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.