A case of leukoencephalopathy, cerebral calcifications and cysts.

Annals of Indian Academy of Neurology
Ajay GulatiN Khandelwal

Abstract

Triad of leukoencephalopathy, cerebral calcifications and cysts (LCC) is a recently reported rare disease named 'Labrune syndrome' after the first case was reported in 1996 by Labrune et al. Herein, we report a case of a 36-year-old man with mild right-sided weakness and seizures for 5 years. CT of brain revealed extensive calcification involving bilateral basal ganglia, right thalamus and bilateral deep cerebellar nuclei. A supratentorial cystic lesion with blood fluid level was seen in left occipitotemporal region. MRI examination revealed diffuse symmetric white matter hyperintensity suggesting leukoencephalopathy. On follow-up, patient reported improvement in the weakness and no further seizure episodes. However, follow-up of MRI revealed persistence of lesions. Differential diagnosis considered were parasitic infections (hydatid, cysticercosis), Coat's plus disease and causes of diffuse cerebral calcification like Fahr's disease and post-radiotherapy/chemotherapy. Serology for parasitic infections was negative. No history of radiotherapy or chemotherapy in the past could be elicited in the history. Another close differential is Coat's plus disease which can mimic LCC pathologically.

Citations

Oct 1, 2014·Pediatric Neurology·Richard L OglesMark D Travis
Jul 19, 2014·Journal of Neurology·Kinga KarlingerAnna Szűcs
Oct 25, 2016·Journal of the Neurological Sciences·Miao WangShengyuan Yu
Jul 21, 2017·Medicine·Yubao MaShengyuan Yu
Nov 22, 2020·Brain Sciences·Magdalena Machnikowska-SokołowskaRafał Płoski
May 22, 2021·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Suzanne MurphyKristian Aquilina
Sep 6, 2021·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Eliseo PicchiFrancesca Di Giuliano
Sep 12, 2021·Neurology India·Souvik DubeyBiman Kanti Ray

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