Craniopharyngiomas: our experience in Lyon

Objective We reviewed our experience in surgical treatment of craniopharyngiomas. Surgical treatment of craniopharyngiomas in children represents a challenge for neurosurgeons because it presents a different set of surgical problems. Results are controversial and debates concerning strategies to ameliorate the rate of success and to decrease the rate of morbidity and mortality are ongoing. Post-surgical quality of life is related to the anatomical relationship between the hypothalamic region, vascular structures and optic pathways.Discussion From 1985 to 2004, 60 children (age range, 4 months to 18 years) were treated in our institution. Since 1987, all patients were studied with pre- and postoperative MRI. Two groups of patients were distinguished: a group of 36 patients treated with surgical direct surgery; a second group of 24 patients treated only with intracystic chemotherapy with bleomycin (18 patients) or associated with surgery (six patients). In the first group, the removal of lesion was total in 74% of cases. Two patients died in the early postoperative period and two more died later (early mortality of 5% and late mortality of 11%). All patients presented ante-pituitary insufficiency and diabetes insipidus, which required substitutive treatment. Twenty-two patients presented with visual problems, including amaurosis in two cases. In the group treated with bleomycin, 18 patients presented a primary cystic or a mixed form of craniopharyngioma and six patients showed a cystic recurrence. Eighteen patients were treated with bleomycin only. The dose used varied from 30 mg to a maximal dose of 150 mg, with a middle dose of 60 mg in the large majority of cases. In this group, the cyst disappeared in 12 patients and reduced to 30% of its initial volume, and stabilization of the lesion was achieved in the other six patients. Only 11 patients presented partial endocrine insufficiency. Of the 18 patients, 16 were of school age, 14 of whom were following a normal educational program and two an assisted program. No mortality was reported.Conclusion Our experience showed that in the group treated with intracystic chemotherapy alone, results were better with a low rate of morbidity and mortality. Endocrinological, visual and neuropsychological evaluations were also correlated with better outcome. In cases of cystic craniopharyngiomas, we considered bleomycin as the treatment of choice. For solid forms or for cases resistant to intracystic chemotherapy with bleomycin, direct surgery has to be proposed.     

Efficacy of zonisamide: our experience

The current overview of zonisamide use and effectiveness is based on both a long-term prospective postmarketing survey and current zonisamide use at the Saitama Medical College, Department of Neuropsychiatry. Survey data, which were collected from individual physicians and 23 survey groups throughout Japan, assessed the effectiveness of zonisamide in 1631 patients. Zonisamide was highly effective for treating partial seizures, with 70% of patients reporting improvement. More than half of patients with generalized seizures (58%) and half of patients with myoclonic and atypical absence seizures showed improvement with zonisamide treatment. Among the different epileptic syndromes, zonisamide was highly effective in treating generalized idiopathic epilepsy (≥78% improvement) and partial epilepsy (≥58% improvement). However, only 28% of patients with West syndrome or Lennox-Gastaut syndrome showed improvement. Among 60 outpatients treated with zonisamide at our facility as of October 1998, most had partial seizures or generalized seizures subsequent to partial seizures. The majority of patients received zonisamide in combination with other antiepilepsy drugs. Patients receiving zonisamide monotherapy showed greater improvement than did patients receiving polytherapy. We conclude that zonisamide is highly effective for partial seizures and generalized seizures, and that there appears to be no decrease in efficacy with long-term use.     

Opsoclonus-polymyoclonia syndrome suppressed with reserpine

A 38-year-old man was admitted to Iwakuni National Hospital on July 6, 1978, with the complaints of difficulty seeing and walking. Two weeks before admission, he first experienced dizziness and it slowly progressed to uncontrollable tremor-like movements of the whole body. On admission, he was alert, oriented and afebrile. He had not experienced nausea, vomiting nor headache. He showed irregular horizontal oscillations of the eyes. Electronystagmographic study showed that this jerky eye movement appeared especially with changes of fixation of the eyes. It was also recorded during conjugate eye movement, and while he closed his eyes. He was ataxic, unable to walk, but no other abnormalities in cerebellar functions were observed. Spinal tap was performed and yielded watery clear cerebrospinal fluid containing 9/mm3 mononuclear cells. Clonazepam was given, 1.5 mg per day, for three days followed by doses of 3 mg per day. Improvement in walking was observed one week after starting the medication, when reserpine was started at a dose of 1 mg per day and increased to a dose of 1.5 mg per day in three days. One week after starting reserpine, opsoclonus improved markedly and he became able to read again. He was discharged home on September 3, 1978. Six months after admission, reserpine was decreased to 0.5 mg per day. Difficulty in reading developed within a month. Reserpine was given 1.0 mg per day and the doses was continuously given for next three months. One year after admission, he is back to his former occupation without medication. He complains of slight difficulty in reading for more than an hour, and in watching TV.(ABSTRACT TRUNCATED AT 250 WORDS)     

Radiation therapy in paediatric gliomas: our institutional experience

Background and purposeThe aim of our retrospective study was to analyze the clinical outcome of paediatric glioma patients treated with radiation therapy (RT) in our institution.Material and methodsWe retrieved the case records of all children with gliomas (age < 18 years) who received RT in our department between 2004 and 2007. We analyzed the information regarding patients' demography, clinical details, treatment given, RT details, and survival. The event-free survival (EFS), the period from the date of completion of RT to the date of the event, i.e. death/recurrence, was calculated with respect to age, sex, location of tumour (brainstem vs. non-brainstem), histopathology (low grade vs. high grade), extent of surgical resection, dose and duration of RT, and use of chemotherapy.ResultsA total of 70 children with glioma received RT during the above-mentioned period. The 3-year EFS rate for all patients was 44% and the median EFS period was 18 months. The 3-year EFS in patients who underwent surgical decompression and no surgery was 58% and 25%, respectively (p < 0.05). Patients with brainstem lesions had statistically significantly lower 3-year EFS to non-brainstem gliomas (28% vs. 56%, p < 0.01). Chemotherapy use showed no statistically significant trend towards better survival.ConclusionsRT is an effective modality of treatment in paediatric glioma patients in our setup. Early use of RT in incompletely resected low-grade gliomas is worth revisiting. Results of chemotherapy in high-grade glioma and brainstem gliomas are encouraging.