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Botulinum A toxin (Oculinum) in ophthalmology.

Botulinum A toxin has been used to treat strabismus and a variety of spasmodic neuromuscular diseases. Botulinum toxin treatment of strabismus is not as definitive and stable as the traditional surgical approach, but it has been found most useful in postoperative overcorrection, small deviations, sensory deviations, and acute sixth nerve palsy. This toxin has been effective in the treatment of essential blepharospasm and hemifacial spasm, for which it produces temporary relief of symptoms. In addition, this treatment has been applied to lower lid entropion, myokymia, aberrant regeneration of the seventh nerve, lid retraction, corneal exposure, nystagmus, spasmodic torticollis, and adductor spastic dysphonia.

Blepharospasm↗

A review of clinical trials of lithium in neurology.

Lithium has been put to clinical trials in no less than fifteen neurological disorders. They are Huntington's chorea, tardive dyskinesia, spasmodic torticollis, Tourette's syndrome, L-dopa induced hyperkinesia and the "on-off" phenomenon in parkinsonism, organic brain disorders secondary to brain-injury, drug induced delusional disorders, migraine and cluster headache, periodic hypersomnolence, epilepsy, meniere's disease and periodic hypokalemic paralysis. This paper gives a brief summary of the clinical trials with lithium salts reported in the literature. There are encouraging results on the use of lithium in cluster headaches, cyclic form of migraine and hypomanic mood disorders due to organic brain disorders. The trials with lithium and amitriptyline in tardive dyskinesia needs independent confirmation. The effect of lithium on seizure disorders needs to be addressed too.

Clinical Trials as Topic↗

Direct recording of somatosensory evoked potentials in the vicinity of the dorsal column nuclei in man: their generator mechanisms and contribution to the scalp far-field potentials.

Somatosensory evoked potentials (SEPs) in the vicinity of the dorsal column nuclei in response to electrical stimulation of the median nerve (MN) and posterior tibial nerve (PTN) were studied by analyzing the wave forms, topographical distribution, effects of higher rates of stimulation and correlation with components of the scalp-recorded SEPs. Recordings were done on 4 patients with spasmodic torticollis during neurosurgical operations for microvascular decompression of the eleventh nerve. The dorsal column SEPs to MN stimulation (MN-SEPs) were characterized by a major negative wave (N1; 13 msec in mean latency), preceded by a small positivity (P1) and followed by a large positive wave (P2). Similar wave forms (P1'-N1'-P2') were obtained with stimulation of PTN (PTN-SEPs), with a mean latency of N1' being 28 msec. Maximal potentials of MN-SEPs and PTN-SEPs were located in the vicinity of the ipsilateral cuneate and gracile nuclei, respectively, at a level slightly caudal to the nuclei. The latencies of P1 and N1 increased progressively at more rostral cervical cord segments and medulla, but that of P2 did not. A higher rate of stimulation (16 Hz) caused no effects on P1 and N1, while it markedly attenuated the P2 component. These findings suggest that P1 and N1 of MN-SEPs, as well as P1' and N1' of PTN-SEPs, are generated by the dorsal column fibers, and P2 and P2' are possibly of postsynaptic origin in the respective dorsal column nuclei.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Impacting infant head shapes.

Infant sleep position impacts the development of head shape. Changes in infant sleep position, specifically the movement toward supine sleep, have led to a redefinition of normal head shape for infants in the United States. Historically, a dolichocephalic (elongated) head shape was the norm. Currently the norm has changed to a more brachycephalic (shorter and broader) shape. Since the American Academy of Pediatrics' Back to Sleep Campaign, the incidence of positional plagiocephaly has increased dramatically with a concurrent rise in the incidence of torticollis. Infants who require newborn intensive care, particularly premature infants, are more prone to positional plagiocephaly and dolichocephaly. Both can be prevented or minimized by proper positioning. The infant with an abnormal head shape requires careful evaluation; treatment varies according to the etiology. Craniosynostosis, a less common but pathological etiology for plagiocephaly, should be considered in the diagnostic process. Successful treatment of positional plagiocephaly and dolichocephaly includes systematic positioning changes to overcome the mechanical forces of repetitive positioning, physical and/or occupational therapy to treat underlying muscle or developmental challenges, and in some cases, molding helmet therapy.

Craniofacial Abnormalities↗

A multi-disciplinary study of the ocular, orthopedic, and neurologic causes of abnormal head postures in children.

