RESUMO
OBJECTIVE: To investigate the morphological features of chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies directed against paranodal junctional molecules, particularly focusing on the fine structures of the paranodes. METHODS: We assessed sural nerve biopsy specimens obtained from 9 patients with CIDP with anti-neurofascin-155 antibodies and 1 patient with anti-contactin-1 antibodies. 13 patients with CIDP without these antibodies were also examined to compare pathological findings. RESULTS: Characteristic light and electron microscopy findings in transverse sections from patients with anti-neurofascin-155 and anti-contactin-1 antibodies indicated a slight reduction in myelinated fibre density, with scattered myelin ovoids, and the absence of macrophage-mediated demyelination or onion bulbs. Teased-fibre preparations revealed that segmental demyelination tended to be found in patients with relatively higher frequencies of axonal degeneration and was tandemly found at consecutive nodes of Ranvier in a single fibre. Assessment of longitudinal sections by electron microscopy revealed that detachment of terminal myelin loops from the axolemma was frequently found at the paranode in patients with anti-neurofascin-155 and anti-contactin-1 antibody-positive CIDP compared with patients with antibody-negative CIDP. Patients with anti-neurofascin-155 antibodies showed a positive correlation between the frequencies of axo-glial detachment at the paranode and axonal degeneration, as assessed by teased-fibre preparations (p<0.05). CONCLUSIONS: Paranodal dissection without classical macrophage-mediated demyelination is the characteristic feature of patients with CIDP with autoantibodies to paranodal axo-glial junctional molecules.
Assuntos
Autoanticorpos/análise , Axônios/patologia , Moléculas de Adesão Celular/imunologia , Contactina 1/imunologia , Bainha de Mielina/patologia , Fatores de Crescimento Neural/imunologia , Neuroglia/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Nós Neurofibrosos/patologia , Nervo Sural/patologia , Adolescente , Adulto , Idoso , Axônios/imunologia , Biópsia , Feminino , Humanos , Masculino , Microscopia Eletrônica , Pessoa de Meia-Idade , Bainha de Mielina/imunologia , Neuroglia/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Nós Neurofibrosos/imunologia , Células de Schwann/imunologia , Células de Schwann/patologia , Nervo Sural/imunologia , Adulto JovemAssuntos
Anticorpos/análise , Carcinoma de Células Escamosas/diagnóstico , Doenças Musculares/diagnóstico , Idoso de 80 Anos ou mais , Anticorpos/sangue , Doenças Autoimunes/sangue , Doenças Autoimunes/diagnóstico , Carcinoma de Células Escamosas/sangue , Feminino , Humanos , Hidroximetilglutaril-CoA Redutases/análise , Hidroximetilglutaril-CoA Redutases/sangue , Doenças Musculares/sangueRESUMO
A 55-year-old woman was admitted to our hospital because of gait disturbance and urinary retention that acutely emerged 1 week after severe acute respiratory syndrome coronavirus 2 infection. Acute inflammatory myelopathy was clinically suspected, based on bilateral lower-limb weakness with an extensor plantar response and an elevated immunoglobulin G level in the cerebrospinal fluid. Whole-spine magnetic resonance imaging findings were normal. The central conduction time was extended, based on somatosensory evoked potentials. Her lower-limb weakness was partially ameliorated with immunosuppressive therapy. Postinfectious myelopathy is a rare neurological complication of coronavirus disease 2019 and can develop with normal radiological findings.
Assuntos
COVID-19 , Mielite , Doenças da Medula Espinal , Feminino , Humanos , Pessoa de Meia-Idade , COVID-19/complicações , COVID-19/patologia , Doenças da Medula Espinal/diagnóstico por imagem , Doenças da Medula Espinal/etiologia , Mielite/complicações , Coluna Vertebral , Debilidade Muscular/complicações , Imageamento por Ressonância MagnéticaRESUMO
Autoantibodies against 3hydroxy-3-methylglutaryl-CoA reductase (HMGCR) and the signal recognition particle (SRP) are representative antibodies causing immune-mediated necrotizing myopathies (IMNM), called as anti-HMGCR and anti-SRP myopathies, respectively. Here, we analyzed the differences in routine blood test results between 56 anti-HMGCR and 77 anti-SRP myopathy patients. A higher alanine transaminase (ALT) level and a lower aspartate transaminase (AST)/ALT ratio were observed in anti-HMGCR myopathy patients [ALT, 265.7⯱â¯213.3â¯U/L (mean ± standard deviation); AST/ALT ratio, 0.88⯱â¯0.32] than in anti-SRP-myopathy patients (ALT, 179.3⯱â¯111.2â¯U/L, p < 0.05; AST/ALT ratio, 1.28⯱â¯0.40, p < 0.01). In the active phase, anti-HMGCR myopathy often showed ALT predominance, whereas anti-SRP myopathy often showed AST predominance. In addition, there were differences in erythrocyte sedimentation rate (ESR), total cholesterol (TChol) level, and high-density lipoprotein (HDL) level between anti-HMGCR and anti-SRP myopathies (ESR: HMGCR, 24.4⯱â¯20.8â¯mm/1â¯h; SRP, 35.7⯱â¯26.7â¯mm/1â¯h, pâ¯=â¯0.0334; TChol: HMGCR, 226.7⯱â¯36.6â¯mg/dL; SRP, 207.6⯱â¯40.8â¯mg/dL, pâ¯=â¯0.0163; HDL: HMGCR, 58.4⯱â¯13.9â¯mg/dL; SRP, 46.2⯱â¯17.3â¯mg/dL, p < 0.01). Additional studies on the differences in routine blood test results may further reveal the pathomechanisms of IMNM.
