J L Montes

McGill University, Montréal, Quebec, Canada

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Publications (41)104.62 Total impact

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    ABSTRACT: Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.
    Neurosurgical FOCUS 06/2013; 34(6):E7. · 2.49 Impact Factor
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    ABSTRACT: Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.
    Neurosurgical FOCUS 06/2013; 34(6):E6. · 2.49 Impact Factor
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    ABSTRACT: Assess the role of radiotherapy (RT) in the management of primary and recurrent myxopapillary ependymoma (MPE). We conducted a retrospective review of patients with MPE treated at the Montreal Children's Hospital/McGill University Health Centre between 1985 and 2008. Seven children under the age of 18 were diagnosed and treated for MPE. All patients were treated with surgery to the primary site. Three patients underwent subtotal resection (STR) and received adjuvant post-operative RT. Only one patient who had spinal drop metastases received post-operative RT to the lumbosacral region following complete resection of the primary tumor. After a median follow up of 78 months (range 24-180 months), all patients were alive with controlled disease. The single patient treated with gross total resection (GTR) and adjuvant local radiation remained recurrence free. One of the three patients treated with STR and adjuvant RT had disease progression that was controlled with re-resection and further RT. Two of the three patients treated with surgery alone developed local and disseminated recurrent spinal disease that was controlled by salvage RT. Our data support the evolving literature which suggests that GTR alone provides suboptimal disease control in MPE. In our patients, RT resulted in control of residual, metastatic and/or recurrent disease. Routine adjuvant RT may improve outcomes in pediatric MPE.
    Pediatric Blood & Cancer 10/2010; 55(4):639-43. · 2.35 Impact Factor
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    ABSTRACT: Congenital brain tumors in the younger pediatric population are rare lesions that are histologically distinct from those in the older pediatric population. Malignant histology is common, with persistently poor outcomes despite accessible neuroimaging and evolving adjuvant therapy. There remains scant literature about the natural history of these patients because of rarity and varied institutional experiences. A retrospective review was performed of congenital brain tumor patients surgically treated at the Montreal Children's Hospital (MCH) over a 22-year period. Patients presenting in the first year of life were evaluated for demographic information, presenting symptoms, lesion location, and management. Analysis was by median rank test and chi(2) statistics. 13 cases of congenital brain tumors were identified: 5 supratentorial and 8 infratentorial. Median age (p = 0.93) and gender (p = 0.57) did not differ by location, and predominant histologies were choroid plexus papilloma and primitive neuroectodermal tumor. Seizure activity was exclusive to supratentorial lesions (40%, p = 0.03), with hypotonia observed only among infratentorial lesions (50%, p = 0.02). There was equal incidence of hydrocephalus (69%, p = 0.57) and increasing head circumference (38%, p = 0.27) by lesion location. Supratentorial lesions were treated by total resection (n = 3), subtotal resection (n = 1), and biopsy (n = 1). Infratentorial lesions were treated by total resection (n = 1), subtotal resection (n = 2), biopsy (n = 1), no operation (n = 2), and decompressive laminectomy for two spinal lesions. Congenital brain tumor patients represent fewer than 2% of patients treated at MCH. An evolving understanding of management objectives for these lesions requires understanding institutional experiences. Patients with supratentorial lesions frequently present with seizures, hydrocephalus, and macrocrania, and more frequently underwent total resection at surgery.
    Pediatric Neurosurgery 11/2009; 45(5):368-74. · 0.42 Impact Factor
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    ABSTRACT: Surgery is an accepted treatment for carefully selected patients with focal epilepsy. In the present study, the authors assessed clinical and surgery-related data obtained in a large series of children suffering from intractable temporal lobe epilepsy (TLE). Etiological, pathological, and clinical features of possible prognostic significance were studied in 109 children who underwent surgery for TLE at the Montreal Neurological Institute and Hospital and the Montreal Children's Hospital between 1985 and 2000. The mean age of patients at seizure onset was 5.5 years and the duration of epilepsy ranged from 0.1 to 17.6 years. Preoperative magnetic resonance imaging revealed mesial sclerosis in 51 patients, a mass lesion in 45, and no visible abnormalities in 12. In six patients invasive monitoring was required. Cortical amygdalohippocampectomy was performed in 72% of patients, whereas 20% underwent transcortical selective amygdalohippocampectomy. In 23 patients a second surgical intervention was necessary. Low-grade tumors were found in 35% and mesial sclerosis was confirmed on pathological evaluation in 45%. Outcome was excellent (seizure free or > 90% reduction) in 94 patients (86%). The patients were followed prospectively for a median of 10.9 years (range 5-20.2 years). There were no permanent neurological complications and no deaths. Successful postsurgical outcomes, especially in patients treated for mesial temporal lobe sclerosis and lesion-related epilepsies, can be obtained in pediatric patients suffering minimal complications. Unfavorable outcomes are most likely to occur when epileptiform discharges are bitemporal or multifocal in distribution and in cases involving incomplete resection of mesiotemporal structures.
