Intracranial hemangioma associated with facial nevus

Abstract

Summary The author is of the opinion that in the majority of cases of facial nevi associated with intracranial hemangioma the motor and psychic phenomena observed are secondary to the intracranial hemangioma and that they are due to the same factors which will produce motor and psychic phenomena in instances in which other intracranial tumors can be demonstrated or other factors which will produce destruction or faulty function of brain cells. He is also of the opinion that in many cases, but not in the majority of cases, a concomitant primary mental deficiency may exist which will add to the severity of the objective mental responses. The rather high percentage of cases in which primary mental deficiency may coexist is probably due to the fact that developmental anomalies are more frequent among primary aments than among those with a normal mentality. The author offers in support of the above statements the following well-established clinical facts: (1) Any intracranial tumor or tumor-like process is capable of producing convulsive phenomena, frequently Jacksonian in type. In the cases reported in the literature of the syndrome under discussion most cases began with Jacksonian attacks and later became general or remained Jacksonian. (2) Jacksonian epilepsy is not the usual type of convulsive phenomena associated with a germ plasm defect responsible for convulsive disorders. (3) Any convulsive disorder persisting over a period of years is capable of producing mental deterioration. In the cases reported in the literature in which mental changes were noted the average age of the patient at time of death was greater than of those with normal intelligence. (4) Mental retardation as a result of primary amentia is exhibited early in life. It is conceded that Yakovlev and Guthrie were correct in classifying the phenomena of a facial nevus associated with an intracranial hemangioma with two other syndromes which present neurocutaneous manifestations and in grouping the three under the term “congenital ectodermatoses”. But we do not believe that in the first mentioned of the three syndromes the concomitant mental changes or epilepsy are embryogenetically associated, but are the result of destruction of anatomic and physiologic units within the brain. This is important from a prognostic standpoint since cases cured by operation or by radium, and by a combination of the two, have been reported.