Neurología. 2010; 25(6) :391-397 0213-4853X/ $ - see front matter © 2009 Sociedad Española de Neurología. Published by Elsevier España, S.L. All rights reserved. NEUROLOGÍA Volumen 25 • Número 1 • Enero-Febrero 2010 Publicación Ofcial de la Sociedad Española de Neurología 25 años NEUROLOGÍA www.elsevier.es/ neurologia LETTERS TO THE EDITOR Posterior leukoencephalopathy syndrome associated with amyloid angiopathy Síndrome de leucoencefalopatía posterior en relación con angiopatía amiloide Dear Editor: In 1996, Hinchey et al1 described the reversible posterior leukoencephalopathy syndrome in relation to arterial hypertension, renal disorders and immunosuppression. Since then, numerous entities have been added as potential triggers. Connective pathologies, haematological diseases, drugs, angiography, blood transfusions, haemodialysis or acute intermittent porphyria are some of examples.2 Recently, there have been some reported cases involving amyloid angiopathy.3-7 We wish to add the case of a patient with this syndrome, who also showed the same underlying disease. This was a hypertensive 73-year-old woman, who had undergone a kidney transplant due to polycystosis, and who was treated with prednisone, tacrolimus, mycophenolate mofetil and atenolol. The baseline study showed no alterations in cognitive function and a normal neurological status. On two occasions, before transplantation, in a state of advanced renal failure, she had presented some episodes of reversible language alterations in the context of elevated blood pressure. Three years after transplantation, she presented for 18 months sudden, recurrent episodes of incapacity for speech and language comprehension, which remitted in about a week. These were not accompanied by headaches or visual alterations; she maintained a good level of consciousness, and the episodes were not associated with strikingly elevated blood pressure or severe renal function alterations. The clinical manifestations were similar in all episodes. We conducted a complete analysis, autoimmunity studies, cerebrospinal fl uid, vascular studies (supra-aortic trunks duplex, transcranial Doppler, echocardiography, ECG, Holter, cerebral angiography), electroencephalogram (EEG), magnetic resonance imaging (MRI), brain SPECT and brain biopsy. The arteriography revealed an ulcerated plaque in the left internal carotid artery (symptomatic), which did not condition haemodynamic alterations. The cranial MRI, in addition to parenchymal atrophy, revealed hyperintensity in the parieto-occipital region on FLAIR and T2-weighted sequences, predominantly on the left side, with a moderate enhancement after contrast administration, and a normal study with dissemination techniques ( fi g. 1). On the EEG and video- EEG, we observed a spike-wave focus in the left hemisphere and some electric crises with no clinical correlation. Other tests performed before the biopsy did not reveal any signi fi cant fi ndings. The patient was admitted to the hospital repeatedly during each of these episodes, and subsequent therapeutic measures were taken due to their recurrence. Firstly, she received antiplatelet therapy, initially with aspirin and then with clopidogrel. Upon the later discovery of the ulcerated carotid plaque, which was not considered a subsidiary of surgery, anticoagulation was established, as well as treatment with statins. Given the the EEG fi ndings and the lack of response to these therapies, empirical anticonvulsant treatment was prescribed, fi r st with phenytoin and then with lamotrigine later on. Everolimus was also given as a replacement for tacrolimus, 8 despite not having found toxic levels of the drug. However, the patient still had recurrent language alterations (up to six episodes), and during the last two, showed residual aphasia. To establish a diagnosis and rule out infectious, neoplastic and infl ammatory processes, a brain biopsy was performed; this revealed amyloid in the leptomeningeal vessels ( fi g. 2). A mild gliosis in the white matter was observed in the brain parenchyma, as well as scarce diffuse cortical plaques, with an absence of neuritic plaques and neurofi brillary tangles. We detected no infl ammatory changes or tumour proliferation. In the case presented, the clinical signs and symptoms initially pointed to vascular episodes of a stroke-like nature due to its acute onset. Despite this, CT and MRI studies never showed parenchymal necrosis, but only non-speci fi c signal changes, hyperintense on FLAIR and T2 sequences. Tacrolimus was replaced because of known potential side effects of this drug, such as encephalopathy and language disorders. 9,10 The patient, despite the residual language impairment, did not present memory defi cits or other additional cognitive impairments. Amyloid angiopathy is a vascular disease, common in the elderly, which often manifests as lobar haemorrhages. However, sometimes it may do so only as leukoencephalopathy, along with a Binswanger-type periventricular distribution, more associated with atrophy, or located in the U- fi bres, at an immediately subcortical level. In this case, it is usually associated with oedema and has a more reversible nature. On the other hand, the deposition of amyloid material tends to be more intense in occipital lobes. 11 The most widely accepted pathophysiological explanation for posterior