Gordon-Firing S, Purriel J A, Pereyra D, Brodbek I
Instituto de Neurología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay.
Acta Neurol Latinoam. 1981;27(1-2):99-111.
The authors present a 47 years old female patient with cutaneomeningospinal angiomatosis or Cobb's syndrome of thoracic location. This entity, included in the primarily mesodermic phacomatose, is extremely rare, and only 17 cases have been sufficiently documented in the literature on the subject. The rare association with vertebral haemangioma of the same metameric level was present in this patient. The diagnostic criteria, possible associations, pathophysiology and nosological situation are discussed. The telangiectasic type of angioma and the principally extradural location are stressed. The authors concluded on the importance of clinical suspicion of this diagnosis based on a spinal cord syndrome in the presence of a cutaneous angioma of the same metameric level. The necessity of a complete paraclinical studies which includes selective spinal angiography and linear tomography of the spine are also stressed. With this methodology the real incidence of this syndrome will be revealed, permitting a planned surgical treatment which will diminish the high mortality and morbidity of the cases published up to the present.
