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CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy) Treatment & Management

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

Medical Care

Neither a cure nor a specific treatment exist for CADASIL. Treatment endeavors should be focused on management of headaches, mood and psychiatric disturbances, and rehabilitation following a brain ischemic injury.

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Surgical Care

There is no specific surgical procedure indicated for the treatment of CADASIL.

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Consultations

See the list below:

  • Vascular neurology: To assist in diagnosis, patients who are suspected to have CADASIL should be seen and later observed by a vascular neurologist.
  • Dermatology: Consultation should be placed to a dermatologist for skin biopsy in those patients in whom biopsy is desired (for example, patients in whom genetic testing is negative but confirmation of the disease would be helpful).
  • Dermatopathology: The skin biopsy specimen should preferentially be investigated by a dermatopathologist.
  • Psychiatry: The many forms of psychiatric manifestations of CADASIL may be managed more appropriately by a psychiatrist.
  • Neuropsychology: A neuropsychologist can assist in gauging cognitive status or rate of deterioration.
  • Physical therapy
  • Occupational therapy
  • Speech therapy
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Diet

No specific diet is recommended for patients with CADASIL. However, because of the risk of developing dysphagia secondary to cerebral infarctions, some may eventually require percutaneous endoscopic gastrostomy (PEG) or jejunal feeding tube. Swallow evaluation should be performed after every ischemic episode or if the swallowing ability of the patient becomes questionable due to progressive pseudobulbar palsy.

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Activity

Physical and cognitive disability is progressive and may be quite severe. In patients with CADASIL, it is important to frequently assess their ability in performing activities of daily living, ambulation, and self-care. A rehabilitation regimen should be prescribed when appropriate.

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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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