RESUMO
Neuromyelitis optica has not been thoroughly studied in Brazilian patients following the discovery of NMO-IgG and its specific antigen aquaporin-4. In this study we aimed to describe the clinical NMO-IgG immunological status and neuroimaging characteristics of recurrent neuromyelitis optica in a series Brazilian patients. We undertook a retrospective study of 28 patients with recurrent neuromyelitis optica, according to 1999 Wingerchuk's diagnostic criteria. Data on NMO-IgG status, clinical features, and MRI findings were analyzed. Three men and 25 women were evaluated. Median age at onset of disease was 26 years (range 7-55); median time of follow-up was 7 years (range 2-14). The mean time elapsed between the first and the second attack was 17 months (median 8.5; range 2-88). NMO-IgG was detected in 18 patients (64.3%). Four patients died due to respiratory failure. Most patients presented with cervical (36%) and cervical-thoracic myelitis (46.4%). Holocord lesion was the most common pattern of involvement (50%) on the axial plane. We did not find a statistical association between myelitis extension and NMO-IgG result. Our series of Brazilian patients showed a younger age of onset than previously reported. In our series, in contrast to previous reports, there was no correlation between the extension of myelitis and NMO-IgG positivity.
Assuntos
Neuromielite Óptica/patologia , Adolescente , Adulto , Idade de Início , Encéfalo/patologia , Brasil/epidemiologia , Criança , Feminino , Humanos , Imunoglobulina G/análise , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/epidemiologia , Neuromielite Óptica/imunologia , Recidiva , Adulto JovemRESUMO
BACKGROUND AND PURPOSE: Hereditary sensory and autonomic neuropathy (HSAN) type V is a very rare disorder. It is characterized by the absence of thermal and mechanical pain perception caused by decreased number of small diameter neurons in peripheral nerves. Recent genetic studies have pointed out the aetiological role of nerve growth factor beta, which is also involved in the development of the autonomic nervous system and cholinergic pathways in the brain. HSAN type V is usually reported not to cause mental retardation or cognitive decline. However, a structured assessment of the cognitive profile of these patients has never been made. METHODS AND RESULTS: We performed a throughout evaluation of four HSAN type V patients and compared their performance with 37 normal individuals. Our patients showed no cognitive deficits, not even mild ones. DISCUSSION AND CONCLUSIONS: Although newer mutations on this and related disorders are continuously described, their clinical characterization has been restricted to the peripheral aspects of these conditions. A broader characterization of this rare disorder may contribute to better understand the mechanisms of the nociceptive and cognitive aspects of pain.
Assuntos
Cognição , Neuropatias Hereditárias Sensoriais e Autônomas/fisiopatologia , Adolescente , Adulto , Criança , Eletromiografia , Feminino , Neuropatias Hereditárias Sensoriais e Autônomas/patologia , Humanos , Masculino , Limiar da DorAssuntos
Encéfalo/patologia , Infecções Bacterianas do Sistema Nervoso Central/diagnóstico , Doença dos Neurônios Motores/diagnóstico , Doenças Musculares/fisiopatologia , Doença de Whipple/diagnóstico , Antibacterianos/uso terapêutico , Encéfalo/microbiologia , Encéfalo/fisiopatologia , Infecções Bacterianas do Sistema Nervoso Central/microbiologia , Infecções Bacterianas do Sistema Nervoso Central/fisiopatologia , Quimioterapia Combinada , Feminino , Humanos , Pessoa de Meia-Idade , Doença dos Neurônios Motores/microbiologia , Doença dos Neurônios Motores/fisiopatologia , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/fisiopatologia , Músculo Esquelético/fisiopatologia , Doenças Musculares/etiologia , Resultado do Tratamento , Doença de Whipple/tratamento farmacológico , Doença de Whipple/fisiopatologia , gama-Globulinas/uso terapêuticoRESUMO
