Neuropathology of human and experimental TSP/HAM: a critical review.

Tropical Spastic Paraparesis/HTLV-1 Associated Myelopathy (TSP/HAM) is clinically characterized by chronic insidious spastic paraparesis associated with variable sensory impairment and sphincter symptoms. Neuropathological studies of this condition are based on a few autopsied cases, and on experimental animal models. However, divergent aspects exist between human and experimental animal neuropathology of TSP/HAM, namely, the site of lesions in the spinal cord, the involvement of peripheral nerves and roots, the nature of histological abnormalities, and the cellular reactions. Moreover, unanswered questions as to the preferential site of spinal affection, the temporal inflammatory picture, the selective damage of the corticospinal tract, the sparing of lower motor neurons, the inconsistent affection of sensory tracts, and the involvement of the brain, are outlined. A long-term, chronological, correlated clinical and neuropathological study in HAM experimental animals is suggested.

Western blot seroindeterminate individuals for human T-lymphotropic virus I/II (HTLV-I/II) in Fortaleza (Brazil): a serological and molecular diagnostic and epidemiological approach.

How to handle Western blot (WB) seroindeterminate individuals for Human T-lymphotropic Virus 1/2 (HTLV-1/2) constitutes a challenge for blood banks and families. We made a cross-sectional study of 191 enzyme linked immunoassay (EIA) reactive individuals from the hematological center (HEMOCE) of Fortaleza (Brazil), examining their serological (WB) and molecular (PCR) diagnosis, and demographic profiles, as well as a possible association of their condition with other infectious pathologies and risk factors. Ethical institutional approval and personal consent were obtained. Out of 191 EIA reactive individuals, 118 were WB seroindeterminate and 73 were seropositive for HTLV-1/2. In the PCR analysis of 41 WB seroindeterminate individuals, 9 (22%) were positive and 32 (78%) were negative for HTLV-1/2. The demographic analysis indicated a trend towards a predominance of males among the seroindeterminate individuals and females in the seropositive ones. The seroindeterminate individuals were younger than the seropositive ones. We did not find any association of these conditions with syphilis, Chagas disease or HIV or hepatitis, and with risk factors such as breast-feeding, blood transfusion, STD (syphilis) and IDU.

Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases.

We analyze the results of surgical treatment of 60 patients presenting syringomyelia (SM) associated with Chiari malformation (CM) who were operated in the period 1982-2000. For each case, analysis covered 15 signs and 16 symptoms included in a protocol that separated SM signs and symptoms from those of CM. A score system was established in parallel with the protocol to make the evaluation of treatment results easier. All cases were submitted to craniovertebral decompression by C1 and eventually C2 laminectomy and cerebellar tonsillectomy with duramater graft. To evaluate the results, statistical proportion difference tests and variance analyses were made to a reliability index of 95% (p=0.05). We conclude that the statistical improvement of CM signs and symptoms was very significant (p=0). Syringomyelia signs and symptoms also improved significantly, except for "upper limb hyporeflexia", which did not improve. No statistical difference in the improvement of SM symptoms as compared to CM symptom was found. Syringomyelia signs improved statistically more than CM signs. In half of patients, the percent improvement of signs and symptoms ranged between 40% and 60%.

Pharmacological, morphological and behavioral analysis of motor impairment in experimentally vitamin C deficient guinea pigs.

The scurvy shows an inflammatory disease and gingival bleeding. Nevertheless, in an animal model for guinea pigs, described by Den Hartog Jager in 1985, scurvy was associated with a motor neuron disease with demyelinization of the pyramidal tract, provoking neurogenic atrophy of muscles. Aiming at searching the protective role of vitamin C in nervous system, a pharmacological, morphological and behavioral study was conducted. Three experimental groups were used: A100, animals receiving 100 mg/ vitamin C/ day; A5.0, animals receiving 5.0 mg/vitamin C/ day; and A0, animals without vitamin C. We analyzed the weight gain, muscular diameter and behavioral tests. In all tests examined, we found significant differences between the supplemented groups in comparison with scorbutic group (p<0.05). Thereafter, the animals were killed for histopathology of gastrocnemius muscle, spinal cord and tooth tissues. In addition, a morphometric study of periodontal thickness and alpha-motor neuron cell body diameter were done. The vitamin C-diet free regimen seemed to induce a disruption in spinal cord morphology, involving the lower motor neuron, as confirmed by a significant reduction in neuron perycaria diameter and muscular atrophy, complicated by increased nutritional deficit.

Application of International Classification of Functioning, Disability and Health (ICF) in individuals with spinal cord injury.

