Siringomielia: revisión a propósito de un caso / Syringomyelia: review, case and report

La siringomielia supone un desafío diagnóstico, ya que es una entidad poco reconocida si no se tiene conciencia de su existencia. Al ser un cuadro progresivo, cuya clínica puede presentarse de forma larvada y ser coincidente con otras patologías neurológicas tales como la esclerosis múltiple, su detección suele realizarse en etapas tardías sobre todo en población adulta y más aún cuando se presenta de forma adquirida. Por lo que el estudio imagenológico con Resonancia Magnética adquiere especial relevancia, permitiendo identificar y clasificar la enfermedad, lo que brindará la base para decidir terapía.

Syringomyelia is a diagnostic challenge, since it is a poorly recognized disease, especially if its existence remains unknown. Being a progressive disease, whose clinic can present in a latent way and be coincident with other neurological pathologies such as multiple sclerosis, its detection is usually conducted in late stages, especially in the adult population and even more when it presents in an acquired way. Therefore, the imaging study with Magnetic Resonance acquires special relevance, allowing to be identified and classified, which will provide the basis for deciding on therapy

A case report of skeletal fluorosis leading to cervical compressive myelopathy and a review of literature

Fluorosis is a public health problem that is caused by excess intake of fluoride through ground/deep bore water. It gets deposited in the bones, teeth, and soft tissues. Fluoride in the human body acts as a “double-edged sword.” Fluoride is beneficial in small amounts but toxic in large amounts. People who have consumed 10–20 mg of fluoride per day for more than 10–20 years may develop crippling skeletal fluorosis. Stiffness and pain in the major joints, including the neck, back, hips, and knees, reduce mobility. The bone structure may change and ligaments may calcify in extreme cases, resulting in muscular weakness and pain. Here, we have reported a case of chronic quadriparesis due to compressive cervical myelopathy. After extensive workup, diagnosis of skeletal fluorosis was made.

Hypokalemic Rhabdomyolysis — A Rare Presentation

Hypokalemia leading to Rhabdomyolysis is a potentially fatal disorder if not identified and treated early. In this case report we present a patient who had one week history of asymmetric painful Quadriparesis with neck drop and preserved reflexes. Evaluation revealed Hypokalemia with raised creatine.

Analysis of muscle activation in children and adolescents with severe cerebral palsy / Análise da ativação muscular em crianças e adolescentes com paralisia cerebral grave

Abstract Introduction: Children and adolescents with spastic quadriparesis have a worse selective motor control, and studies with this population are still very scarce. The same is true with scientific evidence of one of the methods most used as a physiotherapeutic treatment in this population, the Bobath Concept. Objective: To evaluate spine erector muscles activation, gluteus medius and gluteus maximus, through the handling of the Bobath Concept and the sustained kneeling posture in subjects with a diagnosis of severe cerebral palsy; and to compare muscle activation with a reference group, in order to increase the reliability of this study. Methods: A cross-sectional study was carried out with 38 children and adolescents with cerebral palsy, classified by GMFCS at levels IV and V, and 20 healthy participants, aged between 3 and 18 years. They were submitted to the handling of the Bobath Concept and to the sustained kneeling posture, with muscle activation obtained by electromyography. Results: We observed significant muscle activation during handling in side-sitting, with weight transfer and without the help of another therapist, and in the sustained kneeling posture, for the erector of the spine and gluteus medius. Conclusion: The evidence from this study suggests that both the handling in side-sitting and the sustained kneeling posture cause significant muscle activation in the erector of the spine and gluteus medius for severe quadriparesis subjects, GMFCS IV and V, which can contribute to the improvement of postural control and decision-making in physical therapy practice.

