eMedicine Specialties > Neurology > Neuro-vascular Diseases

Cerebral Amyloid Angiopathy: Treatment & Medication

Author: Ravi S Menon, MD, Clinical Fellow in Stroke Diagnostic and Therapeutics, National Institute of Health, National Institute of Neurological Disorders and Stroke
Coauthor(s): Jose G Merino, MD, Medical Director, Suburban Hospital Stroke Program; Vladimir C Hachinski, MD, MSc, DSc, FRCP(C), Professor, Departments of Medicine, Physiology, London Health Sciences Center, University of Western Ontario, Canada
Contributor Information and Disclosures

Updated: Aug 20, 2008

Treatment

Medical Care

  • Cerebral amyloid angiopathy (CAA) is largely untreatable at this time.
  • The management of CAA-related intracranial hemorrhage (ICH) is identical to the standard management of ICH. Pay special attention to reversing anticoagulation, managing intracranial pressure, and preventing complications.
  • If coexisting vasculitis is found on angiography and brain biopsy, long-term treatment (up to 1 y) with steroids and cyclophosphamide is indicated.
  • A syndrome of subacute cognitive decline, seizures, and white matter changes on MRI with perivascular inflammatory changes on biopsy was recently described. Some patients improved clinically (but not to baseline) when given corticosteroids or cyclophosphamide.
  • Although early investigations had shown the safety of Cerebril (Neurochem, Inc), a drug developed to reduce amyloid formation and deposition, this drug is currently not being actively studied for CAA. A small study of patients with amyloidogenic transthyretin (ATTR) Tyr11, a hereditary cause of CAA, assessed effects of liver transplantation. While mortality and occurrence of cerebral hemorrhage and dementia in 3 patients having transplantations were reduced compared with 5 patients not having transplantations, the small number of patients makes it difficult to know how generalized the results will be.4

Surgical Care

  • Hematoma evacuation can be life saving when the hematoma causes significant mass effect and predisposes to herniation, particularly when medical management of increased intracranial pressure yields no response. The goal of therapy is to lower intracranial pressure.
    • No evidence is available from well-designed, randomized clinical trials that can help determine which patients benefit from evacuation of the hematoma. However, that the intervention should be considered in patients with intermediate-sized hematomas (20-60 mL) who have a progressive deterioration in their level of consciousness is agreed.
    • Surgery should be performed before coma develops.
    • Surgery is not beneficial for small or very large hematomas. Patients with small (<20 mL) hematomas and minimally decreased levels of consciousness tend to have good outcomes with conservative treatment. When the hematoma is large (>60 mL) and the patient is lethargic or comatose, the prognosis is poor despite surgical evacuation.
  • Early concerns about the safety of hematoma evacuation in patients with CAA-related ICH were unfounded. Several recent series have reported low rates of mortality and postoperative hematoma; surgical evacuation of the hematoma should be performed when clinically indicated.
    • No evidence supports the belief that evacuation leads to an increased rate of recurrence. A large series that evaluated 50 neurosurgical procedures in 37 patients with CAA-related ICH found a mortality rate of 11% and a 5% rate of postoperative hematoma that required intervention.5 Risk factors associated with an adverse postoperative outcome were age older than 75 years and the presence of a parietal hematoma.
    • Although transoperative oozing from the walls of the hematoma was a common occurrence, it could be controlled easily with an absorbable hemostat (eg, oxidized cellulose, gelatin sponge) or fibrin glue.
  • When determining whether evacuation of the hematoma is appropriate, consider the patient's cognitive status.

Consultations

  • Neurologic evaluation for clinical evaluation, diagnostic workup, and management
  • Neurosurgical consultation in cases of ICH
  • Neuropsychological assessment for cognitive impairment

Diet

No special diet

Activity

Activities should not be restricted. However, patients should avoid head trauma of any degree.

More on Cerebral Amyloid Angiopathy

Overview: Cerebral Amyloid Angiopathy
Differential Diagnoses & Workup: Cerebral Amyloid Angiopathy
Treatment & Medication: Cerebral Amyloid Angiopathy
Follow-up: Cerebral Amyloid Angiopathy
Multimedia: Cerebral Amyloid Angiopathy
References
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References

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Further Reading

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Keywords

cerebrovascular amyloidosis, cerebral amyloid angiopathy, congophilic angiopathy, dysphoric angiopathy, β-amyloid, beta-amyloid, Alzheimer's disease, intracranial hemorrhage, ICH, dementia, transient neurologic events, hereditary cerebral hemorrhage with amyloidosis, hereditary cerebral hemorrhage with amyloidosis-Dutch type, hereditary cerebral hemorrhage with amyloidosis-Icelandic type, HCHWA, cerebral microbleeds, stroke, ischemic strokes

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Contributor Information and Disclosures

Author

Ravi S Menon, MD, Clinical Fellow in Stroke Diagnostic and Therapeutics, National Institute of Health, National Institute of Neurological Disorders and Stroke
Ravi S Menon, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, and American Stroke Association
Disclosure: Nothing to disclose.

Coauthor(s)

Jose G Merino, MD, Medical Director, Suburban Hospital Stroke Program
Jose G Merino, MD is a member of the following medical societies: American Heart Association and American Stroke Association
Disclosure: Nothing to disclose.

Vladimir C Hachinski, MD, MSc, DSc, FRCP(C), Professor, Departments of Medicine, Physiology, London Health Sciences Center, University of Western Ontario, Canada
Vladimir C Hachinski, MD, MSc, DSc, FRCP(C) is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Neurological Association, and Ontario Medical Association
Disclosure: Mitsubi Tanaba Pharma Corporation Honoraria Speaking and teaching; Ferrer Group Honoraria Speaking and teaching

Medical Editor

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.

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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