Autonomic neuropathy and autonomic disorders

Clinical Classification of Autonomic Disorders

Isolated autonomic failure:

    • Autoimmune autonomic ganglionopathy

    • Paraneoplastic autonomic neuropathy

Progressive:

  • Pure autonomic failure

Progressive autonomic failure associated with parkinsonism, ataxia, or dementia:

  • Multiple system atrophy

  • Lewy body disorders (Parkinson disease, dementia with Lewy bodies)

Autonomic failure associated with peripheral neuropathy

  • Acute or subacute:

    • Guillain-Barré syndrome

    • Porphyria

  • Chronic acquired:

    • Diabetes

    • Amyloidosis

    • Immune (Sjögren disease, paraneoplastic)

    • Metabolic (vitamin B12 deficiency, uremia)

    • Toxic (alcohol, chemotherapeutic agents)

    • Infectious (HIV, leprosy)

    • Radiation therapy to neck

  • Hereditary:

    • Hereditary sensory and autonomic neuropathies

    • Fabry disease

Distal autonomic and painful neuropathies:

  • Acquired

    • Idiopathic

    • Inflammatory (vasculitis) or infectious (e.g., HIV)

  • Hereditary (e.g., familial erythromelalgia)

Focal central nervous system disorders (associated with autonomic failure or hyperactivity):

  • Insular stroke

  • Temporal lobe epilepsy

  • Limbic encephalitis

  • Fatal familial insomnia

  • Brainstem lesions

  • Spinal cord injury

  • Syringomyelia

Focal peripheral disorders:

  • Cranial disorders (gustatory sweating, harlequin syndrome)

  • Chronic regional pain syndrome

Syndromes of orthostatic intolerance:

  • Postural tachycardia syndrome

  • Neurally mediated (reflex) syncope

Primary disorders of sweating:

  • Essential anhidrosis

  • Primary focal hyperhidrosis

Drug-induced disorders:

  • Neuroleptic malignant syndrome

  • Serotonin syndrome

  • Anticholinergic toxicity

  • Alcohol withdrawal

  • Stimulant intoxication

Neuropathies with Autonomic Nervous System Involvement

Acute Neuropathies (4 weeks or less)

  • Acute panautonomic neuropathy (acute autonomic ganglionopathy)

    • Widespread failure of adrenergic and cholinergic systems

    • Orthostatic hypotension, xerostomia, anihidrosis, fixed heart rate, fixed pupils, and constipation.

  • Acute paraneoplastic autonomic neuropathy

    • Widespread failure of adrenergic and cholinergic systems

    • Orthostatic hypotension, anihidrosis, fixed heart rate.

  • Acute cholinergic neuropathy

    • Restricted to cholinergic failure

    • Xerostomia, anhidrosis, fixed HR, urinary retention.

  • Acute adrenergic neuropathy

    • Restricted to adrenergic failure

    • Adrenergic supersensitivity, HTN, postural tachycardia

  • Botulism

    • Cholinergic failure

    • Xerostomia, anhidrosis, fixed HR, urinary retention, ileus, orthostatic hypotension

  • Guillain-Barré syndrome

    • Cholinergic failure, adrenergic overactivity

    • Anhidrosis, tachycardia, hypo/hypertension

  • Porphyria

    • Adrenergic overactivity

    • HTN, tachycardia, piloerection

  • Toxic: vincristine, Vacor (rodenticide), cisplatin, amiodarone, heavy metals, etc.

    • Widespread adrenergic and cholinergic failure

    • Orthostatic hypotension, GI hypomotility, urinary retention.

Chronic Neuropathies (8 weeks or more)

  • Diabetes mellitus

  • Amyloid neuropathy (familial and primary)

  • Idiopathic panautonomia

  • Pure autonomic failure (PAF)

  • Paraneoplastic (LEMS)

  • Pure adrenergic neuropathy

  • Chronic idiopathic anhidrosis

  • Sjogren's syndrome

  • HSAN I

  • HSAN III (Riley-Day)

  • Distal small-fiber neuropathy

  • Sensory neuronopathy

  • Adie syndrome

  • Chagas disease

  • Dysautonomia of old age

  • Paraneoplastic sensory neuropathy (malignant inflammatory sensory polyganglionopathy)

  • Human immunodeficiency virus–related autonomic neuropathy

Orthostatic intolerance

  • POTS

  • Mild orthostatic intolerance

  • Syncope

Toxic

  • Organic Solvents (eg, n-Hexane)

  • Acrylamide

  • Heavy Metals (eg, Lead, Arsenic, Thallium, Mercury)

  • N-3-Pyridylmethyl-N′-p-Nitrophenyl Urea Rat Poison (of Historic Relevance)

  • Vinca alkaloids

  • Platinum derivatives

  • Taxanes

  • Epothilones

  • Bortezomib

  • Thalidomide, lenalidomide, pomalidomide

  • Doxorubicin

  • Cytosine arabinoside

  • Perhexiline maleate

  • Amiodarone

  • Pentamidine

  • Gold

  • Podophyllin

  • Marine Toxins (eg, Ciguatera)

Hereditary

  • Familial amyoidosis

  • HSAN (types 1-IV)

  • Fabry's disease

  • Tangier's disease

  • Mitochondrial disorders

The Clinical Evaluation In Autonomic dysfunction

Clinical Manifestations of Autonomic Failure | Output Consequences of Failure | Clinical Manifestation

  • Sympathetic vasomotor | Impaired baroreflex triggered vasoconstriction | Orthostatic hypotension, postprandial hypotension

  • Sympathetic sudomotor | Anhidrosis | Heat intolerance

  • Cranial parasympathetic | Impaired salivary and lacrimal secretion | Dry eyes (xerophthalmia), dry mouth (xerostomia)

Impaired pupillary light reflex | Blurred vision (impaired accommodation)

  • Sacral parasympathetic | Impaired output to the bladder, sexual organs, and rectum | Urinary retention, erectile dysfunction

  • Enteric nervous system | Impaired vagal control of the esophagus and stomach | Nausea and early satiety, abdominal distention

Impaired peristaltic reflexes | Constipation,

Impaired prevertebral ganglion reflexes | Ileus, diarrhea.