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1.
Rev. argent. neurocir ; 30(3): 103-107, ago. 2016. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-982822

RESUMO

Introducción: El cierre de cuero cabelludo en neurocirugías supratentoriales mediante sutura intradérmica es una técnica poco difundida en la actualidad. En contraste, es común que se efectúen suturas de alta tensión con el fin de evitar dehiscencias, fístula de líquido cefalorraquídeo (LCR) y sangrado durante el período posquirúrgico, pero que no tienen buen resultado estético. Objetivo: Comparar las complicaciones relacionadas al cierre de cuero cabelludo que se presentaron en neurocirugías supratentoriales en un período de 19 meses, para determinar si utilizar un cierre de menor tensión y con mejor resultado estético, es igual de seguro que las técnicas de cierre habituales. Materiales y Métodos: Se incluyeron 84 casos de neurocirugías supratentoriales electivas. En 43 casos (control) se realizó el cierre mediante sutura continua tipo surget y en 41 casos (experimental) el cierre fue mediante sutura intradérmica. Resultados: Se presentaron 2 casos de infecciones, 1 en cada grupo (p= 0,97) y 1 caso de sangrado en el grupo control (p= 0,32). No se presentaron dehiscencias ni casos de fístula de LCR. Conclusiones: El cierre de cuero cabelludo mediante sutura intradérmica fue un método seguro que no generó una mayor incidencia de complicaciones y logró un mejor resultado estético a corto y largo plazo.


Background: Scalp closure by means of intradermal suture in patients who underwent supratentorial neurosurgeries is currently an uncommon technique. In contrast, it is usual to perform high tension sutures to avoid skin dehiscence, cerebrospinal fluid leak (CSF) and postoperative bleeding; however it usually results in unsatisfactory aesthetic results. Aim: Comparing complications, over a two year period, related to scalp closure occurred in supratentorial neurosurgeries. The goal is to establish if the intradermal suture is as safe as the current high tension closure technique.Materials and Methods: Eighty-four patients were included. In forty-three cases (control) continuous high tension suture (surget) was performed. The other forty-one cases (experimental) underwent intradermal suture. Results: Two infection cases occurred, one from each group (p= 0, 97). There was one case of bleeding that took place in the control group (p= 0,32). Neither dehiscence nor CSF leak occurred in any of the trial cases.Conclusions: Intradermal suture performed for scalp closure was as safe as surget in terms of complications occurrence and obtained better cosmetic results in the short and long term.


Assuntos
Humanos , Líquido Cefalorraquidiano , Fístula , Neurocirurgia , Couro Cabeludo , Couro Cabeludo/cirurgia , Cirurgia Plástica , Infecção da Ferida Cirúrgica
2.
Rev. argent. neurocir ; 29(4): 159-163, nov. 2015. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-996548

RESUMO

OBJETIVO: Presentar nuestra experiencia en el manejo de los cavernomas de tronco cerebral, de tálamo y de ganglios basales. MATERIAL Y MÉTODO: Analizamos una serie de 16 pacientes asistidos en nuestra Institución, entre enero de 1990 y diciembre del año 2013. De ellos, 9 fueron varones y 7 mujeres. El rango de edad osciló entre 3 y 61 años. RESULTADOS: Siete debutaron con hemorragia cerebral, de ellos 4 se localizaban en protuberancia y 3 en el bulbo raquídeo. Siete pacientes tuvieron cavernomas múltiples, de ellos 3 tenían familiares con la misma enfermedad. El procedimiento diagnóstico de elección fue la resonancia nuclear magnética de cerebro en todos, y en los pacientes con cavernomas múltiples se completó el estudio con resonancia nuclear magnética de médula espinal. El tratamiento fue conservador en 9 enfermos, quirúrgico en 6 y radiocirugía estereotáctica en 1 enfermo; a éste paciente hubo necesidad de operarlo 6 meses después del tratamiento radiante por un resangrado voluminoso en la protuberancia. DISCUSIÓN: La cirugía es exitosa cuando el cavernoma se ubica a 2 mm de la piamadre, o del epéndimo. La radiocirugía puede ser causante de resangrado y de mayor volumen que las hemorragias previas. Por último, el tratamiento conservador sigue teniendo vigencia en los pacientes que se recuperaron neurológicamente y cuando se ubican en la profundidad del tronco cerebral, tálamo óptico o ganglios basales. CONCLUSIÓN: Cada paciente debe evaluarse individualmente para decidir el tipo de tratamiento, teniendo en cuenta la edad, la recuperación de los signos neurológicos, el volumen y la localización precisa del cavernoma


