Neurological development over a period of 25 years and MRI findings are reported in two members of the same family affected by mannosidosis type II. Progressive axial and appendicular cerebellar syndrome, moderate hearing loss and deterioration of gait were present in both patients. Neuropsychological deficiency was severe, but progression over the years was not observed except in the woman's speech capacity. Neither of the patients showed clinical improvement. A progressive corticosubcortical atrophy stands out in the brain neuroimaging studies, especially at the vermian cerebellar level. The osseous cranial deformities are very characteristic and include brachycephaly, thickening of the calvaria at the expense of the diploe, and poor pneumatization of the sphenoid. Neither of our cases showed an empty sella turcica.
The purpose of this prospective study is to show a technique for recording electroencephalographic activity via an endovascular approach in presurgical evaluation of epileptic patients. Technical aspects and insertion strategy are outlined. Advantages of intra-arterial electroencephalography have been demonstrated. It is a semi-invasive procedure that provides information in temporal lobe and extratemporal epilepsy. It allows a dynamic electroencephalographic recording and patient tolerance is excellent. Risks are practically absent. Disadvantages are: in comparison to ovale electrodes, chronic and multicontact recording is not possible and the recording is only intercritical.
Bronchial artery embolization (BAE) is the treatment of choice for massive hemoptysis with rare complications that generally are mild and transient. There are few references in the medical literature with acute cerebral embolization as a complication of BAE. We report a case of intracranial posterior territory infarctions as a complication BAE in a patient with hemoptysis due to bronchiectasis.
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