Preoperative treatment with botulinum A toxin in patients with cervical disk herniation secondary to dystonic cerebral palsy.
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Biomedical subjects
Publications and source records attributed to M Basciani.
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OBJECTIVES: Little is known about factors influencing the spread of blepharospasm to other body parts. An investigation was carried out to deterrmine whether demographic features (sex, age at blepharospasm onset), putative risk, or protective factors for blepharospasm (family history of dystonia or tremor, previous head or face trauma with loss of consciousness, ocular diseases, and cigarette smoking), age related diseases (diabetes, hypertension), edentulousness, and neck or trunk trauma preceding the onset of blepharospasm could distinguish patients with blepharospasm who had spread of dystonia from those who did not. METHODS: 159 outpatients presenting initially with blepharospasm were selected in 16 Italian Institutions. There were 104 patients with focal blepharospasm (mean duration of disease 5.3 (SD 1.9) years) and 55 patients in whom segmental or multifocal dystonia developed (mainly in the cranial cervical area) 1.5 (1.2) years after the onset of blepharospasm. Information was obtained from a standardised questionnaire administered by medical interviewers. A Cox regression model was used to examine the relation between the investigated variables and spread. RESULTS: Previous head or face trauma with loss of consciousness, age at the onset of blepharospasm, and female sex were independently associated with an increased risk of spread. A significant association was not found between spread of dystonia and previous ocular diseases, hypertension, diabetes, neck or trunk trauma, edentulousness, cigarette smoking, and family history of dystonia or tremor. An unsatisfactory study power negatively influenced the validity and accuracy of the negative findings relative to diabetes, neck or trunk trauma, and cigarette smoking. CONCLUSIONS: The results of this exploratory study confirm that patients presenting initially with blepharospasm are most likely to experience some spread of dystonia within a few years of the onset of blepharospasm and suggest that head or face trauma with loss of consciousness preceding the onset, age at onset, and female sex may be relevant to spread. The suggested association between edentulousness and cranial cervical dystonia may be apparent because of the confounding effect of both age at onset and head or face trauma with loss of consciousness. The lack of influence of family history of dystonia on spread is consistent with previous findings indicating that the inheritance pattern is the same for focal and segmental blepharospasm.
We investigated the long-term efficacy and safety of intrasphincteric injections of botulinum toxin (100 U) in 57 patients with esophageal achalasia. One month after treatment, 50 patients had improved (88%); both symptom score and LES pressure were significantly reduced (P < 0.001). After a mean follow-up of 24+/-15 months (range 6-48), 43 patients (75%) are still in remission, although repeat injections of toxin were needed to achieve a stable effect on symptoms.
The detrusor-sphincter dyssynergia (DSD) is a common and significant problem for patients with spinal cord lesions. If not treated, DSD can lead to severe and potentially lethal complications (urinary tract infections and renal damage). BTX inhibits acetylcholine release at the neuromuscular junction, producing muscle chemical denervation in the site of injection. The aim of this preliminary study was to test the safety and the effects of BTX injection in the spastic urethral rhabdosphincter in patients with DSD. Five patients (3 M, 2 F; mean age 43 years, range 22-56) with DSD entered the study. Videourodynamic parameters were controlled before BTX injection at 10 days, at 3 and 6 months after infiltration. The aim of this preliminary study was to test the safety and the effects of BTX injection in the spastic urethral rhabdosphincter in patients with DSD. The post void residual urine volume, the maximum pressure of emptying and the maximum pressure of urethral closure are reported in Tab. II-III-IV. Patients reported subjective improvement of bladder emptying and improved quality of life. No adverse effects related to pharmacological activity of BTX or to infiltration procedures were observed.
BACKGROUND & AIMS: Intrasphincteric injection of botulinum toxin has been suggested as an alternative treatment modality in esophageal achalasia. A controlled trial comparing botulinum toxin, placebo, and pneumatic dilation is reported. METHODS: Sixteen patients received random intrasphincteric injections of either botulinum toxin or saline. The efficacy of treatment was assessed by symptom score, esophageal manometry, and scintigraphy. In case of failure, pneumatic dilation was performed. RESULTS: One month after injection, symptoms had improved in all patients treated with botulinum toxin (symptom score, 0.9 +/- 0.6 vs. 5.5 +/- 1.4; P < 0.02). In the placebo group, symptoms were unchanged in all patients, who were all dilated. Lower esophageal sphincter pressure decreased by 49% after treatment with botulinum toxin (P < 0.03) and by 72% after dilation (P < 0.01). Similarly, esophageal retention decreased by 47% after treatment with botulinum toxin (P < 0.02) and by 59% after dilation (P < 0.02). No significant difference in symptom score and esophageal function test results was found between patients treated with botulinum toxin injections and those undergoing dilation. However, 7 of the 8 patients in the botulinum toxin group required a second injection because of recurrent dysphagia. CONCLUSIONS: Treatment of achalasia with botulinum toxin was as effective as pneumatic dilation in relieving symptoms and improving esophageal function. The effect of the first injection was temporary, but the effect of the second injection lasted longer.