Objective: To present our experience in the management of brainstem, thalamus and basal ganglia cavernous malformations. Material and Method: We analyzed a series of 16 patients admitted to our Institution between January 1990 and December 2013. Nine of them were male and 7 female. Age ranged between 3 and 61. Results: Seven patients presented brainstem hemorrhage, 4 being pontine and the remaining 3 were medullary. Seven patients had multiple cavernomas, and 3 of them had a family background with the disease. The chosen diagnostic procedure was brain MRI in all patients; in patients with multiple cavernomas spine MRI was also requested. Nine patients received conservative treatment, 6 patients underwent surgery and one was treated with stereotactic radiosurgery but had to be operated on six months after radiation treatment due to voluminous re-bleeding at protuberance. Discussion: Surgery is successful when the cavernous malformation is placed 2 mm away from pia mater or ependyma. Radiosurgery can cause re-bleeding and of a greater volume than previous hemorrhages. Finally, conservative treatment is useful in patients who get neurologically recovered and when malformations are placed deep in brainstem, optic thalamus or basal ganglia. Conclusion: Each patient has to be individually assessed to individually assessed to make a decision regarding the type of treatment, taking into account age, recovery of neurological signs, volume, and precise location of cavernous malformation


Assuntos
Humanos , Tálamo , Gânglios da Base , Radiocirurgia , Malformações Vasculares
4.
Neurologia ; 28(9): 550-7, 2013.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-23274119

RESUMO

INTRODUCTION: Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. OBJECTIVE: The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. MATERIAL AND METHODS: We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. RESULTS: Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to-average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. CONCLUSIONS: Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.


Assuntos
Esclerose Tuberosa/patologia , Transtorno Autístico/etiologia , Transtorno Autístico/fisiopatologia , Transtorno Autístico/psicologia , Encéfalo/patologia , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Humanos , Lactente , Deficiência Intelectual/fisiopatologia , Deficiência Intelectual/psicologia , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Convulsões/etiologia , Convulsões/fisiopatologia , Convulsões/psicologia , Esclerose Tuberosa/fisiopatologia
5.
Neurologia ; 27(8): 472-80, 2012 Oct.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-22217526

RESUMO

INTRODUCTION: The term focal cortical dysplasia (FCD) describes a particular migration disorder with a symptomatology mainly characterised by drug-resistant epileptic seizures, typical neuroradiological images, and histological characteristics, as well as a very positive response to surgical treatment in the majority of cases. MATERIAL AND METHODS: A total of 7 patients were studied, comprising 6 children with a mean age of 34.3 months and one 25-year-old male with very persistent focal seizures and MRI images that showed FCD. RESULTS: Three of the patients (all girls) were operated on while very young, with extirpation of the FCD and the surrounding area; with the histopathology study showed agreement between the MRI images and the macroscopic study of the slices. The histology study showed findings typical of a Taylor-type FCD (poor differentiation between the cortical grey matter and the subcortical white matter, and balloon cells). Three years after the FCD extirpation, the same 3 patients remained seizure-free with no anti-epilepsy medication. Two others have seizure control with medication, another (the adult) is on the surgical waiting list, and the remaining patient refused the operation. CONCLUSION: Taylor-type FCD is associated with a high percentage of all drug-resistant focal seizures, and it needs to be identified and extirpated as soon as possible. Well planned and well-performed surgery that leaves no remains of dysplasia can cure the disease it in many cases.


