Dados do Trabalho

Título

Rapidly progressive dementia in a patient with non-hemorrhagic Cerebral Amyloid Angiopathy: a case report

RESUMO

CASE REPORT
A 84 year-old male patient with a history of follicular low grade non-hodgkin's lymphoma treated, evolving for two months with loss of self-care, significant difficulty in walking, total dependence for daily life activities associated with cognitive impairment, especially recent memory. He denied urinary incontinence. Infectious, paraneoplastic, immune-mediated processes were ruled out.
MRI of the brain showed diffuse encephalic volumetric reduction, without lobar predominance. Hyperintense lesions on T2/Flair in the white matter of the cerebral hemispheres, without expansive character and a tendency to confluence in a periventricular situation, suggestive of moderate degree microangiopathy, as well as enlarged perivascular spaces, mainly affecting the basal ganglia, this finding being a marker of small vessel disease.
For diagnostic clarification a brain biopsy was performed with characteristic findings of amyloid angiopathy with an inflammatory pattern. Treatment was performed with methylprednisolone 1g daily for 3 days with significant improvement in the patient's quality of life

DISCUSSION
Cerebral amyloid angiopathy (CAA) is characterized by acellular thickening of the walls of small and medium arteries due to accumulation of amyloid. CAA is often asymptomatic but usually manifests as dementia and vascular syndromes, and there is an important association between CAA and spontaneous intracerebral hemorrhage. In this sense, we present a case of non-hemorrhagic CAA, with an inflammatory pattern, presenting as rapidly progressive dementia.
Histologically analyzed lesions can be of amyloidoma, CAA with primary angiitis of the central nervous system (SPAC) and, as in this case, they consisted of isolated CAA. Its pathogenesis is not very clear, but amyloid deposition is accompanied by inflammation or edema, in contrast to the usual presentation of patients with CAA, involving acute intracerebral hemorrhage.

FINAL COMMENTS
The literature emphasizes the diagnosis of CAA and its association with hemorrhages and dementia, however rare cases of non-hemorrhagic brain lesions are associated with CAA, as exposed above.
Little information is available regarding their treatment. Non-hemorrhagic AAC has shown a satisfactory response to corticosteroid treatment in patients with evidence of inflammation, as we also evidenced in our case.
Thus, although rare, it should be considered in the differential diagnosis of lesions of the central nervous system.

Palavras Chave

Inflammatory cerebral amyloid angiopathy; rapidly progressive dementia;