Trastorno de la marcha del nivel superior como forma de presentación de una parálisis supranuclear progresiva: descripción de un vídeo caso / Higher-level gait disorder as a presenting manifestation of progressive supranuclear palsy: a video case report

Introducción: El trastorno de la marcha frontal/apraxia de la marcha es un déficit motor del nivel superior con diversas causas, caracterizado por dificultades en el inicio de la marcha (congelación). Nuestro objetivo es presentar una paciente con un trastorno de la marcha del nivel superior con episodios de caídas como manifestaciones iniciales de una parálisis supranuclear progresiva (PSP). Sus datos se obtuvieron de los registros médicos del Servicio de Medicina General del Burdwan Medical College & Hospital (Burdwan, Bengala Occidental, India). Caso clínico: Mujer de 58 años sana que consultó por un trastorno de la marcha con caídas. La exploración neurológica mostró una apariencia facial característica (mirada fija, ojos muy abiertos, ceño fruncido y expresión fija hemifacial inferior), e hipocinesia-rigidez simétrica de predominio axial (postura retrocólica del tronco y el cuello). La exploración de la marcha reveló un trastorno de la marcha del nivel superior, caracterizado por una significativa vacilación inicial, que precisaba ayuda de objetos/personas cercanos. Al iniciar la marcha, los pasos mejoraban relativamente, pero reaparecía una deambulación inefectiva al girar. Presentaba zancadas cortas, congelación, base amplia de sustentación, desequilibrio, movimiento lento de las piernas, arrastre de los pies, y pérdida de la cadencia normal del tronco y las extremidades. Los reflejos posturales estaban alterados. La resonancia magnética cerebral desveló atrofia mesencefálica, dilatación de acueducto de Silvio y III ventrículo, atrofia frontal bilateral y el signo típico del colibrí. Finalmente, la paciente fue diagnosticada de una PSP probable. Conclusiones: Varias etiologías, incluida la PSP, deben considerarse, en el contexto clínico apropiado, si la exploración de la deambulación demuestra un trastorno de la marcha del nivel superior.(AU)

Introduction: In the evaluation of drug-resistant epilepsy, a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature. Case report: We present two cases from our series of patients monitored by video-EEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS. A bibliographic review is also carried out to try to establish a localizing and/or lateralizing value of this sign. Conclusion: The sign of the cross is a rare ictal automatism that occurs in patients with temporal lobe epilepsy. The hand used to make the sign of the cross is the dominant one, regardless of the origin of the crises.(AU)

Tourette's Syndrome cervical dystonia induced occipital neuralgia remedied by peripheral nerve stimulation: A case report / Síndrome de Tourette, distonia cervical induzida por neuralgia occipital remediada por estimulação nervosa periférica: relato de caso

BACKGROUND: Dystonia is uncommon in Tourette's syndrome, and occipital neuralgia secondary to Tourette's dystonia is more rare, affecting quality of life. Occipital peripheral nerve stimulation (PNS) is an excellent alternative by being adjustable and minimally invasive. Our case demonstrates occipital PNS as an effective option for refractory Tourette's dystonia. CASE PRESENTATION: A thirty-four-year-old male with poorly controlled Tourette's cervical dystonia presented with severe occipital neuralgia. Various medications were prescribed including propranolol and amitriptyline, and bilateral third-occipital nerve rhizotomies and occipital nerve blocks were trialed. Distal nerve blocks at the occipital protuberance were most effective. Therefore, an occipital PNS trial was done, and a PNS was implanted with no complications. Upon follow-up, the patient reported drastic pain reduction. CONCLUSION: Our case illustrates neuromodulation benefits for a rare presentation of refractory occipital neuralgia secondary to Tourette's-related dystonia. Occipital PNS should be considered for refractory cases because it is safe, easy to implant, and effective.

