Late presentation of leucoencephalopathy with calcifications and cysts: report of two cases

Marelli, Cécilia; Savoiardo, M.; Fini, N.; Bartolomei, Ilaria; Marliani, Anna Federica; Gonda, F De; Agostinis, Cristina; Albini-Riccioli, Luca; Marucci, Gianluca; Giaccone, Giorgio; Chiapparini, Luisa; Salvi, Fabrizio; Pareyson, Davide

doi:10.1136/jnnp.2008.148429

Cited by 24 publications

(20 citation statements)

References 5 publications

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“…LCC is a very rare disease and less than 30 cases reported since the original description by Labrune and colleagues, all of them with characteristically infantile or juvenile onset [1][2][3] .Sener et al were the first to suggest the existence of an adult form of this disease, based on the observation of a slow clinical progression in a patient with onset in late adolescence 4 . Wargon et al report a 30-year-old woman with a lacunar infarct as the first manifestation of LCC 5 .…”

Section: Lettersmentioning

confidence: 99%

“…They differ from brain AVMs in that they lack a true nidus. They differ from dural AVFs in that they derive their arterial supply from pial or cortical arteries and are not located within the dura mater 3 . They may be localized anywhere in the brain but display a preference for the supratentorial regions 3 .…”

Section: Lettersmentioning

confidence: 99%

“…Actually the nodular enhancing lesions constitute pre-cystic lesions as same cases show in the literature. Actually the leucodistrophy described in this entity is rather related to vasogenic edema and gliosis surrounding the cysts and the nodules [1][2][3][4][5] .…”

Section: Lettersmentioning

confidence: 99%

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Leukoencephalopathy, cerebral calcifications, and cysts: entity that can mimic a neoplasm

Bertotti

Linhares

Ferreira³

et al. 2011

Arq. Neuro-Psiquiatr.

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Leukoencephalopathy with intracranial calcifications and cysts (LCC) is a rare and recently described entity characterized radiologically by white matter abnormalities, calcifications, cysts and enhancing nodules 1 . LCC is a disorder of unknown etiology and its neurological manifestations include cognitive decline, seizure, obstructive hydrocephalus, progressive cerebellar, besides extrapyramidal and pyramidal signs with juvenile onset 2 . We report a case of LCC with remarkable radiological and clinical features. CASEA 30-year-old man presenting frontooccipital headache for the last 7 days, worsening in the morning, associated with progressive anorexia and vomiting.Eight years ago, he had an acute neurological event characterized by paresis and aphasia of expression.His past medical history demonstrated moderate cognitive impairment since childhood.Family history, were unremarkable and no consanguinity was known. Neurological examination revealed left paresis grade 4+ with positive Babinski sign besides ataxia. Brain MRI (1.5T) revealed an expansive solid / cystic interhemispheric lesion measuring 55×25 mm with important mass effect. T2 imaging showed remarkable hemossiderin deposition in its walls. MR perfusion demonstrated high capilar density within the walls. Multiple enhancing nodular lesions were observed envolving supra and infratentorial brain parenchyma. Some of these nodules had hemorrhagic component on T2 images. Broad and symmetric calcifications in periventricular white matter, basal ganglia, brainstem and dentate nuclei were observed.Patient underwent surgery for relieving intracranial hypertension and biopsy. Pathological examination was inconclusive due to the large amount of clotted blood within the material. No neoplastic cells were observed. Patient was discharged in good clinical condition, without signs of intracranial hypertension, remaining hemiparesis and aphasia.

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Section: Lettersmentioning

confidence: 99%

Section: Lettersmentioning

confidence: 99%

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Leukoencephalopathy, cerebral calcifications, and cysts: entity that can mimic a neoplasm

Bertotti

Linhares

Ferreira³

et al. 2011

Arq. Neuro-Psiquiatr.

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“…8-10,12,13,15-17 cerebellum, 4,5,7,[11][12][13][14]16 brainstem, 2,12,15 lat eral ventricles, 1,4 basal ganglia, and thalmus. 4,7,12,13,15 The clinical symptoms of this entity include seizures, cognitive decline, and a combination of pyramidal, extrapyramidal, and cerebellar signs, with symptoms primarily related to the progression of leukoencephalopathy, cerebral calcifications, and cyst formation.…”

Section: 3-5mentioning

confidence: 99%

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] Although there are no effective preventive or medical treatments for LCC, surgery has therapeutic value in patients with clinical symptoms due to cyst expansion. 1,2,4,7,9,11,13,15,16 In the present report, we describe the case of a patient with LCC who underwent surgery for repeated cyst formation. The procedures included open surgical removal, stereotactic aspiration, and stereotactic aspiration with Ommaya reservoir placement.…”

Section: 3-5mentioning

confidence: 99%

Repeated cyst formation in a patient with leukoencephalopathy, cerebral calcifications, and cysts: effectiveness of stereotactic aspiration with Ommaya reservoir placement

Ooba

Abé²,

Hisamitsu³

et al. 2013

PED

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The combination of leukoencephalopathy, cerebral calcifications, and cysts (LCC) constitutes a rare cerebral disorder characterized by distinctive neuroradiological and clinical findings. Twenty-eight cases of LCC have been reported. Surgery is usually required to treat progressively expanding cysts, but surgical strategies have been varied. The authors present the case of a patient who underwent 4 surgical procedures for repeated cyst formation. The first operation was performed for the removal of a cyst and to make a histopathological diagnosis. The second and third operations were performed to treat de novo cysts. The fourth operation was performed to treat a recurrence. This is the first reported case of LCC in which it was necessary to perform surgery 4 times, in large part due to de novo cyst formation. It provides evidence that multiple cysts may develop in these patients and that several surgical operations may be needed to treat LCC. Stereotactic aspiration with Ommaya reservoir placement is an appropriate procedure for this condition because it is less invasive and more repeatable than open surgery.

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Contribution of QSM Imaging to the Diagnosis of the Rare Syndrome of Leukoencephalopathy with Cysts and Calcification (LCC)

2017

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Late presentation of leucoencephalopathy with calcifications and cysts: report of two cases

Cited by 24 publications

References 5 publications

Leukoencephalopathy, cerebral calcifications, and cysts: entity that can mimic a neoplasm

Leukoencephalopathy, cerebral calcifications, and cysts: entity that can mimic a neoplasm

Repeated cyst formation in a patient with leukoencephalopathy, cerebral calcifications, and cysts: effectiveness of stereotactic aspiration with Ommaya reservoir placement

Contribution of QSM Imaging to the Diagnosis of the Rare Syndrome of Leukoencephalopathy with Cysts and Calcification (LCC)

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