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NEUROIMAGES
Year : 2019  |  Volume : 67  |  Issue : 3  |  Page : 934-935

Labrune syndrome: A rare cause of reversible hemiparesis


Department of Neuro-Imaging and Interventional Neuro-Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India

Date of Web Publication23-Jul-2019

Correspondence Address:
Dr. Anuj Prabhakar
Department of Neuro-Imaging and Interventional Neuro-Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.263194

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How to cite this article:
Gupta MM, Prabhakar A, Joseph L, Garg A, Gaikwad S. Labrune syndrome: A rare cause of reversible hemiparesis. Neurol India 2019;67:934-5

How to cite this URL:
Gupta MM, Prabhakar A, Joseph L, Garg A, Gaikwad S. Labrune syndrome: A rare cause of reversible hemiparesis. Neurol India [serial online] 2019 [cited 2019 Aug 19];67:934-5. Available from: http://www.neurologyindia.com/text.asp?2019/67/3/934/263194




An 18-year old girl presented with an 8-month history of progressive left upper and lower limb weakness, seizures and facial pain. General physical and visual examinations including visual fields were unremarkable.

Non-contrast computed tomography (NCCT) of the brain showed a large cystic lesion in the right basal ganglia region (arrow, [Figure 1]b) causing mass effect (arrow head, [Figure 1]b). Diffuse confluent deep white matter hypodensities were seen in the fronto-parietal region (arrow, [Figure 1]a) with relative sparing of the juxtacortical white matter. Multiple foci of coarse calcifications in bilateral caudate nuclei heads, right ganglio-capsular region, left lentiform nucleus (arrow, [Figure 1]c, bilateral dentate nuclei (arrow, [Figure 1]d) and deep parieto-occipital white matter were noted. MRI brain-T2W image revealed multiple hyperintense cystic lesions in the supratentorial region, largest in right basal ganglia (arrow, [Figure 2]a) and in the right frontal and left parietal white matter (arrow head, [Figure 2]a), hypointense on T1W [Figure 2]b, partially suppressed on fluid attenuated inversion recovery (FLAIR) images [Figure 2]c and with no diffusion restriction on diffusion weighted images [DWI] [Figure 2]d. Mild smooth peripheral enhancement of the cyst wall on T1W coronal post-contrast image (white arrow, [Figure 2]f) was seen. Diffuse white matter hyperintensities were noted in bilateral fronto-parietal region (arrow, [Figure 2]c and [Figure 2]e).
Figure 1: Non contrast computed tomography brain axial images (a-d): Large cystic lesion in the right basal ganglia region (white arrow, b) causing mass effect over the ipsilateral lateral ventricle and midline shift (arrow head, b). Diffuse confluent deep white matter hypodensity in the fronto-parietal region (white arrow, a) with relative sparing of juxtacortical white matter are seen. Multiple foci of coarse calcifications in bilateral caudate nuclei heads, right ganglio-capsular region, left lentiform nucleus (white arrow, c), bilateral dentate nuclei (white arrow, d) and deep parieto-occipital white matter are also visualised.[1,3]

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Figure 2: MRI brain (a-f): T2W image reveals multiple hyperintense cystic lesions in the supratentorial region, the largest being in the right basal ganglia (white arrow, a) and in the right frontal and left parietal white matter (arrow head, a). These cystic lesions are hypointense on T1W image (b), partially suppressed on FLAIR image (c) and show no restriction on DWI (d). Mild smooth peripheral enhancement of the cyst wall is noted on T1W coronal post-contrast image (white arrow, f). Diffuse, confluent, symmetric white matter hyperintensities are seen in bilateral fronto-parietal regions (white arrow, c and e)[3],[4]

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The triad of leukoencephalopathy, cerebral calcifications and cysts (LCC) was termed the Labrune syndrome in 1996.[1] The clinical presentation includes a cognitive decline, seizures, as well as pyramidal, extrapyramidal and cerebellar signs.[2],[3] The entity may be mistaken for a parasitic or a metabolic disease and following delaying of the treatment. The treatment of choice is cyst resection to reduce the mass effect.[4] These imaging findings are characteristic of Labrune syndrome. This is a rare cause of reversible hemiparesis that is treatable by surgery. Surgical removal of a large cyst can reverse the neurological deficit by reducing the mass effect.

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  References Top

1.
Labrune P, Lacroix C, Goutières F, de Laveaucoupet J, Chevalier P, Zerah M, et al. Extensive brain calcifications, leukodystrophy, and formation of parenchymal cysts: A new progressive disorder due to diffuse cerebral microangiopathy. Neurology 1996;46:1297-301.  Back to cited text no. 1
    
2.
Jenkinson EM, Rodero MP, Kasher PR, Uggenti C, Oojageer A, Goosey LC, et al. Mutations in SNORD118 cause the cerebral microangiopathy leukoencephalopathy with calcifications and cysts. Nature Genet 2016;1185-92.  Back to cited text no. 2
    
3.
Sener U, Zorlu Y, Men S, Bayol U, Zanapalioglu U. Leukoencephalopathy, cerebral calcifications and cysts. AJNR Am J Neuroradiol 2006;27:200-3.  Back to cited text no. 3
    
4.
Nagae-Poetscher LM, Bibat G, Philippart M, Rosemberg S, Fatemi A, Lacerda MT, et al. Leukoencephalopathy, cerebral calcifications, and cysts: New observations. Neurology 2004;62:1206-9.  Back to cited text no. 4
    


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