Severe course of idiopathic intracranial hypertension

Abstract

Idiopathic intracranial pressure (IIH) is a syndrome, characterized by an increase intracranial pressure without an intracranial mass lesion or hydrocephalus and by a normal cerebrospinal composition.
We present a case of a girl, 10 years old, with an initial clinic of a headache, vomiting, followed by worsening of the vision to the extent of an amaurosis, ophthalmoplegia, peripheral facial and hypoglossal palsy, quadriparesis and right-sided hypoesthesia. The fundoscopy reviewed bilateral, atrophic and pale optic discs with unclear borders. The CT showed hypodense left frontal white matter lesions and hypodense lesion in the splenium of corpus callosum. MRI showed hyperintense on T2 and FLAIR and hypointense on T1 left frontal lesions and lesion of splenium of corpus callosum with the characteristics of ischemic.
The case was discussed as a severe course of an idiopathic intracranial pressure, initially misdiagnosed and improperly treated that lead to permanent loss of vision and secondary ischemic lesions which determine the focal neurologic deficit. The patient was treated with an appropriate antiedema therapy that lead to a complete recovery concerning the muscle strength, sensation, cranial nerves functions except the permanent optic nerve atrophy with complete vision loss.
Although IIH has a typical clinical picture and usually benign course, severe complications are also possible that can lead to a complete vision loss. Correct diagnosis and adequate therapy are of a prognostic significance for the disappearance of the symptoms and for the preservation of the vision.