Stretching the limbs? Tonic spasms in multiple sclerosis

Abstract

SummaryA 23-year-old man with a clinically isolated syndrome (right optic neuritis) diagnosed 6 months before, presented with recurrent, brief, painful, stereotyped, involuntary posturing movements of the left upper limb. The neurological examination was otherwise unremarkable (except for right optic atrophy). Intravenous methylprednisolone was initiated; the paroxysms persisted and worsened 7 days later, as the left lower limb and hemiface became affected. A video-EEG showed no epileptiform activity despite the movements. Brain MRI revealed new lesions affecting the right pyramidal tract, contralateral to the clinical manifestations. Valproate was prescribed and the paroxysms were completely resolved 5 days later. Tonic spasms are classically, although infrequently, seen in multiple sclerosis, and may clinically resemble primary paroxysmal dyskinesias or even focal motor epileptic seizures. BACKGROUND

Pain in multiple sclerosis: A systematic review of neuroimaging studies

“…Lesions were identified in the CNS ( i.e. central neuropathic pain) in 21 studies; of these, lesions were located in the spinal cord in six studies ((Alstadhaug et al, 2008; Burkey and Abla-Yao, 2010; Tosi et al, 1998) (de Santi et al, 2009) — three cases (Hellwig et al, 2006) — four cases: only two documented with MRI (Marchettini et al, 2006) — five cases), in the brainstem in 13 studies ((Bentley et al, 2002; Donat, 2012; Gentile et al, 2007; González-Quintanilla et al, 2012; Haas et al, 1993; Leandri et al, 1999; Liu et al, 2008; Tanei et al, 2010) (Meaney et al, 1995; Nakashima et al, 2001; Vilisaar and Constantinescu, 2006) — one case (Fragoso and Brooks, 2007) — two cases (Cordella et al, 2009) — five cases), in the thalamus in one study (Deppe et al, 2013) and in multiple locations throughout the pyramidal tract in another study (Andrade et al, 2012). …”

“…1). Thirty-eight met the inclusion criteria (Alstadhaug et al, 2008; Andrade et al, 2012; Athanasiou et al, 2005; Balasa and Bajko, 2010; Bentley et al, 2002; Broggi et al, 2004; Burkey and Abla-Yao, 2010; Carrieri et al, 2009; Cordella et al, 2009; Cruccu et al, 2009; da Silva et al, 2005; Davey and Al-Din, 2004; Deppe et al, 2013; de Santi et al, 2009; Donat, 2012; Eldridge et al, 2003; Fragoso and Brooks, 2007; Gass et al, 1997; Gee et al, 2005; Gentile et al, 2007; González-Quintanilla et al, 2012; Haas et al, 1993; Hellwig et al, 2006; Kister et al, 2010; Leandri et al, 1999; Liu et al, 2008; Marchettini et al, 2006; Meaney et al, 1995; Minagar and Sheremata, 2000; Nakashima et al, 2001; Pichiecchio et al, 2007; Ramirez-Lassepas et al, 1992; Svendsen et al, 2011; Tanei et al, 2010; Tortorella et al, 2006; Tosi et al, 1998; Vilisaar and Constantinescu, 2006; Yetimalar et al, 2008). Of these, 16 were case reports (Alstadhaug et al, 2008; Andrade et al, 2012; Bentley et al, 2002; Burkey and Abla-Yao, 2010; Carrieri et al, 2009; Davey and Al-Din, 2004; Donat, 2012; Gentile et al, 2007; González-Quintanilla et al, 2012; Haas et al, 1993; Leandri et al, 1999; Liu et al, 2008; Pichiecchio et al, 2007; Tanei et al, 2010; Tosi et al, 1998; Vilisaar and Constantinescu, 2006), nine were case series (Athanasiou et al, 2005; Cordella et al, 2009; de Santi et al, 2009; Fragoso and Brooks, 2007; Hellwig et al, 2006; Marchettini et al, 2006; Meaney et al, 1995; Minagar and Sheremata, 2000; Nakashima et al, 2001), and 13 were investigational studies (<...>…”

“…All lesions thought to explain the body pain syndromes were located in the spinal cord (Table 5), except for the painful tonic spasms where lesions were identified in the pyramidal tract in the brain (Andrade et al, 2012) (Table 5). …”

Paroxysmal Symptoms in Multiple Sclerosis—A Review of the Literature

“…Also known as tonic spasms, these episodes consist of abrupt onset, stereotyped involuntary dystonic posturing. PD can be categorized into painful vs. painless, kinesigenic vs. non-kinesigenic, and generalized vs. focal [ 10 , 11 , 12 , 13 , 14 ]. Generalized dystonia is described as an episode of unilateral upper limb flexion and lower limb extension with a potential to spread to the neck or face [ 14 , 15 , 16 ].…”

“…They last for less than 1 min [ 14 , 15 , 16 , 17 , 18 ], and tend to gradually increase in frequency over time, reaching up to 100 times per day [ 15 ]. Prior to the onset of a dystonic episode, an unpleasant ipsilateral or contralateral sensory aura can occur [ 4 , 12 , 19 ]. This might be due to subtle muscle contractions insufficient to cause visible movements [ 21 ].…”