Maria Lucia Vellutini Pimentel

Multiple sclerosis, spinal cord ependymoma and intracranial meningioma: case report

We report the association a multiple sclerosis (MS), spinal cord tumour and intracranial tumor in a 63 years-old female patient with a 10 years history of relapsing/remitting MS. Symptoms usually remitted in response to costicosteroid therapy. In 1997 the patient presented with paraparesis and paresis of right arm which did not respond to corticotherapy. A spinal RMI revealed in the cervical spinal an intra spinal cord tumour, further diagnosed as ependymoma, and a parietal region meningioma. We call attention to this rare association of central nervous system tumour and MS, enphasizing the need for investigation of new and uncommon symptoms during the evolution of MS.

Immune system markers of neuroinflammation in patients with clinical diagnose of neuromyelitis optica

Neuromyelitis optica (NMO) is an inflammatory, demyelinating disease of the central nervous system characterized by the association of a serious myelitis and unilateral or bilateral optic neuritis. The present study aimed to analyze the immunological parameters of NMO patients with diagnosis established based on Wingerchuck et al. (1999) criteria. Production of IgG and IgA antibodies to antigens of MBP, PLP 95-116, MOG 92-106, and the cytokines interleukin-4 (IL-4) and interferon-gamma (INF-gamma) were assessed by Elisa assay. The cohort was formed by 28 NMO patients and a matched healthy control group. NMO patients had significant high levels of IgG to MOG (p<0.0001), PLP (p=0.0002) and MBP (p<0.0001), and solely IgA to MBP (p<0.0001). INF-gamma (p=0.61) levels were similar to healthy controls. Increased production of IL-4 (p=0.0084) indicates an important role for this cytokine in the activation of Th2 regulatory cells and of the IgA producers B lymphocyte indicating activation of humoral immunity.

Diabetes Effects in Alzheimer Disease: The Interactive Role of Insulin and Aβ Peptide

Insulin resistance, hyper-insulinemia and products associated to insulin metabolism can affect the amyloid cascade and promote the onset of Alzheimer`s disease or aggravate the condition, in early or old age regardless of the development of type 2 diabetes. The changes described in pathological studies and molecular research, classify two types of mechanism involved with cognitive impairment in these cases: one related to cerebrovascular events due the action of vascular risk factors, and the other more controversial, non-cerebrovascular mechanism involving the interaction of insulin with Aβ in the entorhinal cortex and hippocampus, as well as its synaptogenesis action that involves signaling of intracellular molecular paths in the modulating of neurotransmitters such as acetylcholine, norepinephrine and glutamate receptors. Based on a literature review, the role of insulin in the Central Nervous System is examined along with its participation in the amyloidogenesis process in progression to Alzheimer Disease. This review also addresses the consequence of chronic peripheral hyperinsulinemia, leading to down-regulation of insulin receptors in the blood-brain barrier and decreased insulin up-take, causing a state of central hypoinsulinism. This state interferes mainly in the process of Aβ degradation, emphasizing the role of the catalytic enzymes in Aβ clearance, particularly of the insulinase. Among others, increasing synaptic toxicity by disrupting PI3K/Akt inhibition of the GSK3 intracellular molecular pathway increasing tau phosphorylation, as well as PKC synaptogenesis signaling, causing clinical and anatomic changes that favor Alzheimer Disease.

Múltiplos tuberculomas intracerebrais na vigência de terapia específica para tuberculose pulmonar: a propósito de um caso

The classical presentation of neurotuberculosis is meningitis. Intracranial tuberculomas are a rare manifestation of neurotuberculosis and are due to hematogenous dissemination of distant focuses of Mycobacterium tuberculosis infection. Around 1% of patients with central nervous system tuberculosis develop intracranial tuberculomas some weeks or months after the beginning of the specific treatment with tuberculostatic chemotherapy. The involution of the lesions is slow and does not mean drug resistance nor lack of adequate treatment. We describe the case, diagnosed and treated at the 25th Infirmary of Santa Casa da Misericórdia do Rio de Janeiro, of an immunocompetent male patient who developed meningitis and multiple intracranial tuberculomas during the specific treatment of miliary tuberculosis. The literature is revised and the diagnosis, treatment and possible immunological mechanisms are discussed.

Multiple sclerosis in the Southern and Northern hemispheres: the month of birth at different latitudes has the same influence on the prevalence and progression of the disease in the Northern and Southern hemispheres?

