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BACKGROUND: Spinal cord compression is a rare presentation of non-Hodgkin lymphoma (NHL) in children. We aimed to describe the prevalence, histological subtypes, clinical presentation, therapy, and outcome of those children in a population-based cohort. The chemotherapy regimen remained comparable over time. METHODS: We retrospectively identified all children and adolescents with paresis as initial manifestations of the NHL between January 1990 and December 2020 from the NHL-BFM database. Characteristics, therapy, and outcome data were gathered from the database and patient files. RESULTS: Fifty-seven of 4779 children (1.2%) presented with initial paresis due to spinal cord compression. The median age was 10.3 years (range, 3.1-18.0 years), and 33% were female. Initial symptoms were paresis/weakness (n = 50, 88%), back pain (n = 33, 58%), paresthesia (n = 23, 40%), and bladder dysfunction and/or constipation (n = 22, 39%), persisting for a median of 14 days before diagnosis. Subtype distribution was mature B-NHL (n = 41, 72%), precursor B-lymphoblastic lymphoma (LBL) (n = 12, 21%), anaplastic large cell lymphoma (ALCL) (n = 3, 5%), and T-LBL (n = 1, 2%). Initial emergency therapy included surgery (70%) and/or chemotherapy/steroids (63%). Five-year event-free survival and overall survival (80% ± 5% and 82% ± 5%, respectively) were comparable with all other NHL patients. Neurological symptoms persisted in approximately one-third of surviving patients at the last follow-up. CONCLUSION: 1.2% of pediatric NHL patients presented with paresis from spinal cord compression mainly due to B-cell lymphomas. Neurological sequelae were observed in one-third of surviving patients.
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Linfoma no Hodgkin , Compresión de la Médula Espinal , Humanos , Femenino , Masculino , Niño , Adolescente , Estudios Retrospectivos , Preescolar , Compresión de la Médula Espinal/etiología , Linfoma no Hodgkin/complicaciones , Linfoma no Hodgkin/patología , Linfoma no Hodgkin/epidemiología , Tasa de Supervivencia , Pronóstico , Estudios de SeguimientoRESUMEN
BACKGROUND: Severe acute respiratory syndrome virus 2 (SARS-CoV-2) is spreading globally and causes most frequently fever and respiratory symptoms, i.e. Coronavirus disease 2019 (COVID-19), however, distinct neurological syndromes associated with SARS-CoV-2 infection have been described. Among SARS-CoV-2-infections-associated neurological symptoms fatigue, headache, dizziness, impaired consciousness and anosmia/ageusia are most frequent, but less frequent neurological deficits such as seizures, Guillain-Barré syndrome or ataxia may also occur. CASE PRESENTATION: Herein we present a case of a 62-year-old man who developed a subacute cerebellar syndrome with limb-, truncal- and gait ataxia and scanning speech 1 day after clinical resolution of symptomatic SARS-CoV-2 infection of the upper airways. Apart from ataxia, there were no signs indicative of opsoclonus myoclonus ataxia syndrome or Miller Fisher syndrome. Cerebral magnetic resonance imaging showed mild cerebellar atrophy. SARS-CoV-2 infection of the cerebellum was excluded by normal cerebrospinal fluid cell counts and, most importantly, absence of SARS-CoV-2 RNA or intrathecal SARS-CoV-2-specific antibody production. Other causes of ataxia such as other viral infections, other autoimmune and/or paraneoplastic diseases or intoxication were ruled out. The neurological deficits improved rapidly after high-dose methylprednisolone therapy. CONCLUSIONS: The laboratory and clinical findings as well as the marked improvement after high-dose methylprednisolone therapy suggest a post-infectious, immune-mediated cause of ataxia. This report should make clinicians aware to consider SARS-CoV-2 infection as a potential cause of post-infectious neurological deficits with an atypical clinical presentation and to consider high-dose corticosteroid treatment in case that a post-infectious immune-mediated mechanism is assumed.