FUNDAMENTO: A distonia é incomum na síndrome de Tourette, e a neuralgia occipital secundária à distonia de Tourette é mais rara, afetando a qualidade de vida. A estimulação do nervo periférico occipital (SNP) é uma excelente alternativa por ser ajustável e minimamente invasiva. Nosso caso demonstra o SNP occipital como uma opção eficaz para a distonia de Tourette refratária. APRESENTAÇÃO DO CASO: Um homem de 34 anos com distonia cervical de Tourette mal controlada apresentou neuralgia occipital grave. Vários medicamentos foram prescritos, incluindo propranolol e amitriptilina, e foram testadas rizotomias bilaterais do nervo terceiro-occipital e bloqueios do nervo occipital. Os bloqueios dos nervos distais na protuberância occipital foram mais eficazes. Portanto, foi feito um ensaio de PNS occipital e um PNS foi implantado sem complicações. Após o acompanhamento, o paciente relatou redução drástica da dor. CONCLUSÃO: Nosso caso ilustra os benefícios da neuromodulação para uma apresentação rara de neuralgia occipital refratária secundária à distonia relacionada a Tourette. O PNS occipital deve ser considerado para casos refratários porque é seguro, fácil de implantar e eficaz.

Study of the potential toxicity of adrenaline to neurons, using the SH-SY5Y human cellular model

Abstract Prolonged overexposure to catecholamines causes toxicity, usually credited to continuous adrenoceptor stimulation, autoxidation, and the formation of reactive pro-oxidant species. Non-differentiated SH-SY5Y cells were used to study the possible contribution of oxidative stress in adrenaline (ADR)-induced neurotoxicity, as a model to predict the toxicity of this catecholamine to peripheral nerves. Cells were exposed to several concentrations of ADR (0.1, 0.25, 0.5 and 1mM) and two cytotoxicity assays [lactate dehydrogenase (LDH) release and 3-(4,5-dimethylthiazol-2yl)-2,5-diphenyl tetrazolium bromide (MTT) reduction] were performed at several time-points (24, 48, and 96h). The cytotoxicity of ADR was concentration- and time-dependent in both assays, since the lowest concentration tested (0.1mM) also caused significant cytotoxicity at 96h. N-acetyl-cysteine (1mM), a precursor of glutathione synthesis, prevented ADR-induced toxicity elicited by 0.5mM and 0.25mM ADR following a 96-h exposure, while the antioxidant Tiron (100µM) was non-protective. In conclusion, ADR led to mitochondrial distress and ultimately cell death in non-differentiated SH-SY5Y cells, possibly because of ADR oxidation products. The involvement of such processes in the catecholamine-induced peripheral neuropathy requires further analysis.

The Effect of Anakinra on Acrylamide-induced Peripheral Neuropathy and Neuropathic Pain in Rats

Abstract Acrylamide is a neurotoxic compound. Moreover, anakinra is an interleukin-1 (IL-1) receptor antagonist used in rheumatoid arthritis treatment. This study investigated the effect of anakinra on acrylamide-related neuropathy and neuropathic pain. Acrylamide exposure caused a significant decrease in the pain threshold; an increase in malondialdehyde (MDA), tumor necrosis factor-alpha (TNF-α), and interleukin-1 beta (IL-1ß) levels; and a decrease in total glutathione (tGSH) values in the sciatic nerve. This indicates hyperalgesia presence, oxidative stress, and peripheral nerve tissue inflammation. Anakinra treatment significantly reduced the MDA, IL-1ß, and TNF-α levels, and increased the pain threshold and mean tGSH values. The analgesic effect of anakinra was 67.9% at the first hour, increasing to 74.9% and 76.7% at the second and third hours, respectively. The group receiving acrylamide exhibited histopathological changes (e.g., swollen and degenerated axons, hypertrophic and hyperplasic Schwann cells, and congested vessels). The use of anakinra significantly improved these morphological changes. Anakinra is concluded to reduce neuropathic pain and prevent neurotoxic effect of acrylamide on peripheral nerves due to its analgesic, antioxidant, and anti-inflammatory properties

A review of burn symptoms and potential novel neural targets for non-invasive brain stimulation for treatment of burn sequelae.

