PUBLICAÇÕES MÉDICAS
Camila Pupe | Neurologia Geral, e Doenças Neuromusculares
COVID-19 and herpes zoster co-infection presenting with trigeminal neuropathy
Varicella-zoster virus (VZV) is a human neurotropic virus that remains in a latent state within ganglionic neurons throughout the entire neuroaxis after the primary infection. When herpes zoster (HZ) leads to trigeminal involvement, the ophthalmic division is the most implicated. COVID-19 has emerged as a viral cause of severe acute respiratory syndrome that has spread all over the world in the last months. Co-infection with COVID-19 and other viruses has been reported, but sparsely, and involving the respiratory viruses.
Amyotrophic Lateral Sclerosis: New Perpectives and Update
Amyotrophic lateral sclerosis (ALS), Charcot’s disease or Lou Gehrig’s disease, is a term used to cover the spetrum of syndromes caracterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration. Currently, there are approximately 25,000 patients with ALS in the USA, with an average age of onset of 55 years. The incidence and prevalence of ALS are 1-2 and 4-6 per 100,000 each year, respectively, with a lifetime ALS risk of 1/600 to 1/1000.
Definition and diagnosis of small fiber neuropathy: consensus from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology
The aim of this study was to describe the results of a Brazilian Consensus on Small Fiber Neuropathy (SFN). Fifteen neurologists (members of the Brazilian Academy of Neurology) reviewed a preliminary draft. Eleven panelists got together in the city of Fortaleza to discuss and finish the text for the manuscript submission. Small fiber neuropathy can be defined as a subtype of neuropathy characterized by selective involvement of unmyelinated or thinly myelinated sensory fibers. Its clinical picture includes both negative and positive manifestations: sensory (pain/dysesthesias/pruritus) or combined sensory and autonomic complaints, associated with an almost entirely normal neurological examination. Standard electromyography is normal. A growing list of medical conditions is associated with SFN. The classification of SFN may also serve as a useful terminology to uncover minor discrepancies in the normal values from different neurophysiology laboratories. Several techniques may disclose sensory and/or autonomic impairment. Further studies are necessary to refine these techniques and develop specific therapies.
Consenso Brasileiro para o diagnóstico, manejo e tratamento da Polineuropatia Amiloidótica Familiar associada à Transtirretina
Transthyretin familial amyloid polyneuropathy is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy, which if untreated, leads to death in approximately 10 years. In Brazil, liver transplant and tafamidis are the only disease-modifying treatments available. This review consists of a consensus for the diagnosis, management and treatment for transthyretin familial amyloid polyneuropathy from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology. The first and last authors produced a draft summarizing the main views on the subject and emailed the text to 10 other specialists. Relevant literature on this subject was reviewed by each participant and used for the individual review of the whole text. Each participant was expected to review the text and send a feedback review by e-mail. Thereafter, the 12 panelists got together at the city of Fortaleza, discussed the controversial points, and reached a consensus for the final text.
Dor na doença de Charcot-Marie-Tooth: atualização
A doença de Charcot-Marie-Tooth (CMT), a neuropatia periférica hereditária mais comum, tem a dor como uma de suas características clínicas, a qual permanece subdiagnosticada e subtratada. Essa revisão de literatura avaliou os dados relacionados à dor em CMT com objetivo de observar sua prevalência, tipo e importância como sintoma que, em detrimento de outros, é possível ser tratado. O intervalo da pesquisa foi entre 2007 e 2017, através de cinco artigos abordando a dor em CMT. Todos os artigos concordam que a dor é frequente nos pacientes com a doença de CMT e a sua classificação permanece indefinida por não haver consenso na literatura sobre os mecanismos da dor.
Neuropathic Pain Treatment: Still a Challenge
Neuropathic pain (NP) is the result of a series of conditions caused by diseases or lesions to the somatosensory system. Due to the better understanding of NP pathophysiology previously unexplored therapies have been used with encouraging results. In this group, acetyl-L-carnitine, alpha-lipoic-acid, cannabinoids, clonidine, EMA401, botulinum toxin type A and new voltage-gated sodium channel blockers, can be included. Besides, changing paradigms may occur with the advent of optogenetics and a better understanding of epigenetic regulation. We reviewed the published literature on the pharmacological treatment of NP. Despite the interesting results, randomized controlled trials are demanded the majority of the therapies previously mentioned. In spite of several studies for the relief of NP, pain control continues being a challenge.
