Dados do Trabalho

Título

Amyloid-β–Related Angiitis: a case report

RESUMO

Case presentation: 76 years old female,  previously diagnosed with systemic arterial hypertension and diabetes mellitus 2, presented episodic falls, chronically progressing to cognitive impairment, due mainly to apraxia and  loss of motor functionality, without rigidity or other parkinsonian symptoms. After 2 months of worsening of symptoms, patient had new seizure, with tonic posture in four limbs, leading to seek medical care. On admission,cognitive assessment showed ideomotor apraxia, but, due to reduced level of consciouness, the exam was jeopardized.  the patient underwent a non-contrast CT scan, which demonstrated subcortical diffuse hipodensity. For further investigation, brain MRI was performed, revealing T2 and FLAIR hyperintensity of subcortical white matter and SWI several microbleeds in right parietal lobe. Besides, serology, biochemical exams and cerebrospinal fluid did not present any alterations. Diagnosis of amyloid angeitis was proposed.High dose  IV methylprednisolone was started, with significant imprevement of cognitive symptoms, however, after 4 days, the patient had a seizure, wth worsening of awareness. After two weeks of hospitalization, patient was discharged with mild to moderate improvement of cognitive function, but locomotion was disabled. 60-day re-evaluation at neurological clinic showed betterment of walking and cognitive impairment.. Discussion: Cerebral amyloid angeitis, a rare condition, given the evidence of 50 cases in the literature, linked to aging and resulting from the pathogenic mechanism of autoimmunity to β-amyloid, insoluble nature, which are deposited in cerebral vessels, causing a local inflammation with eventual fibrinoid necrosis and, later, cerebral infarction, being demonstrated on MRI a hyperintensity and cortical/subcortical microhemorrhages. Symptoms include altered mental status, focal neurological deficits and seizures which, in a complete analysis of the clinical case, may lead to a diagnosis of amyloid angeitis. Final comments: Amyloid angiitis is a difficult pathology to diagnose and staging due to its clinical presentation, which matches other etiologies, usually being a differential diagnosis. Finally, even in the face of published studies, it is essential to conduct further research on this subject, since there is little current research, besides the lack of probable diagnostic measures.

Palavras Chave

Amyloid beta; angiitis