedial nuclei of tahlamus .
- Presented with disruption of endocrine circadian rhythm
, motor dysfunction , progressive intractable insomnia .
5- Iatrogenic :
1- neuroleptic malignant $ :
- Neurological emergency
- Use of neuroleptic agent followed by 2 of 4 (mental
changes , rigidity, hyperpyrexia , dysautonomia (
tachcardia ,tachypnia,HTN,dysrythmia,profuse
diaphoresis))
2- serotonin $ :
- Use of SSRI followed by fever , rigidity, dysautonomia +
shivering,hyperreflexia,myoclonus,ataxia,nausia,vomiting,
diarrhae

16. 3- brainstem :
1- cerebrovascular disease :
- The commonest cause of dysautonomia in brainstem
dysfunction
=> Vertebrobasilar TIA  may present with paroxysmal
HTN before focal neurological deficit
=> Lateral medullary infarction(wallenberg`s $)  produce:
- Horner`s $
-severe autonomic manifestation e.g. profound bradycardia,
supine hypotension, central hypoventilation
2- tumours :
- present with intractable vomiting, orthostatic hypotension
or paroxysmal HTN
3- syrigobulbia :
- Produce horner`s $ , orthostatic hypotension, labile
bl.pressure , cardiovagal dysfunction, central
hypoventilation
4- arnold-chiari malformation type 1 :
- syncope, sleep apnea, cardiorespiratory arrest

17. 5- inflammation :
1- poliomyeilitis : HTN d.t involvement of medullary
reticular formation
2- tetanus : autonomic hyperactivity d.t disinhibition of
sympathatic and parasympathatic neurons.
3- multiple sclerosis : fulminant neurogenic pulmonary
edema d.t. demyelination of area surrounding NTS

18. 4- spinal cord :
1- trauma : - particularly above the level of T5
=> Acute spinal injury :
- supine and orthostatic hypotension, arrythmias, arrest
during maneuvers that activate vasovagal reflexes.
- Lack of vasomotor and sudomotor thermoregulatory
response below the level of lesion leads to severe
hypo/hyperthermia according to environmental temperature
- Bladdar : paralytic atonic bladdar
=> In Chronic stages :
- Autonomic dysreflexia : it`s massive reflex activation of
both sympathetic and parasympathetic systems during
stimulation of skin ,muscles and viscera innervated by
segments below the lesion.
- Severe hypertension is the prominent feature and may
leads th HTN encephalopathy, IC Hge, SAH or retinal hge
- Vasodilatation above the level of lesion  flushing sif face,
chest, pallor of abdomen and LLs and pupillary dilatation
- Bladdar : Lesions above the sacral parasympathetic
nucleus produce spastic
bladder, commonly with detrusor-sphincter dyssynergia,
whereas lesions of the conus medullaris produce a flaccid,
areflexic bladder.

19. 2- syringomyelia :
- Interrupt sympathetic output
- Produce horner $, sudomotor and vasomotor
dysfunction, and trophic changes in the limbs, especially
the hands.
3- Multiple sclerosis :
- Bladdar dysfunction : detruser hyper-reflexia and
detruser-sphincter dyssynergy
- Subclinical cvs and thermoregulatory dysfunction
4- tetanus & stiff person $ :
- may be associated with sympathetic hyperactivity due to
lack of synaptic inhibition of preganglionic autonomic
neurons.
- This results in hyperpyrexia, sweating, tachycardia,
hypertension, tachypnea, and pupillary dilatation, usually
associated with the muscle spasms

22. 2- physiological (nerve specific) :
1- diffuse autonomic failure
(pandysautonomia) :
2- pure cholinergic dysautonomia
3- pure adrenergic dysautonomia

