Another Perspective on Fasciculations: When is it not Caused by the Classic form of Amyotrophic Lateral Sclerosis or Progressive Spinal Atrophy?

Marco Antonio Araujo Leite, Marco Orsini, Marcos R G de Freitas, João Santos Pereira, Fábio Henrique Porto Gobbi, Victor Hugo Bastos, Dionis de Castro Machado, Sergio Machado, Oscar Arrias-Carrion, Jano Alves de Souza, Acary Bulle Oliveira, Marco Antonio Araujo Leite, Marco Orsini, Marcos R G de Freitas, João Santos Pereira, Fábio Henrique Porto Gobbi, Victor Hugo Bastos, Dionis de Castro Machado, Sergio Machado, Oscar Arrias-Carrion, Jano Alves de Souza, Acary Bulle Oliveira

Abstract

Fasciculations are visible, fine and fast, sometimes vermicular contractions of fine muscle fibers that occur spontaneously and intermittently. The aim of this article is to discuss the main causes for fasciculations and their pathophysiology in different sites of the central/peripheral injury and in particular to disprove that the presence of this finding in the neurological examination is indicative of amyotrophic lateral sclerosis. Undoubtedly, most fasciculations have a distal origin in the motor nerve both in normal subjects and in patients with motor neuron disease. Most of them spread to other dendritic spines often producing an antidromic impulse in the main axon. The clinical and neurophysiological diagnosis must be thorough. It may often take long to record fasciculations with electroneuromyography. In other cases, temporal monitoring is necessary before the diagnosis. The treatment, which may be adequate in some cases, is not always necessary.

Keywords: electromyography treatment; fasciculations; neurological diseases.

Conflict of interest statement

Conflict of interests: the authors declare no potential conflict of interests.