Resumo Introdução: Crianças e adolescentes com quadriparesia espástica apresentam pior controle motor seletivo e estudos com essa população ainda são muito escassos. O mesmo ocorre com as evidências científicas de um dos métodos mais utilizados como tratamento fisioterapêutico nessa população, o Conceito Bobath. Objetivo: Avaliar a ativação dos músculos eretores da coluna, glúteo médio e glúteo máximo por meio do manuseio do Conceito Bobath e da postura ajoelhada sustentada, em indivíduos com diagnóstico de paralisia cerebral grave; e comparar a ativação muscular com um grupo de referência, a fim de aumentar a confiabilidade deste estudo. Métodos: Realizou-se um estudo transversal com 38 crianças e adolescentes com paralisia cerebral, classificados pelo GMFCS nos níveis IV e V, e 20 participantes saudáveis com idade entre 3 e 18 anos. Eles foram submetidos ao manuseio do Conceito Bobath e à postura ajoelhada sustentada, com ativação muscular obtida por eletromiografia. Resultados: Observou-se ativação muscular significativa durante o manuseio na posição sentada de lado, com transferência de peso e sem auxílio de outro terapeuta, e na postura ajoelhada sustentada para o eretor da coluna e glúteo médio. Conclusão: As evidências deste estudo sugerem que tanto o manuseio na posição sentada de lado quanto a sustentação da postura ajoelhada causam significativa ativação muscular no eretor da coluna e glúteo médio para indivíduos com quadriparesia grave, GMFCS IV e V, o que pode contribuir para a melhora do controle postural e tomada de decisão na prática fisioterapêutica.

Bilateral Accessory C1 and C2 Facet Joints; Clinical, Neuroradiological and Peroperative Findings in an Adult with Quadriparesis

Objectives Accessory C1 and C2 facet joints are very rare. Only few cases were reported in the literature.We report a case of bilateral accessory facets in an adult with special attention to clinical, neuroradiological, as well as peroperative findings. Case report A 37-year-old male presented with progressive quadriparesis. Radiology revealed bilateral posterior accessory C1 and C2 facet joints compressing the spinal cord with craniovertebral junction (CVJ) instability. Both accessory C1 and C2 facets with the posterior arch of the C1 were removed. Lateral mass screws and plates fixation at the C1 and C2 level, as well as fusion, were performed. Postoperatively, the patient recovered well. Conclusion In accessory C1 and C2 facet joints, when symptomatic, neuroradiological findings can guide to the proper diagnosis, to pathological understanding, and, ultimately, to management strategy.

Study of incidence and prevalence of hypokalemic periodic paralysis

Background: Hypokalemic periodic paralysis (HPP) is a rare autosomal dominant channelopathy characterised by muscle weakness or paralysis when there is a fall of potassium level in the blood. In individuals with mutation, attack begins during adolescents and most commonly occurs after sleep on awakening, rest after strenuous exercise, high carbohydrate diet and meal with high sodium content. This study was conducted to analyse the age of incidence and prevalence of HPP with various clinical presentations, diagnosis and its effective treatment.Methods: A retrospective analysis of 50 patients of Hypokalemic periodic paralysis was analysed in tertiary care centre “Rajendra institute of medical science” Ranchi, Jharkhand, India, with reference to its clinical presentation, age of incidence and prevalence with laboratory parameters and treatment outcomes.Results: Incidence of attack is more common in men between 26-35 years of age. Sudden onset of flaccid quadriparesis was the most common presentation with history of high carbohydrate diet on the background of strenuous work during summer season. Around 60% had similar history of attacks in the past and most of the patients had serum potassium levels between 2.1-3.0mEq/L. Electrocardiography (ECG) abnormalities associated with hypokalemia had been observed in 90% of patients, and also significant clinical, biochemical changes have been observed as well.Conclusions: Early diagnosis not only helps in definitive management with potassium replacement, but also prevents patient going for life threatening respiratory failure.  Patients recover completely without any clinical sequelae. Therefore, it is imperative for physicians, particularly those working in acute care settings, to be aware of this condition. Further management depends on the cause, frequency of attacks, severity of symptoms and the duration of the illness.

Rehabilitation for Motor Neuron Syndrome and Sjögren's Syndrome: A Case Report / 中国康复理论与实践

@#Objective To explore the rehabilitation and outcome of motor neuron syndrome and Sjögren's syndrome. Methods A patient with motor neuron syndrome and Sjögren's syndrome was reviewed. Results The patient expressed progressive quadriparesis and slurred speech. Electromyogramphy displayed upper and lower motor neuron damage. Sonography showed diffuse lesions in parotid gland and submandibular gland. Laboratory tests displayed markedly elevated levels of SSA and Ro52 antibodies. After a course of intravenous immunoglobulin (IVIG) and four weeks of comprehensive rehabilitation, the patient improved in terms of speech, muscle strength, balance, posture control and activities of daily living. She failed to achieve further improvement after another IVIG and glucocorticoid two months later, but fortunately her symptoms did not deteriorate. Conclusion The patient with motor neuron syndrome and Sjögren's syndrome might benefit from IVIG and appropriate rehabilitation.

