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3.
J Peripher Nerv Syst ; 28(3): 407-414, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37288802

RESUMO

BACKGROUND AND AIMS: Guillain-Barré syndrome (GBS) is a rare, acute neuropathy characterized by ascending muscle weakness. Age, axonal GBS variants, and antecedent Campylobacter jejuni infection are associated with severe GBS, but the detailed mechanisms of nerve damage are only partly explored. Pro-inflammatory myeloid cells express NADPH oxidases (NOX) that generate tissue-toxic reactive oxygen species (ROS) that are implicated in neurodegenerative diseases. This study analyzed the impact of variants of the gene encoding the functional NOX subunit CYBA (p22phox ) on acute severity, axonal damage, and recovery in adult GBS patients. METHODS: Extracted DNA from 121 patients was genotyped for allelic variation at rs1049254 and rs4673 within CYBA using real-time quantitative polymerase chain reaction. Serum neurofilament light chain was quantified by single molecule array. Patients were followed for severity and motor function recovery for up to 13 years. RESULTS: CYBA genotypes linked to reduced formation of ROS, i.e. rs1049254/G and rs4673/A, were significantly associated with unassisted ventilation, shorter time to normalization of serum neurofilament light chain and shorter time to regained motor function. Residual disability at follow-up was confined to patients carrying CYBA alleles associated with high formation of ROS. INTERPRETATION: These findings implicate NOX-derived ROS in GBS pathophysiology and CYBA alleles as biomarkers of severity.


Assuntos
Síndrome de Guillain-Barré , Adulto , Humanos , Alelos , Biomarcadores , Síndrome de Guillain-Barré/genética , Síndrome de Guillain-Barré/fisiopatologia , NADPH Oxidases/genética , Espécies Reativas de Oxigênio , Gravidade do Paciente
4.
Neurology ; 100(23): e2386-e2397, 2023 06 06.
Artigo em Inglês | MEDLINE | ID: mdl-37076309

RESUMO

BACKGROUND AND OBJECTIVES: To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study. METHODS: Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/µL). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%). RESULTS: In 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/µL in 1,005 patients (83%), 5-49 cells/µL in 200 patients (16%), and ≥50 cells/µL in 13 patients (1%). DISCUSSION: ACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/µL, is compatible with GBS after a thorough exclusion of alternative diagnoses. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.


Assuntos
Síndrome de Guillain-Barré , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Contagem de Células , Líquido Cefalorraquidiano/citologia , Estudos de Coortes , Progressão da Doença , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/patologia , Síndrome de Guillain-Barré/fisiopatologia , Internacionalidade , Síndrome de Miller Fisher/líquido cefalorraquidiano , Síndrome de Miller Fisher/diagnóstico , Síndrome de Miller Fisher/patologia , Síndrome de Miller Fisher/fisiopatologia , Prognóstico , Resultado do Tratamento
5.
Clin Neurophysiol ; 133: 20-28, 2022 01.
Artigo em Inglês | MEDLINE | ID: mdl-34800836

RESUMO

OBJECTIVE: This study was undertaken to establish by a multicentric approach the reliability of a new technique evaluating motor axon excitability. METHODS: The minimal threshold, the lowest stimulus intensity allowing a maximal response by 1 mA increments (iUP) and then by 0.1 mA adjustments (iMAX) were prospectively derived from three nerves (median, ulnar, fibular) in four university centers (Liège, Marseille, Fraiture, Nice). iMAX procedure was applied in 28 healthy volunteers (twice) and 32 patients with Charcot-Marie-Tooth (CMT1a), chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barré syndrome (SGB) or axonal neuropathy. RESULTS: Healthy volunteers results were not significantly different between centers. Correlation coefficients between test and retest were moderate (> 0.5). Upper limits of normal were established using the 95th percentile. Comparison of volunteers and patient groups indicated significant increases in iMAX parameters especially for the CMT1a and CIDP groups. In CMT1a, iMAX abnormalities were homogeneous at the three stimulation sites, which was not the case for CIDP. CONCLUSIONS: The iMAX procedure is reliable and allows the monitoring of motor axon excitability disorders. SIGNIFICANCE: The iMAX technique should prove useful to monitor motor axonal excitability in routine clinical practice as it is a fast, non-invasive procedure, easily applicable without specific software or devices.


