RESUMO
BACKGROUND: Anti-IgLON5 disease is a rare neurodegenerative tauopathy that displays heterogeneity in clinical spectrum, disease course, cerebrospinal fluid (CSF) findings, and variable response to immunotherapy. Sleep disorders, bulbar dysfunction, and gait abnormalities are common presenting symptoms, and conventional brain MRI scanning is often unrevealing. OBJECTIVE: To provide a comprehensive overview of the literature and to assess the frequency of symptoms, MRI findings, and treatment response in patients with IgLON5 autoimmunity in the serum and CSF or restricted to serum. METHODS: We examined a 65-year-old woman with bulbar-onset IgLON5 disease with serum-restricted antibodies, and we also performed a systematic review of all confirmed cases reported in the English literature. RESULTS: We identified 93 patients, included our case. Clinical data were obtained in 58 subjects, in whom the most frequent symptoms were sleep-disordered breathing, dysphagia, parasomnias, dysarthria, limb or gait ataxia, stridor or vocal cord paresis, movement disorders, and postural instability. Distinct MRI alterations were identified in 12.5% of cases, as opposed to unspecific or unremarkable changes in the remaining patients. T2-hyperintense non-enhancing signal alterations involving the hypothalamus and the brainstem tegmentum were observed only in the present case. Inflammatory CSF was found in half of the cases and serum-restricted antibodies in 4 patients. Treatment with immunosuppressant or immunomodulatory drugs led to sustained clinical response in 19/52 patients. CONCLUSION: Anti-IgLON5 autoimmunity should be considered in patients with sleep disorders, bulbar syndrome, autonomic involvement, and movement disorders, and high-field brain MRI can be of diagnostic help.
Assuntos
Doenças Autoimunes/imunologia , Paralisia Bulbar Progressiva/imunologia , Moléculas de Adesão Celular Neuronais/imunologia , Doenças Hipotalâmicas/imunologia , Doenças Neurodegenerativas/imunologia , Tauopatias/imunologia , Idoso , Doenças Autoimunes/diagnóstico por imagem , Doenças Autoimunes/patologia , Paralisia Bulbar Progressiva/diagnóstico por imagem , Paralisia Bulbar Progressiva/patologia , Feminino , Humanos , Doenças Hipotalâmicas/diagnóstico por imagem , Doenças Hipotalâmicas/patologia , Doenças Neurodegenerativas/diagnóstico por imagem , Doenças Neurodegenerativas/patologia , Tauopatias/diagnóstico por imagem , Tauopatias/patologiaRESUMO
An 82-year-old woman developed neck weakness and dysarthria with antibodies against acetylcholine receptor (AChR) and low-density lipoprotein receptor-related protein 4 (LRP4). Myasthenia gravis (MG) was diagnosed by edrophonium and repetitive nerve stimulation tests. Her symptoms resolved completely by immunotherapy. One year later, she presented with muscle weakness and bulbar palsy accompanied by atrophy and fasciculation. Her tendon reflexes were brisk, and Babinski's sign was positive. She was diagnosed with probable amyotrophic lateral sclerosis (ALS). Immunotherapy did not improve her symptoms, and she ultimately died of respiratory failure. MG and ALS may share a pathophysiology, including anti-LRP4 antibodies at the neuromuscular junction.
Assuntos
Esclerose Lateral Amiotrófica/imunologia , Autoanticorpos/sangue , Proteínas Relacionadas a Receptor de LDL/imunologia , Miastenia Gravis/imunologia , Receptores Colinérgicos/imunologia , Idoso de 80 Anos ou mais , Esclerose Lateral Amiotrófica/complicações , Esclerose Lateral Amiotrófica/terapia , Paralisia Bulbar Progressiva/imunologia , Disartria/imunologia , Feminino , Humanos , Imunoterapia/métodos , Debilidade Muscular/imunologia , Miastenia Gravis/complicações , Miastenia Gravis/terapia , Exame Neurológico , Reflexo de BabinskiRESUMO
BACKGROUND: Transient bulbar palsy without involvement of the facial or extraocular muscles is a rare presentation. It is considered a form of cranial polyneuropathy, a variant of Guillain-Barré syndrome that is related to the autoimmune mechanisms induced by preceding infections or vaccinations. However, drug-induced cranial polyneuropathy has not previously been reported. We describe a boy with isolated bulbar palsy and positive serum antiganglioside antibodies during aripiprazole treatment. PATIENT DESCRIPTION: This 12-year-old boy was admitted with a seven-day history of dysarthria, tongue discomfort, and tinnitus. Three weeks before symptom onset, aripiprazole was added to the patient's medications for attention-deficit hyperactivity disorder. On examination, he showed curtaining of the pharyngeal wall, tongue fasciculation and deviation, and a weak gag reflex. Cranial magnetic resonance imaging suggested lower cranial nerve involvement. Serum anti-GM1 IgG and anti-GD1b IgG antibodies were positive. After stopping aripiprazole, his bulbar symptoms improved. However, on readministration of aripiprazole seven weeks later, dysarthria recurred and again resolved after stopping the drug. CONCLUSION: We describe the first patient with anti-GM1 IgG and anti-GD1b IgG antibodies-associated transient cranial polyneuropathy presenting as isolated bulbar palsy. These findings could be an adverse effect of aripiprazole treatment.
