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Repositório do Hospital Prof. Doutor Fernando Fonseca (3.582 recursos)

The Hospital Prof. Dr. Fernando Fonseca E.P.E. (HFF) institutional repository collects, preserves and disseminates the intellectual output of its professionals in digital format. Its aims are to increase the HFF’s investigation visibility and citation impact, to foster scientific communication and to preserve the HFF’s intellectual memory.

NEU - Artigos

Mostrando recursos 1 - 20 de 31

  1. Behavioral response to headache: a comparison between migraine and tension-type headache.

    Martins, I; Parreira, E
    OBJECTIVE: To compare patients with migraine and tension-type headache in their behavior during the attacks and the maneuvers used to relieve the pain. BACKGROUND: Patients with headache often perform nonpharmacological measures to relieve the pain, but it is not known if these behaviors vary with the diagnosis, clinical features, and pathogenesis. METHODS: One hundred consecutive patients with either migraine (n = 72 ) or tension-type headache (n = 28) were questioned (including the use of a checklist) concerning their usual behavior during the attacks and nonpharmacological maneuvers performed to relieve the pain. The results of the two types of headache were compared. RESULTS:...

  2. Neuronal migration defects in the Dreher (Lmx1a) mutant mouse: role of disorders of the glial limiting membrane

    Costa, C; Harding, B; Copp, A
    Dreher (dr(J)) is an autosomal recessive mutation in the newly identified LIM homeobox gene, Lmx1a. The homozygous mutant phenotype includes misplaced neurons (heterotopia) in the cerebral cortex, cerebellum and hippocampus, which mimic the mild end of the spectrum of neuronal migration disorders in humans. Heterotopic neurons are found mainly in the normally cell-sparse layer I within the cerebral hemispheres of dr(J) homozygotes. Neu-N immunostaining confirms the neuronal nature of these heterotopic cells, while bromodeoxyuridine-birthdating shows that the misplaced neurons are generated predominantly during the late stages of corticogenesis (E15-E17), suggesting an over-migration of neurons destined for layer II. Immunohistochemistry for...

  3. Romboencefalite e abcessos cerebrais por listeria monocytogenes: um desafio clínico

    Costa, S; Valverde, A
    A Listeria monocytogenes é um bacilo gram positivo, anaeróbio facultativo e responsável por envolvimento do sistema nervoso central em 30 a 55% dos casos. As manifestações mais frequentes são a meningite aguda e subaguda, seguida da bacteriémia, romboencefalite e abcessos cerebrais ou medulares. Apresentamos um caso clínico de uma romboencefalite e abcessos cerebrais por L. monocytogenes. Concluímos que é importante considerar sempre o diagnóstico de infecção por este agente em doentes imunossuprimidos e/ou com mais de 65 anos com envolvimento infratentorial do SNC ou meningite, devendo ser iniciada antibioterapia assim que possível. Todavia, perante a suspeita clínica deve ser feita a colheita de hemoculturas antes...

  4. Romboencefalite e abcessos cerebrais por listeria monocytogenes: um desafio clínico

    Costa, S; Valverde, A
    A Listeria monocytogenes é um bacilo gram positivo, anaeróbio facultativo e responsável por envolvimento do sistema nervoso central em 30 a 55% dos casos. As manifestações mais frequentes são a meningite aguda e subaguda, seguida da bacteriémia, romboencefalite e abcessos cerebrais ou medulares. Apresentamos um caso clínico de uma romboencefalite e abcessos cerebrais por L. monocytogenes. Concluímos que é importante considerar sempre o diagnóstico de infecção por este agente em doentes imunossuprimidos e/ou com mais de 65 anos com envolvimento infratentorial do SNC ou meningite, devendo ser iniciada antibioterapia assim que possível. Todavia, perante a suspeita clínica deve ser feita a colheita de hemoculturas antes...