Burn survivors experience myriad associated symptoms such as pain, pruritus, fatigue, impaired motor strength, post-traumatic stress, depression, anxiety, and sleep disturbance. Many of these symptoms are common and remain chronic, despite current standard of care. One potential novel intervention to target these post burn symptoms is transcranial direct current stimulation (tDCS). tDCS is a non-invasive brain stimulation (NIBS) technique that modulates neural excitability of a specific target or neural network. The aim of this work is to review the neural circuits of the aforementioned clinical sequelae associated with burn injuries and to provide a scientific rationale for specific NIBS targets that can potentially treat these conditions. We ran a systematic review, following the PRISMA statement, of tDCS effects on burn symptoms. Only three studies matched our criteria. One was a feasibility study assessing cortical plasticity in chronic neuropathic pain following burn injury, one looked at the effects of tDCS to reduce pain anxiety during burn wound care, and one assessed the effects of tDCS to manage pain and pruritus in burn survivors. Current literature on NIBS in burn remains limited, only a few trials have been conducted. Based on our review and results in other populations suffering from similar symptoms as patients with burn injuries, three main areas were selected: the prefrontal region, the parietal area and the motor cortex. Based on the importance of the prefrontal cortex in the emotional component of pain and its implication in various psychosocial symptoms, targeting this region may represent the most promising target. Our review of the neural circuitry involved in post burn symptoms and suggested targeted areas for stimulation provide a spring board for future study initiatives.

Transperineal Ultrasound Assessment of a Cystocele's Impact on the Bladder Neck Mobility in Women with Stress Urinary Incontinence.

Background and objectives: As pelvic floor disorders are often difficult to assess thoroughly based on clinical examination alone, the use of imaging as a complementary technique is helpful. This study's aim was to investigate by transperineal ultrasound (US) if there was any significant difference in the mobility of the bladder neck in women with stress urinary incontinence (SUI) without a cystocele and in those with SUI and an associated cystocele. The study also investigated whether the number of vaginal births and/or the heaviest newborn's birth weight was correlated with the bladder neck mobility. Materials and Methods: A total of 71 women suffering from SUI were included in the study and divided into two groups based on the presence of a cystocele. Their bladder neck mobility was evaluated by transperineal US, calculating the distance from the inferior margin of the symphysis pubis to the bladder neck (SPBN), and the dorsocaudal linear movement (DLM), term used to illustrate the displacement of the bladder neck by subtracting rest and Valsalva values. GraphPad Prism 8 was used for statistical analysis. Results: Within both study groups, the SPBN values were significantly higher and the DLM values were significantly lower at rest as compared to Valsalva maneuver (p < 0.05). No significant difference between the groups regarding SPBN and DLM values at rest, Valsalva, or subtraction was demonstrated. A significant positive correlation was found between the bladder neck mobility and the heaviest newborn's birth weight, regardless of the presence of a cystocele (p = 0.042). Conclusions: The presence of a cystocele had no significant impact on the bladder neck mobility measurements in patients with SUI. The heaviest newborn's birth weight positively correlated with bladder neck hypermobility, as quantified by SPBN.

Peripheral nerve abnormality in HIV leprosy patients.

BACKGROUND: The geographical overlap of HIV (human immunodeficiency virus) and leprosy infection has become increasingly frequent and worrying, bringing many clinical issues. Peripheral neuropathy is very frequent in leprosy because of the predilection of its etiologic agent by Schwann cells of the peripheral nervous system, and it also affects individuals with HIV as one of the most common neurological manifestations. METHODOLOGY/PRINCIPAL FINDINGS: The present study compared a cohort of 63 patients diagnosed with leprosy and coinfected with HIV with a cohort of 64 patients with leprosy alone, who were followed at the outpatient clinic of the Nucleus of Tropical Medicine of the Federal University of Pará, Brazil. We observed that HIV-coinfected leprosy patients presented greater odds of overall peripheral nerve damage (nerve function impairment-NFI) than patients with leprosy alone. More sensitive damage was observed, especially in patients coinfected with multibacillary forms. Leprosy patients coinfected with HIV presented higher chances of motor damage with improvement over time using multidrug therapy (MDT) and highly active antiretroviral therapy (HAART), along with a greater extent of damage and occurrence of neuritis. The data suggest that in addition to patients presenting possible damage caused by leprosy, they also had a greater damage gradient attributable to HIV disease, but not related to HAART because most of these patients had been on the treatment for less than a year. Neuritis was treated with prednisone at doses recommended by the WHO, and coinfected patients had the highest rate of clinical improvement in the first 60 days. CONCLUSIONS/SIGNIFICANCE: The clinical characteristics of the two diseases should be considered in leprosy patients coinfected with HIV for better diagnosis and treatment of peripheral neuropathy. We suggest that new simplified assessment tools that allow the evaluation of the NFI of these patients be developed for use in the service.

Spinal Cord and Peripheral Nerve Abnormalities of the Ruminant.