    Journal of Neurosurgery 11/2005; 103(5 Suppl):401-12. · 3.15 Impact Factor
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    ABSTRACT: Purpose: To assess the efficacy of functional hemispherectomy in promoting language acquisition in a child with severe language delay and continuous left hemispheric epileptiform discharge in the absence of clinical seizures. We report a 6-month-old girl who initially presented with a right hemiparesis secondary to a lesion of probable prenatal origin in the distribution of the left middle cerebral artery. Weeks later, she developed infantile spasms. At 1.5 years of age, because of intractable seizures, the patient had fenestration of the left porencephalic cyst and anterior temporal lobectomy. The seizures ceased; however, language development remained limited to 35 words at 3.5 years of age. The EEG showed almost continuous epileptiform activity over the left hemisphere with no independent epileptiform potentials contralaterally. She underwent a functional left hemispherectomy.Methods: Imaging, electroencephalographic investigation, pre- and postoperative psychological and speech assessment were carried out.Results: Considerable language, speech, and behavior improvement was noted within 2 months of surgery.Conclusions: Interictal epileptiform discharges can interfere with the development of contralateral hemispheric function in the absence of clinical seizures. Early functional hemispherectomy may have a role in promoting optimal language development in appropriately selected patients. Although the primary indication for functional hemispherectomy is to control intractable seizures, a secondary proposed indication is to reduce functional impairment of the other hemisphere by electrical interference.
    Epilepsia 08/2005; 39(7):787 - 792. · 3.96 Impact Factor
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    ABSTRACT: Posterior quadrantic dysplasia (PQD), a developmental malformation involving the temporal, parietal, and occipital lobes of one cerebral hemisphere, leads to intractable epilepsy. To characterize the clinical features of 19 patients with PQD and analyze the postsurgical outcome of those who underwent resection of dysplastic tissue. The extent and nature of the malformation were primarily assessed with high-resolution brain imaging. Fourteen patients underwent complete or partial temporoparieto-occipital resection or temporal resection associated with parieto-occipital disconnection. Postoperative follow-up period ranged from 8 months to 7 years. The authors used the Engel classification for postoperative outcome. All patients were sporadic. Clinical features included infantile spasms, partial seizures, mental retardation, mild hemiparesis, and visual field defects. Neuroimaging localized the malformation within the posterior cerebral quadrant contralateral to the neurologic deficit and demonstrated hemi-hemimegalencephaly in 14 of 19 patients and multilobar cortical dysplasia in 5 of 19 patients. The authors observed class I outcome in six patients. Two patients had class II and four patients had class III outcome. Class IV outcome was seen in two patients. After surgery, two patients developed mild hemiparesis, and two developed a visual field defect. Widespread cortical dysplasia is more frequent in the posterior quadrant. In our series, posterior quadrantic dysplasia represents either hemi-hemimegalencephaly or multilobar cortical dysplasia. Individuals with posterior quadrantic dysplasia share a spectrum of clinical features. The intractable epilepsy in these patients may be alleviated by a large quadrantic temporoparieto-occipital resection.
    Neurology 07/2004; 62(12):2214-20. · 8.25 Impact Factor
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    ABSTRACT: Twelve patients suffering from intractable epilepsy and presenting with radiological evidence of diffuse hemispheric involvement of a dysplastic process, were treated by disconnective hemispherectomy, either functional hemispherectomy or peri-insular hemispherotomy. The median age at surgery was 4.5 years old and the interval between seizure onset and surgery, 3 years. All patients underwent a presurgical evaluation that led to the suggestion of disconnective hemispherectomy. Over 70% of patients have remained in Engel's seizure outcome class I since surgery and another 18% have had a satisfactory seizure outcome. There was one unexplained death and one case of early hydrocephalus. Hemispherectomy offers the possibility to improve seizure control in the majority of patients undergoing surgery for extensive dysplastic pathology of the hemisphere. Disconnective techniques reduce the rate of complications in this specific pathology.