Assuntos
Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Malformações do Desenvolvimento Cortical/psicologia , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Lactente , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/cirurgia , Procedimentos Neurocirúrgicos , Tomografia por Emissão de Pósitrons , Radiografia , Convulsões/etiologia , Tomografia Computadorizada de Emissão de Fóton Único , Resultado do Tratamento , Adulto Jovem
6.
Neurologia ; 27(8): 491-9, 2012 Oct.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-21890242

RESUMO

OBJECTIVE: To present 16 patients with schizencephaly and neurological involvement, and analyse their characteristics and neuroimages. MATERIAL AND METHODS: The study included 16 patients, 8 males and 8 females, all of whom were diagnosed with schizencephaly at less than 3 years of age; 2 patients were diagnosed prenatally. Schizencephaly was identified by computerized tomography (CT) in 1 patient and by MR or three-dimensional MR (3DMR) with a 1.5tesla apparatus in the others. Most patients were referred for evaluation because of psychomotor delay, motor disabilities and/or seizures. RESULTS: Five patients had bilateral schizencephaly with open lips (2 of them had suffered intrauterine cytomegalovirus infections); 2 showed unilateral schizencephaly with separated lips, 8 presented unilateral schizencephaly with fused lips, and 1 had schizencephaly with open lips on one side and fused lips on the other. Prenatal cytomegalovirus infection was diagnosed in 2 patients. A cerebral malformation that affected the midline was diagnosed by routine ultrasound studies in 2 patients. Eight patients (50%) presented with seizures that were focal, except for one patient who showed secondary generalisation. The latter was the only patient whose disease was refractory to complete seizure control with antiepileptic medication. All patients had some degree of motor deficit, which was either unilateral (hemiparesis) or bilateral (tetraparesis). CONCLUSION: 3D MR imaging was very important in diagnosing of schizencephaly in our patients because it showed the polymicrogyria that covered the area of the cleft and permitted us to rule out porencephaly. Neuronal migration disorders such as heterotopias and, more frequently, cortical dysplasias, were observed in several patients. Half of the patients had epilepsy which was controlled with antiepileptic medication, except in 1 patient.


Assuntos
Malformações do Desenvolvimento Cortical/patologia , Pré-Escolar , Deficiências do Desenvolvimento/etiologia , Eletroencefalografia , Feminino , Cabeça/anatomia & histologia , Humanos , Processamento de Imagem Assistida por Computador , Imageamento Tridimensional , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/psicologia , Tomografia Computadorizada por Raios X
8.
Magn Reson Med ; 65(2): 329-39, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20939087

RESUMO

Proton magnetic resonance spectroscopic imaging ((1) H-MRSI) has been advocated as a valuable tool for prostate cancer diagnosis. However, a barrier to widespread clinical use of this technique is the lack of robust quantification methods that yield reproducible results in an institution-independent manner. The main goal of this study was to develop a standardized and fully automated approach (LCModel-based) for quantitative prostate (1) H-MRSI. To this end, a dedicated basis set was constructed by the combination of simulated (citrate, Cit; choline, Cho, and creatine, CR) and experimentally acquired (spermine, Spm) spectra. The overlapping Spm, Cho, and Cr could be resolved and quantified individually, thus allowing for the independent assessment of glandular (Cit and Spm) and proliferative (Cho) components. Several metabolite ratios were calculated and compared to the histologic findings of prostatectomy specimens from 10 prostate cancer patients with Gleason scores (3 + 3) and (3 + 4). The Cho mole fraction and the Cho/(Cit + Spm) ratio were found to best discriminate between prostate cancer and healthy tissue. The comparison between the quantitative MRSI results and the histologic findings suggests that no correlation exists between the detected metabolic alterations and the Gleason score of low-grade tumors.


Assuntos
Adenocarcinoma/metabolismo , Espectroscopia de Ressonância Magnética , Próstata/metabolismo , Próstata/patologia , Neoplasias da Próstata/metabolismo , Neoplasias da Próstata/patologia , Adenocarcinoma/patologia , Colina/análise , Ácido Cítrico/análise , Creatina/análise , Humanos , Técnicas In Vitro , Masculino , Imagens de Fantasmas , Espermina/análise
9.
Neuroradiol J ; 24(4): 577-87, 2011 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-24059716

RESUMO

We review several clinical and radiological entities that present with abnormalities of the spinal dura that may be related to each other. These include: the syndrome of spontaneous intracranial hypotension, neuroenteric cysts, spontaneous spinal cord herniation, degenerative disc disease associated with disc clefts or associated with intradural complications, and Hirayama disease. Our observations focus on common imaging findings of dural tears, abnormal peridural fluid and associated degenerative disc disease. We propose that T2 fat sat sequences should be routinely used in spinal imaging protocols to evaluate extradural fluid.

