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1.258 Singing-induced cervical dystonia
Poster Presentations: Related Disorders Results: Our two patients showed typical XP symptoms with neurological manifestations as well as described in the literature. The major neurological features of XP include microcephaly, progressive mental deterioration, choreoathetosis, ataxia, sensorineural deafness, areflexia, spasticity, abnormal EEG findings, neuropathy and cerebral atrophy. The progressive cognitive deficit is the most frequent neurological symptom in 80% of cases. Conclusion: The mechanism of neurological abnormalities remains unknown. It is suggested that ineffective DNA repair could lead to cell death and be responsible for neurological manifestations.
1.257 Cervical dystonia: Effect of botulinum toxin on trajectory formation E. Pelosin1° , M. Bove, L. Marinelli, C. Moisello, A. Di Rocco, M.F. Ghilardi, G. Abbruzzese 1 Genoa, Italy Objective: To ascertain whether the abnormal processing of information from muscle spindles present in asymptomatic muscles of patients with focal dystonia impair trajectory formation and whether botulinum toxin has restorative effects. Method: Ten patients with cervical dystonia (CD) (age range: 35−65 years) were tested with a motor task before (baseline) and 3 weeks after (Test) botulinum toxin injections in the overactive neck muscles. Ten aged-matched normal controls were tested twice three weeks apart. During the motor task, subjects performed out-and-back reaching movements on a digitizing tablet from a central starting point to one of eight targets displayed on a computer screen. Screen cursor was blanked and vision of the limb was blocked to prevent corrections during movement. Results: At baseline, hand-paths of controls were straight, with sharp reversals and overlapping out-and-back strokes. In patients with CD hand paths were more curved (p < 0.001) with increased area (p < 0.001) and longer reversal lags (time between end of the out motion and the beginning of the return, p < 0.0001) and increased asymmetry in the temporal velocity profile (p < 0.001). Botulinum treatment improved head posture in all patients. At test, hand-paths curvature, normalized areas, reversal lags and symmetry indexes decreased in CD although without reaching the controls’ range. Conclusion: Patients with CD, without clinically evident involvement of the upper limbs, display defects in trajectory formation that partially recover after treatment. Altogether, these findings suggest the presence of abormal interjoint coordination. They support the hypothesis that CD impairs the processing of proprioceptive input from upper limb. Treatment with botulinum toxin is helpful in restoring sensory processes.
1.258 Singing-induced cervical dystonia G. Fabiani1° , H. Teive 1 Curitiba, Brazil Objective: Our objective is to report on an unusual presentation of a task-specific cervical dystonia in a 24-year-old male. Method: We report on an unusual presentation of a task-specific dystonia in a 24-year-old male. After beginning a singer-career after the shows he suffered from cervical discomfort. He also presented difficulties to keep the voice normally pitched when singing, as he presented a turning of the head to the left. When he tried to keep his head on the right position his voice changed to a high-pitched, coarse or strangled tone. Neurological exam at rest was normal, but when singing Brazilian countrymusic, demonstrated a left-torticollis with severe right sternocleidomastoid muscle (SCM) hypertrophy. Results: Laboratory tests and cranial-CT scan were normal. Electromyography demonstrated prolonged bursts on the right SCM only when singing. A diagnosis of a task-specific cervical dystonia (spasmodic torticollis) was done. We decided on for intramuscular botulinum toxin type A (BT-A; Dysport) treatment. A total dose of 450 IU was injected, being 300 IU on
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the left splenius capitis and 150 IU on the right SCM. After 3 weeks he underwent a new neurological examination and the dystonic symptoms and also the SCM hypertrophy had vanished. Conclusion: Task-specific focal dystonia, occurs mainly in highly skilled manual tasks, and are usually related to occupation (piano players, architects, writers, shoemakers). Rare cases were described in oromandibular region (e.g., embrochure dystonia in brass players). The present case illustrates an unusual and to the best of our knowledge, never before reported case of task specific cervical dystonia elicited by the singing act.
