This paper deals with signs, symptoms and course in spasmodic torticollis (ST). Two hundred and f... more This paper deals with signs, symptoms and course in spasmodic torticollis (ST). Two hundred and fifty-six patients were included in the study, 59.3% women, 40.7% men. The mean age was 49.1 years. Rotating torticollis out-numbered latero- and antero-retrocollis. A family history of ST occurred in 3.1% of the total sample. First degree relatives were affected in 2.3%. Thirty-four per cent of the patients had additional dystonic symptoms. Most frequently these affected the upper extremities (13%), and less often the legs. Of the patients 19.1% had experienced a period of complete remission. The correlations between the severity of the signs and the neurological symptoms are surprisingly weak.
Evaluate changes in active and passive function with onabotulinumtoxinA + standard of care within... more Evaluate changes in active and passive function with onabotulinumtoxinA + standard of care within goal-oriented rehabilitation programmes in adults with focal post-stroke spasticity. Prospective, 24-week double-blind study with an open-label extension. Subjects were randomized to onabotulinumtoxinA + standard of care or placebo + standard of care, at baseline and at 12 weeks, if judged appropriate, with follow-up to 52 weeks. The primary endpoint was the number of patients achieving their principal active functional goal at 24 weeks (or 10 weeks after an optional second injection). Secondary endpoints included achievement of a different active or a passive goal at this timepoint. The intent-to-treat population comprised 273 patients. The proportion of patients achieving their principal active functional goal and secondary active functional goal with onabotulinumtoxinA + standard of care was not statistically different from placebo + standard of care. Significantly more patients achi...
Journal of neurology, neurosurgery, and psychiatry, 2000
To define a safe and effective dose of Dysport for treating hip adductor spasticity. Patients wit... more To define a safe and effective dose of Dysport for treating hip adductor spasticity. Patients with definite or probable multiple sclerosis, and disabling spasticity affecting the hip adductor muscles of both legs, were randomised to one of four treatment groups. Dysport (500, 1000, or 1500 Units), or placebo was administered by intramuscular injection to these muscles. Patients were assessed at entry, and 2, 4 (primary analysis time-point), 8, and 12 weeks post-treatment. A total of 74 patients were recruited. Treatment groups were generally well matched at entry. The primary efficacy variables-passive hip abduction and distance between the knees-improved for all groups. The improvement in distance between the knees for the 1500 Unit group was significantly greater than placebo (p = 0.02). Spasm frequency was reduced in all groups, but muscle tone was reduced in the Dysport groups only. Pain was reduced in all groups, but improvements in hygiene scores were evident only in the 1000 ...
Dystonia is a syndrome characterized by sustained muscle contractions frequently causing twisting... more Dystonia is a syndrome characterized by sustained muscle contractions frequently causing twisting and repetitive movements or abnormal posture. For diagnosis, prognosis and therapy, it is useful to classify dystonia with regard to types of abnormal movements present, their mode of activation and topographical distribution taking into account age of onset, and etiology. The majority of cases are idiopathic, or primary dystonias, while in a minority environmental, structural, or metabolic causes can be identified. Primary dystonias can be familial or sporadic. The most important neurophysiological phenomenon in dystonia is pathological cocontraction of antagonistic muscles, while there is no consistent neuropathological abnormality in idiopathic dystonia. Causal therapies for dystonia are only possible in a few symptomatic forms (M. Wilson, Segawa-syndrome). As a rule, treatment has to be symptomatic but results of systemic pharmacotherapies remain disappointing. For adult onset focal...
Eleven patients with chronic spastic hemiparesis were treated with biosignal processing (BSP), a ... more Eleven patients with chronic spastic hemiparesis were treated with biosignal processing (BSP), a modified biofeedback method in which the patient practices useful tasks, not isolated individual movements. A surface EMG measures sequential movements, and an acoustic signal monitors muscle exertion. The patient first learns how the signal develops by using the unaffected limb. Then the patient tries to reproduce the course of the signal in the paretic limb. Patients received 12 to 30 treatments for upper and/or lower extremities. We measured maximum strength as expressed through the EMG signal; ability to perform the trained action as measured by specific grading systems; and general increase in movement competence during a Bobath movement test. Ten patients showed improved strength; four made marked progress in the performance of specific tasks with the upper extremity, as did four with the lower extremity. Four patients in each group improved in general movement. We recommend the in...
