eMedicine Specialties > Neurology > Neuro-vascular Diseases

Metabolic Disease and Stroke - Fabry Disease: Follow-up

Author: Pitchaiah Mandava, MD, PhD, Assistant Professor, Department of Neurology, Baylor College of Medicine; Consulting Staff, Department of Neurology, Michael E DeBakey Veterans Affairs Medical Center
Coauthor(s): Thomas A Kent, MD, Professor, Department of Neurology, Baylor College of Medicine; Neurology Care Line Executive, Michael E DeBakey Veterans Affairs Medical Center
Contributor Information and Disclosures

Updated: Dec 11, 2008

Follow-up

Inpatient & Outpatient Medications

  • Antiplatelet agents: Aspirin, ticlopidine, clopidogrel, and aspirin-dipyridamole in doses mentioned in the Medication section are used routinely to prevent recurrent ischemic strokes of thrombotic type.
  • Anticoagulant: Warfarin is often used to prevent cardioembolic strokes.

Transfer

  • Fabry disease is uncommon, and its diagnosis and treatment can be challenging.
  • Treatment strategies involve combined efforts from multiple specialties. The diagnosis and care of these patients usually is best handled at tertiary care centers.
  • Acute strokes may be managed adequately in community hospitals in the initial phases.
  • Aggressive efforts to diagnose the etiology of stroke are necessary to plan secondary prevention strategies. In this context, unusual presentations with multiple organ involvement or lack of traditional vascular risk factors should suggest consideration of Fabry disease. Traditional secondary stroke prevention strategies are still necessary.
  • Further care can be accomplished by means of consultation with tertiary care centers.

Prognosis

  • Fabry disease affects multiple organ systems in the body. Death, a result of renal failure, heart involvement, or stroke, usually occurs by the fifth decade.
  • After a first stroke, recurrent stroke is frequent, with a median interval to first recurrence of 6.4 years in hemizygotes.

Patient Education

For excellent patient education resources, visit eMedicine's Stroke Center. Also, see eMedicine's patient education article Stroke.

Miscellaneous

Medicolegal Pitfalls

  • Signs and symptoms of Fabry disease may be nonspecific. If manifestations in different organs are considered in isolation, the unifying diagnosis may be missed.
  • If the family history suggests a diagnosis of Fabry disease, genetic testing and counseling should be offered to all family members, regardless of their sex.
 


More on Metabolic Disease and Stroke - Fabry Disease

Overview: Metabolic Disease and Stroke - Fabry Disease
Differential Diagnoses & Workup: Metabolic Disease and Stroke - Fabry Disease
Treatment & Medication: Metabolic Disease and Stroke - Fabry Disease
Follow-up: Metabolic Disease and Stroke - Fabry Disease
References
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References

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Further Reading

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Keywords

Fabry's disease, angiokeratoma corporis diffusum, glycolipid lipidosis, metabolic disease and stroke, Fabry disease, lysosomal disorder, glycosphingolipids

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Contributor Information and Disclosures

Author

Pitchaiah Mandava, MD, PhD, Assistant Professor, Department of Neurology, Baylor College of Medicine; Consulting Staff, Department of Neurology, Michael E DeBakey Veterans Affairs Medical Center
Pitchaiah Mandava, MD, PhD is a member of the following medical societies: American Academy of Neurology, Sigma Xi, and Stroke Council of the American Heart Association
Disclosure: Nothing to disclose.

Coauthor(s)

Thomas A Kent, MD, Professor, Department of Neurology, Baylor College of Medicine; Neurology Care Line Executive, Michael E DeBakey Veterans Affairs Medical Center
Thomas A Kent, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, New York Academy of Sciences, Royal Society of Medicine, Sigma Xi, and Stroke Council of the American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey L Saver, MD, Director, Stroke Center, Professor, Department of Neurology, University of California at Los Angeles Medical Center
Jeffrey L Saver, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Neurological Association, and National Stroke Association
Disclosure: Boehringer-Ingelheim - Secondary Prevention Consulting fee Consulting; Talacris Consulting fee Consulting; ImaRx Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association
Disclosure: Boehringer Ingelheim Honoraria Speaking and teaching; BMS/Sanofi Honoraria Speaking and teaching; Novartis Honoraria Speaking and teaching

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD, Professor, Department of Neurology, Oregon Health and Science University; Associate Director, Oregon Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association
Disclosure: Co-Axia Consulting fee Review panel membership; Talecris Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Concentric Medical Consulting fee Review panel membership; Abbott Consulting fee Consulting; Sanofi  Consulting

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