Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 28
Filtrar
Más filtros

Banco de datos
Tipo del documento
País de afiliación
Intervalo de año de publicación
1.
Pediatr Neurosurg ; 55(6): 432-438, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33401272

RESUMEN

INTRODUCTION: Occult tethered cord syndrome (OTCS) and its surgical treatment are controversial. A previous study with lumbar magnetic resonance imaging (MRI) in the prone position had found statistically significant differences in morphological parameters between pediatric OTCS patients and a control group. Filum terminale internum (FTI) sectioning is currently the gold standard for the treatment of OTCS. CASE PRESENTATION: We present four cases of adolescents with OTCS, of which three cases were associated with low-lying cerebellar tonsils (LLCT). The patients presented various symptoms of OTCS from pain to sensory disturbances to alterations in sphincter functions, but none had disorders belonging to all three categories. A T2-weighted axial MRI in the prone position supported the clinical diagnosis of OTCS. The patients were treated with minimal skin incision and filum terminale externum (FTE) sectioning through the sacral hiatus under local anesthesia. DISCUSSION: We describe for the first time the association between lumbar MRI in the prone position and FTE sectioning in OTCS. The FTE sectioning has resulted in the disappearance of the pain and sensory disturbance symptoms. The results on the sphincter function are mixed. Three of our four patients with OTCS had LLCT, and all three also reported headache episodes, which, surprisingly, disappeared postoperatively. This minimally invasive surgery involves only minimal discomfort for the patient and minimizes the risks related to skin incision only. The postoperative lumbar MRI in the prone position has shown that there is no mobilization of the FTI or changes in the other preoperative morphological parameters.


Asunto(s)
Cauda Equina , Defectos del Tubo Neural , Adolescente , Cauda Equina/diagnóstico por imagen , Cauda Equina/cirugía , Niño , Humanos , Región Lumbosacra , Imagen por Resonancia Magnética , Defectos del Tubo Neural/diagnóstico por imagen , Defectos del Tubo Neural/cirugía , Posición Prona
2.
Headache ; 54(5): 899-908, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24766291

RESUMEN

BACKGROUND: Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children. OBJECTIVE: The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up. METHODS: Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders. RESULTS: Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (<5 mm, N = 12), mild (5-9 mm, N = 27), and severe tonsillar ectopia (≥10 mm, N = 6), respectively. Twenty-four out of 33 (73%) cases with Chiari 1 malformation complained of headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation. CONCLUSIONS: In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia.


Asunto(s)
Malformación de Arnold-Chiari/complicaciones , Cefalea/etiología , Adolescente , Niño , Preescolar , Femenino , Cefalea/diagnóstico , Humanos , Lactante , Estudios Longitudinales , Masculino , Neuroimagen , Estudios Retrospectivos
3.
J Neurol ; 271(5): 2149-2158, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38289534

RESUMEN

INTRODUCTION: Ocrelizumab (OCR) and Fingolimod (FGL) are two high-efficacy treatments in multiple sclerosis which, besides their strong anti-inflammatory activity, may limit neurodegeneration. AIM: To compare the effect of OCR and FGL on clinical and MRI endpoints. METHODS: 95 relapsing-remitting patients (57 OCR, 38 FGL) clinically followed for 36 months underwent a 3-Tesla MRI at baseline and after 24 months. The annualized relapse rate, EDSS, new cortical/white matter lesions and regional cortical and deep grey matter volume loss were evaluated. RESULTS: OCR reduced the relapse rate from 0.48 to 0.04, FGL from 0.32 to 0.05 (both p < 0.001). Compared to FGL, OCR-group experienced fewer new white matter lesions (12% vs 32%, p = 0.005), no differences in new cortical lesions, lower deep grey matter volume loss (- 0.12% vs - 0.66%; p = 0.002, Cohen's d = 0.54), lower global cortical thickness change (- 0.45% vs - 0.70%; p = 0.036; d = 0.42) and reduced cortical thinning/volume loss in several regions of interests, including those of parietal gyrus (d-range = 0.65-0.71), frontal gyrus (d-range = 0.47-0.60), cingulate (d-range = 0.41-0.72), insula (d = 0.36), cerebellum (cortex d = 0.72, white matter d = 0.44), putamen (d = 0.35) and thalamus (d = 0.31). The effect on some regional thickness changes was confirmed in patients without focal lesions. CONCLUSIONS: When compared with FGL, patients receiving OCR showed greater suppression of focal MRI lesions accumulation and lower cortical and deep grey matter volume loss.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Clorhidrato de Fingolimod , Sustancia Gris , Imagen por Resonancia Magnética , Esclerosis Múltiple Recurrente-Remitente , Humanos , Femenino , Masculino , Adulto , Sustancia Gris/diagnóstico por imagen , Sustancia Gris/patología , Sustancia Gris/efectos de los fármacos , Anticuerpos Monoclonales Humanizados/farmacología , Anticuerpos Monoclonales Humanizados/administración & dosificación , Anticuerpos Monoclonales Humanizados/uso terapéutico , Esclerosis Múltiple Recurrente-Remitente/tratamiento farmacológico , Esclerosis Múltiple Recurrente-Remitente/diagnóstico por imagen , Esclerosis Múltiple Recurrente-Remitente/patología , Persona de Mediana Edad , Clorhidrato de Fingolimod/farmacología , Clorhidrato de Fingolimod/uso terapéutico , Corteza Cerebral/diagnóstico por imagen , Corteza Cerebral/patología , Corteza Cerebral/efectos de los fármacos , Moduladores de los Receptores de fosfatos y esfingosina 1/farmacología , Factores Inmunológicos/farmacología , Factores Inmunológicos/administración & dosificación , Estudios de Seguimiento
4.
Neurol Neuroimmunol Neuroinflamm ; 11(6): e200301, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39178066

