Case reports

Cerebral amyloid angiopathy and spontaneous intra- cerebral haemorrhage

Report of a sporadic case in a young Chinese

Maria P. Wang*, Thomas H.K. Ng*, and Kwan-Hon Ghan* *.

Introduction

Cerebral amyloid angiopathy (CAA), also re- ferred to as congophilic angiopathy, is a special form of microvascular amyloidosis affecting ce- rebral vessels mainly in the leptomeninges and superficial cerebral cortex of the elderly’.*. In- volvement of the systemic vasculature is excep- tiona13,4. Since the description of the condition early this century5,6, it has been found in fre- quent association with Alzheimer’s disease (AD), senile dementia of Alzheimer type (SDAT) and other neurodegenerative disor- ders’,7. Other recognised presentations include cerebrovascular haemorrhage*x9 or cerebral mi- croinfarcts of spontaneous or hereditory nature. This report describes a young Chinese adult in whom intracerebral haemorrhage is attributable to CAA independent of any associated family history or features of neurological disorders.

Case Summary

A 49-year-old Chinese male was admitted into hospital because of acute onset of headache, vomiting and clouding of consciousness. There was no history of head trauma. Past health was unremarkable. In particular, there was no hy- pertension, neurological or mental symptoms. There was no family history of dementia, mental

* Department of Pathology, University of Hong Kong.

Summary

A 49-year-old Chinese male with spontaneous intracerebral haemorrhage due to cerebral amyloid angiopathy is presented. This is the first case reported in the Chinese population, and the youngest patient described without a family history or associated mental disorder.

Key words: Cerebral amyloid angiopathy, con- gophilic angiopathy, intracerebral haemor- rhage, Chinese

disorder, intracranial haemorrhage, stroke or foreign ancestry.

Examination Physical examination revealed a semiconscious man with right hemiparesis. Examination of other organ systems was unremarkable. A com- puterised tomography scan of the brain showed lobar intracerebral haemorrhage of the left occi- pitoparietal region extending into the left lateral ventricle (Fig. 1). No other focal lesion was observed.

Operation The patient underwent a left craniotomy, which showed subdural and subarachnoid extension

* * Division of Surgical Neurology, Department of Surgery, University of Hong Kong.

Address for correspondence and reprint requests: Dr. M. P. Wong, Department of Pathology, University of Hong Kong, Queen Mary Hospital. Pokfulam Road, Hong Kong.

Accepted 3-8-89

Clin Neural Neurosurg 1991. Vol. 93-2

133

Fig. 1. CT scan of brain showing lobar haemorrhage over left occipitoparietal area with intraventricular extension.

Fig. 2. Photomicrograph showing thickened leptomenin- geal vessels by amorphous material (Haematoxylin & eosin x 200).

Fig. 3. Photomicrograph showing apple green birefringence of leptomeningeal vessels (Congo red x 100, under polarized light).

from the intracerebral haematoma. The evac- uated blood clot and biopsies from the wall of the haematoma were submitted for pathological examination,

Pathological Study The submitted material consisted of small pieces of cortical brain tissue and about 1Oc.c. of bloot clot. Histologically, a few leptomeningeal arter- ies and small cortical vessels showed thickening of the wall by amorphous eosinophilic material (Fig. 2) which gave a salmon red colour with congo red stain. Apple green birefringence was observed under polarized light (Fig. 3). There was no fibrinoid necrosis. microaneurysm, or senile plaque.

Electron microsopic examination of the blood vessels showed a haphazard arrangement of non-branching non-parallel fibrils with a mean diameter of approximately 9nm (Fig. 4) charac- teristic of amyloidosis. The fibrils replaced smooth muscle cells in the media and produced variable separation of the internal elastic lamina and the external basement membrane (Fig. 5). The overall features were those of cerebral amy- loid angiopathy.

Discussion

Cerebral amyloid angiopathy (CAA) was ini- tially described as one form of cerebral amyloi- dosis early in this century5v6. Despite numerous cases reported2Tr0, including one of a Japanese patient , l1 CAA as a specific pathological finding

Fig. 4. Electron-micrograph showing non-branching, non- parallel deposits of amyloid fibrils in wall of blood vessel (x 50,ooo).

Fig. 5. Electron-micrograph showing the amyloid fibriis re- placing the smooth muscle cells in the media (x 2,OOU).

in the Chinese populating has never been de- scribed. We believe the present case is the first Chinese patient reported in the English litera- ture.

Amyloidosis can be detected by a number of histochemical, immunological and electron mi- croscopic methods. The tinctorial characteris- tics of congophilia, and the demonstration of apple green birefringence under polarised light remains one of the simplest and most reliable methods of amyloid detectioni.‘2. In this case, the diagnosis of CAA is further confirmed by the ultrastructural demonstration of character- istic amyloid fibrils in the vessel wall. CAA most commonly involves the leptomeningeal and su- perficial cortical small vessels of the occipital and parietal grey matterI”. The finding in this patient also conforms to this characteristic topo- graphic distribution. Moreover, the absence of features of systemic amyloidosis elsewhere in the body, and the rarity of simultaneous in- volvement of cerebral vessels in systemic amy- loidosis support our belief that this patient suf- fers from primary cerebrovascular amyloidosis.

This unique case also describes the youngest patient to be affected by sporadic CAA. Most other reported cases portray elderly patients above sixty years oId2.“‘, whereby the incidence mounts with increasing age. All other young patients presenting in the second or third dec- ades of life either suffer from unexplained or hereditary neuropsychiatric disorders’.‘” such as

Down’s syndrome, or belong to families of Ice- 1andiP or Dutch16 ancestry where well-defined familial forms of CAA occur.

Although CAA is commonly associated with the clinical and pathological features of Alz- heimer’s disease and senile dementia of Alz- heimer type7.‘2, recently accumulated evidence has revealed that spontaneous intracerebral haemorrhage is an important independent man- ifestation of the condition8.9. The diagnosis in these patients would easily be missed if material obtained during clot evacuation was not careful- ly studied. In accordance with our present case, other authors have stressed the importance of making the correct diagnosis by histological ex- amination of the submitted blood clot”~“, as this might warn of recurrent multifocal haemorrhag- eS9.r3

A case of sporadic CAA presenting as intrace- rebral haemorrhage in a young Chinese is re- ported. The importance of histological exam- ination of craniotomy material obtained from intracerebral haemorrhage of unknown cause is stressed. It is hoped that with improved aware- ness of the condition and advances in newer biochemical and molecular biology techniques, the relation between normal aging, dementia and amyloid angiopathy can be revealed.

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