Amyloid-ß related angiopathy: a case report

A 68-year-old man with a history of progressive headache, intermittent periods of disorientation and slight personality change for several weeks presented with an acute onset of aphasic speech disturbances and a latent hemiparesis on the right side. His medical history was remarkable for hypertension, atrial fibrillation and clipping of an aneurysm of the anterior communicating artery three years previously. Upon admission, the speech disturbances and the hemiparesis appeared to be regressive. Magnetic resonance imaging demonstrated hyperintense subcortical signal-changes of the left frontal lobe in the T2-weighted images. Recent ischemia or hemorrhage could be excluded. The cerebral MR-angiogram was normal. Lumbar puncture revealed elevated protein content and a slight pleocytosis. The results of cerebrospinal fluid bacterial cultures, virology and cytology were negative. In the following days the patient's condition deteriorated dramatically. He developed frontal seizures, signs of frontal disinhibition and a clouding of consciousness to coma grade I. Correspondingly, magnetic resonance imaging controls displayed a progression of the fronto-parietal signal changes and new lesions bilaterally in the occipital lobe. Despite normal findings in a conventional angiography, the clinical diagnosis of granulomatous angiitis was made. An open brain biopsy verified this diagnosis showing a severe granulomatous meningo-encephalitis and vasculitis with extensive deposit of amyloid ß4. An immunosuppressive treatment with corticosteroids followed by cyclophosphamide was initiated. The patient responded well to the therapy and improved over the following weeks to the extent that only mild cognitive disabilities remained present.

Conclusion: Amyloid associated angiitis of the central nervous system is a rare disease one should have in mind when confronted with cerebral vasculitis because treatment in the early stage promises a good outcome. Brain biopsy should be performed as early as possible whenever granulomatous angiitis is suspected because a definite diagnosis can solely be based on this procedure.