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INTRODUCTION: Neuralgic amyotrophy (NA) is a painful non-traumatic peripheral nervous system condition affecting the brachial plexus. Signal abnormalities in nerves and muscles have been detected in these patients using magnetic resonance neurography (MRN). METHODS: Electronic medical records and MRN images obtained in a 3 T scanner, in 14 adult patients diagnosed with NA at our Neurological institution (Neuromuscular Disorders Section), between December 2015 and December 2019 were retrospectively reviewed. The study was first approved by our Institutional Ethics Committee. RESULTS: Subclinical, multifocal, and bilateral nerve signal anomalies were recorded in the brachial plexus of these patients. We identified four different types of nerve constriction without entrapment, which we categorized as follows: incomplete focal (type I), complete focal or hourglass (type II), multifocal or string of pearls (type III) and segmental (type IV). CONCLUSIONS: Given that MRN is an accurate diagnostic tool to detect nerve damage, we believe abnormal findings could improve early detection of NA patients.

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The present academic work aims to contribute to an early diagnosis of neuralgic amyotrophy (NA) because of its high prevalence in the population. This disease is a neuromuscular syndrome with unclear etiology; it affects mostly the brachial plexus, causing acute pain in the affected shoulder, paralysis, and disabilities. Considering the importance of an early treatment that can modify the prognosis of the patient, knowing the last updates about the syndrome as its clinical presentation is important. Data analysis was conducted through an online non-systematic review that indicated the epidemiology, pathophysiology, and differential diagnosis and prognosis of NA. Knowledge of the clinical features of NA is not common; however, it is important in orthopedic practice because it requires differentiation from spine pathologies.

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BACKGROUND: Hirayama's disease (HD) is characterized by an insidious onset asymmetric weakness and atrophy of the forearm and hand. Taking as a premise, the etiopathogenesis of the disease is attributed to forward displacement of posterior wall of lower cervical dural canal in neck flexion causing marked compression and flattening of lower spinal cord. This may result in compression of the posterior column of the spinal cord and seems likely to result in somatosensory evoked potentials (SSEPs) abnormalities. In the present study, we studied the possible involvement of the lemniscal dorsal pathway in patients with HD. METHODS: SSEPs in upper and lower extremities were prospectively performed in eight patients with HD. All the patients were recruited from the outpatient clinic of a neuromuscular disorder center from South Brazil. SSEPs were obtained by transcutaneous electrical stimulation of the median and posterior tibial nerves, on both sides. We collected the amplitude and the latency of the different components obtained in each channel. The interpretation was based on Brazilian study standards. RESULTS: We evaluated seven men and one woman (mean age 27). The data obtained were compared to a control group consisting of eight patients with spondylotic cervical myelopathy, 6 men and 2 women with mean age of 59 years. The measurements of obtained by the SSEP were also compared between the groups and no significant difference was found for any of them. CONCLUSION: SSEP did not turn out to be an electrophysiological marker in our HD patients.

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Although relatively rare, post-operative nerve injuries may occur after cervical spine procedures. The most common post-operative neural disorder is C5 nerve palsy. The risk factors for C5 nerve palsy are male gender, OPLL, and posterior cervical approaches. It generally presents with deltoid and/or biceps weakness, and may present immediately or several days after surgery. Treatment is generally conservative due to transient duration of symptoms, but evaluation of residual compression at C4-5 is essential. PTS (Parsonage-Turner syndrome) is an idiopathic plexopathy generally presenting with severe neuropathic pain in the shoulder, neck, and arms, followed by neurological deficits involving the upper brachial plexus. The deficits typically present in a delayed fashion after the onset of pain. Once residual nerve compression is ruled out, initial treatment is based on pain control and physical therapy. Post-operative C8-T1 nerve palsies occur with weakness of the five intrinsic muscles of the hand innervated by the medial nerve, with sensory symptoms in the territory innervated by the ulnar nerve (ulnar two digits of the hand), and also the medial forearm. The risk factors for C8-T1 nerve injuries after surgery are C7 pedicle subtraction osteotomies and posterior fixation of the cervico-thoracic junction, especially in patients with preoperative C7-T1 stenosis. A wide foraminal decompression at C7-T1 region is necessary to minimize risk of this complication. Finally, Horner's syndrome can occur post-operatively, especially after anterolateral approaches to the middle and lower levels of the cervical spine. It is characterized by ipsilateral papillary miosis, facial anhydrosis, and ptosis secondary to injury of the cervical sympathetic nerves. Avoid using the cautery on the lateral border of the longus colli muscle, where the sympathetic chain lies and place the retractors properly underneath the muscle to decrease the chance of sympathetic injuries. It can also occur from iatrogenic compression or injury to the T1 nerve root, as the sympathetic chain gets some of its fibers from that level. Understanding the most common potential nerve injuries after cervical spine procedures is helpful in prevention, early diagnosis, and appropriate management.