RESUMEN

BACKGROUND AND OBJECTIVES: Cognitive impairment (CI) in multiple sclerosis (MS) is frequent and determined by a complex interplay between inflammatory and neurodegenerative processes. We aimed to investigate whether CSF parvalbumin (PVALB), measured at the time of diagnosis, may have a prognostic role in patients with MS. METHODS: In this cohort study, CSF analysis of PVALB and Nf-L levels was performed on all patients at diagnosis (T0) and combined with physical, cognitive, and MRI assessment after an average of 4 years of follow-up (T4) from diagnosis. Cognitive performance was evaluated with a comprehensive neuropsychologic battery: both global (cognitively normal, CN, mildly CI, mCI, and severely CI, sCI) and domain cognitive status (normal/impaired in memory, attention/information processing speed, and executive functions) were considered. Cortical thickness and gray matter volume data were acquired using 3T MRI scanner. RESULTS: A total of 72 patients with MS were included. At diagnosis, PVALB levels were higher in those patients who showed a worsening physical disability after 4 years of follow-up (p = 0.011). CSF PVALB levels were higher in sCI patients than in CN (p = 0.033). Moreover, higher PVALB levels significantly correlated with worse global cognitive (p = 0.024) and memory functioning (p = 0.044). A preliminary clinical threshold for PVALB levels at diagnosis was proposed (2.57 ng/mL), which maximizes the risk of showing CI (in particular, sCI) at follow-up, with a sensitivity of 91% (specificity 30%). No significant results were found for these associations with Nf-L. In addition, patients with higher levels of PVALB at diagnosis showed higher cognitive (p = 0.024) and global fatigue (p = 0.043) at follow-up. Finally, higher PVALB levels also correlated significantly with more pronounced CTh/volume at T4 in the inferior frontal gyrus (p = 0.044), postcentral gyrus (p = 0.025), frontal pole (p = 0.042), transverse temporal gyrus (p = 0.008), and cerebellar cortex (p = 0.041) and higher atrophy (change T0-T4) in the right thalamus (p = 0.038), pericalcarine cortex (p = 0.009), lingual gyrus (p = 0.045), and medial frontal gyrus (p = 0.028). DISCUSSION: The significant association found between parvalbumin levels in the CSF at diagnosis and cognitive, clinical, and neuroradiologic worsening after 4 years of follow-up support the idea that parvalbumin, in addition to Nf-L, might represent a new potential prognostic biomarker, reflecting MS neurodegenerative processes occurring since early disease stages.