23. 1- diffuse autonomic failure
(pandysautonomia) :
Preganglionic
(central neuro-
degenerative disorder)
Ganglionic and postganglionic
(peripheral neuro-
degenerative disorder)
1- MSA (shy - drager $)
2- PD with autonomic failure
Pure autonomic
failure
Neuropathies
with autonomic failure
Acute & subacute chronic
- Acute pandysautonomia
- GB$
- Paraneoplastic $
- Others (porphyria, toxins, drugs)
Gangliopathies Postganglionic
(ch. Smallfibre
neuropathies)
- Para neoplastic $
- Sjogren $
- HIV, SLE
- Metabolic & nutritional
- others
- DM
- amyloidosis
- Hereditary sensory and
autonomic neuropathies
(familial dysautonomia,
fabry`s disease, others )

24. 2- pure cholinergic dysautonomia
Associated with NMT defect Not associated with NMT defect
1- acute ( botulism )
2- chronic (Eaton lambert $)
1- acute cholinergic
dysautonomia
2- chronic idiopathic anhidrosis
3- Adie`s $
4- Ross`s $ : combination of adie`s
pupils and segmental anhidrosis.
4- chagas disease : caused by
trepanosoma cruzi that affects the
neurons of the parasympathetic
ganglion and produces
megaesophagus, megacolon, and
cardiomyopathy .
- acetylcholine release from motor as well
as autonomic neurons fails.
- Thus, neuromuscular and autonomic
cholinergic neurotransmission is impaired,
and muscle weakness, reflex loss, and
autonomic failure result

25. 3- pure adrenergic dysautonomia
1- pure adrenergic neuropathy 2- dopamine B-hydroxylase deficiency
 Dopamine B-hydroxylase deficiency :
- a hereditary disease characterized by an inability to convert dopamine
(DA) to NE.
- Typically, the serum NE/DA ratio is 0.1 (normal, 10).
- Autonomic manif.  severe orthostatic hypotension accompanied by ptosis,
ejaculatory failure, nocturia, nasal congestion, and hyperextensible joints.

26. 1- multiple system atrophy :
- Degenerative disorder of CNS
- Pathologically  cell loss and gliosis in striatonigral, olivopontocerebellar, and autonomic neurons
are evident, and intracytoplasmic oligodendroglial and neuronal inclusions are frequently present.
- C/P :diffuse autonomic + cerebellar + extrapyramidal + pyramidal manifestation
- Autonomic features : orthostatic hypotension, erectile dysfunction in males,
bowel hypomotility, urinary incontinence and respiratory disturbances
(sleep apnea and laryngeal stridor).
- poor response to levodopa.
2- parkinson disease :
- Pathologically : lewy body in both central and peripheral neurons.
- Clinically : Less severe than MSA, occurs later in the disease usually associated with levodopa and
dopamine agonist therapy.
3- pure autonomic failure :
- idiopathic, sporadic, degenerative disorder.
- Slowly progressive and has better prognosis than MSA.
- No motor manifestations.
- Pathologically : intracytoplasmic eosinophilic inclusion bodies in neurons of the substantia nigra,
locus ceruleus, thoracolumbar and sacral spinal cord, as well as sympathetic ganglia and
postganglionic nerves.

28. 4- acute pandysautonomia :
- Prominent sympathatic and parasympathatic involvement.
- May be isolated or accompangied with GBS, acute paraneoplastic $.
- CSF ptn. May be elevated
- Respond to IVIG.
5- Guillian barre $ : - Autonomic manifestations :
- Cardiovascular signs may include tachycardia, bradycardia, dysrhythmias, wide fluctuations in blood
pressure, and postural hypotension.
- Urinary retention due to urinary sphincter disturbances may be noted.
- Constipation due to bowel paresis and gastric dysmotility may be present.
- Facial flushing and venous pooling secondary to abnormal vasomotor tone may be present.
- Hypersalivation - Anhydrosis - Tonic pupils
6- paraneoplastic dysautonomia :
- Subacute onset
- Autonomic manif.  dry eyes and dry mouth, large pupils that react poorly to light and
accommodation, and neurogenic bladder.
7- Others :
- Acute autonomic dysfunction may also be a feature of porphyria and of the neuropathies due to drugs
(e.g., vincristine, taxol, and cisplatin), industrial toxins (e.g., thallium and acrylamide), marine toxins,
and infections such as botulism and diphtheria.
8- diabetes mellitus :
- the most common cause of autonomic neuropathy in the developed world.
-autonomic manif.  orthostatic intolerance, impotence, voiding difficulties, gastroparesis,
constipation alternating with diarrhea, and gustatory sweating.

