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1.
Clin Neuroradiol ; 27(2): 199-203, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26350588

RESUMO

PURPOSE: Intra-arterial (IA) administration of nimodipine has been shown to be an effective treatment for subarachnoid hemorrhage-related cerebral vasospasm. The concentrations achieved in cerebral arteries during this procedure, though, are unknown. Therefore, there are no clinical studies investigating dose-dependent effects of nimodipine. We aimed at providing a pharmacokinetic model for IA nimodipine therapy for this purpose. METHODS: A two-compartment pharmacokinetic model for intravenous nimodipine therapy was modified and used to assess cerebral arterial nimodipine concentration during IA nimodipine infusion into the internal carotid artery (ICA). RESULTS: According to our simulations, continuous IA nimodipine infusion at 2 mg/h and 1 mg/h resulted in steady-state cerebral arterial concentrations of about 200 ng/ml and 100 ng/ml assuming an ICA blood flow of 200 ml/min and a clearance of 70 l/h. About 85 % of the maximal concentration is achieved within the first minute of IA infusion independent on the infusion dose. Within the range of physiological and pharmacokinetic data available in the literature, ICA blood flow has more impact on cerebral arterial concentration than nimodipine clearance. CONCLUSION: The presented pharmacokinetic model is suitable for estimations of cerebral arterial nimodipine concentration during IA infusion. It may, for instance, assist in dose-dependent analyses of angiographic results.


Assuntos
Modelos Cardiovasculares , Nimodipina/administração & dosagem , Nimodipina/farmacocinética , Hemorragia Subaracnóidea/tratamento farmacológico , Hemorragia Subaracnóidea/metabolismo , Vasoespasmo Intracraniano/tratamento farmacológico , Vasoespasmo Intracraniano/metabolismo , Simulação por Computador , Humanos , Injeções Intra-Arteriais , Taxa de Depuração Metabólica , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Hemorragia Subaracnóidea/etiologia , Vasodilatadores/administração & dosagem , Vasodilatadores/farmacocinética , Vasoespasmo Intracraniano/complicações
2.
Clin Neuroradiol ; 22(1): 39-45, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22138815

RESUMO

The patency of a bypass plays an important role in the postoperative recovery of patients especially when dealing with complicated intracranial aneurysms. In this study two-dimensional phase contrast magnetic resonance angiography (PC-MRA) was used to measure cerebral blood flow in 23 patients before extracranial-intracranial high-flow bypass surgery using the excimer laser-assisted non-occlusive anastomosis (ELANA) technique and in 15 patients following surgery. The results showed that PC-MRA is a suitable technique for assessing bypass patency and that with the ELANA technique the bypass has the capability of compensating the blood flow of an occluded internal carotid artery (ACI) in cases of complex aneurysms.


Assuntos
Circulação Cerebrovascular , Interpretação de Imagem Assistida por Computador/métodos , Aneurisma Intracraniano/fisiopatologia , Aneurisma Intracraniano/cirurgia , Lasers de Excimer/uso terapêutico , Angiografia por Ressonância Magnética/métodos , Veia Safena/transplante , Velocidade do Fluxo Sanguíneo , Feminino , Humanos , Aumento da Imagem/métodos , Aneurisma Intracraniano/diagnóstico , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Reologia/métodos , Sensibilidade e Especificidade , Resultado do Tratamento
3.
Radiologe ; 50(8): 706-10, 2010 Aug.
Artigo em Alemão | MEDLINE | ID: mdl-20652214

RESUMO

The case of an adolescent female patient with acute lymphoblastic leukemia and stroke-like hemiparesis demonstrates a typical manifestation of methotrexate-induced acute encephalopathy. This rare entity occurs both in children and adults and can result from intrathecal as well as high dose intravenous administration of methotrexate. Diagnosis can confidently be made using cerebral MRI including diffusion-weighted imaging (DWI), so that patients can be informed about the favorable prognosis.


