Neurology, Lyell McEwin Hospital, Adelaide, South Australia, Australia
Medicine, Lyell McEwin Hospital, Adelaide, South Australia, Australia.
BMJ Case Rep. 2021 Nov 19;14(11):e244440. doi: 10.1136/bcr-2021-244440.
We discuss an extremely rare case of low-grade Schwann cell leptomeningeal neoplasm with no evident intradural primary, presenting with rapid neurological decline leading to death reflecting the aggressive biological behaviour of this entity despite its low-grade morphology. Notwithstanding extensive investigations, the diagnosis was only established on autopsy as clinical presentation is non-specific making diagnosis challenging. This condition could be considered in patients presenting with leptomeningeal disease if initial workup of more common causes is non-revealing.
我们讨论了一例极为罕见的低级神经鞘瘤性脑脊膜神经瘤病例，无明显的颅内原发性病变，表现为迅速的神经功能下降导致死亡，反映了该实体的侵袭性生物学行为，尽管其形态为低级。尽管进行了广泛的调查，但仅在尸检时才确诊，因为临床表现不特异，诊断具有挑战性。如果最初对更常见原因的检查结果不明确，这种情况可考虑用于出现脑脊膜疾病的患者。