Neuropatia de início tardio: uma apresentação incomum da hanseníase
Clinical and pathological findings in leprosy are determined by the natural host immune response to Mycobacterium leprae. We previously described cases of painful neuropathy (PN) with no concurrent cause apart from a past history of leprosy successfully treated. Four leprosy previously treated patients who developed a PN years after multidrug therapy (MDT) are reported. The mean patient age was 52.75 years (47-64). The mean time interval of the recent neuropathy from the previous MDT was 19 years (12-26). A painful multiplex neuritis or polyneuropathy were observed respectively in two cases. Electrophysiological studies disclosed a sensory axonal neuropathy in two cases. Microvasculitis with no bacilli was seen in nerve biopsy. Neuropathic symptoms were improved with prednisone. We consider these cases as being a leprosy late-onset neuropathy (LLON) form of presentation. A delayed immune reaction could explain the late appearance of LLON.
A utilização do Xbox/Kinect na reabilitação pós-acidente vascular cerebral: uma revisão sistemática
Active games based on virtual reality have been widely used in the rehabilitation of many clinical conditions. However, studies on the use of Xbox/Kinect are rare, and technology application in stroke treatment is not clear yet.
Is the “lactormone” a key-factor for exercise-related neuroplasticity? A hypothesis based on an alternative lactate neurobiological pathway
For many years lactate was seen as a metabolite from glucose metabolism. However, since the last century researchers have shown that this molecule has an important role on liver, muscle, and brain metabolism. Lactate traffics along whole body mediating many biological processes depending on specific situations. For example, glucose is the main substrate used during exercise but lactate released by striated skeletal muscle is used by own muscle as secondary fuel.
Microcephaly and arthrogryposis multiplex congenita: The full-blown CNS spectrum in newborns with ZIKV infection
The recent alarming statements concerning the newborn ZIKV-induced microcephaly epidemics in the Northeast of Brazil, released by the Brazilian Ministry of Health, as well as important international health agencies, such as the World Health Organization and the Pan American Health Organization, raised many “why and how” questions so far, that will hopefully be scientifically answered, as more researches in that regard come up in the long term.
Revisiting the Term Neuroprotection in Chronic and Degenerative Diseases
Thanks to the development of several new researches, the lifetime presented a significant increase, even so, we still have many obstacles to overcome − among them, manage and get responses regarding neurodegenerative diseases. Where we are in the understanding of neuroprotection? Do we really have protective therapies for diseases considered degeneratives such as amyotrophic lateral sclerosis and its variants, Parkinson’s disease, Alzheimer’s disease and many others? Neuroprotection is defined by many researches as interactions and interventions that can slow down or even inhibit the progression of neuronal degeneration process. We make some considerations on this neuroprotective effect.
Definição e diagnóstico de neuropatia de fibras finas: consenso do Departamento Científico de Neuropatias Periféricas da Academia Brasileira de Neurologia
O objetivo deste estudo é descrever os resultados de um Consenso Brasileiro sobre Neuropatia de Fibras Finas (NFF). Quinze neurologistas (membros da Academia Brasileira de Neurologia) revisaram uma versão preliminar do artigo. Onze panelistas se reuniram na cidade de Fortaleza para discutir e terminar o texto para a submissão do manuscrito. NFF pode ser definida como um subtipo de neuropatia caracterizada pelo envolvimento seletivo de fibras sensitivas amielínicas ou pouco mielinizadas. Seu quadro clínico inclui manifestações negativas e positivas: sensitivas (dor/disestesias/prurido) ou queixas sensitivas e autonômicas combinadas, associadas a exame neurológico quase totalmente normal. A eletromiografia convencional é normal. Uma lista crescente de condições médicas causa NFF. NFF também pode servir como uma terminologia útil para referenciar pequenas discrepâncias nos valores normais de diferentes laboratórios de neurofisiologia. Diferentes técnicas podem evidenciar anormalidades sensitivas e/ou autonômicas. São necessários mais estudos para refiná-las e para o desenvolvimento de terapias específicas.
