Diagnosis and treatment of 11 patients with cevical spondylotic amyotrophy / 中国骨伤

OBJECTIVE@#To explore clinical features, treatment methods and clinical effects of cervical spondylosis with proximal muscular atrophy.@*METHODS@#Eleven patients with proximal-type cervical spondylotic amyotrophy were retrospectively studied from September 2016 to November 2020, including 7 males and 4 females, aged 38 to 68 years old. Clinical symptoms, MRI and neuroelectrophysiological manifestations were analyzed, and patients were treated with conservative treatment or anterior cervical decompression fusion surgery, respectively. The efficacy was evaluated by manual muscle test (MMT) before and after treatment, and patients' satisfaction was followed up at the same time.@*RESULTS@#All patients were followed up for 6 to 19 months. All 11 patients were unilateral, mainly manifested by atrophy of deltoid muscle, supraspinatus muscle and infraspinatus muscle, and may be accompanied by ipsilateral neck and shoulder pain at early stage. MRI showed lesions at C4,5, C5,6 segments were more common. Electrophysiological examination showed the affected muscle was denervated, and amplitude of compound muscle action potential (CMAP) of innervated nerve on the affected side was lower than that on the healthy side. All patients were obtained bone fusion. One patient who were underwent anterior cervical corpectomy and fusion (ACCF) occurred developed contralateral C5 nerve root paralysis after operation, which recovered completely after 10 weeks of symptomatic treatment. At 12 months after operation, the efficacy was evaluated according to MMT, 3 patients were treated conservatively, 2 patients excellent and 1 good;in 8 patients treated by operation, 3 patients were excellent, 4 good, and 1 moderate.@*CONCLUSION@#The incidence of cervical spondylosis with proximal muscular atrophy is low, which is manifested as unilateral proximal muscle atrophy and may be accompanied by ipsilateral neck and shoulder pain in the early stage. Combined with MRI and neuroelectrophysiological examination, misdiagnosis could be reduced. In the early stage of disease, especially in the case of nucleus pulposus protrusion leading to nerve compression, conservative treatment could be taken. When the conservative treatment is ineffective or the pain cannot be tolerated, anterior decompression surgery is recommended, and the overall effect is satisfactory.

Course of Rehabilitation Treatment of Neuralgic Amyotrophy Presenting with Collet-Sicard Syndrome / The Japanese Journal of Rehabilitation Medicine

Typical cases of neuralgic amyotrophy present with sudden onset of excruciating pain in the shoulders and upper extremities, followed by marked muscle weakness and atrophy over a period of hours to days. Neuralgic amyotrophy is not confined to the brachial plexus, and difficulties in its diagnosis may delay the start of rehabilitation therapy. Here, we report a case of rehabilitation of a patient with neuralgic amyotrophy presenting with Collet-Sicard syndrome (9th, 10th, 11th, and 12th cranial nerve disorder)．A 44-year-old man developed severe pain from the left posterior neck to the occipital region, followed by sporadic onset of dysarthria, dysphagia, and difficulty in raising the left upper limb over several weeks. Nerve conduction studies showed marked bilateral differences in the amplitude of the compound muscle action potential recorded from the trapezius during accessory nerve stimulation. Needle electromyography showed abnormal resting potentials in the left trapezius and left side of the tongue and a decrease in the interference pattern during voluntary contraction. Based on the clinical course, neurological and laboratory findings, a diagnosis of neuralgic amyotrophy was made. Speech language hearing therapy was performed for dysarthria and dysphagia, and physical therapy was performed for difficulty in raising the left upper limb due to accessory nerve palsy. Rehabilitation along with recovery from inflammation-induced neuropathy allowed the patient to become independent in activities of daily living.

Course of Rehabilitation Treatment of Neuralgic Amyotrophy Presenting with Collet-Sicard Syndrome / The Japanese Journal of Rehabilitation Medicine

Typical cases of neuralgic amyotrophy present with sudden onset of excruciating pain in the shoulders and upper extremities, followed by marked muscle weakness and atrophy over a period of hours to days. Neuralgic amyotrophy is not confined to the brachial plexus, and difficulties in its diagnosis may delay the start of rehabilitation therapy. Here, we report a case of rehabilitation of a patient with neuralgic amyotrophy presenting with Collet-Sicard syndrome (9th, 10th, 11th, and 12th cranial nerve disorder)．A 44-year-old man developed severe pain from the left posterior neck to the occipital region, followed by sporadic onset of dysarthria, dysphagia, and difficulty in raising the left upper limb over several weeks. Nerve conduction studies showed marked bilateral differences in the amplitude of the compound muscle action potential recorded from the trapezius during accessory nerve stimulation. Needle electromyography showed abnormal resting potentials in the left trapezius and left side of the tongue and a decrease in the interference pattern during voluntary contraction. Based on the clinical course, neurological and laboratory findings, a diagnosis of neuralgic amyotrophy was made. Speech language hearing therapy was performed for dysarthria and dysphagia, and physical therapy was performed for difficulty in raising the left upper limb due to accessory nerve palsy. Rehabilitation along with recovery from inflammation-induced neuropathy allowed the patient to become independent in activities of daily living.

