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

Leucoenfalopatia, calcificações cerebrais e cistos: entidade que pode simular uma neoplasia

LETTERS

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

Leucoenfalopatia, calcificações cerebrais e cistos: entidade que pode simular uma neoplasia

Melina Moré Bertotti^I; Marcelo Neves Linhares^I; Rafael Ferreira^II; Daniel Sousa Santos^I; Athos Tiradentes Athayde Jr^I; Antonio Cesar Melo Mussi^I; Jorge W Moritz^I; Thiago Siminiano Jung^I; Humberto Kluge Schroeder^I; Igor Kunze Rodrigues^I

^IServiço de Neurocirurgia, Hospital Governador Celso Ramos, Florianópolis SC, Brazil

^IIServiço de Radiologia, Clínica Imagem, Florianópolis SC, Brazil

^{Correspondence}

Leukoencephalopathy with intracranial calcifications and cysts (LCC) is a rare and recently described entity characterized radiologically by white matter abnormalities, calcifications, cysts and enhancing nodules¹. 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 onset². We report a case of LCC with remarkable radiological and clinical features.

CASE

A 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.

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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.

DISCUSSION

This is the first report of MR perfusion findings in a case of LCC. The enhancing walls presented a striking high capillary density that could mislead for an erroneously diagnosis of neoplastic lesion. In fact, this finding may be explained for one of the theories of cerebral microangiopathy as the etiology of this entity. This theory could explain the hemorrhagic content of the lesions.Furthermore high vascular lesions may present high perfusion as well as brain cancer.

The symmetrical calcifications and the diffuse enhancing nodular lesions are other important findings that suggest a diffuse cerebral condition. 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^1-5.

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^1-3 .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 adolescence⁴. Wargon et al. report a 30-year-old woman with a lacunar infarct as the first manifestation of LCC⁵. In our patient, the diagnosis of LCC was based on the highly suggestive neuroradiological findings which were almost identical to previous report . Our biopsy showed no neoplastic cells, but clotted blood within the lesion.

LCC is an entity can present with cystic expansive lesions with mass effect, hemorrhagic content and high vascular density that can be misunderstood as neoplastic lesions. Symmetrical brain calcifications and diffuse enhancing hemorrhagic nodules are the other crucial findings. These abnormalities points out that this enity should rather be descrided as a vascular entity in the books than as a primary leucodistrophy.

Conflicts of interest

The authors report no conflict of interest

Received 12 March 2011

Accepted 30 March 2011

• 1. Labrune P, Lacroix C, Goutieres F, et al. Extensive brain calcifications, leukodystrophy, and formation of parenchymal cysts: a new progressive disorder due to diffuse cerebral microangiopathy. Neurology 1996;46: 1297-1301.
• 2. Kaffengerger T, Valko PO, von Meyenburg J, et al. A case of late onset leukoencephalopathy with cerebral calcifications and cysts in 59-year-old woman. Eur J Neurol 2009;16:278-281.
• 3. Marelli C, Savoriardo M, Fini N, et al. Late presentation of leucoencephalopathy with calcification and cysts: report of two cases. J Neurol Neurosurg Physchiatry 2008;79:1303-1304.
• 4. Sener U, Zorlu Y, Men S, Bayol U, Zanapalioglu U .Leukoencephalopathy, cerebral calcifications and cysts. Amer J Neuroradiol 2006;27:200-203.
• 5. Wargon I, Lacour MC, Adams D, Denier C. A small deep infarct revealing leukencephalopathy, calcifications and cysts in an adult patient. J Neurol Neurosurg Psychiatry 2008;79:224-225.

Publication Dates

• Publication in this collection
  01 Sept 2011
• Date of issue
  Aug 2011