Assuntos
Aripiprazol/efeitos adversos , Paralisia Bulbar Progressiva/induzido quimicamente , Gangliosídeo G(M1)/imunologia , Gangliosídeos/imunologia , Imunoglobulina G/sangue , Psicotrópicos/efeitos adversos , Aripiprazol/uso terapêutico , Transtorno do Deficit de Atenção com Hiperatividade/tratamento farmacológico , Transtorno do Deficit de Atenção com Hiperatividade/imunologia , Paralisia Bulbar Progressiva/sangue , Paralisia Bulbar Progressiva/diagnóstico por imagem , Paralisia Bulbar Progressiva/imunologia , Criança , Humanos , Masculino , Psicotrópicos/uso terapêuticoAssuntos
Autoanticorpos/imunologia , Paralisia Bulbar Progressiva/induzido quimicamente , Edema/induzido quimicamente , Hidroximetilglutaril-CoA Redutases/imunologia , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Músculo Esquelético/efeitos dos fármacos , Doenças Musculares/induzido quimicamente , Sinvastatina/efeitos adversos , Idoso , Azatioprina/efeitos adversos , Biópsia , Paralisia Bulbar Progressiva/diagnóstico , Paralisia Bulbar Progressiva/tratamento farmacológico , Paralisia Bulbar Progressiva/imunologia , Edema/diagnóstico , Edema/tratamento farmacológico , Edema/imunologia , Glucocorticoides/administração & dosagem , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/imunologia , Imunossupressores/administração & dosagem , Masculino , Debilidade Muscular , Músculo Esquelético/patologia , Músculo Esquelético/fisiopatologia , Doenças Musculares/diagnóstico , Doenças Musculares/tratamento farmacológico , Doenças Musculares/imunologia , Necrose , Prednisolona/administração & dosagem , Recuperação de Função Fisiológica , Sinvastatina/imunologia , Resultado do TratamentoRESUMO
We herein report a case of Human T-lymphotropic virus type-I (HTLV-I)-associated myelopathy with bulbar palsy-type amyotrophic lateral sclerosis-like symptoms. A 52-year-old woman developed dyslalia at approximately 40 years of age, which slowly progressed. She presented with muscular atrophy and increased tendon reflexes of the extremities as well as bulbar palsy, from which motor neuron disease was suspected. Cerebrospinal fluid (CSF) testing revealed no abnormalities except for an elevated neopterin concentration at 143.17 pmol/mL (normal ≤30 pmol/mL). Her serum and CSF anti-HTLV-I antibody titers were also high. Intravenous infusions of methylprednisolone decreased the CSF neopterin concentration to 50.33 pmol/mL. Subsequent oral prednisolone therapy was effective in alleviating the symptoms.