Bilateral Hirayama Disease: A Case Report

Hirayama disease is a rare restricted form of motor neuron disease. It commonly affects young males. Patients typically presentwith the insidious onset of unilateral weakness and atrophy of the hand muscles that often progresses to the forearm. In somecases, the syndrome is bilateral but often asymmetrical. Of note, the brachioradialis muscle is usually spared. The syndromeaffects C7–C8–T1 muscles with sparing of the C5–6 muscles. We report a case of a 25-year-old male who presented with 2years of history of progressive wasting and weakness of muscles of bilateral hands and forearms. Based on clinical features,electrodiagnostic studies and dynamic magnetic resonance imaging cervical spine diagnosis of Hirayama disease were made.The patient was treated conservatively with a cervical collar. Over a period of 8 months follow-up, no progression was seen.

Significance of Sufficient Neck Flexion During Magnetic Resonance Imaging in the Diagnosis of Hirayama Disease: Report of Two Cases

It is difficult to distinguish Hirayama disease (HD) from other mimicking disorders in adolescent patients with distal upper limb weakness. The prevailing theory of HD postulates that the lower cervical cord is susceptible to compression during neck flexion because of insufficient growth of the dura relative to the spinal column. Confirmation of a dynamic change in the dorsal epidural space on magnetic resonance imaging (MRI) during neck flexion is essential for diagnosing HD. However, neck flexion MRI has not been routinely performed in juvenile patients with distal upper limb weakness in the absence of suspected HD. We report two cases of HD that were initially confused with other diseases because of insufficient or absent cervical flexion during MRI. Full-flexion MRI showed typical findings of HD in both cases. Our cases suggest that dynamic cervical MRI in the fully flexed position is necessary for evaluating suspected HD.

Parsonage-Turner syndrome following chikungunya virus infection: A case report

Rationale: Parsonage-Turner syndrome is a rare syndrome of unknown etiology, affecting mainly the lower motor neurons of the brachial plexus. Chikungunya fever is a mosquito-borne viral disease characterized by acute fever and polyarthritis/polyarthralgia. Patient concerns: A 54-year-old Brazilian male patient who presented with a 2-day history of fever (temperature 38.8 °C), arthralgia, erythematous rash, diffuse osteomuscular pain and headache, which evolved into left shoulder pain associated with morning stiffness. Diagnosis: Parsonage-Turner syndrome and chikungunya fever. Interventions: Symptomatic treatment (a combination of short-acting dypirone (500 mg every 6 h) and slow-release opioids (tramadol 100 mg every 4 h) and physiotherapy/rehabilitation with improvement. Outcomes: The patient was improved and discharged, remaining with symptomatic treatment and physiotherapy/rehabilitation. Lessons: To the best of our knowledge, there were no reports of Parsonage-Turner syndrome following chikungunya virus infection. Awareness of the possibility of this rare association is important. The present case report highlights the importance of awareness of this association as a new cause of morbidity in patients with chikungunya virus infection.

Parsonage-Turner syndrome following chikungunya virus infection: A case report

Rationale: Parsonage-Turner syndrome is a rare syndrome of unknown etiology, affecting mainly the lower motor neurons of the brachial plexus. Chikungunya fever is a mosquito-borne viral disease characterized by acute fever and polyarthritis/polyarthralgia. Patient concerns: A 54-year-old Brazilian male patient who presented with a 2-day history of fever (temperature 38.8 °C), arthralgia, erythematous rash, diffuse osteomuscular pain and headache, which evolved into left shoulder pain associated with morning stiffness. Diagnosis: Parsonage-Turner syndrome and chikungunya fever. Interventions: Symptomatic treatment (a combination of short-acting dypirone (500 mg every 6 h) and slow-release opioids (tramadol 100 mg every 4 h) and physiotherapy/rehabilitation with improvement. Outcomes: The patient was improved and discharged, remaining with symptomatic treatment and physiotherapy/rehabilitation. Lessons: To the best of our knowledge, there were no reports of Parsonage-Turner syndrome following chikungunya virus infection. Awareness of the possibility of this rare association is important. The present case report highlights the importance of awareness of this association as a new cause of morbidity in patients with chikungunya virus infection.

