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BACKGROUND AND PURPOSE: Many single cases and small series of Guillain-Barré syndrome (GBS) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection were reported during the coronavirus disease 19 (COVID-19) outbreak worldwide. However, the debate regarding the possible role of infection in causing GBS is still ongoing. This multicenter study aimed to evaluate epidemiological and clinical findings of GBS diagnosed during the COVID-19 pandemic in northeastern Italy in order to further investigate the possible association between GBS and COVID-19. METHODS: Guillain-Barré syndrome cases diagnosed in 14 referral hospitals from northern Italy between March 2020 and March 2021 were collected and divided into COVID-19-positive and COVID-19-negative. As a control population, GBS patients diagnosed in the same hospitals from January 2019 to February 2020 were considered. RESULTS: The estimated incidence of GBS in 2020 was 1.41 cases per 100,000 persons/year (95% confidence interval 1.18-1.68) versus 0.89 cases per 100,000 persons/year (95% confidence interval 0.71-1.11) in 2019. The cumulative incidence of GBS increased by 59% in the period March 2020-March 2021 and, most importantly, COVID-19-positive GBS patients represented about 50% of the total GBS cases with most of them occurring during the two first pandemic waves in spring and autumn 2020. COVID-19-negative GBS cases from March 2020 to March 2021 declined by 22% compared to February 2019-February 2020. CONCLUSIONS: Other than showing an increase of GBS in northern Italy in the "COVID-19 era" compared to the previous year, this study emphasizes how GBS cases related to COVID-19 represent a significant part of the total, thus suggesting a relation between COVID-19 and GBS.
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COVID-19 , Síndrome de Guillain-Barré , COVID-19/complicações , COVID-19/epidemiologia , Síndrome de Guillain-Barré/etiologia , Humanos , Incidência , Pandemias , SARS-CoV-2RESUMO
OBJECTIVE: Single cases and small series of Guillain-Barré syndrome (GBS) have been reported during the SARS-CoV-2 outbreak worldwide. We evaluated incidence and clinical features of GBS in a cohort of patients from two regions of northern Italy with the highest number of patients with COVID-19. METHODS: GBS cases diagnosed in 12 referral hospitals from Lombardy and Veneto in March and April 2020 were retrospectively collected. As a control population, GBS diagnosed in March and April 2019 in the same hospitals were considered. RESULTS: Incidence of GBS in March and April 2020 was 0.202/100 000/month (estimated rate 2.43/100 000/year) vs 0.077/100 000/month (estimated rate 0.93/100 000/year) in the same months of 2019 with a 2.6-fold increase. Estimated incidence of GBS in COVID-19-positive patients was 47.9/100 000 and in the COVID-19-positive hospitalised patients was 236/100 000. COVID-19-positive patients with GBS, when compared with COVID-19-negative subjects, showed lower MRC sum score (26.3±18.3 vs 41.4±14.8, p=0.006), higher frequency of demyelinating subtype (76.6% vs 35.3%, p=0.011), more frequent low blood pressure (50% vs 11.8%, p=0.017) and higher rate of admission to intensive care unit (66.6% vs 17.6%, p=0.002). CONCLUSIONS: This study shows an increased incidence of GBS during the COVID-19 outbreak in northern Italy, supporting a pathogenic link. COVID-19-associated GBS is predominantly demyelinating and seems to be more severe than non-COVID-19 GBS, although it is likely that in some patients the systemic impairment due to COVID-19 might have contributed to the severity of the whole clinical picture.
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COVID-19/complicações , Síndrome de Guillain-Barré/epidemiologia , Adulto , Idoso , COVID-19/diagnóstico , COVID-19/terapia , Feminino , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Hospitalização , Humanos , Incidência , Itália , Masculino , Pessoa de Meia-Idade , Encaminhamento e Consulta , Estudos RetrospectivosRESUMO
BACKGROUND AND PURPOSE: In its initial stages, Guillain-Barré syndrome (GBS) is difficult to identify, because diagnostic criteria may not always be fulfilled. With this retrospective study, we wanted to identify the most common electrophysiological abnormalities seen on neurophysiological examination of GBS patients and its variants in the early phases. METHODS: We reviewed the clinical records of patients admitted to our Neurology Unit with a confirmed diagnosis of GBS. The study sample was divided in two subgroups according to whether the neurophysiological examination was performed: within 7 days (very early group) or within 7-15 days (early group). H reflex, F waves, and motor and sensory conduction parameters were judged abnormal if they were outside the normal range for at least two nerves. We evaluated neurophysiological findings in Miller-Fisher syndrome (MFS) separately. RESULTS: The study sample comprised 36 patients. In GBS, the most frequent abnormal neurophysiological parameter was the bilateral absence of the H reflex, followed by F wave abnormalities. Motor conduction parameters were altered in less than 50% of patients, and even less common were sensory nerve action potential reduction and the "sural-sparing" pattern. In MFS, H reflex was absent bilaterally in 100% of patients, followed by a predominant peripheral sensory involvement, whereas motor conduction parameters were frequently normal. CONCLUSIONS: Bilateral absence of the H reflex is the most sensitive parameter in early diagnosis of GBS and its variants.
