Autonomic nervous system specific diseases

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Symptoms of autonomic dysfunction occur in many common conditions which affect both the parasympathetic and sympathetic pathways e.g. cerebrovascular disease.

The following arc less common disorders which primarily may affect the autonomic nervous system -

IDIOPATHIC ORTHOSTATIC HYPOTENSION

Two types of this condition are recognised:

1. Due to degeneration of sympathetic postganglionic neurons.

2. Due to degeneration of sympathetic preganglionic neurons of the intermediolateral column T1-T12 -SHY-DRAGER SYNDROME.

In the latter disorder, features of extrapyramidal system involvement arc also found. Both disorders are characterised by: postural hypotension: anhidrosis (absent sweating): impotence:

sphincter disturbance: pupillary abnormalities. The disorders may be separated pharmacologically; the postganglionic disorder shows hypersensitivity (denervation hypersensitivity) to noradrenaline infusion. Treatment

Drugs such as fludrocortisone increase blood volume and may prevent postural hypotension. DIABETIC AUTONOMIC NEUROPATHY

Symptoms of autonomic dysfunction are common in long-standing insulin-dependent diabetics: Impotence/retrograde ejaculation.

Bladder dysfunction - decreased detrusor muscle action - resulting in increased residual volume. Nocturnal diarrhoea.

G1 dysfunction - vomiting from gastroparcsis. Orthostatic hypotension.

These problems arise from damage to both sympathetic and parasympathetic postganglionic neurons. Treatment

Improve diabctic control and treat symptoms e.g. fludrocortisone for BP control.

POST-INFECTIOUS POLYNEUROPATHY - Guillain-Barré syndrome (see previous chapter). Autonomic involvement occurs commonly in this disorder and may present major problems in patient management. The lesion may involve the afferent or efferent limbs of the cardiovascular reflexes (baroreceptor reflexes) resulting in postural hypotension, episodes of hypertension and cardiac dysrhythmias. Occasionally the postinfectious neuropathy is purely autonomic.

HEREDITARY AUTONOMIC NEUROPATHY e.g. RILEY-DAY Syndrome

This autosomal recessive disorder occurs in persons of Jewish descent.

Features of autonomic dysfunction: postural hypotension, oesophageal reflux, hyperpyrexia - present from birth. Insensitivity to pain results with associated sensory neuropathy.

PRIMARY AMYLOIDOSIS

Autonomic involvement with orthostatic hypotension, impotence, diarrhoea and bladder involvement may accompany sensimotor neuropathy in the primary and hereditary forms. Amyloid infiltration affects autonomic ganglia.

ADIE'S SYNDROME

A tonic pupil (page 140) associated with areflexia and occasionally widespread autonomic dysfunction, e.g. segmental hypohidrosis (absent sweating) and diarrhoea.

AUTONOMIC DYSFUNCTION IN QUADRIPLEGIA (autonomic dysreflexia)

A high cervical lesion which completely severs the spinal cord, e.g. traumatic cervical fracture/dislocation will isolate all but the cranial parasympathetic outflow. As a result, disturbed autonomic function is inevitable but variable.

Autonomic reflexes arc retained - Passive movement or tactile stimulation of limbs may result in blood pressure rise, bradycardia, sweating, reflex penile erection (priapism).

Automatic Nerves The Penis

sympathetic parasympathetic

Detrusor muscle

Hypogastric plexus

Pelvic nerves (nervi erigentes) \

i Hypogastric 4 nerve

bP,nal sphincter cord ^¿s

Function: somatic efferent

Detrusor muscle relaxation

Internal sphincter contraction

Hypogastric External Plexus sphincter ^^

Function

Detrusor muscle contraction Internal sphincter relaxation

Origin: anterior horn cells S2,3,4 -Voluntary innervation corticai. control

Frontal lobe: paracentral lobe

- initiates micturition

- inhibits micturition

Afferent innervation parasympathetic sympathetic

Enter through posterior rami and terminate in anteromediolateral column T9-L2

Pudendal nerve

BLADDER

Hypogastric plexus

Enter through \ posterior rami ^ and terminate in anterolateral \ column, S2,3,4.

Function:

Sensation of painful distension conveyed from bladder wall

Function:

Sensation of pain and distension conveyed from bladder wall and internal sphincter

The afferent pathways are responsible for the sensation of bladder fullness

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