Calcium pyrophosphate dihydrate deposition disease of the filum terminale.

INTRODUCTION: Calcium pyrophosphate dihydrate crystal deposition disease (CPPDD) is a rare benign inflammatory joint disorder characterized by the presence of calcium pyrophosphate dihydrate crystal in the interarticular and periarticular tissue. It has been rarely described with spinal localization. METHODS: A 50-year-old woman, affected by CPPDD, presented a progressive weakness of both lower limbs associated with neurogenic claudication. Neuroradiological examinations revealed the presence of two intradural calcified lesions at level L3-L4, with no post-contrast enhancement. RESULTS: Surgery was performed and the histopathological exams documented the presence of rod-shaped crystals embedded in a fibrocartilaginous stroma. The postoperative course was uneventful and the patient experienced complete symptoms relief with a 5-year follow-up. CONCLUSION: Intradural CPPD localization at the filum terminale is an extremely rare occurrence. Total removal should be preferably attempted with a long-term focal control of the disease as we observed in our case.

Occurrence and distribution of pilomyxoid astrocytoma.

PURPOSE: To know the occurrence and distribution of Pilomyxoid Astrocytomas amongst tumours previously diagnosed histologically as Pilocytic Astrocytoma and to assess the clinical impact of this new entity. METHODS: Retrospective Diagnostic review of all cases histologically diagnosed as WHO Grade I Astrocytoma at a single Neurosurgical unit between 1990 and 2003. RESULTS: Of a total of 91 cases identified, 9 were found to have Pilomyxoid histology. Of these, 8 were children (mean age 3.33 years) and 1 adult. 6 tumours were hypothalamochiasmatic in location. The clinical course of Pilomyxoid tumours was aggressive marked by maturation, multiple recurrences and disease control was rarely achieved with single treatment modality as opposed to typical pilocytics. The overall survival of the pilomyxoid group was not statistically different from the pilocytic tumours. CONCLUSIONS: Encompassing all age-groups and locations, Pilomyxoid Astrocytomas constitute about 10% of all tumours previously diagnosed as Pilocytic Astrocytoma. Nearly two-thirds are hypothalamo-chiasmatic in location. Knowledge of this entity is essential for appropriate aggressive treatment and follow-up.

Chiari type I malformation in children.

The diagnosis of Chiari type I malformation (CIM) is more and more frequent in clinical practice due to the wide diffusion of magnetic resonance imaging. In many cases, such a diagnosis is made incidentally in asymptomatic patients, as including children investigated for different reasons such as mental development delay or sequelae of brain injury. The large number of affected patients, the presence of asymptomatic subjects, the uncertainties surrounding the pathogenesis of the malformation, and the different options for its surgical treatment make the management of CIM particularly controversial.This paper reports on the state of the art and the recent achievements about CIM aiming at providing further information especially on the pathogenesis, the natural history, and the management of the malformation, which are the most controversial aspects. A historial review introduces and explains the current classification. Furthermore, the main clinical, radiological, and neurophysiological findings of CIM are described to complete the picture of this heterogeneous and complex disease.

Epilepsy surgery of posterior quadrant dysplasia in the first year of life: experience of a single centre with long term follow-up.

Posterior quadrant dysplasia (PQD) is a rare variant of cortical dysplasia involving the posterior regions of a single hemisphere. It is always associated with early onset, refractory epilepsy often characterized by a "catastrophic" evolution. The experience on its surgical management during the first year of life is limited to sporadic, isolated cases. Between 2002 and 2005, four children less than one-year-old and affected by drug-resistant epilepsy associated with PQD were admitted to our Institution and underwent surgical treatment. One patient remained seizure-free during all the follow-up (Engel I). The remaining three children showed a recurrence of the seizures, requiring subsequent surgical procedures in two cases. In one case (Engel II), the seizure control has been obtained thanks to pharmacological treatment. The other two patients respectively had only a partial (Engel III) and a less relevant reduction of the number of seizures (Engel IV). Both the epileptic and the neuropsychological outcome of our series were significantly influenced by persistent contralateral interictal anomalies rather than by the timing of the surgical procedure. Unpredictable results should be expected in this kind of patients if there is the detection of contralateral independent epileptiform activities on the EEG at diagnosis. Parents and relatives should be aware of the results' variability, even though a reduction of seizures may be expected, enabling an easier handling of the child's condition.

Mini-invasive surgery for Chiari type I malformation.

Surgical treatment of Chiari I malformation and associated syringomyelia includes several different techniques with various degrees of invasiveness. Most extensive procedures may provide good long-term outcome in a good proportion of cases but are burdened by a quite high risk of postoperative complications. Thirty children operated on by simple bone decompression are retrospectively reviewed to assess the effectiveness of a less invasive technique. The present series comprises 30 children (18 females, 12 males; mean age: 68 months) treated from 1993 to 2005. All patients underwent foramen magnum decompression by means of suboccipital craniectomy and resection of the fibrous band at the level of foramen itself. Twenty-one children also required C1 laminectomy while a dural delamination was performed in 11 cases. The mean current follow-up is 4.3 years (1-12.6 years). Head and/or neck pain was the most frequent preoperative finding (56.7%), followed by upper and lower extremity weakness (20.0%), ataxia (20.0%) and vertigo (27.7%). Syringomyelia was present in 12 patients. A significant improvement of preoperative clinical symptoms and signs was observed in 28 patients (93.3%). Two children required adjunctive surgery. Neuroimaging revealed minor postoperative modifications in most cases regardless of tonsils location, while syringomyelia was reduced in size in 50% of the cases. Complication rate and length of hospital stay were significantly reduced compared with the literature data and our own experience using more invasive techniques. These data, compared with the literature, allow us to conclude that suboccipital craniectomy and Cl laminectomy (possibly integrated by dural delamination) is an effective and safe treatment for symptomatic children with Chiari I malformation and syringomyelia.