    Epileptic disorders: international epilepsy journal with videotape 10/2003; 5 Suppl 2:S125-30. · 1.17 Impact Factor
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    ABSTRACT: Children with neurofibromatosis type 1 (NF1) undergo costly surveillance scanning for a variety of asymptomatic central nervous system lesions whose natural history is poorly understood. We performed a 20-year retrospective chart review of 25 patients with clinically proven NF1 who required surgery (group A) and contrasted this cohort with 150 NF1 patients who did not require surgery (group B). In group A, 52% of patients underwent multiple procedures for more than one lesion (p = 0.043). Group A patients were further distinguished from those in group B by exhibiting a greater number of optic gliomas (p = 0.015), nonoptic intracranial tumors (p = 0.006), cranial nerve (p = 0.000), paraspinal (p = 0.0062), craniofacial (p = 0.001) and visceral (p = 0.03) neurofibromas and moyamoya disease (p = 0.00), as well as a higher frequency of seizure disorder, sphenoid wing dysplasia and poor academic performance. Gadolinium enhancement occurred in 43% of optic gliomas, 50% of parenchymal gliomas, 100% of cranial nerve, 100% of plexus, 67% of paraspinal, 50% of craniofacial and 50% of visceral neurofibromas in group A, while only 1 group B tumor enhanced. In group A, radiological progression occurred after a median of 4 years from initial diagnosis for optic gliomas as well as cranial nerve, plexus and visceral neurofibromas, 2 years for paraspinal neurofibromas and brainstem gliomas and 2.7 years for craniofacial neurofibromas. Only 1 tumor progressed in group B. Therefore, a small subgroup of NF1 patients (12.5%) who require treatment are at risk of subsequently needing further surgical attention, whereas a larger group of NF1 patients (87.5%) carry an indolent form of the disease. We recommend imaging for asymptomatic, gadolinium-enhancing lesions every 2 years for optic pathway and parenchymal gliomas and cranial nerve and visceral neurofibromas, and every year for brainstem gliomas and paraspinal as well as craniofacial neurofibromas. Nonenhancing optic pathway lesions could be followed up radiographically much less often since they do not show progression.
    Pediatric Neurosurgery 10/2002; 37(3):122-36. · 0.42 Impact Factor
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    ABSTRACT: Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently. To report a series of 13 patients in whom the hamartoma itself was resected. All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y). Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate. Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.
    Neurology 06/2002; 58(9):1338-47. · 8.25 Impact Factor
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    ABSTRACT: To present the ophthalmic features and visual prognosis of patients with slit-ventricle syndrome (SVS). Observational case series. Six patients diagnosed and treated with SVS at the Montreal Children's Hospital between 1985 and 1999. Patients were included in this study if they had an appropriate ophthalmologic follow-up and if they fulfilled the criteria for the diagnosis of SVS based on intracranial pressure monitoring and neuroimaging studies. Features studied included patients' baseline ophthalmologic evaluation, visual outcome, and neurosurgical characteristics. The follow-up ranged from 1 to 14 years. The average number of shunting procedures was 3.7. Ocular examination performed on the initial visit revealed a normal visual acuity in four patients, esotropia in three of six patients, and nystagmus in two of six patients. Cycloplegic refraction was normal in all the subjects. Initial funduscopic evaluation revealed optic atrophy in a 4-month-old infant, whereas two children developed optic atrophy later in the course of the disease. One child developed severe visual field defects. The two children with optic atrophy had moderate to severe loss of visual acuity associated with SVS. A prompt recognition of patients with SVS is crucial, because these individuals are at an increased risk for significant visual loss.
    Ophthalmology 04/2002; 109(3):520-4; discussion 524-5. · 5.56 Impact Factor
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    ABSTRACT: Case records of 37 patients with a diagnosis of brainstem glioma treated at the Montreal Children's Hospital from June 1989 to June 1999 were reviewed. 15 patients had diffuse pontine gliomas and 22 patients had focal forms of brainstem gliomas. The two groups were compared with respect to age, clinical evolution, radiological appearance, type of surgery practised, histological diagnosis, adjuvant treatments and survival. A non-pontine brainstem location, a cystic or exophytic component, bright enhancement with gadolinium injection, a histological diagnosis of pilocytic astrocytoma or ganglioglioma were favourable prognostic factors. Progression-free survival and overall survival were significantly worse in the group of patients with diffuse pontine gliomas. The relative impact of radical surgery and/or radiotherapy is analysed. Surgery coupled to adjuncts such as navigation, ultrasound and monitoring plays an important role for focal brainstem lesions. Focal/conformal radiotherapy has an adjuvant role but better treatments are needed for the diffuse pontine brainstem lesions.
