Hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, surgery is often unsuccessful and associated with considerable risks. In the two cases presented, gamma knife radiosurgery was applied as a safe and noninvasive alternative to obtain seizure control. Two patients, a 13-year-old boy and a 6-year-old girl, presented with medically intractable gelastic epilepsy and increasing episodes of secondary generalized seizures. Abnormal behavior and precocious puberty were also evident. Magnetic resonance (MR) imaging revealed hypothalamic hamartomas measuring 13 and 11 mm, respectively. After general anesthesia had been induced in the patients, radiosurgical treatment was performed with margin doses of 12 Gy to 90% and 60% of isodose areas, covering volumes of 700 and 500 mm3, respectively. After follow-up periods of 54 months in the boy and 36 months in the girl, progressive decrease in both seizure frequency and intensity was noted (Engel outcome scores IIa and IIIa, respectively). Both patients are currently able to attend public school. Follow-up MR imaging has not revealed significant changes in the sizes of the lesions. Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas.
The combination of two minimally invasive therapies, endoscopic sinus surgery and stereotactic radiosurgery, provide a reliable new approach to the treatment of a series of olfactory neuroblastomas that offers excellent quality of life, less injury to the patient, fewer side-effects, and fewer long-term effects than other treatment strategies.
Objective-Synovial cysts of the vertebral facet joints are a source of nerve root compression. DiVerent surgical procedures are in use, but no consensus has been formed so far as to which method should be used in synovial cysts. To clarify the role of surgical management, the eYcacy of operative procedures and factors influencing the outcome in our own series of 19 patients treated between 1994 and 1998 were analysed. Methods-Nineteen patients with a mean age of 65 years underwent surgery for medically intractable radicular pain or neurological deficits caused by synovial cysts. The patients' records were retrospectively analysed for neurological deficits, cysts diameter, operative approach, segmental hypermobility, and clinical outcome; CT and MRI were analysed for additional degenerative changes. Results-In 17 patients an excellent result and in two patients a good postoperative result was achieved. Twelve patients were found to have hypermobility of the facet joints and six had spondylolisthesis. There was no correlation between cyst diameter, operative approach, and outcome. No intraoperative or postoperative complications occurred. Conclusions-Age and hypermobility may play a part in the aetiology of facet joint synovial cysts. As all operative strategies showed equally good clinical outcome, total excision via a small flavectomy as the least invasive approach should be considered therapy of choice in patients with cysts causing neurological deficits. (J Neurol Neurosurg Psychiatry 2001;70:74-77) Keywords: facet joint; flavectomy; lumbar spine; spondylolisthesis; radicular pain; synovial cyst Space occupying lesions of the spinal canal often cause radicular pain or neurological deficits and are most commonly caused by either disc herniation or a bony stenosis of the spinal canal. In a few cases pain in the leg and accompanying paraesthesia is caused by neurinomas or inflammatory processes. A rare source of extradural mass eVects are cysts of the facet joints (accounting for an incidence of 0.8% in our institution). They are cited in the literature as synovial or ganglion cysts.
The aim of this clinical study was to determine the tumour control rate, clinical outcome and complication rate following gamma knife treatment for glomus jugulare tumours. Between May 1992 and May 1998, 13 patients with glomus tumours underwent stereotactic radiosurgical treatment in our department. The age of these patients ranged from 21 to 80 years. The male:female ratio was 2:11. Six patients had primary open surgery for partial removal or recurrent growth and subsequent radiosurgical therapy. Radiosurgery was performed as primary treatment in 7 cases. The median tumour volume was 6.4 cm3 (range: 4.6-13.7 cm3). The median marginal dose applied to an average isodose volume of 50% (30-50%) was 13.5 Gy (12-20 Gy). In 10 patients, a total of 48 MRI and CT follow-up scans were available. The remaining three patients have been excluded from the postradiosurgical evaluation since the observation time (t < 12 months) was too short or patients were lost to follow up. The median interval from Gamma Knife treatment to the last radiological follow-up was 37.6 months (5-68 months). In 4 patients (40%) decreased tumour volumes were observed and in 6 cases (60%) the tumour size remained unchanged. Neurological follow-up examinations revealed improved clinical status in 5 patients (50%), a stable neurological status in 5 patients (50%) and no complications occurred. According to our preliminary experience Gamma Knife radiosurgery represents an effective treatment option for glomus jugulare tumours.
Based on the favourable results recorded so far, the combination of endoscopic sinus surgery and radiosurgery can be considered a promising treatment option for esthesioneuroblastoma that merits further consideration.
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