UNLABELLED: After spinal cord injury is common functionality is affected. OBJECTIVE: To evaluate the functionality of patients with spinal cord injury. METHOD: Cross-sectional study by means of the International Classification of Functionality (ICF). 109 adults with spinal cord injury in the city of Curitiba, Brazil were evaluated. RESULTS: The categories most compromised in body were intestines and bladder, sexuality, energy, sleep, emotion and weight. In the domain activities and participation, there was greater difficulty in tasks of bathing, toilet and dressing, self care and leisure. In the domain environmental factors, the categories classified as facilitators were: medications, orthoses and wheelchair, attitude of family, transport, social foresight and health services. The categories classified as barriers were: attitude of authorities, social attitudes, education and work. CONCLUSION: The application of the ICF in persons with spinal cord injury demonstrated a series of disabilities and limitations.

Neuropathic pain characteristics in patients from Curitiba (Brazil) with spinal cord injury.

This was a descriptive cross-sectional study on patients with spinal cord injuries living in Curitiba, Paraná, Brazil. The aim was to evaluate the pain characteristics among such patients seen at referral care centers for spinal cord injury patients in Curitiba. A total of 109 adults with spinal cord injury in this city were evaluated regarding the presence of pain, especially neuropathic pain. Neuropathic pain was evaluated using the DN4 questionnaire, a universal instrument that has been translated and validated for Portuguese. A visual analog scale (VAS) was used to evaluate the intensity of pain. The prevalence of pain among these 109 patients was 31.2% (34 patients). The nociceptive pain presented was classified as musculoskeletal pain (nine patients), visceral pain (four patients) and mixed pain (one patient), thus totaling 14 patients (12.8%). Another 20 patients (18.3%) showed symptoms of neuropathic pain and fulfilled the criteria for neuropathic pain with scores greater than 4 out 10 in the DN4 questionnaire. Regarding the characteristics of the patients with neuropathic pain, most of them were male, younger than 40 years of age and paraplegic with incomplete lesions. They had become injured from 1 to more than 5 years earlier. The predominant etiology was gunshot wounds, and the intensity of their pain was high, with VAS scores greater than 5. This study partially corroborates other studies conducted on this subject. Studies of this type are important for understanding the profile of these patients, for the purpose of designing strategies for their rehabilitation, with a focus on the appropriate treatment and management of pain.

Myasthenia gravis in Ceará, Brazil: clinical and epidemiological aspects.

A retrospective chart review was performed on patients diagnosed as having myasthenia gravis in Ceará State, Brazil and who were followed from October 1981 to June 2009. Clinical and epidemiologic aspects were evaluated. In this work, 122 patients were studied, of whom 85 (69.7%) were females and 37 (30.3%) were males. The disease duration ranged from five months to 50 years (8.9±8.1 years). Age at the first symptoms varied from 0 to 74 years (31.9±14.4 years). The first main symptoms and signs were ptosis, diplopia and limb weakness. Generalized myasthenia was the most common clinical presentation, but 5.1% (n=6) persisted as ocular myasthenia. Thymectomy was performed in 42.6% (n=52) of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR) antibodies were present in 80% (n=20) of specimens tested. The data presented are similar to those of studies performed in other countries.

Application of International Classification of Functioning, Disability and Health (ICF) in individuals with spinal cord injury / CIF - Classificação Internacional de Funcionalidade após lesão medular

After spinal cord injury is common functionality is affected. OBJECTIVE: To evaluate the functionality of patients with spinal cord injury. METHOD: Cross-sectional study by means of the International Classification of Functionality (ICF). 109 adults with spinal cord injury in the city of Curitiba, Brazil were evaluated. RESULTS: The categories most compromised in body were intestines and bladder, sexuality, energy, sleep, emotion and weight. In the domain activities and participation, there was greater difficulty in tasks of bathing, toilet and dressing, self care and leisure. In the domain environmental factors, the categories classified as facilitators were: medications, orthoses and wheelchair, attitude of family, transport, social foresight and health services. The categories classified as barriers were: attitude of authorities, social attitudes, education and work. CONCLUSION: The application of the ICF in persons with spinal cord injury demonstrated a series of disabilities and limitations.