Two Cases of Hirayama Disease in a Pediatric Clinic / 대한소아신경학회지

We report two pediatric cases with Hirayama disease—a 16-year-old boy with a left wrist drop and a 14-year-old-boy with weakness and muscle atrophy of right hand. Motor nerve conduction study revealed decreased motor nerve action potential amplitudes in the ulnar nerve and radial nerve of the affected hands. The former patient showed normal magnetic resonance imaging (MRI) of the cervical spine, but the latter showed mild, asymmetric thinning of the anterior spinal cord at levels C5 to C7. Following active rehabilitation and avoidance of neck flexion, no further progression of neurological findings was noticed. These clinical findings were typical of Hirayama disease. We show that timely and accurate diagnosis for Hirayama disease is possible with awareness of disease history, careful physical examination, and the use of neurophysiological studies and MRI studies.

The clinical manifestations and outcomes of neuralgic amyotrophy

Background & Objective: Although the clinical manifestations and outcomes of neuralgic amyotrophy have been previously described, some controversies remain. Thus, we evaluated clinical manifestations and outcomes of patients with neuralgic amyotrophy. Methods: We evaluated the clinical and electrodiagnostic data, and the outcomes, of 32 patients with neuralgic amyotrophy.Of the 32 patients, 26 were followed-up for one year after onset of the disease.Results:The initial symptoms were pain (50.0%), pain with weakness (21.9%), other sensory symptoms without weakness (6.3%), and painless weakness or atrophy (21.9%). The commonly involved nerves were the median (75.0%), radial (68.8%), suprascapular (50.0%), ulnar (50.0%), axillary (46.9%), and musculocutaneous (40.6%) nerves. The initial symptoms were not associated with nerve involvement. Of all patients, 59% recovered fully, 16% had residual mild weakness without functional disability, and 6% experienced persistent severe weakness and were unable to return to work. Some patients were not evaluated because they were lost to follow-up. Conclusions: Painless weakness as an initial symptom of neuralgic amyotrophy may be more common than previously noted. Of all patients, 75% enjoyed favorable outcomes by one year after disease onset. These results will be useful when planning treatment strategies and will deepen our understanding of prognosis of neuralgic amyotrophy.

Amiotrofia muscular espinal, terminalidade e desligamento do programa de equoterapia: reflexão sobre o rompimento de vínculos / Spinal muscular amyotrophy, termination and shutdown of the equinetherapy program: reflection on the breaking of bonds

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.

Síndrome de Davidenkow: Una neuropatía periférica rara / Davidenkow syndrome. A rare peripheral neuropathy

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.

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.

Rare case of Hirayama’s disease.

Hirayama‟s disease is a rare benign neurological disorder also known as monomelic amyotrophy, Sobue disease, Juvenile Muscular Atrophy of Distal Upper Extremity (JMADUE). It mainly affects young males in their second or third decades and is most commonly seen in Asian countries like Japan, Malaysia and India. In majority of the cases the cause of the disease is unknown. An 18 year male came with weakness in his right hand and forearm since 1 year. Examination revealed weakness and wasting of muscles of forearm and hand without lower limb involvement and normal deep tendon reflexes. MRI showed focal short segment hyperintense signal in the ventral and right lateral aspect of the cervical cord at C5-C6 level with the involved segment measuring 4x3mm in size. Based on clinical and radiological features a diagnosis of focal amyotrophy was made. Patient is given a cervical collar to prevent flexion at the neck and physiotherapy in the form of hand and forearm exercises were started. Regular follow up of the patient once every 2 months is being done. Hirayama‟s disease is a rare, benign, self-limiting neurological disorder. Early diagnosis and management by preventing cervical flexion with the help of a cervical collar has shown to halt the progression of the disease.

Monomelic Amyotrophy after Permethrin Poisoning.

Monomelic amyotrophy (MMA) known as Hirayama disease (HD). The first report appeared in 1959 when Hirayama described 12 patients [1] then, a large group of patients was found from Japan [2,3]. MMA from South India was also reported in 1984 [4]. The disease accounted for males over 80% of cases, especially between 15 and 25 years of age. The disorder has been recognized as, unilateral or bilateral asymmetric atrophy of hand and forearm with sparing of brachioradialis giving the characteristic appearance of oblique amyotrophy. Symmetrically bilateral disease has also been recognized. It is believed to be a cervical flexion myelopathy [5]. Pyrethroids are used as insecticides due to their high potency. These are highly toxic to a wide range of insects but have low toxicity to humans. Pyrethroids are known to cause neurotoxicity in humans like seizures, tremors, and dizziness. Motor neuron damage has been reported in acute toxicity due to ingestion of pyrethroids and organochlorines [6] and MND (Motor neuron disease) like features after chronic exposure has been reported [7]. Here a case of monomelic amyotrophy following massive ingestion of permethrin, amytriptyline and benzodiazepine tablets is reported.