Assuntos
Esclerose Lateral Amiotrófica/imunologia , Anti-Inflamatórios/administração & dosagem , Paralisia Bulbar Progressiva/imunologia , Vírus Linfotrópico T Tipo 1 Humano/imunologia , Atrofia Muscular/imunologia , Paraparesia Espástica Tropical/imunologia , Prednisolona/administração & dosagem , Esclerose Lateral Amiotrófica/complicações , Biomarcadores/sangue , Paralisia Bulbar Progressiva/tratamento farmacológico , Paralisia Bulbar Progressiva/fisiopatologia , Feminino , Anticorpos Anti-HTLV-I/sangue , Vírus Linfotrópico T Tipo 1 Humano/efeitos dos fármacos , Humanos , Pessoa de Meia-Idade , Atrofia Muscular/fisiopatologia , Paraparesia Espástica Tropical/tratamento farmacológico , Paraparesia Espástica Tropical/fisiopatologia , Reflexo Anormal , Resultado do TratamentoRESUMO
BACKGROUND: Reactivity against terminal NeuNAc(alpha2-3)Gal, common to several gangliosides such as GD1a, GT1b and GM3, has rarely been reported. The authors recently described a patient with a clinical picture of acute relapsing sensory ataxic neuropathy and bulbar involvement in whom they demonstrated concomitant reactivity against NeuNAc(alpha2-3)Gal and disialosyl epitopes. The authors suggested a correlation between NeuNAc(alpha2-3)Gal reactivity and bulbar involvement. AIM: To determine the frequency of reactivity against terminal NeuNAc(alpha2-3)Gal in acute and chronic immune-mediated disorders, and its possible correlation with bulbar involvement. METHODS: The authors retrospectively reviewed reactivity in the serum of more than 3000 consecutive patients with acute and chronic disorders in which antiganglioside antibodies were studied. The authors selected those patients who were simultaneously positive, by ELISA or thin-layer chromatography, for IgG or IgM antibodies anti-GM3, GD1a and GT1b, and reviewed their clinical features. RESULTS: Reactivity against NeuNAc(alpha2-3)Gal, shared by GM3, GD1a and GT1b gangliosides, was detected in 10 patients: isolated in one patient, and concomitant with reactivity against other gangliosides in the remaining patients. Reactivity against NeuNAc(alpha2-3)Gal was frequently associated (8/10) with symptoms suggestive of bulbar involvement, such as dysphagia, dysarthria or dysphonia. Severe respiratory failure requiring mechanical ventilation was observed in four patients. CONCLUSIONS: Reactivity against the NeuNAc(alpha2-3)Gal epitope is rare and is generally found in association with reactivity against the disialosyl epitope. It can be detected in patients with acute or chronic disorders and could be a serological marker of clinical bulbar involvement and, to a lesser extent, associated with the development of severe respiratory failure.
Assuntos
Paralisia Bulbar Progressiva/imunologia , Paralisia Bulbar Progressiva/fisiopatologia , Gangliosídeo G(M3)/imunologia , Gangliosídeos/imunologia , Imunoglobulina G/imunologia , Imunoglobulina M/imunologia , Especificidade de Anticorpos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosAssuntos
Autoanticorpos/sangue , Músculos Laríngeos/fisiopatologia , Miastenia Gravis/complicações , Miastenia Gravis/fisiopatologia , Receptores Proteína Tirosina Quinases/imunologia , Receptores Colinérgicos/imunologia , Paralisia das Pregas Vocais/imunologia , Biomarcadores/sangue , Paralisia Bulbar Progressiva/sangue , Paralisia Bulbar Progressiva/imunologia , Paralisia Bulbar Progressiva/fisiopatologia , Transtornos de Deglutição/sangue , Transtornos de Deglutição/imunologia , Transtornos de Deglutição/fisiopatologia , Músculos Faciais/inervação , Músculos Faciais/fisiopatologia , Humanos , Músculos Laríngeos/inervação , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/sangue , Debilidade Muscular/imunologia , Debilidade Muscular/fisiopatologia , Miastenia Gravis/imunologia , Junção Neuromuscular/imunologia , Junção Neuromuscular/fisiopatologia , Prednisolona/uso terapêutico , Insuficiência Respiratória/sangue , Insuficiência Respiratória/imunologia , Insuficiência Respiratória/fisiopatologia , Traqueostomia , Resultado do Tratamento , Paralisia das Pregas Vocais/sangue , Paralisia das Pregas Vocais/fisiopatologiaRESUMO
We describe a patient with acute oropharyngeal-facial diplegia, tongue palsy and albuminocytological dissociation following upper respiratory tract infection. Electrophysiological abnormalities in blink reflex suggested a brainstem lesion. High titers of anti-GM3, GD1a and GT1b IgG class serum antibodies were initially detected. Absorption studies indicated that antibodies were directed to a common terminal epitope NeuNAc(alfa 2-3)Gal. This novel antiganglioside antibody specificity may play a role in this unusual regional form of acute bulbar palsy of possible central origin. These data are supportive for extending the panel of antiganglioside specificities with anti-GM3.