Idiopathic spasmodic torticollis (IST) is one of the most frequent dystonic movement disorders. I... more Idiopathic spasmodic torticollis (IST) is one of the most frequent dystonic movement disorders. Its classification as a focal dystonia, as well as its treatment with botulinum toxin resulted in groups of patients being regularly seen by neurologic specialists. In a multicentre study, we investigated psychosocial changes, coping and psychopathology, and their interrelations with signs, symptoms and course. 256 patients were included in the study (59.3% women, 40.7% men). The mean age was 49.1 years. Rotating torticollis occurred more often than latero-retrocollis and antero-retrocollis. A family history of IST was seen in 3.1% of the total sample. 34% of the patients had additional dystonic symptoms. Most frequently, these affected the upper extremities (13%), less often the legs. 19.1% of the patients had experienced a period of complete remission. The General Symptom Index of the SCL 90-R in 27% of the patients ranged above the double standard deviation of the normal controls, indi...
Injections with botulinum toxin type A (BTX) are considered the first-line treatment for spasmodi... more Injections with botulinum toxin type A (BTX) are considered the first-line treatment for spasmodic torticollis (ST), blepharospasm (BL) and hemifacial spasm (HFS). Because BTX brings only temporary and partial relief, patients frequently try other additional therapies to minimize their symptoms. The subjective rating of all therapies ever tried by patients with ST, BL and HFS was evaluated by using a simple questionnaire. Two hundred questionnaires were considered (112 TS, 54 BL, 34 HFS). BTX was rated subjectively the best therapy in all three diagnostic groups (median: 2 = good effect). Despite Citalopram and physiotherapy (median: 3 = average effect), all other therapies were rated with a median of > or = 4 (= minimal effect). Patients with ST tried 7.7, patients with BL 2.4 and patients with HFS 2.6 different types of therapy. In conclusion, BTX is the most effective treatment for patients with ST, BL and HFS, as rated subjectively. Further evaluation of therapies additional ...
Idiopathic cervical dystonia, like other adult-onset focal dystonias, has been notoriously diffic... more Idiopathic cervical dystonia, like other adult-onset focal dystonias, has been notoriously difficult to treat. Multiple approaches, including systemic drug treatment with anticholinergics, antidopaminergics, anticonvulsants, muscle relaxants and many other drugs as well as physiotherapy and psychotherapy, usually lead to only temporary amelioration in a minority of patients. Surgical selective EMG-controlled denervation of dystonic neck muscles produces better and, in the hands of some, lasting improvement. However, the procedure is invasive and as such less well accepted by patients. Local injections of botulinum toxin are strikingly successful in improving postural deviation and pain in about 80% of patients. They can be made on an outpatient basis and appear to be safe even over many repeat sessions. Dysphagia is potentially the most serious side-effect but can be decreased in incidence and severity by injecting lower doses, particularly into the sternomastoid. Major drawbacks at...
Local injections with Botulinum toxin A (BtxA) are safe and effective in the treatment of focal d... more Local injections with Botulinum toxin A (BtxA) are safe and effective in the treatment of focal dystonia. In cervical dystonia and blepharospasm, BtxA injections have become the treatment of choice. However, good results have also been reported with oromandibular dystonia, spasmodic dysphonia and writer's cramp. In cervical dystonia, muscles for injection are selected by clinical presentation or in complex forms with EMG guidance. Several studies have shown that 500 units Dysport are safe and effective in the treatment of cervical dystonia. In blepharospasm, injections are performed in the periorbital part of the orbicularis oculi muscle with good results for 12-14 weeks. The most frequently employed starting dose is 120 units Dysport per eye, divided in three periorbital injection sites. In case of levator inhibition, the pretarsal part of the orbicularis oculi muscle should be injected in a lower dose. EMG guidance is not necessary. By contrast, BtxA treatment of spasmodic dys...