10.
Neurologia ; 25(5): 314-21, 2010 Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-20643042

RESUMO

OBJECTIVE: Presentation of 8 patients with subependymal giant-cell astrocytomas (SGCA) associated with tuberous sclerosis complex (TSC). MATERIAL AND METHODS: There are 8 patients, 6 males and 2 females with TSC, who presented with the tumour between the neonatal period and 24 years. RESULTS: All patients showed bilateral hypersignalised areas in zones close to the foramen of Monro. Three of the patients were admitted urgently due to blindness and increased intracranial pressure. Incomplete removal of the tumour has always been bad solution as it resulted in the death of the patient (in one case) or further surgery operation in the short term. Only one patient developed the tumour suddenly from pre-existing subependymal nodules from the childhood and they had to be removed at 24 years of age. By contrast, 32 patients with TSC and images of subependymal nodules whose CT or MR progress was followed up for between 10 and 30 years did not develop a tumour. One patient had to be operated four times over 20 years. CONCLUSIONS: SGCA associated with TSC is a severe complication which as likely to develop and careful monitoring is required from neonatal age with periodicclinical and imaging studies in order to avoid its irreversible complications. Hydrocephaly, blindness and even the death can be the main consequences. Reintervention of the recurrent tumour is often necessary.


Assuntos
Astrocitoma/etiologia , Astrocitoma/patologia , Neoplasias Encefálicas/etiologia , Neoplasias Encefálicas/patologia , Esclerose Tuberosa , Adolescente , Astrocitoma/cirurgia , Neoplasias Encefálicas/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Esclerose Tuberosa/complicações , Esclerose Tuberosa/patologia , Adulto Jovem
11.
Neuropediatrics ; 40(3): 148-51, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-20020403

RESUMO

Conventional arteriography in an 11-month-old boy with cardiopathy, aortic arch coarctation and haemangiomas showed the absence of the right internal carotid and vertebral arteries, hypertrophy of the right external carotid artery, with enlargement of the internal maxillary and ophthalmic arteries that supplied the right cerebral hemisphere. An MRI study showed an infarcted area in the posterior zone of the left cerebral hemisphere vascularised by the middle cerebral artery that was caused by a thrombosis during a severe bout of gastroenteritis. MRA studies performed at 16 and 23 years of age revealed progressive narrowing of the left carotid and vertebral arteries, persistence of the proatlantal and trigeminal arteries, and poor cerebral vascularisation that, at adult age, was entirely supplied through collateral arteries, branches of both external carotids, the presence of unilateral duplication of the vertebral arteries and ascending pharyngeal artery.


Assuntos
Anormalidades Cardiovasculares/complicações , Transtornos Cerebrovasculares/complicações , Hemangioma/congênito , Neoplasias Cutâneas/complicações , Angiografia/métodos , Angiografia Cerebral/métodos , Transtornos Cerebrovasculares/diagnóstico por imagem , Transtornos Cerebrovasculares/patologia , Humanos , Lactente , Estudos Longitudinais , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética/métodos , Masculino , Adulto Jovem
12.
Neurologia ; 24(7): 457-61, 2009 Sep.
Artigo em Espanhol | MEDLINE | ID: mdl-19921555