    Pediatric Neurosurgery 05/2001; 34(4):206-14. · 0.42 Impact Factor
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    ABSTRACT: Residual seizures after functional hemispherectomy occur in approximately 20% of patients with catastrophic epilepsy. These episodes are traditionally attributed to incomplete disconnection, persistent epileptogenic activity in the ipsilateral insular cortex, or bilateral independent epileptogenic activity. The authors report on the case of an 8-year-old boy with an intractable seizure disorder who had classic frontal adversive seizures related to extensive unilateral left hemispheric cortical dysplasia. The initial intervention consisted of extensive removal of the epileptic frontal and precentral dysplastic tissue and multiple subpial transections of the dysplastic motor strip, guided by intraoperative electrocorticography, Subsequently, functional hemispherectomy including insular cortex resection was performed for persistent attacks. After a seizure-free period of 6 months, a new pattern ensued, consisting of an aura of fear, dystonic posturing of the right arm, and unusual postictal hyperphagia coupled with an interictal diencephalic-like syndrome. Electroencephalography and ictal/interictal single-photon emission computerized tomography were used to localize the residual epileptic discharges to deep ipsilateral structures. Results of magnetic resonance imaging indicated a complete disconnection except for a strip of residual frontobasal tissue. Therefore, a volumetric resection of the epileptogenic frontal basal tissue up to the anterior commissure was completed. The child has remained free of seizures during 21 months of follow-up review. Standard hemispherectomy methods provide extensive disconnection, despite the presence of residual frontal basal cortex. However, rarely, and especially if it is dysplastic, this tissue can represent a focus for refractory seizures. This is an important consideration in determining the source of ongoing seizures posthemispherectomy in patients with extensive cortical dysplasia. It remains important to assess them fully before considering their disease refractory to surgical treatment.
    Journal of Neurosurgery 04/2001; 94(3):510-4. · 3.15 Impact Factor
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    ABSTRACT: Mesial temporal sclerosis (MTS) is the most frequently encountered lesion in adult patients with intractable temporal epilepsy; it is found in isolation in approximately two-thirds of surgically treated cases. Whereas the exact etiology of MTS is still controversial, several reports suggest that this pathologic lesion is both the cause and the consequence of chronic seizures and develops progressively during childhood secondary to recurrent seizures. In order to evaluate the clinical importance of MTS in children, we retrospectively reviewed the clinical charts of children who underwent surgery for medically intractable temporal epilepsy and report cases presenting an amygdalo-hippocampic dual pathology. Six children aged 1.5-16 years (mean +/- SD: 7.5 +/- 3 years) presenting with partial complex seizures (5 cases) or extension spasms (1 case), with onset from 6 months to the age of 8.5 years (mean seizure onset +/- SD: 3 +/- 5 years) underwent anterior temporal lobectomy including resection of the amygdala and hippocampus. All patients exhibited variable degrees of severity of neuronal loss and gliosis in the amygdala and/or hippocampus. The pathological picture of MTS was not isolated, however. Careful pathological examination has thus shown foci of amygdalo-hippocampic neuronal dysplasia in six patients, with concomitant bilaminated fascia dentata in two cases. Postoperatively, no mortality or morbidity was encountered. After a mean follow-up of 2.5 years, four patients are seizure free. One patient had a 80% rate of improvement in seizure frequency, though still having occasional febrile convulsions. In another patient, complex partial seizures resolved, but rare episodes of absence were still observed. These data are in keeping with the hypothesis that MTS could be secondary to repeated seizures. The analysis of this series of patients could suggest that mesiotemporal dysplastic lesions within the amygdalo-hippocampic structures induce seizures, which, in turn, will favor the development of MTS during childhood. MTS could then lead to synaptic reorganization, which can express abnormal hyperexcitability and result in more recurrent seizures. In this way a vicious circle is set up, which may explain the progression of seizures in some patients.
    Child s Nervous System 12/2000; 16(10-11):719-23. · 1.24 Impact Factor
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    ABSTRACT: The management of craniopharyngioma has been controversial for years. We review our 20 year experience in the treatment of paediatric craniopharyngioma. Twenty-five patients were treated for craniopharyngioma at The Montreal Children's Hospital from 1972 to 1991. They included 15 males and 10 females whose median age was 10 years. The initial clinical manifestations were remarkable for a predominance of symptoms and signs related to intracranial hypertension, followed in frequency by visual and endocrinological deficits. Radiologically, there were five cystic craniopharyngiomas, one solid and 19 mixed. Several therapeutic approaches were used including stereotactic drainage followed by radiotherapy or radiosurgery (three cases), transsphenoidal removal (six cases) and subfrontal and/or pterional craniotomy for total (three cases) or partial (13 cases) removal, followed by radiotherapy in 10 cases. The follow up period averaged 11 years. Eight patients recurred, three after total and three after partial removal; none of these six patients had received radiotherapy. Two cases treated by stereotactic drainage recurred, one received adjuvant radiosurgery and the other conventional radiotherapy. Morbidity was lower, and quality of survival better, with more conservative approaches. More conservative approaches in the management of craniopharyngioma are reasonable alternatives for treatment. Similar rates of disease control are observed with less morbidity and better quality of survival. Copyright 1999 Harcourt Publishers Ltd.