Após uma lesão medular, a funcionalidade do indivíduo é comumente afetada e fatores diversos estão envolvidos neste processo. OBJETIVO: Classificar a funcionalidade de pacientes com lesão medular. MÉTODO: Estudo transversal por meio da aplicação da Classificação Internacional de Funcionalidade (CIF). Foram avaliados 109 adultos com lesão medular na cidade de Curitiba, Brasil. RESULTADOS: As categorias mais comprometidas em relação às funções do corpo foram intestino e bexiga, sexualidade, energia, sono, emoção e peso. No domínio atividades e participação, as maiores dificuldades foram nas tarefas de tomar banho, toalete, vestuário, autocuidado e lazer. No domínio fatores ambientais, as categorias classificadas como facilitadores foram: medicamentos, órteses e cadeira de rodas, atitude da família, transporte, previdência social e serviços de saúde. As categorias classificadas como barreiras foram: atitude de autoridades, atitudes sociais, educação e trabalho. CONCLUSÃO: A aplicação da CIF em pessoas com lesão medular permitiu demonstrar uma série de limitações nesses pacientes.

Neuropathic pain characteristics in patients from Curitiba (Brazil) with spinal cord injury / Dor neuropática em pessoas com lesão medular residentes em Curitiba (Brasil)

This was a descriptive cross-sectional study on patients with spinal cord injuries living in Curitiba, Paraná, Brazil. The aim was to evaluate the pain characteristics among such patients seen at referral care centers for spinal cord injury patients in Curitiba. A total of 109 adults with spinal cord injury in this city were evaluated regarding the presence of pain, especially neuropathic pain. Neuropathic pain was evaluated using the DN4 questionnaire, a universal instrument that has been translated and validated for Portuguese. A visual analog scale (VAS) was used to evaluate the intensity of pain. The prevalence of pain among these 109 patients was 31.2 percent (34 patients). The nociceptive pain presented was classified as musculoskeletal pain (nine patients), visceral pain (four patients) and mixed pain (one patient), thus totaling 14 patients (12.8 percent). Another 20 patients (18.3 percent) showed symptoms of neuropathic pain and fulfilled the criteria for neuropathic pain with scores greater than 4 out 10 in the DN4 questionnaire. Regarding the characteristics of the patients with neuropathic pain, most of them were male, younger than 40 years of age and paraplegic with incomplete lesions. They had become injured from 1 to more than 5 years earlier. The predominant etiology was gunshot wounds, and the intensity of their pain was high, with VAS scores greater than 5. This study partially corroborates other studies conducted on this subject. Studies of this type are important for understanding the profile of these patients, for the purpose of designing strategies for their rehabilitation, with a focus on the appropriate treatment and management of pain.

Estudo transversal descritivo em pacientes com lesão medular que residem em Curitiba, Paraná, Brasil. O objetivo foi avaliar as características da dor em pacientes com lesão medular acompanhados em Centros de Reabilitação de referência na cidade. Os sujeitos envolvidos na pesquisa foram 109 pacientes adultos com lesão medular. Foi avaliada a presença de dor, principalmente dor neuropática, mediante aplicação do Questionário DN4, um instrumento universal traduzido e validado para o português. A Escala Visual Analógica (EVA) também foi usada para avaliar a intensidade da dor. A prevalência de dor nos 109 pacientes avaliados foi de 31,2 por cento, ou seja, 34 pacientes. Destes, nove apresentaram dor nociceptiva classificada como músculo-esquelética, quatro dor visceral e um paciente apresentou dor mista, totalizando 14 pacientes (12,8 por cento). Além disso, 20 pacientes (18,3 por cento) apresentaram dor neuropática, cumprindo os critérios para este tipo de dor com uma contagem superior a 4/10 para o DN4. Sobre as características dos pacientes com dor neuropática, a maioria era do sexo masculino, abaixo de 40 anos, paraplégico com lesão incompleta e tempo de evolução entre um e cinco anos de lesão. A etiologia predominante foi perfuração por arma de fogo e a intensidade da dor era severa, com EVA superior a cinco. Este estudo confirma parcialmente outros estudos publicados sobre o tema. Pesquisas deste tipo são importantes para entender o perfil destes pacientes com a finalidade de estabelecer estratégias para uma reabilitação com foco no tratamento e manejo adequado da dor.

Comportamentos espontâneos e induzidos em ratos com dor neuropática por constrição crônica do nervo ciático / Spontaneous and induced behaviors in rats with neuropathic pain by chronic sciatic nerve constriction