  5. Cefalea en paciente con neurofibromatosis tipo 1

    Herrero Valverde, A; Moiron Simões, R; Mera Campillo, J; Palma, T
    INTRODUCTION: Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome due to a mutation in chromosome 17 (at 17q11.2). The association of NF-1 with Arnold-Chiari I malformation has been previously described. CASE REPORT: A 23 year-old woman diagnosed of NF-1 based on the presence of cutaneous lesions and a familiar background consistent with this was referred to the neurologist due to oppressive frontooccipital bilateral headache that irradiated to both shoulders. It worsened on physical effort and Valsalva maneuver and had more than one year of evolution. Physical examination showed multiple café au lait spots in the trunk and cutaneous neurofibromas...

  6. What should you know about limbic encephalitis?

    Machado, S; Pinto, A; Irani, S
    Autoimmune encephalitis is an inflammatory disorder characterized by a subacute impairment of short-term memory, psychiatric features and seizures. It is often associated with a variety of other neurological symptoms, and its differential diagnosis is wide, leading to challenges in its recognition. It used to be regarded as a rare disease, usually paraneoplastic and with poor prognosis. However, with the recent recognition of membrane-surface directed antibodies, it is now known that in a substantial proportion of cases there is no association with any malignancy and there is a good prognosis if treated. Hence, early recognition and prompt initiation of immunotherapies are of great importance.

  7. Diagnosis of stroke by the nonneurologist: a validation study.

    Ferro, J; Pinto, A; Falcão, I; Rodrigues, G; Ferreira, J; Falcão, F; Azevedo, E; Canhão, P; Melo, T; Rosas, MJ; Oliveira, V; Salgado, AV
    BACKGROUND AND PURPOSE: The first medical contact of an acute stroke victim is often a nonneurologist. Validation of stroke diagnosis made by these medical doctors is poorly known. The present study seeks to validate the stroke diagnoses made by general practitioners (GPs) and hospital emergency service physicians (ESPs). METHODS: Validation through direct interview and examination by a neurologist was performed for diagnoses of stroke made by GPs in patients under their care and doctors working at the emergency departments of 3 hospitals. RESULTS: Validation of the GP diagnosis was confirmed in 44 cases (85%); 3 patients (6%) had transient ischemic attacks and...

  8. Acquired immunodeficiency syndrome and the risk of stroke

    Cole, J; Pinto, A; Hebel, J; Buchholz, D; Earley, C; Johnson, C; Macko, R; Price, T; Sloan, M; Stern, B; Wityk, R; Wozniak, M; Kittner, S
    BACKGROUND AND PURPOSE: Although acquired immunodeficiency syndrome (AIDS) is thought to increase the risk of stroke, few data exist to quantify this risk. This is the first population-based study to quantify the AIDS-associated risk of stroke. METHODS: We identified all incident ischemic stroke (IS) and intracerebral hemorrhage (ICH) cases among young adults 15 to 44 years of age in central Maryland and Washington, DC, who were discharged from any of the 46 hospitals in the study area in 1988 and 1991. Using data from the medical records, 2 neurologists reviewed each case to confirm the diagnosis. Cases of AIDS among these...

  9. Headache as the sole presentation of cerebral venous thrombosis: a prospective study

    Timóteo, A; Inácio, N; Machado, S; Pinto, A; Parreira, E
    Headache is the most frequent presenting symptom of cerebral venous thrombosis (CVT), most commonly associated with other manifestations. It has been described as its only clinical presentation in 15 % of patients. There is no typical pattern of headache in CVT. The objective of this study was to study the characteristics of headache as the sole manifestation of CVT. From a prospective study of 30 consecutive patients diagnosed with CVT over 18 months, we selected those who presented with headache only: they had a normal neurological examination, no papilloedema and no blood or any parenchymal lesion on CT scan. All...

  10. AIDS/HIV infection and cerebrovascular disease

    Pinto, A
    The occurrence of cerebrovascular disease in patients with human immunodeficiency virus (HIV) infection has been reported mainly in advanced stages of the disease and was generally associated with nonbacterial thrombotic endocarditis, opportunistic infections, or tumors, although in recent series a large number of cryptogenic strokes were found, probably related to HIV vasculopathy. Recently a population-based study reported a strong association between acquired immunodeficiency syndrome (AIDS) and both ischemic stroke and intracerebral hemorrhage, with an incidence of 0.2% per year. However, with the advent of highly active retroviral therapy (HAART)-causing immune restoration in HAARTtreated patients and avoiding early death and leading to a lengthening...

