[Atypical pseudotumoral neuromeningeal cryptococcosis in an immunocompromised patient treated for pontine glioma. Case report and literature review]. / Cryptococcose neuroméningée pseudotumorale chez une enfant immunodéprimée traitée pour un gliome infiltrant du tronc cérébral. A propos d'un cas et revue de la littérature.

BACKGROUND AND PURPOSE: We report an atypical feature of neuromeningeal cryptococcosis presenting as spinal cystic arachnoiditis and cerebellar cryptococcoma in a child treated for pontine glioma. CASE REPORT: In November 2003, we diagnosed a pontine glioma in a six-year-old female child. She was initially treated with radiotherapy (54Gy for six weeks) and dexamethasone until July 2006. From January 2004 to September 2006, the patient received 30 cycles of chemotherapy including vincristine 1.5mg/m(2) Day 1, carboplatin 150mg/m(2) Day 1, and temozolomide 150mg/m(2) Days 2-6 every 28 days. In October 2006, the patient suffered spontaneous acute low back pain radiating into both lower limbs revealing lumbar cystic arachnoiditis and cerebellar cryptococcoma. The cerebrospinal fluid (CSF) sample showed lymphocytic pleocytosis and Cryptococcus neoformans; glucose and protein levels were low. First-line medical treatment including liposomal amphotericin B, then fluconazole effectively decreased the pain. However, in February 2007, she presented with cauda equina syndrome and the spinal MRI showed that the lumbar cyst had increased in size. The patient underwent a lumbar laminectomy and cyst removal. Histology confirmed the arachnoiditis with no cancer cells or pathogenic agents. CONCLUSIONS: Arachnoiditis and cryptococcoma are rare. They can appear to be a brain neoplasm because of their pseudotumoral aspect. Often, the diagnosis can be made from the CSF sample.

[Neurosurgical treatment in childhood dystonias and dyskinesias]. / Tratamientos neuroquirúrgicos en las distonías y discinesias de la infancia.

AIM: To present the post-surgery clinical results of deep brain stimulation of the internal globus pallidus (GPi) in a group of paediatric patients with primary and secondary dystonic-dyskinetic syndrome. PATIENTS AND METHODS: Between November 1996 and May 2006, 121 patients were submitted to interventions with bilateral deep brain stimulation of the GPi, 58 of whom were children (age range: 5-16 years); 35 were cases of primary dystonia, of which 17 were carriers of the DYT1 mutation and 10 were not. A further 8 cases were myoclonic dystonias, 3 of whom presented the DYT11 mutation. With regard to the secondary dystonias, 4 were carriers of PKAN (pantothenate kinase-associated neurodegeneration) syndrome and 9 were cases of post-anoxic encephalopathies. RESULTS: Percentage improvements were as follows: in the case of DYT1+ primary dystonias, 80% at one year maintained at 3 years; in cases of DYT1-, 70% at one year maintained at 3 years, and in the myoclonic dystonias, 50% at one year and 85% at 3 years. In the group of patients with secondary dystonias due to post-anoxic encephalopathies, we found an improvement of 30% at one year and 40% at 3 years; in the group with PKAN syndrome, the figures were 60% at one year and 50% at two years. CONCLUSIONS: Treatment involving bilateral deep brain stimulation of the GPi nuclei in childhood cases of generalised dystonias is highly effective, the best prognoses being those offered by the group with primary dystonias and especially those related to the DYT1 mutation. Treatment also proved to be effective in the case of secondary dystonias, although with lower degrees of improvement.

Tratamientos neuroquirúrgicos en las distonías y discinesias de la infancia / Neurosurgical treatment in childhood dystonias and dyskinesias

Objetivo. Presentar los resultados clínicos poscirugíade estimulación cerebral profunda del globo pálido interno (Gpi)en un grupo de enfermos pediátricos con síndrome distonodiscinéticoprimario y secundario. Pacientes y métodos. Desde noviembrede 1996 hasta mayo de 2006 se operaron con estimulación cerebralprofunda del Gpi, de forma bilateral, 121 enfermos, de los cuales58 eran niños (rango de edad: 5-16 años): 35 eran distonías primarias,de los que 17 eran portadores de la mutación DYT1, 10 no y 8eran distonías mioclónicas, tres de ellas con la mutación DYT11.En cuanto a las distonías secundarias, 4 eran portadores del síndromePKAN (pantothenate kinase-associated neurodegeneration) y9 eran encefalopatías postanóxicas. Resultados. La mejoría porcentualfue la siguiente: en el caso de las distonías primariasDYT1+, 80% al año mantenida a los 3 años; en los DYT1–, 70% alaño mantenida a los 3 años, y en las distonías mioclónicas, 50%al año y 85% a los 3 años. En el grupo de enfermos con distoníassecundarias por encefalopatías postanoxicas encontramos una mejoríadel 30% al año y 40% a los 3 años, y en el grupo con síndromePKAN, 60% al año y 50% a los dos años. Conclusiones. El tratamientopor estimulación cerebral profunda bilateral de los núcleosdel Gpi en los casos de distonías generalizadas de la infanciaes altamente eficaz, siendo el grupo de las distonías primarias y especialmentelas relacionadas con la mutación DYT1 las de mejorpronóstico. En el caso de las distonías secundarias, la eficaciatambién se ha probado, aunque con grados de mejoría inferiores

