Downloaded from http://jnnp.bmj.com/ on June 27, 2015 - Published by group.bmj.com Short report 9. 10. 11. 12. Mygland A, Monstad P. Chronic polyneuropathies in Vest-Agder, Norway. Eur J Neurol 2001;8:157–65. Strasberg PM, Triggs-Raine BL, Warren IB, et al. Genotype–phenotype pitfalls in Gaucher disease. J Clin Lab Anal 1994;8:228–36. Giraldo P, Pocovi M, Pérez Calvo JI, et al. Report of the Spanish Gauchers disease Registry: Clinical and genetic characteristics. Haematologica 2000;85:792–9. Halperin A, Elstein D, Zimran A. Increased incidence of Parkinson disease among relatives of patients with Gaucher disease. Blood Cells Mol Dis 2006;36:426–8 13. 14. 15. Perkin GD. The likely impact of demographic changes on the incidence and prevalence of neurological disease: demography in the United Kindom. J Neurol Neurosurg Psychiatry 1997;63(Suppl 1):8–10. Mistry P, Germanin DP. Therapeutic goals in Gaucher disease. Rev Med Interne 2006;27(Suppl 1):S30–8. Connock M, Burls A, Frew E, et al. The clinical effectiveness and cost-effectiveness of enzyme replacement therapy for Gaucher’s disease: a systematic review. Health Technol Assess 2006;10:iii–iv, ix–136. Neurological picture Medulla compression caused by vertebral artery dolichoectasia A 75-year-old man was admitted with recently developed hiccough and a 1 year history of ataxia. He denied drug overuse, stroke, head trauma and a family history of ataxia. He had hypertension for 10 years. He showed left-sided ptosis, miosis, right-sided hypaesthesia and bilateral limb ataxia, which was more prominent on the left side. He lost balance and leaned to the left side while standing when putting his feet together. Brain MRI and CT angiography showed prominent left-sided vertebral artery dolichoectasia compressing the left side of the ventrolateral portion of the medulla to the right (fig 1). Since the patient had no evidence of gastrointestinal disease, diaphragm irritation or alcohol consumption, the hiccough was assumed to be the result of a lesion of the nucleus ambiguous, dorsal muscular nucleus of the vagus nerve or solitary tract nucleus. Ipsilateral Horner’s symptom and contralateral hypaesthesia are typical features of lateral medullar syndrome. The limb and truncal ataxia, more prominent ipsilaterally, was interpreted as a consequence of bilateral vestibular nucleus and cerebellar peduncle lesions, which were more profound ipsilaterally. In the elderly, hypertension seems to cause progressive vascular wall damage and subsequent dolichoectasia.1 2 Also, brain injury by vertebral artery dolichoectasia is usually attributed to direct compression of the brainstem or ischaemia caused by pressure on perforating branches of the vertebral artery.1 2 No parenchymal lesion of the medulla, and the insidiously developed symptoms and signs of our case suggest direct compression as a possible pathophysiology of the clinical findings rather than ischaemia. The patient was treated conservatively with antiplatelet therapy1 and is stable to date. In the case of chronic ataxia with combined medullar sings, medulla compression caused by vertebral artery dolichoectasia needs to be included in the differential diagnosis encompassing cerebellar atrophy, stroke or drug overuse. J H Roh, Y S Koo, S-H Jang, M H Park Department of Neurology, Korea University College of Medicine, Korea Correspondence to: Dr M H Park, Department of Neurology, Korea University Ansan Hospital, Gojan-1dong, Danwon-gu, Ansan-city, Gyeonggi-do (425–707), South Korea; parkmuno@yahoo.co.kr Competing interests: None. J Neurol Neurosurg Psychiatry 2008;79:222. doi:10.1136/jnnp.2007.123885 REFERENCES Figure 1 T2 weighted axial MR image (A), gadolinum enhanced MR angiography source image (B) and T1 weighted enhanced sagittal image (C) show impingement of the left side of the ventral medulla with secondary cistern enlargement caused by a dolichoectactic left-sided vertebral artery. CT angiography showing a posterior view of the intracranial vessels (D) reveals a prominently angulated, dilated and tortous vertebral artery. 222 1. 2. Savitz SI, Ronthal M, Caplan LR. Vertebral artery compression of the medulla. Arch Neurol 2006;63:234–41. Caplan LR. Dilatative arteriopathy (dolichoectasia): what is known and not known. Ann Neurol 2005;57:469–71. J Neurol Neurosurg Psychiatry February 2008 Vol 79 No 2 Downloaded from http://jnnp.bmj.com/ on June 27, 2015 - Published by group.bmj.com Medulla compression caused by vertebral artery dolichoectasia J H Roh, Y S Koo, S-H Jang and M H Park J Neurol Neurosurg Psychiatry 2008 79: 222 doi: 10.1136/jnnp.2007.123885 Updated information and services can be found at: http://jnnp.bmj.com/content/79/2/222 These include: References Email alerting service Topic Collections This article cites 2 articles, 0 of which you can access for free at: http://jnnp.bmj.com/content/79/2/222#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article. Articles on similar topics can be found in the following collections Radiology (1625) Radiology (diagnostics) (1225) Stroke (1356) Brain stem / cerebellum (632) Hypertension (353) Injury (453) Neurological injury (371) Ophthalmology (763) Trauma (454) Trauma CNS / PNS (371) Neuroimaging (359) Notes To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/