PURPOSE: To determine the relative frequency that abnormal head postures in children are caused by orthopedic, ophthalmologic, or neurologic disorders, respectively. DESIGN: A prospective, consecutive case series. METHODS: Children found to have an abnormal head posture on routine pediatric examination underwent an evaluation by a pediatric ophthalmologist, pediatric orthopedist, and pediatric neurologist. The study was conducted in northwestern Italy. RESULTS: In the 63 children evaluated, the cause of the abnormal head posture was orthopedic in 35, ocular in 25, and neurologic in 5. In 8 patients, no specific cause could be found. The most common orthopedic cause was congenital muscular torticollis, which accounted for 31 patients. The most common ocular cause was superior oblique muscle palsy, which accounted for 12 patients. In 2 patients neck muscle contracture suggested an orthopedic cause, however, the tight neck muscles were secondary to a head tilt caused by superior oblique muscle palsy. CONCLUSIONS: When the cause of an abnormal head posture is not obvious, a multi-disciplinary approach including ophthalmologic, neurologic, and orthopedic specialists may be helpful.

Child, Preschool↗

Treatment of cervical dystonia and focal hand dystonia by high cervical continuously infused intrathecal baclofen: a report of 2 cases.

We describe 2 patients, one with cervical dystonia (CD) combined with focal hand dystonia (writer's cramp) and another with idiopathic CD, who were unresponsive to oral medications and became resistant to botulinum toxin type A and B injections. Both patients were successfully treated with high cervical (C1-3) continuously infused intrathecal baclofen (ITB). Neck range of motion (ROM) was measured by using a 3-dimensional electromagnetic cervical ROM system. Pain, disability, and severity were assessed by using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). The patient with CD and writer's cramp did well on a continuous baclofen dose of 186.1 microg/d. Her total TWSTRS score improved significantly, her electromagnetic measurements showed an increased in total neck flexion and extension, and her handwriting improved. Unfortunately, this patient (a heavy smoker) developed small cell carcinoma of the lung and died 9 months after her pump was placed. Total TWSTRS score and electromagnetic measurements also significantly improved after pump implant in the patient with CD. He continues to do well on a periodic bolus dose using a combination of 50 microg of baclofen and 25 microg of hydromorphone (Dilaudid) every 4 hours. Our findings suggest the potential usefulness of this therapy in other patients with focal dystonia. To our knowledge, this is the first reported successful treatment of CD and CD combined with writer's cramp with high cervical continuously infused ITB.

Baclofen↗

Three extraordinary complications of adenotonsillectomy.

In a 6-month period, three patients aged 5-11 years were transferred to our tertiary care children's hospital for management of severe complications following adenotonsillectomy. The first patient presented with headaches and lethargy and was found to have a sagittal sinus thrombosis from severe dehydration. The second patient was admitted immediately following an intra-operative oral cavity fire due to electrocautery malfunction. She suffered partial-thickness burns to the buccal mucosa, palate, and lips. The third patient was admitted with torticollis. Grisel's syndrome was initially suspected, but a thorough work up resulted in the diagnosis of a conversion disorder. These cases comprise an interesting cohort of three little-known complications of adenotonsillectomy.

Adenoidectomy↗

Reduction of cervical dystonia after an extended course of chiropractic manipulation: a case report.

OBJECTIVE: The diminution of the signs and symptoms of cervical dystonia following an extended course of specific chiropractic manipulation is described. CLINICAL FEATURES: A 38-year-old man had gross anterior-lateral torticollis, focal dystonia of the head and neck, and radicularlike pains which failed to respond to physical therapy, medication, and injection. INTERVENTIONS AND OUTCOMES: Two specific spinal manipulative technique systems unique to the chiropractic profession (Applied Biostructural Therapy [ABT] and Atlas Coccygeal Technique [ACT]) were applied to the patient. The patient's grading on a modified cervical dystonia scale dropped from a grade 16 to a grade 5 after an extended course of these specific chiropractic manipulative techniques. CONCLUSIONS: The application of Advanced Biostructural Therapy and Atlas Coccygeal chiropractic techniques for management of cervical dystonia is presented. Substantial reduction in the cervical dystonia rating scale was observed with this approach, even after standard medical interventions had failed.

Adult↗

Isolated cervical esophageal duplication: a rarity.

Duplication of alimentary canal is a well-known entity seen commonly in the abdomen and the thorax. Esophageal duplications are the second most common ones after the ideal duplications, but those in the neck are rarer-only 8 cases have been seen earlier. Cervical esophageal duplication in its isolated form causing torticollis and other chronic pressure symptoms has not been reported previously. Here, the authors present a 3-year-old male patient who had cystic duplication of the esophagus in the neck associated with the cervical vertebral defect and Horner syndrome. The authors hereby discuss the various embryological theories of etiopathogenesis of this malformation in brief.