In food animals, spinal cord damage is most commonly associated with infection or trauma. Antemortem diagnosis is based on clinical signs, history, cerebrospinal fluid analysis, and imaging. As clinical signs are often severe, and prognosis is grave, necropsy may provide a postmortem diagnosis. Peripheral nerve abnormalities are most often the result of trauma. Calving paralysis or paresis is the most common condition affecting the sciatic or obturator nerve and often concurrently involves the peroneal branch of the sciatic. Damage to peripheral nerves is often transient and resolves within a few days as long as the nerve is not severed.

Espectro clínico y valor diagnóstico de los anticuerpos contra el complejo proteico asociado a canales de potasio / Clinical spectrum and diagnostic value of antibodies against the potassium channel related protein complex

Introducción: Los anticuerpos contra un complejo proteico que incluye a los canales de potasio dependientes de voltaje (CKVD) se han descrito en pacientes con encefalitis límbica, hiperexcitabilidad del nervio periférico, síndrome de Morvan, así como en un creciente grupo de síndromes neurológicos. Desarrollo: En este artículo revisamos los síndromes asociados a anticuerpos contra proteínas relacionadas con los CKVD y los 2 antígenos principales de este complejo, las proteínas leucine rich glioma inactivated protein 1 (LGI1) y contactin-associated protein-like 2 (Caspr2). Así mismo describimos los problemas conceptuales y las implicaciones diagnósticas de la descripción de anticuerpos contra CKVD diferentes de LGI1 y Caspr2. Aunque inicialmente se consideró que existían anticuerpos dirigidos contra CKVD, recientemente se ha identificado que, en la mayor parte de los casos, los antígenos son una proteína neuronal secretada denominada LGI1, involucrada en el control de la excitabilidad sináptica, y la proteína Caspr2, localizada en la superficie neuronal de varias regiones cerebrales y en la región yuxtaparanodal de axones mielinizados. Mientras que los anticuerpos contra LGI1 se asocian preferentemente a un cuadro clásico de encefalitis límbica, los anticuerpos contra Caspr2 muestran un espectro clínico más amplio, incluyendo el síndrome de Morvan, la hiperexcitabilidad del nervio periférico o neuromiotonía, o una encefalitis límbica o difusa. Existen además casos descritos de pacientes con anticuerpos contra el complejo CKVD que no tienen anticuerpos contra LGI1 o Caspr2. En estos casos, la identidad y la localización de los antígenos es desconocida, la asociación sindrómica inespecífica y la respuesta al tratamiento, incierta. Conclusiones: El descubrimiento de los antígenos LG1 y Caspr2 ha permitido delimitar clínica y molecularmente el amplio grupo de síndromes previamente atribuidos a anticuerpos contra CKVD. Frente a la literatura que describe la presencia de anticuerpos contra CKVD diferentes a LGI1 y Caspr2, proponemos un algoritmo práctico para el diagnóstico y el tratamiento de estos pacientes

Introduction: Antibodies against a protein complex that includes voltage-gated potassium channels (VGKC) have been reported in patients with limbic encephalitis, peripheral nerve hyperexcitability, Morvan's syndrome, and a large variety of neurological syndromes. Review summary: In this article, a review is presented of the syndromes associated with antibodies against VGKC-related proteins and the main antigens of this protein complex, the proteins LGI1 (leucine rich glioma inactivated protein 1) and Caspr2 (contactin-associated protein-like 2). The conceptual problems and clinical implications of the description of antibodies against VGKC-related proteins other than LGI1 and Caspr2 are also discussed. Although initial studies indicated the occurrence of antibodies against VGKC, recent investigations have shown that the main antigens are a neuronal secreted protein known as LGI1 which modulates synaptic excitability, and a protein called Caspr2 located on the cell surface and processes of neurons of different brain regions, and at the juxtaparanodal region of myelinated axons. While antibodies against LGI1 preferentially associate with classical limbic encephalitis, antibodies against Caspr2 associate with a wider spectrum of symptoms, including Morvan's syndrome, peripheral nerve hyperexcitability or neuromyotonia, and limbic or more extensive encephalitis. In addition there are reports of patients with antibodies against VGKC-related proteins that are different from LGI1 or Caspr2. In these cases, the identity and location of the antigens are unknown, the syndrome association is not specific, and the response to treatment uncertain. Conclusions: The discovery of antigens such as LGI1 and Caspr2 has resulted in a clinical and molecular definition of the broad group of diseases previously attributed to antibodies against VGKC. Considering the literature that describes the presence of antibodies against VGKC other than LGI1 and Caspr2 proteins, we propose a practical algorithm for the diagnosis and treatment of these patients

Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature.