Assuntos
Antimetabólitos Antineoplásicos/efeitos adversos , Metotrexato/efeitos adversos , Síndromes Neurotóxicas/diagnóstico , Paresia/induzido quimicamente , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamento farmacológico , Adolescente , Antimetabólitos Antineoplásicos/administração & dosagem , Encéfalo/efeitos dos fármacos , Encéfalo/patologia , Citarabina/administração & dosagem , Imagem de Difusão por Ressonância Magnética , Relação Dose-Resposta a Droga , Feminino , Humanos , Infusões Intravenosas , Injeções Espinhais , Metotrexato/administração & dosagem , Exame Neurológico/efeitos dos fármacos , Paresia/diagnóstico
4.
J Inherit Metab Dis ; 30(1): 5-22, 2007 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-17203377

RESUMO

Glutaryl-CoA dehydrogenase (GCDH) deficiency is an autosomal recessive disease with an estimated overall prevalence of 1 in 100 000 newborns. Biochemically, the disease is characterized by accumulation of glutaric acid, 3-hydroxyglutaric acid, glutaconic acid, and glutarylcarnitine, which can be detected by gas chromatography-mass spectrometry of organic acids or tandem mass spectrometry of acylcarnitines. Clinically, the disease course is usually determined by acute encephalopathic crises precipitated by infectious diseases, immunizations, and surgery during infancy or childhood. The characteristic neurological sequel is acute striatal injury and, subsequently, dystonia. During the last three decades attempts have been made to establish and optimize therapy for GCDH deficiency. Maintenance treatment consisting of a diet combined with oral supplementation of L: -carnitine, and an intensified emergency treatment during acute episodes of intercurrent illness have been applied to the majority of patients. This treatment strategy has significantly reduced the frequency of acute encephalopathic crises in early-diagnosed patients. Therefore, GCDH deficiency is now considered to be a treatable condition. However, significant differences exist in the diagnostic procedure and management of affected patients so that there is a wide variation of the outcome, in particular of pre-symptomatically diagnosed patients. At this time of rapid expansion of neonatal screening for GCDH deficiency, the major aim of this guideline is to re-assess the common practice and to formulate recommendations for diagnosis and management of GCDH deficiency based on the best available evidence.


Assuntos
Glutaril-CoA Desidrogenase/deficiência , Glutaril-CoA Desidrogenase/genética , Erros Inatos do Metabolismo/diagnóstico , Erros Inatos do Metabolismo/terapia , Criança , Pré-Escolar , Feminino , Glutaril-CoA Desidrogenase/metabolismo , Humanos , Lactente , Recém-Nascido , Espectrometria de Massas , Erros Inatos do Metabolismo/dietoterapia , Erros Inatos do Metabolismo/genética , Mutação , Triagem Neonatal , Fenótipo , Risco
5.
Neurology ; 67(4): 587-91, 2006 Aug 22.
Artigo em Inglês | MEDLINE | ID: mdl-16924009

RESUMO

BACKGROUND: The authors report a three-generation family with four male patients presenting with a novel type of X-chromosomal leukoencephalopathy associated with skeletal abnormalities. METHODS: The index patient and his brother reached their early motor milestones in due time and had normal language development. Between the ages of 2 and 3 years, first signs of spastic paraplegia were noticed. Furthermore, the patients developed tremor, ataxia, optic atrophy, and spastic tetraparesis. Both boys had broad wrists and knees without significant contractures. A maternal uncle and a granduncle had the same disease. RESULTS: Leukoencephalopathy (MRI, MRS) and metaphyseal chondrodysplasia (X-ray, MRI) were diagnosed. MRS showed a reduction of choline-containing compounds in the white matter. An autopsy on one of the patients, who died at age 37 years, revealed an orthochromatic type of leukoencephalopathy. In bone and cartilage tissue, unspecific signs of a mild chondrodysplasia were found. At the PLP gene locus an obligate recombination was observed, which excludes the Pelizaeus-Merzbacher locus on Xq21-22. However, affected males share a fragment of the long arm of chromosome X. CONCLUSION: The authors report a new type of leukoencephalopathy associated with metaphyseal chondrodysplasia located on Xq25-q27.


Assuntos
Encefalopatias/diagnóstico , Encefalopatias/genética , Cromossomos Humanos X/genética , Osteocondrodisplasias/diagnóstico , Osteocondrodisplasias/genética , Adulto , Encefalopatias/complicações , Mapeamento Cromossômico , Predisposição Genética para Doença/genética , Heterozigoto , Humanos , Masculino , Osteocondrodisplasias/complicações , Linhagem
6.
Neurology ; 64(8): 1411-6, 2005 Apr 26.
Artigo em Inglês | MEDLINE | ID: mdl-15851732

RESUMO

OBJECTIVE: To describe a distinctive syndrome of nonprogressive encephalopathy, normo- or microcephaly, and early onset of severe psychomotor impairment in 15 white patients, including two siblings and two first cousins. METHODS AND RESULTS: MRI revealed bilateral cysts in the anterior part of the temporal lobe and white matter abnormalities with pericystic abnormal myelination and symmetric lesions in frontal and occipital periventricular regions. None of the usual inborn errors of metabolism/infectious diseases associated with leukoencephalopathy and bilateral anterior temporal lobe cysts were detected. CONCLUSIONS: These patients' clinical signs and cranial MRI abnormalities are strikingly similar and may represent a distinctive disease with autosomal-recessive inheritance: cystic leukoencephalopathy without megalencephaly.


