Recovery From Cervical Dystonia With Topiramate

Abstract

Primary cervical dystonia (CD), spasmodic torticollis, is the most common focal dystonia. In the US between 60,000 and 90,000 people have been estimated to suffer from this painful disorder with rare recovery and high psychiatric co-morbidity. The therapy of CD has been symptomatic. The primary treatment, repeated chemodenervation of cervical muscles with botulinum toxin injections, is expensive and not available to the majority of persons with CD. Neither controlled studies of oral pharmacotherapy of dystonia, nor reports of sustained full remission were published. Thus, it was unclear if any effective and safe oral pharmacotherapy for CD exists, and if it is possible to remain asymptomatic. I report on a 48-year-old woman who has been symptom-free during nearly 4 years treatment with topiramate, after previously suffering from familial CD during 29 years. Her left-sided lateral postural and rotating dystonia increased in stressful situations and during physical exercise, and had not responded to any drugs including Botox injections. Topiramate (150 mg/day) was administered for her schizo-affective disorder in 2011, after 11 months of treatment with clozapine (150 mg/day) in a forensic psychiatric hospital. Postural and rotating symptoms subsided within 2 months, and muscular hypertrophy, pain, dysphagia, and daily headache recovered. The author examined her lifetime health care referrals and documented her symptoms in the hospital during 6 years. Informed consent was obtained. CD is associated with reduction of gamma-aminobutyric acidergic (GABAergic) neurons in the basal ganglia. Topiramate, a sulfamate-substituted monosaccharide, modulates both GABAergic and glutaminergic neurotransmission. It has been used since the 90s for epilepsy and many neurological and psychiatric disorders, on the basis of randomized controlled trials. My case study demonstrates that some patients recover from CD during treatment with topiramate, and remain asymptomatic for years. This is in accordance with the pharmacodynamic mechanisms of topiramate, the pathophysiology of involuntary movements, and the clinical evidence of other hyperkinetic movement disorders with similar pathophysiology. It would be important to conduct randomized controlled studies of topiramate in CD, and to define the subgroups who may benefit from it.




J Med Cases. 2015;6(8):373-375
doi: http://dx.doi.org/10.14740/jmc2088w