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Original article| Volume 45, 102408, October 2020

Painful tonic spasm in Chinese patients with neuromyelitis optica spectrum disorder: Prevalence, subtype, and features

      Highlights

      • PTS associated with myelitis was a common symptom in NMOSD patients.
      • PTS exerts a great impact on daily life and requires active intervention.
      • Of the 5 PTS subtypes, flexor tonic spasm were observed more often in the upper extremity, while extensor tonic spasm were more frequent in the lower extremity; and isometric tonic spasm were principally found in the trunk.
      • Among the subtypes, complex tonic spasm was the most common and serious subtype.

      Abstract

      Objectives

      Painful tonic spasm (PTS) is a common symptom in patients with neuromyelitis optica spectrum disorder (NMOSD). We herein aimed to explore the prevalence, subtype, and features of PTS in Chinese Han patients with NMOSD.

      Methods

      We retrospectively reviewed medical records and interviewed patients with NMOSD who were admitted to Huashan Hospital and the Jing'an District Center Hospital of Fudan University in Shanghai, China, from February 2017 to May 2019. The primary questionnaires included a Numerical Rating Scale (NRS), Spinal Cord Injury Health Questionnaire (SCI-HQ), Penn Spasm Frequency Scale (PSFS), and Patient Global Impression of Improvement (PGI-I).

      Results

      Sixty-seven of 153 (43.79%) patients with NMOSD showed PTS, only when spinal cord was involved. PTS had a negative impact on daily life in 97.01% (65/67) of the patients, and 92.54% (62/67) of the patients needed symptomatic treatment. Pain intensity and frequency of PTS were significantly different (P = 0.018 with NRS, P = 0.045 with PSFS) among flexor tonic spasm, extensor tonic spasm, isometric tonic spasm and complex tonic spasm subtype. Forty patients (59.71%) manifested complex tonic spasms, which indicated more severe pain and frequent spasms comparing to other subtypes. The locations of PTS were significantly different among the 5 subtypes (P<0.001), i.e.,77.78% (7/9) of flexor tonic spasms appeared in the upper extremity, 100% (9/9) of extensor tonic spasm occurred in the lower extremity, and isometric tonic spasms principally occurred in the trunk (87.5%). Forty-one patients (66.13%) demonstrated good responses to the symptomatic treatments, and there were no statistical differences with respect to the therapeutic responses among the 5 PTS subtypes (P = 0.509).

      Conclusions

      PTS was associated with myelitis, and was a common symptom in NMOSD. Intensity, frequency and location were different among the PTS subtypes. Complex tonic spasm was the most common and serious subtype.

      Keywords

      Abbreviations:

      NMO (neuromyelitis optica), LETM (longitudinally extensive transverse myelitis), AQP4-ab (aquaporin 4-antibody), NMOSD (neuromyelitis optica spectrum disorders), ON (optic neuritis), PTS (painful tonic spasm), MS (multiple sclerosis), EEG (electroencephalograms), IgG (Immunoglobulin G), MOG (myelin oligodendrocyte glycoprotein), IIFT (immune-fluorescence test), MRI (magnetic resonance imaging), NRS (numerical rating scale), PSFS (Penn Spasm Frequency Scale), SCI-HQ (Spinal Cord Injury Health Questionnaire), PGI-I (Patient Global Impression of Improvement), TM (transverse myelitis), IVIG (intravenous immunoglobulin G)
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      References

        • Abboud H.
        • Macaron G.
        • Yu X.X.
        • Knusel K.
        • Fernandez H.H.
        • Bethoux F.
        Defining the spectrum of spasticity-associated involuntary movements.
        Parkinsonism Relat. Disord. 2019; 65: 79-85
        • Abboud H.
        • Fernandez H.H.
        • Mealy M.A.
        • Levy M.
        Spinal Movement disorders in neuromyelitis optica: an under-recognized phenomenon.
        Mov. Disord. Clin. Pract. 2016; 3: 596-602
        • Andrade C.
        • Massano J.
        • Guimaraes J.
        • Garrett M.C.
        Stretching the limbs? Tonic spasms in multiple sclerosis.
        BMJ Case Rep. 2012; 30 (2012)
        • Carnero C.E.
        • Leguizamon F.
        • Hryb J.P.
        • Celso J.
        • Pace J.L.
        • Ferrari J.
        • et al.
        . Neuromyelitis optica: association with paroxysmal painful tonic spasms.
        Neurologia. 2016; 31: 511-515
        • Holtz K.A.
        • Lipson R.
        • Noonan V.K.
        • Kwon B.K.
        • Mills P.B.
        Prevalence and effect of problematic spasticity after traumatic spinal cord injury.
        Arch. Phys. Med. Rehabil. 2017; 98: 1132-1138
        • Kim S.M.
        • Go M.J.
        • Sung J.J.
        • Park K.S.
        • Lee K.W.
        Painful tonic spasm in neuromyelitis optica: incidence, diagnostic utility, and clinical characteristics.
        Arch. Neurol. 2012; 69: 1026-1031
        • Kessler R.A.
        • Mealy. M.A.
        • Levy M.
        Treatment of neuromyelitis optica spectrum disorder: acute, preventive, and symptomatic.
        Curr. Treat. Options Neurol. 2016; 18: 2
        • Liu J.
        • Zhang Q.
        • Lian Z.
        • Chen H.
        • Shi Z.
        • Feng H.
        • et al.
        Painful tonic spasm in neuromyelitis optica spectrum disorders: prevalence, clinical implications and treatment options.
        Mult. Scler. Relat. Disord. 2017; 17: 99-102
        • Matthews W.B.
        Tonic seizures in disseminated sclerosis.
        Brain. 1958; 81: 193-206
        • Priebe M.M.
        • Sherwood A.M.
        • Thornby J.I.
        • Kharas N.F.
        • Markowski J.
        Clinical assessment of spasticity in spinal cord injury: a multidimensional problem.
        Arch. Phys. Med. Rehabil. 1996; 77: 713-716
        • Usmani N.
        • Bedi G.
        • Lam B.L.
        • Sheremata W.A.
        Association between paroxysmal tonic spasms and neuromyelitis optica.
        Arch. Neurol. 2012; 69: 121-124
        • Viktrup L.
        • Hayes. R.P.
        • Wang P.
        • Shen W.
        Construct validation of patient global impression of severity (PGI-S) and improvement (PGI-I) questionnaires in the treatment of men with lower urinary tract symptoms secondary to benign prostatic hyperplasia.
        BMC Urol. 2012; 12: 30
        • Wingerchuk D.M.
        • Banwell B.
        • Bennett J.L.
        • Cabre P.
        • Carroll W.
        • Chitnis T.
        • et al.
        . International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
        Neurology. 2015; 85: 177-189
        • Wingerchuk D.M.
        • Lennon. V.A.
        • Lucchinetti. C.F.
        • Pittock. S.J.
        • Weinshenker. B.G.
        The spectrum of neuromyelitis optica.
        Lancet Neurol. 2007; 6: 805-815
        • Wingerchuk D.M.
        • Lennon V.A.
        • Pittock S.J.
        • Lucchinetti C.F.
        • Weinshenker B.G.
        Revised diagnostic criteria for neuromyelitis optica.
        Neurology. 2006; 66: 1485-1489
        • Wingerchuk D.M.
        • Hogancamp W.F.
        • O’Brien P.C.
        • Weinshenker B.G.
        The clinical course of neuromyelitis optica (Devic’s syndrome).
        Neurology. 1999; 53: 1107-1114