A case of multiple intracerebral hemorrhage due to early-onset cerebral amyloid angiopathy with Alzheimer`s disease neuropathological change three decades after childhood neurosurgery

Abstract

Background Cerebral amyloid angiopathy (CAA) is a disease in which Amyloid β (Aβ) is deposited in blood vessels and meninges in the brain. CAA typically occurs in the elderly but is also known to occur in younger patients with a history of childhood head trauma or dura graft following neurosurgical procedures. In some cases, pathological examination of brain biopsies has identified not only Aβ but also phosphorylated tau (p-tau) deposits. This is a pathological feature characteristic of Alzheimer`s disease (AD). There are few reports of cases in which AD has already developed based on cognitive decline symptoms and the presence of p-tau in cerebrospinal fluid (CSF). This is the first case of amyloid angiopathy after graft surgery in which the patient already had mild cognitive decline and pathological findings showed not only amyloid β accumulation but also abnormal tau accumulation. Case presentation The patient is a 39-year-old female who underwent neurosurgery for an arachnoid cyst in the right temporal lobe at the age of 2 years. The patient probably underwent dural transplantation, although this is unclear because no operative record remains. Severe headache, dizziness and right leg weakness developed abruptly. Neurological finding showed aprosexia and cognitive impairment of 21/30 on MMSE. The Aβ42/40 ratio had decreased in CSF. Brain MRI showed multiple cerebral hemorrhages. ¹²³I -iodoamphetamine single-photon emission computed tomography (IMP-SPECT) showed hypoperfusion dominantly in the bilateral temporal lobe and posterior cingulate gyrus. Considering of CAA, brain biopsy was performed. Pathological examination showed severe CAA in many leptomeningeal and cortical vessels. Neurofibrillary tangles, neuropil threads and tau-positive neurites around plaques were also present. Conclusion We reported early-onset CAA after neurosurgery in childhood. In addition to pathological findings characteristic of AD, cognitive impairment was also observed, suggesting that the patient may have developed AD. In pathological finding, p-tau deposition was observed in neurons. These finding suggested the possibility of progression to AD in this patient. We believe that this is an extremely rare case of early-onset CAA with cognitive decline, abnormalities of Aβ and tau in cerebrospinal fluid, AD pathology including deposition of p-tau in neurons, and characteristic findings on IMP-SPECT.