We sought to define an effective and safe dose of botulinum toxin type A (Dysport) for the treatm... more We sought to define an effective and safe dose of botulinum toxin type A (Dysport) for the treatment of upper limb muscle spasticity due to stroke. This was a prospective, randomized, double-blind, placebo-controlled, dose-ranging study. Patients received either a placebo or 1 of 3 doses of Dysport (500, 1000, 1500 U) into 5 muscles of the affected arm. Efficacy was assessed periodically by the Modified Ashworth Scale and a battery of functional outcome measures. Eighty-three patients were recruited, and 82 completed the study. The 4 study groups were comparable at baseline with respect to their demographic characteristics and severity of spasticity. All doses of Dysport studied showed a significant reduction from baseline of muscle tone compared with placebo. However, the effect on functional disability was not statistically significant and was best at a dose of 1000 U. There were no statistically significant differences between the groups in the incidence of adverse events. The pr...
The aim of the study was to evaluate and compare health-related quality of life (HR-QoL) and depr... more The aim of the study was to evaluate and compare health-related quality of life (HR-QoL) and depression in essential blepharospasm (BSP) and idiopathic cervical dystonia (CD), to identify the clinical and demographic factors associated with poor HR-QoL in both disorders and to analyse the effect of Botulinum Toxin A (BtxA) therapy. Two hundred-twenty consecutive patients with BSP (N = 89, 62 % women, mean age 64 years, mean disease duration 7 years) and CD (N = 131, 64 % women, mean age 53 years, mean disease duration 8 years) recruited from routine referrals to eight Austrian dystonia clinics were included. HR-QoL was measured by the Short Form 36 (SF-36) and depression by the Beck Depression Inventory (BDI). At baseline, patients with CD and BSP scored significantly worse in all eight SF-36 domains compared with an age-matched community sample. In addition, 47 % of patients with CD and 37 % of those with BSP were depressed. Women with BSP scored significantly lower in all SF-36 domains and were more depressed than male patients. In contrast, there was no significant effect of gender on HR-QoL and depression in CD. Neck pain had a significant impact on all SF-36 domains and represented the main determinant of depression in CD. Although BtxA therapy resulted in a significant improvement of clinical symptoms in BSP and CD, HR-QoL did not improve in BSP and only two of the eight SF-36 domains improved significantly in patients with CD. The present study for the first time demonstrated that BSP has a substantial impact on health status emphasizing the need for psychological support with interventions aimed at treating depression in these patients. Our results provide further evidence for the profound impact of CD on HR-QoL and indicate the importance of an adequate management of neck pain in addition to reducing the severity of dystonia in CD. The mismatch between objective BtxA derived improvement of dystonia and lack of change of HR-QoL as determined by the SF-36 illustrates the need for optimized disease specific quality of life rating scales in patients with craniocervical dystonia.
Spasticity is one of the major causes of functional impairment in adults with lesions of the cent... more Spasticity is one of the major causes of functional impairment in adults with lesions of the central nervous system. For instance, approximately 30% of post-stroke patients suffer from different degrees of spasticity with possible consecutive impairments. Numerous studies or meta-analyses showed that local injections of botulinum toxin in spastic muscles lead to dose-dependent reduction in muscle tone and improvement of passive movements (e. g. facilitated care), especially following repeated injections.However, country-specific regulations and patient-remote administration in German health care often do not allow adequate provision of this therapy. Thus, the present consensus statement based on the EBM analyses of the published international literature tries to highlight recent advances and the standard in the field of local spasticity treatment, aiming to facilitate communication between the decision makers and German reimbursement institutions in health care. Prior to initiation ...