The product of human T-cell lymphotropic virus type 1 (HTLV-1) tax gene has a transactivating effect of the viral and cellular gene expression. Genetic variations in this gene have been correlated with differences in clinical outcomes. Based upon its diversity, two closely related substrains, namely tax A and tax B, have been described. The tax A substrain has been found at a higher frequency among human T-cell leukemia virus type 1 (TSP/HAM) patients than among healthy HTLV-I-infected asymptomatic subjects in Japan. In this study, we determined the distribution of tax substrains in HTLV-I-infected subjects in the city of São Paulo, Brazil. Using the ACCII restriction enzyme site, we detected only tax A substrain from 48 TSP/HAM patients and 28 healthy HTLV-I carriers. The sequenced tax genes from nine TSP/HAM patients and five asymptomatic HTLV-I carriers showed a similar pattern of mutation, which characterizes tax A. Our results indicate that HTLV-I tax subtypes have no significant influences on TSP/HAM disease progression. Furthermore, monophyletic introduction of HTLV-I to Brazil probably occurred during the African slave trade many years ago.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Mutação , Paraparesia Espástica Tropical/virologia , Produtos do Gene tax/genética , Vírus Linfotrópico T Tipo 1 Humano/genética , Variação Genética , Polimorfismo de Fragmento de RestriçãoRESUMO
To evaluate the human T-cell lymphotropic virus type I (HTLV-I) proviral DNA load among asymptomatic HTLV-I-infected carriers and patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP), real time PCR using TaqMan probes for the pol gene was performed in two million peripheral blood mononuclear cells (PBMC). The albumin gene was the internal genomic control and MT2 cells were used as positive control. The results are reported as copies/10,000 PBMC, and the detection limit was 10 copies. A total of 89 subjects (44 HAM/TSP and 45 healthy HTLV-I-infected carriers) followed up at the Institute of Infectious Diseases "Emilio Ribas" and in the Neurology Division of Hospital of Clínicas were studied. The asymptomatic HTLV-I-infected carriers had a median number of 271 copies (ranging from 5 to 4756 copies), whereas the HAM/TSP cases presented a median of 679 copies (5-5360 copies) in 10,000 PBMC. Thus, HAM/TSP patients presented a significantly higher HTLV-I proviral DNA load than healthy HTLV-I carriers (P = 0.005, one-way Mann-Whitney test). As observed in other persistent infections, proviral DNA load quantification may be an important tool for monotoring HTLV-I-infected subjects. However, long-term follow-up is necessary to validate this assay in the clinical setting.
Assuntos
DNA Viral/análise , Vírus Linfotrópico T Tipo 1 Humano/genética , Paraparesia Espástica Tropical/virologia , Provírus/genética , Estudos de Casos e Controles , DNA Viral/genética , DNA Viral/imunologia , Vírus Linfotrópico T Tipo 1 Humano/imunologia , Humanos , Leucócitos Mononucleares/imunologia , Paraparesia Espástica Tropical/imunologia , Reação em Cadeia da Polimerase , Provírus/imunologia , Carga ViralRESUMO
To evaluate the human T-cell lymphotropic virus type I (HTLV-I) proviral DNA load among asymptomatic HTLV-I-infected carriers and patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP), real time PCR using TaqMan probes for the pol gene was performed in two million peripheral blood mononuclear cells (PBMC). The albumin gene was the internal genomic control and MT2 cells were used as positive control. The results are reported as copies/10,000 PBMC, and the detection limit was 10 copies. A total of 89 subjects (44 HAM/TSP and 45 healthy HTLV-I-infected carriers) followed up at the Institute of Infectious Diseases "Emilio Ribas" and in the Neurology Division of Hospital of Clínicas were studied. The asymptomatic HTLV-I-infected carriers had a median number of 271 copies (ranging from 5 to 4756 copies), whereas the HAM/TSP cases presented a median of 679 copies (5-5360 copies) in 10,000 PBMC. Thus, HAM/TSP patients presented a significantly higher HTLV-I proviral DNA load than healthy HTLV-I carriers (P = 0.005, one-way Mann-Whitney test). As observed in other persistent infections, proviral DNA load quantification may be an important tool for monotoring HTLV-I-infected subjects. However, long-term follow-up is necessary to validate this assay in the clinical setting.
