Mathis Stéphane, Dupuis-Girod Sophie, Plauchu Henri, Giroud Maurice, Barroso Bruno, Ly Kim Heang, Ingrand Pierre, Gilbert Brigitte, Godenèche Gaëlle, Neau Jean-Philippe
Department of Neurology, CHU Poitiers, Université de Poitiers, 2 rue de la Milétrie, 86021 Poitiers, France.
Clin Neurol Neurosurg. 2012 Apr;114(3):235-40. doi: 10.1016/j.clineuro.2011.10.036. Epub 2011 Nov 16.
Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder that can lead to neurological manifestations including strokes and cerebral abscesses. Our objectives were to describe clinical, radiological, bacteriological, and outcome characteristics of patients with cerebral abscess and HHT, and to concurrently compare this group with a control group with cerebral abscess, but without HHT.
Patients with HHT and cerebral abscess in 5 French medical centers were included. Their clinical, radiological, biological data and prognosis were compared to the data of unselected patients with cerebral abscesses but without HHT included during the same period of time.
Twenty-six patients (13 men and 13 women; 44.7±17.2; range 12-79 years), with HHT and cerebral abscess were included. A pulmonary arteriovenous malformation (AVM) was present in all cases. Cerebral abscesses were solitary, supratentorial, and mostly lobar. In all cases, pathogens were anaerobic or facultative anaerobic germs (particularly streptococcus). No death was observed, but various sequels were present in up to two-thirds of the patients. We observed a recurrence of the cerebral abscess in 4 patients with a mean delay of 81 months. In comparison with the control group, cerebral abscesses were generally of later recurrence and significantly more often unique and less often due to staphylococcus.
HHT cerebral abscesses are particularly linked to pulmonary arteriovenous malformations and anaerobic germs. Their clinical, radiological and bacteriological characteristics are quite different than in a control group with more solitary brain localizations, no staphylococcus infection and a significantly longer interval to recurrence.
遗传性出血性毛细血管扩张症（HHT）是一种罕见的常染色体显性疾病，可导致包括中风和脑脓肿在内的神经学表现。我们的目的是描述脑脓肿合并HHT患者的临床、放射学、细菌学及预后特征，并同时将该组患者与脑脓肿但无HHT的对照组进行比较。
纳入法国5个医学中心的HHT合并脑脓肿患者。将他们的临床、放射学、生物学数据及预后与同期纳入的未选择的脑脓肿但无HHT患者的数据进行比较。
纳入26例（13例男性和13例女性；年龄44.7±17.2岁，范围12 - 79岁）HHT合并脑脓肿患者。所有病例均存在肺动静脉畸形（AVM）。脑脓肿为单发、幕上，且大多位于脑叶。所有病例中，病原体均为厌氧菌或兼性厌氧菌（尤其是链球菌）。未观察到死亡病例，但多达三分之二的患者出现各种后遗症。我们观察到4例患者脑脓肿复发，平均间隔时间为81个月。与对照组相比，脑脓肿通常复发较晚，且明显更常为单发，由葡萄球菌引起的情况较少见。
HHT相关性脑脓肿尤其与肺动静脉畸形和厌氧菌有关。其临床、放射学和细菌学特征与对照组有很大不同，脑定位更单一，无葡萄球菌感染，复发间隔时间明显更长。