29. 9- hereditar sensory and autonomic neuropathies (HSAN) :
 5 types:
- Type I & II mild autonomic manifestations.
- Type III (familial dysautonomia ; relay-day $) 
- autosomal recessive, seen in in Ashkenazi Jewish children.
- C/P : insensitivity to pain and temperature, the absence of tears, hypoactive corneal and
tendon reflexes, and the absence of fungiform papillae of the tongue. poor suck and feeding responses,
esophageal reflux with vomiting and aspiration, uncoordinated swallowing, episodic hyperhidrosis,
vasomotor instability, postural hypotension, hypertensive crises, supersensitivity to cholinergic and
adrenergic agents, and absent histamine flare .
- Type IV & V  characterized by congenital insestivity to pain and temperature.
10- Fabry`s disease :
- X-linked recessive caused by a deficiency of α-galactosidase A .
- C/P : painful distal peripheral neuropathy; a truncal reddish-purple macular papular rash;
angiectases of the skin, conjunctiva, nail bed, and oral mucosa; progressive renal disease; corneal
opacities; and cerebrovascular accidents.
- Autonomic manifestations  hypohidrosis or anhidrosis, reduced saliva and tear formation, impaired
histamine flare, and gastrointestinal dysmotility.
- Enzyme replacement therapy with recombinant human α-galactosidase A improved small fiber
function in one study.

30. 3- Topographically (organ specific) :
1- pupil disoreders
2- Vasomotor & sudomotor disorders
4- Neurogenic Bladdar
3- GIT dysmotility
5- Sexual dysfunction

31. 1- pupil disoreders
Affecting sympathatic outyflow Affecting parasympathatic outflow
3rd nerve lesion
Argyll Robertson pupil
Adie`s pupil
Horner syndrome Porfour du petit
syndrome

32. 1- 3rd CN lesion : see later
2- argyll robertson pubil :
- small irregular pupil, respond to accomodation & not respond to light reflex.
- indicates lesion in the rostral midbrain at the level of posterior commissurehorner $.
3- adie`s pupil :
- large irregular tonic pupil, poorly reactive to light.
- indicate lesion of the ciliary ganglion or the short ciliary nerves.
- associated with reduced or absent tendon reflex.
- slit lamp exam: regular palsies of the iris sphincter.
- pilocarpine test :hypersensitive constrictor response.
4- horner $ : see later
5- Porfour du petit syndrome :
- a dilated pupil with flushing and hyperhidrosis due to sympathetic hyperactivity.
- seen in patients with sympathetic nervous system lesions, frequently following
injuries of the neck that damage the sympathetic plexus around the carotid artery.

34. 2- Vasomotor & sudomotor disorders
Face Limb
1- gustatory sweating
2- harlequin $
3- cluster headache
Vasomoyor disorders
Sudomotor disorders
1- Raynaud`s phenomenon
2- Acrocyanosis
3- Livedo reticularis
4- Erythromelalgia
Essential hyperhydrosis
Complex neuropathic
pain $
1- painful distal peripheral neuropathy
2- nerve trauma(causalgia)
3- complex regional pain syndrome
4- reflex sympathatic dystrophy

