Top

Published in:

01-02-2010 | Short Commentary

Effects of immunotherapy on motor cortex excitability in Stiff Person Syndrome

Authors: Simone Rossi, Monica Ulivelli, Maria Malentacchi, Giuseppe Greco, Sabina Bartalini, Patrizia Borgogni, Fabio Giannini

Published in: Journal of Neurology | Issue 2/2010

Abstract

A number of cortical and spinal excitability variables have been tested in a patient with Stiff Person Syndrome (SPS), before and after immunotherapy with mycophenolate mofetil, intravenous immunoglobulin and corticosteroids, which normalized plasma levels of anti-GAD antibodies and dramatically improved the clinical picture. The overlapping time-course of neurophysiological, clinical and bio-umoral findings suggests that immunotherapy might have changed GABA/Glutamate balance at cortical level, favoring the former, as reflected by normalization of the startle reflex, lengthening of the cortical silent period and clear-cut reduction of intracortical facilitation to paired-pulse transcranial magnetic stimulation. This represents the first report investigating effects of immunotherapy on cortical excitability in SPS.

Dalakas MC, Fuji M, Li M, McRoy B (2000) The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome. Neurology 55:1531–1535PubMed

Meinck HM, Ricker K, Conrad B (1984) The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine. J Neurol Neurosurg Psychiatry 47:280–287CrossRefPubMed

Perani D, Garibotto V, Moresco RM et al (2007) PET evidence of central GABAergic changes in stiff-person syndrome. Mov Dis 22:1030–1033CrossRef

Levy LM, Levy-Reis I, Fujii M, Dalakas MC (2005) Brain aminobutyric acid changes in stiff-person syndrome. Arch Neurol 62:970–974CrossRefPubMed

Rossini PM, Rossi S (2007) Transcranial magnetic stimulation. Diagnostic, therapeutic and research potential. Neurology 68:484–488CrossRefPubMed

Chen R, Cros D, Curra A et al (2008) The clinical diagnostic utility of transcranial magnetic stimulation: report of an IFCN committee. Clin Neurophysiol 119:504–532CrossRefPubMed

Koerner C, Wieland B, Richter W, Meinck HM (2004) Stiff-person syndromes. Motor cortex hyperexcitability correlates with anti-GAD autoimmunity. Neurology 62:1357–1362PubMed

Ziemann U, Rothwell JC, Ridding MC (1996) Interaction between intracortical inhibition and facilitation in human motor cortex. J Physiol (Lond) 496:873–881

Di Lazzaro V, Oliviero A, Meglio M et al (2000) Direct demonstration of the effect of lorazepam on the excitability of the human motor cortex. Clin Neurophysiol 111:794–799CrossRefPubMed

10.

Yu M (2007) Recent developments of the PET imaging agents for metabotropic glutamate receptor subtype 5. Curr Top Med Chem 7:1800–1805CrossRefPubMed

11.

Dalakas MCJ (2005) The role of IVIg in the treatment of patients with stiff person syndrome and other neurological diseases associated with anti-GAD antibodies. J Neurol 252(Suppl 1):119–125

12.

Rossini PM, Barker AT, Berardelli A et al (1994) Non-invasive electrical and magnetic stimulation of the brain, spinal cord and roots: basic principles and procedures for routine clinical application. Report of an IFCN committee. Electroencephalogr Clin Neurophysiol 91:79–92CrossRefPubMed

13.

Ziemann U, Lo¨nnecker S, Steinhoff BJ, Paulus W (1996) Effects of antiepileptic drugs on motor cortex excitability in humans: a transcranial magnetic stimulation study. Ann Neurol 40:367–378CrossRefPubMed

14.

Tokimura H, Di Lazzaro V, Tokimura Y et al (2000) Short latency inhibition of human hand motor cortex by somatosensory input from the hand. J Physiol (Lond) 523:503–513CrossRef

15.

Di Lazzaro V, Oliviero A, Saturno E et al (2005) Effects of lorazepam on short latency afferent inhibition and short latency intracortical inhibition in humans. J Physiol 564:661–668CrossRefPubMed

16.