Assuntos
Encéfalo/patologia , Cistos do Sistema Nervoso Central/patologia , Malformações do Sistema Nervoso/patologia , Adolescente , Adulto , Idade de Início , Encéfalo/fisiopatologia , Cistos do Sistema Nervoso Central/fisiopatologia , Córtex Cerebral/patologia , Córtex Cerebral/fisiopatologia , Criança , Pré-Escolar , Deficiências do Desenvolvimento/patologia , Deficiências do Desenvolvimento/fisiopatologia , Progressão da Doença , Epilepsia/patologia , Epilepsia/fisiopatologia , Face/anormalidades , Feminino , Humanos , Deficiência Intelectual/patologia , Deficiência Intelectual/fisiopatologia , Transtornos do Desenvolvimento da Linguagem/patologia , Transtornos do Desenvolvimento da Linguagem/fisiopatologia , Imageamento por Ressonância Magnética , Masculino , Fibras Nervosas Mielinizadas/patologia , Malformações do Sistema Nervoso/fisiopatologia , Fenótipo , Quadriplegia/patologia , Quadriplegia/fisiopatologia , Síndrome , Turquia , População Branca
7.
J Clin Endocrinol Metab ; 88(10): 4590-5, 2003 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-14557427

RESUMO

The pathogenesis of central precocious puberty (PP) and/or gelastic seizures due to a hypothalamic hamartoma (HH) is still under debate. We evaluated the association of clinical symptoms with morphology and localization of the HH in 34 patients. The majority (86.4%) of HHs in patients with isolated PP (n = 22; 68.2% females) revealed a parahypothalamic position without affecting the third ventricle (91%). Half of them were pedunculated, and 40.9% showed a diameter less than 10 mm. In contrast, 11 of 12 patients with seizures, eight of whom were male, presented with a sessile intrahypothalamic hamartoma, 10 of which distorted the third ventricle. Logistic regression analysis revealed an increased relative risk (RR) for epilepsy in males (RR, 4.3; 95% confidence interval, 0.96-19). However, combination of the risk factor gender with intrahypothalamic position (RR, 19; 1.3-285) and distortion of the third ventricle (RR, 10; 0.6-164) reduced the risk associated with male gender to 1.1. The position of a HH and involvement of the third ventricle are likely to be more predictive for clinical characteristics than size and shape. Male gender was associated with an intrahypothalamic HH and epilepsy, suggesting a sexually dimorphic developmental pattern of this heterotopic mass.


Assuntos
Epilepsia Parcial Complexa/etiologia , Hamartoma/complicações , Doenças Hipotalâmicas/complicações , Puberdade Precoce/etiologia , Estatura , Criança , Pré-Escolar , Epilepsia Parcial Complexa/epidemiologia , Epilepsia Parcial Complexa/patologia , Estrogênios/sangue , Feminino , Gonadotropinas/sangue , Hamartoma/epidemiologia , Hamartoma/patologia , Humanos , Doenças Hipotalâmicas/epidemiologia , Doenças Hipotalâmicas/patologia , Lactente , Modelos Logísticos , Imageamento por Ressonância Magnética , Masculino , Puberdade Precoce/epidemiologia , Puberdade Precoce/patologia , Estudos Retrospectivos , Fatores de Risco , Distribuição por Sexo , Testosterona/sangue , Tomografia Computadorizada por Raios X
8.
Neuroradiology ; 42(9): 685-91, 2000 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-11071445