35. 1- gustatory sweating and flushing :
- occur in the following conditions:
1- idiopathic hemifacial hyperhidrosis associated with hypertrophy
of the sweat glands
2- following bilateral cervicothoracic sympathectomy with reinnervation of the superior
sympathetic ganglion by preganglionic sympathetic fibers destined for the sweat glands
3- after local damage to the autonomic fibers traveling with the peripheral branches of the
trigeminal nerve (e.g., in parotid or submaxillary gland surgery or V3 zoster) with
reinnervation of sweat glands and blood vessels by parasympathetic vasodilator fibers
destined for the salivary glands
4- accompanying peripheral neuropathies, most frequently diabetes mellitus.
2- harlequin syndrome :
- sudden onset of flushing and sweating on one side of the face.
- may be due to lesions in the contralateral central or peripheral
sympathetic nervous system pathways.
3- cluster headache :
- Attacks of cluster headache may be accompanied by ipsilateral signs of parasympathetic
hyperactivity (lacrimation and nasal discharge), sympathetic overactivity (forehead
sweating), and ocular sympathetic paralysis (miosis and ptosis).

36. 4- Raynaud`s phenomenon :
- it`s episodic, bilateral, symmetrical change in skin color (pallor, followed by cyanosis and
terminating in rubor after rewarming).
- It is provoked by cold or emotional stimuli. This response is due to episodic closure of the
digital arteries.
- Causes :
1- may be associated with connective tissue disease (e.g., scleroderma, rheumatoid
arthritis, and psoriasis),
2- occupational trauma (e.g., the use of pneumatic hammers
or chain saws producing vibration),
3- the thoracic outlet syndrome,
4- the carpal tunnel syndrome
5- certain drugs (e.g., β-blockers, ergot alkaloids, methysergide, vinblastine, bleomycin,
amphetamines, bromocriptine, and cyclosporine).
5- Acrocyanosis :
- is a symmetrical distal blue discoloration, usually occurring
below the wrists and ankles,
- is due to arteriolar constriction and is aggravated by cold.

37. 6- Livedo reticularis :
- a vasomotor disorder that affects the extremities and the trunk and is due
to vasospasm with obstruction of the perpendicular arterioles or stasis of
blood in the superficial veins.
- It may be a benign disorder or may be associated with vasculitis, anti-
phospholipid antibodies (Sneddon's syndrome), connective tissue disease,
hyperviscosity syndrome, thrombocythemia,
and drugs (e.g., amantadine, quinine, and quinidine).
7- vasomotor paralysis :
- lesions of the sympathetic pathways
- surgical sympathectomy , trauma , and tumor infiltration .
8- Erythromelagia (painful acrus eryhromatous):
- A condition cutaneous warming and is associated with an intense burning
sensation.
- It may occur in patients with small-fiber neuropathies.
- The mechanism may be activation of neurogenic flare by a polymodal C-
nociceptor axon and release of vasodilator and algogenic neuropeptides
such as substance P.

39. 9- Hyperhydrosis : may be generalized or localized
- Generalized hyperhidrosis :
- may be secondary to infections, malignancies, or neuroendocrine disorders (e.g.,
pheochromocytoma, thyrotoxicosis, acromegaly, carcinoid, anxiety, hypotension,
hypoglycemia, and cholinergic agents) .
- Localized hyperhydrosis :
- rare and may occur with injury to the spinal cord
(e.g., in syringomyelia), peripheral nerves (e.g., with partial
median or sciatic nerve injury), or eccrine sweat glands.
- Perilesional hyperhidrosis : may surround an anhidrotic
region produced by a lesion of the sympathetic ganglia or rami.
- Primary or essential hyperhidrosis : affects the axillary, palmar, and plantar
regions and may be familial.
- Treatment :
- medical : systemic anticholinergic and B-blocker and topical agents as
aluminium antiperspirants. ineffective
- surgical : sympathectomy  effective with more side effects (horner $,
pneumothorax, compensatory sweating in othe areas)
- intradermal injection of botulinium toxin type A  effective mainly in 1ry
palmar hyperhydrosis .