JUSTIFICATIVA E OBJETIVOS: A dor neuropática é uma condição frequente e desafiadora no que concerne ao tratamento, necessitando assim do uso de modelos experimentais para a melhor compreensão de seus mecanismos. O objetivo deste estudo foi reproduzir cirurgicamente o modelo de constrição crônica do nervo ciático e quantificar os comportamentos auto-dirigidos sugestivos de dor neuropática crônica experimental durante quatro semanas.MÉTODO: Foram utilizados 21 ratos Wistar divididos em três grupos: normal (n = 7), pseudo-cirúrgico (n = 7) e cirúrgico (n = 7). No grupo pseudo-cirúrgico, procedeu-se apenas a exposição bilateral do nervo ciático, sem lesioná-los. No grupo cirúrgico procedeu-se a exposição do nervo ciático à direita e então constrição com fio mononylon 4.0 em quatro locais, separados por 2 mm de distância. No membro esquerdo, fez-se apenas exposição do nervo ciático, sem lesioná-lo. RESULTADOS: A análise dos comportamentos revelou um aumento significativo (p < 0,05) de coçar-se e morder-se nos membros posteriores direito e esquerdo (em espelho), com predomínio à direita. Além disso, houve diminuição significativa de empinar-se e exploratórios como correr, andar, farejar, e aumento significativo de descansar/dormir e congelar-se. A análise dos testes térmicos revelou presença de alodínia (40º C) e hiperalgesia (46º C) nos membros posteriores direito e esquerdo.CONCLUSÃO: O modelo induziu mononeuropatia ciática dolorosa expressa por comportamentos espontâneos sugestivos de dor crônica, além de alodínia e hiperalgesia.

BACKGROUND AND OBJECTIVES: Neuropathic pain is a frequent and challenging condition in terms of management, thus requiring the use of experimental models to better understand its mechanisms. This study aimed at surgically reproducing chronic sciatic nerve constriction model and at quantifying self-directed behaviors suggestive of experimental chronic neuropathic pain during four weeks.METHOD: Twenty-one Wistar rats were divided in three groups: normal (n = 7), pseudo-surgical (n = 7) and surgical (n = 7). The pseudo-surgical group was submitted to bilateral sciatic nerve exposure without injury. The surgical group was submitted to right sciatic nerve exposure and constriction with mononylon thread 4.0 in four sites 2 mm apart from each other. Left sciatic nerve was exposed without injury. RESULTS: Behavioral analysis has shown significant increase (p < 0.05) in scratching and biting right and left hind limbs (in mirror), predominantly to the right. In addition there has been significant decrease in rearing up and exploratory behaviors such as running, walking and sniffing, and significant increase in resting / sleeping and freezing. Thermal tests analysis has shown allodynia (40º C) and hyperalgesia (46º C) in right and left hind limbs. CONCLUSION: The model has induced painful sciatic single neuropathy expressed by spontaneous behaviors suggestive of chronic pain, in addition to allodynia and hyperalgesia

Myasthenia gravis in Ceará, Brazil: clinical and epidemiological aspects / Miastenia gravis no Ceará, Brasil: aspectos clínicos e epidemiológicos

A retrospective chart review was performed on patients diagnosed as having myasthenia gravis in Ceará State, Brazil and who were followed from October 1981 to June 2009. Clinical and epidemiologic aspects were evaluated. In this work, 122 patients were studied, of whom 85 (69.7 percent) were females and 37 (30.3 percent) were males. The disease duration ranged from five months to 50 years (8.9±8.1 years). Age at the first symptoms varied from 0 to 74 years (31.9±14.4 years). The first main symptoms and signs were ptosis, diplopia and limb weakness. Generalized myasthenia was the most common clinical presentation, but 5.1 percent (n=6) persisted as ocular myasthenia. Thymectomy was performed in 42.6 percent (n=52) of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR) antibodies were present in 80 percent (n=20) of specimens tested. The data presented are similar to those of studies performed in other countries.

Foram analisados, retrospectivamente, os prontuários de pacientes miastênicos, diagnosticados e seguidos entre outubro de 1981 e junho de 2009 no Estado do Ceará, Brasil. Foram coletados dados clínicos e epidemiológicos. Na casuística foram estudados 122 pacientes: 85 (69,7 por cento) do sexo feminino e 37 (30,3 por cento) do sexo masculino. O tempo de doença variou de 5 meses a 50 anos (8,9±8,1 anos). A idade de inicio da doença variou de 0 a 74 anos (31,9±14,4 anos). Na amostra estudada, os primeiros sintomas foram principalmente ptose, diplopia e fraqueza dos membros. A maioria dos pacientes apresentou a forma generalizada, enquanto 5,1 por cento (n= 6) persistiram com miastenia ocular. Timectomia foi realizada em 42,6 por cento (n=52) dos pacientes. Timoma estava presente em 10 pacientes. Anticorpo anti-receptor de acetilcolina foi positivo em 80 por cento (n=20) das amostras testadas. Os aspectos clínicos e epidemiológicos da amostra estudada têm semelhança com aqueles estudados em outros países.