Assuntos
Humanos , DNA Viral/análise , Paraparesia Espástica Tropical/virologia , Provírus/genética , Vírus Linfotrópico T Tipo 1 Humano/genética , Estudos de Casos e Controles , DNA Viral/genética , DNA Viral/imunologia , Leucócitos Mononucleares/imunologia , Reação em Cadeia da Polimerase , Paraparesia Espástica Tropical/imunologia , Provírus/imunologia , Carga Viral , Vírus Linfotrópico T Tipo 1 Humano/imunologiaRESUMO
Hypertrophic pachymeningitis is a rare disorder characterized by meningeal thickness, that can be caused by infection, tumoral infiltration, inflammatory disorders or idiopathic. We report the case of a 40 year-old man that presented with longstanding headache and progressive bilateral visual loss and proptosis. Cranial and orbital magnetic resonance imaging revealed diffuse dural thickness and bilateral extraconal orbital lesion. Extensive investigation did not reveal any systemic condition. Histopathological study after meningeal and orbital biopsy disclosed a chronic inflammatory process compatible respectively with idiopathic hypertrophic pachymeningitis (IHP) and orbital pseudotumor. This case emphasizes that orbital involvement can occur in IHP and that its early identification is of great importance in order to improve the visual prognosis of this condition.
Paquimeningite hipertrófica se caracteriza por espessamento das meninges, podendo ser decorrente de infecção, infiltração tumoral, doença inflamatória ou idiopática. Relatamos sobre um homem de, 40 anos, com queixa de cefaléia de longa data e perda progressiva da visão em ambos os olhos acompanhadas de proptose bilateral. A imagem por ressonância magnética de crânio e órbitas revelou espessamento dural difuso e lesão orbitária bilateral. Extensa investigação não revelou qualquer afecção sistêmica. Estudo anatomopatológico realizado após biópsias de meninges e da massa orbitária evidenciou processo inflamatório crônico compatível com paquimeningite hipertrófica idiopática (PHI) e com pseudotumor orbitário respectivamente. Este caso evidencia que o acometimento orbitário pode ocorrer na PHI e que a sua identificação precoce é de fundamental importância para o prognóstico visual.
Assuntos
Adulto , Humanos , Masculino , Dura-Máter , Doenças Orbitárias/etiologia , Hipertrofia/complicações , Meningite/complicações , Biópsia , Doenças Orbitárias/patologia , Dura-Máter/patologia , Hipertrofia/patologia , Imageamento por Ressonância Magnética , Meningite/patologia , Órbita/patologiaRESUMO
The most frequent inherited peripheral neuropathy is the peripheral myelin protein 22 (PMP22) gene related disease. Duplication, deletion, and point mutations in that gene are associated with phenotypic variability. Here we report a family carrying a novel mutation in the PMP22 gene (c. 327C>A), which results in a premature stop codon (Cys109stop). The family members who carry this mutation have a Charcot-Marie-Tooth type 1 variable phenotype, ranging from asymptomatic to severely affected. These findings suggest that the fourth transmembrane domain of the PMP22 gene may play an important role, although the intrafamilial clinical variability reinforces the observation that pathogenic mutations are not always phenotype determinant and that other factors (genetic or epigenetic) modulate the severity of the clinical course.
Assuntos
Doença de Charcot-Marie-Tooth/genética , Códon de Terminação/genética , Mutação , Proteínas da Mielina/genética , Fenótipo , Adolescente , Adulto , Idoso , Axônios/patologia , Axônios/ultraestrutura , Biópsia/métodos , Doença de Charcot-Marie-Tooth/fisiopatologia , Cisteína/genética , Análise Mutacional de DNA/métodos , Saúde da Família , Feminino , Humanos , Masculino , Microscopia Eletrônica/métodos , Pessoa de Meia-Idade , Exame Neurológico/métodos , Linhagem , Reação em Cadeia da Polimerase , Polimorfismo Conformacional de Fita Simples , Nervo Sural/patologia , Nervo Sural/ultraestruturaRESUMO
Many physical agents cause neuropathies. The most common are chronic pressure, vibration and temperature. In general, these lesions occur at work, as a result of accidents or through chronic exposure to the physical agent. Radiation leading to peripheral neuropathy is also related to radiotherapy in cancer treatment, as an undesirable side-effect. We present here a case report of short, intense UV radiation exposure at work, leading to delayed-onset ocular neuropathy. A clear cause-effect relationship is shown, demonstrated using magnetic resonance imaging scans. We suggest that the mechanism was thermal and ischaemic.
