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      Rol de los estudios neurofisiológicos en el diagnóstico diferencial entre la esclerosis lateral amiotrófica y la mielopatía espondilótica cervical Translated title: Role of neurophysiological studies in the differential diagnosis of amyotrophic lateral sclerosis and cervical spondylotic myelopathy

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          Abstract

          La esclerosis lateral amiotrófica (ELA) es una enfermedad degenerativa y progresiva que afecta a las motoneuronas del sistema nervioso central. Se caracteriza por un comienzo insidioso, habitualmente en individuos de mediana edad de sexo masculino. Los síntomas iniciales son: debilidad y atrofia muscular distal, fasciculaciones, hiperreflexia, espasticidad, signo de Babinski, y alteraciones progresivas de la deglución y la fonación; su pronóstico es reservado. El diagnóstico diferencial más importante es con la mielopatía espondilótica cervical; los estudios neurofisiológicos juegan un papel importante en esta diferenciación y las imágenes por resonancia magnética confirman este diagnóstico. Para demostrar la importancia de los estudios neurofisiológicos en la realización del diagnóstico diferencial entre la esclerosis lateral amiotrófica y la mielopatía espondilótica cervical, se presenta un caso clínico con diagnóstico presuntivo de esclerosis lateral amiotrófica, sin embargo los resultados de los estudios neurofisiológicos lo pusieron en duda, sugiriendo compresión de la médula espinal cervical, sin poder precisar la etiología exacta. Las imágenes de resonancia magnética confirmaron la existencia de una mielopatía espondilítica cervical severa. Se concluye que los estudios neurofisiológicos juegan un papel importante en la realización del diagnóstico diferencial entre las dos entidades y que los estudios de imagen facilitan la identificación de las lesiones espondilóticas.

          Translated abstract

          Amytrophic lateral sclerosis (ALS) is a degenerative and progressive disease. Motoneurone of central nervous system are affected. It has an insidious start, and is more frequently in men. Patients suffer of muscular weakness and atrophy, hyperreflexia, Babinski sign, fasciculations of the muscles, it prognosis is reserved. One of the most important differential diagnoses is the compression of the spinal cord by spondylosis, neurophysiologic tests are very important in this differentiation and magnetic resonance images confirm last condition. This paper show a case, who's had presumptive diagnostic of amytrophic lateral sclerosis, but neurophysiologic tests results doubt this diagnosis and suggested spinal compression at cervical region, magnetic resonance images (MRI) confirmed severe cervical spondilotic mielopathy. In conclusion neurophysiologic tests have an important roll to differentiate between amytrophic lateral sclerosis and cervical spondilotic myelopathy.

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          Most cited references28

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          Electrodiagnostic studies in amyotrophic lateral sclerosis and other motor neuron disorders.

          J R Daube (2000)
          The clinical electrodiagnostic medicine (EDX) consultant asked to assess patients with suspected amyotrophic lateral sclerosis (ALS) has a number of responsibilities. Among the most important is to provide a clinical assessment in conjunction with the EDX study. The seriousness of the diagnoses and their enormous personal and economic impact require a high-quality EDX study based on a thorough knowledge of and experience with motor neuron diseases (MNDs) and related disorders. Clinical evaluation will help determine which of the EDX tools available to the EDX consultant should be applied in individual patients. Although electromyography (EMG) and nerve conduction study are the most valuable, each of the following may be helpful in the assessment of selected patients based on their clinical findings: repetitive nerve stimulation, motor unit number estimate, single-fiber EMG, somatosensory evoked potential, autonomic function test, and polysomnography. The pertinent literature on these is reviewed in this monograph. The selection and application of these EDX tools depend on a thorough knowledge of the MNDs and related disorders.
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            Nerve conduction studies in amyotrophic lateral sclerosis.

            Nerve conduction studies (NCS) are an integral part of the evaluation of amyotrophic lateral sclerosis (ALS) patients and are useful in distinguishing ALS from disorders that mimic it. The question often arises whether in the presence of severe atrophy and reduction of the compound muscle action potential amplitude, abnormal conduction velocity (CV), distal latency (DL), or F-wave latency (F) exceeds what can be expected from ALS alone. To determine the limits of abnormality in classic ALS, we prospectively evaluated NCS data from 61 patients who met a strict clinical definition of ALS. We related CV, DL, and F to distal evoked amplitude (AMP) in peroneal (n = 63 observations), median (n = 50), and ulnar (n = 52) nerves. In nerves with reduced AMP, CV rarely fell to less than 80% of the lower limit of normal, and DL and F rarely exceeded 1.25 times the upper limit of normal. Utilizing the entire data set and regression analyses, 95% confidence limits for expected values for CV, F, and DL as a function of AMP were calculated. These limits thus derived suggest criteria for NCS abnormalities in ALS and may be useful in differentiating ALS from other illnesses.
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              Incidence and characteristics of spinal decompression surgery after the onset of symptoms of amyotrophic lateral sclerosis.

              The high incidence of spondylosis in patients at the mean age of onset (55.7 yr) of amyotrophic lateral sclerosis (ALS) can make recognition of ALS as a cause of weakness difficult. To assess the impact of this diagnostic dilemma on neurosurgical practice, we performed a retrospective analysis of a database of more than 1500 patients with motor neuron disease. Of 1131 patients with typical, sporadic ALS, 47 (4.2%) underwent decompressive spinal surgery after the onset of retrospectively recognized symptoms of ALS. Among 55 operations in 47 ALS patients, 86% yielded no improvement, 9% produced minor improvement, and only 5% provided significant benefit. Cervical decompression was performed in 56%, lumbar in 42%, and thoracic in 2%. Foot drop was a symptom prompting surgery in 11 patients, and in 10, this finding was subsequently attributed solely to ALS. No differences between ALS patients who underwent spinal decompression and other ALS patients were noted regarding age at symptom onset, severity of impairment at time of diagnosis, or rate of disease progression. Not surprisingly, patients who had spinal surgery tended to have a longer interval between retrospectively recognized symptom onset and diagnosis of ALS. A small but significant number of patients with unrecognized ALS undergo spinal surgery that in retrospect may be inappropriate. The possibility of ALS must be considered in the evaluation of patients with weakness even in the presence of radiographic evidence of spondylosis and nerve root or spinal cord impingement.
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                Author and article information

                Journal
                anco
                Acta Neurológica Colombiana
                Acta Neurol Colomb.
                Asociación Colombiana de Neurología (Bogotá, Distrito Capital, Colombia )
                0120-8748
                2422-4022
                January 2013
                : 29
                : 1
                : 53-59
                Affiliations
                [01] orgnameFinlay-Albarrán de la Habana orgdiv1facultad de medicina orgdiv2CCOI Frank País
                Article
                S0120-87482013000100007 S0120-8748(13)02900107
                2f11544c-55c4-4f49-a8b2-a6a1c3a56d54

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 28 March 2012
                : 09 August 2012
                : 25 September 2012
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 18, Pages: 7
                Product

                SciELO Colombia

                Categories
                Presentación de casos clínicos

                Neurofisiología,Enfermedades de la Médula Espinal,Esclerosis Lateral Amiotrófica,Neurophysiology,Spinal Cord Disease,Amyotrophic Lateral Sclerosis ALS

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