Assuntos
Axônios/fisiologia , Nervo Mediano/fisiologia , Neurônios Motores/fisiologia , Condução Nervosa/fisiologia , Nervo Fibular/fisiologia , Nervo Ulnar/fisiologia , Adulto , Idoso , Doença de Charcot-Marie-Tooth/fisiopatologia , Feminino , Síndrome de Guillain-Barré/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/fisiopatologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Estudos Prospectivos , Adulto Jovem
6.
Neurol Res ; 44(2): 121-127, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34382919

RESUMO

OBJECTIVE: This study aimed to explore the correlation of elevated glucose levels in the blood and cerebrospinal fluid with the progression and short-term prognosis of Guillain-Barré syndrome (GBS). METHODS: The medical records of 982 patients who were diagnosed with GBS in 31 representative tertiary hospitals, located in 14 provinces in southern China, were collected and retrospectively reviewed. Patients were grouped according to the levels of fasting plasma glucose (FPG) and cerebrospinal fluid (CSF) glucose, as well as the concentration of blood hemoglobinAlc (HbA1c). The Hughes grade scale was used to quantify functional outcomes. RESULTS: Compared to patients with normal FPG and CSF glucose levels, those in the high FPG and high CSF glucose groups were characterized by a higher proportion of severe patients (HFGS ≥ 3) at admission (58.8 vs. 73.1, P = 0.000; 57.6 vs. 71.2, P = 0.000), at nadir (67.4 vs. 83.0, P = 0.000; 66.2 vs. 80.4, P = 0.000), and at discharge (29.8 vs. 46.3, P = 0.000; 26.4 vs. 45.0, P = 0.000). Patients in the high HbA1c group also had more severe disability at admission (74.6 vs. 56.1, P = 0.005) and at nadir (80.3 vs. 64.3, P = 0.012) compared to the normal HbA1c group. Moreover, elevated levels of FPG and CSF glucose were significantly correlated with more severe disability at admission, at nadir, and at discharge. CONCLUSIONS: The present study showed that elevated glucose levels in the blood and cerebrospinal fluid were associated with the severity and short-term prognosis of GBS. TRIAL REGISTRATION: chicTR-RRc-17,014,152. ABBREVIATIONS: GBS, Guillain-Barré syndrome; FPG, fasting plasma glucose; CSF, cerebrospinal fluid; HFGS, Hughes Functional Grading Scale; HbA1c, hemoglobin A1c. DM, diabetes mellitus; NCS, nerve conduction study; AIDP, acute inflammatory demyelinating polyneuropathy; AMAN, acute motor axonal neuropathy; AMSAN, acute motor sensory axonal neuropathy; MV, mechanical ventilation.


Assuntos
Progressão da Doença , Glucose/metabolismo , Síndrome de Guillain-Barré , Adulto , Glicemia , Feminino , Glucose/líquido cefalorraquidiano , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Gravidade do Paciente , Prognóstico , Estudos Retrospectivos , Fatores de Tempo
7.
Diabetes Metab Syndr ; 15(6): 102326, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34731822

RESUMO

BACKGROUND AND AIMS: The COVID-19 pandemic has turned the world topsy turvy since its emergence and has claimed innumerable lives worldwide. Neurological manifestations of the disease have raised several eyebrows around the world among which Guillain-Barré syndrome (GBS) deserve special mention. Although majority of the cases of the coronavirus disease 2019 (COVID-19) present with respiratory symptoms, extrapulmonary manifestations are being increasingly reported. We conducted this study to analyze detailed clinical presentations and outcome in a series of eight cases (n = 8) with COVID-19 associated GBS. METHODS: An observational prospective study was conducted among patients with post-infectious/para-infectious GBS. 8 patients were subclassified into acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN) as per electrodiagnostic criteria and were followed up from admission to 6 months post discharge, to obtain a comprehensive clinical profile and outcome in these patients. RESULTS: The diagnosis of GBS was confirmed as per Asbury criteria, supported by electrodiagnostic features in nerve conduction velocity test. Among the series of 8 patients, 3 were diagnosed as AIDP, 3 had AMAN and the remaining 2 patients had AMSAN. 3 patients of GBS were afebrile and were diagnosed as COVID-19 after a positive assay on routine screening. Cerebro-spinal fluid analysis for SARS-Cov-2 RT-PCR and serum anti-ganglioside antibodies were negative in all the patients. CONCLUSION: GBS in patients with COVID-19 should be differentiated from critical illness neuropathy and myopathy. Early diagnosis is important as it is associated with poor outcome and prolonged invasive ventilation.


