Luiz Fernando Fonseca

Early-onset acute transverse myelitis following hepatitis B vaccination and respiratory infection: case report

Acute transverse myelitis is an acute inflammatory process of the spinal cord and it is a rare clinical syndrome in childhood. In this paper, we report a case of 3 years-old boy who developed acute onset tetraparesia following a viral respiratory infecction and hepatitis B vaccination. Magnetic resonance imaging of the spinal cord disclosed signal-intensity abnormalities from C4 to C3. A diagnosis of acute transverse myelitis was made and the patient was treated with IV methylprednisolone and IV immunoglobulin. The child had a fair outcome despite of the very acute course of the disease and the presence of a cervical sensory level which usually harbor a poor prognosis.

Kearns-Sayre syndrome "plus": classical clinical findings and dystonia

We present a boy of eight years of age with symptoms of Kearns-Sayre syndrome (KSS) characterised by ophthalmoparesis, palpebral ptosis, mitochondrial myopathy, pigmentous retinitis, associated to short stature, cerebellar signs, cardiac blockade, diabetes mellitus, elevated cerebrospinal fluid protein concentration, and focal hand and foot dystonia. The skeletal muscle biopsy demonstrated ragged red fibers, cytochrome C oxidase-negative and succinate dehydrogenase-positive fibers. The magnetic resonance imaging showed symmetrical signal alteration in tegmentum of brain stem, pallidum and thalamus. Mitochondrial DNA analysis from skeletal muscle showed a deletion in heteroplasmic condition. The association of dystonia to KSS, confirmed by molecular analysis, is first described in this case, and the importance of oxidative phosphorylation defects in the physiopathogenesis of this type of movement disorder is stressed.

Choroid plexus papilloma and Aicardi syndrome: case report

A case of Aicardi syndrome associated with a choroid plexus papilloma of the third and both lateral ventricles in a 15 months old baby girl is repported. The tumor was completely removed via three craniotomies. Reports of the literature with the association of choroid plexus papilloma and Aicardi syndrome are rare. We suggest that children diagnosed with Aicardi syndrome should routinely undergo imaging studies of the brain, such as computed tomography or magnetic resonance.

Tumores do plexo coróideo

This analysis comprises 15 children under 16 years of age, with choroid plexus tumors, seen in the Service of Paediatric Neurosurgery, Hospital das Clinicas and Hospital Sao Francisco de Assis in Belo Horizonte, Brazil, between 1981 and 1996. The patients were aged between 4 months and 16 years (average of 3 years and a half); 10 were less than 2 years, 9 were female; 14 children had clinical evidence of intracranial hypertension. All the children underwent CT scan and the choroid plexus tumors were clearly demonstrated in 14 of then. In 8 children the tumors were located in one lateral ventricle, 5 in the fourth ventricle and 2 had the tumors in more than one ventricle, 11 children required ventriculo-peritoneal shunt; 14 cases were operated on, 13 with total excision; 2 children died, respectively 7 days and one year after the surgery. Pathological examination revealed papillomas in 12 cases and carcinoma in two cases.

Spontaneous acute subdural hematoma contralateral to an arachnoid cyst

Arachnoid cysts (AC) are extra-cerebral cerebrospinal fluid collections of unknown origin. They correspond to 1% of all intracranial nontraumatic space-occupying lesions and appear more frequently in the middle fossa (50%). More than 25% of these cysts are incidental findings and the majority of patients are asymptomatic. Seizures, intracranial hypertension signs, neurological deficits, macrocrania, developmental delay and bulging of the skull are the main signs and symptoms of the lesion. AC rupture and bleeding are rare, usually occurring in young adults and associated with trauma. The risk of hemorrhage does not exceed 0.04% / year. We describe the case of a ten-year-old boy who presented with acute signs of intracranial hypertension secondary to a spontaneous acute subdural hematoma, contralateral to an AC of the middle fossa. Three factors were significant in this case: signs and symptoms occurred spontaneously; the presence of an acute subdural hematoma exclusively contralateral to the AC; successful outcome of the conservative treatment.

Hemisferectomia funcional precoce na hemimegalencefalia associada à epilepsia refratária

Hemimegalencephaly (HME) is a rare congenital brain malformation of unknown etiology. Patients with HME can present with an early onset epileptic syndrome which is often refractory to medical treatment and associated to impaired neurological development. Functional hemispherectomy (FH) has proven to be a valuable treatment alternative in patients with refractory epilepsy in this setting. Very few children operated under the age of 6 months and bearing HME and catastrophic epilepsy (CE) have been described in the literature. This study reports on 2 kids younger than 6 months with HME and CE submitted to FH with good clinical outcome.

Compressão medular alta por cisto neuroentérico: relato de caso

The authors report the case of a child with a neuroenteric cyst compressing the high cervical spinal cord at the level of C1 to C3. The tumor was completely excised through a laminotomy with good results. The literature is revised, and the embryology, symotomatology, treatment and results are discussed.

Perfuração vaginal como complicação de derivação ventrículo-peritoneal apresentação de um caso

Report of a case with vaginal perforation by the catheter following a ventri-culoperitoneal shunt. The possible causative factor and results observed are discussed.Report of a case with vaginal perforation by the catheter following a ventriculoperitoneal shunt. The possible causative factor and results observed are discussed.

Espasmos infantis: experiência em treze casos

Infantile spasms are typical crisis found in the childhood being a serious pathology with an obscure prognosis. We present the experience of the Service of Neuropediatrics in the Pediatric General Center, Belo Horizonte - Brasil, during the year of 1997, with a review of the literature about. After appropriate propaedeutics, were found 12 cases considered symptomatics and 1 case cryptogenic. All these cases were treated with ACTH during 6 weeks associated with oral antiepileptic drugs in mono or polytherapy for maintenance. We had excellent results using ACTH at the beginning of the treatment, obtaining good results in all of the cases with some side effects that were not important enough to interrupt the treatment. However, there was a recurrence rate of 55% and vigabatrin was used as second option, controlling 4 of the 5 cases. All the cases showed retardation of the neuropsychomotor development.Infantile spasms are typical crisis found in the childhood being a serious pathology with an obscure prognosis. We present the experience of the Service of Neuropediatrics in the Pediatric General Center, Belo Horizonte ¾ Brasil, during the year of 1997, with a review of the literature about. After appropriate propaedeutics, were found 12 cases considered symptomatics and 1 case cryptogenic. All these cases were treated with ACTH during 6 weeks associated with oral antiepileptic drugs in mono or polytherapy for maintenance. We had excellent results using ACTH at the beginning of the treatment, obtaining good results in all of the cases with some side effects that were not important enough to interrupt the treatment. However, there was a recurrence rate of 55% and vigabatrin was used as second option, controlling 4 of the 5 cases. All the cases showed retardation of the neuropsychomotor development.