Clinical aspects of cerebral amyloid angiopathy.

[hereditary cerebral hemorrhage with amyloidosis]

Amyloid depositions mainly consist of proteins with a fibrillary structure . A large number of different proteins have amyloidogenic properties . Amyloids are now categorized on the basis of their chemical structure , but the clinical classification of localized and systemic amyloid is still useful . The proteins that can be found in cerebral amyloid angiopathy are cystatin C , beta /A 4 and transthyretin . Cerebral amyloid angiopathy can remain symptom-free , but can also give a broad spectrum of clinical and radiological manifestations , including (vascular ) dementia , cerebellar and cerebral hemorrhage , subarachnoid hemorrhage , and leukoencephalopathy . It is debated whether amyloid angiopathy plays a causative role in Alzheimer 's disease , but it is strongly correlated with the presence of cerebral plaques . In this review , the clinical spectrum of cerebral amyloid angiopathy will be described , based on retrospective studies from the literature . Hereditary cerebral hemorrhage with amyloidosis (Dutch ) will be presented as a 'prospective ' model to study the clinical effects of amyloid angiopathy .

Diseases
Validation

Diseases presenting "large number" symptom

acute rheumatic fever

adrenal incidentaloma

allergic bronchopulmonary aspergillosis

canavan disease

coats disease

cowden syndrome

dedifferentiated liposarcoma

dracunculiasis

epidermolysis bullosa simplex

fabry disease

familial mediterranean fever

gm1 gangliosidosis

heparin-induced thrombocytopenia

hereditary cerebral hemorrhage with amyloidosis

hirschsprung disease

kindler syndrome

legionellosis

malignant atrophic papulosis

neuralgic amyotrophy

phenylketonuria

pleomorphic liposarcoma

primary effusion lymphoma

scrub typhus

severe combined immunodeficiency

triple a syndrome

waldenström macroglobulinemia

well-differentiated liposarcoma

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

zellweger syndrome

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