This paper deals with signs, symptoms and course in spasmodic torticollis (ST). Two hundred and f... more This paper deals with signs, symptoms and course in spasmodic torticollis (ST). Two hundred and fifty-six patients were included in the study, 59.3% women, 40.7% men. The mean age was 49.1 years. Rotating torticollis out-numbered latero- and antero-retrocollis. A family history of ST occurred in 3.1% of the total sample. First degree relatives were affected in 2.3%. Thirty-four per cent of the patients had additional dystonic symptoms. Most frequently these affected the upper extremities (13%), and less often the legs. Of the patients 19.1% had experienced a period of complete remission. The correlations between the severity of the signs and the neurological symptoms are surprisingly weak.
Evaluate changes in active and passive function with onabotulinumtoxinA + standard of care within... more Evaluate changes in active and passive function with onabotulinumtoxinA + standard of care within goal-oriented rehabilitation programmes in adults with focal post-stroke spasticity. Prospective, 24-week double-blind study with an open-label extension. Subjects were randomized to onabotulinumtoxinA + standard of care or placebo + standard of care, at baseline and at 12 weeks, if judged appropriate, with follow-up to 52 weeks. The primary endpoint was the number of patients achieving their principal active functional goal at 24 weeks (or 10 weeks after an optional second injection). Secondary endpoints included achievement of a different active or a passive goal at this timepoint. The intent-to-treat population comprised 273 patients. The proportion of patients achieving their principal active functional goal and secondary active functional goal with onabotulinumtoxinA + standard of care was not statistically different from placebo + standard of care. Significantly more patients achi...
Journal of neurology, neurosurgery, and psychiatry, 2000
To define a safe and effective dose of Dysport for treating hip adductor spasticity. Patients wit... more To define a safe and effective dose of Dysport for treating hip adductor spasticity. Patients with definite or probable multiple sclerosis, and disabling spasticity affecting the hip adductor muscles of both legs, were randomised to one of four treatment groups. Dysport (500, 1000, or 1500 Units), or placebo was administered by intramuscular injection to these muscles. Patients were assessed at entry, and 2, 4 (primary analysis time-point), 8, and 12 weeks post-treatment. A total of 74 patients were recruited. Treatment groups were generally well matched at entry. The primary efficacy variables-passive hip abduction and distance between the knees-improved for all groups. The improvement in distance between the knees for the 1500 Unit group was significantly greater than placebo (p = 0.02). Spasm frequency was reduced in all groups, but muscle tone was reduced in the Dysport groups only. Pain was reduced in all groups, but improvements in hygiene scores were evident only in the 1000 ...
Dystonia is a syndrome characterized by sustained muscle contractions frequently causing twisting... more Dystonia is a syndrome characterized by sustained muscle contractions frequently causing twisting and repetitive movements or abnormal posture. For diagnosis, prognosis and therapy, it is useful to classify dystonia with regard to types of abnormal movements present, their mode of activation and topographical distribution taking into account age of onset, and etiology. The majority of cases are idiopathic, or primary dystonias, while in a minority environmental, structural, or metabolic causes can be identified. Primary dystonias can be familial or sporadic. The most important neurophysiological phenomenon in dystonia is pathological cocontraction of antagonistic muscles, while there is no consistent neuropathological abnormality in idiopathic dystonia. Causal therapies for dystonia are only possible in a few symptomatic forms (M. Wilson, Segawa-syndrome). As a rule, treatment has to be symptomatic but results of systemic pharmacotherapies remain disappointing. For adult onset focal...
Eleven patients with chronic spastic hemiparesis were treated with biosignal processing (BSP), a ... more Eleven patients with chronic spastic hemiparesis were treated with biosignal processing (BSP), a modified biofeedback method in which the patient practices useful tasks, not isolated individual movements. A surface EMG measures sequential movements, and an acoustic signal monitors muscle exertion. The patient first learns how the signal develops by using the unaffected limb. Then the patient tries to reproduce the course of the signal in the paretic limb. Patients received 12 to 30 treatments for upper and/or lower extremities. We measured maximum strength as expressed through the EMG signal; ability to perform the trained action as measured by specific grading systems; and general increase in movement competence during a Bobath movement test. Ten patients showed improved strength; four made marked progress in the performance of specific tasks with the upper extremity, as did four with the lower extremity. Four patients in each group improved in general movement. We recommend the in...
