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Background: Vulpian-Bernhardt syndrome is an atypical form of the motor neuron disease described since the 19th century. The importance of a timely diagnosis lies in the increased survival present in this variant. Due to the clinical rarity and complex diagnosis we report a clinical case of this disease, which is why we describe the typical clinical presentation, the diagnostic approach, and we make a bibliographic review of this neurodegenerative disorder as well. Clinical case: Latin American man whose clinical case onset was characterized by thoracic asymmetric and increasing limb weakness, showing affection from distal to proximal upper limbs area. Subsequently, symptoms worsened to the point of limiting day-to-day activities and conditioning patient's physical independence. Physical examination was consistent with motor neuron disease. Nerve conduction studies were performed and confirmed findings compatible with motor neuron involvement limited to thoracic limbs. Conclusion: Vulpian-Bernhardt syndrome is an uncommon form of motor neuron disease. Due to the rarity of its presentation, it is frequent to confuse clinical profile even for trained physicians. The importance of electrodiagnosis relies in identifying the neurogenic origin of the disease, as well as the active denervation and reinnervation data. Considering that with this syndrome patients have a longer survival than with the classic form of amyotrophic lateral sclerosis, it is important to have a clear diagnosis approach in order to provide a better quality of life and supportive treatment.

Introducción: el síndrome de Vulpian-Bernhardt es una forma atípica de la enfermedad de la motoneurona descrita desde el siglo XIX. La importancia de un diagnóstico oportuno radica en la mayor supervivencia que presenta esta variante. Debido a la rareza clínica y al diagnóstico complejo presentamos un caso clínico de esta enfermedad, por lo que describimos el cuadro clínico típico, el abordaje diagnóstico y hacemos una revisión bibliográfica de este trastorno neurodegenerativo. Caso clínico: hombre de origen latinoamericano que comenzó su padecimiento con debilidad de miembros torácicos, asimétrica y progresiva de distal a proximal. Los síntomas progresaron hasta limitar sus actividades de la vida diaria y su independencia física. La exploración física fue compatible con enfermedad de motoneurona. Se hicieron estudios de extensión y neuroconducción que confirmaron hallazgos compatibles con afectación en motoneurona limitada a miembros torácicos. Conclusión: el síndrome Vulpian-Bernhardt es una forma clínica poco común. Debido a su rareza, es fácil confundir el cuadro clínico, incluso por parte de experimentados. La importancia del electrodiagnóstico radica en identificar el origen neurogénico de la enfermedad, los datos de denervación activa y reinervación. Al ser una forma en la que se presenta una supervivencia mayor que en la forma clásica, es importante el diagnóstico claro para dar una mejor calidad de vida y tratamiento de soporte.

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BACKGROUND: Diabetic peripheral neuropathy (DPN) is the most common complication of type 2 diabetes mellitus (T2DM); its diagnosis and treatment are based on symptomatic improvement. However, as pharmacological therapy causes multiple adverse effects, the implementation of acupunctural techniques, such as electroacupuncture (EA) has been suggested as an alternative treatment. Nonetheless, there is a lack of scientific evidence, and its mechanisms are still unclear. We present the design and methodology of a new clinical randomized trial, that investigates the effectiveness of EA for the treatment of DPN. METHODS: This study is a four-armed, randomized, controlled, multicenter clinical trial (20-week intervention period, plus 12 weeks of follow-up after concluding intervention). A total of 48 T2DM patients with clinical signs and symptoms of DPN; and electrophysiological signs in the Nerve Conduction Study (NCS); will be treated by acupuncture specialists in outpatient units in Mexico City. Patients will be randomized in a 1:1 ratio to one of the following four groups: (a) short fibre DPN with EA, (b) short fibre DPN with sham EA, (c) axonal DPN with EA and (d) axonal DPN with sham EA treatment. The intervention will consist of 32 sessions, 20 min each, per patient over two cycles of intervention of 8 weeks each and a mid-term rest period of 4 weeks. The primary outcome will be NCS parameters, and secondary outcomes will include DPN-related symptoms and pain by Michigan Neuropathy Screening Instrument (MNSI), Michigan Diabetic Neuropathy Score (MDNS), Dolour Neuropatique Score (DN-4), Semmes-Westein monofilament, Numerical Rating Scale (NRS) for pain assessment, and the 36-item Short Form Health Survey (SF-36). To measure quality of life and improve oxidative stress, the inflammatory response; and genetic expression; will be analysed at the beginning and at the end of treatment. DISCUSSION: This study will be conducted to compare the efficacy of EA versus sham EA combined with conventional diabetic and neuropathic treatments if needed. EA may improve NCS, neuropathic pain and symptoms, oxidative stress, inflammatory response, and genetic expression, and it could be considered a potential coadjutant treatment for the management of DPN with a possible remyelinating effect. TRIAL REGISTRATION: ClinicalTrials.gov. NCT05521737 Registered on 30 August 2022. International Clinical Trials Registry Platform (ICTRP) ISRCTN97391213 Registered on 26 September 2022 [2b].