Assuntos
Alanina Transaminase/sangue , Hidroximetilglutaril-CoA Redutases/sangue , Doenças Musculares/sangue , Adulto , Idoso , Autoanticorpos/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
We herein report a case of recurrent multifocal, distal-dominant-sensorimotor neuropathy with ophthalmoplegia, IgM anti-GM1 antibody, and pyrexia-associated relapse. The patient developed sensory disturbance in her limbs after febrile disease at 50 years old. She had experienced several similar episodes and was admitted to the hospital at 56 years old. Based on a pathological study and electrophysiological findings consistent with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), maintenance IVIg therapy was administered and produced partial improvement with no relapse at one-year follow-up. Immunohistochemical studies suggested the presence of IgG (not IgM) anti-myelin antibodies. Chronic neuropathy with ophthalmoplegia and pyrexia-associated relapse may be a unique variant of CIDP.
Assuntos
Oftalmoplegia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Polirradiculoneuropatia , Doença Crônica , Feminino , Febre , Gangliosídeos , Humanos , Imunoglobulina M , Fenótipo , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , RecidivaRESUMO
Objective: To provide evidence that idiopathic inflammatory myopathy (IM) with myasthenia gravis (MG) frequently shows thymoma association and polymyositis (PM) pathology and shares clinicopathologic characteristics with IM induced by immune checkpoint inhibitors (ICIs). Methods: We analyzed the clinicopathologic features of 10 patients with idiopathic IM and MG identified in 970 consecutive patients with biopsy-proven IM. Results: Seven patients (70%) had thymoma. IM and MG were diagnosed with more than 5-year time difference in 6 thymomatous patients and within 1 year in 1 thymomatous and 3 nonthymomatous patients. Seven thymomatous patients showed rhabdomyolysis-like features with respiratory failure (4/7), dropped head (3/7), cardiac involvement (2/7), and positive anti-acetylcholine receptor (anti-AChR) and anti-titin antibodies (7/7 and 4/6, respectively) but rarely showed ocular symptoms (2/7) or decremental repetitive nerve stimulation (RNS) responses (1/7) at IM diagnosis. Three nonthymomatous patients showed acute cardiorespiratory failure with rhabdomyolysis-like features (1/3), positive anti-AChR and anti-titin antibodies (3/2 and 2/2, respectively), and fluctuating weakness of the skeletal muscle without ocular symptoms (3/3). Muscle pathology showed a PM pathology with infiltration of CD8-positive CD45RA-negative T-lymphocytes (9/9), scattered endomysial programmed cell death 1 (PD-1)-positive cells (9/9), and overexpression of programmed cell death ligand 1 (PD-L1) on the sarcolemma of muscle fibers around the infiltrating PD-1-positive cells (7/9). Conclusion: Rhabdomyolysis-like features, positive anti-AChR antibody without decremental RNS responses, and PD-L1 overexpression are possible characteristics shared by ICI-induced IM. Frequent thymoma association in patients with idiopathic IM and MG may suggest thymoma-related immunopathogenic mechanisms, including dysregulation of the immune checkpoint pathway.