Asunto(s)
Disfunción Cognitiva , Fatiga , Sustancia Gris , Esclerosis Múltiple , Parvalbúminas , Humanos , Masculino , Femenino , Disfunción Cognitiva/líquido cefalorraquídeo , Disfunción Cognitiva/etiología , Disfunción Cognitiva/diagnóstico , Persona de Mediana Edad , Sustancia Gris/diagnóstico por imagen , Sustancia Gris/patología , Adulto , Esclerosis Múltiple/líquido cefalorraquídeo , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/diagnóstico , Esclerosis Múltiple/diagnóstico por imagen , Parvalbúminas/líquido cefalorraquídeo , Fatiga/líquido cefalorraquídeo , Fatiga/etiología , Imagen por Resonancia Magnética , Pronóstico , Estudios de Seguimiento , Estudios de Cohortes , Progresión de la Enfermedad , Biomarcadores/líquido cefalorraquídeo , Proteínas de Neurofilamentos
5.
PLoS One ; 17(1): e0262174, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35025944

RESUMEN

OBJECTIVES: The aim of this study was to evaluate the clinical performance of the Fluorecare SARS-CoV-2 Spike Protein Test Kit, a rapid immunochromatographic assay for SARS-CoV-2 detection. Moreover, we sought to point out the strategy adopted by a local company to lift the lockdown without leading to an increase in the number of COVID-19 cases, by performing a precise and timely health surveillance. METHODS: The rapid Fluorecare SARS-CoV-2 Spike Protein Test was performed immediately after sampling following the manufacturer's instructions. RT-PCRs were performed within 24 hours of specimen collection. A total amount of 253 nasopharyngeal samples from 121 individuals were collected between March 16 and April 2, 2021 and tested. RESULTS: Of 253 nasopharyngeal samples, 11 (9.1%) were positive and 242 (90.9%) were negative for SARS-CoV-2 RNA by RT-PCR assays. The rapid SARS-CoV-2 antigen detection test's mean sensitivity and specificity were 84,6% (95% CI, 54.6-98.1%) and 100% (95% CI, 98.6-100%), respectively. Two false negative test results were obtained from samples with high RT-PCR cycle threshold (Ct). CONCLUSION: Our study suggested that Fluorecare SARS-CoV-2 Spike Protein Test can be introduced into daily diagnostic practice, as its mean sensitivity and specificity follow the standards recommended by WHO and IFCC Task Force. In addition, we underlined how the strategy adopted by a local company to risk assessment and health surveillance was appropriate for infection containment. This real-life scenario gave us the possibility to experience potential approaches aimed to preserve public health and work activities.


Asunto(s)
Prueba de COVID-19/métodos , COVID-19/diagnóstico , COVID-19/virología , Nasofaringe/virología , Glicoproteína de la Espiga del Coronavirus/genética , Antígenos Virales/inmunología , Control de Enfermedades Transmisibles/métodos , Reacciones Falso Negativas , Reacciones Falso Positivas , Humanos , ARN Viral/genética , Estudios Retrospectivos , SARS-CoV-2/genética , Sensibilidad y Especificidad , Manejo de Especímenes
6.
Cephalalgia ; 31(6): 751-6, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21172953

RESUMEN

BACKGROUND: Hemiplegic migraine (HM) is a rare variety of migraine with aura, characterized by motor deficits during the aura, often beginning in childhood. The hemiplegic attacks can be severe and prolonged but the prognosis is usually good. Data on neuroimaging, including diffusion-weighted imaging (DWI) and spectroscopy, during prolonged attacks of HM are quite limited, particularly in children. CASE: An eight-year-old female had a prolonged attack of sporadic HM characterized by right-sided hemiplegia, global aphasia, fever and impairment of consciousness. MRI nine hours after hemiplegia onset was negative, while the following MRI scans (days 4 and 11) documented a progressive increase in cortical swelling in the left hemisphere with mild hyperintensity on DWI and mild reduction of apparent diffusion coefficient values. Proton MRI spectroscopy (MRS) (day 15) showed a decrease in the N-acetylaspartate/creatine ratio in the left hemisphere. (99m)Tc-ECD single-photon emission tomography (SPET) (day 27) showed marked left hemispheric hypoperfusion. The patient recovered completely after 40 days and neuroimaging follow-up (MRI and SPET) after six months was normal. The patient carried a missense mutation of the ATP1A2 gene. CONCLUSION: Multimodal neuroimaging (MRI, DWI, MRS, SPET) in a prolonged HM attack supports evidence for a primary neuronal dysfunction.