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BACKGROUND: Spinocerebellar ataxia type 2 (SCA2) is due to a CAG expansion (CAGexp) at ATXN2. SCA2 presents great clinical variability, alongside characteristic ataxia with saccadic slowness. AIMS: To study parkinsonism, dementia, dystonia, and amyotrophy as subphenotypes of SCA2, and to explore the effect of CAG repeats at different loci and of mitochondrial polymorphism A10398G as modifiers of phenotype. METHODS: Symptomatic subjects were classified by presence/absence of neurological signs mentioned above; SARA and NESSCA scores were obtained. CAG repeats at ATXN1, ATXN2, ATXN3, CACNA1A, ATXN7 and RAI1, and polymorphism A10398G at mtDNA were established. Group characteristics were compared, with a p < 0.05. RESULTS: Forty-eight SCA2 individuals were included. Age at onset, CAGexp, and disease duration explained 53% and 43% of SARA and NESSCA variations, respectively. CAGexp of subjects with and without parkinsonism were different (medians of 42 and 39 repeats) as well as of subjects with and without dystonia (44 and 40 repeats). Amyotrophy was not significantly related to any variable under study. Concerning polymorphism A10398G, 83% of subjects with and 34% of those without cognitive decline carried 10398G at (p = 0.003). DISCUSSION: Treating the four phenotypic subgroups as outcomes was a valid strategy to identify modifiers of disease. Among correlations found, some confirmed previous reports, such as that between dystonia and CAGexp. Of note was the association between cognitive decline and the variant G at mitochondrial polymorphism A10398G, a variant formerly related to earlier ages at onset in SCA2.

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Refletir sobre os aspectos que envolvem o desligamento de um praticante portador de Amiotrofia Muscular Espinhal do programa de equoterapia, quando se vê confrontado com prognóstico desfavorável à sua permanência no mesmo, especialmente se este prognóstico indica terminalidade com previsão de morte próxima, é o objetivo do presente estudo, de caráter bibliográfico, que descreve aspectos do tratamento fisioterápico, psicológico e de cuidados de responsabilidade do equitador que podem beneficiar o portador;apresenta sucintamente a Teoria do Apego de John Bowlby que versa sobre vínculos psicológicos e afetivos; demonstra a importância da formação desses vínculos na equoterapia e possíveis implicações de seu rompimento com foco nos aspectos bioéticos envolvidos, especialmente quanto à autonomia do sujeito em relação à própria morte. Conclui-se pela indicação de que equipe interdisciplinar de equoterapia e família considerem a possibilidade de buscar alternativas para a manutenção do convívio do praticante com o cavalo e com o ambiente equoterápico, ainda que sem a montaria, pelo maior tempo possível, se assim for o seu desejo e, também, pela indicação de que se invista em pesquisas sobre o tema, que, por sua incipiência e importância carece do olhar atento dos pesquisadores da área.

Reflecting on the aspects that involve the detachment of a practitioner of Spinal Muscular Amyotrophy from the equine therapy program, when confronted with a prognosis unfavorable to its permanence in the same, especially if this prognosis indicates terminality with near death prediction, is the objective of the Present study, of bibliographical character, that describes aspects of the physiotherapeutic, psychological and care treatment of the equitador that can benefit the bearer; Presents succinctly John Bowlby's Theory of Attachment that deals with psychological and affective bonds; Demonstrates the importance of forming these links in equine therapy and the possible implications of their disruption with a focus on the bioethical aspects involved, especially regarding the autonomy of the subject in relation to death itself. The conclusion is that the interdisciplinary equine and family team consider the possibility of seeking alternatives for the maintenance of the practitioner's life with the horse and the equotherapeutic environment, even if without riding, for as long as possible, if this is the case. His desire and joint decision of the family and team and also by the indication that he is investing in research on the subject, which, due to his incipience and importance, needs the close attention of researchers in the area.