Assuntos
COVID-19/complicações , Síndrome de Guillain-Barré/virologia , Adulto , Idoso , Feminino , Síndrome de Guillain-Barré/classificação , Síndrome de Guillain-Barré/fisiopatologia , Síndrome de Guillain-Barré/terapia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Respiração Artificial
8.
Rev Neurol ; 73(9): 315-320, 2021 11 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34676529

RESUMO

INTRODUCTION: To describe clinical characteristics and electrophysiological variants of GBS cases during the pandemic, we carried out a comparative analysis between SARS-CoV2 related GBS and non-SARS-CoV2 patients and then compared to the 2019 cases. PATIENTS AND METHODS: We carried out a cross-sectional study of GBS patients diagnosed according to Asbury and Cornblath criteria. We collected information on clinical and paraclinical variables. We defined a SARS-CoV-2 related GBS case according to the description of Ellul et al. We used Hadden criteria to classify the electrophysiological variants. We performed a comparative analysis between groups. RESULTS: Fourty-two patients were diagnosed with GBS in 2020, men 64.2%, age 46 ± 17.4 years, patients with obesity/overweight 42.8%, previous diarrhea 31%, history of respiratory tract infection 14.2%. Guillain Barre Disability Scale = 3 points 71.4% and, cranial nerve involvement 69%. The most frequent electrophysiological variant was acute inflammatory demyelinating polyradiculoneuropathy (AIDP) 53.5%. Seven (16.6%) cases were SARS-CoV2 related, four men, age 43.4 ± 13.4 years. When comparing patients with GBS in 2020 vs patients in 2019, we observed a decrease in the previous infection history during 2020 (45.2% vs 73.3%, p-value = 0.005) and a decrease in previous respiratory infection (14.2% vs 33.3%, p = 0.045), as well as a higher frequency of cranial nerve involvement, and albuminocytologic dissociation. CONCLUSIONS: SARS-CoV2 virus infection preventive measures may be impacting the presentation of post-infectious diseases such as GBS. We did not observe an increase in GBS cases during 2020. Also, the AIDP variant were more frequent in our population in the COVID-19 pandemic.


TITLE: Síndrome de Guillain-Barré durante la pandemia de COVID-19: experiencia de un centro de referencia en México.Introducción. Se trata de describir las características clínicas y variantes electrofisiológicas de los casos de síndrome de Guillain-Barré (SGB) durante la pandemia. Llevamos a cabo un análisis comparativo entre pacientes con SGB relacionado con el SARS-CoV-2 y sin antecedente del virus, y posteriormente realizamos una comparación con los casos de 2019. Pacientes y métodos. Se llevó a cabo un estudio transversal de los pacientes con diagnóstico de SGB según los criterios de Asbury y Cornblath. Se recolectaron información clínica y variables paraclínicas. Definimos el SGB relacionado con el SARS-CoV-2 conforme a la descripción de Ellul et al. Se utilizaron los criterios de Hadden para la clasificación de las variantes electrofisiológicas. Por último, realizamos un análisis comparativo entre grupos. Resultados. Se diagnosticó a 42 pacientes con SGB en 2020, un 64,2% hombres, con una edad de 46 ± 17,4 años, un 42,8% con obesidad/sobrepeso, un 31% con historia de diarrea previa y un 14,2% con infección respiratoria previa. El 71,4% tuvo una puntuación en la Guillain-Barré Disability Score igual o mayor que 3 puntos y el 69% tenía afectados los nervios del cráneo. La variante electrofisiológica más común fue la polirradiculoneuropatía desmielinizante inflamatoria aguda (PDIA; 53,5%). Siete (16,6%) casos tuvieron relación con el SARS-CoV-2, cuatro hombres, con edad de 43,4 ± 13,4 años. Al realizar la comparación entre pacientes con SGB de 2020 frente a los de 2019, observamos un decremento en el antecedente de infección previa en 2020 (45,2 frente a 73,3%; p = 0,005) y un decremento específico en la historia de infección respiratoria (14,2 frente a 33,3%; p = 0,045), así como una mayor frecuencia de afectación de los nervios del cráneo y de disociación albuminocitológica. Conclusiones. Las maniobras preventivas para la infección por el SARS-CoV-2 impactan directamente en la presentación de enfermedades postinfecciosas como el SGB. No observamos un incremento en los casos de SGB durante 2020. Asimismo, la variante de PDIA fue la más frecuente en nuestra población durante la pandemia de COVID-19.


