eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases
Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes: Follow-up
Updated: Nov 18, 2009
Follow-up
Further Inpatient Care
- Physical therapy is useful for gait training. Patients also should be instructed in safety precautions.
- Patients should be monitored closely for drug efficacy and adverse effects. Doses should be adjusted accordingly.
Prognosis
- The prognosis for autoimmune autonomic neuropathy (AAN) is poor without treatment, and many patients have residual autonomic symptoms. With IVIg therapy, a few patients who are treated early in the disease course can have excellent recovery of function. However, additional patients must be treated to confirm the initial favorable findings.
- Patients with pure autonomic failure have symptoms that remain confined to the autonomic nervous system. These patients generally improve little over time, and their symptoms may worsen. Some may later develop multiple system atrophy or Parkinson's disease.
- The prognosis for patients with multiple system atrophy is poor overall. Neurologic function declines gradually over time. The autonomic symptoms often become debilitating. Survival is typically 6-9 years from the time of diagnosis.
Miscellaneous
Medicolegal Pitfalls
- Failure to diagnose and treat acute inflammatory demyelinating polyneuropathy
- Failure to detect an associated malignancy at the time of presentation
- Failure to prevent fatal cardiac events
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| Treatment & Medication: Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes |
Follow-up: Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes |
| References |
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References
Klein CM, Vernino S, Lennon VA, et al. The spectrum of autoimmune autonomic neuropathies. Ann Neurol. 2003;53:752-8. [Medline].
Sandroni P, Vernino S, Klein CM, et al. Idiopathic autonomic neuropathy: comparison of cases seropositive and seronegative for ganglionic acetylcholine receptor antibody. Arch Neurol. Jan 2004;61(1):44-8. [Medline].
Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. Feb 1 1999;163(1):94-8. [Medline].
Sommer C, Lauria G. Skin biopsy in the management of peripheral neuropathy. Lancet Neurol. Jul 2007;6(7):632-42. [Medline].
Freeman R. Clinical practice. Neurogenic orthostatic hypotension. N Engl J Med. Feb 7 2008;358(6):615-24. [Medline].
Schroeder C, Vernino S, Birkenfeld AL, et al. Plasma exchange for primary autoimmune autonomic failure. N Engl J Med. Oct 13 2005;353(15):1585-90. [Medline].
Heafield MT, Gammage MD, Nightingale S, Williams AC. Idiopathic dysautonomia treated with intravenous gammaglobulin. Lancet. Jan 6 1996;347(8993):28-9. [Medline].
Quan D, Rich MM, Bird SJ. Acute idiopathic dysautonomia: electrophysiology and response to intravenous immunoglobulin. Neurology. Feb 8 2000;54(3):770-1. [Medline].
Gibbons C, Vernino S, Freeman R. Combined immunomodulation therapy in autoimmune autonomic ganglionopathy. Arch Neurol. Feb 2008;65(2):213-217.
Bannister R, Mathias CJ, Polinsky R. Autonomic failure--A comparison between UK and US experience. In: Autonomic Failure: A Textbook of Clinical Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1988:282-8.
Daniel SE. The neuropharmacology and neurochemistry of multiple system atrophy. In: Autonomic Failure: A Textbook of Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1992:564-85.
Hoeldtke RD, Bryner KD, Hoeldtke ME, Hobbs G. Treatment of autonomic neuropathy, postural tachycardia and orthostatic syncope with octreotide LAR. Clin Auton Res. Dec 2007;17(6):334-40. [Medline].
Kaufmann H. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure and multiple system atrophy. Clin Auton Res. Apr 1996;6(2):125-6. [Medline].
Mathias CJ. Disorders of the autonomic nervous system. In: Neurology in Clinical Practice. Boston, Mass: Butterworth-Heinemann; 1996:1953-81.
Matthews MR. Autonomic ganglia in multiple system atrophy and pure autonomic failure. In: Autonomic Failure: A Textbook of Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1992:593-621.
Quinn NP, Wenning G, Marsden CD. The Shy-Drager syndrome. What did Shy and Drager really describe?. Arch Neurol. Jul 1995;52(7):656-7. [Medline].
Smit AA, Vermeulen M, Koelman JH, Wieling W. Unusual recovery from acute panautonomic neuropathy after immunoglobulin therapy. Mayo Clin Proc. Apr 1997;72(4):333-5. [Medline].
Suarez GA, Fealey RD, Camilleri M, Low PA. Idiopathic autonomic neuropathy: clinical, neurophysiologic, and follow-up studies on 27 patients. Neurology. Sep 1994;44(9):1675-82. [Medline].
van Ingelghem E, van Zandijcke M, Lammens M. Pure autonomic failure: a new case with clinical, biochemical, and necropsy data. J Neurol Neurosurg Psychiatry. Jun 1994;57(6):745-7. [Medline].
Vernino S, Freeman R. Peripheral Autonomic Neuropathies. Continuum Lifelong Learning Neurol. Dec 2007;13(6):89-110.
Further Reading
Keywords
acute idiopathic dysautonomia, multiple system atrophy, olivopontocerebellar atrophy, pure autonomic failure, Shy-Drager syndrome, striatonigral degeneration, postural tachycardia syndrome, autoimmune autonomic neuropathy, autoimmune autonomic ganglionopathy, acute pandysautonomia, acute panautonomic neuropathy
Follow-up: Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes