Revista de neurologia · 1998
[Lhermitte-Duclos disease in a ten-year-old child: clinical follow-up and neuroimaging data from birth].
Клинический случайАннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Verdú A, Garde T, Madero S
Аннотация
INTRODUCTION: Lhermitte-Duclos disease is a rare lesion characterized by enlarged cerebellar folia containing abnormal ganglion cells. It typically affects young adults, and reports in the pediatric age are exceptional.
CLINICAL CASE: This case report describes a child with slowly progressive macrocephaly, which was already present at birth, delayed motor development, and clumsiness. At 7 years of age a mild ataxia appeared, which remained stationary until 10 years of age when he developed signs of intracranial pressure (headache and papilloedema). A CT scan at 2 years of age showed an enlarged posterior fossa with asymmetrical cerebellum; another study at 7 years of age disclosed mild ventricular dilatation. IRM at 10 years of age showed aqueductal obliteration by an enlarged cerebellum. The cerebellar lesion consisted of abnormally thickened folia with abnormal hyperintensity of the central cerebellum on the T2-weighted images. The lesion did not enhance with gadolinium. The histological findings were typical of Lhermitte-Duclos disease.
CONCLUSIONS: In our patient the lesion behaved like a slowly growing mass, but both neuroimaging and pathological studies were suggestive of a hamartomatous rather than a true neoplastic disorder. Furthermore, there was clinical evidence that the disorder was already present at birth. All the evidences in our case support the concept that this growing lesion represents a congenital abnormality.
CLINICAL CASE: This case report describes a child with slowly progressive macrocephaly, which was already present at birth, delayed motor development, and clumsiness. At 7 years of age a mild ataxia appeared, which remained stationary until 10 years of age when he developed signs of intracranial pressure (headache and papilloedema). A CT scan at 2 years of age showed an enlarged posterior fossa with asymmetrical cerebellum; another study at 7 years of age disclosed mild ventricular dilatation. IRM at 10 years of age showed aqueductal obliteration by an enlarged cerebellum. The cerebellar lesion consisted of abnormally thickened folia with abnormal hyperintensity of the central cerebellum on the T2-weighted images. The lesion did not enhance with gadolinium. The histological findings were typical of Lhermitte-Duclos disease.
CONCLUSIONS: In our patient the lesion behaved like a slowly growing mass, but both neuroimaging and pathological studies were suggestive of a hamartomatous rather than a true neoplastic disorder. Furthermore, there was clinical evidence that the disorder was already present at birth. All the evidences in our case support the concept that this growing lesion represents a congenital abnormality.