Assuntos
COVID-19/complicações , Síndrome de Guillain-Barré/complicações , Adulto , Estudos Transversais , Feminino , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/fisiopatologia , Instalações de Saúde , Humanos , Masculino , México , Pessoa de Meia-Idade , Encaminhamento e Consulta
9.
Biomed Res Int ; 2021: 9800488, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34458371

RESUMO

Immune checkpoint inhibitors (ICIs) have been increasingly used in the treatment of various types of tumors with favorable results. But these treatments also led to a variety of immune-related adverse events (irAEs). Neurological irAEs such as Guillain-Barré Syndrome are rare and may have serious consequences once they occur. A systematic literature search was performed in PubMed and Embase for all case reports of GBS associated with ICIs published in English reporting on human beings from 1990 up to date. A total of 30 case reports (total patients = 33) were used for final analysis. The included cases were from 11 countries, covering 10 tumor types, with melanoma accounting for the largest number. The mean age was 62.2 ± 11.1 years old, and males were dominant (male: 26 and female: 7). The median time of initial symptoms was 8.2 weeks after the 1st dose of ICIs. The most common manifestations of GBS associated with ICIs were weakness, hyporeflexia or areflexia, and paresthesia in order. The GBS subtypes suggested by electrophysiological results were acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and Miller Fisher syndrome (MFS). The protein level of CSF in patients with GBS related to ICIs was 180.68 ± 152.51 mg/dl. Immediate termination of ICIs followed by intravenous immunoglobulin was the preferred treatment option. 72.7% of patients recovered or had residual mild dysfunction after treatment. Elderly male patients with melanoma were most likely to develop ICI-related GBS. The specific neurological symptoms, CSF analysis, and electrophysiological examination were important means of diagnosis.


Assuntos
Síndrome de Guillain-Barré/induzido quimicamente , Inibidores de Checkpoint Imunológico/efeitos adversos , Neoplasias/tratamento farmacológico , Polineuropatias/etiologia , Fenômenos Eletrofisiológicos , Síndrome de Guillain-Barré/imunologia , Síndrome de Guillain-Barré/fisiopatologia , Humanos , Inibidores de Checkpoint Imunológico/imunologia , Inibidores de Checkpoint Imunológico/uso terapêutico , Imunoterapia/métodos , Neoplasias/metabolismo , Neoplasias/patologia , Farmacovigilância , Polineuropatias/patologia , Resultado do Tratamento
11.
Pan Afr Med J ; 38: 356, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34367435

RESUMO

Pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré Syndrome (GBS) is characterized by weakness in cervicobrachial and oropharyngeal region, together with areflexia of upper limbs. Being an uncommon variant, it is often misdiagnosed as other neurological conditions resembling GBS. Although most of the cases occur as a post-infectious complication, no reports describing its development following dengue-chikungunya co-infection have been documented. A young female presented with a progressive history of swallowing difficulty, bilateral arm weakness and neck weakness. Three weeks earlier, she was presented with clinical features corresponding to dengue and was symptomatically treated. Currently, hypotonia and decreased muscle strength were observed in both upper limbs and neck. Detailed investigation revealed the presence of Immunoglobulin M (IgM) antibodies against dengue antigen (NS 1) and Chikungunya virus (CHIKV), confirming the possibility of previous dengue-chikungunya co-infection. Nerve conduction studies and electromyography of upper limbs pointed towards findings consistent with the early stages of acute motor demyelinating and possible axonal neuropathy. The detection of antiganglioside antibodies (anti-GT1a antibodies), confirmed the diagnosis of the pharyngeal-cervical-brachial variant of GBS. A five days treatment of intravenous immunoglobulin (IVIG) along with physical rehabilitation was started which led to significant improvement and the patient was discharged after 15 days. PCB is an unfamiliar variant of GBS for many clinicians. Diagnosis can be made by a thorough history, clinical examination and investigations that can rule out other potential causes of cervicobrachial and oropharyngeal weakness. It also necessitates careful monitoring and followups after mono- and co-arboviral infections to prevent any debilitating neurological complications.


Assuntos
Febre de Chikungunya/complicações , Dengue/complicações , Síndrome de Guillain-Barré/diagnóstico , Adulto , Febre de Chikungunya/diagnóstico , Febre de Chikungunya/terapia , Coinfecção , Dengue/diagnóstico , Dengue/terapia , Feminino , Síndrome de Guillain-Barré/fisiopatologia , Síndrome de Guillain-Barré/terapia , Humanos , Imunoglobulina M/imunologia , Imunoglobulinas Intravenosas/administração & dosagem
12.
Neurol Res ; 43(10): 793-801, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34101535