Assuntos
Miastenia Gravis/complicações , Miosite/complicações , Polimiosite/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/diagnóstico , Miastenia Gravis/patologia , Miosite/diagnóstico , Miosite/patologia , Polimiosite/diagnóstico , Polimiosite/patologia , Timoma/diagnóstico , Timoma/patologia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologiaRESUMO
We describe a twenty-year follow-up study of antiglycolipid antibodies and electrophysiological results in a 36-year-old man with Campylobacter jejuni-associated Guillain-Barré syndrome (GBS). The patient had a high titer of IgG antibodies to GM1 and GA1 20 years ago. Plasma exchange resulted in full recovery from a bedridden status to independent walking in three weeks, except for residual mild weakness of the bilateral extensor hallucis longus muscles and atrophy of the plantar muscles. Twenty years later, he is unable to run at full pace due to neurological sequelae, and IgG antibodies to GM1 and GA1 were still slightly positive. This case suggests that marked improvement in the acute phase does not necessarily guarantee a subsequent good quality of life (QOL). Optional treatment such as complement inhibitors in the acute phase may be required to achieve better QOL in subsets of patients with GBS.
Assuntos
Gangliosídeo G(M1)/imunologia , Síndrome de Guillain-Barré/imunologia , Imunoglobulina G/sangue , Qualidade de Vida , Adulto , Biomarcadores/sangue , Infecções por Campylobacter , Campylobacter jejuni , Enterite/complicações , Enterite/microbiologia , Seguimentos , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/fisiopatologia , Síndrome de Guillain-Barré/terapia , Humanos , Masculino , Condução Nervosa , Troca Plasmática , Prognóstico , Fatores de TempoRESUMO
A 15-year-old girl presented with non-progressive sensorineural hearing impairment in her right ear since her early childhood. She had normal intellectual development. When she was 15 years old, small deep white matter lesions around the lateral ventricles were incidentally detected in brain MRI studies through further investigation of auditory organs. Laboratory data including cerebrospinal fluid analysis and antibodies to aquaporin-4 or myelin-oligodendrocyte glycoprotein were normal. She was diagnosed as a congenital cytomegalovirus (CMV) infection based on the detection of CMV DNA from preserved umbilical cord tissue by real-time polymerase chain reaction. It should be kept in mind that a case of congenital CMV infection with normal intelligence may be underdiagnosed and that sensorineural hearing impairment from early childhood and deep white matter abnormalities can be key features giving rise to suspicion on congenital CMV infection.
Assuntos
Infecções por Citomegalovirus/congênito , Infecções por Citomegalovirus/diagnóstico , Citomegalovirus/isolamento & purificação , Perda Auditiva Neurossensorial/etiologia , Cordão Umbilical/virologia , Substância Branca/diagnóstico por imagem , Substância Branca/patologia , Adolescente , Citomegalovirus/genética , Infecções por Citomegalovirus/patologia , DNA Viral/isolamento & purificação , Diagnóstico Diferencial , Feminino , Perda Auditiva Neurossensorial/diagnóstico por imagem , Humanos , Inteligência , Testes de Inteligência , Imageamento por Ressonância Magnética , Reação em Cadeia da Polimerase em Tempo Real , Valores de ReferênciaRESUMO
A 19-year-old woman developed high fever, headache, and nausea after taking Loxoprofen for pharyngitis, followed by disturbed consciousness and nuchal stiffness. The patient and her mother had a history of Raynaud's phenomenon. Cerebrospinal fluid (CSF) examination indicated a diagnosis of aseptic meningitis and revealed high levels of Q albumin and IgG index. Anti-RNP antibodies were positive in serum and CSF. Her symptoms disappeared immediately after cessation of Loxoprofen and a drug lymphocyte stimulation test was negative, confirming a diagnosis of non-steroidal anti-inflammatory drugs (NSAIDs)-induced aseptic meningitis. It should be kept in mind that an immune abnormality such as serum and CSF anti-RNP antibodies may play a role in development of NSAIDs-induced aseptic meningitis. A history of usage of NSAIDs and a thorough examination of collagen diseases are useful for identification of the origin of aseptic meningitis in a young woman.
Assuntos
Anti-Inflamatórios não Esteroides/efeitos adversos , Anticorpos Antinucleares/sangue , Anticorpos Antinucleares/líquido cefalorraquidiano , Meningite Asséptica/diagnóstico , Meningite Asséptica/etiologia , Fenilpropionatos/efeitos adversos , Ribonucleoproteínas/imunologia , Acetaminofen/administração & dosagem , Adulto , Anti-Inflamatórios não Esteroides/administração & dosagem , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Autoimunidade , Biomarcadores/sangue , Biomarcadores/líquido cefalorraquidiano , Diagnóstico Diferencial , Substituição de Medicamentos , Feminino , Humanos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: To determine the clinical features of myositis patients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers. METHODS: All 93 myositis patients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed. RESULTS: The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). For these PM, IBM, and unclassifiable patients, their mean ages at diagnosis were 63, 70, and 64 years; autoimmune disease was present in 7 (41%), 13 (19%), and 4 (67%); hepatitis C virus infection was detected in 0%, 13 (20%), and 2 (33%); and p62 was immunopositive in 0%, 66 (94%), and 2 (33%), respectively. Of the treated patients, 11 of 16 PM patients and 4 of 6 p62-immunonegative patients in the unclassifiable group showed responses to immunotherapy, whereas all 44 patients with IBM and 2 p62-immunopositive patients in the unclassifiable group were unresponsive to immunotherapy. CONCLUSIONS: CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis.