  11. Involuntary rhythmic leg movements time-locked with the respiratory cycle

    Leal, A; Calado, E
    Involuntary rhythmic leg movements in childhood is an uncommon condition, the generators of which remain unknown. We report on a male 3 years of age with distinct features providing important clues concerning the location of one of these generators. At the age of 7 months, the previously healthy young male started with low frequency, rhythmic, and continuous (both during wakefulness and sleep) flexion/extension movements of the lower limbs. Movements interfered significantly with gait acquisition, and, despite normal cognitive development, he was able to walk only at age 2 years, 4 months. The neurologic examination revealed the absence of automatic stepping...

  12. Citicoline in the treatment of acute ischaemic stroke: an international, randomised, multicentre, placebo-controlled study (ICTUS trial)

    Dávalos, A; Alvarez-Sabin, J; Castillo, J; Diez-Tejedor, E; Ferro, J; Martinez-Vila, E; Serena, J; Segura, T; Cruz, V; Masjuan, J; Cobo, E; Secades, J; Salgado, AV; International Citicoline Trial on acUte Stroke (ICTUS) trial investigators
    BACKGROUND: Citicoline is approved in some countries for the treatment of acute ischaemic stroke. The drug has shown some evidence of efficacy in a pooled analysis. We sought to confirm the efficacy of citicoline in a larger trial. METHODS: We undertook a randomised, placebo-controlled, sequential trial in patients with moderate-to-severe acute ischaemic stroke admitted at university hospitals in Germany, Portugal, and Spain. Using a centralised minimisation process, patients were randomly assigned in a 1:1 ratio to receive citicoline or placebo within 24 h after the onset of symptoms (1000 mg every 12 h intravenously during the first 3 days and orally...

  13. Examining the motor phenotype of patients with both essential tremor and Parkinson's disease.

    Simões, R; Constantino, A; Gibadullina, E; Houghton, D; Louis, E; Litvan, I
    Previous reports have suggested that essential tremor (ET) represents a risk factor for the development of Parkinson's disease (PD). Patients with long-standing ET who develop PD tend to have a tremor-dominant subtype. To further clarify this association, we examined patients from kindreds with autosomal dominant ET who had signs of isolated PD but did not meet criteria for overlapping ET. We identified 22 patients with PD meeting these diagnostic criteria, and 90% (20 of 22) had tremor-predominant subtype of PD. Unilateral rest tremor was the presenting symptom in 15 of 22 patients, bradykinesia or rigidity in 5 of 22, and...

  14. Rapidly progressive corticobasal degeneration syndrome.

    Valverde, A; Costa, S; Ginestal, R; Pimentel, J; Timóteo, A
    Introduction: Corticobasal syndrome (CBS) has a heterogeneous clinical presentation with no specific pathologic substratum. Its accurate diagnosis is a challenge for neurologists; in order to establish CBS definitively, postmortem confirmation is required. Some clinical and radiological features can help to distinguish it from other neurodegenerative conditions, such as Creutzfeldt-Jakob disease (CJD). Clinical Case: A 74-year-old woman presented with language impairment, difficulty in walking and poor attentiveness that had begun 10 days before. Other symptoms, such as asymmetrical extra-pyramidal dysfunction, limb dystonia and ‘alien limb’ phenomena, were established over the next 2 months, with rapid progression. Death occurred 3 months after symptom onset. Laboratory results were normal. Initially, imaging only showed...

  15. Posterior reversible encephalopathy syndrome and anti-angiogenic agents: a case report

    Silva, F; Pêgo, P; Vendrell, MC; Farias, MJ; Timóteo, A; Costa, MC; Cravo, I; Gomes, F
    Posterior reversible encephalopathy syndrome is an increasingly recognised clinico-radiological entity, associated with several medical conditions (such as systemic arterial hypertension) and characterised by seizures, altered mental status, headaches, and visual symptoms. Magnetic resonance imaging is a key component in this diagnosis, with hyperintense foci in T2-weighted images, corresponding to vasogenic oedema. The pathophysiology is not fully understood but probably involves loss of auto-regulation of cerebral vasculature or endothelial dysfunction or both. A 56-year-old male, suffering from a gastro-intestinal stromal tumour with hepatic metastasis resistant to imatinib, on therapy with sunitinib, came to the Emergency Department because of headaches, hallucinations, and loss of vision....