The pathogenic mechanisms of motor weakness following aneurysmal subarachnoid hemorrhage: A review

Motor weakness is one of the neurological complication that can occur after aneurysmal subarachnoid hemorrhage (SAH); incidence of motor weakness of 14~29% has been reported. Detailed information on the pathogenic mechanism of motor weakness is essential for brain rehabilitation because it enables estimation of the severity of injury, establishment of scientific rehabilitative strategies, and prediction of motor outcomes by clinicians. However, the exact pathogenic mechanisms of motor weakness following aneurysmal SAH have not been clearly elucidated. In this article, 14 previous studies on pathogenic mechanisms in patients with aneurysmal SAH were reviewed according to the location of the lesion (cerebral cortex, brainstem, spinal cord, and peripheral nerve). The following pathogenic mechanisms have been suggested: vasospasm, cerebral ischemia, hydrocephalus, compression of cerebral cortex, neural injury, spinal cord infarction, and radiculo-neuropathy. Considering the high incidence of aneurysmal SAH and motor weakness following aneurysmal SAH, we believe that the pathogenic mechanisms of motor weakness have been relatively understudied. More effort should be taken to investigate this important topic.

Skeletal fluorosis with progressive quadriparesis U.M.N. type, non compressive myelopathy, chronic kidney disease and secondary hyperparathyroidism and hypothyroidism – A case report.

Endemic fluorosis leads to dental fluorosis, skeletal fluorosis and visceral fluorosis. This case described about the crippling skeletal fluorosis with neurological manifestations in the form of myelopathy, due to exposure to high fluoride level in water for many years. The complications of fluoride deposition in bones resulted in generalised sclerosis, osteophytosis, with narrowing of inter vertebral disc spaces in vertebral column. In this case the skeletal fluorosis leads to severe spastic quadriparesis. This case had features of visceral fluorosis; the affected organs include thyroid gland with hypothyroidism, parathyroid gland with secondary hyperparathyroidism, pancreas with intra pancreatic calcification and the kidneys with chronic kidney disease.

Acute Cervical Subdural Hematoma with Quadriparesis after Cervical Transforaminal Epidural Block

Cervical epidural steroid injection is frequently used in the conservative management of neck pain and cervical radiculopathy. Epidural cervical transforaminal injections are usually well-tolerated with mild side effects such as transient decreased sensory and motor function, or headache due to dural puncture. Although there are a few case reports about adverse effects of cervical epidural injection in the literature, it can cause severe complications such as large hematoma, infarction by spinal vascular injury. Subdural hematoma has been occurred much less common rather than epidural hematoma in the spinal cord. We report a rare catastrophic case of cervical spinal subdural hematoma with quadriparesis after cervical transforaminal epidural block.

A rare presentation of dengue fever: acute motor quadriparesis due to hypokalemia.

Hypokalemia can lead to acute onset pure motor flaccid quadriparesis. Although there are many causes of hypokalemia but dengue is a one rare cause of them which can cause hypokalemia. Simply correction of potassium improve the weakness dramatically. We reported a case who presented to us with acute motor quadriparesis, finally diagnosed hypokalemia due to dengue

Role of Decompression in Late Presentation of Cervical Spinal Cord Disorders

STUDY DESIGN: Prospective study conducted at Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, India. PURPOSE: To show the efficacy of decompression in the late presentation of cervical spinal cord disorders. OVERVIEW OF LITERATURE: Studies by various authors have shown that early spinal decompression results in better neurological outcomes. METHODS: From January 2003 to January 2005, 11 of the 41 patients with cervical spinal cord compression, meeting the inclusion criteria, underwent anterior decompression; interbody graft placement and stabilization by anterior cervical locking plate. The neurologic and functional outcomes were recorded. RESULTS: Five patients had spinal cord injury and 6 patients had compressive cervical myelopathy. Complications included 1 death and 1 plate loosening. No patient lost their preoperative neurological status. One patient had no improvement, 2 patients showed full recovery. The mean follow-up is 28.3 month. At the of rehabilitation, 6 were able to walk without support), 2 could walk with support, and 1 needed a wheelchair. The average American Spinal Injury Association motor score on admission to the hospital, 32.8 (standard deviation [SD], 30.5); admission to rehabilitation, 38.6 (SD, 32.4); discharge from rehabilitation, 46.2 (SD, 33.7). The most recent follow-up was 64.0 (SD, 35.3). CONCLUSIONS: The anterior approach for cervical decompression allows for adequate decompression. This decompression is the best chance offered in even late reported cases, including posttraumatic cases where there is no evidence of cord transactions. The use of anterior cervical plates reduces the chances of graft loosening, extruding, or collapsing.

