Ese reportate un caso de syndrome de Ehlers-Danlos associate con . presence de mastocytes dans les tumeurs cutanees de la maladie de Recklinghausen. We report a case of von Recklinghausen's disease complicated by a pineal region tumor . nerveux au cours de la maladie de Recklinghausen: A propos d'une. Manifestations Art Rielles de La Maladie de Von Recklinghausen. Manal Benlahbib, Abbes El Mesnaoui, Younes Bensa D. Manifestations Art.


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In our patient multiple intracranial tumors were associated with only one small subcutaneous neurilemoma and three intrathoracic neurilemomas.

Nodules de Lisch dans la maladie de Von Recklinghausen

Neurilemoma of the peripheral nerves was not reported in any of these 49 cases. Several reports of different types of intracranial and peripheral nerve tumors in various combinations have been published sincebut we could not find any case presenting with the combination of maladie de recklinghausen seen in our maladie de recklinghausen.

Of the six tumors found on the CT scan of the head, only the two intraventricular meningiomas and maladie de recklinghausen suprasellar meningioma were identified on the skull x-rays. All of these were calcified. The right convexity meningioma, even though calcified on the CT scan, was not visible on the skull x-rays.

Ehlers-Danlos Syndrome and Multiple Neurofibromatosis

The two acoustic tumors were not maladie de recklinghausen on the plain x-rays but complex motion tomograms of the petrous bones did show widening of the internal auditory meatus on both sides, consistent with bilateral acoustic tumors. Cystic degeneration is known to occur in both meningiomas and neurilemomas.

In the presence of intracranial lesions, the skin lesions of neurofibromatosis have often been few and inconspicuous,1 as they were in this patient. Although this condition is considered to be an inherited neuroectodermal abnormality determined by a dominant gene, in nearly 50 percent of the cases it seems to develop by mutation.

The hearing and visual losses in this patient were most probably due to the bilateral acoustic tumors and the suprasellar meningioma.

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  • Introduction

The rate of progression of this disease is usually very slow,2 and malignant change occurs infrequently. With modern neurosurgical techniques it is possible to remove acoustic tumors and conserve hearing, maladie de recklinghausen the prognosis for conservation of hearing decreases with increasing size maladie de recklinghausen the tumor.

Including our case, we identified 28 cases of intracranial aneurysms in the literature [Table - 1]. Predominantly, the aneurysms were located in the ICA circulation.

In three cases, the aneurysms was in the vertebrobasilar circulation, and eight patients had multiple aneurysms. Intracranial aneurysms in NF-1 might occur as a result of the radiation therapy of gliomas of the optic chiasm.


At least the intimal aneurysm form was typical for cerebral aneurysms and had marked, eccentric, fibrous intimal proliferation, a small number of sparsely distributed spindle cells in the intima and media, smooth muscle fibrosis, and elastica fragmentation. Elle permet d' ameliorer la qualite de la vie et de restaurer 1' autonomie des malades.

La sensibilisation contre la scarification eviterait la surinfection. Traditional scarification, superinfected royal tumors and surgery in the Von Recklinghausen disease: It is rare and an ancient practice; surgical resection does not maladie de recklinghausen the risk of malignant degeneration.

Beside the margin of the 3rd ventricle there is diffuse infiltration of the pre-optic nuclei. Where the glioma gains the meningeal surface it forms a focus of fibrillary thickening, but there is no extensive spread in the meninges. Thalamic tumour, showing cells with astrocytic nuclei and short glial processes.

Edge of thalamic tumour, above and to the right, reaching medial aspect of internal capsule on the left. The mid-brain at the level of the decussation of brachium conjunctivum is diffusely swollen and the aqueduct is narrow, distorted and shifted towards the right. Throughout the section at this level there is infiltration by proliferating glial cells; only a portion of the left cerebral peduncle is spared.