RESUMO

OBJECTIVE: The aim of this paper is to present the cases of 7 young children and young adult with type 2 neurofibromatosis (NF2). MATERIAL AND METHODS: Seven patients, 5 females and 2 males, aged 2 to 33 years, were studied by intracranial and spinal magnetic resonance (MRI) and clinically. In 5 patients, the symptoms because around the time of the diagnosis while 2 patients, who had no previous symptoms, were diagnosed by MRI after undergoing the test because a parent had been diagnosed of NF2 by MRI. RESULTS: All 7 patients had bilateral vestibular schwannomas (VS) and only two had no associated intracranial and/or spinal tumors. Four patients had intracranial meningiomas, mainly located in the lesser wing of the temporal fossa and in the falx, and 5 had spinal cord tumors (ependymomas and meningiomas). An attempt to made to remove the VS in all but one case, however, it was not possible to remove completely the tumors in any case. Deafness was the common sequel in all operated cases, associated with permanent bilateral facial paralysis in one and unilateral facial parenthesis in another. CONCLUSION: A comparison of our series of NF1 and NF2 cases shows that the ratio of NF2:NF1 in childhood is approximately 1:100, and that the clinical features of NF2 are considerably more severe than in NF1.


Assuntos
Neurofibromatose 2/patologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/genética , Neurofibromatose 1/patologia , Neurofibromatose 2/diagnóstico , Neurofibromatose 2/genética , Adulto Jovem
14.
Rev Neurol ; 47(8): 399-403, 2008.
Artigo em Espanhol | MEDLINE | ID: mdl-18937200

RESUMO

INTRODUCTION: It is known as segmental neurofibromatosis type 1 (NF1) a type of NF1 characterized by the features circumscribed to one or more body cutaneous and/or subcutaneous segments. This entity is recognized from recently and it is related with somatic mosaicism. PATIENTS AND METHODS: 43 patients (29 females and 14 males) with ages below 16 years were retrospectively studied. Image study of the affected region of the body was performed in all patients to discard a subjacent organic disease, and a neurofibroma was histologically demonstrated in some cases. Somatic or gonosomal mosaicism was not investigated in any of the patients. RESULTS: Only 8 patients showed cutaneous lesion--7 with café-au-lait spots (3 of freckles type, 4 of large spots, of which, 3 were bilateral and 1 unilateral ) and 1 presented neurofibromas-. The other cases (81%) had cutaneous lesion with subjacent lesion (neurofibromas and bone dysplasia in most cases). The subcutaneous lesions were seen in all parts of the body without a preferent location. In cases with only cutaneous lesion, the clinical features were seen on the trunk skin. CONCLUSION: Segmental NF1 is considered to be the result of a somatic or gonosomal mosaicism and still is underdiagnosed. Features of segmental NF1 can be found in as many regions of the body as NF1 without mosaicism. The types of segmental NF1 that were seen less frequently in this series were those with only cutaneous features (café-au-lait spots and neurofibromas). Types with subcutaneous features that involved subjacent organs were seen in 81% of patients. Familial patients, with NF1 or segmental NF1 was shown in 15 patients (35%) and bilateral lesion in 4 cases (9.3%).


Assuntos
Neurofibromatoses/patologia , Adolescente , Manchas Café com Leite/genética , Manchas Café com Leite/patologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Mosaicismo , Neurofibromatoses/genética , Estudos Retrospectivos , Pele/patologia
16.
Rev Neurol ; 46(9): 530-6, 2008.
Artigo em Espanhol | MEDLINE | ID: mdl-18446694

RESUMO

PATIENTS AND METHODS: From a series of 530 patients with neurofibromatosis type 1 (NF1), we performed a retrospective assessment of the long-term neurologic, visual, neuroimaging and evolution of 80 patients (15%) with optic pathway gliomas (OPG). All the 80 patients, 58 (72.5%) females and 22 (27.5%) males were diagnosed during childhood (below age 16 years), range 13 months to 15 years (average: 4.6 years). RESULTS: Image studies showed the distribution of the lesions among optic nerves, chiasm, tracts and radiations demonstrated that only 25% of the tumors involved only one optic nerve and 11.5% were located only in the chiasm, while 40% involved one or both optic nerves and chiasm, tracts and radiations. Two patients showed pilocytic astrocytoma in the histological study. Late diagnosis (after 7 years of age) of OPG was made in three patients and late progression was evident in three others who required surgical resection, radiotherapy or chemotherapy. CONCLUSIONS: All patients were diagnosed during childhood (below 16 years of age). Incidence was double in girls than in boys. Despite the apparent tumoral agressivity of the magnetic resonance and magnetic resonance spectroscopy images, histological findings corresponded to benign pilocytic astrocytoma. Some tumors follow the growth after 7 years. Continued monitoring of patients with NF1 into adulthood is advisable.