Aim. To present the post-surgery clinical results of deep brain stimulation of the internal globus pallidus (GPi) ina group of paediatric patients with primary and secondary dystonic-dyskinetic syndrome. Patients and methods. BetweenNovember 1996 and May 2006, 121 patients were submitted to interventions with bilateral deep brain stimulation of the GPi,58 of whom were children (age range: 5-16 years); 35 were cases of primary dystonia, of which 17 were carriers of the DYT1mutation and 10 were not. A further 8 cases were myoclonic dystonias, 3 of whom presented the DYT11 mutation. With regardto the secondary dystonias, 4 were carriers of PKAN (pantothenate kinase-associated neurodegeneration) syndrome and 9were cases of post-anoxic encephalopathies. Results. Percentage improvements were as follows: in the case of DYT1+primary dystonias, 80% at one year maintained at 3 years; in cases of DYT1–, 70% at one year maintained at 3 years, and inthe myoclonic dystonias, 50% at one year and 85% at 3 years. In the group of patients with secondary dystonias due to postanoxicencephalopathies, we found an improvement of 30% at one year and 40% at 3 years; in the group with PKAN syndrome,the figures were 60% at one year and 50% at two years. Conclusions. Treatment involving bilateral deep brain stimulation ofthe GPi nuclei in childhood cases of generalised dystonias is highly effective, the best prognoses being those offered by thegroup with primary dystonias and especially those related to the DYT1 mutation. Treatment also proved to be effective in thecase of secondary dystonias, although with lower degrees of improvement

Treatment of dystonic syndromes by chronic electrical stimulation of the internal globus pallidus.

AIM: Dystonia is a medically intractable condition causing twisting or myoclonic movements and abnormal postures. There is an important heterogeneity among etiologies of dystonia. The electrical stimulation of the globus pallidus has been used successfully in primary generalized dystonia. The aim of this study was to examine the long-term efficacy and safety of deep brain stimulation (DBS) in the treatment of primary and secondary generalized dystonia in children and adults. METHODS: Fifty-three patients were included. Electrodes were bilaterally implanted under stereotactic guidance and connected to neurostimulators, subcutaneously inserted. Efficacy was evaluated by comparing scores on the clinical and functional Burke-Marsden-Fahn dystonia rating scales (BMFDRS) before and after implantation. Patients were divided into 3 groups: group 1 comprised 15 patients with DYT1 dystonia; group 2, 17 patients with dystonia of unknown etiology and group 3, 21 patients with secondary dystonia. The mean follow-up was 26.6+/-12.3 months for primary dystonia and 23.1+/-11.8 for secondary dystonia. RESULTS: After 1 year, the improvement of the clinical score is 71% in group 1, 74% in group 2 and 31% in group 3. The functional score was improved by 63% in group 1, 49% in group 2 and 7% in group 3. We did not find any significant difference between children and adults. In secondary dystonia, efficacy of the stimulation is more limited. The efficacy of the stimulation improved with time for the 3 groups. COMCLUSION: Electrical stimulation of the internal globus pallidus proved to be an effective treatment for generalized dystonia and should be considered as first-line therapy.

[Percutaneous sacral thermorhizotomy to treat equinism of spastic cerebral palsy children]. / Thermorhizotomie sacrée percutanée pour équinisme spastique de l'enfant infirme moteur cérébral.

We propose to evaluate the effect of sacral percutaneous thermorhizotomy on cerebral palsy children. A prospective study including 29 children followed by a multidisciplinary team was conducted from 1990 to 2000. A thermal radiofrequency lesion of the first sacral root was obtained by percutaneous puncture of the posterior intervertebral foramen with evaluation by stimulation of the best motor response for the minimal threshold (<0.5 volts). The efficacy of the lesion was evaluated by testing and modification of the stimulation threshold obtained (x 2). The results on spasticity was evaluated using the Held score and the functional effect on walking, and classified as "good" (9 cases), "efficacy" (6 cases) and "nil" (4 cases). Indications of the procedure are discussed during the walking acquisition period and growing period of the cerebral palsy child.