Abstract
The amyotrophic lateral sclerosis (ALS) is declared for muscular weakness, fasciculation and upper and lower motor neuron signs as: paresis, dysphagia, and dysarthria and breath shortness or paralysis of respiratory function, without sensitive compromise or sphincters alteration.
The neurophysiologic diagnosis requires as a minimum: sensitive and motor neuron-conductions in the median, cubital, radial and sural nerves (peroneal and posterior tibial nerves must be included in motor studies). Proximal and distal stimuli are necessary for motor action potential (PAM), also an electromyography. The electromyography shows increase of insertion activity, positive waves and fasciculation. The PAM has normal amplitude with greater duration. F wave and H reflex diminish its amplitude. The neurophysiologic finds change with the disease course and by them is necessary to maintain presents other diagnostic possibilities.
The medical history must be extent, exhaustive the neurological examination and reasonable the use of neurophysiologic studies, to support a clear diagnosis
References
Jau-Shin Lou. Amiotrophic lateral sclerosis. In CD Medlink. Neurobase 2006.ICD. cod 335.20
Brooks BR, Shodis KA, Lewis DH, Rawling JD, Sanjak M, Belden DS, Hakim H,DeTan Y, Gaffney JM. Natural history of amyotrophic lateral sclerosis. Quantification of symptoms, signs, strength, and function. Adv Neurol;1995;68
:63-84.
Preston DC, Shapiro BE. Amyotrophic lateral sclerosis and its variants. In: Preston DC, Shapiro BE, eds. Electromyography and neuromuscular disorders. Philadelphia: Elsevier Butterworth Heinemann, 2005: 423-437
Ramírez SF, Hernandez JF. Diagnóstico electrofisiológico de la enfermedad de motoneurona. Guia Neurolológica. Neuro-Electro- Diagnóstico. Asociación Colombiana de Neurología. 2005: 89-101.
Dumitru D, Amato AA. Introduction to Myopathies and Muscle Tissues’s Reaction to Injury. In: Dumitru D, Amato AA, Swartz MJ eds. Electrodiagnostic Medicine. 2nd Ed. Philadelphia: Hanley &Belfus, Inc., 2002:1229-1264.
Jilllapalli D, Shefner JM. Single motor unit variability with threshold stimulation in patients with amyotrophic lateral sclerosis and normal subjects. Muscle Nerve 2004; 30: 578 - 584.
Miller RG, Rosenberg JA, Gelinas DF et al. and the ALS Practice Parameters Task Force. Practice Parameter: The care of the patient with amyotrophic lateral sclerosis (an evidence-based review). Report of the Quality Standards Subcommittee of the AAN. Neurology 1999; 52:1311-1323.
Olney RK, Aminoff MJ, So YT. Clinical and electrodiagnostic features of X-linked recessive bulbospinal neuronopathy. Neurology 1991; 41:823-8.
Griggs RC, Askanas V, DiMauro S, et al. Inclusion body myositis and myopathies. Ann Neurol 1995;38:705-13.
Lambert EH. Electromyography in amyotrophic lateral sclerosis. In: Norris FH, Kurland LT, ed. Motor neuron diseases. New York: Grune & Stratton 1969;135-53.
Katz JS, Wolfe GI, Bryan WW, Jackson CE, Amato AA, Barohn RJ. Electrophysiologic findings in multifocal motor neuropathy. Neurology 1997; 48:700-7
Stalberg E, Trontelj JV. Singel Fiber Electro- myography findings in Diferent Disorders. Singel Fiber Electromyography Studies in Healthy and Diseased Muscle Raven Press. Second Edition. 1994: 130-219.
Hentati A, Bejaoui K, Pericak-Vance MA, et al. Linkage of recessive familial amyotrophic lateral sclerosis to chromosome 2q33-q35. Nat Genet 1994;7(3):425-8.
Preston DC, Shapiro BE. Atypical motor neuron disorders. In: Preston DC, Shapiro BE, eds. Electromyography and neuromuscular disorders. Philadelphia: Elsevier Butterworth Heinemann, 2005: 439-455.
Strong M, Rosenfeld J. Amyotrophic lateral sclerosis: A review of current concepts. Amyotroph Lateral Scler Other Motor Neuron Disord 2003;4:136-43.
World Federation of Neurology (WFN) Research Group on Neuromuscular Diseases Subcommittee on Motor Neuron Disease. Airlie House Guidelines. Therapeutic trials in amyotrophic lateral sclerosis. Airlie House “Therapeutic Trials in ALS” Workshop Contributors. J Neurol Sci 1995;129:1-10.
Hu MT, Ellis CM, Al Chalabi A, et al. Flail arm syndrome: a distinctive variant of amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 1998;65:950-1.
Khwaja S, Sripathi N, Ahmad BK, Lennon VA. Paraneoplastic motor neuron disease with type 1 Purkinje cell antibodies. Muscle Nerve 1998;21:943-5
Gordon PH, Rowland LP, Younger DS, et al. Lymphoproliferative disorders and motor neuron disease: an update. Neurology 1997;48:1671-8.
Ferracci F, Fassetta G, Butler MH, Floyd S, Solimena M, De Camilli P. A novel antineuronal antibody in a motor neuron syndrome associated with breast cancer. Neurology 1999;53:852-5.
Younger DS, Rowland LP, Latov N, et al. Lymphoma, motor neuron diseases, and amyotrophic lateral sclerosis. Ann Neurol 1991;29:78-86.
Ramírez SF. Gamopatías monoclonales. Act Neurol Colomb 2005: 28-32
Moulignier A, Moulonguet A, Pialoux G, Rozenbaum W. Reversible ALS-like disorder in HIV infection. Neurology 2001;57:995-1001.
Gessain A, Gout O. Chronic myelopathy associated with human-T lymphotrophic virus type 1 (HTLV-1). Ann Int Med 1992;117:933
Mitsumoto H, Sliman RJ, Schafer IA, et al. Motor neuron disease in adult hexosaminidase A deficiency in two families: evidence for multisystem degeneration. Ann Neurol 1985;17:378-85.
Parry GJ. AAEM case report #30: multifocal motor neuropathy. Muscle Nerve 1996;19:269-76.
Rowland LP, Shneider NA. Amyotrophic lateral sclerosis. New Engl J Med 2001;344:1688-700.
Brais B, Bouchard J, Xie YG, et al. Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy. Nat Genet 1998;18:164-7.
Brais B, Rouleau GA, Bouchard JP, et al. Oculopharyngeal muscular dystrophy. Semin Neurol 1999;19:59-66.
Gil IW. Treatment of MyastheniaGravis. En CD: Americam Acadmy of Neurology. 2005.
Steven V. Parenoplastic Myasthenia and related disorders. En CD: Americam Acadmy of Neurology. 2005.
Syringomyelia associated with intradural arachnoid cysts. J Neurosurg Spine 2006 Aug; 5(2):111-6.
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