Ein Fall Neuroangiomatose bei Neurofibromatose Typ I

Abstract

We present the case of a patient suffering from Neurofibromatosis type I (NF-1) with acute, very painful neuropathy of the right lower extremity. The preoperative electro neuro- physiological study showed an impaired function of the peroneal nerve. The MRI revealed an extended diffuse plexiform tumour of the sciatic nerve and at thigh level. Biopsies showed marked diffuse angiomatosis within the sciatic nerve.To our knowledge, this is the first description of an intraneural vascular malformation in NF-1.Treatment of such an entity is a challenge and must be individually defined.