medigraphic.com
Instituto Nacional de Neurología y Neurocirugía

2008, Number 3

Next >>

Arch Neurocien 2008; 13 (3)

Neurophysiologic studies for the identification of alterations in superior and inferior motor neurons in patients with amyotrophic lateral sclerosis

Martínez HHR, Fernández González-Aragón MC, Burgos CJ

Language: Spanish
References: 29
Page: 145-154
PDF size: 145.39 Kb.


ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that causes death in a small amount of time. The main pathophysiology feature of this entity is the affection of the inferior and superior motor neurons in the same period of time. The diagnosis of ALS is founded in clinical criteria and sustained with neurophysiologic studies, such as electromyography, which identifies the loss of motor units due to the affection of the inferior motor neuron; recently the transcraneal magnetic stimulation tecnique allows the measurement of the integrity of cortical motor neuron pathways. Objectives: to associate the evoked motor potentials with motor conduction studies in ALS patients. Methodology: cross-sectional study in ALS patients with a probable or definitive diagnosis using the El Escorial criteria. We performed TMS and neuroconduction studies measuring the evoked motor potentials and compound action muscular potentials. We applied a functional scale for ALS. The analysis was performed for descriptive and correlation statistics using SPSS version 15.0. Results: nine cases were studied. In all of them we observed at least one alteration in the TMS tests. The more frequent finding was a prolongation of the central conduction time and of the minimal central latencies. Four of the cases did not present a recognizable response or a partial evoked motor response. The TMS alterations did not correlate with disease duration or clinical scales. Conclusions: we confirmed the utility of TMS for the detection of the cortico-spinal pathway alterations. Our results did not found a correlation between the evoked motor potentials and disease´s progression. It will be necessary to continue our investigations , expanding the number of cases and try to correlate this diagnostic tool with a biological marker.


REFERENCES

  1. Rowland LP, Shneider NA. Amyotrophic lateral sclerosis. N Engl J Med 2001. 344; 22: 1688-700.

  2. Brooks BR, Subcommitte on Motor Neuron Disease / Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases. El Escorial, World Federation of Neurology Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis. J Neurol Sci 1994;24(suppl.): 96-107.

  3. Miscio G, Pisano F, Mora G, Mazzini L. Motor neuron disease: usefulness of transcranial magnetic stimulation in improving the diagnosis. Clin Neurophysiol 1999; 110: 975-81.

  4. Komissarow L, Rollnik JD, Bogdanova D, Krampfl K, Khabirov FA, Kossev A, et al. Triple stimulation technique (TST) in amyotrophic lateral sclerosis. Clin Neurophys 2004;115:356-60.

  5. Kaufmann P, Pullman SL, Shungu DC, Chan S, Hays AP, Del Bene ML, et al. Objective test for upper motor neuron involvement in amyotrophic lateral sclerosis (ALS). Neurology 2004; 62: 1753-7.

  6. Boll MC, Alcaraz-Zubledía M, Montes S, Murillo-Bonilla L, Ríos C. Raised nitrated concentration and low SOD activity in the CSF of sporadic ALS patients. Neurochem Research 2003; 28:5: 699-703.

  7. Jacobson J, Jonsson PA, Andersen PM, Forsgren L, Marklund SL. Superoxide dismutase in CSF from amyotrophic lateral sclerosis patients with and without CuZn- superoxide dismutase mutations. Brain 2001; 124: 1461-66.

  8. Ihara Y, Nobukuni K, Takata H, Hayabara T. Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Znsuperoxide dismutase mutation. Neurological Research 2005; 27: 105-8.

  9. Devos D, Moreau C, Lassalle P, Perez T, De Seze J, Brunand-Danel V, et al. Low levels of the vascular endothelial growth factor in CSF from early ALS patients. Neurology 2004; 62: 2127-9.

  10. Cronin S, Greenway MJ, Ennis S, Kieran D, Green A, Prehn JHM, et al. Elevated serum angiogenin levels in ALS. Neurology 2006; 67: 1833-36.