Assuntos
Acidentes de Trabalho , Cegueira/etiologia , Doenças Orbitárias/etiologia , Lesões por Radiação/etiologia , Raios Ultravioleta/efeitos adversos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Doenças Orbitárias/diagnósticoRESUMO
In order to assess the suitability of cryopreserved neoplastic tissues for xenografting into nude (nu/nu) mice, we compared the take rate in 28 samples of pancreatic ductal carcinoma. Eleven fresh samples were implanted in nu/nu mice, and 17 were frozen in cryopreserving solution and implanted at a later time. All samples were examined for the presence of neoplastic tissue in cryostat sections. A total of 15 tumors grew in the animals; five from the freshly implanted samples and ten from those cryopreserved. Ten xenografted tumors were characterized for alterations in p53, K-ras, and p16 genes, which were found in six, eight, and nine cases, respectively. Our results demonstrate that the take rate for xenografting is comparable between cryopreserved and fresh tissue samples. The procedure allows for the exchange of tumor material between institutions and permits the establishment of centralized facilities for the storage of an array of different primary tumor samples suitable for the production of in vivo models of cancers.
Assuntos
Carcinoma de Células das Ilhotas Pancreáticas/patologia , Criopreservação , Ductos Pancreáticos/patologia , Neoplasias Pancreáticas/patologia , Adulto , Idoso , Animais , Carcinoma de Células das Ilhotas Pancreáticas/genética , Análise Mutacional de DNA , DNA de Neoplasias/análise , Feminino , Deleção de Genes , Genes p16/genética , Genes p53/genética , Genes ras/genética , Humanos , Masculino , Camundongos , Camundongos Nus , Pessoa de Meia-Idade , Transplante de Neoplasias , Neoplasias Pancreáticas/genética , Reação em Cadeia da Polimerase , Transplante HeterólogoRESUMO
Hyperthermia, skeletal muscle rigidity, rhabdomyolysis, acidosis and multiple system insufficiency characterize malignant hyperthermia. Anaesthetic malignant hyperthermia follows halogenated volatile agents and/or depolarizing muscle relaxants utilization. Diagnosis is based on in vitro muscle contracture in response to halothane and/or caffeine exposure. Neuroleptic malignant syndrome affects patients taking neuroleptic drugs; clinical findings include hyperthermia, extrapyramidal rigidity, acidosis, neurovegetative instability and neurological signs. We report three neuroleptic malignant syndrome patients with positive muscle contracture tests which shows that muscle from neuroleptic malignant syndrome patients may in some instances show alterations similar to those of anaesthetic malignant hyperthermia.
Assuntos
Hipertermia Maligna/etiologia , Síndrome Maligna Neuroléptica/complicações , Adulto , Cafeína , Contratura/etiologia , Suscetibilidade a Doenças/diagnóstico , Feminino , Halotano , Humanos , Masculino , Hipertermia Maligna/diagnósticoRESUMO
We evaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36%) were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80% of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs were less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.
Assuntos
Miastenia Gravis/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/cirurgia , Timoma/cirurgia , Neoplasias do Timo/cirurgiaRESUMO
Five patients with a tumefactive lesion were clinically followed from 1992 to 1993. Four patients were female; age ranged from 32 to 57 years, the duration of symptoms varied from 3 days to 3 years. Neurological examination disclosed dementia in two patients, aphasia in three, hemiparesis in four, hemihypoaesthesia in three, optical neuritis in two, tetraparesis with sensitive level and neurogenic bladder in one. MRI disclosed lesions with a hypersignal on images assessed at T2 and hyposignal at T1, and gadolinium heterogeneous enhancement; these lesions were located in the: a) temporooccipital region bilaterally and brain stem, b) frontoparietal white matter, c) basal ganglia, bilateral white matter and brain stem, d) left parietal region, e) cervical spinal cord, with enlargement of this region. Cerebral biopsy was performed in three patients; acute and subacute demyelinating disease was diagnosed by histological examination. Two patients had an evolutive diagnosis; exclusion of other pathologies and clinical and radiological improvement after corticotherapy, pointed to an inflammatory disease.
