Granuloma eosinófilo da coluna cervical com manifestação neurológica, liquorica e radiológica atípica

Abstract

A case of eosinophilic granuloma of the cervical spine in a ten year-old white boy is reported. The patient complained of posterior cervical pain at the level of C5-C6 and right hemiparesis that progressed to mixed tetraparesis (lower motor neuron in upper limbs and upper motor neuron in lower limbs). Examination of the cerebrospinal fluid (CSF) disclosed an inflammatory reaction; radiologic studies of the cervical spine showed an irregular aspect with demineralization at the base of spinous process of C6. The patient was treatet intermitently with corticosteriods (prednisone); there was an improvement of neurologic symptoms and the examination of the CSF was normal within a few days, each time he took the drug. Neurologic symptons and abnormalities of the CSF recurred after discontinuation of corticotherapy. Examination of a fragment of the spinous process and neighboring tissues of C6, taken by biopsy, disclosed eosinophilic granuloma. Radiotherapy was then indicated, plus corticosteroids. There was remission of all neurologic symptoms and signs. The unusual features of the case are discussed in relation to others reported in the literature.

Granuloma eosinofílico de coluna cervical: relato de caso

“…Histiocitose X é um grupo de doenças de origem desconhecida, caracterizado por proliferação anormal de histiócitos, que inclui a doença de LettererSiwe, Hand-Schuller-Christian e o granuloma eosinofílico (GE) 1,6,8 . O GE seria a forma mais benigna das histiocitose X, acometendo principalmente crianças e adolescentes 6,8,9 .…”

“…Granuloma eosinofílico é doença descrita como condição benigna de proliferação histiocitária localizada ou multifocal, acometendo, principalmente, os ossos do crânio, costelas, mandíbulas, fêmur e coluna vertebral, sendo mais frequente em crianças e adolescentes [1][2][3][4][5] . Representa 50-60% de um grupo de enfermidades denominadas histiocitose X, cujo denominador comum é o desenvolvimento de lesões granulomatosas em diversos tecidos 6,7 . Estas enfermidades seriam diferentes expressões do mesmo processo patológico de origem ainda desconhecida 3,6,8 .…”

“…Representa 50-60% de um grupo de enfermidades denominadas histiocitose X, cujo denominador comum é o desenvolvimento de lesões granulomatosas em diversos tecidos 6,7 . Estas enfermidades seriam diferentes expressões do mesmo processo patológico de origem ainda desconhecida 3,6,8 . O presente relato tem como finalidade lembrar que uma criança com torcicolo persistente e achados de lesão óssea em coluna cervical, sem história prévia de trauma, pode apresentar granuloma eosinofílico.…”

Cervical spine cord compression by eosinophilic granuloma: case report

“…Such pathological entities have in common a histopathological characteristic pattern of histiocytary nature (Langerhans cells) associated with eosinophils [1][2][3][4][5][6][7][8][9][10] .…”

“…In adults the localization in the cervical region, associated to symptoms of spinal cord compression or myelorradiculopathy, is still rarer 9,12 . A review of the recent literature referable to these condition, related to the cervical localization disease, there were compiled only five cases with characteristics signs of spinal cord compression 3,4,8,12,13 . We are reporting an additional case of eosinophilic granuloma, the sixth to add to the literature, issuing a displacement of the spinal cervical cord and compression of the roots, whose diagnosis was established after the complete investigation and also by the lesion histopathology.…”