**Author details**

Vikas Chaudhary1\*, Shahina Bano2 , Sachchidanand Yadav2 , Umesh Chandra Garga2 and Sachin Kumar Singh3

\*Address all correspondence to: dr\_vikaschaudhary@yahoo.com

1 Department of Radiodiagnosis, Employees' State Insurance Corporation (ESIC) Model Hospital, Gurgaon, Haryana, India

2 Department of Radiodiagnosis, PGIMER, Dr. Ram Manohar Lohia (RML) Hospital, India

3 Department of Radiodiagnosis, Jawahar Lal Nehru (JLN) Medical College, Bhagalpur, Bi‐ har, India

#### **References**


[4] Andreas C, Qiang G, David JW. Sporadic cerebral amyloid angiopathy revisited: re‐ cent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psy‐ chiatry 2012;83:124-37.

**6. Summary**

48 Intracerebral Hemorrhage

cortical hemorrhage.

**Author details**

Sachin Kumar Singh3

har, India

**References**

Vikas Chaudhary1\*, Shahina Bano2

Hospital, Gurgaon, Haryana, India

in Rural Practice 2011;1:87-91.

**•** Cerebral amyloid angiopathy (CAA) is an important cause of spontaneous cortical-

**•** Leukoencephalopathy in conjunction with acute or chronic ICH in a cortical-subcortical

**•** Computed tomography is the imaging study of choice for evaluation of suspected acute

**•** MRI is best suited for identification of chronic cortical-subcortical hemorrhages, ischemic

**•** The burden of asymptomatic cerebral microhemorrhages detectable by GRE MRI in patients with CAA is a good predictor of hemorrhage recurrence, and therefore highlights the

, Sachchidanand Yadav2

1 Department of Radiodiagnosis, Employees' State Insurance Corporation (ESIC) Model

2 Department of Radiodiagnosis, PGIMER, Dr. Ram Manohar Lohia (RML) Hospital, India

3 Department of Radiodiagnosis, Jawahar Lal Nehru (JLN) Medical College, Bhagalpur, Bi‐

[1] Bano S, Yadav SN, Garga UC, Chaudhary V. Sporadic cerebral amyloid angiopathy: An important cause of cerebral hemorrhage in the elderly. Journal of Neurosciences

[2] Vinters HV. Cerebral amyloid angiopathy: a critical review. Stroke 1987;18:311-24.

[3] Chao CP, Kotsenas AL, Broderick DF. Cerebral amyloid angiopathy: CT and MR

, Umesh Chandra Garga2

and

subcortical intracranial hemorrhage in the normotensive elderly.

sequelae of the disease and assessment of disease progression.

importance of secondary prevention in CAA-related PICH.

\*Address all correspondence to: dr\_vikaschaudhary@yahoo.com

imaging findings. Radiographics 2006; 26:1517-31.

location increases the diagnostic specificity for CAA.


[18] Linn J, Herms J, Dichgans M, Brückmann H, Fesl G, Freilinger T, et al. Subarachnoid hemosiderosis and superficial cortical hemosiderosis in cerebral amyloid angiopathy. AJNR Am J Neuroradiol 2008;29:184-6.

**Chapter 5**

**Genetics of Sporadic Cerebral Amyloid Angiopathy**

Cerebral amyloid angiopathies (CAA) can be divided into sporadic and hereditary forms. This chapter is focused on the genetics of sporadic CAA, but will first consider hereditary forms in

Amyloid-β protein (Aβ), the commonest amyloid subunit implicated in sporadic forms of CAA, is also involved in certain hereditary forms. Several other proteins are also associated with rare familial diseases in which CAA is a characteristic morphological feature. [1] Missense mutations within or just outside the Aβ peptide coding region of the APP gene result in clinicopathological phenotypes of early onset Alzheimer's disease (AD) and are associated with a neuropathological phenotype which includes prominent CAA – for example hereditary cerebral haemorrhage with amyloidosis of Dutch type (HCHWA-D), or with Italian, Arctic, Iowa, Piedmont and Flemish mutations. Severe Aβ CAA has also been well documented in cases of familial AD due to mutations in the presenilin (PSEN1 and PSEN2) genes. Familial CAAs associated with other proteins include BRI2 gene-related dementias (familial British dementia and familial Danish dementia), cystatin C gene mutations in hereditary cerebral haemorrhage with amyloidosis of Icelandic type, TTR gene mutations in meningo-vascular amyloidosis, hereditary prion disease with premature stop codon mutations and mutated

Sporadic cerebral amyloid angiopathy is characterised by deposition of Aβ in leptomeningeal and cortical blood vessels. It has a prevalence in population-based autopsy studies of 20-40%

> © 2014 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Kristiina Rannikmäe and Cathie Sudlow

http://dx.doi.org/10.5772/58870

**1. Introduction**

**1.1. Hereditary CAA**

**1.2. Sporadic CAA**

brief.

Additional information is available at the end of the chapter

gelsolin gene in familial amyloidosis of Finnish type. [1]