Myelopathy and Quadriparesis due to Spinal Cord Compression of C1 Laminar Osteochondroma

The aim of this paper is to show that osteochondromas of the cervical vertebrae can cause myelopathy and neck pain.The reported etiology, diagnosis, treatment and differential diagnosis were reviewed. Osteochondromas may present as a solitary lesion with no genetic component or as multiple lesions as a part of a genetic disorder known as hereditary multiple exostosis. Osteochondromas of the spine are rarely encountered in clinical practice. These lesions are reported more commonly with neural compression in cases associated with hereditary multiple exostosis. The authors describe a unusual clinical manifestation of a solitary osteochondroma located in the right posterior arch of the atlas. Complete removal of the tumor was performed resulting in the relief of neck pain and spastic quadriparesis. Although unusual, osteochondromas of the cervical spine must be considered in patients with persistent neck pain and progressive symptoms of myelopathy. Computed tomography and magnetic resonance imaging in conjunction with plain radiograms is the neuroradiological modality of choice. The diagnosis and surgical excision of these tumors are important because they can cause spinal stenosis resulting in neural tissue compression and myelopathy.

Langerhans Cell Histiocytosis with Extensive Spinal and Thyroid Gland Involvement Presenting with Quadriparesis: An Unusual Case in an Adult Patient

Langerhans cell histiocytosis of the spine frequently presents as a solitary lesion and rarely results in neurological deficit. Involvement of thyroid tissue is also rare, even in multifocal disease presentations. We present an unusual variant of Langerhans cell histiocytosis in a 37-year-old man presenting with profound quadriparesis and incidental thyroid involvement. MR imaging and skeletal radiographs revealed widespread involvement of the spine. The patient underwent surgical resection of the tumour and stabilization of the spine. Diagnosis was confirmed by histological and immunohistochemistry analysis. Langerhans cell histiocytosis should be included in the differential diagnosis of radiolucent lesions of the spine in adults.

Cervical Spinal Metastasis of Merkel Cell Carcinoma

Merkel cell carcinoma is a skin disease that rarely involves the spine. The common presentation of spinal involvement is quite confusing and may be easily misdiagnosed. However, once it spreads, the disease progresses rapidly, with an extremely poor outcome. We report on the case of a 30-year-old male with cervical spinal metastasis of Merkel cell carcinoma causing quadriparesis.

A Case of Cervical Epidural Abscess Presenting Rapidly Progressing Quadriplegia without any other Symptom or Sign of CEA

Cervical epidural abscess (CEA) is a very uncommon disease with diverse clinical presentations. Clinically, it is difficult to diagnose this disease, especially in early stage. We report an atypical case of CEA showing only progressive quadriparesis without any other symptoms or signs of CEA. From this experience, CEA must be considered when progressive quadriparesis without any specific cause is developed in immuno-compromised patients and diabetic patients with polyneuropathies.

A Case of Cervical Epidural Abscess Presenting Rapidly Progressing Quadriplegia without any other Symptom or Sign of CEA

Cervical epidural abscess (CEA) is a very uncommon disease with diverse clinical presentations. Clinically, it is difficult to diagnose this disease, especially in early stage. We report an atypical case of CEA showing only progressive quadriparesis without any other symptoms or signs of CEA. From this experience, CEA must be considered when progressive quadriparesis without any specific cause is developed in immuno-compromised patients and diabetic patients with polyneuropathies.

A Case of Neurologic Symptom in Association with Intoxication after Ingestion of Codfish Intestine

No abstract available.

Fatal gastro-intestinal bleeding: A complication following spinal injury.

On 30.08.2003, a 20-year-old boy met with a vehicular accident and sustained spinal injury and left upper limb fracture. He was immediately hospitalized and seemed to recover well after treatment for a period of 15 days, when just before discharge he suddenly had hematemesis and bleeding per rectum and succumbed within another 24 hours. The case is discussed in detail.

Transient Quadriparesis due to Dysgenesis of the Posterior Arch of the Atlas: Case Report

Acase of 14-year-old student is presented with the complaint of recurrent attack of transient quadriparesis during hyperextension of the neck. On 3-dimensional spinal CT and MRI, the authors confirmed intrusion of posterior tubercle of the atlas with increased signal on T1- and T2- weighted image was found. The clinical manifestations were improved without having cervical instability after a posterior laminectomy of the atlas.