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

Updated: Oct 09, 2018
  • Author: Reza Behrouz, DO, FACP; Chief Editor: Helmi L Lutsep, MD  more...
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Treatment

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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