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Median nerve somatosensory evoked potentials (SEP) were recorded in 7 Cynomolgus monkeys, before and after the administration of N-Methyl 1,4 Phenyl 1,2,3,6 tetrahydropiridine (MPTP), a neurotoxin which induces a parkinsonian syndrome in primates. Following MPTP administration, the amplitude of the negative component recorded at 15 ms over the frontal derivations (N15) decreased by 70% or more. This amplitude reduction was not modified by administration of dopamine precursors. These findings shed light on recent findings in human parkinsonian patients.
We report a case of myoclonus from overnight exposure to methyl bromide. Myoclonus was either spontaneous or induced by somatosensory stimulation or voluntary movements, multifocal and sometimes generalized. Median SEP showed normal size P14-N20, but giant parietal P25, N33 and frontal P22-N30 waves. Back-averaging showed a biphasic EEG spike of maximal amplitude at the central region contralateral to the corresponding myoclonic jerk recorded from abductor pollicis brevis and preceding it by a short interval consistent with conduction in corticospinal pathways. Long latency reflexes from cutaneous and mixed nerve stimulation were enhanced. The above electrophysiological findings suggest that myoclonus following methyl bromide poisoning belongs to the cortical reflex myoclonus category.
Two patients with legs tremor present on standing, but none on walking or sitting, are reported. Tremor was not exclusive or orthostatism and was also evoked by strong tonic contraction of leg muscles. Synchronous EMG bursts were recorded in antagonistic muscle groups at 8-10 Hz in the first patient and at 16 Hz in the second. EMG activity was synchronous in corresponding muscles of both legs. The occurrence of EMG activity was not influenced by stimulation of nerve afferent fibers. We suggest that this movement disorder may be an exaggeration of physiological tremor due to synchronization of motor units by spontaneous oscillations in central structures.
A case is reported in which retrobulbar neuritis preceded Guillain-Barré syndrome by 4 weeks. The visual evoked potential latencies were prolonged. After peripheral nervous system signs had cleared, median and peroneal somatosensory evoked potentials showed prolonged cervical N13, scalp N20 and L3-scalp conduction times.
Three patients with autosomal dominant Strümpell's familial spastic paraplegia (SFSP) were evaluated by means of somatosensory evoked potentials (SEPs) from upper and lower limb and determination of sural nerve conduction velocity. Findings of normal sural nerve conduction but reduced amplitude and poor definition of SEPs with normal latencies on peroneal nerve stimulation support a pattern of central nervous system degeneration characterized by a selective involvement of centrally directed axons within the gracile fasciculi.
VEPs to different spatial frequencies (1,2,4 cycles per degree) and different stimulus configurations (vertical, horizontal gratings and checkerboard) were recorded in 47 Multiple Sclerosis patients and a corresponding group of control subjects. Serial recordings were performed in periods of 1-6 years. We show that the highest diagnostic yield in MS is for VEPs of higher spatial frequency than 1 cpd, and that vertical gratings give reliable results when high spatial frequencies are used, whereas checkerboard stimuli elicit consistent VEPs only when the check size is 30'-15' of arc of visual angle. The use of fine vertical grating patterns dissipates controversies dependent on "W" shaped VEPs.
VEPs were recorded with three different spatial frequencies of stimulation in patients affected by idiopathic Parkinsonism and by Parkinsonian syndromes. The detection of VEP abnormalities in Parkinson's disease was dependent on the spatial frequency of the visual stimulus (a vertical square wave grating). The VEP latency was normal in Parkinsonian syndrome patients (except in one patient affected by familial Parkinsonism). Dopamine precursor therapy differently reduced the VEP latency, depending on the spatial frequency of the visual stimulus. These findings suggest that the dopaminergic mechanism involved in the generation of VEP delays is sensitive to stimulus spatial frequency. The study of VEPs before and after the administration of haloperidol confirmed this hypothesis. VEP latency did not correlate with the major clinical symptoms of Parkinson's disease and could not predict the results of chronic dopaminergic therapy.
Two patients with a neurologic syndrome of vitamin B12 deficiency and delayed Visual Evoked Potentials are reported. VEP latencies returned to normal values from 90 to 110 days following parenteral treatment with cyanocobalamin.
