[Botulinum toxin type B in the management of dystonia non-responsive to botulinum toxin type A]. 2003

Francisco Cardoso
Serviço de Neurologia, Universidade Federal de Minas Gerais, Belo Horizonte, Brasil. cardosof@metalink.com.br

BACKGROUND Botulinum toxin (BTX) injection is the first choice treatment for focal dystonias. However 10% or more of patients who receive repetitive injections of BTX type A (BTX-A) lose response (secondary non-responders). One of the strategies to manage such patients is to treat them with another serotype. The aim of this article is to describe my experience with BTX type B (BTX-B) in the management of patients with focal dystonia who became secondary non-responders to BTX-A. METHODS Open-label non-controlled use of BTX-B injections to treat dystonia patients who developed secondary nonresponse to BTX-A Response to treatment was rated on a 0-4 scale (Jankovic). RESULTS Four patients entered the study. Pacient 1- At age 48 this man developed idiopathic cervical dystonia. Five years later he also presented with blepharospasm and idiopathic oromandibular dystonia. He was treated with 7604U of BTX-A along 23 sessions separated by a mean interval of 18.8 weeks (range 6-39). Loss of response was noticed after the seventh session. First treatment with BTX-B consisted of injection of 20000U with response rated 3 but duration of 3 weeks. Second session, 23500U, resulted in score 4 with response lasting 12 weeks. Patient 2- This man, with Tourette syndrome since age 8 years, developed tardive blepharospasm at age 51. On 8 sessions of BTX-A injections he received a cumulative dosage of 550U with a mean interval between sessions of 8.8 weeks (range 6-12). Decline of response was noticed after the fifth session. First treatment with BTX-B, 3000U, had a response rated 3 with duration of 12 weeks. Second session, 6000U, resulted in score 4. Patient 3- This woman noticed onset of blepharospasm at age 58 and developed oromandibular and laryngeal dystonia as well as cervical dystonia, respectively, at ages 59 and 65. In other institutions she received 6 sessions of BTX-A. In my service she received a dosage of 1404U along 8 sessions with a mean interval between sessions of 17.4 weeks (range 16-18). She became secondary non-responder after the ninth session. First treatment with BTX-B, 6000U, was rated 0. Second session, 12000U, was rated 4. Patient 4- At age 69 this man developed idiopathic cranial dystonia. Prior to follow up with me, he received 6 sessions of BTX-A in other services. In my institution he was treated with a cumulative dosage of 730U along 4 sessions with a mean interval between sessions of 16.3 weeks (range 15-18). He developed loss of response on the sixth session. Treatment with BTX-B, 12000U, was rated 4 and lasted 20 weeks. Side-effects: local pain (all patients) and dryness of mouth and ptosis (one patient each). CONCLUSIONS My findings confirm that BTX-B injections are a safe and effective option for the management of dystonia patients who become secondary non-responders to BTX-A. The results also underscore the need of individualizing dosage regimens before optimum results are achieved.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009465 Neuromuscular Agents Drugs used for their actions on skeletal muscle. Included are agents that act directly on skeletal muscle, those that alter neuromuscular transmission (NEUROMUSCULAR BLOCKING AGENTS), and drugs that act centrally as skeletal muscle relaxants (MUSCLE RELAXANTS, CENTRAL). Drugs used in the treatment of movement disorders are ANTI-DYSKINESIA AGENTS. Skeletal Muscle Relaxants,Neuromuscular Effect,Neuromuscular Effects,Agents, Neuromuscular,Effect, Neuromuscular,Effects, Neuromuscular,Muscle Relaxants, Skeletal,Relaxants, Skeletal Muscle
D001905 Botulinum Toxins Toxic proteins produced from the species CLOSTRIDIUM BOTULINUM. The toxins are synthesized as a single peptide chain which is processed into a mature protein consisting of a heavy chain and light chain joined via a disulfide bond. The botulinum toxin light chain is a zinc-dependent protease which is released from the heavy chain upon ENDOCYTOSIS into PRESYNAPTIC NERVE ENDINGS. Once inside the cell the botulinum toxin light chain cleaves specific SNARE proteins which are essential for secretion of ACETYLCHOLINE by SYNAPTIC VESICLES. This inhibition of acetylcholine release results in muscular PARALYSIS. Botulin,Botulinum Neurotoxin,Botulinum Neurotoxins,Clostridium botulinum Toxins,Botulinum Toxin,Neurotoxin, Botulinum,Neurotoxins, Botulinum,Toxin, Botulinum,Toxins, Botulinum,Toxins, Clostridium botulinum
D004351 Drug Resistance Diminished or failed response of an organism, disease or tissue to the intended effectiveness of a chemical or drug. It should be differentiated from DRUG TOLERANCE which is the progressive diminution of the susceptibility of a human or animal to the effects of a drug, as a result of continued administration. Resistance, Drug
D004421 Dystonia An attitude or posture due to the co-contraction of agonists and antagonist muscles in one region of the body. It most often affects the large axial muscles of the trunk and limb girdles. Conditions which feature persistent or recurrent episodes of dystonia as a primary manifestation of disease are referred to as DYSTONIC DISORDERS. (Adams et al., Principles of Neurology, 6th ed, p77) Muscle Dystonia,Dystonia, Diurnal,Dystonia, Limb,Dystonia, Paroxysmal,Diurnal Dystonia,Dystonia, Muscle,Limb Dystonia,Paroxysmal Dystonia
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes

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