Evaluation of quality of life in patients with type 2 diabetes Mellitus with symptomatic distal symmetric polyneuropathy / Avaliação da qualidade de vida em doentes com diabetes Mellitus tipo 2 com polineuropatia simétrica distal sintomática
The complications of Diabetes Mellitus (DM) are traditionally categorized as micro and macrovascular disorders. Among them, diabetic polyneuropathy (DPN) is one of the most common, presenting with or without associated neuropathic pain, and its morbidity exerts a significant impact on the quality of life (QOL) of these patients. About 50% of individuals with type 2 DM (T2DM) suffer from this condition and the distal symmetric polyneuropathy (DSPN) constitutes its most frequent clinical form. OBJECTIVE: To demonstrate the effect of symptomatic DSPN on the QOL of T2DM patients in a sample of the Brazilian population, correlating clinical and electrophysiological findings, besides comparing the results obtained by the Medical Outcomes Study Questionaire 36-Item Short Form Health Survey (SF-36) among patients with painful and non-painful diabetic DSPN
Tópicos atuais no tratamento cardiorrespiratório de pacientes com síndrome pós-poliomielite
Post-polio syndrome (PPS) is a condition that affects polio survivors years after recovery from an initial acute attack of the poliomyelitis virus. Most often, polio survivors experience a gradual new weakening in muscles that were previously affected by the polio infection. The actual incidence of cardiovascular diseases (CVDs) in individuals suffering from PPS is not known. However, there is a reason to suspect that individuals with PPS might be at increased risk.
Chikungunya Virus Meningoencephalitis: A Case Report
Chikungunya is a mosquito-transmitted viral infection. Its main clinical manifestations include flu-like symptoms, rash and joint pain that can last a few days to years after the resolution of the infection. Besides these systemic manifestations, different kinds of neurological syndromes have been described in association with this virus.
Consenso Brasileiro para o diagnóstico, manejo e tratamento da Polineuropatia Amiloidótica Familiar associada à Transtirretina
Transthyretin familial amyloid polyneuropathy is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy, which if untreated, leads to death in approximately 10 years. In Brazil, liver transplant and tafamidis are the only disease-modifying treatments available. This review consists of a consensus for the diagnosis, management and treatment for transthyretin familial amyloid polyneuropathy from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology.
Encefalomiopatia neurogastrointestinal mitocondrial mimetizando polirradiculoneuropatia inflamatória desmielinizante crônica
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare multisystemic autosomal recessive disease caused by mutations in the gene encoding thymidine phosphorylase1 . The deficiency of this enzyme produces plasma accumulations of its substrates, thymidine and deoxyuridine, that have toxic effect on mitochondrial DNA, leading to multiple deletions and depletion
Síndrome de Guillain-Barré: avanços e perspectivas futuras
The first case of Guillain-Barré syndrome was described in 1916. Since then, knowledge about the pathophysiology and immunogenesis of this acquired inflammatory polyradiculoneuropathy has been growing steadily, especially after the advent of nerve conduction studies and the discovery of pathogenic autoantibodies. In the present study, we conducted a review of the main information available in the literature to date about the syndrome, including its diagnosis and management.
Palm to Finger Ulnar Sensory Nerve Conduction
Ulnar neuropathy at the wrist (UNW) is rare, and always challenging to localize. To increase the sensitivity and specificity of the diagnosis of UNW many authors advocate the stimulation of the ulnar nerve (UN) in the segment of the wrist and palm. The focus of this paper is to present a modified and simplified technique of sensory nerve conduction (SNC) of the UN in the wrist and palm segments and demonstrate the validity of this technique in the study of five cases of type III UNW.
Esclerose Lateral Amiotrófica com achados sensitivos
Uma doença Multisistêmica?
A esclerose lateral amiotrófica (ELA) clássica não apresenta dificuldades para o correto diagnóstico, entretanto, quando se faz presente em forma atípica de doença do neurônio motor (DNM), fato consumado em 20% dos casos, gera confusão entre formas atípicas de ELA/DNM e outras patologias, tais como neuropatia motora multifocal, neuropatia desmielinizante inflamatória crônica a espondilose cervical. Associações entre neuropatias de causas várias e ELA têm sido relatadas raramente.