1.259 Frontalis, corrugator and procerus dystonia: A blepharospasm variant? G. Fabiani1° , H. Teive Brazil
1 Curitiba,
Objective: Our objective is to report on an unusual presentation of dystonia in a 46-year-old male, affecting the frontalis, corrugator and procerus muscles. Method: We report on an unusual presentation of dystonia, affecting the frontalis, corrugator and procerus muscles, without any involvement of orbicularis oculi muscles. The patient is a 46-year-old male. Neurological exam disclosed involuntary movements of frontalis, corrugator and procerus muscles, bilaterally, without any compromising of orbicularis oculi muscles. He also presented oculogyric deviations (up). Results: Laboratory tests and cranial CT scan were normal. We decided on for intramuscular botulinum toxin type A (BT-A; Dysport) treatment. A total dose of 200/IU was injected in the frontalis, corrugator and procerus muscles. After 3 weeks he underwent a new neurological examination and the dystonic symptoms had vanished. Conclusion: The hallmark of the blepharospasm is the involvement of the orbicularis oculi muscles. For the diagnosis this feature is essential. Nonetheless the unusual features the patient presented, it was obvious to us that he presented a cranial dystonia with sole involvement of frontalis, corrugator and procerus muscles bilaterally. A total dose of 200/IU of botulinum toxin type A (BT-A; Dysport) was injected in the frontalis, corrugator and procerus muscles. After 3 weeks he underwent a new neurological examination and the dystonic symptoms had vanished.
1.260 Venlafaxine-induced bruxism H. Alonso Navarro1° , M. Mart´ın-Prieto, J.J. Ruiz-Ezquerro, F.J. Jim´enezJim´enez 1 Salamanca, Spain Objective: Bruxism is considered to be a focal dystonia which usually improved with botulinum toxin infiltrations in masticatory muscles. We report a patient who developed a typical bruxism during a treatment with venlafaxine, which disappeared after withdrawal of this drug. Method: A 62 years-old male patient without previous personal or familial history of movement disorders was evaluated because of a severe bruxism of 6 months of evolution. Results: Blood count, routine biochemistry, copper metabolism studies, thyroid hormones levels and serological studies for syphilis and Brucella were normal or negative. Brain MR imaging was normal. He was under therapy with venlafaxine 150 mg/day since the two weeks previous to the clinical onset of bruxism because of “depression” and “anxiety”. Two essays with botulinum toxin infiltrations in temporalis and masseter muscles (25 IU each) had been unsuccessful. Following venlafaxine withdrawal, bruxism improved gradually, and disappeared 2 months later. Conclusion: The temporal relation between the development of bruxism, together with its disappearance following venlafaxine-withdrawal, suggests a possible cause–effect relationship of bruxism with venlafaxine therapy. Venlafaxine should be considered as a possible cause of drug inducedbruxism.
1.257 Cervical dystonia: Effect of botulinum toxin on trajectory formation E. Pelosin1° , M. Bove, L. Marinelli, C. Moisello, A. Di Rocco, M.F. Ghilardi, G. Abbruzzese 1 Genoa, Italy Objective: To ascertain whether the abnormal processing of information from muscle spindles present in asymptomatic muscles of patients with focal dystonia impair trajectory formation and whether botulinum toxin has restorative effects. Method: Ten patients with cervical dystonia (CD) (age range: 35−65 years) were tested with a motor task before (baseline) and 3 weeks after (Test) botulinum toxin injections in the overactive neck muscles. Ten aged-matched normal controls were tested twice three weeks apart. During the motor task, subjects performed out-and-back reaching movements on a digitizing tablet from a central starting point to one of eight targets displayed on a computer screen. Screen cursor was blanked and vision of the limb was blocked to prevent corrections during movement. Results: At baseline, hand-paths of controls were straight, with sharp reversals and overlapping out-and-back strokes. In patients with CD hand paths were more curved (p < 0.001) with increased area (p < 0.001) and longer reversal lags (time between end of the out motion and the beginning of the return, p < 0.0001) and increased asymmetry in the temporal velocity profile (p < 0.001). Botulinum treatment improved head posture in all patients. At test, hand-paths curvature, normalized areas, reversal lags and symmetry indexes decreased in CD although without reaching the controls’ range. Conclusion: Patients with CD, without clinically evident involvement of the upper limbs, display defects in trajectory formation that partially recover after treatment. Altogether, these findings suggest the presence of abormal interjoint coordination. They support the hypothesis that CD impairs the processing of proprioceptive input from upper limb. Treatment with botulinum toxin is helpful in restoring sensory processes.
1.258 Singing-induced cervical dystonia G. Fabiani1° , H. Teive 1 Curitiba, Brazil Objective: Our objective is to report on an unusual presentation of a task-specific cervical dystonia in a 24-year-old male. Method: We report on an unusual presentation of a task-specific dystonia in a 24-year-old male. After beginning a singer-career after the shows he suffered from cervical discomfort. He also presented difficulties to keep the voice normally pitched when singing, as he presented a turning of the head to the left. When he tried to keep his head on the right position his voice changed to a high-pitched, coarse or strangled tone. Neurological exam at rest was normal, but when singing Brazilian countrymusic, demonstrated a left-torticollis with severe right sternocleidomastoid muscle (SCM) hypertrophy. Results: Laboratory tests and cranial-CT scan were normal. Electromyography demonstrated prolonged bursts on the right SCM only when singing. A diagnosis of a task-specific cervical dystonia (spasmodic torticollis) was done. We decided on for intramuscular botulinum toxin type A (BT-A; Dysport) treatment. A total dose of 450 IU was injected, being 300 IU on
S67
the left splenius capitis and 150 IU on the right SCM. After 3 weeks he underwent a new neurological examination and the dystonic symptoms and also the SCM hypertrophy had vanished. Conclusion: Task-specific focal dystonia, occurs mainly in highly skilled manual tasks, and are usually related to occupation (piano players, architects, writers, shoemakers). Rare cases were described in oromandibular region (e.g., embrochure dystonia in brass players). The present case illustrates an unusual and to the best of our knowledge, never before reported case of task specific cervical dystonia elicited by the singing act.
1.259 Frontalis, corrugator and procerus dystonia: A blepharospasm variant? G. Fabiani1° , H. Teive Brazil
1 Curitiba,
Objective: Our objective is to report on an unusual presentation of dystonia in a 46-year-old male, affecting the frontalis, corrugator and procerus muscles. Method: We report on an unusual presentation of dystonia, affecting the frontalis, corrugator and procerus muscles, without any involvement of orbicularis oculi muscles. The patient is a 46-year-old male. Neurological exam disclosed involuntary movements of frontalis, corrugator and procerus muscles, bilaterally, without any compromising of orbicularis oculi muscles. He also presented oculogyric deviations (up). Results: Laboratory tests and cranial CT scan were normal. We decided on for intramuscular botulinum toxin type A (BT-A; Dysport) treatment. A total dose of 200/IU was injected in the frontalis, corrugator and procerus muscles. After 3 weeks he underwent a new neurological examination and the dystonic symptoms had vanished. Conclusion: The hallmark of the blepharospasm is the involvement of the orbicularis oculi muscles. For the diagnosis this feature is essential. Nonetheless the unusual features the patient presented, it was obvious to us that he presented a cranial dystonia with sole involvement of frontalis, corrugator and procerus muscles bilaterally. A total dose of 200/IU of botulinum toxin type A (BT-A; Dysport) was injected in the frontalis, corrugator and procerus muscles. After 3 weeks he underwent a new neurological examination and the dystonic symptoms had vanished.
1.260 Venlafaxine-induced bruxism H. Alonso Navarro1° , M. Mart´ın-Prieto, J.J. Ruiz-Ezquerro, F.J. Jim´enezJim´enez 1 Salamanca, Spain Objective: Bruxism is considered to be a focal dystonia which usually improved with botulinum toxin infiltrations in masticatory muscles. We report a patient who developed a typical bruxism during a treatment with venlafaxine, which disappeared after withdrawal of this drug. Method: A 62 years-old male patient without previous personal or familial history of movement disorders was evaluated because of a severe bruxism of 6 months of evolution. Results: Blood count, routine biochemistry, copper metabolism studies, thyroid hormones levels and serological studies for syphilis and Brucella were normal or negative. Brain MR imaging was normal. He was under therapy with venlafaxine 150 mg/day since the two weeks previous to the clinical onset of bruxism because of “depression” and “anxiety”. Two essays with botulinum toxin infiltrations in temporalis and masseter muscles (25 IU each) had been unsuccessful. Following venlafaxine withdrawal, bruxism improved gradually, and disappeared 2 months later. Conclusion: The temporal relation between the development of bruxism, together with its disappearance following venlafaxine-withdrawal, suggests a possible cause–effect relationship of bruxism with venlafaxine therapy. Venlafaxine should be considered as a possible cause of drug inducedbruxism.