Giant aneurysm of the intracavernous carotid artery and bilateral carotid fibromuscular dysplasia.
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Biomedical subjects
Publications and source records attributed to O Combarros.
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INTRODUCTION AND OBJECTIVE: The objective of the study was to analyze the EEG tracings during the seizures of two monozygotic twins, shown on molecular analysis, diagnosed as having infantile epilepsy with absences. The recordings are particularly interesting since the genotypes of the patients are identical. This allowed comparison between the similarities and differences in discharges so that they could be better defined. RESULTS: The 3 Hz spike and wave complexes were the same as the classical descriptions in typical absences. No 'W' spike and wave complexes morphology, discharge fragmentation or light induction of the discharges were observed. There was some differences in the duration of absences, which were much shorter in one twin girl. The duration of the seizures was variable, and sometimes similar to that described as characteristic in other types of generalized idiopathic epilepsies with absences. It would seem therefore that the duration of the seizure is not a criterion for differentiation between the different types of generalized idiopathic epilepsies. CONCLUSION: Concordance, both clinical and of seizure EEG recordings in our patients confirmed that there is a specific electroclinical phenotype in infantile epilepsy with absences, although individual variations may also occur.
Although the lingual nerve and the chorda tympani are the components of the classic peripheral gustatory pathway, loss of taste in patients after surgery for trigeminal neuralgia supports for the existence of an accessory gustatory pathway through the trigeminal sensory root and the gasserian ganglion. Bell's palsy is the most common pathology of the peripheral gustatory pathway. The central gustatory pathway ascends from the solitary tract nucleus in the medulla up to the upper pons in the ipsilateral central tegmental tract, rather than in the medial lemniscus as proposed in the past. It is not possible to specify whether the central gustatory pathway decussates or not at the lower midbrain level. Interruption of the gustatory pathway in the brainstem usually occurs with stroke or demyelination. The thalamic gustatory relay is located in the most medial aspect of the ventroposteromedial nucleus, immediately adjacent to the somatosensory area for the oral cavity and fingers. Therefore, ageusia associated with the sensory cheiro-oral syndrome may occur with a thalamic lesion. The laterality of the gustatory representation in the thalamus remains unresolved. Studies on epileptic gustatory aura have demonstrated that the insula and the anteromedial temporal lobe are the primary and secondary gustatory cortex, respectively. Taste perception results in patients with corpus callosum section and strokes or tumors involving the insula support the hypothesis that there is a gustatory representation of both hemitongues in the left cerebral hemisphere, whereas only the right hemitongue is represented in the right hemisphere.
Malignant peripheral nerve sheath tumors (MPNST) are rare. We report a case of a MPNST of the proximal sciatic nerve in the thigh, unassociated with pre-existing type 1 neurofibromatosis or history of radiation therapy. A 71-year-old man had a 6 month history of constant, severe, burning pain affecting the sole of the left foot. One month after the onset, the pain radiated to the left calf, posterior aspect of the thigh and buttock, and distal leg weakness followed. Three months prior to admission, the patient developed a large and painful mass in the buttock, that occupied the entire left gluteal region on examination. There was severe weakness of ankle and toe dorsiflexion and plantarflexion, decreased sensation on the lateral and posterior aspect of the left leg as well as on the dorsal and plantar surfaces of the foot, and absent ankle jerk. EMG showed denervation and motor unit loss in the short head of biceps femoris and muscles supplied by tibial and peroneal nerves on the left side. Magnetic resonance imaging revealed a 10-cm enhancing mass of the left sciatic nerve from the upper thigh to the greater sciatic notch. In surgery, a large MPNST with a high Ki67 labeling index (> 60 %) was subtotally removed from the sciatic nerve, and adjuvant radiation therapy was administered. In the ensuing months the tumor invaded the entire pelvic region. A high sciatic malignant tumor can present with a rapidly growing buttock mass and unilateral, neuropathic foot pain imitating the tarsal tunnel syndrome.
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The aim of this prospective study was to determine the etiologic factors leading to stroke in a group of young adults. We studied 95 patients aged 50 years or under who were admitted with the diagnosis of stroke over a 2-year period. These patients underwent complete clinical and laboratory assessment for stroke. The etiology was established in 73 (76.8%) out of 95 cases. Arterial hypertension, embolism and atherosclerosis were found to be the most frequent causes in patients with ischemic stroke, whereas hypertension and aneurysm rupture were the most prevailing etiologies in patients with hemorrhagic stroke. Miscellaneous causes represented one fourth of all cases of the series. Coagulation abnormalities occurred in some patients, but in these cases there were also other well established causes. Paradoxical embolism was not observed. This study corroborates the importance of thorough diagnostic evaluation to establish an accurate diagnosis.