References

    1. Buainain RP, Souza Moura L, Oliveira ASB.Fasciculations. Rev Neurocienc 2000;8:31-4
    1. Masland RL, Wigiton RS.Nerve activity accompanying fasciculations produced by prostigmin. J Neurophysiol 1940;3:269-75
    1. Mills KR.Characteristics of fasciculationss in amyotrophic lateral sclerosis and the benign fasciculations syndrome. Brain 2010;133:3458-69
    1. Kleine BU, Stegeman DF, Schelhaas HJ, Zwarts MJ.Firing pattern of fasciculationss in ALS: evidence for axonal and neuronal origin. Neurology 2008;70:353-9
    1. Orsini M, Sztajnbok FR, Oliveira AB, et al. Benign fasciculationss and corticosteroid use: possible association? An update. Neurol Int 2011;3:e11.
    1. Masland RL, Wigiton RS.Nerve activity accompanying fasciculations produced by prostigmin. J Neurophysiol 1940;3:269-75
    1. Muravchick S, Burkett L, Gold MI.Succinycholine-induced fasciculationss and intragastric pressure during induction of anesthesia. Anesthesiology 1981;55: 180-3
    1. Reed DM, Kurland LT.Muscle fasciculations in a healthy population. Arch Neurol 1963;9:363-7
    1. Simon NG, Kiernan MC.Fasciculations anxiety syndrome in clinicians. J Neurol 2013;260:1743-7
    1. Forcelini CM, Rotta FT, Posenato N.Benign fasciculationss responsive to gabapentin. Arq Neuropsiquiatr 2007;65: 1015-7
    1. Rigby J, Holton A, Partridge J, Satchithananda D.Dyspnoea fasciculations syndrome: the clue is in the title. BMJ Case Rep 2012:2012
    1. Singh V, Gibson J, McLean B, et al. Fasciculationss and cramps: how benign? Report of four cases progressing to ALS. J Neurol 2011;258:573-8
    1. Blexrud MD, Windebank AJ, Daube JR.Long term follow-up of 121 patients with benign fasciculationss. Ann Neurol 1993;34:622-5
    1. Okuda B, Kodama N, Tachibana H, Sugita M.Motor neuron disease following generalized fasciculationss and cramps. J Neurol Sci 1997;150:129-31
    1. Fermont J, Arts IM, Overeem S, et al. Prevalence and distribution of fasciculationss in healthy adults: effect of age, caffeine consumption and exercise. Amyotroph Lateral Scler 2010;11:181-6
    1. Arias M, Arias-Rivas S, Blanco-Arias P, et al. [SCA from the Costa da Morte: A new SCA. Description of the phenotype]. Neurologia 2008;23:628-9 [Article in Spanish]
    1. Tan EK, Tong J, Pavanni R, et al. Genetic analysis of SCA 2 and 3 repeat expansions in essential tremor and atypical Parkinsonism. Mov Disord 2007;22:1971-4
    1. França MC, Jr, D’Abreu A, Nucci A, Lopes-Cendes I.Muscle excitability abnormalities in Machado-Joseph disease. Arch Neurol 2008;65:525-9
    1. Kanai K, Kuwabara S.Motor nerve hyper-excitability and muscle cramps in Machado-Joseph disease. Arch Neurol 2009;66:138-141
    1. Kobayashi H, Abe K, Matsuura T, et al. Expansion of Intronic GGCCTG hexanucleotide repeat in NOP56 causes SCA36, a type of spinocerebellar ataxia accompanied by motor neuron involvement. Am J Hum Genet 2011;89:121-30
    1. Ikeda Y, Ohta Y, Kobayashi H, et al. Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan). Neurology 2012;79:333-41
    1. García-Murias M, Beatriz Quintáns B, Arias M, et al. Costa da Morte ataxia is spinocerebellar ataxia 36: clinical and genetic characterization. Brain 2012:135; 1423-35
    1. Shy GM, Drager GA.A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 1960;2:511-27
    1. Montagna P, Martinelli P, Rizzuto N, et al. Amyotrophy in Shy-Drager syndrome. Acta Neurol Belg 1983;83:142-57
    1. Luo W, Ouyang Z, Guo Y, et al. Spinal muscular atrophy combined with sporadic olivopontocerebellar atrophy. Clin Neurol Neurosurg 2008;110:855-8
    1. Blunt SB, Khalil NM, Perkin GD.Facial myokymia in multiple system atrophy. Mov Disord 1997;12:235-8
    1. Machida Y, Tsuchiya K, Anno M, et al. Sporadic amyotrophic lateral sclerosis with multiple system degeneration: a report of an autopsy case without respirator administration. Acta Neuropathol 1999;98:512-5
    1. Le Forestier N, Lacomblez L, Meininger V.Syndromes parkinsoniens et scléroselatéraleamyotrophique: Tauopathies, TDP-43 et mutations SOD. Rev Neurol (Paris) 2009;165:15-30
    1. Brooks BR, Miller RG, Swash M, et al. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;1:293-9
    1. Hughes AJ, Daniel SE, Kilford L, Lees AJ.Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinicopathological study of 100 cases. J NeurolNeurosurg Psychiatry 1992;55:181-4
    1. van Es MA, Schelhaas HJ, van Vught PW, et al. Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Ann Neurol 2011;70:964-73
    1. Manno C, Lipari A, Bono V, et al. Sporadic Parkinson disease and Amyotrophic Lateral Sclerosis complex (Brait-Fahn-Schwartz Disease). J Neurol Sci 2013;326:104-6
    1. Park HK, Lim YM, Kim JS, et al. Nigrostriatal dysfunction in patients with amyotrophic lateral sclerosis and parkinsonism J Neurol Sci 2011;301:12-3
    1. Brait K, Fahn S, Schwarz GA.Sporadic and familial parkinsonismand motor neuron disease. Neurology 1973;23:990-002
    1. Annesi G, Savettieri G, Pugliese P, et al. DJ-1 mutations and parkinsonism-dementia-amyotrophic lateral sclerosis complex. Ann Neurol 2005;58:803-7
    1. Deymeer F, Akca S, Kocaman G, et al. Fasciculationss, autonomicsymptoms and limbic encephalitis: a thymoma-associated Morvan’s-like syndrome. Eur Neurol 2005;54:235-7
    1. Irani SR, Pettingill P, Kleopa KA, et al. Morvansyndrome: clinical and serological observations in 29 cases. Ann Neurol. 2012,72:241-55
    1. Krivickas LS.Amyotrophic lateral sclerosis and other motor neuron diseases. Phys Med Rehabil Clin N Am 2003;14:327-45
    1. Higashihara M, Sonoo M. [Electrodiagnosis of ALS]. Brain Nerve 2007;59:1031-41 [Article in Japanese]
    1. de Carvalho M, Swash M.Fasciculations potentials and earliest changes in motor unit physiology in ALS. J Neurol Neurosurg Psychiatry 2013;84:963-8
    1. Hirota N, Eisen A, Weber M.Complex fasciculationss and their origin in amyotrophic lateral sclerosis and Kennedy’s disease. Muscle Nerve 2000;23:1872-5
    1. Kohara N.Abnormal hyperexcitability in ALS. Rinsho Shinkeigaku 1999;39:61-4 [Article in Japanese].
    1. Godoy JM, Oliveira MAR, Moraes Neto JBM, et al. Esclerose lateral amiotrófica esporádica. Critérios diagnósticos. Arq Neuropsiquiatr 1993;51:236-42
    1. Finelli PF.Drug-induced Creutzfeldt-Jakob like syndrome. J Psychiatr Neurosci 1992;17:103-5
    1. Alonso-Navarro H, Jiménez-Jiménez FJ.Reversible tremor, myoclonus, and fasciculationss associated with topiramate use for migraine. Clin Neuropharmacol 2006;29:157-9
    1. Adams CR, Ziegler DK, Lin JT.Mercury intoxication simulating amyotrophic lateral sclerosis. JAMA 1983;250:642-3
    1. Hara K, Watanabe O, Shibano K, Ishiguro H. [A case of anti VGKC-complex antibody associated disorder presenting with severe pain and fasciculations predominant in unilateral upper extremity]. Rinsho Shinkeigaku 2012; 52(9):677-80 [Article in Japanese]
    1. Kizilay F, Ekmekci B, Gungor H, et al. Flunarizine-induced fasciculations-myokymia. J Clin Neuromuscul Dis 2011; 12:246-7
    1. Chaouch N, Mejid M, Zarrouk M, et al. [Isoniazid-induced myopathy]. Rev Pneumol Clin 2011;67:354-8 [Article in French]
    1. Reddy PL, Grewal RP.Resolution of muscle cramps and fasciculations with treatment of sleep apnea. J Clin Neuromuscul Dis 2009;11:66-7

Source: PubMed

Sponsorit ja yhteistyökumppanit

Sairaudet

Huumeiden interventiot

3
Tilaa