Abnormalities of cerebral arteries are frequent in patients with late-onset Pompe disease.

Cerebral aneurysms and arteropathies causing severe cerebrovascular events have been reported as rare complications in patients with late-onset Pompe disease. We investigated the frequency of cerebrovascular anomalies in six patients with late-onset Pompe disease followed at our institution. Clinical data collection and magnetic resonance angiography were performed as part of routine annual examinations. Four out of six patients had brain vascular anomalies including dolichoectasia of the basilar artery and ectasia of internal carotids. These patients also complained of gastrointestinal symptoms (chronic constipation and gastrointestinal reflux). Two patients had clinical signs related to the arteriopathy, including partial paralysis of the third cranial nerve and transient ischemic attacks. At 1 year follow-up, enzyme replacement therapy did not modify the size of cerebral vessels, but patients reported a marked improvement of intestinal symptoms. In conclusion, neurologists should be aware that intracranial artery abnormalities are not infrequent in patients with late-onset Pompe disease, and they should be specifically investigated in the presence of unexplained CNS symptoms.

[The coracoid block: demonstration of a simple approach using the pectoralis minor as landmark]. / Le bloc coracoïdien : mise en évidence d'une voie d'abord simple utilisant le petit pectoral.

OBJECTIVE: Despite their recognized advantages, infraclavicular blocks, especially with the pericoracoid approach, are underutilized because the lack of simple anatomical landmarks. The goal of this study is to determine a simple, reliable and reproducible reference point by means of magnetic resonance imaging (MRI), using the anterior extremity of the coracoid process, and the pectoralis minor as a tactile reference point. METHOD: Sagittal and para-sagittal MRI sections at the coracoid process were performed on 11 patients. The relationships between the coracoid process, the nerve-vessel bundle, the pectoralis minor and the skin were measured. RESULTS: The optimal puncture point for a needle introduced strictly in an anterio-posterior direction in a supine, alert patient is located 2 cm within and 2.5 cm below the coracoid process. The injection point, defined as the distance between the skin and the tip of the needle lying in the centre of the visualized nerve-vessel bundle, is located an average of 5 cm from the skin (mean 5.02 cm, with a standard deviation of 1.03 and a confidence interval of 0.91). This injection point goes from 0.4 cm (at the most anterior part of the bundle) and 1.75 cm (at the most posterior part), with an average 1 cm from the posterior surface of the pectoralis minor (tactile reference point). Neither body mass index nor sex influence significantly the variables measured. CONCLUSIONS: The reference points defined here could allow easy and reproducible performance of this relatively simple block and make it more effective.