E. Lee Murray, MD

OVERVIEW

Autonomic difficulty is usually a component of a systemic disease with other neurologic manifestations. A commonality is orthostatic hypotension for most of these. Once the diagnosis of autonomic insufficiency has been made, the differential diagnosis includes the following:

•Idiopathic orthostatic hypotension: Pure autonomic failure without other deficits

•Diabetic autonomic insufficiency: Most common autonomic disorder. Autonomic neuropathy is typically a component of a multimodal neuropathy.

•Multiple system atrophy: Autonomic failure associated with other neurologic deficits including extrapyramidal, corticospinal, or cerebellar dysfunction

•Paraneoplastic autonomic neuropathy: Autonomic neuropathy before or after diagnosis of cancer. Often with other neurologic deficits from peripheral neuropathy, cognitive change, seizures, or Lambert-Eaton myasthenic syndrome.

•Hereditary autonomic neuropathy: Family of disorders of which autonomic involvement is a component. Type 3 is familial dysautonomia.

IDIOPATHIC ORTHOSTATIC HYPOTENSION

Idiopathic orthostatic hypotension (IOH) is prominent orthostasis with no other associated neurologic or medical disease.

PRESENTATION is with presyncopal sensation on standing. There may also be noticeable anhydrosis. Erectile dysfunction can occur also. The orthostasis can be disabling.

DIAGNOSIS is suspected with a history and exam compatible with orthostasis. Electromyogram (EMG) is done if the autonomic difficulty is believed to be a component of a peripheral neuropathy. Labs for other causes may include Hgb A1C (diabetes), paraneoplastic antibodies, VGCC antibodies (LEMS), and porphyrins if these disorders are suspected.

MANAGEMENT includes use of agents to reduce the orthostasis, including especially midodrine and fludrocortisone. Fluid and salt management is important also. Cessation of drugs that can contribute to autonomic insufficiency is recommended.

DIABETIC AUTONOMIC INSUFFICIENCY

Diabetic autonomic neuropathy can have a wide range of effects; these can include:

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