Assuntos
Especificidade de Anticorpos , Paralisia Bulbar Progressiva/imunologia , Gangliosídeos/imunologia , Doença Aguda , Ensaio de Imunoadsorção Enzimática , Gangliosídeo G(M3)/imunologia , Humanos , Masculino , Pessoa de Meia-IdadeAssuntos
Autoanticorpos/imunologia , Autoantígenos/imunologia , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/imunologia , Oftalmoplegia/imunologia , Insuficiência Respiratória/etiologia , Especificidade de Anticorpos , Paralisia Bulbar Progressiva/imunologia , Reações Cruzadas , Síndrome de Guillain-Barré/complicações , Humanos , Imunoglobulina G/sangue , Imunoglobulina G/imunologia , Modelos Logísticos , Valor Preditivo dos Testes , Respiração Artificial , Insuficiência Respiratória/imunologia , Insuficiência Respiratória/terapiaRESUMO
We describe a patient with acute isolated bulbar palsy following enteritis. A 29-year-old man developed dysphagia and nasal voice without limb weakness, ataxia, or areflexia. High titres of serum anti-GT1a and anti-Campylobacter jejuni IgG antibodies were detected. He was treated with plasmapheresis, resulting in rapid clinical improvement. This case suggests that an acute isolated bulbar palsy may be caused by a pathology relating to Guillain-Barré syndrome (GBS), in which anti-GT1a IgG antibody may have a role.
Assuntos
Paralisia Bulbar Progressiva/imunologia , Infecções por Campylobacter/imunologia , Campylobacter jejuni/imunologia , Enterite/imunologia , Gangliosídeos/imunologia , Imunoglobulina G/imunologia , Adulto , Autoanticorpos/análise , Paralisia Bulbar Progressiva/sangue , Paralisia Bulbar Progressiva/complicações , Infecções por Campylobacter/complicações , Enterite/complicações , Síndrome de Guillain-Barré/complicações , Humanos , Imunoglobulina G/análise , Masculino , PlasmafereseRESUMO
We reported a 44-year-old woman with Guillain-Barré syndrome (GBS) showing elevations of serum anti-GD1a and anti-GT1b antibody levels. A few days after an upper respiratory infection, she felt numbness in her hands and feet, dysphagia and dysarthria, and weakness in her limbs. On admission, examination showed the paralysis of pharynx and neck, moderate weakness of face and upper limbs, and mild weakness of lower limbs. Sensory deficits were minimal on the distal side of extremities. Deep reflexes were decreased or absent. Laboratory examinations revealed the albumino-cytological dissociation in cerebrospinal fluid and the increase of anti-GD1a and anti-GT1b antibodies in serum. Nerve conduction studies demonstrated axonal damage to the motor nerves. With immunoadsorption therapy, she gradually recovered and the anti-GD1a and anti-GT1b antibodies were normalized. It was reported that the anti-GT1a antibody may be associated with a pharyngeal-cervical-brachial (PCB) variant of GBS (Ropper) and the similar cases including the present case. However, in the present case, the serum anti-GT1a antibody level was not increased, whereas those of anti-GD1a and anti-GT1b antibodies did. Therefore, these antibodies may also play a role in the development of PCB signs in GBS.
Assuntos
Autoanticorpos/sangue , Paralisia Bulbar Progressiva/imunologia , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/imunologia , Adulto , Paralisia Bulbar Progressiva/terapia , Feminino , Síndrome de Guillain-Barré/terapia , Humanos , Técnicas de Imunoadsorção , Resultado do TratamentoRESUMO
Some patients with Guillain-Barré syndrome (GBS) develop bulbar palsy, which may lead to serious complications during the acute phase of the illness. A serological marker that could predict the occurrence of bulbar palsy would be valuable for the treatment of acute GBS. We examined the serum levels of various IgG antiganglioside antibodies in the sera of 16 patients with GBS with bulbar palsy [GBS-BP(+)] and 72 patients with GBS without bulbar palsy [GBS-BP(-)]. Anti-GT1a antibodies were detected in a higher percentage of the GBS-BP(+) patients (10/16, 63%) than the GBS-BP(-) patients (2/72, 3%). In addition to GT1a, a new disialosylganglioside antigen was recognized by the sera of four GBS-BP(+) patients. Anti-GM1b antibodies were also frequently detected in the sera of the GBS-BP(+) cases. However, anti-GM1 and anti-GalNAc-GD1a antibodies, which are highly associated with acute axonal motor neuropathy (AMAN), were not detected in any of the GBS-BP(+) cases, while anti-GM1 antibodies were detected in 29% (21/72) and anti-GalNAc-GD1a antibodies were detected in 8% (6/72) of the GBS-BP(-) cases. These findings suggest that the presence of particular antiganglioside antibodies might be related with certain clinical manifestations of GBS. In patients who are diagnosed with GBS, the presence or absence of anti-GT1a and anti-GM1b antibodies should be tested at the early stage of GBS so that appropriate therapies that prevent the development of bulbar palsy and improve the outcome of GBS, may be initiated.
