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A small deep infarct revealing leukoencephalopathy, calcifications and cysts in an adult patient
  1. I Wargon1,
  2. M-C Lacour2,
  3. D Adams1,
  4. C Denier1
  1. 1
    APHP, Bicêtre Hospital, Department of Neurology, le Kremlin-Bicêtre, France
  2. 2
    APHP, Bicêtre Hospital, Department of Neuroradiology (M-C.L), le Kremlin-Bicêtre, France
  1. Dr Christian Denier, MD, PhD, Department of Neurology, Centre Hospitalier de Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin-Bicêtre Cedex, France; christian.denier{at}bct.aphp.fr

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Leukoencephalopathy with intracranial calcifications and cysts (LCC) is an uncommon entity; it was first described in 1996 in three unrelated children.1 Following this report, six other cases have been reported, including one adult-onset case.25 The clinical presentations of this cerebral disorder include progressive extrapyramidal, cerebellar, pyramidal signs, slowed cognitive performances and/or seizures.15 LCC is characterised by a striking triad of neuroimaging findings: asymmetric calcifications, diffuse abnormal white-matter signal and parenchymal brain cysts.15 Histopathological examination in LCC reveals “angiomatous-like rearrangements of the microvessels”, with proliferation of abnormal small vessels associated with Rosenthal fibres, gliosis and calcifications.1 The admitted primary pathological feature of LCC is cerebral angiomatous rearrangements involving the microvessels, whereas perivascular foci of calcifications, hyaline deposits and formation of Rosenthal fibres appear compatible with secondary changes.1 3 5 Extensive cerebral calcifications and leukoencephalopathy have been also reported in another rare disorder, “Coats plus”, which is characterised by additional features: intrauterine growth retardation, bilateral retinal telangiectasias and exudations (Coats disease), sparse hairs, dysplastic nails, …

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