Assuntos
Linfócitos T CD8-Positivos/metabolismo , Linfócitos T CD8-Positivos/patologia , Genes MHC Classe I , Miosite/patologia , Miosite/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Imunoterapia , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/metabolismo , Músculo Esquelético/patologia , Miosite/complicações , Miosite/terapia , Proteínas de Ligação a RNA/metabolismo , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Limb girdle muscular dystrophy type 2L (LGMD2L) is an adult-onset slowly progressive muscular dystrophy associated with anoctamin 5 (ANO5) gene mutation, mainly reported from Northern and Central Europe. We report the case of a Japanese male patient with a novel homozygous mutation of c.2394dup, p.Arg799Thrfs in ANO5 gene, the second patient in the Asian population. He had had marked elevation of creatine kinase (CK) level for more than 10 years with minimal muscular symptoms consisting of muscle stiffness and occasional cramps, preceding the onset of proximal limb weakness. Calf hypertrophy and selective fatty replacement of the adductor magnus and gastrocnemius muscles were prominent clinical and muscle imaging features. This case suggests that LGMD2L may affect a broader population than has been previously thought, physicians should consider the possibility of ANO5 mutation even in patients showing elevated CK level with no apparent muscle weakness but muscle stiffness or cramps.
Assuntos
Anoctaminas/genética , Debilidade Muscular/genética , Distrofia Muscular do Cíngulo dos Membros/genética , Mutação , Mialgia/genética , Idade de Início , Povo Asiático/genética , Homozigoto , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/diagnóstico por imagem , Debilidade Muscular/patologia , Debilidade Muscular/fisiopatologia , Músculo Esquelético/patologia , Distrofia Muscular do Cíngulo dos Membros/diagnóstico por imagem , Distrofia Muscular do Cíngulo dos Membros/patologia , Distrofia Muscular do Cíngulo dos Membros/fisiopatologia , Mialgia/diagnóstico por imagem , Mialgia/patologia , Mialgia/fisiopatologiaRESUMO
We aimed to validate the diagnostic utility of enzyme-linked immunosorbent assay (ELISA) for the detection of anti-neurofascin (NF) 155 antibody in 191 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Human NF155-based ELISA clearly distinguished between anti-NF155 antibody-positive and -negative sera. Fifteen CIDP patients (8%) were IgG4 anti-human NF155 antibody-positive, which were confirmed by western blot, cell-based assay and immunohistochemical study. None of disease controls or healthy subjects had positive results. Clinical presentation of IgG4 anti-NF155 antibody-positive patients was consistent with those in previous reports. This ELISA combined with determination of the IgG4 subclass is useful in screening for anti-NF155 antibodies.
Assuntos
Moléculas de Adesão Celular/imunologia , Imunoglobulina G/sangue , Fatores de Crescimento Neural/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Adolescente , Adulto , Idoso , Animais , Moléculas de Adesão Celular/genética , Moléculas de Adesão Celular Neuronais/metabolismo , Ensaio de Imunoadsorção Enzimática , Feminino , Citometria de Fluxo , Proteínas de Fluorescência Verde/genética , Proteínas de Fluorescência Verde/metabolismo , Células HEK293 , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Crescimento Neural/genética , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Ratos , Nervo Isquiático/metabolismo , Estatísticas não Paramétricas , Transfecção , Adulto JovemRESUMO
OBJECTIVE: To show cancer association is a risk factor other than statin exposure for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase autoantibody-positive (anti-HMGCR Ab+) myopathy. METHODS: We analyzed the clinical features and courses of 33 patients (23 female and 10 male) with anti-HMGCR Ab+ myopathy among 621 consecutive patients with idiopathic inflammatory myopathies. RESULTS: Among the 33 patients, 7 (21%) were statin-exposed and 26 were statin-naive. In relation with cancer, there were 12 patients (statin-exposed, n = 4) with cancers detected within 3 years of myopathy diagnosis (cancer association), 3 patients (all statin-naive) with cancers detected more than 3 years before myopathy diagnosis (cancer history), 10 cancer-free patients followed up for more than 3 years (all statin-naive), and 8 patients without cancer detection but followed up for less than 3 years (statin-exposed, n = 3). Therefore, 12 patients with cancer association (36%) formed a larger group than that of 7 statin-exposed patients (21%). Among 12 patients with cancer association, 92% had cancer detection within 1 year of myopathy diagnosis (after 1.3 years in the remaining patient), 83% had advanced cancers, and 75% died of cancers within 2.7 years. Of interest, 1 patient with cancer history had sustained increase in creatine kinase level over 12 years from cancer removal to the development of weakness. CONCLUSIONS: Patients with cancer association formed a large group with poor prognosis in our series of patients with anti-HMGCR Ab+ myopathy. The close synchronous occurrence of cancers and myopathies suggested that cancer association is one of the risk factors for developing anti-HMGCR Ab+ myopathy.
