RESUMO
BACKGROUND: Spinal stenosis (SS) is a manifestation associated with cardiac amyloidosis (CA). However, there is a lack of studies assessing the prevalence of CA among patients with SS. We aimed to address the prevalence of CA among patients with SS and YLH. METHODS: We performed a cross-sectional study of consecutive patients older than 65 years with SS and yellow ligament hypertrophy (YLH). All the patients were assessed with an electrocardiogram, echocardiogram and biohumoral evaluation. Patients with CA red flags was further studied with cardiac magnetic resonance and 99mTc-DPD scintigraphy. A cohort of patients with confirmed CA and SS was used to assess clinical features associated with CA. RESULTS: 105 patients (75.0 ± 6.6 years old; 45.7% males) with SS and YLH [5.5 [5-7] mm] were screened. Prevalence of red flags of CA was high and 58 patients presented clinical suspicion of CA. One patient (0.95%) was finally diagnosed of CA. Patients with confirmed CA presented a more expressive phenotype than the screened population. Patients with suspected CA had greater YLH than patients without suspicion of CA (6.4 ± 1.3 vs. 5.0 ± 0.8 mm; p < 0.001) and patients with confirmed CA presented greater YLH than the screening population (6.7 ± 1.8 vs. 5.7 ± 1.2 mm; p = 0.018). CONCLUSION: Despite red flags of CA are common among patients with SS, the prevalence of confirmed CA was low in our sample of screened patients.
RESUMO
Background: Lower cranial nerve schwannomas are rare and only 63 cases originating from the accessory nerve have been documented. Case Description: We report a 61-year-old man who presented with a 3-month history of dysmetria, ataxic gait, and frequent falls. Magnetic resonance imaging revealed a giant rim-enhancing cystic lesion at the right cerebellomedullary cistern, which markedly displaced the brainstem and caused a critical compression on surrounding structures and mild hydrocephalus. Even though the nature of this lesion was not clear, it received a radiological diagnosis of meningioma as first option. Surgery was performed through an extended far lateral retrosigmoid approach with C1 hemilaminectomy, with intraoperative neurophysiological monitoring. A near-total resection was achieved due to the adhesion of the lesion to the brainstem and to the cranial nerves VII, VIII, IX, X, XI, and XII. Intraoperatively, the tumor was found to arise from the accessory nerve. The histopathological analysis concluded with a final diagnosis of ancient schwannoma, a rare histological subtype characterized by degenerative changes, typical from long-standing tumors. Conclusion: Very few cases of intracranial ancient schwannomas have been described. To the best of our knowledge, this is the first report of this extremely rare histological variant arising from the intracisternal component of the XI nerve. The rarity of this disease at this location may lead to preoperative misdiagnosis.
