Cochlear Implantation in Brown-Vialetto-Van-Laere syndrome.

OBJECTIVE: To report outcomes for the first known cochlear implantation procedures in two patients with Brown-Vialetto-Van-Laere syndrome. PATIENTS: Two adult patients (a brother and sister) with post-lingual sensorineural deafness associated with Brown-Vialetto-Van-Laere syndrome. The female patient presented with a milder form of the syndrome. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURE: Post-implantation speech discrimination scores. RESULTS: Auditory evoked potential testing suggested pathological changes in both patients' cochleae, auditory nerves, brainstem and (probably) central auditory pathways. In the male patient, despite implantation of the better ear, the Bamford-Kowal-Bench sentence score was zero at 21 months post-implantation. In the female patient, Bamford-Kowal-Bench sentence scores at six months post-implantation were 25 per cent in quiet and 3 per cent in noise. CONCLUSION: These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.

Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.

This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. Biopsy sampling should be considered when technically feasible.

Anterior corpus callosotomy: effects in a patient with congenital bilateral perisylvian syndrome and oromotor seizures.

Anterior corpus callosotomy was performed in a patient with pseudobulbar palsy, mild mental retardation and intractable epilepsy related to congenital bilateral perisylvian cortical dysplasia. Before surgery, she had daily atonic drop attacks, rare and mainly sleep-related oromotor seizures, and multifocal and diffuse paroxysmal EEG discharges; after callosotomy, less abrupt atonic drop attacks recurred monthly and the EEG epileptiform abnormalities disappeared. Video-EEG sleep recordings revealed the clinically unsuspected serial recurrence of oromotor seizures, probably related to the clinically observed aggravation of dysarthria. New surgical techniques, in addition or alternative to callosotomy, should be developed in order to avoid or reduce the risk of aggravating some types of partial seizures in patients with bilateral cortical displastic lesions, intractable epilepsy and epileptic falls.

An arachnoid cyst extending above and below the foramen magnum.

A case of progressive bulbar and long tract symptoms with hydrocephalus of eleven years duration in a 23-year-old man, in whom an arachnoid cyst of the cisterna magna cerebellomedullaris was disclosed at operation.

Long-term follow-up of traumatic unilateral superior oblique palsy.

Twenty-four cases of traumatic unilateral superior oblique palsy were followed-up during a mean period of 5.3 years. Hypertropia in the primary position and a positive Bielschowsky test were present in 100% of the cases, compensating head posture in 71%, and excyclotorsion in 25%. Six patients needed surgical correction; 18 were cured spontaneously during a mean period of 9.2 months.

[Thymectomy in myasthenia gravis pseudoparalytica]. / Thymektomie bei Myasthenia gravis pseudoparalytica.

From 1967 to 1982, 109 thymectomies were performed mainly in patients with moderate severity of the disease. The age of patients averaged 30,3 years. Operative lethality amounted to 2.7%. Another 4 patients died of the basic disease 6 months after the operation. During the subsequent course 70% of the patients showed a longlasting improvement after thymectomy, with complete recovery occurring in 23% and a significant amelioration in 40% of thymectomized patients. In another 32% there was a slight improvement of complaints. The best results could be observed in myasthenic patients with thymic hyperplasia whereas only a slight remission occurred after removing thymomas. 5 to 10 years after thymectomy the improvement rates remained approximately at the same level. Consequently, thymectomy can be regarded as a successful therapy in myasthenia gravis pseudoparalytica.