The etiopathogenesis of multiple sclerosis (MS) remains unclear in spite of the several attempts to predict the risk of acquiring the disease. Genetic and environmental factors related to disease susceptibility, suggested by many recent studies that configured a multifactorial etiology to MS, reflect its geographical distribution and changes in risk with migration. The incidence of MS seems to decrease with migration in early life, from high-risk to low-risk areas1,2. It’s already known that specific haplotypes represent a genetic risk factor for MS. Exposure to sunlight and production of vitamin D appear to be important factors for MS susceptibility. Vitamin D seems to promote Th2 function and to inhibit the proinflammatory cytokines IL-1a, IL 2β and TNFα. Its role in immunological response was the focus of many studies concerning autoimmune diseases, including MS3. Vitamin D was also identified as a promoter of some alleles related to the month of birth and risk of MS4. According to latitude, Kurtzke5 and Bharanidharan6 stratified the prevalence of MS in three groups, and the Northern parts of Europe and North America presented high range (>30/100,000), Southern Europe and USA had medium range (5–30/100,000), South America presented low to medium range and Asia had low range (<5/100,000 inhabitants). Many studies suggested that less exposure to sunlight in high latitudes increased the risk of MS1. Several countries in the Northern hemisphere analyzed the mother’s exposure to sunlight during pregnancy, the month of birth and the risk of developing MS, and found a higher number of MS patients born in winter time and in higher latitude regions7,8. Conversely, recent studies have not found a significant variation in the prevalence of MS with the latitude in Latin America. Risco et al.9 considered specific environmental factors and different ethnicity also as being related to the lower influence of latitude in the prevalence of MS in Latin America, compared to Europe and North America. In this issue of Arquivos de Neuro-Psiquiatria, Fragoso YD et al.10 published the first study to evaluate the risk of MS according to latitude and month of birth, which included many different regions in the same continent. The authors analyzed 1,207 MS patients and 1,207 controls in four countries of South America: Argentina, Brazil, Chile and Peru, and compared the month of birth in a large range of latitudes (zero to 10° South; 11° to 20° South; 21° to 30° South; and 31° to 40° South) and the prevalence of MS. In spite of the relatively low number of patients due to the lower prevalence of MS in the Southern hemisphere compared to the Northern hemisphere, children born in high latitudes in the Southern hemisphere did not present higher incidence of MS in adult life. There is not a major variation of exposure to daylight between different seasons in the Southern hemisphere as there is in the Northern Head of Clinic, Neurology Outpatient Clinic, Santa Casa da Misericórdia do Rio de Janeiro, Rio de Janeiro RJ, Brazil. Correspondence Maria Lucia Vellutini Pimentel Rua Santa Luzia 206, Centro 24a / 25a Enfermarias 20020-022 Rio de Janeiro RJ Brasil E-mail: mlvpimentel@gmail.com Conflict of interest There is no conflict of interest to declare.

Tremor: adaptação de uma escala clínica

The Fahn, Tolosa e Maríns clinical rating scale for tremor was adapted to our country for evaluation of tremor. This scale was applied to 123 patients with this movement disorder, selected from the Ambulatory of General Medicine at Hospital Universitário Pedro Ernesto da Universidade do Estado do Rio de Janeiro. These patients had been chosen no matter the etiology of their condition or their age. Although some variants may had influenced the scores of this particular scale, they did not seem to change its standardization and applicability.

Síndrome de Parinaud e sinal de Collier: manifestaçäo neurológica inicial em paciente HIV-1 positivo

We describe the third case in the literature of Parinaud`s syndrome as the sole manifestation of brainstem toxoplasmosis, in a positive HIV-1 patient and review the literature concerning the possible etiologies of Parinaud`s syndrome, enforcing that it is a rare manifestation of central nervous system toxoplasmosis. We refer to its occurrence without simultaneous obstructive hydrocephalus and increased intracranial pressure, pointing out the Collier signal which is not very well known, but has great semiotic value once it locates the lesion in the brainstem.

Tremor essencial: observações clínicas

The authors have studied 53 patients with essential tremor, focusing its clinical and epidemiological aspects. There were familial history in 37.70% of all cases, prevailing in females (56.60%) and white people (69.80%); nevertheless this difference can not be assured from the statistical point of view due to lack of population data. We agreed that the main incidence of this syndrome occurred beyond the 5th decade, specially during the 6th and 7th decades. Topographically, we could observe that the hand tremor predominanted, with an incidence of 96.20% of the total number of cases, followed by head tremor (28.30%), voice tremor (16.99%), leg tremor (11.30%), tongue tremor (3.78%) and trunk tremor (1.88%). These focal tremors were seldom observed alone and we noted frequent association with hand tremor.

Dystonia induced by peripheral trauma: organic or psychogenic?

Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. A cause-and-effect relationship between brain injury and subse-quent movement disorder is well established, but a link with peripheral trauma is more controversial

Primary progressive freezing gait: case report

The clinical features of the primary progressive freezing gait, a rare and distinct neurological entity, have been described in some articles. This paper describes this gait disorder affecting one patient, whose conditions long course provoked no postural instability. As stated in the literature, the pathophysiology of the primary progressive freezing gait still remains unclear, although the lack of response to levodopa therapy suggests a possible involvement of nondopaminergic pathways. Alteration in the visual perception may be a potential cause for the condition.