Idiopathic spasmodic torticollis (IST) is one of the most frequent dystonic movement disorders. I... more Idiopathic spasmodic torticollis (IST) is one of the most frequent dystonic movement disorders. Its classification as a focal dystonia, as well as its treatment with botulinum toxin resulted in groups of patients being regularly seen by neurologic specialists. In a multicentre study, we investigated psychosocial changes, coping and psychopathology, and their interrelations with signs, symptoms and course. 256 patients were included in the study (59.3% women, 40.7% men). The mean age was 49.1 years. Rotating torticollis occurred more often than latero-retrocollis and antero-retrocollis. A family history of IST was seen in 3.1% of the total sample. 34% of the patients had additional dystonic symptoms. Most frequently, these affected the upper extremities (13%), less often the legs. 19.1% of the patients had experienced a period of complete remission. The General Symptom Index of the SCL 90-R in 27% of the patients ranged above the double standard deviation of the normal controls, indi...
Injections with botulinum toxin type A (BTX) are considered the first-line treatment for spasmodi... more Injections with botulinum toxin type A (BTX) are considered the first-line treatment for spasmodic torticollis (ST), blepharospasm (BL) and hemifacial spasm (HFS). Because BTX brings only temporary and partial relief, patients frequently try other additional therapies to minimize their symptoms. The subjective rating of all therapies ever tried by patients with ST, BL and HFS was evaluated by using a simple questionnaire. Two hundred questionnaires were considered (112 TS, 54 BL, 34 HFS). BTX was rated subjectively the best therapy in all three diagnostic groups (median: 2 = good effect). Despite Citalopram and physiotherapy (median: 3 = average effect), all other therapies were rated with a median of > or = 4 (= minimal effect). Patients with ST tried 7.7, patients with BL 2.4 and patients with HFS 2.6 different types of therapy. In conclusion, BTX is the most effective treatment for patients with ST, BL and HFS, as rated subjectively. Further evaluation of therapies additional ...
Idiopathic cervical dystonia, like other adult-onset focal dystonias, has been notoriously diffic... more Idiopathic cervical dystonia, like other adult-onset focal dystonias, has been notoriously difficult to treat. Multiple approaches, including systemic drug treatment with anticholinergics, antidopaminergics, anticonvulsants, muscle relaxants and many other drugs as well as physiotherapy and psychotherapy, usually lead to only temporary amelioration in a minority of patients. Surgical selective EMG-controlled denervation of dystonic neck muscles produces better and, in the hands of some, lasting improvement. However, the procedure is invasive and as such less well accepted by patients. Local injections of botulinum toxin are strikingly successful in improving postural deviation and pain in about 80% of patients. They can be made on an outpatient basis and appear to be safe even over many repeat sessions. Dysphagia is potentially the most serious side-effect but can be decreased in incidence and severity by injecting lower doses, particularly into the sternomastoid. Major drawbacks at...
Local injections with Botulinum toxin A (BtxA) are safe and effective in the treatment of focal d... more Local injections with Botulinum toxin A (BtxA) are safe and effective in the treatment of focal dystonia. In cervical dystonia and blepharospasm, BtxA injections have become the treatment of choice. However, good results have also been reported with oromandibular dystonia, spasmodic dysphonia and writer's cramp. In cervical dystonia, muscles for injection are selected by clinical presentation or in complex forms with EMG guidance. Several studies have shown that 500 units Dysport are safe and effective in the treatment of cervical dystonia. In blepharospasm, injections are performed in the periorbital part of the orbicularis oculi muscle with good results for 12-14 weeks. The most frequently employed starting dose is 120 units Dysport per eye, divided in three periorbital injection sites. In case of levator inhibition, the pretarsal part of the orbicularis oculi muscle should be injected in a lower dose. EMG guidance is not necessary. By contrast, BtxA treatment of spasmodic dys...
