A Collet–Sicard syndrome due to internal carotid artery dissection associated with cerebral amyloid angiopathy–related inflammation

Abstract

Background:Cerebral amyloid angiopathy–related inflammation is a rare condition with approximately 100 reported cases. Its clinical manifestations are varied. We report here a novel presentation of this disease.Case presentation:A 61-year-old Caucasian man presented with rapidly progressive paralysis of the IX, X, XI and XII right cranial nerves associated with right central facial nerve palsy. Brain computed tomography angiography and cerebral catheter angiography found a focal fusiform enlargement of the distal cervical portion of the right internal carotid artery, related to a pseudo-aneurysm suggesting an evolution of a dissection and intra-cranial vessel dysplasia. Brain magnetic resonance imaging showed multiple asymmetrical subcortical regions of hyperintensity on T2 fluid-attenuated inversion recovery sequences. Punctiform cortical hyposignals on T2-weighted gradient echo magnetic resonance imaging sequences were mostly congruent with the white matter hyperintensities. There was a decreased cerebral perfusion at the frontal hyperintense fluid-attenuated inversion recovery region. Spectrometry identified a lactate–lipid peak. A brain biopsy showed intravascular amyloid deposits. Corticosteroid therapy was initiated, leading to a dramatic improvement of both clinical condition and magnetic resonance imaging brain lesions.Conclusion:This case report suggests that extra-cranial vasculitis and dysplasia can exceptionally be found in patients satisfying cerebral amyloid angiopathy–related inflammation criteria.

Association Between Autoimmune Diseases and Spontaneous Cervicocranial Arterial Dissection

“…However, immune-related inflammatory infiltration was absent in traumatic dissection (15,19). In some case reports, these arterial anomalies disappeared rapidly after immunosuppressive therapy, which also supports the link between autoimmunity and dissection (3,17,20).…”

“…Inflammatory markers were elevated in stroke patients with SCCAD and correlated with clinical prognosis (16). In addition, two reports also showed that inflammation in the arterial wall was associated with SCCAD and aspirin/steroids treatment resulted in dramatic improvement of both clinical condition and magnetic resonance imaging brain lesions (3,17). Failure to demonstrate a specific infection agent led to a hypothesis that the activation of specific immune-mediated mechanisms rather than a specific infective agent may be responsible for a local inflammatory alteration linked to SCCAD.…”

Cerebral amyloid angiopathy-related inflammation: current status and future implications

“…Some cases presented with involuntary movement, [ 35 , 36 ] while others had systemic diseases, [ 14 ] cerebral hernia caused by severe edema, [ 37 ] uveitis, [ 21 ] multiple malignancies, [ 14 , 15 , 38 ] extracranial vasculitis, or vascular dysplasia at baseline. [ 39 ] One patient with a history of Parkinson's disease (PD) was mistakenly thought to have developed the mental manifestation of PD when he presented with the symptoms of CAA-RI. [ 40 ] Whether the etiology of these comorbidities, such as autoimmunity, or their treatment, such as radiation therapy, [ 41 ] are related to CAA-RI requires further study.…”

Unraveling the Links between Chronic Inflammation, Autoimmunity, and Spontaneous Cervicocranial Arterial Dissection

“…In the context of non-traumatic CCAD, it is noteworthy that there is a dearth of pertinent in vivo study models available. However, some case reports show significant improvement in the clinical profiles of arterial dissection and MRI brain-related lesions in SCCAD patients with the combined autoimmune disease (Takayasu arteritis and SCCAD, cerebral amyloid angiopathy-related inflammation and SCCAD) following the use of high-dose hormones and/or anti-inflammatory drugs [37,38].…”

“…This may be due to the differing pathogenesis between these two disease entities. A history of antecedent infection is thought to be significantly associated with SCCAD development, with inflammation potentially playing a critical role [6,[30][31][32][33][38][39][40][41][42]. Interestingly, cervicocranial arterial dissection (CAD) is more likely to occur in colder months, regardless of geographic location, suggesting that transient seasonal factors such as infections may play a role in pathophysiology [43,44].…”