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A different and rare type of atrophy with a predominant pattern in scapulo-peroneal distribution was described by Davidenkow in 1939. The syndrome was characterized by some researchers as a variant of Charcot-Marie-Tooth disease, however Davidenkow noticed that clinical and laboratorial manifestations did not corroborate exactly with this hypothesis. We describe a case of a female patient, 39 years-old, clinical picture similar to the syndrome described by Davidenkow, presenting scapulo-peroneal atrophy. Her first symptoms had appeared when she was 24, initially with proximal motor weakness in the upper limbs. This patient did not have family history of myopathy or neuropathy. Several tests were performed to exclude other syndromes that could be included in the differential diagnosis, by testing gene mutation, in addition to the physical examination and electromyography. The large spectrum of neuromuscular diseases makes difficult the diagnosis of Davidenkow's syndrome which always should be considered in the differential diagnosis.

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En 1939 Davidenkow describió un tipo de atrofia diferente y rara con un patrón predominante en distribución escápulo-peroneal. Algunos investigadores caracterizaron el síndrome como una variante de la enfermedad de Charcot-Marie-Tooth; sin embargo, Davidenkow percibió que las manifestaciones clínicas y de laboratorio no corroboraban exactamente esta hipótesis. Describimos el caso de una mujer de 39 años, con cuadro clínico semejante al síndrome descrito por Davidenkow, presentando atrofia escápulo-peroneal. Sus primeros síntomas comenzaron cuando tenía 24 años, inicialmente con debilidad motora proximal en los miembros superiores. No tenía historia familiar de miopatía o neuropatía y se excluyeron otros síndromes que se podrían incluir entre los diagnósticos diferenciales mediante la realización de pruebas de mutación genética, además del examen físico y electromiografía. El amplio espectro de enfermedades neuromusculares a veces dificulta su diagnóstico y debe ser siempre considerado en el diagnóstico diferencial.

A different and rare type of atrophy with a predominant pattern in scapulo-peroneal distribution was described by Davidenkow in 1939. The syndrome was characterized by some researchers as a variant of Charcot-Marie-Tooth disease, however Davidenkow noticed that clinical and laboratorial manifestations did not corroborate exactly with this hypothesis. We describe a case of a female patient, 39 years-old, clinical picture similar to the syndrome described by Davidenkow, presenting scapulo-peroneal atrophy. Her first symptoms had appeared when she was 24, initially with proximal motor weakness in the upper limbs. This patient did not have family history of myopathy or neuropathy. Several tests were performed to exclude other syndromes that could be included in the differential diagnosis, by testing gene mutation, in addition to the physical examination and electromyography. The large spectrum of neuromuscular diseases makes difficult the diagnosis of Davidenkow’s syndrome which always should be considered in the differential diagnosis.

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La Enfermedad de Hirayama —o amiotrofia monomélica— es una afección de baja frecuencia y escasamente reportada en la edad pediátrica. Se presenta el caso clínico de un adolescente de 15 años de edad con disminución de la fuerza muscular y pérdida de la masa muscular, que comenzó a los 10 años de edad por la mano izquierda, y le afectó posteriormente el antebrazo. Mantuvo un curso progresivo durante 3 años, para luego mantenerse estable. El electromiograma de aguja arrojó lesión de axones motores o motoneuronas dependientes de los miotomas C7-T1, y en menor grado, C5-C6. En la tomografía axial computarizada con contraste endovenoso en marcada flexión cervical, se observó desde C7-T2 una evidente ectasia venosa posmedular asimétrica, predominantemente del lado izquierdo, por congestión del plexo venoso vertebral posterior interno. En este paciente la enfermedad se detuvo espontáneamente, en otros casos es necesario limitar la motilidad de la columna con el uso de un collar cervical, y solo llegar a la cirugía en los casos más severos de evolución rápida(AU)