Abnormalities, Multiple↗

Use and complications of high-dose disodium etidronate therapy in fibrodysplasia ossificans progressiva.

A boy with fibrodysplasia ossificans progressiva received nearly twice the usual therapeutic dose of disodium etidronate for 13 months in an attempt to prevent reossification following a second operation to correct severe torticollis. The operation was successful, but during therapy he developed weakness and distinctive bone lesions characterized by general osteopenia, widened physes, and unique radiolucent bands in the metaphyses. The osseous abnormalities were distinct from those of rickets and healed after withdrawal of the drug.

Child, Preschool↗

Biperiden hydrochlorate ameliorates dystonia of rats produced by microinjection of sigma ligands into the red nucleus.

It has been reported that the imbalance of anticholinergic and antidopaminergic activity of each neuroleptic drug correlates with the capacity to produce neuroleptic-induced acute dystonia (NAD) and the major focus of NAD is thought to be the striatum. Anticholinergic drugs are highly effective on NAD, but they are partially effective on neuroleptic-induced tardive dystonia and their effect on idiopathic dystonia is disappointing. Recently, it has been reported that the unilateral microinjection of sigma (sigma) ligands into the red nucleus induces torticollis of rats. This animal model appears to be a model of dystonia, but it is not clear whether it is suitable for NAD in man. To clarify this issue, we investigated the effect of an anticholinergic drug, biperiden hydrochlorate (BH), on this animal model. This study revealed that BH dose-dependently ameliorated dystonia of rats induced by two sigma ligands, whether each sigma ligand had dopaminergic affinity or not. This animal model of dystonia appears to be a model of NAD in man from the viewpoint of treatment-response. The results also suggest that not only dopaminergic and cholinergic systems but also sigma system, and not only the striatum but also the red nucleus, may play an important role in the pathophysiology of NAD.

Animals↗

Serum antibody production to botulinum A toxin.

PURPOSE: Conflicting data have been reported regarding development of serum antibodies to botulinum A toxin. The purpose of this study is to determine conclusively whether antibody production to this toxin occurs in humans, and, if so, to determine its relationship, if any, to length of treatment, total cumulative dose, and clinical response to treatment. METHODS: Sixty-five sera samples from 42 adults treated with botulinum A toxin for essential blepharospasm, hemifacial spasm, or spasmodic torticollis were analyzed via a sphere-linked immunodiagnostic assay for antibody production. Results were plotted against length of treatment, number of injections, cumulative dose, and treatment effect produced. RESULTS: Twenty-four (57%) of the 42 patients produced antibodies in all three diagnostic groups. No significant differences were found between antibody producers and nonproducers with respect to age (P = 0.216), length of treatment (P = 0.586), number of injections (P = 0.619), or total cumulative dose (P = 0.286). Within the antibody-producing group, there was no significant correlation between amount of antibody and length of treatment (P = 0.081), number of injections (P = 0.134), or cumulative dose (P = 0.250). The presence of demonstrable antibodies in serum did not affect the clinical responsiveness to injection. CONCLUSION: Antibody production is present in a majority of patients treated with botulinum A toxin. The sphere-linked immunodiagnostic assay is a reliable and reproducible method for detecting and quantifying these antibodies. When antibody production occurs, it is likely due to variations in individual immune responsiveness and appears to have no direct effect on the patient's clinical response to treatment.

Adult↗

Facial scoliosis--a diagnostic dilemma.

Facial scoliosis may be caused by frontal plagiocephaly or muscular torticollis. Determining the correct cause has implications for patient management. Past confusion in the literature has led to difficulty in determining appropriate treatment plans. The correct diagnosis may be readily obtained by using a series of clinical and radiological features peculiar to each condition. Four cases are shown demonstrating these diagnostic features.

Adolescent↗

Selective peripheral denervation of the levator scapulae muscle for laterocollic cervical dystonia.

Laterocollis, as a symptom of idiopathic cervical dystonia may be due to abnormal contraction of the levator scapulae muscle (LSM). The purpose of this study is to define the effects of selective peripheral denervation of LSM in patients with laterocollic cervical dystonia.The clinical records of 10 patients who underwent LSM denervation for laterocollic torticollis were reviewed. Preoperatively, all patients showed sustained lateral tilt of the head and elevation of the shoulder. Abnormal contraction of LSM was observed and the muscle was denervated surgically in the posterior cervical triangle. The mean age at operation was 38.4 years (range, 20-58 years) and the mean duration of the symptom ranged from 14 to 80 months (mean, 41months). The postoperative follow up ranged from 6 to 72 months (mean, 29.4months). The cervical dystonia rating score of Tsui was 10.8 on average before the operation, and the postoperative follow up score was 1.3. According to the changes of the rating score, success rates, including excellent and good results, were 40% and 60%, respectively. Five patients (50%) postoperatively showed transient dysaesthesia in the neck. There was no disturbance of shoulder elevation or arm raising. When abnormal contraction of LSM is responsible for laterocollis in patients with cervical dystonia, selective peripheral denervation of this muscle is a safe and effective procedure for alleviation of the symptom.