Asunto(s)
Imagen por Resonancia Magnética , Espectroscopía de Resonancia Magnética , Migraña con Aura , Tomografía Computarizada de Emisión de Fotón Único , Niño , Femenino , Humanos , Migraña con Aura/diagnóstico por imagen , Migraña con Aura/metabolismo , Migraña con Aura/patología
7.
Neurosurg Focus ; 30(4): E1, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21456920

RESUMEN

OBJECT: Although a rarely reported occurrence, late failure of endoscopic third ventriculostomy (ETV) may occur in children as a result of a variety of factors. Delay in recognition of symptoms can lead to harmful deterioration in the patient's condition. The authors undertook this study to assess the capacity of cine phase-contrast MR imaging to identify late failure in asymptomatic pediatric patients treated with ETV for hydrocephalus. METHODS: This study was a retrospective evaluation of cases involving patients who underwent ETV between January 1, 1999, and December 31, 2008, at the pediatric neurological surgery service of the University of Padua. Before 2004, patients were routinely followed up with cine MR imaging at 3, 6, and 12 months after ETV. In 2004, a protocol of annual cine MR follow-up was instituted as a result of a case of fatal late failure. The authors evaluated all cases of late failure identified through cine MR imaging and performed a statistical analysis to investigate the relationship between ETV failure and several variables, including the cause of hydrocephalus for which ETV was originally indicated. RESULTS: In a series of 84 patients (age range 6 days-16 years), 17 patients had early ETV failure. Of the remaining 67 patients, 5 (7%) were found to have no CSF flow through the fenestration and recurrent ventriculomegaly when assessed with cine MR imaging at 1, 2, 3, 4, and 7 years after ETV. The patient in whom ETV failure was identified 1 year postoperatively had Dandy-Walker malformation. The patients in whom ETV failure was identified 2, 3, and 4 years postoperatively all had undergone ETV for treatment of postinfective hydrocephalus. The patient in whom ETV failure was identified 7 years postoperatively had a cystic arachnopathy in the fourth ventricle after cerebellar astrocytoma removal. CONCLUSIONS: Patients who undergo ETV for infective hydrocephalus and Dandy-Walker malformation should receive long-term follow-up, because late closure of the stoma may occur progressively and slowly. Intraoperative observation of thickened arachnoid membranes at the level of the interpeduncular cisterns at the first ETV and a progressive decreasing of CSF flow through the stoma on routine cine MR imaging should be considered unfavorable elements entailing a significant risk of deterioration.


Asunto(s)
Endoscopía/efectos adversos , Complicaciones Posoperatorias/diagnóstico , Tercer Ventrículo/cirugía , Ventriculostomía/efectos adversos , Adolescente , Niño , Preescolar , Estudios de Cohortes , Síndrome de Dandy-Walker/etiología , Síndrome de Dandy-Walker/mortalidad , Femenino , Humanos , Hidrocefalia/cirugía , Lactante , Recién Nacido , Estimación de Kaplan-Meier , Imagen por Resonancia Magnética/métodos , Masculino , Pediatría , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/fisiopatología , Estudios Retrospectivos , Factores de Tiempo
8.
Pediatr Blood Cancer ; 55(6): 1083-8, 2010 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-20979170

RESUMEN

BACKGROUND: Little is known about the visual outcome of children affected by an optic pathway glioma (OPG). PROCEDURES: We evaluated the long-term visual outcome of 32 consecutive children affected by OPG without neurofibromatosis type-1 referred to the Pediatric Department of Padua University and managed according to standardized strategies. RESULTS: Eight children received chemotherapy, 10 radiotherapy, 7 both chemotherapy and radiotherapy, whereas 7 were untreated. At presentation, visual acuity (VA) was normal in 22 children (13 unilaterally and 9 bilaterally), and reduced in 10. At follow-up, VA had improved in 6 patients; it was stable in 8 and worse in 18. Visual field, assessed in 29 children, was normal in 9 and reduced in 20. The number of children with some grade of visual impairment increased from 7 to 10 during follow-up. Of the 17 children in whom the tumor became significantly smaller, VA improved in 6, was stable in 3, and worse in 8. Of the 6 children with improved VA, 5 received radiotherapy, and their papilla was normal or mildly pale. After a median follow-up of 6 years, 26 patients are alive with stable disease. CONCLUSIONS: The visual prognosis of children with OPG is unsatisfactory. Older children treated with radiotherapy seem to have a better visual outcome than younger children. Severe optic pallor at diagnosis or during follow-up may be indicative of a negative prognosis.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Glioma del Nervio Óptico/fisiopatología , Neoplasias del Nervio Óptico/fisiopatología , Agudeza Visual/fisiología , Campos Visuales/fisiología , Vías Visuales/fisiopatología , Adolescente , Niño , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Masculino , Neurofibromatosis 1/fisiopatología , Glioma del Nervio Óptico/terapia , Neoplasias del Nervio Óptico/terapia , Dosificación Radioterapéutica , Resultado del Tratamiento
9.
Acta Neurochir (Wien) ; 152(11): 1943-6, 2010 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-20878531