RESUMO

Objective: In the young generations with nitrous oxide abuse (N2O), featured electrophysiological response of the peripheral neuropathy caused by nitrous oxide remains to be defined.Methods: Patients with nitrous oxide abuse (20 cases), two variants of Guillain-Barré syndrome (GBS), that is, acute inflammatory demyelinating polyradiculoneuropathy (GBS-AIDP, 19 cases) and acute motor axonal neuropathy (GBS-AMAN, 18 cases), as well as diabetic peripheral neuropathy (DPN, 20 cases) were enrolled into this study. Electrophysiological parameters including distal motor latency (DML), motor nerve conduction velocity (MNCV), sensory nerve conduction velocity (SNCV), amplitudes of compound muscle action potential (CMAP), and sensory nerve action potential (SNAP) were measured and analyzed by comparing the parameters between the aforementioned patients groups as well as normal control group (20 subjects).Results: Compared to normal control subjects, patients with nitrous oxide abuse showed prolonged DML, slower MNCV and SNCV in the limbs, lower amplitudes of CMAP in the median, tibial and peroneal nerves, and lower SNAP in median and ulnar nerves. Abnormalities of MNCV and amplitudes of CMAP in the lower limbs were significantly higher than that in the upper limbs . Abnormal electrophysiological features of patients with nitrous oxide abuse were dramatically different from those in GBS-AIDP or DPN patients, but similar to those in GBS-AMAN patients.Conclusions: Nitrous oxide abuse could cause abnormal electrophysiological response in the limbs. Some of the parameters (DML, MNCV, SNCV, CMAP and SNAP) appeared significantly different between the patients with nitrous oxide abuse, GBS with AIDP or AMAN, and DPN patients.Significance: Electrophysiological examination could be considered as an important supporting factor in differential diagnosis for nitrous oxide abuse, GBS with AIDP or AMAN, and DPN.


Assuntos
Neuropatias Diabéticas/fisiopatologia , Síndrome de Guillain-Barré/fisiopatologia , Condução Nervosa/fisiologia , Óxido Nitroso/toxicidade , Adolescente , Adulto , Extremidades/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Relacionados ao Uso de Substâncias/fisiopatologia
13.
Sci Rep ; 11(1): 11578, 2021 06 02.
Artigo em Inglês | MEDLINE | ID: mdl-34079013

RESUMO

Guillain-Barré syndrome (GBS) is a neurological disorder characterized by paralysis. Identifying the severity, appropriate therapeutic method, and prognosis of GBS at an early stage is highly important. This study aimed to investigate the modifiable risk factors for the severity of GBS and consequent need for mechanical ventilation (MV) and to identify clinical predictive factors for poor short-term outcomes of severe GBS. 155 GBS patients who were admitted to the Affiliated Yantai Yuhuangding Hospital of Qingdao University during 2014-2020 were enrolled. Demographic, clinical, therapeutic and evolutionary data were collected and were then analyzed using univariate and multivariate regression analyses. Our analytic data demonstrated that the significant clinical predictors of severe GBS were recent history of surgery, older age, cranial nerve impairment, and elevated levels of liver enzymes (p < 0.05). Furthermore, autonomic dysfunction, lower Medical Research Council (MRC) score at nadir, and elevated levels of liver enzymes were significantly associated with MV for severe GBS (p < 0.05), and lower MRC score at nadir and autonomic dysfunction remained significant predictors of MV in severe GBS (p < 0.05). Lastly, recent history of surgery, lower MRC score at admission and at nadir, requirement for MV, and pneumonia during hospitalization were significantly associated with the short-term outcome of severe GBS and that lower MRC score at admission and need for MV were confirmed to be predictors of poor short-term prognosis (p < 0.05). Of note, this study suggested that recent history of surgery is a predictor of severity in GBS patients and is associated with the poor short-term prognosis of severe GBS.


Assuntos
Síndrome de Guillain-Barré/fisiopatologia , Adulto , Idoso , Síndrome de Guillain-Barré/terapia , Humanos , Pessoa de Meia-Idade , Prognóstico , Respiração Artificial , Fatores de Risco , Índice de Gravidade de Doença
14.
Clin Neurol Neurosurg ; 206: 106691, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34022690

RESUMO

Miller-Fisher syndrome (MFS) together with Guillan-Barré syndrome (GBS) and Bickerstaff brainstem encephalitis (BBE) are considered to form a continuous clinical spectrum of the same disease, possibly affecting the peripheral and/or central nervous systems, with monophasic symptoms. The frequency of overlapping clinical signs and the risk of recurrence are independent and very low, but no cases of GQ1b-seropositive recurrent MFS overlapping with GBS and BBE have been described so far. Here, we describe for the first time an atypical case of recurrent GQ1b-seropositive MFS overlapping GBS and BBE, 12 years after a previous GQ1b-seronegative typical MFS episode. Our case expands the clinical spectrum of recurrent MFS, and it should prompt clinicians to investigate the presence of anti-ganglioside antibodies in recurrent MFS even when these were negative in the previous episode, especially in those presenting with overlapping spectrum symptoms and a critically ill picture during the second episode.


