Flemming Kelly D, Rinaldo Lorenzo, Lanzino Giuseppe
Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota.
J Neurosurg Case Lessons. 2025 Mar 24;9(12). doi: 10.3171/CASE24746.
The clinical and radiological evolution of large capillary telangiectasias is largely unknown.
A 30-year-old female presented with progressive ataxia and nystagmus. She was found to have a large, diffuse enhancing pontomedullary lesion with associated hypointensity on susceptibility-weighted imaging consistent with a large capillary telangiectasia. In addition, she had a second capillary telangiectasia in the right frontal lobe, hypertrophic olivary degeneration (HOD), and multiple susceptibility lesions throughout the brain. The patient's condition continued to deteriorate and she became nonambulatory over 8 years, with right-sided hemiparesis and hemiataxia, nystagmus, palatal myoclonus, and a left abducens palsy. Over the follow-up period, serial MR images showed increased cavitation of the lesion and significant brainstem atrophy.
Capillary telangiectasias are generally small, benign lesions composed of dilated capillaries. However, when large (≥ 1 cm), they can be symptomatic. The authors present the case of a diffuse capillary telangiectasia of the pons with HOD and a progressively declining course due to brainstem atrophy and enlarging cavitation of the lesion. The radiological evolution of an aggressive capillary telangiectasia has not been previously reported. https://thejns.org/doi/10.3171/CASE24746.
大毛细血管扩张症的临床和影像学演变情况在很大程度上尚不明确。
一名30岁女性出现进行性共济失调和眼球震颤。她被发现有一个大的、弥漫性强化的脑桥延髓病变，在磁敏感加权成像上伴有低信号，符合大毛细血管扩张症。此外，她在右侧额叶还有第二个毛细血管扩张症、肥大性橄榄体变性（HOD）以及全脑多处磁敏感病变。患者的病情持续恶化，8年中她无法行走，出现右侧偏瘫和偏身共济失调、眼球震颤、腭肌阵挛以及左侧展神经麻痹。在随访期间，系列磁共振图像显示病变空洞增加以及明显的脑干萎缩。
毛细血管扩张症通常是由扩张的毛细血管组成的小的良性病变。然而，当病变较大（≥1厘米）时，可能出现症状。作者报告了一例伴有HOD的脑桥弥漫性毛细血管扩张症病例，该病例因脑干萎缩和病变空洞扩大而病程逐渐恶化。此前尚未报道过侵袭性毛细血管扩张症的影像学演变情况。https://thejns.org/doi/10.3171/CASE24746 。