Assuntos
Paralisia Bulbar Progressiva/imunologia , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/imunologia , Imunoglobulina G/sangue , Adulto , Idoso , Feminino , Gangliosídeo G(M1)/imunologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
OBJECTIVES: To identify valuable antiganglioside antibodies that support the diagnosis of Guillain-Barré syndrome (GBS) and its variants in patients showing bulbar palsy as an initial symptom. METHODS: Medical records of 602 patients with GBS or its variants were reviewed. Fifteen patients had bulbar palsy as an initial symptom. Serum antibodies against GM1, GM1b, GD1a, GalNAc-GD1a, GT1a, and GQ1b were examined in 13 of them. RESULTS: Serum antiganglioside antibodies were positive in 11 (85%) patients. IgG anti-GT1a (n=8; 62%) and anti-GM1b (n=7; 54%) antibodies were often present, whereas all the patients had low or no anti-GM1 antibody activity. High anti-GD1a and anti-GQ1b IgG antibody titres were also present in some patients, but most had higher IgG antibody titres to GM1b or GT1a. All five patients with high IgG antibody titre to GM1b or GT1a only had had antecedent diarrhoea. Some patients with pharyngeal-cervical-brachial weakness (PCB) had IgG antibody to GT1a which did not cross react with GQ1b. Other patients with PCB had antibody to GT1a which cross reacted with GQ1b or antibody to GM1b, but anti-GM1b and anti-GT1a antibodies were not associated with the presence of bulbar palsy. All the patients who had no IgG antiganglioside antibodies recovered completely. CONCLUSIONS: Measurement of serum IgG anti-GT1a and anti-GM1b antibodies gives helpful support for the diagnosis of GBS and its variants when there is early involvement of the oropharyngeal function independently of other neurological findings which appear as the illness progresses.
Assuntos
Anticorpos/sangue , Paralisia Bulbar Progressiva/imunologia , Gangliosídeos/imunologia , Polirradiculoneuropatia/diagnóstico , Adolescente , Adulto , Reações Antígeno-Anticorpo , Criança , Ensaio de Imunoadsorção Enzimática , Feminino , Gangliosídeo G(M1)/análogos & derivados , Gangliosídeo G(M1)/imunologia , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Masculino , Pessoa de Meia-Idade , Polirradiculoneuropatia/imunologiaRESUMO
The pharyngeal-cervical-brachial variant (PCB) of Guillain-Barré syndrome (GBS) has clinical features similar to those of botulism and diphtheria. Mizoguchi et al. (1994) [Mizoguchi, K., Hase, A., Obi, T., Matsuoka, H., Takatsu, M., Nishimura, Y., Irie, F., Seyama, Y., Hirabayashi, Y., 1994. Two species of antiganglioside antibodies in a patient with a pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. J. Neurol. Neurosurg. Psychiatry 57, 1121-1123] reported a patient with PCB-like symptoms who had serum IgG anti-GT1a antibodies which did not cross-react with GQ1b. We assumed that PCB is associated with anti-GT1a antibodies that do not have reactivity to GQ1b and made a serological study of a PCB patient. We searched for PCB patients prospectively and found one with PCB. This patient had IgG anti-GT1a antibodies which were not absorbed with GQ1b in an absorption study, whereas IgG anti-GT1a antibodies from Fisher's syndrome patients were. The frequency of positive IgG anti-GT1a antibody did not differ in patients with and without bulbar palsy. Our findings indicate that IgG anti-GT1a antibodies which do not cross-react with GQ1b are specifically detectable in PCB and can be used as a diagnostic marker of PCB.