Peripheral (cauda-lumbar, wrist-Erb, Erb-cervical) and central (cauda-vertex, cervical-scalp) nervous impulse propagation velocities and times to peroneal and median nerve stimulation were investigated in 34 patients suffering from definite (17 cases), probable (6 cases) and possible (11 cases) forms of multiple sclerosis (MS). In 6 cases short- and intermediate-latency scalp somatosensory evoked potentials to peroneal nerve stimulation were recorded with 'open' (1-5000 Hz, -6 dB) bandpass filters and subsequently digitally filtered through a 'narrow' bandpass (200-5000 Hz, -6 dB). The lumbar response was abnormal in 2.95% of legs, while the Erb response was always within normal limits. The cauda-vertex conduction was altered in 75% of the examined limbs (86.2% definite, 58.3% probable, 63.6% possible MS). Absent scalp responses to peroneal stimulation were often encountered during narrow bandpass recording (54.9%), while a slowed central conduction was less frequent (33.3%). Scalp responses when recorded with open bandpass were always identifiable, being delayed in 3 out of 6 cases. In 5 of these the short-latency wavelets were either absent or showed a prolonged interpeak time even when open filter records were normal. Median nerve SEPs were altered in 60.3% of cases, more frequently because of a delayed scalp response or of a prolonged cervical-scalp conduction time than because of an absent cervical or scalp response. When peroneal and median nerve data were considered together, the rate of abnormality rose to 88.2% of patients. Due to their length, afferent pathways from the lower limb might suffer from a loss of high frequency impulse coding as an early sign of defective impulse propagation.
Brain-stem auditory evoked responses have been investigated in 28 patients suffering from chronic renal failure (17 on diet, 11 on chronic dialysis) during monaural stimulation with 70 dB HL clicks. These responses were abnormal in 32.15% of cases. In 13 out of 15 years with altered BAERs a normal response morphology was present, with progressively more significant latency delays involving all peaks following wave I, while in the remaining two ears some of the components always found in healthy controls were not identifiable. The most prominent abnormality in patients with symptoms referable to peripheral neuropathy was the presence of a prolonged I-II interpeak latency, while in those without symptomatology the I-V and III-V interpeak times and the peak V latency were the most strikingly altered parameters. The incidence of altered BAERs was higher in the group of patients treated with diet than in the dialysed group. When BAERs were followed-up in the hours preceding and following the first dialysis in two patients not included in the previous group, the I-V interpeak time significantly decreased after 26 h from the end of dialysis.
Bit mapped color imaging of SEPs was recorded from 19 derivations in 11 healthy volunteers after electrical stimulation of the median nerve at the wrist, index finger digital nerve stimulation, and mechanical stimulation of the index fingertips by an electromechanically driven vibrating thin metallic plate. The latencies of SEP components increased for the various stimulation modalities, being shortest after median nerve stimulation at the wrist and longest after mechanical stimulation of the index fingertips. The scalp distribution of SEPs to mechanical stimuli was, however, the same as other SEPs, independently of the stimulation employed, and components corresponding to N20 and P22 were recorded only contralaterally to the stimulated side.
BACKGROUND AND PURPOSE: Electrical stimulation, physical therapy and occupational therapy remain the main treatment for children with upper brachial plexus palsy (UBPP), when surgery has been excluded. A pilot study was undertaken to investigate whether botulinum toxin type A (BoNT-A) and plaster casting, as adjunct to the physical therapy, decreased muscle contracture and improved the position and function of the impaired arm. METHOD: Twenty-two children (mean age 5.6 +/- 3.4 years) with mild UBPP who previously underwent serial cast treatment, unsuccessfully, were enrolled. Neurological impairment and functional status were quantified using Medical Research Council (MRC) and Mallet scales and the Nine-Hole Peg Test (NHPT). Elbow extension was measured using a goniometer. Biceps brachii, brachialis, pronator teres and pectoralis major muscles were injected with 22 units kg(-1) BoNT-A (Dysport, Ipsen). After injection, the treated arm was fixed with a plaster cast and progressively lengthened over 14 days. The cast was maintained for 30 days. Assessments of elbow extension, MRC, Mallet Scale and NHPT were made at baseline, 3, 6 and 12 months. RESULTS: After BoNT-A injection, children had significant improvement of active elbow extension (15.5 degrees +/- 17.1 at 12 months after injection, compared with 42.0 degrees +/- 10.4 at baseline; p < 0.001). NPHT scores improved significantly over the 12 months (51.1 +/- 21.8 seconds compared with 56.7 +/- 19.3 seconds at baseline, p < 0.01). MRC and Mallet scale scores of the paretic muscles were unchanged. CONCLUSION: The children showed a reduction in muscular contracture and improvements of the arm position and elbow extension. The data support the use of BoNT-A and plaster casting as an adjunct to physical therapy, in the treatment of children with mild UBPP.