Magnetic Resonance Neurography Findings in Idiopathic Neuralgic Amyotrophy / 계명의대학술지

Idiopathic neuralgic amyotrophy (INA) is known as Parsonage-Turner syndrome or idiopathic brachial plexitis and is characterized by sudden onset of severe limb pain, followed by weakness and atrophy of limb. There is no specific tests for the diagnosis of INA. The diagnosis of INA is mainly dependent on the clinical history and electrodiagnostic study. It is often confused with more common disorders such as acute cervical radiculopathy, rotator cuff tear, or acute calcific tendinitis. A few recent reports have revealed that magnetic resonance image (MRI) of brachial plexus and shoulder can be helpful in the diagnosis of INA. We report two cases of classic INA in which MRI enhanced specificity and confidence in the diagnosis. MRI of the brachial plexus and corresponding limb as well as cervical spine should be included in patients clinically suspected of INA.

Severe Spinal Injury in Hirayama Disease

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.

Monomelic Amyotrophy (Hirayama Disease) With Upper Motor Neuron Signs: A Case Report

Monomelic amyotrophy (MMA), also known as Hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities. This disorder rarely involves proximal upper extremities and presents minimal sensory symptoms with no upper motor neuron (UMN) signs. It is caused by anterior displacement of the posterior dural sac and compression of the cervical cord during neck flexion. An 18-year-old boy visited our clinic with a 5-year history of left upper extremity pain and slowly progressive weakness affecting the left shoulder. Atrophy was present in the left supraspinatus and infraspinatus. On neurological examination, positive UMN signs were evident in both upper and lower extremities. Electrodiagnostic study showed root lesion involving the fifth to seventh cervical segment of the cord with chronic and ongoing denervation in the fifth and sixth cervical segment innervated muscles. Cervical magnetic resonance imaging (MRI) showed asymmetric cord atrophy apparent in the left side and intramedullary high signal intensity along the fourth to sixth cervical vertebral levels. With neck flexion, cervical MRI revealed anterior displacement of posterior dural sac, which results in the cord compression of those segments. The mechanisms of myelopathy in our patient seem to be same as that of MMA. We report a MMA patient involving proximal limb with UMN signs in biomechanical concerns and discuss clinical importance of cervical MRI with neck flexion. The case highlights that clinical variation might cause misdiagnosis.

Spinal Cord Stimulation for Refractory Neuropathic Pain of Neuralgic Amyotrophy / 대한신경손상학회지

The aim of this paper was to report the effect of temporary and chronic spinal cord stimulation for refractory neuropathic pain in neuralgic amyotrophy (NA). A 35-year-old female presented with two-months history of a severe, relentless neuropathic pain of the left shoulder, forearm, palm, and fingers. The neuropathic pain was refractory to various medical treatments, including nonsteroidal anti-inflammatory drugs, opiates, epidural and stellate ganglion blocks, and typically unrelenting. The diagnosis of NA was made with the characteristic clinical history and magnetic resonance imaging. The patient underwent a temporary spinal cord stimulation to achieve an adequate pain relief because her pain was notoriously difficult to control and lasted longer than the average duration (about 4 weeks on average) of a painful phase of NA. Permanent stimulation was given with paddle lead. The neuropathic pain in her NA persisted and she continued using the spinal cord stimulation with 12 months after development of NA. The temporary spinal cord stimulation was effective in a patient with an extraordinary prolonged, acute painful phase of NA attack, and the subsequent chronic stimulation was also useful in achieving an adequate analgesia during the chronic phase of NA.

Neuralgic Amyotrophy Manifesting as Mimicking Posterior Interosseous Nerve Palsy

The upper trunk of the brachial plexus is the most common area affected by neuralgic amyotrophy (NA), and paresis of the shoulder girdle muscle is the most prevalent manifestation. Posterior interosseous nerve palsy is a rare presentation in patients with NA. It results in dropped finger on the affected side and may be misdiagnosed as entrapment syndrome or compressive neuropathy. We report an unusual case of NA manifested as PIN palsy and suggest that knowledge of clinical NA phenotypes is crucial for early diagnosis of peripheral nerve palsies.

Response of Hirayama disease to surgical intervention: case report.

Hirayama disease also known as monomelic amyotrophy, primarily involves distal upper limb extremities. It differs from the known types of motor neuron diseases because of its nonprogressive behavior and pathologic findings of focal ischemic changes in the anterior horn of the lower cervical cord. We present a young male with Hirayama disease who had a left upper extremity involvement which was progressive in nature. He didn’t respond with initial treatment of cervical collar. Consequently surgical intervention improves muscle weakness and decrease the neurological deficit.