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Abnormalities of cerebral arteries are frequent in patients with late-onset Pompe disease.
- Source :
-
Journal of neurology [J Neurol] 2010 Oct; Vol. 257 (10), pp. 1730-3. Date of Electronic Publication: 2010 Jun 18. - Publication Year :
- 2010
-
Abstract
- 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.
- Subjects :
- Adult
Aged
Cerebral Arterial Diseases diagnosis
Cerebral Arteries diagnostic imaging
Female
Humans
Magnetic Resonance Angiography methods
Magnetic Resonance Imaging methods
Male
Middle Aged
Muscle, Smooth physiopathology
Radiography
Cerebral Arterial Diseases etiology
Cerebral Arteries pathology
Glycogen Storage Disease Type II complications
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1459
- Volume :
- 257
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 20559845
- Full Text :
- https://doi.org/10.1007/s00415-010-5618-0