Adult↗

Remote F-wave changes after local botulinum toxin application.

OBJECTIVE: Although the therapeutic effects of botulinum toxin A can be explained by its action at the neuromuscular junction, central or more proximal effects have also been discussed. METHODS: Eleven patients with torticollis spasmodicus and 3 patients with writer's cramp were studied before and 1 and 5 weeks after the first treatment with botulinum toxin. We measured compound muscle action potentials (CMAPs), motor conduction velocities (MCVs), the shortest (SFL) and the mean F-wave latencies (MFL) and F-wave persistence (30 trials) of untreated muscles for each side (ulnar nerve-abductor digiti minimi muscle, peroneal nerve-tibialis anterior muscle). RESULTS: CMAPs and MCVs showed no significant changes. For both nerves, however, SFL and MFL were prolonged slightly 1 week after treatment and returned to about baseline after 5 weeks (t test). The F-wave persistence was reduced 1 week after treatment for the right ulnar and both peroneal nerves (t test). CONCLUSIONS: These results are not likely due to an impairment of neuromuscular transmission. Instead, we propose a decreased excitability of alpha-motoneurons supplying non-treated muscles. A reduction of muscle spindle activity or changes of the recurrent inhibition are discussed as possible causes.

Action Potentials↗

Atlanto-axial subluxation (Grisel's syndrome) following otolaryngological diseases and procedures.

Grisel's syndrome is a rare condition of uncertain aetiology characterized by atlanto-axial subluxation following an infection in the head and neck region. The condition most frequently affects children and characteristically presents in the post-operative period with cervical pain and torticollis. We describe three cases and discuss the aetiology and pathogenesis. A high index of suspicion may lead to early diagnosis in its natural history thereby preventing potentially serious complications.

Adenoidectomy↗

Pallidal deep brain stimulation in cervical dystonia: clinical outcome in four cases.

OBJECTIVE: Report on the clinical results following bilateral globus pallidus interna deep brain stimulation in four patients (one female and three males) with severe cervical dystonia, mean age 48 years (range 37-67). METHODS: All four patients had failed extensive medical and botulinum toxin treatment. The mean duration of the disease was nine years (range 4-15 years). Patients were assessed pre and postoperatively using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Pre-operatively, the mean TWSTRS total score was 43.2 (range 28-60.5). Posteroventral pallidal deep brain stimulators were inserted using MRI and microelectrode recording guidance. Last follow-up was 15 months for the four patients. RESULTS: Mean reduction in the TWSTRS total scores at last follow- up was 73% (range 61- 85%). Improvement in pain occurred soon after deep brain stimulation surgery. Motor improvement was delayed and prolonged over several months. Frequent adjustment in the stimulation parameters was necessary in the first three months. CONCLUSION: Bilateral pallidal stimulation is effective in management of selected cases of intractable cervical dystonia.

Adult↗

Surgical management of anomalous head posture because of horizontal gaze palsy or acquired vertical nystagmus.

PURPOSE: To confirm the effectiveness of the Kestenbaum-Anderson principle in the surgical management of compensatory head posture because of horizontal gaze palsy and acquired vertical nystagmus. METHODS: Nine patients with anomalous head posture because of horizontal gaze palsy, and four patients with acquired vertical nystagmus and oscillopsia and compensatory torticollis underwent surgery according to the Kestenbaum-Anderson principle. As in the treatment of congenital nystagmus, the eyes have to be shifted in the orbits, in the direction of anomalous head posture. Homonymously based prisms were used preoperatively to assess the potential benefit of surgery. At the time of surgery, the clinical conditions of the patients had been stable for at least 1 year. RESULTS: After surgery, compensatory head posture and visual performances improved in all cases and the results remained stable for at least 2 years. CONCLUSIONS: Contrary to what is generally believed, the ocular condition of the patients with compensatory head posture secondary to neurological causes can be often improved with surgery. The aim of surgery is obviously not to modify ocular motility, but rather to improve the head position.

Adult↗