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COVID-19/complicaciones , Ataxia Cerebelosa/complicaciones , Cerebro/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , ARN ViralRESUMEN
Background: Vaccination against SARS-CoV-2 has been conducted frequently to limit the pandemic but may rarely be associated with postvaccinal autoimmune reactions or disorders. Case presentation: We present a 35-year-old woman who developed fever, skin rash, and headache 2 days after the second SARS-CoV-2 vaccination with BNT162b2 (Pfizer/Biontech). Eight days later, she developed behavioral changes and severe recurrent seizures that led to sedation and intubation. Cerebral magnetic resonance imaging showed swelling in the (para-) hippocampal region predominantly on the left hemisphere and bilateral subcortical subinsular FLAIR hyperintensities. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis of 7 cells/µl and normal protein and immunoglobulin parameters. Common causes of encephalitis or encephalopathy such as viral infections, autoimmune encephalitis with well-characterized autoantibodies, paraneoplastic diseases, and intoxications were ruled out. We made a diagnosis of new-onset refractory status epilepticus (NORSE) due to seronegative autoimmune encephalitis. The neurological deficits improved after combined antiepileptic therapy and immunomodulatory treatment including high-dose methylprednisolone and plasma exchange. Conclusions: Although a causal relationship cannot be established, the onset of symptoms shortly after receiving the SARS-CoV-2 vaccine suggests a potential association between the vaccination and NORSE due to antibody-negative autoimmune encephalitis. After ruling out other etiologies, early immunomodulatory treatment may be considered in such cases.
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Background: In patients with myocardial infarction, atypical symptoms at onset have been demonstrated in women. We aimed to investigate the presence of sex-related differences in symptom presentation in patients with aneurysmal subarachnoid hemorrhage (aSAH) to enable earlier diagnosis and treatment. Methods: We assessed symptoms on admission to hospital in 343 patients with aSAH in this retrospective single-center cohort-study. Univariate statistical analysis was performed by comparing sexes including the whole study population and subgroups (dichotomized using Fisher scale 1-2 vs. 3-4, WFNS grade 1-3 vs. 4-5, and anterior vs. posterior circulation aneurysms, respectively). Results: The majority of patients was female (63.6%, n=218, vs. 36.4%, n=125), the mean age 57.4 years (standard deviation (SD) 13.3) with older women compared to men (59.2, SD 13.8, vs. 54.4, SD 11.6; p=0.003). Anterior communicating artery (AcomA) aneurysms were most common (30.9%, n=106), predominantly in men (43.2%, n=54, vs. 23.9%, n=52; p=0.0002), whereas posterior communicating artery (PcomA) aneurysms were more frequent in women (19.3%, n=42, vs. 8.8%, n=11; p=0.005). Exercise-induced headache was more often reported by men (10.4%, n=13, vs. 5%, n=11; p=0.04) in all patients as well as in the subgroup of WFNS 1-3. Anisocoria was more frequent in women within the subgroup of severely impaired consciousness (WFNS 4-5; 25.3%, n=22, vs. 10.7%, n=6; p=0.032). For all other symptoms, there was no evidence for sex-specific differences in the whole study group as well as in subgroups. Conclusion: Our results show no evidence for relevant sex-related differences in symptom presentation at onset in aSAH patients. Women presenting with an acute onset anisocoria should be screened even more carefully for an underlying ruptured Pcom aneurysm.
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Aneurisma Roto , Aneurisma Intracraneal , Hemorragia Subaracnoidea , Masculino , Humanos , Femenino , Anciano , Persona de Mediana Edad , Hemorragia Subaracnoidea/complicaciones , Hemorragia Subaracnoidea/diagnóstico , Hemorragia Subaracnoidea/epidemiología , Estudios Retrospectivos , Anisocoria , Aneurisma Intracraneal/complicacionesRESUMEN
BACKGROUND: Artistic gymnastics is a popular Olympic discipline where female athletes compete in four and male athletes in six events with floor exercise having the longest competition duration in Women's and Men's artistic gymnastics (WAG, MAG). To date no valid information on the energetics of floor gymnastics is available although this may be important for specific conditioning programming. This study evaluated the metabolic profile of a simulated floor competition in sub-elite gymnasts. METHODS: 17 (9 male, 8 female) sub-elite gymnasts aged 22.5 ± 2.6y took part in a floor-training-competition where oxygen uptake was measured during and until 15 min post-exercise. Additionally, resting and peak blood lactate concentration after exercise were obtained. The PCr-LA-O2 method was used to calculate the metabolic energy and the relative aerobic (WAER), anaerobic alactic (WPCr) and anaerobic lactic (WBLC) energy contribution. Further, the athletes completed a 30 s Bosco-jumping test, a countermovement jump and a drop jump. RESULTS: The competition scores were 9.2 (CI:8.9-9.3) in WAG and 10.6 (CI:10.4-10.9) in MAG. The metabolic profile of the floor routine was mainly aerobic (58.9%, CI: 56.0-61.8%) followed by the anaerobic alactic (24.2%, CI: 21.3-27.1%) and anaerobic lactic shares (16.9%, CI:14.9-18.8%). While sex had a significant (p = .010, d = 1.207) large effect on energy contribution, this was not the case for competition duration (p = .728, d = 0.061). Relative energy contribution of WAG and MAG differed in WAER (64.0 ± 4.7% vs. 54.4 ± 6.8%, p = .004, d = 1.739) but not in WPCr (21.3 ± 6.1% vs. 26.7 ± 8.0%, p = .144, d = 0.801) and WBLC (14.7 ± 5.4% vs. 18.9 ± 4.2%, p = .085, d = 0.954). Further no correlation between any energy share and performance was found but between WPCr and training experience (r = .680, p = .044) and WBLC and competition level (r = .668, p = .049). CONCLUSION: The results show a predominant aerobic energy contribution and a considerable anaerobic contribution with no significant difference between anaerobic shares. Consequently, gymnastic specific aerobic training should not be neglected, while a different aerobic share in WAG and MAG strengthens sex-specific conditioning. All in all, the specific metabolic share must secure adequate energy provision, while relative proportions of the two anaerobic pathways seem to depend on training and competition history.