RESUMEN

BACKGROUND: The growing teratoma syndrome (GTS) consists of a mature teratoma paradoxically enlarging during or after chemotherapy for malignant nongerminomatous germ cell tumors. METHODS AND RESULTS: We report two cases of GTS occurring in association with NSGCT of the pineal gland. Although an unusual event, clinicians and radiologists should be aware of its natural history. CONCLUSIONS: When normalized tumor markers after chemotherapy are associated with imaging features of a growing mass, the hypothesis of GTS must be taken in consideration. When early diagnosed, GTS can be managed surgically with good results.


Asunto(s)
Antineoplásicos/efectos adversos , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Neoplasias de Células Germinales y Embrionarias/complicaciones , Neoplasias de Células Germinales y Embrionarias/tratamiento farmacológico , Pinealoma/complicaciones , Pinealoma/tratamiento farmacológico , Teratoma/inducido químicamente , Adolescente , Preescolar , Quimioterapia/métodos , Humanos , Masculino , Invasividad Neoplásica/patología , Invasividad Neoplásica/fisiopatología , Neoplasias de Células Germinales y Embrionarias/patología , Pinealoma/patología , Síndrome , Teratoma/patología , Teratoma/cirugía , Resultado del Tratamiento
10.
Neurol Sci ; 30(6): 525-6, 2009 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-19768371

RESUMEN

An 18-year-old man with progressive paraparesis, thermal hypoesthesia, sweating abnormalities, bladder dysfunction, severe orthostatic hypotension, bilateral Babinski sign, underwent a brain MRI scan that showed downward displacement of cerebellar tonsils through the foramen magnum, consistent with Chiari I malformation, compression of the brainstem-spinal cord junction, and C1-D11 syringomyelia (6.5 mm diameter at C2 level) consistent with Chiari I syndrome. Suboccipital craniectomy and duraplasty were performed. A C2 partial laminectomy and ablation of posterior arch of the atlas was performed. MRI scans 4 days and 1 month after surgery showed a dramatic syringomyelia reabsorption (2.5 and 1 mm, respectively) associated with complete clinical recovery.


Asunto(s)
Malformación de Arnold-Chiari/patología , Malformación de Arnold-Chiari/cirugía , Siringomielia/patología , Siringomielia/cirugía , Adolescente , Encéfalo/patología , Encéfalo/cirugía , Vértebras Cervicales , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Médula Espinal/patología , Médula Espinal/cirugía , Factores de Tiempo , Resultado del Tratamiento
11.
Pediatr Blood Cancer ; 50(6): 1154-8, 2008 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-18300319

RESUMEN

BACKGROUND: Parameningeal rhabdomyosarcoma (PM-RMS) is a rare, highly malignant pediatric tumor arising from locations adjacent to the meninges, from where it can spread intracranially. PROCEDURE: We reviewed 109 children with non-metastatic PM-RMS enrolled in the Italian RMS79, RMS88 and RMS96 protocols over a 24-year period. All patients received intensive chemotherapy and standard or hyperfractionated and accelerated radiotherapy. Some had delayed surgery. RESULTS: Five-year overall survival rose from 40% in the RMS79 to 72% in the RMS88 and RMS96 protocols (P = 0.01), where more intensive chemotherapy and hyperfractionated accelerated radiotherapy (HART) was used. Delayed surgery after initial treatment was statistically associated with a better prognosis. Unfavorable tumor characteristics for RMS arising in other sites, for example, histology, invasiveness or node involvement, did not predict outcome for PM-RMS. CONCLUSION: Outcome in PM-RMS patients enrolled in three consecutive Italian protocols has progressively improved, as a result of intensive chemotherapy, delayed surgery and, possibly, HART, though improved imaging and radiotherapeutic tools may have had a role as well.