Hereditary sensory and autonomic neuropathy (HSAN) IV is a rare autosomal recessive disorder which is characterized by a decrease in the number of myelinated and non-myelinated nerve fibers of peripheral nerves which causes diminished or absent pain sensation leading to increase in self-mutilative habits. A retrospective study of eight cases ranging from age group of 4 to 17 years for oral and digital signs and symptoms is presented. All the patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe bite injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa) were found in most patients. Our study suggests that early diagnosis and specific treatment plan are important for prevention of characteristic of the oral as well as digital trauma associated with this disorder.

[RESEARCH PROGRESS OF AUTOLOGOUS VEIN NERVE CONDUIT FOR REPAIR OF PERIPHERAL NERVE DEFECT].

OBJECTIVE: To summarize the research progress of autologous vein nerve conduit for the repair of peripheral nerve defect. METHODS: The recent domestic and foreign literature concerning autologous vein nerve conduit for repair of peripheral nerve defect was analyzed and summarized. RESULTS: A large number of basic researches and clinical applications show that the effect of autologous venous nerve conduit is close to that of autologous nerve transplantation in repairing short nerve defect, especially the compound nerve conduit has a variety of autologous nerve tissue, cells, and growth factors, etc. CONCLUSION: Autologous vein nerve conduit for repair of non-nerve defect can be a good supplement of autologous nerve graft, improvement of autologous venous catheter to repair peripheral nerve defect is the research direction in the future.

Variable origin and ramification pattern of the lateral femoral cutaneous nerve: a case report and neurosurgical considerations.

Variations in the anatomy of the lateral femoral cutaneous nerve (LFCN) have been reported in the literature. LFCN is vulnerable to injury during several surgical operations, therefore any surgeon intervening in the area should be familiar to its topographic variability. Lesion of the nerve leads to a condition known as "meralgia paresthetica". We present a cadaveric case of a variant LFCN where two LFCN branches were encountered arising from the lumbar plexus. In specific the anterior LFCN branch originated from the femoral nerve, whereas at the level of the inguinal ligament, four nerve branches were present. The existence of multiple LFCN branches could lead to diagnostic confusion in case of "meralgia paresthetica", while if the neurosurgeon is not aware of the potential variability during surgical decompression of the nerve, postoperative complications may occur. The supernumerary LFCN branches could be identified by ultrasound imaging and be used as optimum vascularized grafts for sensory nerve repair.

Regeneración de las lesiones críticas del nervio periférico con factores de crecimiento. Estudio experimental / Regeneration of critical injuries of the peripheral nerve with growth factors

Introducción. El objetivo del proyecto es estudiar la regeneración de las lesiones no reparables del nervio periférico, mediante un injerto muscular enriquecido con factores de crecimiento. Material y método. La experimentación se desarrolla en 2 fases: primero, comparamos la sutura directa del defecto crítico en el nervio ciático de 10 ratas, con la interposición de un injerto de músculo autólogo desnaturalizado por calor en otras 10. En la segunda, se comparan 10 ratas con reparación mediante injerto muscular acelular, con la inyección de 2 cc de IGF-1 (10 mg/ml de mecasermina, en solución inyectable) dentro del injerto acelular de otras 10. Realizamos el seguimiento clínico y el control funcional de la marcha, medición de la huella plantar y «Grasping Test». Fueron sacrificadas a los 90-100 días, obteniendo muestras para macro y microscopía, con tinciones de azul de toluidina, hematoxilina-eosina y tricrómico de Masson. Resultados. La primera experimentación demostró el hallazgo de tejido de características nerviosas en las secciones del injerto muscular. La segunda supuso una potenciación de los resultados: mejoría clínica posquirúrgica, precoz deambulación, descenso en la tasa de úlceras por presión en partes acras, recuperación de la huella plantar, e incremento del porcentaje de terminaciones nerviosas en regeneración del cabo distal (47-62%). Conclusiones. Exponemos en este trabajo las posibilidades experimentales y clínicas de la reparación del defecto nervioso mediante músculo desnaturalizado, confirmando la adecuación de la técnica. Además, confirmamos nuestra hipótesis con clínica y determinaciones celulares enriquecidas por la adicción de factores de crecimiento que impulsan la regeneración nerviosa (AU)