Clinical and Epidemiological Profile of a Brazilian’s cohort of patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy
To analyze the clinico-epidemiological profile of patients with Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a neuromuscular center of a University Hospital in Rio de Janeiro.
Gender Disparity Between Neurologists in Brazil: Is It Really Decreasing?
The main aim of this paper was to present the lack of a women’s advancement into leadership and academic positions in neurology in Brazil, despite the growing proportion of them among practicing neurologists and the fact that more than half the medical students in the country are women.
Contact Heat Evoked Potentials Stimulator (CHEPS) In The Assessement Of Painful Leprosy Neuropathy
To report the electrophysiological findings, including CHEPS, in consecutive series of 10 leprosy patients with neuropathic pain. Background: Leprosy is one of the most common causes of nontraumatic peripheral neuropathy in the developing countries. The causative agent, Mycobacterium leprae, has a predilection for Schwann cells, where the organism multiplies unimpeded by organism-specific host immunity, resulting in destruction of myelin, secondary inflammatory changes, and destruction of the nerve architecture.
Two Atypical Cases of Stiff-Person Syndrome
Report two cases of SPS with atypical manifestations Background: Stiff-person syndrome (SPS) is a rare autoimune movement disorder, characterized by muscular rigidity, painful muscle spasms predominantly affecting paraspinal muscles. Methods: Collect data from medical records and compare to medical literature.
The Anatomical Variation between the Superficial Radial and Lateral Antebrachial Cutaneous Nerves. A Nerve Conduction Study
The focus of this paper is to call attention for a poorly known anatomical variation in which the lateral antebrachial cutaneous nerve (LACN) innervates the radial border of the dorsum of the hand and thumb in addition to or replacing the superficial radial nerve (RSN). Our goal is to recommend a technique of nerve conduction to the recognition of this variation.
Neuropathic Pain Treatments and Quality of Life Results in Leprosy Patients
To evaluate the impact of two treatment regimens in health-related quality of life in patients with neuropathic pain due to leprosy presenting active neuritis. BACKGROUND: Leprosy is a chronic infectious disease caused by Mycobacterium leprae. In addition with a large social stigma, it causes neuropathic pain, among other comorbidities.
Contact Heat Evoked Potentials in Painful Autoimmune Neuropathy
To analyse CHEPS findings in PAiN patients. BACKGROUND: The analysis of contact heat evoked potentials (CHEPs) latency and amplitude is being considered of clinical utility for the assessment of neuropathic pain. It is a secure neurophysiological tool for patients complaining of pain, eliciting a dysfunction in small nerve fibers. It has been recently reported that prolonged latency and decreased CHEPs amplitude correlates with the intraepidermal nerve fiber density observed in skin biopsy performed in the area involved in the neuropathic symptoms.
Pompe Disease: Distal Myopathy In A Previously Unreported Heterozygous Variant
Pompe disease (PD) is an autosomal recessive, rare disease, caused by a deficiency on lysosomal enzyme alpha-glucosidase (GAA). It causes progressive muscular weakness and may lead to respiratory impairment. Presentation may occur as soon as in newborns (early-onset) or later in children, adolescents or adults (late-onset).
Corneal Confocal Microscopy (CCM) and Contact Heat-Evoked Potential Stimulator (CHEPS) Are Useful and Complementary Tools for Neuropathic Pain/Small-Fiber Neuropathy Diagnosis
To report the applicability of CHEPS complemented with CCM in neuropathic pain (NP) / small-fiber peripheral neuropathy (SFPN) diagnosis.