Asunto(s)
Neoplasias Meníngeas/mortalidad , Rabdomiosarcoma/mortalidad , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Terapia Combinada , Femenino , Neoplasias de Cabeza y Cuello/mortalidad , Neoplasias de Cabeza y Cuello/terapia , Humanos , Lactante , Masculino , Neoplasias Meníngeas/terapia , Pronóstico , Rabdomiosarcoma/terapia , Tasa de Supervivencia
12.
Neuro Oncol ; 9(4): 430-7, 2007 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-17704361

RESUMEN

We evaluated the visual outcome of a cohort of children with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG) treated according to standardized therapeutic guidelines. The study population consisted of all consecutive patients with NF1 and OPG referred to a specialized pediatric neuro-oncology program between 1994 and 2004. Treatment was instituted only in cases of progressive disease or clinical deterioration. Treatment modalities were chemotherapy (based on vincristine/carboplatin) for children younger than 5 years and radiotherapy for all others. Ten boys and 10 girls (seven with a positive family history) entered the trial (median age at diagnosis of OPG, 29 months). At a median follow-up time of 78 months, seven patients had been treated with chemotherapy only, four with radiotherapy, and four with chemotherapy plus radiotherapy. Five patients were observed only. Currently, 18 are alive and two have died. Eight patients were treated for progressive visual loss in the face of stable disease, five for tumor volume increase without visual deterioration, and two for symptomatic tumor volume increase. At referral, six children had a visual acuity (VA) of < 30% in both eyes; eight children had 100% VA bilaterally. At referral, the visual field (VF) could be assessed in three children: One had VF loss in both eyes, one had VF loss in one eye, and one had normal VF. At last follow-up, eight children had VA < 20% in both eyes; only two children had 100% VA in both eyes. Among 11 children who had some visual function, three had VF loss in one eye and three in both eyes, and five had an intact VF. Contrast and color sensitivity were abnormal in seven and six patients, respectively. Thirteen children fell into the WHO hypovision category. In summary, among the 15 children treated, one had a definitive and two a mild improvement in VA. In conclusion, the visual outcome of this selected cohort of NF1 patients with OPG is unsatisfactory. A critical reappraisal of the therapeutic strategy adopted is needed.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Neurofibromatosis 1/terapia , Glioma del Nervio Óptico/terapia , Radioterapia/efectos adversos , Trastornos de la Visión/etiología , Carboplatino/administración & dosificación , Carboplatino/efectos adversos , Niño , Preescolar , Terapia Combinada , Sensibilidad de Contraste/efectos de los fármacos , Sensibilidad de Contraste/efectos de la radiación , Potenciales Evocados Visuales , Femenino , Humanos , Lactante , Masculino , Neurofibromatosis 1/complicaciones , Glioma del Nervio Óptico/complicaciones , Vincristina/administración & dosificación , Vincristina/efectos adversos , Campos Visuales/efectos de los fármacos , Campos Visuales/efectos de la radiación
13.
Brain Dev ; 27(1): 66-9, 2005 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-15626545

RESUMEN

We report the occurrence of symmetrical thalamic calcifications (STC) in one of a pair of monozygotic twins born at term without evidence of pre- or peri-natal asphyxia. STC is known to be an extremely rare condition in infants. Judging from the few cases reported in the literature, the clinical presentation is very severe: low Apgar score, no spontaneous movements, spasticity or marked hypotonia, impaired suck and swallow, facial diplegia. The prognosis is also very poor. The etiology is still a matter of debate: genetic, infectious, toxic or hypoxic-ischemic insults have been hypothesized. In our case, the presence of the lesion in one of a pair of monozygotic twins would rule out any genetic origin, nor was there any evidence of toxic or infectious disease. The only potential risk factor for fetal damage was hypoxic-ischemic insult related to the twin pregnancy.


Asunto(s)
Calcinosis/patología , Hipoxia Fetal/complicaciones , Hipoxia-Isquemia Encefálica/etiología , Hipoxia-Isquemia Encefálica/patología , Tálamo/patología , Puntaje de Apgar , Calcinosis/diagnóstico por imagen , Calcinosis/fisiopatología , Corteza Cerebral/patología , Corteza Cerebral/fisiopatología , Disartria/etiología , Disartria/patología , Disartria/fisiopatología , Distonía/etiología , Distonía/patología , Distonía/fisiopatología , Músculos Faciales/fisiopatología , Humanos , Hipoxia-Isquemia Encefálica/fisiopatología , Lactante , Imagen por Resonancia Magnética , Masculino , Espasticidad Muscular/etiología , Espasticidad Muscular/patología , Espasticidad Muscular/fisiopatología , Tractos Piramidales/patología , Tractos Piramidales/fisiopatología , Tálamo/diagnóstico por imagen , Tálamo/fisiopatología , Tomografía Computarizada por Rayos X
14.
J Child Neurol ; 27(4): 536-9, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22378677