Assuntos
Autoanticorpos/análise , Paralisia Bulbar Progressiva/imunologia , Gangliosídeos/imunologia , Polirradiculoneuropatia/imunologia , Adulto , Plexo Braquial/imunologia , Plexo Braquial/fisiopatologia , Plexo Cervical/imunologia , Plexo Cervical/fisiopatologia , Reações Cruzadas , Ensaio de Imunoadsorção Enzimática , Humanos , Imunoglobulina G/análise , Masculino , Fatores de Crescimento Neural/imunologia , Músculos Faríngeos/imunologia , Músculos Faríngeos/inervação , Músculos Faríngeos/fisiopatologia , Estudos ProspectivosAssuntos
Autoanticorpos/sangue , Paralisia Bulbar Progressiva/fisiopatologia , Gangliosídeos/imunologia , Polirradiculoneuropatia/fisiopatologia , Idoso , Ataxia/imunologia , Ataxia/fisiopatologia , Paralisia Bulbar Progressiva/imunologia , Paralisia Bulbar Progressiva/terapia , Humanos , Masculino , Oftalmoplegia/imunologia , Oftalmoplegia/fisiopatologia , Polirradiculoneuropatia/imunologia , SíndromeRESUMO
Immunoglobulin (Ig)-mediated activity in plasma directed towards normal blood type matched red blood cells (RBC) inducing haemolysis in vitro has earlier been demonstrated to be a characteristic feature in ALS-patients. In this study, saliva of ALS-patients, normal and diseased controls was tested with the same in vitro test. An increased degree of haemolysis was induced by the ALS-patient as compared with control samples. The activity thus found in saliva had the same basic characteristics as that earlier described for plasma; it reacted similarly to serial dilution and was retained in salivary Ig. The effect on red blood cells of saliva from patients with bulbar paralysis was larger than that of saliva from ALS-patients lacking bulbar symptoms. It is discussed whether cytotoxic Ig in saliva could be pathophysiologically active in bulbar paralysis by means of passage through the oral mucosa and local action on motor end plates in perioral muscles.
Assuntos
Esclerose Lateral Amiotrófica/imunologia , Autoanticorpos/metabolismo , Paralisia Bulbar Progressiva/imunologia , Membrana Eritrocítica/imunologia , Atrofia Muscular Espinal/imunologia , Saliva/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Histocompatibility antigen (HLA A, B and DR) serotyping was performed on 65 patients with motor neuron disease in the northern region of England and compared to a large control population from the Newcastle upon Tyne area. Thirty two patients had amyotrophic lateral sclerosis, 17 had progressive bulbar palsy and 16 had progressive muscular atrophy. Ten patients had a more slowly progressive course. No significant HLA associations were observed in the motor neuron disease patients. Subdivision of the patients by the clinical course of their disease did not reveal any significant associations. Forty six motor neuron disease patients from the Newcastle upon Tyne area had a reduced frequency of HLA DR4 compared to the local control population. The relevance of histocompatibility antigens to the pathogenesis of motor neuron disease is discussed.
Assuntos
Esclerose Lateral Amiotrófica/imunologia , Paralisia Bulbar Progressiva/imunologia , Antígenos HLA/análise , Antígenos de Histocompatibilidade Classe II/análise , Atrofia Muscular/imunologia , Adulto , Idoso , Esclerose Lateral Amiotrófica/genética , Paralisia Bulbar Progressiva/genética , Inglaterra , Antígenos HLA-DR , Humanos , Pessoa de Meia-Idade , Atrofia Muscular/genética , FenótipoRESUMO
Neurofibrillary degeneration is an argyrophilic intraneuronal lesion found in several unrelated neurologic conditions. The relationship between different types of neurofibrillary tangles is investigated with two monoclonal antibodies raised against Alzheimer neurofibrillary tangles (anti-ANT). Using the peroxidase-antiperoxidase technique, the authors demonstrate that neurofibrillary tangles of progressive supranuclear palsy, containing 15-nm straight filaments, share an antigenic determinant with ANTs. Ultrastructural studies localize the antigenic determinant to filamentous elements in the parakarya. The determinant is not present in normal brain, aluminum-induced experimental tangles in the rabbit, Lewy bodies, Hirano bodies, or axonal filamentous inclusions of amyotrophic lateral sclerosis and giant axonal neuropathy. It is, however, present in ANTs regardless of the pathologic condition in which they are found, including Alzheimer's disease, Down's syndrome, and postencephalitic Parkinson's disease.