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.

Surgical Care

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

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

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.

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.

Medication

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Media Gallery

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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Author

Reza Behrouz, DO, FACP Department of Neurology, UT Health San Antonio

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

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 Morsani College of Medicine

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK life science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Eisai, Jazz, LivaNova, Neurelis, SK life science, Stratus, Sunovion, UCB<br/>Received research grant from: Cerevel Therapeutics, Ovid Therapeutics, Neuropace, Greenwich Jazz, SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Neuroelectrics Corporation, Longboard, Janssen, Equilibre Biopharmaceuticals.

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 in St Louis 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; Physician Advisory Board for Coherex Medical; National Leader and Steering Committee Clinical Trial, Bristol Myers Squibb; Abbott Laboratories, advisory group.

Additional Contributors

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

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

Disclosure: Nothing to disclose.