43. 10- Regional pain syndromes :
- Reflex sympathetic dystrophy (chronic regional pain syndrome type 1):
- is a pain syndrome defined as a continuous burning pain, hyperpathia
(exaggerated response to painful stimuli), and allodynia (perception of an
innocuous stimulus as painful) in a portion of an extremity.
- It is associated with signs of sympathetic hypoactivity or hyperactivity
and usually follows minor trauma that does not involve major nerves.
- Causalgia (chronic regional pain syndrome type 2):
- is a similar syndrome that occurs after partial injury of a nerve or one of
its major branches.
- Vasomotor and sudomotor instability, manifestations of sympathetic
hyperactivity or hypoactivity, are commonly seen.
- Treatment : Sympathetic blockade may be of
benefit.

46. 3- GIT dysmotility
GIT hypomotility GIT hypermotility
management management
1-  dietary fibres, water intake, exercise
2- stool softeners (decusate Na), lubricants
(mineral oil), osmotic agents (lactulose).
3- prokinetic drugs e.g domperidone,
Metoclopramide, erythromycon
4- colon surgery
1- In unexplained diarrhea  tetracycline
or metronidazole.
2- Opioids(loperamide, diphenoxylate) 
↓peristalsis, rectal sphincter tone.
3- α2-agonist(clonidine) ttt of diarrhea
assoc. with diabetec dysautonomia.
4- Idiopathic fecal incontinence  high
fiber bulking, daily tap water enemas
5- surgical  sphincter repair, colostomy,
Artificial sphincter, creation of new
Sphincter with muscle graft

47. 4- Neurogenic Bladdar
Spastic (UMNL)
(reflex type)
Flaccid (LMNL)
(non-reflex type)
Acute Gradual
The terms reflex and nonreflex denote the presence or absence,
respectively, of bulbocavernosus and anal reflexes.
Afferent Efferent
Spinal
center
Retention with
overflow
precipitancy
Automatic
bladdar
Sensory atonic
bladdar
Motor atonic
bladdar
Autonomic
bladdar

50.  Spastic bladdar :
1- Uninhibited bladder : ( detrucer hyperreflexia without outlet obstruction )
- associated with lesions of the medial frontal region that results in urinary incontinence but not
urinary retention because the detrusor-sphincter synergy is preserved.
- Ttt : - anticholinergic e.g oxybutenine, tolteredine
- Timed voiding & fluid restriction
- in severe cases: both intrathecal baclofen infusion and dorsal rhizotomy may be effective.
- in MS & refractory detruser hyperreflexia : Augmantaion cystoplasty
2- Automatic bladder : ( detrucer hyperreflexia with outlet obstruction )
- results from lesions of the spinal cord that interrupt the pathway from the pontine micturition
centers.
- An automatic bladder is associated with urgency, frequency, incontinence, and urinary retention that
is due to detrusor-sphincter dyssynergia.
- Ttt : - alpha1- antagonist e.g prazosin  ↓ bladdar outlet sphincter tone
- baclofen , dantrolene  ↓ tone of striated external sphincter
- the most useful : anticholinergic + intermittent self catheterization
- recent ttt : surgical sphincterotomy.

51.  Flaccid bladdar : ( Detrusor Areflexia or Poor Bladder Contractility )
- occurring with lesions of the cauda equina or peripheral nerves,
- characterized by incomplete bladder emptying, urinary retention, and overflow
incontinence
- Ttt :
- The simplest & most effective method  intermittent self catheterization
- If there`s motor disability  indwelling catheter or suprapupic dfiversion
- In women  crede`s method
- Medical ttt  bethanechol HCL : muscarinic agonist with selective action on U.B.
- In cauda equina or conus lesion  α-adrenergic agonist e.g ephedrine &
phenylpropanolamine

52. 5- Sexual dysfunction
Erectile dysfunction Ejaculation dysfunction