Nineteen patients with juvenile nasopharyngeal angiofibroma (JNA) were surgically treated with different techniques from January 1968 through December 1985. Two patients had undergone a previous operation at another hospital; all patients were males (mean age 15.4), and the most common symptom was nasal obstruction (84.2%). Lateral extension into the pterygomaxillary fossa occurred in 14 patients (73.6%), and 2 also had intracranial invasion (10.5%). In five cases, the tumor's cytosol was analyzed for hormonal receptors. Negative values for estrogen and progesterone receptors were obtained, although the content of dehydrotestosterone receptors was highly positive. These results tend to support the hypothesis of JNA's androgen-dependence. The authors emphasize the need of a preoperative staging classification based on clinicoradiological data in selecting the most adequate surgical approach. Tumors with lateral extension into the pterygomaxillary fossa can be easily removed through a midface degloving; large involvement of the infratemporal fossa requires, also, a transzygomatic dissection. In JNAs with intracranial extension a combined intracranial-extracranial approach is advisable.

Brain atrophy and WM tract abnormalities in frontal-parietal circuits can be detected at least a decade before the estimated symptom onset in asymptomatic mutation carriers.

No abstract

The findings of the present study can be summed up in the following points: (1) brainstem auditory evoked potentials (BAEP), as compared with magnetic resonance imaging (MRI), has a greater capacity and a lower cost in disclosing brainstem plaques both in MS patients with symptoms or signs of actual brainstem involvement and in clinically silent ones. This makes BAEP a useful technique for the neurologist, who can confirm the clinical suspicion of a brainstem lesion and follow the evolution of the disease in the patient. (2) The sensitivity of BAEP is lower than that of MRI as far as the anterior lesions of the brainstem are concerned. (3) MRI is more specific than BAEP, inasmuch as several types of injuries can alter the BAEP, while the demyelinating plaque has a specific image and can only be confused with little lacunar infarcts. (4) Plaques that produced symptoms or signs in the past can eventually disappear and be no longer detected by a subsequent MRI.

We report two cases of lower cranial nerve palsies (XII in case 1, IX-X-XII in case 2) associated with abnormalities of the internal carotid artery at the base of the skull. In case 1 a limited dissection of the carotid wall produced both paresis of the hypoglossal nerve and Horners syndrome by compression of the nerve trunk against the base of the skull and stretching of the periarterial sympathetic fibres respectively. In case 2 we speculate that a narrow angled kinking of the internal carotid artery may have damaged cranial nerves IX, X and XII by interfering with the blood supply to the nerve trunks. In both cases the outcome was favorable with almost complete regression of the initial symptoms. We conclude that the association between lower cranial nerve disturbances and internal carotid artery abnormalities is probably more common than was thought. We suggest that the pathogenesis of the damage to the cranial nerves may differ from one case to the next.