César Vinícius Grande

Bilateral pallidotomy for generalized dystonia

OBJECTIVE To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. BACKGROUND Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus) pallidotomies for Parkinsons disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. METHODS Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Scale (BFM). Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM) by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. RESULTS The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58% was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. CONCLUSIONS Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure.

Ventral extradural spinal meningeal cyst causing cord compression: Neurosurgical treatment

Spinal extradural meningeal cysts are typically formed by a thin fibrotic membranous capsule, macroscopically similar that of an arachnoid membrane, filled by cerebro spinal fluid and related to a nerve root or to the posterior midline. Ventral location is extremely rare and when it occurs they usually cause spinal cord herniation through the ventral dural gap. A 61 year-old man who began with a two years long history of insidious tetraparesis, spasticity and hyperreflexia in lower extremities, and flaccid atrophy of upper limbs, without sensory manifestations, is presented. Investigation through magnetic resonance imaging demonstrated an extensive spinal ventral extradural cystic collection from C6 to T11. The lesion was approached through a laminectomy and a cyst-peritoneal shunt was introduced. The cyst reduced in size significantly and the patient is asymptomatic over a 48 months follow-up. This is the first reported case of a spontaneous ventral extradural spinal meningeal cyst causing cord compression. Cyst-peritoneal shunt was effective in the treatment of the case and it should be considered in cases in which complete resection of the cyst is made more difficult or risky by the need of more aggressive surgical maneuvers.

Fatores prognósticos no tratamento dos tumores intradurais extramedulares: estudo de 44 casos

Between 1993 and 1999, 44 patients submitted to resection of an expansible intradural extramedullary lesion who filled protocol requirements of appropriate follow up were studied. Patients were constituted by 43.2% female and 56.8% male. The mean age was 32.9 years old. Lesion most common location was at the thoracic spine, with 45.5% of the cases, followed by the lumbar level with 18.2%. Tumor extension varied from 1 to 7 vertebral segments, with an average of 2.5 levels. Schwannoma, with 65,9% of the cases, was the most frequent lesion, followed by meningioma with 20.5%. There were 2 cases of neurofibroma and 1 case of paraganglioma, neuroenteric cyst, metastasis and malignant schwannoma. The evolution was of improvement in 56.8%, stability in 31.8% and of worsening in 11.4%. There was no mortality related to the surgical procedure. All cases of worsening had total resection and they had lesions located in the thoracic segment. Total resection is the ideal modality of surgical treatment. However, at the thoracic level, where the peculiarities of spine irrigation prevail, surgical morbidade may be higher (p=0.014).

Ressonância magnética dos cavernomas intraventriculares: aspectos diagnósticos

Cavernomas are vascular malformations that typically affect the white matter of cerebral hemispheres and brain stem. They are angiographically occult lesions that depend on magnetic resonance imaging (MRI) for their diagnosis, presenting a hypointense perilesional ring caused by hemossiderin deposition as seen in T2 sequences. The ventricular location is rare, and image features may differ. We present two cases with diagnosis made only by histopathologic examination, due to a lack of classic image findings. Cavernous hemangiomas must be included in the differential diagnosis of intraventricular tumors, and total surgical resection is the treatment of choice. Perilesional ring as demonstrated by MRI, must not be expected when dealing with such lesions.

Fatores prognósticos no tratamento dos tumores intramedulares

Between 1993 and 1999, in the Divisions of Neurosurgery of the Hospital Nossa Senhora das Gracas and the Hospital de Clinicas in Curitiba, Parana, Brazil, 35 patients harboring intramedullary spinal cord tumors who were submitted to microsurgery were analyzed. There were 24 males (68.6%) and average age was 32.9 years. The main location, with 40% of cases, was the thoracic level, followed by cervical and cervico-thoracic levels with 25.7%. Neurological exam, carried out between 6 and 12 months after surgery, showed that 42.9% of patients improved, 34.3% were stable and 22.9% presented neurological worsening. Total resection was obtained in 57.2% of cases and subtotal in 37.1%. In 5.7% of patients a biopsy was the accomplished procedure. Total resection was more often obtained among patients with ependymomas (13 out of 17) than with astrocytomas (5 out of 12). However, degree of resection and tumor histology did not interfer in postoperative morbidity. Factors as sex, age and tumors size also did not demonstrate significance in predicting prognostic after surgery, whereas tumor in a thoracic level was associated with higher morbidity (p=0.021).