RESUMO

Our purpose was to evaluate the surgical and endovascular treatment outcomes of ruptured intracranial vertebral artery aneurysms (RIVAA). The outcomes of 44 patients with RIVAA treated between 1983 and 1998 surgically (26), endovascularly (20) or both (2) were evaluated. The aneurysms were clipped in 24 patients, and clipped and wrapped in two. We treated 20 by the endovascular approach, 12 with Guglielmi detachable coils (GDC), and eight by parent-vessel occlusion using detachable balloons. Three patients had endovascular treatment after a failed or inadequate surgical attempt. Posttreatment follow-up was 17-183 months (mean 101 months) for surgically treated patients. For the GDC-treated group angiographic follow-up was carried at 8-49 months (mean 19 months). The condition of seven (27 %) of the surgically treated patients worsened due to procedure-related complications, compared with 10% in the endovascular treatment group. Of the patients initially presenting with Hunt and Hess grade IV or V, three of five (60%) died who were treated surgically and two of eight (25%) who were treated endovascularly. A good outcome was achieved in 17 surgically treated patients (85% of the survivors) and in 16 of the endovascular group (89% of the survivors). This present "same-site" report on treatment of a specific abnormality, RIVAA, treated surgically or by an endovascular approach indicates that especially in poorer Hunt and Hess grade patients, the latter may offer a clinical outcome as good as that of surgery, although long-term efficacy of GDC treatment is still to be determined.


Assuntos
Aneurisma Roto/terapia , Oclusão com Balão/métodos , Aneurisma Intracraniano/terapia , Artéria Vertebral/cirurgia , Adolescente , Adulto , Idoso , Aneurisma Roto/diagnóstico , Aneurisma Roto/cirurgia , Angiografia Cerebral , Criança , Feminino , Humanos , Aneurisma Intracraniano/diagnóstico , Aneurisma Intracraniano/cirurgia , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Hemorragia Subaracnóidea/diagnóstico , Artéria Vertebral/diagnóstico por imagem
9.
J Child Neurol ; 14(4): 222-8, 1999 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-10334395

RESUMO

Metachromatic leukodystrophy refers to a group of genetic neurologic diseases caused by deficiencies of the enzyme arylsulfatase A and the resulting accumulation of sulfatides in white matter. Bone marrow transplantation has been advocated as a treatment in an attempt to correct the enzyme deficiency. Such a transplant was performed in 1991 in a 16-year-old girl with a form of late juvenile metachromatic leukodystrophy caused by a homozygous P426L mutation in the arylsulfatase A gene. Engraftment was prompt and resulted in constant enzymatic normalization of circulating lymphocytes. The elevated urinary excretion of sulfatides remained unaffected. Clinical findings up until transplantation consisted of gait disturbances, impairment of cognitive functioning, and deterioration in school performance over several years. During a 6-year follow-up period, the patient's condition was subject to major fluctuations but, on the whole, findings showed slow neurologic and neurophysiologic deterioration. The clinical course observed after bone marrow transplantation probably more or less reflects the natural course expected in this form of late-onset metachromatic leukodystrophy.


Assuntos
Transplante de Medula Óssea , Leucodistrofia Metacromática/diagnóstico , Leucodistrofia Metacromática/cirurgia , Adolescente , Progressão da Doença , Feminino , Seguimentos , Humanos , Leucodistrofia Metacromática/tratamento farmacológico , Testes Neuropsicológicos , Resultado do Tratamento
10.
Wien Med Wochenschr ; 147(7-8): 186-93, 1997.
Artigo em Alemão | MEDLINE | ID: mdl-9297368

RESUMO

Patients with cerebral arteriovenous malformations (cAVMs) are usually young at the time of diagnosis with a mean age of 32 years. These patients are in a crucial phase of their lives, starting a family and establishing a professional position. Facing a relative annual risk of 2 to 3% for a severe hemorrhage which cumulates to a 50% risk of hemorrhage over the next four decades of their lives, these patients have to make the decision about possible treatment of their cAVM. Advice can only be given to the patients on an individual basis because there are almost no two identical lesions. Nevertheless, based on a grading system and within established treatment regimens, risk estimations are possible and particularly with close neuroradiological/neurosurgical cooperation these risks can be defined and minimized. In this context we report a series of 184 patients with cAVMs which were taken care of at the Departments of Neurosurgery and Neuroradiology at the University of Hamburg between February 1988 and July 1996 and who were treated according to a standardized joint protocol with interventional and microsurgical options.


Assuntos
Neoplasias Encefálicas/terapia , Embolização Terapêutica , Hemangioma/terapia , Malformações Arteriovenosas Intracranianas/terapia , Microcirurgia , Adulto , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/mortalidade , Feminino , Hemangioma/diagnóstico por imagem , Hemangioma/mortalidade , Humanos , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Malformações Arteriovenosas Intracranianas/mortalidade , Masculino , Equipe de Assistência ao Paciente , Radiografia , Taxa de Sobrevida , Resultado do Tratamento
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