  16. Kinesiophobia in migraine

    Martins, I; Gouveia, R; Parreira, E
    Pain aggravation by movement and avoidance of movement (kinesiophobia) is often reported by patients during migraine attacks. Yet its specific contribution to migraine diagnosis is undetermined. To characterize the frequency and severity of kinesiophobia during migraine and its role in the diagnosis of primary headaches, we questioned 150 patients (126 women and 24 men, average age 38.5 yrs) with migraine (n = 111) or tension-type headache (TTH) (n = 39) about aggravation of pain by bending forward, brisk head movements (jolt), and avoidance of movement during the attacks. The degree of pain worsening by each stimulus was measured through a...

  17. Mutations of the GLA gene in young patients with stroke: the PORTYSTROKE study-screening genetic conditions in Portuguese young stroke patients

    Baptista, V; Ferreira, S; Pinho-e-Melo, T; Carvalho, M; Cruz, V; Carmona, C; Silva, F; Tuna, A; Rodrigues, M; Ferreira, C; Pinto, A; Leitão, A; Gabriel, J; Calado, S; Oliveira, J; Ferro, J
    BACKGROUND AND PURPOSE: Fabry disease is an X-linked monogenic disorder caused by mutations in the GLA gene. Recent data suggest that stroke in young adults may be associated with Fabry disease. We aimed to ascertain the prevalence of this disorder among young adult patients with stroke in Portugal by GLA genotyping. METHODS: During 1 year, all patients aged 18 to 55 years with first-ever stroke, who were admitted into any of 12 neurology hospital departments in Portugal, were prospectively enrolled (n=625). Ischemic stroke was classified according to Trial of Org 10172 in Acute Stroke Treatment criteria. Alpha-galactosidase activity was further assayed...

  18. Neuralgic amyotrophy following botulinum toxin injection

    Alcalay, R; Simões, R; Feigin, A; Frucht, S

  19. Nonsense mutation in TITF1 in a Portuguese family with benign hereditary chorea

    Costa, MC; Costa, C; Silva, A; Evangelista, P; Santos, L; Ferro, A; Sequeiros, J; Maciel, P
    Benign hereditary chorea (BHC) is an autosomaldominant disorder of early onset characterized by a slowly progressing or nonprogressing chorea, without cognitive decline or other progressive neurologic dysfunction, but also by the existence of heterogeneity of the clinical presentation within and among families. The genetic cause of BHC is the presence of either point mutations or deletions in the thyroid transcription factor 1 gene (TITF1). We studied a Portuguese BHC family composed of two probands: a mother and her only son. The patients were identified in a neurology out-patient clinic showing mainly involuntary choreiform movements since childhood, myoclonic jerks, falls, and...

  20. Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products

    Silva-Fernandes, A; Costa, MC; Duarte-Silva, S; Oliveira, P; Botelho, C; Martins, L; Mariz, J; Ferreira, T; Ribeiro, F; Correia-Neves, M; Costa, C; Maciel, P
    Machado-Joseph disease (MJD) is a late-onset neurodegenerative disorder caused by a polyglutamine (polyQ) expansion in the ataxin-3 protein. We generated two transgenic mouse lineages expressing the expanded human ataxin-3 under the control of the CMV promoter: CMVMJD83 and CMVMJD94, carrying Q83 and Q94 stretches, respectively. Behavioral analysis revealed that the CMVMJD94 transgenic mice developed motor uncoordination, intergenerational instability of the CAG repeat and a tissue-specific increase in the somatic mosaicism of the repeat with aging. Histopathological analysis of MJD mice at early and late stages of the disease revealed neuronal atrophy and astrogliosis in several brain regions; however, we found...

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