RESUMEN

Chiari malformation type 2 is characterized by hindbrain protrusion and a constellation of supratentorial malformations. Chiari malformation type 2 is thought to be causally related to myelomeningocele due to intrauterine cerebrospinal fluid overdrainage. This relationship is so strong that it has become a rule. A 14-year-old girl affected by mental retardation, spastic triparesis, and epilepsy is presented. Brain magnetic resonance imaging disclosed a severe Chiari malformation type 2, whereas spine magnetic resonance imaging was unremarkable. The authors discuss previous literature describing rare cases of Chiari malformation type 2 without open spinal dysraphism and the relevance of concomitant, sometimes overlooked, neuroimaging findings, underlying how exceptions might be hurdles but might also eventually strengthen the rules.


Asunto(s)
Agenesia del Cuerpo Calloso/complicaciones , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/diagnóstico , Malformaciones del Desarrollo Cortical/complicaciones , Adolescente , Agenesia del Cuerpo Calloso/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Malformaciones del Desarrollo Cortical/patología
15.
Brain Pathol ; 22(6): 865-8, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-23050873

RESUMEN

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor that usually occurs in the superficial cerebral hemispheres of children and young adult and has a favorable prognosis. We report a case of a 14-year-old girl with a recent history of sciatica and ataxic gait. Pre- and post-contrast brain and spinal MRI revealed the presence of multiple solid lesions with a cystic component in the cerebellum and the spinal cord with a concomitant massive leptomeningeal involvement Histological and immunohistochemical findings were concordant with a final diagnosis of WHO grade II PXA. Even the biological indolent PXAs' behavior, this is the third report in the literature of such an unusual multicentric PXA with leptomeningeal dissemination.


Asunto(s)
Astrocitoma/patología , Neoplasias Encefálicas/patología , Carcinomatosis Meníngea/patología , Adolescente , Femenino , Humanos
16.
Neurologist ; 17(2): 83-5, 2011 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-21364359

RESUMEN

INTRODUCTION: Thrombolytic therapy for acute ischemic stroke is based on clinical assessment, blood tests, and computed tomography (CT) findings. The presence of early CT ischemic changes may help support therapeutic decision. Our aim is to call attention to a likely overlooked "stroke mimic," Gliomatosis Cerebri (GC). CASE SERIES: Clinical and neuroimaging analysis of 3 patients with documented GC, whose acute clinical onset and CT data (lenticular and ribbon sign and a ipsilateral dot sign) were suggestive of ischemic stroke. CONCLUSION: Noncerebrovascular conditions may present with both acute onset and false CT signs of early ischemic stroke. Although rare, GC needs to be considered among the possible "stroke mimics" to avoid inappropriate thrombolytic therapy.


Asunto(s)
Neoplasias Neuroepiteliales/diagnóstico , Accidente Cerebrovascular/diagnóstico , Tomografía Computarizada por Rayos X/métodos , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Neoplasias Neuroepiteliales/patología , Accidente Cerebrovascular/tratamiento farmacológico , Terapia Trombolítica
17.
Clin Appl Thromb Hemost ; 17(6): E127-30, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21159706

RESUMEN

Varicella zoster virus (VZV) is the only human virus known to replicate in arteries. After the acute infection, the virus persists in a noninfectious latent form in ganglia along the neuraxis, with intermittent periods of reactivation. Both primary and secondary reactivation are associated with stroke in children. These patients, regardless of the chosen treatment, have a high risk of recurrence, particularly those with worsening arterial stenosis. There are no specific therapy protocols for varicella-associated stroke in children, and the use of steroids or antiviral drugs is still controversial. We present a series of 4 children with stroke following varicella infection, with no recurrence and stable vascular stenosis at a mean follow-up of 18 months without steroid treatment. We also analyze possible correlations between anti-protein C, protein S and protein Z autoantibodies, and post-varicella arteriopathy.