Assuntos
Encefalopatias/patologia , Doenças Desmielinizantes/patologia , Imageamento por Ressonância Magnética , Doenças da Medula Espinal/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
We report the case of a 49-year-old woman who has the rare combination of myasthenia gravis and cervical dystonia. She was treated with botulinum toxin type A with good response and no evidence of deterioration of the myasthenic symptoms. We therefore conclude that it is possible to use botulinum toxin in the presence of defective neuromuscular transmission.
Assuntos
Toxinas Botulínicas Tipo A/uso terapêutico , Distonia/tratamento farmacológico , Miastenia Gravis/complicações , Fármacos Neuromusculares/uso terapêutico , Vértebras Cervicais , Distonia/complicações , Feminino , Humanos , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
The peripheral nervous system is frequently involved in systemic vasculitis and it may be helpful in the disease diagnosis. We report on eight patients: seven women and one man; five white, two black and one yellow; age mean 55.9 years; four had polyarteritis nodosa, one had systemic lupus erythematosus, one had isolated peripheral nerve vasculitis and one had livedoid vasculitis. All of them received endovenous therapy with "pulse" of methylprednisolone (1 g/day/3 days) and cyclophosphamide (1 g/1 day). Five patients improved, two remained unchanged and one died. The neurological improvement occurred after the third or fourth pulse and in the patients who have had a shorter time of disease.
Assuntos
Anti-Inflamatórios/administração & dosagem , Ciclofosfamida/administração & dosagem , Metilprednisolona/administração & dosagem , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Vasculite/tratamento farmacológico , Idoso , Feminino , Humanos , Injeções Intravenosas , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/diagnóstico , Nervo Sural/patologia , Vasculite/diagnósticoRESUMO
BACKGROUND: Infection with HTLV-I is etiologically linked with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). However some patients with chronic progressive paraparesis resembling HAM/TSP have been shown to be infected with HTLV-II. OBJECTIVE: To clarify the role of each of these human retroviruses in the etiology of HAM/TSP in São Paulo, Brazil. STUDY DESIGN: A detailed serological and molecular analysis of HTLV-I/II infection was performed in a cohort of 19 patients with HAM/TSP attending a neurological clinic. RESULTS: Plasma samples analyzed for anti-HTLV-I/II antibodies using a Western blot assay, comprising HTLV-I (rgp46I)- and HTLV-II (rgp46II)-specific recombinant env epitopes, demonstrated reactivity to rgp46I and hence were typed as seropositive for HTLV-I. Presence of HTLV genomic sequences in peripheral blood mononuclear cells (PBMC) was sought after by PCR using consensus primers SK 110 and SK 111 for the pol region of HTLV proviral DNA followed by hybridization with type-specific probes--SK 112 (HTLV-I) and SK 188 (HTLV-II). Southern blots from all individuals hybridized with SK 112 but not with SK 188, further confirming HTLV-I infection. Cocultivation of PBMC from eight of these patients with activated lymphocytes from normal individuals resulted in active viral production, detected as presence of soluble p24gag antigen in culture supernatants. Investigation of risk factors for HTLV-I infection in these individuals revealed that five out of 19 patients studied (26.3%) had received blood transfusions previous to disease onset.