We sought to define an effective and safe dose of botulinum toxin type A (Dysport) for the treatm... more We sought to define an effective and safe dose of botulinum toxin type A (Dysport) for the treatment of upper limb muscle spasticity due to stroke. This was a prospective, randomized, double-blind, placebo-controlled, dose-ranging study. Patients received either a placebo or 1 of 3 doses of Dysport (500, 1000, 1500 U) into 5 muscles of the affected arm. Efficacy was assessed periodically by the Modified Ashworth Scale and a battery of functional outcome measures. Eighty-three patients were recruited, and 82 completed the study. The 4 study groups were comparable at baseline with respect to their demographic characteristics and severity of spasticity. All doses of Dysport studied showed a significant reduction from baseline of muscle tone compared with placebo. However, the effect on functional disability was not statistically significant and was best at a dose of 1000 U. There were no statistically significant differences between the groups in the incidence of adverse events. The pr...
The aim of the study was to evaluate and compare health-related quality of life (HR-QoL) and depr... more The aim of the study was to evaluate and compare health-related quality of life (HR-QoL) and depression in essential blepharospasm (BSP) and idiopathic cervical dystonia (CD), to identify the clinical and demographic factors associated with poor HR-QoL in both disorders and to analyse the effect of Botulinum Toxin A (BtxA) therapy. Two hundred-twenty consecutive patients with BSP (N = 89, 62 % women, mean age 64 years, mean disease duration 7 years) and CD (N = 131, 64 % women, mean age 53 years, mean disease duration 8 years) recruited from routine referrals to eight Austrian dystonia clinics were included. HR-QoL was measured by the Short Form 36 (SF-36) and depression by the Beck Depression Inventory (BDI). At baseline, patients with CD and BSP scored significantly worse in all eight SF-36 domains compared with an age-matched community sample. In addition, 47 % of patients with CD and 37 % of those with BSP were depressed. Women with BSP scored significantly lower in all SF-36 domains and were more depressed than male patients. In contrast, there was no significant effect of gender on HR-QoL and depression in CD. Neck pain had a significant impact on all SF-36 domains and represented the main determinant of depression in CD. Although BtxA therapy resulted in a significant improvement of clinical symptoms in BSP and CD, HR-QoL did not improve in BSP and only two of the eight SF-36 domains improved significantly in patients with CD. The present study for the first time demonstrated that BSP has a substantial impact on health status emphasizing the need for psychological support with interventions aimed at treating depression in these patients. Our results provide further evidence for the profound impact of CD on HR-QoL and indicate the importance of an adequate management of neck pain in addition to reducing the severity of dystonia in CD. The mismatch between objective BtxA derived improvement of dystonia and lack of change of HR-QoL as determined by the SF-36 illustrates the need for optimized disease specific quality of life rating scales in patients with craniocervical dystonia.
Spasticity is one of the major causes of functional impairment in adults with lesions of the cent... more Spasticity is one of the major causes of functional impairment in adults with lesions of the central nervous system. For instance, approximately 30% of post-stroke patients suffer from different degrees of spasticity with possible consecutive impairments. Numerous studies or meta-analyses showed that local injections of botulinum toxin in spastic muscles lead to dose-dependent reduction in muscle tone and improvement of passive movements (e. g. facilitated care), especially following repeated injections.However, country-specific regulations and patient-remote administration in German health care often do not allow adequate provision of this therapy. Thus, the present consensus statement based on the EBM analyses of the published international literature tries to highlight recent advances and the standard in the field of local spasticity treatment, aiming to facilitate communication between the decision makers and German reimbursement institutions in health care. Prior to initiation ...
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Papers by Jörg Wissel