Enfermedad de hirayama en un adolescente / Hirayama disease observed in an adolescent

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.

Brachioradial pruritus in a patient with cervical disc herniation and Parsonage-Turner syndrome

Abstract Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-yearold woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.

Extended Neuralgic Amyotrophy Syndrome: voice therapy in one case of vocal fold paralysis / Amiotrofia Nevrálgica Estendida: fonoterapia em um caso de paralisia de prega vocal

Neuralgic Amyotrophy (NA) is a rare disturb of the peripheral nervous system that can include extreme pain, multifocal paresis and atrophy of the muscles of the upper limbs. When the nerves located outside of the brachial plexus are involved, the term Neuralgic Amyotrophy Extended (ANE) is used. Diagnosis of NA is clinical and has a series of inclusion and compatibility criteria established by the European CMT Consortium. On this study the clinical history, multidimensional vocal assessment data and the vocal techniques used in five-weeks voice therapy for one patient, professional voice, with ANE are presented. In this case, sudden and recurrent paralysis of his right vocal fold was the only manifestation of the disease. At the end of the fifth week the patient's voice was normal, the spoken and sung vocal ranges were same as before the current episode of ANE and scores of his vocal self-assessment were appropriate.

A Amiotrofia Nevrálgica (AN) é um distúrbio raro do sistema nervoso periférico que pode incluir dor extrema, paresia multifocal e atrofia dos músculos dos membros superiores. Quando há o envolvimento de nervos localizados fora do plexo braquial, o termo Amiotrofia Nevrálgica Estendida (ANE) é utilizado. O diagnóstico da AN é clínico e possui uma série de critérios de inclusão, bem como critérios de compatibilidade estabelecidos pelo European CMT Consortium. Neste estudo são apresentados a história clínica, os dados da avaliação vocal multidimensional e as técnicas vocais utilizadas na terapia vocal de cinco semanas de um paciente, profissional da voz, com ANE. A paralisia súbita e recorrente da prega vocal direita foi a única manifestação da doença. Ao término da quinta semana, a voz do paciente estava adaptada, as extensões de voz falada e cantada eram as mesmas de antes da lesão e os escores de sua autoavaliação vocal estavam adequados.

Radiofrequency Neurotomy of Cervical Medial Branches for Chronic Cervicobrachialgia

Chronic neck and arm pain or cervicobrachialgia commonly occurs with the degeneration of cervical spine. Authors investigated the usefulness of radiofrequency (RF) neurotomies of cervical medial branches in patients with cervicobrachialgia and analyzed the factors which can influence the treatment outcome. Demographic data, types of pain distribution, responses of double controlled blocks, electrical stimulation parameters, numbers and levels of neurotomies, and surgical outcomes were evaluated after mean follow-up of 12 months. Pain distribution pattern was not significantly correlated with the results of diagnostic blocks. Average stimulation intensity was 0.45 V, ranging from 0.3 to 0.69, to elicit pain response in cervical medial branches. The most common involvement of nerve branches was C4 (89%), followed by C5 (82%), C6 (75%), and C7 (43%). Among total of 28 patients, nineteen (68%) reported successful outcome according to outcome criteria after 6 months of followup (p=0.001), and eight (42%) of 19 patients reported complete relief (100%) of pain. Four patients showed recurrence of pain between 6 and 12 months. It was therefore concluded that cervical medial branch neurotomy is considered useful therapeutic modality for the management of cervicobrachialgia in selected patients, particularly in degenerative zygapophyseal disorders.

Acute brachial neuropathy: electrophysiological study and clinical profile

Acute brachial neuropathy (ABN) is a rare disease, characterized by an acute or subacute onset of pain followed by weakness of shoulder or arm muscles without trauma or traction injury. So the diagnosis of this clinical entity is not easy. The purpose of this study was to analyze retrospectively the ABN in 14 cases focusing on the clinical profile and to evaluate the effectiveness of electrophysiologic study in diagnosis of ABN with a new result helpful in localizing a brachial plexus disorder. The most helpful electrophysiologic data of ABN in my patients seemed to be abnormalities of low amplitude, abnormal right to left difference of compound motor action potentials (CMAPs) and sensory nerve action potentials (SNAPs) in axillary nerve, ulnar or median nerves. Results of nerve conduction velocity, terminal and F-wave latency were not as useful. But the electromyogram was most helpful in localization of upper or lower plexus lesions and cervical radiculopathy. The most striking clinical feature of ABN was the rapid onset of pain followed by the development of muscle weakness of shoulder girdle after a variable period or within four days. In contrast to other reports, intrinsic hand muscle weakness was observed in 3 cases with sensory changes in ulnar nerve distribution. The cervical radiculopathies (C5-C7 roots) were simultaneously combined with ipsilateral axillary neuropathy in 3 cases. In this study, decreased amplitude, abnormal right to left difference of SNAPs and CMAPs, and neurogenic EMG findings with normal data of NCV, terminal and F-wave latencies suggest that the pathology of ABN might not be a demyelinating process, but axonopathy.