Hirayama disease or monomelic amyotrophy is a low frequent, barely reported illness at pediatric ages. Here is the clinical case of 15 years-old boy that presented with reduced muscular strength and loss of muscle mass; this condition began at the age of 10 year in his left hand and then affected the forearm. The illness progressed for three years and then remained stable. The needle electromyogram showed a lesion in C7-T1 myotome-depending motor axons or motoneurons and to less extent in those C5-C6 depending ones. The venous contrast computed tomography on a marked cervical cord flexion position; it was observed an evident asymmetric postmedullary vein ectasia from the C7-T2 myotomes, mainly on the left side, caused by the internal posterior vertebral vein plexus congestion. There was spontaneous remission of the disease in this patient, but it is necessary in other cases to limit the cervical cord motility with the use of a collar and to only perform surgery in the most rapidly evolving and severe cases(AU)

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La Enfermedad de Hirayama -o amiotrofia monomélica- es una afección de baja frecuencia y escasamente reportada en la edad pediátrica. Se presenta el caso clínico de un adolescente de 15 años de edad con disminución de la fuerza muscular y pérdida de la masa muscular, que comenzó a los 10 años de edad por la mano izquierda, y le afectó posteriormente el antebrazo. Mantuvo un curso progresivo durante 3 años, para luego mantenerse estable. El electromiograma de aguja arrojó lesión de axones motores o motoneuronas dependientes de los miotomas C7-T1, y en menor grado, C5-C6. En la tomografía axial computarizada con contraste endovenoso en marcada flexión cervical, se observó desde C7-T2 una evidente ectasia venosa posmedular asimétrica, predominantemente del lado izquierdo, por congestión del plexo venoso vertebral posterior interno. En este paciente la enfermedad se detuvo espontáneamente, en otros casos es necesario limitar la motilidad de la columna con el uso de un collar cervical, y solo llegar a la cirugía en los casos más severos de evolución rápida.

Hirayama disease or monomelic amyotrophy is a low frequent, barely reported illness at pediatric ages. Here is the clinical case of 15 years-old boy that presented with reduced muscular strength and loss of muscle mass; this condition began at the age of 10 year in his left hand and then affected the forearm. The illness progressed for three years and then remained stable. The needle electromyogram showed a lesion in C7-T1 myotome-depending motor axons or motoneurons and to less extent in those C5-C6 depending ones. The venous contrast computed tomography on a marked cervical cord flexion position; it was observed an evident asymmetric postmedullary vein ectasia from the C7-T2 myotomes, mainly on the left side, caused by the internal posterior vertebral vein plexus congestion. There was spontaneous remission of the disease in this patient, but it is necessary in other cases to limit the cervical cord motility with the use of a collar and to only perform surgery in the most rapidly evolving and severe cases.

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Hirayama disease is a rare neurological disorder characterized by an insidious progressive subacute unilateral or bilateral weakness of the hands and forearm muscles leading to a painless amyotrophy. The disease primarily affects young men in the second to third decades of life. It has always been described as a second motor neuron disease, thus sparing the pyramidal and sensitive pathways. It usually has a slow progression course of 3 to 5 years followed by stabilization. Since its initial description by Keyzo Hirayama in 1959, most cases have been reported in Asia, particularly Japan and India, although the disease reportedly has worldwide distribution.

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Introducción: la neuralgia amiotrófica o síndrome de Parsonage-Turner se asocia a vacunaciones en 15 por ciento de los casos. No existen reportes que vinculen esta condición con la vacuna contra la leptospirosis humana. Objetivo: describir las características clínicas de un paciente que presentó una plexopatía braquial aguda posterior a la inmunización contra la leptospirosis humana. Presentación del caso: un varón de 31 años de edad, obrero agrícola en 2008 recibió una dosis de la vacuna cubana contra la leptospirosis humana (vax-Spiral); 3 semanas después presentó dolor intenso localizado en ambos hombros, a la semana siguiente aparece debilidad muscular y posteriormente atrofia del músculo deltoides derecho. La evaluación neurológica era compatible con afección aguda asimétrica de la porción superior del plexo braquial, con mayor intensidad en el lado derecho, en el cual también estaba involucrado el diafragma. Se comprobó el compromiso de la porción superior del plexo braquial con estudios de neuroconducción y electromiografía. El paciente es tratado con analgésicos y fisioterapia, evolucionando de modo favorable. Conclusiones: el evento ocurrido en este caso sugiere que la vacuna cubana contra leptospirosis humana pudiera producir inmunorreactividad cruzada contra antígenos del sistema nervioso periférico(AU)