Assuntos
Vírus Linfotrópico T Tipo 1 Humano/isolamento & purificação , Paraparesia Espástica Tropical/virologia , Adulto , Idoso , Antígenos Virais/imunologia , Western Blotting , Brasil , Feminino , Produtos do Gene gag/imunologia , Vírus Linfotrópico T Tipo 1 Humano/genética , Vírus Linfotrópico T Tipo 1 Humano/imunologia , Humanos , Leucócitos Mononucleares/virologia , Masculino , Pessoa de Meia-Idade , Análise de Sequência de DNA , Proteínas do Envelope Viral/imunologiaRESUMO
Human T cell lymphotropic virus type-1 (HTLV-1) associated myelopathy/tropical spastic paraparesis (HAM/TSP) has been epidemiologically linked to prior blood transfusion. The prevalence of transfusion as a risk factor for infection varies among endemic areas. Here we report the relative frequency of reported history of blood transfusion among 52 patients evaluated in Sao Paulo, Brazil. A patient reported history of blood transfusion prior to the onset of symptoms, found in 15 (28.8%) of the patients, was the most important risk factor identified in this group of patients when compared with a history of sexually transmitted diseases, homo/bisexuality, sexual promiscuity (three or more sexual partners a year), and intravenous drug use. The mean time between reported transfusions and the onset of symptoms was longer than previously reported. There was no trend toward a more severe evolution to motor inability among the HAM/TSP patients with a history of previous transfusion.
Assuntos
Paraparesia Espástica Tropical/etiologia , Reação Transfusional , Adulto , Idade de Início , Idoso , Brasil , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paraparesia Espástica Tropical/transmissão , Fatores de RiscoRESUMO
The case of 22-year old, white woman with bilateral orbital myositis following an acute upper respiratory tract infection is reported. The most important clinical findings were ocular pain, proptosis, restricted eye motility and swelling of the eyelids. The enlarged eye muscles were seen on orbital computerized tomography scan. The clinical findings of inflammatory orbital myositis and clinical response to corticotherapy are emphasized.
Assuntos
Pseudotumor Orbitário , Doença Aguda , Adulto , Feminino , Glucocorticoides/uso terapêutico , Humanos , Pseudotumor Orbitário/diagnóstico por imagem , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/virologia , Prednisona/uso terapêutico , Infecções Respiratórias/complicações , Tomografia Computadorizada por Raios XRESUMO
Creutzfeldt-Jakob disease (CJD) is a transmissible disease of the nervous system causatively related to the presence of an abnormal prion protein, with dementia, myoclonic jerks, and periodic EEG activity. Fourteen patients (7 females and 7 males) ranging from 26 to 76 years of age (median 59 years) were evaluated between 1974 and 1995 at the Neurologic Clinic of São Paulo University School of Medicine. The average duration of the disease was 12 months (3.5-34 months). Early clinical findings were: behaviour changes in 7 patients, dementia in 4, visual disturbances in 4, vertigo in 2, tremor in 9, and dystonia in one. Advanced symptoms were dementia and myoclonus in all patients. Pyramidal tract dysfunction was found in 6, cerebellar ataxia in 2, seizures in 3, nystagmus and vertigo in 4, and peripheral nervous system involvement in 2. Atypical clinical forms were found in 5 patients. Periodic EEG activity was found in 10 patients. Cerebrospinal fluid evaluation showed pleocytosis in 1 patient, higher protein content in 2, and higher gamma globulin level in 2. In 10 patients anatomopathological evidence in the central nervous system confirmed the clinical diagnosis by presenting with status spongiosus. All except one patient presented with the sporadic form of the disease.
Assuntos
Síndrome de Creutzfeldt-Jakob/diagnóstico , Adulto , Idoso , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Myasthenic gravis may affect both inspiratory and expiratory muscles. Respiratory involvement occurred in almost all patients with myasthenia gravis in all clinical forms of the disease: 332 lung function tests done in 324 myasthenic patients without respiratory symptoms (age 34.6 +/- 18.3 years) were examined. Lung volumes analysis showed that all the patients of both sexes with generalized or ocular myasthenia gravis showed "myasthenic pattern". Male patients with "ocular" form only presented the "myasthenic pattern" with lung impairment and had, from the lung function point of view, a more benign behaviour. Female patients with the "ocular" form exhibited a behaviour of respiratory variables similar to that of the generalized form. It was not observed modification of the variables that suggested obstruction of the higher airways. The "myasthenic pattern" was rarely observed in other neuromuscular diseases, except in patients with laryngeal stenosis.