Meningiomas em pacientes pediátricos: relato de 2 casos

Meningiomas correspond to 1% - 4% of primary intracranial tumors in pediatric group, with their incidence raising according to age. There is not gender prevalence, in spite of some authors describe a male tendency opposed to female one in adulthood. At present study we describe two cases of pediatric meningiomas reviewing clinical, radiological and histological aspects of these lesions. The authors review also treatment options and prognosis of childhood meningiomas. A two-year-old boy was admitted with seizures. Computerized tomography showed a right parietal lesion, which was totally resected. Histological features were compatible with meningioma. After 17 months the child is doing well, with no deficits or seizures. The second case is a 12-year-old girl, with a headache complain. During investigation, a CT revealed a right frontal lesion. She was operated under a right frontal craniotomy with total tumor resection. Nowadays she is asymptomatic, 20 months after surgery. Despite meningiomas in pediatric group are uncommon; they should be included in differential diagnosis list of expansive intracranial lesions of childhood.

Combination micrografting and tattooing in the reconstruction of eyebrows of patients with craniofacial clefts.

The absence of an eyebrow, either partial or total, has been observed in patients with craniofacial clefts, such as the Tessier 9 to 13 cleft. Several techniques have been used to improve the appearance of the region, such as island scalp flaps and scalp strip grafting, with limited or marginally satisfactory aesthetic results. The authors report 2 patients with craniofacial clefts in whom a novel technique combining 2 separate surgical approaches, micrografting and tattooing, was used. The use of micrografting with single or double hair units, properly angulated, produces natural-looking and satisfactory results with a minimum of morbidity. Excellent volume and appearance of the eyebrow may be achieved in a single session using this technique. Tattooing performed subsequently over the microimplanted hairs provides the illusion of greater density to the eyebrow, resulting in an appearance closer to normal.

Spinal cord arteriovenous malformation: a pediatric presentation.

Dra. Rosana Herminia Scola – Serviço de Doenças Neuromusculares / Hospital de Clínicas da UFPR Rua General Carneiro 181 / 3o andar 80060-900 Curitiba PR – Brasil. E-mail: scola@hc.ufpr.br Spinal vascular malformations in child are rare and still under-diagnosed entities that typically lead to progressive spinal cord symptoms and myelopathy if not properly treated. This malformation can be differentiated into congenital (arteriovenous malformations and cavernomas) or acquired (dural arteriovenous fistulae) forms. Spinal cord arteriovenous malformations (SCAVM) comprise a complex and heterogeneous group of lesions with a number of subtypes that have distinct clinical presentations, anatomic features and natural histories. While there are important differences between subtypes, the majority of patients with SCAVM present first clinical manifestations only in adolescence or adulthood. We describe the characteristics on child with SCAVM and show the possible treatment of this disease on childhood because only few cases have been published.

Ganglioglioma da medula espinhal: relato de caso

Os gangliogliomas sao tumores raros do SNC, compreendendo apenas 2,7 a 3,8% dos tumores primarios do SNC e as lesoes medulares perfazem 7,6 a 14,3 % do total de gangliogliomas. O tratamento preconizado pela literatura e a resseccao total, ficando a radioterapia reservada apenas em casos de progressao da doenca apos a cirurgia ou em casos de lesoes de comportamento histologico mais agressivo. Neste artigo relatamos um caso de uma paciente portadora de um ganglioglioma medular envolvendo os niveis T5 a T10 que foi submetida a tratamento cirurgico em nosso servico, sendo tambem realizada revisao da literatura analisando diversos aspectos, incluindo as diversas modalidades de tratamento indicadas neste tipo incomum de lesao.