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Efficacy Of Dysport In The Treatment Of Tremulous Cervical Dystonia Due To Vitamin E Deficiency, About Two Families

Chahrazad Boukadir, Wahiba Amer El Khedoud

Neurology Department, Ben Aknoun Hospital, Algiers, Algeria

Ben Aknoun, 16028. Algiers. Algeria

Chahrazed.Doc@gmail.com

Introduction : Vitamin E deficiency ataxia is an autosomal recessive neurodegenerative disorder manifesting as ataxia, areflexia, and proprioceptive disturbances, rarely cervical dystonia, which is often tremulous [1], [2], [3].

High-dose vitamin E supplementation may halt disease progression and improve clinical signs. In some cases, improvement may be incomplete, requiring symptomatic treatment of the various signs, especially if treatment was not initiated early in the course of the disease [1], [2].

Botulinum toxin is the first-line symptomatic treatment for cervical trembling dystonia and requires precisely identifying and targeting of the cervical muscles involved in both the movement and the dystonic tremor [4].

Methods :We report the case of three patients from two families followed for vitamin E deficiency: patient C.M., aged 27, and her sister C.R., aged 25,and patient MS, aged 23, who presented with laterocaput-type cervical dystonia associated with dystonic tremor, resistant to oral medical treatment and vitamin E. They received botulinum toxin sessions with Dysport every three months for two years, targeting the splenius, sternocleidomastoid, or oblique captis inferioris ()OCI muscles of the head on both sides under ultrasound guidance for the treatment of dystonic tremor, and other muscles for the treatment of dystonic movement (laterocaput defined according to the Col-Cap concept), such as the ipsilateral trapezius. The mean total dose was 700 Speywood units.

Results : A progressive improvement was noted in the TWSTRS score [5] and especially the TSUI scale [6] [which also assesses the intensity of the associated dystonic tremor, which were carried out before each toxin session and one month after.

The efficacy of botulinum toxin in the treatment of idiopathic and secondary cervical trembling dystonia [4], particularly those associated with Parkinsonian syndromes, has already been proven [7]. No publications have been published regarding its efficacy in the treatment of cervical trembling dystonia due to vitamin E deficiency.

Cervical dystonia is rare in the clinical presentation of vitamin E deficiency ataxias [1]. It is often associated with dystonic tremor of the head [1], involving other muscles in the dystonic movement such as the splenius, sternocleidomastoid or inferior oblique of the head on both sides [4].

Conclusion : Botulinum toxin may represent an effective therapeutic alternative in the symptomatic treatment of cervical dystonia in patients with vitamin E deficiency refractory to oral medical treatment.

Keywords : Dysport; Trembling cervical dystonia; Vitamin E deficiency; Col-cap concept; Ultrasound-guidance; The TSUI scale.

References

[1] - Andrew E and colleagues. Ataxia with Vitamin E Deficiency May Present with Cervical Dystonia. Tremor and Other Hyperkinetic Movements.2016.

[2] - Moualek D et al. Ataxie par carence en vitamine E avec dystonie cervicale prédominante. Association nationale des neurologues privés (ANNP).Guide de la santé en Algérie.2022.

[3]- Toni S. Pearson* More Than Ataxia: Hyperkinetic Movement Disorders in Childhood Autosomal Recessive Ataxia Syndromes. Tremor and Other Hyperkinetic Movements.2016.

[4]- Sanjay Pandey* and colleagues. Tremor in Idiopathic Cervical Dystonia – Possible Implications for Botulinum Toxin Treatment Considering the Col-Cap Classification. Tremor and Other Hyperkinetic Movements. 2020.

[5] - D. Gayraud, F. Viallet. Évaluation d’une dystonie cervicale. Utilité des échelles d’évaluation : échelle TWSTRS. Revue Neurologique FMC - © 2008.

[6] -Wolfgang H. Jost •and colleagues.Rating scales for cervical dystonia: a critical evaluation of tools for outcome assessment of botulinum toxin therapy. J Neural

Transm (2013) 120:487-496. [7]- Wolfgang H. Jost.Use of Botulinum Neurotoxin in Parkinson’s Disease: A Critical Appraisal. Toxins 2021, 13, 87.