Asunto(s)
Varicela/complicaciones , Varicela/tratamiento farmacológico , Herpesvirus Humano 3/fisiología , Esteroides/uso terapéutico , Accidente Cerebrovascular/virología , Antivirales/uso terapéutico , Autoanticuerpos/inmunología , Varicela/inmunología , Varicela/virología , Preescolar , Femenino , Humanos , Masculino , Recurrencia , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/inmunología , Resultado del Tratamiento
18.
J Child Neurol ; 26(3): 361-5, 2011 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-21273507

RESUMEN

Polymicrogyria (involving or not the sylvian scissure) with cerebellar cortical dysplasia or vermis hypoplasia has been reported in few cases. In addition, the association between ectopic neurohypophysis and other cortical malformations, including bilateral perisylvian polymicrogyria, has been documented. We describe a girl affected by focal epilepsy since the age of 2 years. Magnetic resonance imaging (MRI) at 11 and 22 years of age showed bilateral perisylvian polymicrogyria, dysplasia of the left cerebellar hemisphere, and ectopic neurohypophysis. Genetic tests, including fluorescent in situ hybridization 22q11.2 and array-comparative genomic hybridization, and pituitary hormones (at the age of 20 years) were normal. The patient is now 22 years old, and she is seizure free under therapy with lamotrigine and levetiracetam. To the best of our knowledge, this is the first description of this complex cerebral malformation. This finding confirms that bilateral perisylvian polymicrogyria can be associated with other cerebral malformations; cerebellum and neurohypophysis must be carefully evaluated in patients with polymicrogyria.


Asunto(s)
Malformaciones del Desarrollo Cortical/complicaciones , Neurohipófisis/patología , Anomalías Múltiples , Femenino , Humanos , Discapacidad Intelectual/complicaciones , Estudios Longitudinales , Adulto Joven
19.
J Child Neurol ; 25(8): 1024-8, 2010 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-20299698

RESUMEN

Fibrocartilaginous embolization is a rare cause of ischemic myelopathy caused by embolization of intersomatic disk nucleus pulposus into spinal vasculature during Valsalva-like maneuvers. Diagnostic criteria are based on patient's clinical history, magnetic resonance evidence of T2-hyperintense spinal cord lesion, and exclusion of other causes of ischemic myelopathy. These criteria do not take into account the development of magnetic resonance techniques able to enhance signal abnormalities within the neighboring intersomatic disc or vertebral body and to early characterize central nervous system lesions according to the presence of cytotoxic edema. We present 2 pediatric cases of progressive paraplegia attributed to fibrocartilaginous embolization in which short-tau inversion recovery and diffusion-weighted imaging sequences played a pivotal role showing the ischemic nature of spinal cord lesions. Due to its specificity, diffusion-weighted imaging should be included in the magnetic resonance criteria of fibrocartilaginous embolization and in standard magnetic resonance analysis when dealing with acute transverse myelopathy.


Asunto(s)
Imagen de Difusión por Resonancia Magnética/métodos , Embolia/patología , Fibrocartílago/patología , Disco Intervertebral/patología , Isquemia de la Médula Espinal/patología , Médula Espinal/patología , Adolescente , Niño , Embolia/etiología , Embolia/fisiopatología , Femenino , Fibrocartílago/fisiopatología , Humanos , Disco Intervertebral/fisiopatología , Masculino , Vías Nerviosas/irrigación sanguínea , Vías Nerviosas/patología , Vías Nerviosas/fisiopatología , Valor Predictivo de las Pruebas , Pronóstico , Médula Espinal/irrigación sanguínea , Médula Espinal/fisiopatología , Isquemia de la Médula Espinal/etiología , Isquemia de la Médula Espinal/fisiopatología
20.
J Child Neurol ; 25(6): 748-51, 2010 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-19808992

RESUMEN

Several studies on opiates demonstrated that selected brain areas as cerebellum and limbic system have the greatest density of opioid receptors. Recently, few cases of severe cerebellitis following methadone poisoning have been reported in children. We present the case of a 30-month-old girl who developed a delayed encephalopathy after methadone intoxication. She was admitted to our emergency department in coma, and after naloxone infusion, she completely recovered. Five days after intoxication, she developed psychomotor agitation, slurred speech, abnormal movements, and ataxia despite a negative neuroimaging finding. A repeat magnetic resonance imaging (MRI) performed 19 days after the intoxication for persistent symptoms showed signal abnormalities in the temporomesial regions, basal ganglia, and substantia nigra. To our knowledge, this is the first report of these delayed MRI findings associated with synthetic opioid intoxication.


Asunto(s)
Encéfalo/patología , Encefalitis/patología , Metadona/envenenamiento , Síndromes de Neurotoxicidad/patología , Encéfalo/efectos de los fármacos , Preescolar , Encefalitis/inducido químicamente , Femenino , Humanos , Imagen por Resonancia Magnética , Examen Neurológico , Recurrencia
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA