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CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy) Follow-up

  • Author: Reza Behrouz, DO, FACP; Chief Editor: Helmi L Lutsep, MD  more...
 
Updated: Nov 25, 2015
 

Further Outpatient Care

Patients with CADASIL should be followed routinely by a neurologist or a vascular neurologist to monitor their degree of disability and rate of progression. Management is primarily symptom-based and addressing rehabilitation needs is paramount. Routine psychiatric follow-up is also reasonable if the patient suffers from a complex mental disorder.

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Further Inpatient Care

Inpatient care of patients with CADASIL depends on the reason for admission. Inpatient care of patients with CADASIL who present with a stroke is not entirely different than patients without CADASIL who have a stroke (see Acute Stroke Management). However, safety and efficacy of recombinant tissue plasminogen activator (rtPA) in patients with CADASIL who present with an acute cerebral infarction within the therapeutic window has not been systematically assessed.

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Inpatient & Outpatient Medications

See Medication.

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Prognosis

Exact mortality rate in patients with CADASIL is unknown.

Age at onset for stroke is 45-50 years.

Mean age at death has been reported to be 61 years after a mean disease duration of approximately 23 years.[6]

Close to 80% of patients are completely dependent immediately before death.[7]

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Patient Education

Genetic counseling

Because CADASIL has an autosomal dominant inheritance, patients and their families should be counseled on the nature of the disorder and the probability of developing or transmitting it.

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Contributor Information and Disclosures
Author

Reza Behrouz, DO, FACP Assistant Professor, Division of Cerebrovascular Diseases and Neurological Critical Care, Department of Neurology, The Ohio State University College of Medicine

Reza Behrouz, DO, FACP is a member of the following medical societies: American Academy of Neurology, American College of Physicians, Society of Critical Care Medicine, Society for Vascular Medicine, Neurocritical Care Society

Disclosure: Nothing to disclose.

Coauthor(s)

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Glenn Lopate, MD Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association

Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2.

Additional Contributors

Paul E Barkhaus, MD Professor of Neurology and Physical Medicine and Rehabilitation, Department of Neurology, Medical College of Wisconsin; Section Chief, Neuromuscular and Autonomic Disorders, Department of Neurology, Director, ALS Program, Medical College of Wisconsin

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

References
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FLAIR MRI of the brain showing hyperintensities involving the temporal poles in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). (Reprinted with permission from Mayo Clin Proc, Meschia, 2005.)
FLAIR MRI of the brain showing hyperintensities involving bilateral external capsules in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). (Reprinted with permission from Mayo Clin Proc, Meschia, 2005.)
 
 
 
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