  11. Pasinetti GM, Ungar LH, Lange DJ, Yemul S, Deng H, Yuan X, et al. Identification of potential CSF biomarkers in ALS. Neurology 2006; 66: 1218-22.

  12. Brooks BR, Miller RG, Swash M, Munsat TL. World Federation of Neurology Research group on motor neuron disease. Amyotroph Lateral Scler Motor Neuron Disord 2000;1(5):293-9.

  13. Beghi E, Balzarini C, Bogliun G, Logroscino G, Manfredi L, Mazzini L, et al. The Italian ALS study group; reliability of the El Escorial diagnostic criteria for amyotrophic lateral sclerosis. Neuroepidemiology 2002; 21: 265-70.

  14. Mitchell JD, Borasio GD. Amyotrophic lateral sclerosis. Lancet 2007; 369: 2031-41.

  15. Baek WS, Desai NP. ALS: pitfalls in the diagnosis. Prac Neurol 2007;7;74-81.

  16. Schulte-Mattler JM, Müller T, Zierz S. Transcranial magnetic stimulation compared with upper motor neuron signs in patients with amyotrophic lateral sclerosis. J Neurol Sci 1999;170: 51-6.

  17. Eisen A. Clinical electrophysiology of the upper and lower motor neuron in amyotrophic lateral sclerosis. Semin Neurol 2001;21 (2):141-54.

  18. Day BL, Dressler D, Maertens DN. Electric and magnetic stimulation of human motor cortex: surface EMG and single motor unit responses [published erratum appears in J Physiol (Lond) 1990; 430:617]. J Physiol (Lond) 1989; 412:449-73.

  19. Cedarbaum JC, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sci 1999;169:13-21.

  20. Claus D, Brunh C, Kerling FP, Henschel S. Transcranial magnetic stimulation as a diagnostic and prognostic test in amyotrophic lateral sclerosis. J Neurol Sci 1995; 129 (Suppl.): 30-4.

  21. Urban PP, Wicht S, Hopf HC. Sensitivity of transcranial magnetic stimulation of cortico-bulbar vs. cortico-spinal tract involvement in amyotrophic lateral sclerosis (ALS) J Neurol 2001;248:850-5.

  22. Wilbourn AJ. Clinical neurophysiology in the diagnosis of amyotrophic lateral sclerosis: The Lambert and the El Escorial criteria. J Neurol Sci 1998; 160 (Suppl. 1): S25–S9.

  23. Osei-Lah AD, Mills KR. Optimising the detection of upper motor neuron function dysfunction in amyotrophic lateral sclerosis – a transcranial magnetic stimulation study. J Neurol 2004; 251:1364-9.

  24. Komissarow F, Rollnik JD, Bogdanova D, Krampfl K, Khabirov FA, et al. Triple stimulation technique (TST) in amyotrophic lateral sclerosis. Clin Neurophysiol 2004; 115: 356-60.

  25. Rösler KM, Truffert A, Hess CW, Magistris MR. Quantification of upper motor neuron loss in amyotrophic lateral sclerosis. Clin Neurophysiol 2000; 111: 2208-18.

  26. Attarian S, Azulay JP, Lardillier D, Verschueren A, Pouget J. Transcranial magnetic stimulation in lower motor neuron diseases. Clin Neurophysiol 2005; 116: 35-42.

  27. Carvalho M, Turkman A, Swash M. Motor responses evoked by transcranial magnetic stimulation and peripheral nerve stimulation in the ulnar innervation in amyotrophic lateral sclerosis: the effect of upper and lower motor neuron lesion. J Neurol Sci 2003; 210: 83-90.

  28. Mitsumoto H, Ulug Am, Pullman SL, GoochCL, Chan S,Tang MX, et al. Quantitative objective markers for upper and lower motor neuron dysfunction in ALS. Neurol 2007;68:1402-10.

  29. Truffert A, Rösler KM, Magistris MR. Amyotrophic lateral sclerosis versus cervical spondylotic myelopathy: a study using transcranial magnetic stimulation with recordings from the trapezius and limb muscles. Clin Neurophysiol 2000; 111:1031-8.




2020     |     www.medigraphic.com