RESUMO
OBJECTIVE: We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association. METHODS: We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis. RESULTS: Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs. CONCLUSIONS: CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM.
Assuntos
Proteínas Reguladoras de Apoptose/imunologia , Autoanticorpos/imunologia , Miosite/complicações , Miosite/imunologia , Neoplasias/complicações , Neoplasias/imunologia , Proteínas Nucleares/imunologia , Autoanticorpos/sangue , Biomarcadores/sangue , Feminino , Humanos , Masculino , Miosite/sangue , Miosite/diagnóstico , Neoplasias/sangue , Neoplasias/diagnóstico , Estudos RetrospectivosRESUMO
We herein describe the case of a 48-year-old woman who presented with nonconvulsive status epilepticus refractory to antiepileptic drugs caused by anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis without any tumors. She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness. On admission to our hospital, she presented with involuntary orofacial movements and central hypoventilation, and an electroencephalogram showed a generalized slow activity consistent with nonconvulsive status epilepticus. The patient's drug-resistant status epilepticus markedly improved following second-line immunotherapy with rituximab and cyclophosphamide. Physicians should consider the early initiation of second-line therapy in certain cases of anti-NMDAR encephalitis.
Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato/complicações , Encefalite Antirreceptor de N-Metil-D-Aspartato/tratamento farmacológico , Anticorpos Monoclonais Murinos/uso terapêutico , Ciclofosfamida/uso terapêutico , Estado Epiléptico/etiologia , Quimioterapia Combinada , Eletroencefalografia , Feminino , Humanos , Pessoa de Meia-Idade , RituximabRESUMO
We describe the case of a 46-year-old man with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis with prominent parkinsonism. The patient presented with psychiatric symptoms followed by epileptic seizure and parkinsonism including micrographia. Magnetic resonance imaging (MRI) revealed lesions in the bilateral medial temporal lobes and basal ganglia on fluid-attenuated inversion recovery images. His symptoms and MRI findings were ameliorated by immunotherapy but then relapsed. After retreatment, his parkinsonism gradually improved except for the micrographia. This is an atypical case of anti-NMDAR encephalitis in that the patient showed prominent and refractory parkinsonism, thus indicating that the clinical diversity of anti-NMDAR encephalitis is greater than expected.
Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato/complicações , Encefalite Antirreceptor de N-Metil-D-Aspartato/fisiopatologia , Transtornos Parkinsonianos/complicações , Transtornos Parkinsonianos/fisiopatologia , Adulto , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/terapia , Discinesias , Humanos , Imunoterapia , Imageamento por Ressonância Magnética , MasculinoRESUMO
We report an 85-year-old man presenting with wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome with cerebellar ataxia and facial dysesthesia. He experienced an abrupt onset of double vision and exotropia of the right eye with unsteady gait and dysesthesia around upper lip. He was admitted to our hospital ten days after the onset of the double vision. On admission, he presented with WEBINO, left limb ataxia, and dysesthesia around upper lip on the right side. His exotropia was prominent on the right side. Diffusion weighted images of MRI revealed a high intensity lesion in the paramedian pontine tegmentum involving bilateral medial longitudinal fasciculus (MLF), consistent with acute ischemic lesion. Four months after the onset, the WEBINO persisted, without cerebellar ataxia and facial dysesthesia. Putative lesions of the WEBINO, cerebellar ataxia and facial dysesthesia were bilateral MLF, left superior cerebellar peduncle and trigeminothalamic tract, respectively, which were broader than the MRI lesion. Neurological examination is critical for evaluation of accurate ischemic area.