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BACKGROUND: Dermatomyositis belongs to an infrequent group of diseases predominantly found in patients older than 40 years old and is characterized by dermal and muscular findings. This disease presents itself as proximal, ascending and symmetric weakness and typical dermatosis with findings such as elevated muscle enzymes, altered electromyography and typical changes in muscle biopsy; as of today, the etiology of the disease in unknown. The COVID-19 vaccine has been a fundamental tactic to achieve control of the coronavirus (SARS CoV2), and it's clear that the benefits of getting the vaccine overweight the risks that might come along with it. Although rare, all adverse effects should be reported, this could help us to understand the elusive pathophysiology of inflammatory idiopathic myopathy. CASE PRESENTATION: In this text we will describe the case of a patient with dermatomyositis who was vaccinated against SARS CoV2 with BNT162b2 mRNA (Pfizer-BioNTech), showing a temporal relation between the vaccination and the beginning of her symptoms. We realized all the diagnostic approach to the suspected disease including electromyography, muscle biopsy and laboratory findings, corroborating the diagnosis. The patient received standard treatment for this disease (steroid therapy) and have a classic slow improvement. CONCLUSIONS: Although it´s not possible to confirm a direct correlation between the vaccine and the onset of the disease, we considered that there are enough data to suspect that this could be a trigger event and therefore should always be considered a possible cause for a case of inflammatory idiopathic myopathy.

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INTRODUCTION: Intraoperative neurophysiological monitoring (IONM) is a procedure that uses neurophysiological techniques in order to evaluate the motor and sensitive systems during surgeries that endanger the nervous system. METHOD: The approach, scope, target population, and clinical questions to be answered were defined. A systematic search of the evidence was conducted step by step; during the first stage, clinical practice guidelines were collected, during the second stage systematic reviews were obtained, and during the third stage, clinical trials and observational studies were procured. The MeSH nomenclature and free related terminology were used, with no language restrictions and a 5-10 years frame. The quality of the evidence was graded using the CEPD and SIGN scales. RESULTS: Obtained using the search algorrhythms of 892 documents. Fifty-eight were chosen to be included in the qualitative synthesis. A meta-analysis was not possible due to the heterogeneity of the studies. CONCLUSIONS: Eighteen recommendations were issued and will support the adequate use of the IONM.

INTRODUCCIÓN: El monitoreo neurofisiológico intraoperatorio (MNIO) es un procedimiento que emplea técnicas neurofisiológicas con la finalidad de evaluar los sistemas motor y sensitivo durante cirugías que ponen en riesgo al sistema nervioso. MÉTODO: Se definieron el enfoque, los alcances, la población diana y las preguntas clínicas por resolver. Se realizó una búsqueda sistematizada de la evidencia por etapas. En la primera, se buscaron guías de práctica clínica; en la segunda, revisiones sistemáticas; y en la tercera, ensayos clínicos y estudios observacionales. Se utilizaron los términos MeSH y libres correspondientes, sin restricciones de lenguaje y con una temporalidad de 5 a 10 años. Se graduó la calidad de la evidencia utilizando las escalas CEPD y SIGN. RESULTADOS: Mediante los algoritmos de búsqueda se obtuvieron 892 documentos, y se seleccionaron 58 para la inclusión de la síntesis cualitativa. Debido a la heterogeneidad entre los estudios, no fue posible realizar metaanálisis. CONCLUSIONES: Se emitieron 18 recomendaciones, las cuales servirán como apoyo para la adecuada utilización del MNIO.