Assuntos
Encefalite , Síndrome de Guillain-Barré , Síndrome de Miller Fisher , Adulto , Autoanticorpos/imunologia , Autoantígenos/imunologia , Tronco Encefálico , Encefalite/imunologia , Encefalite/fisiopatologia , Feminino , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/imunologia , Síndrome de Guillain-Barré/fisiopatologia , Humanos , Síndrome de Miller Fisher/imunologia , Síndrome de Miller Fisher/fisiopatologia , Recidiva
15.
Neurophysiol Clin ; 51(2): 183-191, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33685769

RESUMO

OBJECTIVE: To assess whether patients with acute inflammatory demyelinating polyneuropathy (AIDP) associated with SARS-CoV-2 show characteristic electrophysiological features. METHODS: Clinical and electrophysiological findings of 24 patients with SARS-CoV-2 infection and AIDP (S-AIDP) and of 48 control AIDP (C-AIDP) without SARS-CoV-2 infection were compared. RESULTS: S-AIDP patients more frequently developed respiratory failure (83.3% vs. 25%, P=0.000) and required intensive care unit (ICU) hospitalization (58.3% vs. 31.3%, P=0.000). In C-AIDP, distal motor latencies (DMLs) were more frequently prolonged (70.9% vs. 26.2%, P=0.000) whereas in S-AIDP distal compound muscle action potential (dCMAP) durations were more frequently increased (49.5% vs. 32.4%, P=0.002) and F waves were more often absent (45.6% vs. 31.8%, P=0.011). Presence of nerves with increased dCMAP duration and normal or slightly prolonged DML was elevenfold higher in S-AIDP (31.1% vs. 2.8%, P=0.000);11 S-AIDP patients showed this pattern in 2 nerves. CONCLUSION: Increased dCMAP duration, thought to be a marker of acquired demyelination, can also be oserved in critical illness myopathy. In S-AIDP patients, an increased dCMAP duration dissociated from prolonged DML, suggests additional muscle fiber conduction slowing, possibly due to a COVID-19-related hyperinflammatory state. Absent F waves, at least in some S-AIDP patients, may reflect α-motor neuron hypoexcitability because of immobilization during the ICU stay. These features should be considered in the electrodiagnosis of SARS-CoV-2 patients with weakness, to avoid misdiagnosis.


Assuntos
COVID-19/complicações , COVID-19/fisiopatologia , Síndrome de Guillain-Barré/etiologia , Síndrome de Guillain-Barré/fisiopatologia , Potenciais de Ação , Adulto , Idoso , Idoso de 80 Anos ou mais , Cuidados Críticos/estatística & dados numéricos , Eletrodiagnóstico , Fenômenos Eletrofisiológicos , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Neurônios Motores , Músculo Esquelético/fisiopatologia , Condução Nervosa , Insuficiência Respiratória/etiologia , Células Receptoras Sensoriais
16.
BMC Neurol ; 21(1): 135, 2021 Mar 23.
Artigo em Inglês | MEDLINE | ID: mdl-33757457

RESUMO

BACKGROUND: During the Coronavirus disease 2019 (COVID-19) pandemic, different neurological manifestations have been observed. However, only a few cases of Guillain-Barre syndrome (GBS) and COVID-19 have been reported. Therefore, the aim of this study is to investigate a case of concomitant GBS with COVID-19 in Colombia. CASE PRESENTATION: A 39-year-old woman was admitted to a teaching hospital in Barranquilla, Colombia with a history of progressive general weakness with lower limb dominance. Previous symptoms such as ageusia, anosmia and intense headache were reported. Upon admission, facial diplegia, quadriparesis with lower extremity predominance and Medical Research Council muscular strength of 2/5 in the lower limbs and 4/5 in the upper limbs were reported. During clinical evolution, due to general areflexia, hypertensive emergency and progressive diaphragmatic weakness, the patient was admitted to an intensive care unit. The cerebrospinal fluid analysis showed protein-cytological dissociation and the GBS diagnosis was confirmed via a nerve conduction and electromyography test. With regard to the symptoms before hospitalisation, SARS-CoV-2 diagnostic testing was performed with positive results in the second test. The patient was managed with supportive care and was discharged after 20 days of hospitalization with clinical improvement. CONCLUSIONS: Only a few cases of COVID-19 with GBS have been reported. Different subtypes have been previously identified, such as Miller-Fisher syndrome and dysautonomic GBS with SARS-CoV-2 infection. This study investigated the first confirmed case of COVID-19 with concomitant GBS in Colombia. In patients with GBS, several viral and bacterial pathogens have been found in case-control studies but the factors that induce the immune-mediated destruction of the nerve tissues have not been determined. Further studies are needed to determine the possible association between COVID-19 exposure and GBS.