Introduction. This project aims to study the regeneration of non-repairable lesions of peripheral nerve by muscle grafts enhanced with growth factors. Material and methods. The experiment was carried out in two phases. The first one compared direct suture of a critical defect in the sciatic nerve of ten rats, with the interposition of autologous muscle graft, denatured by heat, in another ten. The second phase compared ten rats with nerve repair using an acellular muscle graft, with injection of 2 cc of IGF-1 (10 mg/ml mecasermin, Injectable solution) into the acellular graft of another ten. A clinical and functional follow-up was carried out including, ambulation, footprint measurement, and «Grasping Test». The animals were sacrificed at 90-100 days, and samples obtained for macro- and microscopic studies with toluidine blue, haematoxylin-eosin and Masson's trichrome staining. Results. The first experiment showed the characteristic findings of nerve tissue in muscle graft level sections. The second was an enhancement of the results: post-surgical clinical improvement, early ambulation, decrease in the rate of pressure ulcers in toes, recovery of the footprint, and increasing the percentage of nerve endings in distal sciatic regeneration (47-62%). Conclusions. In this study the experimental and clinical possibilities of nerve defect repair by denatured muscle are demonstrated, confirming the suitability of the technique. Furthermore, it confirms our hypothesis with clinical and cellular determinations enriched by the addition of growth factors that promote nerve regeneration (AU)

Symptomatic neural loop causing hemidigital anesthesia: case report.

Digital neural loops were identified over a century ago and are common findings in cadaveric studies of palmar and digital anatomy. Symptomatic digital neural loops are rare. We report a case of hemidigital anesthesia resulting from a proper digital nerve neural loop penetrated by its common digital artery in the palm. After neurolysis of the median nerve and the common and proper digital nerves to the third webspace, we transected the common digital artery, transposed it out of the neural loop, and repaired it. The patient's sensory symptoms fully resolved over 6 weeks. The differential diagnosis, diagnostic workup, and surgical treatment are reviewed.

Inervación anómala múltiple de la mano en una paciente. Diagnóstico electrofisiológico / Multiple abnormal innervation of the hand in one patient. Electrophysiological diagnosis

Introducción. Las anastomosis nerviosas en la mano constituyen desviaciones de la norma anatómica. No son motivo deenfermedad, pero sí eventos relacionados con dificultades electrodiagnósticas, y por su presentación inesperada tambiéncon yatrogenia en cirugías regionales. Caso clínico. Describimos el estudio neurofisiológico de una mujer de 45 años en la que demostramos una anastomosis motora medianocubital en la palma de tipo Riche-Cannieu y dos variantes sensitivas, inervación completa del dedo IV por el cubital e inervación completa por el radial superficial del dorso de la mano.Conclusiones. Las descripciones anatómicas en la mano de inervaciones anómalas son frecuentes, en especial las sensitivas. No suelen provocar problemas, aun cuando se lesione la rama comunicante, probablemente por la habitual escasa participación de axones en la anastomosis. Saber reconocerlas mediante electroneurografía no es difícil si estamos al tanto de sus tipologías y conocemos con rigor los recorridos anatómicos; de este modo podremos desarrollar precisosprotocolos diagnósticos. El conocimiento de estas variantes evitará errores electrodiagnósticos y yatrogenia quirúrgica (AU)

Introduction. Nerve anastomoses in the hand are deviations from the anatomical norm. They do not lead to illness, but still they are events related with electrodiagnostic difficulties and, due to their unexpected presentation, also withiatrogenesis in regional surgical interventions. Case report. We report the neurophysiological study conducted on a 45-year-old female who was found to have Riche-Cannieu-type motor anastomosis between the median and ulnar branches in the palm of the hand, complete innervation of the 4th finger by the ulnar nerve and complete innervation by the superficial radial of the back of the hand. Conclusions. Anatomical descriptions of abnormal innervations in the hand are frequent, especially of the sensorykind. They do not usually give rise to any problems, even when the communicating branch is injured, probably owing to the scant participation of axons that usually occurs in anastomosis. They are not difficult to recognise by means ofelectroneurography if we are aware of their different types and we have a good knowledge of the anatomical routes. This will enable us to develop accurate diagnostic protocols. Knowledge of these variants will prevent electrodiagnostic errors and surgical iatrogenic effects from occurring (AU)

Morphological abnormalities of embryonic cranial nerves after in utero exposure to valproic acid: implications for the pathogenesis of autism with multiple developmental anomalies.