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Background: Guillain-Barré Syndrome (GBS) is an acute polyneuropathy characterized by symmetrical weakness of the limbs with hyporeflexia or areflexia with a maximum progression within four weeks and can impair respiratory function and implies disability at a long. The aim of this paper was to describe the clinical, epidemiological and neurophysiological features of patients with GBS at the Hospital de Especialidades del Centro Medico Nacional Siglo XXI (HECMNSXXI) Methods: An observational, retrospective cross-sectional study, data were collected form clinical records of adults with GBS hospitalized in HECMNSXXI from March 2012 to March 2016. The recorded variables were demographics, previous infection, clinical presentation, disability scores, prognosis scores and neurophysiological subtypes. Results: Clinical records of 94 patients were analysed with a mean age of 53 years, 61% male, with previous infection in 80%. Albumin cytologic dissociation was present in 50%. Medical Research Council (MRC) sum scores mean was 32, the SGB disability score at admittance with a mean of 3.63. The axonal subtype was in 68%, and demyelinating in 29%, not conclusive in 3%. Conclusions: In this study the demographic and clinical features are similar to other previous reports, we documented a greater proportion of axonal subtype, which are related with important disability and worse prognosis.

Introducción: El síndrome de Guillain-Barré (SGB) es una polirradiculoneuropatía aguda caracterizada por debilidad simétrica progresiva de las extremidades con hipo o arreflexia, que progresa en un máximo de 4 semanas y que puede llevar a la falla respiratoria y a discapacidad a largo plazo. El objetivo de este trabajo fue describir las características clínicas, epidemiológicas y neurofisiológicas de pacientes con SGB del Hospital de Especialidades del Centro Médico Nacional Siglo XXI (HECMNSXXI). Métodos: Se realizó un estudio observacional de tipo transversal retrolectivo. Se obtuvieron datos de expedientes clínicos de adultos con diagnóstico de SGB hospitalizados en el HECMNSXXI en el periodo de marzo de 2012 a marzo de 2016. Se registraron variables demográficas, clínicas y neurofisiológicas. Resultados: Se incluyeron 94 pacientes con un promedio de edad de 53 años, en su mayoría hombres, con infección previa en 80%. La fuerza muscular medida con la escala del Medical Research Council (MRC) fue en promedio de 32, la escala de discapacidad del SGB (Hughes) al ingreso tuvo un promedio de 3.63. La variedad axonal se encontró en 68%, la desmielinizante en 29% y en 3% no concluyente. Conclusiones: Se documentan en esta muestra características demográficas y clínicas similares a lo reportado en la literatura, así como la mayor proporción de variedades axonales, las cuales tienen mayor severidad en la presentación clínica así como mal pronóstico.

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Despite steady progress in therapeutics of liver disease, portal systemic encephalopathy remains to be a great challenge for clinicians because of the heterogeneity of neuropsychiatric symptoms, multiple risk factors and complexity on achieving a sustained response. We aimed to evaluate the efficacy of L-Ornithin, L-Aspartate versus lactulose in Mexican patients with hyperammonemic hepatic encephalopathy. A total of 20 patients were randomly allocated to receive either lactulose(n = 10) or L-ornithine - L-aspartate (n = 10) for 2 weeks. At baseline, patients of both groups were comparable in age (64 +/- 7 versus 60 +/- 6) and degree of hepatic failure according to the Child-Pugh scale (9.2 +/- 1.3 versus 9.2 +/- 1.1). A significant decrease in ammonia levels was observed both in the lactulose group (120.4 +/- 8.1 versus 91.4 +/- 10, p < 0.05) and in the LOLA group (141.6 +/- 9.1 versus 96.9 +/- 9.3, p < 0.05). Moreover, in patients who received LOLA a significant improvement was observed in mental status (1.0 +/- 0.14 versus 0.4 +/- 0.16, p < 0.05), Number Connection Test (184 +/- 43 versus 88 +/- 7, p < 0.05), asterixis (14.6 +/- 2.8 versus 6.7 +/- 1.5, p < 0.05), as well as EEG findings (6.8 +/- 0.6 versus 8.1 +/- 0.2 cycles per second, p < 0.05). Compliance with study medications was similar between the lactulose group (94%) and the LOLA group (100%). No serious adverse events were reported in the two groups; however, in the lactulose group an increase in the number of weekly defecations was reported, as well as a higher incidence of abdominal pain or flatulence. Finally, both patient groups reported an improvement in the Visual Analogue Scale for EuroQol index (51.1 +/- 24.1 versus 61.5 +/- 15.8, p < 0.05, in the lactulose group; 56.5 +/- 24.5 versus 70 +/- 19.4, p < 0.05, in the LOLA group). In conclusion, oral administration of lactulose or L-ornithine - L-aspartate to Mexican patients with cirrhosis and hyperammonemic encephalopathy significantly reduced serum ammonia levels in study groups and additionally improved mental status parameters, number connection test, asterixis scores, and EEG activity in the group receiving L-ornithine-L-aspartate.

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