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.

https://pmc.ncbi.nlm.nih.gov/articles/PMC4222929/

https://pharmaceutical-journal.com/article/feature/hacking-the-vagal-system-to-treat-chronic-cough

https://pmc.ncbi.nlm.nih.gov/articles/PMC7996811/