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The aim of the present study was to compare the activity patterns of young, healthy right- (RH, n = 25) and left-handed (LH, n = 20) subjects in high-density electroencephalograpic (EEG) recordings during a deliberation task. The deliberation task consisted of pressing one of two keys depending on a color-word Stroop task (Stroop, 1935) presented on a computer screen. Depending on the color shown and the meaning of the color word, participants responded with the index finger of the dominant or non-dominant hand. This leads to different activities in the hemispheres depending on the acting hand and on subject's handedness. Presenting the word "black" in black color, subjects were not to press any key (no-go-trial). Prior to this, subjects were tested for simple motor tasks, during which they were informed about the motor action to be performed. The temporal activity patterns obtained from RH and LH were very similar in shape and constituent components. The comparison of the three types of trials lead to the assumption that the deliberation process is based on a two-step decision: The first decision was characterized by the choice between move (match-trials, mismatch-trials) or not to move (no-go-trials). The second decision resulted in the final judgment of which index finger has to be used. The latter decision, in particular, can be tracked via the local spread of activity over the scalp. Our hypothesis is based on a comparison of activities and locations of RH and LH and yields some insights about processing a two-step decision in a deliberation task.
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Lateralidad Funcional , Mano , Humanos , DedosRESUMEN
OBJECTIVE: To expand the spectrum of anti-IgLON5 disease by adding 5 novel anti-IgLON5-seropositive cases with bulbar motor neuron disease-like phenotype. METHODS: We characterized the clinical course, brain MRI and laboratory findings, and therapy response in these 5 patients. RESULTS: Patients were severely affected by bulbar impairment and its respiratory consequences. Sleep-related breathing disorders and parasomnias were common. All patients showed clinical or electrophysiologic signs of motor neuron disease without fulfilling the diagnostic criteria for amyotrophic lateral sclerosis. One patient regained autonomy in swallowing and eating, possibly related to immunotherapy. CONCLUSION: IgLON5 disease is an important differential diagnosis to evaluate in patients with bulbar motor neuron disease-like phenotype and sleep disorders. There is need for a deeper understanding of the underlying pathobiology to determine whether IgLON5 disease is an immunotherapy-responsive condition.
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Autoanticuerpos/sangre , Moléculas de Adhesión Celular Neuronal/sangre , Enfermedad de la Neurona Motora/sangre , Enfermedad de la Neurona Motora/diagnóstico , Anciano , Autoanticuerpos/inmunología , Moléculas de Adhesión Celular Neuronal/inmunología , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Inmunoterapia/métodos , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/inmunología , Enfermedad de la Neurona Motora/terapia , SíndromeRESUMEN
The aim of the study was to determine whether a deliberative process, leading to a motor action, is detectable in high density EEG recordings. Subjects were required to press one of two buttons. In a simple motor task the subject knew which button to press, whilst in a color-word Stroop task subjects had to press the right button with the right index finger when meaning and color coincided, or the left button with the left index finger when meaning and color were disparate. EEG recordings obtained during the simple motor task showed a sequence of positive (P) and negative (N) cortical potentials (P1-N1-P2) which are assumed to be related to the processing of the movement. The sequence of cortical potentials was similar in EEG recordings of subjects having to deliberate over how to respond, but the above sequence (P1-N1-P2) was preceded by slowly increasing negativity (N0), with N0 being assumed to represent the end of the deliberation process. Our data suggest the existence of neurophysiological correlates of deliberative processes.