Di Lazzaro V, Pilato F, Di leone M, Proficue P, Ranieri F, Ricci V et al (2007) Segregating two inhibitory circuits in human motor cortex at the level of GABAA receptor subtypes: a TMS study. Clin Neurophysiol 118:2207–2214CrossRefPubMed

17.

Ziemann U, Lonnecker S, Steinhoff BJ, Paulus W (1996) The effect of lorazepam on the motor cortical excitability in man. Exp Brain Res 109:127–135CrossRefPubMed

18.

Siebner HR, Dressnandt J, Auer C, Conrad B (1998) Continuous intrathecal baclofen infusions induced a marked increase of the transcranially evoked silent period in a patient with generalized dystonia. Muscle Nerve 21:1209–1212CrossRefPubMed

19.

Hanajima R, Ugawa Y, Terao Y et al (1998) Paired-pulse magnetic stimulation of the human motor cortex: differences among I waves. J Physiol (Lond) 509:607–618CrossRef

20.

Ziemann U, Chen R, Cohen LG, Hallett M (1998) Dextromethorphan decreases the excitability of the human motor cortex. Neurology 51:1320–1324PubMed

21.

Rossi S, De Capua A, Tavanti M, Calossi S, Polizzotto NR, Mantovani A, Falzarano V, Bossini L, Passero S, Bartalini S, Ulivelli M (2009) Dysfunctions of cortical excitability in drug-naive posttraumatic stress disorders patients. Biol Psychiatry 66:54–61CrossRefPubMed

22.

Sandbrink F, Ali Syed N, Fuji MD, Akas C, Floeter MK (2000) Motor cortex excitability in stiff-person syndrome. Brain 123:2231–2239CrossRefPubMed

23.

Matsumoto JY, Caviness JN, McEvoy KM (1994) The acoustic startle reflex in stiff-man syndrome. Neurology 44:1952–1955PubMed

24.

Meinck HM, Ricker K, Hulser PJ, Solimena M (1995) Stiff man syndrome: neurophysiological findings in eight patients. J Neurol 242:134–142CrossRefPubMed

25.

Floeter MK, Valls Solè JP, Toro C, Hallett M (1998) Physiologic studies of spinal inhibitory circuits in patients with stiff-person syndrome. Neurology 51:85–93PubMed

26.

Lorish TR, Thorsteinsson G, Howard FM Jr (1989) Stiff-man syndrome updated [Review]. Mayo Clin Proc 64:624–636

27.

Baker MR, Das M, Isaacs J, Fawcett PRW, Bates D (2005) Treatment of stiff person syndrome with rituximab. J Neurol Neurosurg Psychiatry 76:999–1001CrossRefPubMed

28.

Lockman J, Burns TM (2007) Stiff-person syndrome. Curr Treat Options Neurol 9:234–240CrossRefPubMed

Title: Effects of immunotherapy on motor cortex excitability in Stiff Person Syndrome
Authors: Simone Rossi
Monica Ulivelli
Maria Malentacchi
Giuseppe Greco
Sabina Bartalini
Patrizia Borgogni
Fabio Giannini
Publication date: 01-02-2010
Publisher: D. Steinkopff-Verlag
Published in: Journal of Neurology / Issue 2/2010
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-009-5331-z

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Effects of immunotherapy on motor cortex excitability in Stiff Person Syndrome

Abstract

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 2/2010

The social impact of dizziness in London and Siena

Bilateral brainstem activation by thermal stimulation of the face in healthy volunteers

Blind spot heterotopia by automated static perimetry to assess static ocular torsion: centro-cecal axis rotation in normals

Musty odour, mental retardation, and spastic paraplegia revealing phenylketonuria in adulthood

Demographic and clinic characteristics of French patients treated with natalizumab in clinical practice

Frequency of the FKRP mutation c.826C>A in isolated hyperCKemia and in limb girdle muscular dystrophy type 2 in German patients