Avaliação de fatores preditores para diagnóstico de esclerose lateral amiotrófica em pacientes com doença do neurônio motor na primeira consulta neurológica
A doença do neurônio motor (DNM) é uma doença sistêmica de amplo espectro clínico. É caracterizada por acometimento primário do neurônio motor inferior ou superior, ou ambos, simultaneamente, representado pela forma mais comum, a esclerose lateral amiotrófica (ELA). A ELA é uma doença rara e fatal, que leva o indivíduo ao óbito entre três a cinco anos, em média, após o aparecimento dos primeiros sintomas, devido falência respiratória
NEUROPATIA DIABÉTICA E SEUS CUIDADOS EM PACIENTE DESCOMPENSADO: RELATO DE CASO
Diabetes Mellitus pode acometer qualquer idade, mas predomina na população adulta e é responsável por elevada mortalidade por doença cardiovascular e complicações microvasculares. Visando aumentar a sobrevida é importante o seu diagnóstico precoce. A complicação mais prevalente do Diabetes Mellitus é a neuropatia diabética.
TERMOGRAFIA COMO INSTRUMENTO DE RASTREIO E PREVENÇÃO DAS COMPLICAÇÕES DO PÉ DIABÉTICO: REVISÃO DE LITERATURA
O diabetes mellitus é uma desordem metabólica crônica que necessita de constante supervisão médica para prevenir graves complicações. A hiperglicemia pode afetar a visão, nervos, vasos sanguíneos, coração e até os rins.
Tratamento farmacológico da dor neuropática central: consenso da Academia Brasileira de Neurologia
A dor neuropática central (DNC) é frequentemente refratária às estratégias terapêuticas disponíveis e há poucas opções de tratamento baseado em evidência. Muitos pacientes com dor neuropática não são diagnosticados ou tratados adequadamente. Desse modo, recomendações baseadas em consenso, adaptadas à disponibilidade de medicamentos no país, são necessárias para guiar decisões clínicas.
Autocuidado praticado por idosos com diabetes mellitus em uma unidade básica de saúde – Parintins- AM
O presente artigo objetivou avaliar as práticas de autocuidado por pacientes idosos diabéticos na prevenção do pé diabético. Trata-se de uma pesquisa quantitativa, realizada com 51 idosos diabéticos atendidos por uma Unidade Básica de Saúde do município de Parintins, Amazonas, Brasil. A coleta de dados realizou-se nos meses de Agosto e Setembro de 2017, utilizando o Questionário de Atividades de Autocuidado com o Diabetes.
Rehabilitation in Amyotrophic Lateral Sclerosis: “Prudence and Limit of Programs”
The rehabilitation treatment approach for patients with Amyotrophic Lateral Sclerosis should be individualized, held in submaximal limits and changed according to the stages of the disease. The spectrum of presentation and disease progression speed may vary according to each patient and the transdisciplinary approach promotes measures focused mainly on improving the quality of life of patients.
Protótipo para a Avaliação Sensorial da Neuropatia Diabética Através de Testes Térmicos
Este estudo teve o objetivo de realizar o desenvolvimento de um Protótipo para Avaliação Sensorial da Neuropatia Diabética Periférica através de Testes Térmicos e o estudo das tecnologias envolvidas para a realização de um dispositivo desse tipo. Nele inclui a explicação teórica sobre a Diabetes Mellitus, Neuropatias, Neuropatia Diabética Periférica, Testes Diagnósticos da Neuropatia Diabética, Efeito Termoelétrico e Módulo Peltier.
Doença de Anderson Fabry
A hematologist once exclaimed that his specialty was unique because “blood is ubiquitous.” A neurologist immediately claimed similar ground explaining that the nervous system is also ubiquitous and likely more so. In all its components, the length of the peripheral nervous system is extraordinary from its sensory extensions into every segment of the skin, motor supply into every muscle fiber and autonomic innervation of every blood vessel, smooth muscle and cardiac muscle along with many secreting glands. The totality of this network is so extensive that it remains unmeasured to date.
Achados bucais em crianças com a Síndrome Congênita Zika: uma série de casos
O objetivo deste trabalho é descrever os principais achados orais observados em uma série de casos de crianças com a Síndrome Congênita do Zika (CZS). Os participantes foram selecionados dentre os pacientes atendidos em uma Unidade Básica de Saúde de um município do Nordeste do Brasil, os quais foram avaliados (anamnese e exame clínico oral) por um único pesquisador. A amostra final foi composta por 12 sujeitos (seis homens e seis mulheres), com idade entre 36 e 40 meses.