    Journal of Clinical Neuroscience 05/1999; 6(3):199-203. · 1.25 Impact Factor
  • Frontiers of radiation therapy and oncology 02/1999; 33:114-22.
  • Frontiers of Radiation Therapy and Oncology - FRONT RADIAT THER ONCOL. 01/1999; 33:114-122.
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    ABSTRACT: Patients with hypothalamic hamartomas present with epileptic attacks of laughter and later experience multiple seizure types and cognitive decline, suggestive of secondary generalized epilepsy. It has been suggested in the past that gelastic seizures originate in the temporal lobes rather than in the hamartoma, but temporal resections have been ineffective. Recent electrophysiologic evidence suggests that the epileptogenic discharges may originate in the hamartoma itself. We used proton magnetic resonance spectroscopic imaging to quantify the amount of neuronal damage in the temporal lobes and hamartomas of patients with hypothalamic hamartomas and gelastic seizures. Five patients were studied and the relative intensity of N-acetylaspartate to creatine (NAA/Cr) was determined for both temporal lobes as well as for the hamartoma. These values were compared with signals from the temporal lobes and hypothalami of normal control subjects. NAA/Cr was not significantly different from normal control subjects for either temporal lobe, nor was there a significant asymmetry between the two temporal lobes for any of the patients. NAA resonance signals were present in the hamartomas, and the ratio of NAA to Cr was decreased in the hamartomas compared with the hypothalami of normal control subjects (t = 4.5, p = 0.005). We found no detectable neuronal damage in the temporal lobes of patients with hypothalamic hamartomas and gelastic epilepsy. This is further evidence that gelastic seizures do not originate in the temporal lobes of these patients.
    Neurology 11/1998; 51(4):1046-50. · 8.25 Impact Factor
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    ABSTRACT: We evaluated the role of magnetic resonance imaging (MRI) of the lumbosacral spinal cord in children with complicated voiding dysfunction and normal neuro-orthopedic examination. We reviewed the records of 32 consecutive children with complicated enuresis who were referred for neurosurgical evaluation, including those with a history of refractory voiding dysfunction or incontinence associated with persistent vesicoureteral reflux, encopresis, or associated leg or back pain. Nine patients were excluded from study because of urethral or anorectal anomalies, or failure to meet the inclusion criteria. Eligible for study inclusion were 23 children with a mean age of 8.9 years. Complete neurological and orthopedic examinations were normal in all patients except 1 with mild scoliosis and 1 with congenital facial palsy. Urodynamic studies revealed instability in 14 cases, hypertonia in 7, hyporeflexia in 2 and detrusor-sphincter dyssynergia in 4. Skeletal abnormalities, mostly spina bifida occulta, were detected in 16 of the 23 children (70%). Spinal MRI was normal in 21 patients (91.3%), including 1 with a tethered cord and lipoma associated with a complex skeletal abnormality, and 1 with a nonprogressive, nonsurgical T7 to T9 syrinx. Only the case of lipoma required neurosurgical intervention. The value of MRI is limited in children with voiding dysfunction and a normal neuro-orthopedic assessment. This study should be reserved for patients with associated neuroorthopedic findings or complex skeletal deformity on plain x-ray.
    The Journal of Urology 10/1998; 160(3 Pt 2):1080-3. · 3.70 Impact Factor
  • C R Freeman, J P Farmer, J Montes
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    ABSTRACT: Developments in imaging and in neurosurgical techniques over the past decade have substantially altered the management of children with low-grade astrocytoma. Indications for surgery have become more clearly defined, and a larger proportion of children undergo complete or subtotal resection than in the past. Fewer receive adjuvant therapy, even though the options in this regard are more numerous now and theoretically likely to result in less morbidity than conventional external beam radiotherapy. This review will address in particular the correlations between location, imaging appearance, and behavior that need to be more widely appreciated, and present recommendations regarding the management of these tumors.
    International Journal of Radiation OncologyBiologyPhysics 08/1998; 41(5):979-87. · 4.52 Impact Factor