Autism is often associated with multiple developmental anomalies including asymmetric facial palsy. In order to establish the etiology of autism with facial palsy, research into developmental abnormalities of the peripheral facial nerves is necessary. In the present study, to investigate the development of peripheral cranial nerves for use in an animal model of autism, rat embryos were treated with valproic acid (VPA) in utero and their cranial nerves were visualized by immunostaining. Treatment with VPA after embryonic day 9 had a significant effect on the peripheral fibers of several cranial nerves. Following VPA treatment, immunoreactivity within the trigeminal, facial, glossopharyngeal and vagus nerves was significantly reduced. Additionally, abnormal axonal pathways were observed in the peripheral facial nerves. Thus, the morphology of several cranial nerves, including the facial nerve, can be affected by prenatal VPA exposure as early as E13. Our findings indicate that disruption of early facial nerve development is involved in the etiology of asymmetric facial palsy, and may suggest a link to the etiology of autism.

Ultrasound diagnosis of peroneal nerve variant in a child with compressive mononeuropathy.

We report on a 6-year-old child presenting with subacute foot drop. Neurophysiologic and radiologic studies revealed a peroneal nerve compression secondary to fibular exostosis. Before undergoing surgical removal of the exostosis, the patient underwent further neurophysiologic and ultrasonographic evaluation that showed the presence of an accessory peroneal nerve branch that caused gastrocnemius involvement. Findings at surgery confirmed the supposed anatomical variant. Both nerve components were carefully preserved during the operative procedure. The association of ultrasonographic and neurophysiologic studies was crucial in identifying the etiopathologic mechanism and anatomical picture and provided clinicians and surgeons with important information in planning the procedure.

Plexopatía braquial secundaria a tumor maligno de la vaina del nervio periférico / Brachial plexopathy secondary a malignant tumor of the peripheral nerve sheath

Introducción: el plexo braquial puede verse afectado por patología neoplásica tanto primaria como secundaria. Los tumores primarios del plexo braquial son entidades poco frecuentes, aunque algunos, como el tumor maligno de la vaina del nervio periférico pueden tener un comportamiento agresivo. Caso clínico: se presenta una mujer de 31 años con disestesias y debilidad progresiva en el miembro superior izquierdo. El estudio neurofisiológico mostró afectación del plexo braquial izquierdo. En la resonancia magnética se observó una masa de tejido blando que invadía el plexo braquial. El estudio histológico fue compatible con un tumor maligno de la vaina del nervio periférico. Conclusiones: el tumor maligno de la vaina del nervio periférico es un tumor altamente agresivo que puede aparecer en pacientes sin datos clínicos de neurofibromatosis tipo 1. Debe mantenerse un elevado nivel de sospecha con el objetivo de no retrasar el diagnóstico para así poder realizar un tratamiento lo más conservador posible.

Introduction. Malignant peripheral nerve sheath tumor (MPNST) are sarcomas that are rarely located in the upper limb. Clinical case. We present a 31- year-old woman with progressive dysesthesia and weakness of the left upper limb. The neurophysiological study showed damage in the left brachial plexus. A soft tissue mass that was invading the plexus was observed in the magnetic resonance image. The anatomopathological study was compatible with MPNST diagnosis. Conclusions. Intrinsic tumors of the brachial plexus are uncommon. A MPNST is an extremely aggressive mesenchymal tumor that is seldom rooted in the brachial plexus.

Clinical sonopathology for the regional anesthesiologist: part 1: vascular and neural.

The use of ultrasound to facilitate regional anesthesia is an evolving area of clinical, education, and research interests. As our community's experience grows, it has become evident that anesthesiologists performing "routine" ultrasound-guided blocks may very well be confronted with atypical or even pathologic anatomy. As an educational resource for anesthesiologists, the following articles present examples of common sonopathology that may be encountered during ultrasound-guided regional anesthesia. This present article describes sonopathology related to blood vessels and nerves.