Chewing-like as a possible behavior of persistent orofacial pain in the experimental temporomandibular dysfunction / Mastigação (chewing-like) como possível comportamento de dor orofacial persistente na disfunção temporomandibular experimental.

BACKGROUND AND OBJECTIVES: Stimulations with formalin in the orofacial region can be related to transient or subacute nociceptive activity and behavioral changes. The evaluation of behavioral changes induced by persistent or chronic irritating nociceptive substance has not yet been described.METHOD: Complete Freund's Adjuvant (CFA) was injected in the temporomandibular joint (TMJ) region of rats and analyzed comparing it to the groups treated with saline and 2.5% formalin. In addition, behaviors such as grooming, freezing, rest/sleeping and chewing-like were electronically observed and quantified.RESULTS: It was shown that the chewing-like behavior was significantly increased and that it was inhibited by indometacin (5 mg/kg) and morphine (4 mg/kg). CONCLUSION: These results suggest that chewing-like may be a possible behavior of persistent or chronic orofacial pain, and may be a tool for clinical-pharmacological studies.

JUSTIFICATIVA E OBJETIVOS: Estímulos com formalina na região orofacial podem estar relacionados com a atividade nociceptiva e as alterações comportamentais transitórias ou subagudas. A avaliação de comportamentos sob ação de substância irritante nociceptiva persistente e crônica ainda não foi descrita.MÉTODO: Foi feita injeção de adjuvante completo de Freund (ACF) na região da articulação temporomandibular (ATM) de ratos e foi analisada comparando-a com os grupos tratados com salina e formalina a 2,5%. Além disso, foram observados e quantificados eletronicamente os comportamentos grooming, freezing, rest/sleeping e chewing-like (mastigação). RESULTADOS: Observou-se que o comportamento mastigação (chewing-like) estava significativamente aumentado e que ele foi inibido pela indometacina (5 mg/kg) e morfina (4 mg/kg).CONCLUSÃO: Esses resultados sugerem ser o chewing-like um possível comportamento de dor orofacial persistente, oferecendo-se como instrumento para análise clínico-farmacológica

A hypothetical immunemediated unifying mechanism for the holmes-Adie syndrome

A síndrome de Holmes-Adie consiste de pupila tônica, arreflexia patelar e disfunçäo autômica. As explicaçöes desses diferentes síntomas têm-se baseado em achados neuropatológicos e eletrofisiológicos. Neste trabalho apresenta-se uma hipótese imunológica para a fisiopatogenia da síndrome, de modo semelhante à da síndrome de Guillain-Barré

Discrepancy, coincidence or evidence in chronic idiopathic spastic paraparesis throughout the world: a meta-analysis on 2811 patients

HTLV-I has been associated with a chronic idiopatic spastic paraparesis (CHISPA) in man; however, a complete undestanding of this association is still debated. We selected the most comprehensible papers on this topic between 1985 and 1996, and found that 1261 out of 2811 patients (44.9 percent) reported, throughout the world, were HTLV-I positive. The mean age was 39.5 years and there was a female predominance of 1.9:1. These results do not exclude the causality of HTLV-I as a germen associated to CHISPA; however, other causes) e.g., toxic, immunosuppressors) must be considered as participating in the multistep neurodegeneration observed in CHISPA throughout the world.

Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases

Analisamos os resultados do tratamento cirúrgico de 60 pacientes com a associação de siringomielia (SM) e malformação de Chiari (MC) operados no período de 1982 a 2000. Em cada caso foram analisados 15 sinais e 16 sintomas, incluídos em um protocolo que separou os sinais e sintomas decorrentes da SM dos sinais e sintomas decorrentes da MC. Paralelamente ao protocolo, foi elaborado um sistema de pontuação que facilitou a avaliação dos resultados do tratamento. Todos os casos foram submetidos à descompressão crânio vertebral com laminectomia de C1 e eventualmente de C2, e tonsilectomia cerebelar com plástica de dura-máter. Para avaliação dos resultados, foram aplicados testes estatísticos de diferença de proporções e análise de variância com confiabilidade de 95% (p=0,05). Concluímos que os sinais e sintomas da MC apresentaram melhora estatística muito significativa (p=0). Os sinais e sintomas decorrentes da SM também apresentaram melhora significativa, sendo a exceção o sinal "hiporreflexia de membros superiores" que não melhorou. Não encontrada diferença estatística da melhora dos sintomas da SM quando comparados aos da MC. Os sinais na SM melhoraram estatisticamente mais que os sinais da MC. A metade dos pacientes apresentou percentual de melhora de seus sinais e sintomas na faixa entre 40 e 60%.