Progressing Spasticity, Cognitive Deficit and Non-elicitable Cortical Motor Evoked Potentials as Signs of Probable Primary Lateral Sclerosis – a Case Report

Authors: A. Kopal 1;  E. Ehler 1;  I. Rektorová 2,3
Authors‘ workplace: Neurologická klinika, Pardubická krajská nemocnice 1;  Výzkumná skupina Aplikované neurovědy, Středoevropský technologický institut, CEITEC MU, Brno 2;  I. neurologická klinika LF MU a FN u sv. Anny v Brně 3
Published in: Cesk Slov Neurol N 2011; 74/107(5): 579-583
Category: Case Report


Motor neurone diseases comprise a heterogeneous group of disorders that affect the upper motor neurone and/or the lower motor neurone. Both amyotrophic lateral sclerosis and primary lateral sclerosis fall into the category of motor neurone diseases. Amyotrophic lateral sclerosis is usually defined as a fatal neurodegenerative disorder that progressively affects both the upper and lower motor neurone, with mean survival between three and five years, although long-term survival may occur, albeit infrequently. Primary lateral sclerosis is an idiopathic, non-familial neurodegenerative disorder of the upper motor neurone.

Key words:
motor neurone diseases – primary lateral sclerosis – amyotrophic lateral sclerosis – spasticity – motor evoked potentials – cognitive deficit


1. Tartaglia MC, Rowe A, Findlater K, Orange JB, Grace G, Strong MJ. Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up. Arch Neurol 2007; 64(2): 232–236.

2. Singer MA, Statland JM, Wolfe GI, Barohn RJ. Primary lateral sclerosis. Muscle Nerve 2007; 35(3): 291–302.

3. Keller O. Amyotrofická laterální skleróza, onemocnění motoneuronu. Ces Slov Neurol N 2006; 69/102(6): 408–417.

4. Nasreddine ZS, Phillips NA, Bédirian V, Charbonneau S, Whitehead V, Collin I et al. The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc 2005; 53(4): 695–699.

5. Gordon PH, Cheng B, Katz IB, Mitsumoto H, Rowland LP. Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS. Neurology 2009; 72(22): 1948–1952.

6. Floeter MK, Mills R. Progression in primary lateral sclerosis: a prospective analysis. Amyotroph Lateral Scler 2009; 10(5–6): 339–346.

7. Brugman F, Veldink JH, Franssen H, de Visser M, de Jong JM, Faber CG et al. Differentiation of hereditary spastic paraparesis from primary lateral sclerosis in sporadic adult-onset upper motor neuron syndromes. Arch Neurol 2009; 66(4): 509–514.

8. Warnecke T, Duning T, Schirmacher A, Mohammadi S, Schwindt W, Lohmann H et al. A novel splice site mutation in the SPG7 gene causing widespread fiber damage in homozygous and heterozygous subjects. Mov Disord 2010; 25(4): 413–420.

9. Rektorová I. Frontotemporální lobární degenerace – diagnóza z neuro-psychiatrického pomezí. Neurol Prax 2006; 7(4): 208–211.

10. Meier SL, Charleston AJ, Tippett LJ. Cognitive and behavioral deficits associated with the orbitomedial prefrontal cortex in amyotrophic lateral sclerosis. Brain 2010; 133(11): 3444–3457.

11. Pringle CE, Hudson AJ, Munos DG, Kiernan JA, Brown WF, Ebers GC. Primary lateraly sclerosis. Clinical features, neuropatology and diagnostic criteria. Brain 1992; 115(Pt 2): 495–520.

12. Claassen DO, Josephs KA, Peller PJ. The stripe of primary lateral sclerosis: frontal primary motor cortex hypometabolism seen on fluorodesoxyglucose F18 positron emission tomography. Arch Neurol 2010; 67(1): 122–125.

13. Turner MR, Hammers A, Al-Chalabi A, Shaw CE, Andersen PM, Brooks DJ et al. Cortical involvement of four cases of primary lateral sclerosis using [11C]--flumazenil PET. J Neurol 2007; 254(8): 1033–1036.

14. Dufek J. Transkraniální magnetická stimulace a onemocnění motorického neuronu. In: Bareš M, Kaňovský P, Dufek J et al (eds). Transkraniální magnetická stimulace. Brno: NCO NZO 2003: 140–148.

15. Hromada J, Keller O. Primární laterální skleróza. Neurol Prax 2006; 7(1): 28–29.

16. Milano JB, Neto MC, Hunhevicz SC, Arruda WO, Ramina R, Barros E Jr. Intrathecal baclofen for spasticity in primary lateral sclerosis. J Neurol 2005; 252(6): 740–741.

17. Mabuchi N, Watanabe H, Atsuta N, Ito H, Fukatsu H, Kato T et al. Primary lateral sclerosis presenting parkinsonian symptoms without nigrostriatal involvement. J Neurol Neurosurg Psychiatry 2004; 75(12): 1768–1771.

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