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Síndrome de Guillain-Barré , Síndrome de Miller Fisher , Síndrome de Guillain-Barré/diagnóstico , Frequência Cardíaca , Humanos , Condução Nervosa , Neurofisiologia , Estudos RetrospectivosAssuntos
Neurite do Plexo Braquial/diagnóstico por imagem , Atrofia Muscular/diagnóstico por imagem , Nervo Musculocutâneo/diagnóstico por imagem , Idoso , Braço , Neurite do Plexo Braquial/fisiopatologia , Eletrodiagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/patologia , Atrofia Muscular/patologia , Nervo Musculocutâneo/fisiopatologia , UltrassonografiaAssuntos
Traumatismos dos Nervos Periféricos/diagnóstico , Nervo Radial/diagnóstico por imagem , Nervo Radial/lesões , Adulto , Eletrodiagnóstico , Humanos , Masculino , Traumatismos dos Nervos Periféricos/diagnóstico por imagem , Traumatismos dos Nervos Periféricos/fisiopatologia , Nervo Radial/fisiopatologia , UltrassonografiaRESUMO
Botulinum toxin (BT), a first-line treatment for focal dystonias in adults, has gained USA Food and Drug Administration approval for pediatric upper and lower extremity spasticity and sialorrhea, though its use in children younger than 2 years old is still considered off-label treatment for all pathologies. Dosing, treatment strategies and outcome measures lack international consensus, and they are often extrapolated from adult or spasticity guidelines. This review aims to evaluate the best available evidence on the efficacy and safety of BT therapy in pediatric dystonia (age under 21 years old), isolated or associated with other medical conditions. A comprehensive search in PubMed, Scopus and Web of Science was conducted, including only articles in English. Although no randomized controlled trials are still present, 12 articles were included with an overall of 57 patients. All the papers demonstrate that BT can improve motor function, decrease pain and ameliorate quality of life, with minimal adverse effects in pediatric patients affected by pure or mixed dystonic motor disorders. Despite the low level of evidence, our review shows that BT could be an efficacious treatment for these pediatric patients. The frequent generalized involvement, together with the heterogeneous nature of childhood dystonic forms, sometimes intermingled with spasticity, prompts further multicenter clinical trials or prospective studies with a higher level of evidence to shed light on the efficacy and safety profile of BT in pediatric dystonia.
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Toxinas Botulínicas , Distonia , Humanos , Criança , Distonia/tratamento farmacológico , Toxinas Botulínicas/uso terapêutico , Toxinas Botulínicas/administração & dosagem , Toxinas Botulínicas/efeitos adversos , Fármacos Neuromusculares/uso terapêutico , Fármacos Neuromusculares/efeitos adversos , Fármacos Neuromusculares/administração & dosagem , Adolescente , Resultado do Tratamento , Pré-Escolar , Distúrbios Distônicos/tratamento farmacológico , Qualidade de VidaRESUMO
INTRODUCTION: "Tethered cord syndrome" (TCS) refers to a congenital abnormality associated with neurological signs and symptoms. The aim of surgery is to prevent or arrest their progression. This study reports a retrospective case series of tethered cord syndrome surgeries, supported by intraoperative neurophysiological monitoring. METHODS: The case series comprises 50 surgeries for tethered cord syndrome in which multimodal intraoperative neurophysiological monitoring was performed using motor evoked potentials (transcranial motor evoked potentials [TcMEPs]), tibial nerve somatosensory evoked potentials (TNSEPs), and pudendal-anal reflex (PAR). The intraoperative neurophysiological monitoring results are reported and correlated with clinical outcomes. RESULTS: Sensitivity, specificity, and negative predictive value were high for TcMEPs and TNSEPs, while PAR exhibited low sensitivity and positive predictive value but high specificity and negative predictive value. Fisher's exact test revealed a significant correlation between changes in TcMEPs, TNSEPs, and clinical outcome ( P < 0.000 and P = 0.049 respectively), but no correlation was detected between PAR and urinary/anal function ( P = 0.497). CONCLUSIONS: While TcMEPs and TNSEPs were found to be reliable intraoperative neurophysiological monitoring parameters during tethered cord syndrome surgery, PAR had low sensitivity and positive predictive value probably because the reflex is not directly related to bladder function and because its multisynaptic pathway may be sensitive to anesthetics. New onset muscle weakness and sensory deficits were related to postoperative changes in TcMEPs and TNSEPs, whereas changes in PAR did not predict bladder/urinary impairment. Urinary deficits may be predicted and prevented with other neurophysiological techniques, such as the bladder-anal reflex.