Assuntos
COVID-19/diagnóstico , Síndrome de Guillain-Barré/diagnóstico , Adulto , Paralisia de Bell/diagnóstico , Colômbia , Eletromiografia , Feminino , Síndrome de Guillain-Barré/fisiopatologia , Hospitalização , Humanos , Condução Nervosa , Quadriplegia/diagnóstico
17.
Curr Pain Headache Rep ; 25(5): 32, 2021 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-33760994

RESUMO

PURPOSE OF REVIEW: Chest pain is a very common presenting complaint among patients in the hospital, a large proportion of whom have non-cardiac chest pain (NCCP). Neurological causes of NCCP have not been previously reviewed although several causes have been identified. RECENT FINDINGS: Chest pain has been reported as a symptom of multiple neurological conditions such as migraine, epilepsy, and multiple sclerosis, with varying clinical presentations. The affected patients are often not formally diagnosed for long periods of time due to difficulties in recognizing the symptoms as part of neurological disease processes. This paper will briefly summarize well-known etiologies of chest pain and, then, review neurological causes of NCCP, providing an overview of current literature and possible pathophysiologic mechanisms.


Assuntos
Dor no Peito/etiologia , Doenças do Sistema Nervoso/complicações , Sensibilização do Sistema Nervoso Central , Dor no Peito/fisiopatologia , Síndromes da Dor Regional Complexa/complicações , Síndromes da Dor Regional Complexa/fisiopatologia , Epilepsia/complicações , Epilepsia/fisiopatologia , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/fisiopatologia , Herpes Zoster/complicações , Herpes Zoster/fisiopatologia , Humanos , Transtornos de Enxaqueca/complicações , Transtornos de Enxaqueca/fisiopatologia , Doenças do Sistema Nervoso/fisiopatologia , Neuralgia Pós-Herpética/complicações , Neuralgia Pós-Herpética/fisiopatologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Radiculopatia/complicações , Radiculopatia/fisiopatologia , Raízes Nervosas Espinhais , Rigidez Muscular Espasmódica/complicações , Rigidez Muscular Espasmódica/fisiopatologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/fisiopatologia
19.
Rev. neurol. (Ed. impr.) ; 72(6): 203-212, 16 mar., 2021. tab
Artigo em Espanhol | IBECS | ID: ibc-202703

RESUMO

INTRODUCCIÓN: La pandemia por la enfermedad por coronavirus 2019 (COVID-19) es un importante problema para la salud mundial. Hay un incremento en las complicaciones neurológicas reconocidas por la COVID-19, incluyendo el síndrome de Guillain-Barré (SGB) y sus variantes. DESARROLLO: Se realizó una revisión de los casos publicados en los últimos meses de SGB asociado a infección por COVID-19. Incluimos a 48 pacientes (31 hombres; edad media: 56,4 años). Los síntomas de COVID-19 más comunes fueron tos (60,4%) y fiebre (56,3%). El tiempo promedio entre los síntomas de COVID-19 y el SGB fue de 12,1 días, pero nueve pacientes (18,8%) desarrollaron SGB en menos de siete días. Once pacientes (22,9%) presentaron afectación de los nervios craneales en ausencia de debilidad muscular, 36 presentaron la variante clásica sensitivomotora (75%) y uno tuvo una variante motora pura (2,1%). El patrón electrofisiológico se consideró desmielinizante en el 82,4% de las variantes generalizadas. La presencia de hiposmia/disgeusia estuvo asociada con una latencia menor a los siete días hasta el inicio de los síntomas del SGB (30 frente a 15,6%) y a la afectación de los nervios craneales en ausencia de debilidad (30,8 frente a 17,1%). La mayoría de los pacientes (87,5%) fueron tratados con inmunoglobulina endovenosa. La evolución neurológica fue favorable en el 64,6%, el 29,2% tuvo insuficiencia respiratoria y hubo un 4,2% de muertes. CONCLUSIONES: El SGB en pacientes con infección por SARS-CoV-2 es similar clínica y electrofisiológicamente a las formas clásicas. Se requieren más estudios para comprender si la frecuencia del SGB realmente aumentó debido a la pandemia por COVID-19 y explorar los mecanismos patógenos involucrados


INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic is a major worldwide health disorder. There is an increasing number of neurological complications recognized with COVID-19 including patients with GBS and its variants. DEVELOPMENT: A review of the clinical cases of GBS associated to COVID-19 infection published in the last months has been developed. We included 48 patients (31 men, mean age 56.4 years). The most common COVID-19 symptoms were cough (60.4%) and fever (56.3%). Mean time from COVID-19 symptoms to neurologic manifestations was 12.1 days, but in nine patients (18.8%) developed GBS within seven days. Eleven patients (22.9%) presented cranial nerve involvement in the absence of muscle weakness; 36 presented the classic sensory motor variant (75%) and one had a pure motor variant (2.1%). The electrodiagnostic pattern was considered demyelinating in 82.4% of the generalized variants. The presence of hyposmia/dysgeusia was associated with a latency shorter than seven days to GBS onset of symptoms (30% vs 15.6%), and cranial nerve involvement in the absence of weakness (30.8% vs 17.1%). Most patients (87.5%) were treated with intravenous immunoglobulin. Neurological outcome was favorable in 64.6%; 29.2% had respiratory failure and 4.2% died shortly after being admitted. CONCLUSIONS: GBS in patients with SARS-CoV-2 infection resembles clinically and electrophysiology the classical forms. Further studies are necessary to understand whether GBS frequency is actually increased due to SARS-CoV-2 infection and explore pathogenic mechanisms


Assuntos
Humanos , Masculino , Feminino , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Síndrome de Guillain-Barré/virologia , Pandemias , Betacoronavirus , Síndrome de Guillain-Barré/fisiopatologia , Disgeusia/virologia , Transtornos do Olfato/virologia
20.
Neuroepidemiology ; 55(2): 109-118, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33631765

RESUMO

BACKGROUND: COVID-19 can be accompanied by acute neurological complications of both central and peripheral nervous systems (CNS and PNS). In this study, we estimate the frequency of such complications among hospital inpatients with COVID-19 in Assiut and Aswan university hospitals. MATERIALS AND METHODS: We screened all patients with suspected COVID-19 admitted from 1 June to 10 August 2020 to the university hospitals of Assiut and Aswan in Upper Egypt. Clinical and laboratory tests, CT/MRI of the chest and brain, and neurophysiology study were performed for each patient if indicated. RESULTS: 439 patients had confirmed/probable COVID-19; neurological manifestations occurred in 222. Of these, 117 had acute neurological disease and the remainder had nonspecific neuropsychiatric symptoms such as headache, vertigo, and depression. The CNS was affected in 75 patients: 55 had stroke and the others had convulsions (5), encephalitis (6), hypoxic encephalopathy (4), cord myelopathy (2), relapse of multiple sclerosis (2), and meningoencephalitis (1). The PNS was affected in 42 patients: the majority had anosmia and ageusia (31) and the others had Guillain-Barré syndrome (4), peripheral neuropathy (3), myasthenia gravis (MG, 2), or myositis (2). Fever, respiratory symptoms, and headache were the most common general symptoms. Hypertension, diabetes mellitus, and ischemic heart disease were the most common comorbidities in patients with CNS affection. CONCLUSION: In COVID-19, both the CNS and PNS are affected. Stroke was the most common complication for CNS, and anosmia and/or ageusia were common for PNS diseases. However, there were 6 cases of encephalitis, 2 cases of spinal cord myelopathy, 2 cases of MG, and 2 cases of myositis.


Assuntos
Anosmia/fisiopatologia , COVID-19/fisiopatologia , Doenças do Sistema Nervoso Central/fisiopatologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Acidente Vascular Cerebral/fisiopatologia , Adulto , Idoso , Anosmia/epidemiologia , Encéfalo/diagnóstico por imagem , COVID-19/diagnóstico , COVID-19/epidemiologia , Doenças do Sistema Nervoso Central/diagnóstico , Doenças do Sistema Nervoso Central/epidemiologia , Progressão da Doença , Egito/epidemiologia , Encefalite/epidemiologia , Encefalite/fisiopatologia , Feminino , Síndrome de Guillain-Barré/epidemiologia , Síndrome de Guillain-Barré/fisiopatologia , Hospitais Universitários , Humanos , Hipóxia Encefálica/epidemiologia , Hipóxia Encefálica/fisiopatologia , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Esclerose Múltipla Recidivante-Remitente/fisiopatologia , Miastenia Gravis/epidemiologia , Miastenia Gravis/fisiopatologia , Miosite/epidemiologia , Miosite/fisiopatologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/epidemiologia , SARS-CoV-2 , Convulsões/epidemiologia , Convulsões/fisiopatologia , Medula Espinal/diagnóstico por imagem , Doenças da Medula Espinal/epidemiologia , Doenças da Medula Espinal/fisiopatologia , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/epidemiologia , Tomografia Computadorizada por Raios X
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