Introduction: neuralgic amyotrophy or Parsonage-Turner syndrome is associated with vaccination in 15 percent of cases. There are no reports linking this condition to the vaccine against human leptospirosis. Objective: describe the clinical characteristics of a patient who developed acute brachial plexopathy after vaccination against human leptospirosis. Case presentation: a male 31 year-old agricultural worker received a dose of the Cuban vaccine against human leptospirosis (vax-SPIRAL) in 2008. Three weeks later he presented intense pain in both shoulders. The following week he had muscular weakness, and then atrophy of the right deltoid muscle. Neurological evaluation showed acute asymmetric damage to the upper brachial plexus, with greater intensity on the right side, and involvement of the diaphragm. Nerve conduction examination and electromyography revealed involvement of the upper brachial plexus. The patient was treated with analgesics and physical therapy, and was found to evolve favorably. Conclusions: the events described suggest that the Cuban vaccine against human leptospirosis might produce immunological cross-reactivity against antigens of the peripheral nervous system(AU)

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Introducción: la neuralgia amiotrófica o síndrome de Parsonage-Turner se asocia a vacunaciones en 15 por ciento de los casos. No existen reportes que vinculen esta condición con la vacuna contra la leptospirosis humana. Objetivo: describir las características clínicas de un paciente que presentó una plexopatía braquial aguda posterior a la inmunización contra la leptospirosis humana. Presentación del caso: un varón de 31 años de edad, obrero agrícola en 2008 recibió una dosis de la vacuna cubana contra la leptospirosis humana (vax-Spiral); 3 semanas después presentó dolor intenso localizado en ambos hombros, a la semana siguiente aparece debilidad muscular y posteriormente atrofia del músculo deltoides derecho. La evaluación neurológica era compatible con afección aguda asimétrica de la porción superior del plexo braquial, con mayor intensidad en el lado derecho, en el cual también estaba involucrado el diafragma. Se comprobó el compromiso de la porción superior del plexo braquial con estudios de neuroconducción y electromiografía. El paciente es tratado con analgésicos y fisioterapia, evolucionando de modo favorable. Conclusiones: el evento ocurrido en este caso sugiere que la vacuna cubana contra leptospirosis humana pudiera producir inmunorreactividad cruzada contra antígenos del sistema nervioso periférico

Introduction: neuralgic amyotrophy or Parsonage-Turner syndrome is associated with vaccination in 15 percent of cases. There are no reports linking this condition to the vaccine against human leptospirosis. Objective: describe the clinical characteristics of a patient who developed acute brachial plexopathy after vaccination against human leptospirosis. Case presentation: a male 31 year-old agricultural worker received a dose of the Cuban vaccine against human leptospirosis (vax-SPIRAL) in 2008. Three weeks later he presented intense pain in both shoulders. The following week he had muscular weakness, and then atrophy of the right deltoid muscle. Neurological evaluation showed acute asymmetric damage to the upper brachial plexus, with greater intensity on the right side, and involvement of the diaphragm. Nerve conduction examination and electromyography revealed involvement of the upper brachial plexus. The patient was treated with analgesics and physical therapy, and was found to evolve favorably. Conclusions: the events described suggest that the Cuban vaccine against human leptospirosis might produce immunological cross-reactivity against antigens of the peripheral nervous system

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Benign monomelic amyotrophy (BMA) is a rare form of motor neuron disease of unknown cause and it?s characterized by weakness and muscular atrophy restricted to an upper or lower limb, usually in the distal part, absence of upper motor neuron signs and self limited course. In this report we describe the clinical and electromyographic features of two young men with BMA in the proximal portion of the right upper limb, a location of BMA rarely described in the literature.

Amiotrofia Monomélica Benigna (AMB) é uma entidade rara do neurônio motor, de causa desconhecida, caracterizada por fraqueza muscular e amiotrofia restritas a 1 membro superior ou inferior, frequentemente em terço distal, sem comprometimento da via piramidal e cursoautolimitado. Descrevemos os achados clínicos e eletroneuromiográficos de 2 pacientes jovens com AMB na porção proximal do membro superior, localização raramente descrita na literatura.

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