Assuntos
Neoplasias Primárias Múltiplas/diagnóstico , Neurofibromatose 1/diagnóstico , Glioma do Nervo Óptico/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos
17.
Neurologia ; 23(2): 114-8, 2008 Mar.
Artigo em Espanhol | MEDLINE | ID: mdl-18322831

RESUMO

OBJECTIVE: To present a left-handed patient who had an acute encephalopathy, possibly of viral etiology, followed by remote and recent memory loss, several types of apraxia and emotional disturbance, without any motor abnormalities. He had cerebral lesions that involved both temporal lobes and other brain regions. All features corresponded to the Klüver-Bucy syndrome. After a seven year follow-up, no improvement of the neurological and neuroradiological, mainly by magnetic resonance imaging (MRI) features was observed. CASE REPORT: A 14 year-old left-handed boy suffered sudden onset of fever (40.4 degrees C), headache, vomiting, focal and generalized seizures and coma. After the acute illness, the patient had severe neurological sequels consisting in total loss of memory without any capacity to recognize persons (including family members) and remote events and he was not capability of remember hardly anything that he was taught after his disease, these alterations continued almost completely during the seven years (from 14 to 21 years) that we followed him up. The only abilities that he conserved in similar conditions to those prior to his acute disease were his capacity to swim (including the style of jumping into the water), bike riding, playing football, dominoes, cards, etc., which he had learned during his childhood, to say the numbers and the alphabet letters rapidly by heart (without knowing them) and to avoid cars on the street. MRI showed post-inflammatory lesions in the temporal and the parieto-temporo-occipital regions bilaterally (cortical and subcortical regions) and in the left occipital region. He presented almost all types of apraxia. CONCLUSION: Klüver-Bucy syndrome, which can be secondary to more than 50 different causes, not only presents remote memory loss but also recent memory loss as in our patient, who appeared to be isolated from the surrounding world. Motor function and automated activities learned before the acute brain illness were not affected and could be recovered. Etiology, location and extent of the anatomic brain lesion appear to be the most important prognostic conditions.


Assuntos
Síndrome de Kluver-Bucy/diagnóstico , Síndrome de Kluver-Bucy/fisiopatologia , Adolescente , Adulto , Apraxias/etiologia , Apraxias/fisiopatologia , Seguimentos , Humanos , Síndrome de Kluver-Bucy/patologia , Masculino , Transtornos da Memória/etiologia , Transtornos da Memória/fisiopatologia , Testes Neuropsicológicos , Prognóstico
18.
Neuropediatrics ; 39(6): 341-3, 2008 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19568998

RESUMO

We present a 3-month-old girl who showed segmental NF1 and Cobb syndrome. She has a cutaneous vascular malformation located on the middle T (4)-T (6) region superimposed on a giant cutaneous café-au-lait spot. Magnetic resonance arteriography (MRA) revealed bilateral renal artery stenosis, extensive hypertrophy of the spinal epidural venous plexus, coarctation and tubular hypoplasia of the aortic arch and proximal portion of descending aorta. To the best of our knowledge the association of both neurocutaneous disorders has not being previously described.


Assuntos
Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas Intracranianas/diagnóstico , Neurofibromatose 1/diagnóstico , Pele/irrigação sanguínea , Coartação Aórtica/diagnóstico , Coartação Aórtica/genética , Malformações Arteriovenosas/genética , Pré-Escolar , Feminino , Humanos , Lactente , Malformações Arteriovenosas Intracranianas/genética , Angiografia por Ressonância Magnética , Doença de Moyamoya/diagnóstico , Doença de Moyamoya/genética , Neurofibromatose 1/genética , Síndrome
19.
AJNR Am J Neuroradiol ; 28(8): 1609-12, 2007 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17846222