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Our case report underscores the importance of electroneuromyography (ENMG) combined with peripheral nerve high-resolution ultrasound (HRUS) in the evaluation of neurofibromatosis type 1 (NF1). A 49-year-old woman affected by NF1 came to our attention because of new-onset left arm weakness and atrophy. Debulking of a cervicothoracic C7-T1 neurofibroma had been performed 8 years earlier. On current admission, magnetic resonance imaging disclosed increased lesion volume that was thought to cause the neurologic deficits by compressing the C8 root. Findings from intraoperative neurophysiologic monitoring during repeat debulking suggested that C8 root integrity had been compromised during the first operation and that the new-onset symptoms probably stemmed from peripheral nervous system damage distal to the cervical roots. Postoperative ENMG showed chronic denervation signs in the muscles innervated by C7-C8-T1 roots, moderate carpal tunnel syndrome (CTS), and ulnar nerve conduction block at the elbow. HRUS confirmed the CTS and revealed multiple neurofibromas involving the distal tract of the radial, ulnar, and median nerves. Surgical debulking was considered unnecessary in this case. ENMG combined with nerve and plexus HRUS evaluation may help identify the cause of neurologic deficits and choose the best surgical option in such complex clinical conditions as NF1.
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Eletrodiagnóstico/métodos , Neurofibromatose 1/diagnóstico , Ultrassonografia/métodos , Feminino , Humanos , Nervo Mediano/fisiopatologia , Pessoa de Meia-Idade , Condução Nervosa , Neurofibromatose 1/diagnóstico por imagem , Neurofibromatose 1/cirurgia , Período Pré-OperatórioRESUMO
BACKGROUND: The 'pain-inhibits-pain' effect stems from neurophysiological mechanisms involving endogenous modulatory systems termed diffuse noxious inhibitory controls (DNIC) or conditioned pain modulation (CPM). Laser-evoked potentials (LEPs) components, the N2/P2 complex, and the N1 wave, reflect the medial and lateral pain pathway, respectively: anatomically, the lateral thalamic nuclei (LT) project mainly to the somatosensory cortex (N1 generator), while the medial thalamic nuclei (MT) are bound to the limbic cortices (N2/P2 generators). METHODS: We applied a CPM protocol in which the test stimulus was laser stimulation and the conditioning stimulus was a cold pressor test. LEPs recordings were obtained from 15 healthy subjects in three different conditions: baseline, during heterotopic noxious conditioning stimulation (HNCS) and post-HNCS. RESULTS: We observed a significant reduction in N2/P2 amplitude during HNCS and a return to pre-test amplitude post-HNCS, whereas the N1 wave remained unchanged during and post-HNCS. CONCLUSIONS: Our results indicate that CPM affects only the medial pain system. The spinothalamic tract (STT) transmits to both the LT and the MT, while the spinoreticulothalamic (SRT) projects only to the MT. The reduction in the amplitude of the N2/P2 complex and the absence of change in the N1 wave suggest that DNIC inhibition on the dorsal horn neurons affects only pain transmission via the SRT, while the neurons that give rise to the STT are not involved. The N1 wave can be a reliable neurophysiological parameter for assessment of STT function in clinical practice, as it does not seem to be influenced by CPM. SIGNIFICANCE: No reports have described the effect of DNIC on lateral and medial pain pathways. We studied the N1 wave and the N2/P2 complex to detect changes during a CPM protocol. We found a reduction in the amplitude of the N2/P2 complex and no change in the N1 wave. This suggests that the DNIC inhibitory effect on dorsal horns neurons affects only pain transmission via the SRT, whereas the neurons that give rise to the STT are not involved.