RESUMO

BACKGROUND AND PURPOSE: The association of cortical organization disorders with facial hemangiomas or vascular malformations has been described in only a few reports. The purpose of this study was to show the close association of these cutaneous anomalies with cortical dysplasias and intracranial vascular abnormalities. MATERIALS AND METHODS: Five patients, all women, with cutaneous vascular abnormalities, 4 with hemangioma and 1 with vascular malformation, were studied with MR and MR angiography. RESULTS: All 5 of the patients showed cortical dysplasia. The cutaneous lesions involved the left frontal region, ipsilateral to the cerebral hemisphere with cortical dysplasia, in all of the patients. Four patients had seizures that responded well to antiepileptic drugs. Hemispheric hypoplasia was associated with the cortical dysplasia in all 5 of the patients. Arterial abnormalities were found in all of the patients, consisting of aplasia of the ipsilateral internal carotid artery in 2, persistence of the trigeminal artery in 2, persistence of both proatlantal arteries and double kinking in the internal carotid artery in 1, and origin of both anterior cerebral arteries from the same internal carotid in all 5 of the patients, 1 of whom also showed an intracavernous anterior cerebral artery origin of the same side of the hemispheric hypoplasia and polymicrogyria. Seizures and mild psychomotor delay could be caused by the cortical dysplasia and the hemispheric hypoplasia. CONCLUSIONS: The presence of many congenital vascular abnormalities in this series suggests that facial hemangioma and vascular malformations may be in close relationship with cortical and vascular abnormalities. The reason that the vascular and cortical abnormalities occurred in the left side in all 5 of the patients and the mechanism underlying the association of both malformations are unclear. A genetic origin is suggested.


Assuntos
Encéfalo/anormalidades , Neoplasias Faciais/complicações , Hemangioma/complicações , Adolescente , Encéfalo/patologia , Artérias Carótidas/anormalidades , Artérias Cerebrais/anormalidades , Córtex Cerebral/anormalidades , Córtex Cerebral/patologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética
20.
Neurologia ; 22(10): 846-52, 2007 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-17671854

RESUMO

OBJECTIVE: To describe the clinical and imaging findings of 20 patients (12 women and 8 men) with brain stem tumors associated with neurofibromatosis type 1 (NF1). PATIENTS AND METHODS: All patients were first time studied before 11 years old. Clinical and magnetic resonance (MR) study were made in all 20 patients, and spectroscopic MR (SMR) was performed in 7 patients. Thirteen of the 20 patients (65 %) also had optic pathway tumor. Brain stem tumor identification occurred at the same time as NF1 in the patients who were studied by MR at the time of the first consult. RESULTS: Brain stem identification occurred at the same time as that of the NF1 in patients who were studied by MR from the beginning. Diffuse or localized medullary enlargement was the most frequent MR imaging and appeared in 13 patients (65%), followed by the tumor that involved all brain stem (pontine and medullary areas) that appeared in 6 patients (30 %). In the last group, one tumor showed extension through brain stem and medial cerebellar parts, another was located in the aqueduct and in the periaqueductal areas and showed slow progressive growth, and one third patient had a tumor with aggressive signs in the SMR study. Another patient had an aggressive tumor that involved the left optic nerve, chiasm, mesencephalon and upper right pontine areas. The histological study of the tumoral biopsic tissue of the two last patients showed astrocitoma degree 1 (benign tumor). The two aggressive tumors were treated with radiotheraphy and chemotherapy and they are still alive 4 and 7 years respectively after treatment. Three patients who had aqueductal obstruction and hydrocephalus were treated with shunt. The rest of patients did not receive treatment. Only one of the 20 patients died, although it was due to a malignant chiasmatic tumor, that had been treated twenty years before, and not by the brain stem tumor. CONCLUSIONS: In NF1, brain stem tumors are the most frequent tumors of the posterior fossa and the second most frequent of the central nervous system (CNS). MR and SMR are necessary to a correct identification of the tumor in some patients. Most of these tumors are benign.


Assuntos
Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico , Tronco Encefálico , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Neurofibromatose 1/complicações , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
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