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A transverse lower uterine incision was made at cesarean section in 79 term patients with a singleton fetus in a transverse lie. The mean Apgar score was 9.5 (SD 0.8). One infant sustained a fractured femur, and another had a torticollis. In no case did the incision extend into the uterine vessels.
The neurological manifestations and toxicities of 12 antituberculosis drugs [isoniazid, rifampicin (rifampin), ethambutol, p-aminosalicylic acid, pyrazinamide, streptomycin, kanamycin, ethionamide, cycloserine, capreomycin, viomycin and thiacetazone] are reviewed. Their effects upon the central nervous system, cranial nerves, peripheral nerves and the neuromuscular junction are examined, and drug interactions of neurological concern are briefly discussed. Isoniazid is well known to increase the concentrations of gamma-aminobutyric acid in neural tissues. Although conflicting data have been published, isoniazid may play a limited future role in reducing the degree of adventitious movements noted in certain neurological diseases such as multiple sclerosis, spasmodic torticollis, and other segmental dystonic syndromes. With rifampicin neurological complications have been observed infrequently. Rifampicin penetrates into the CSF and has been shown to have useful activity against various micro-organisms in the CSF, including certain viruses; however, contrary to earlier suggestions, it appears to have no role in the treatment of subacute sclerosing panencephalitis. A number of studies have indicated that isoniazid is associated with a large number of accidental and intentional poisonings. The highest incidence has been observed with Southwestern American Indians in which this agent was involved in 7% of all suicide attempts and 19% of the suicide deaths. Degeneration of the optic chiasma and nerve is a well-known adverse effect of ethambutol; toxicity is manifested by impairment of visual acuity, marked loss of colour discrimination, constricted visual fields, and central and peripheral scotoma. Ototoxicity is a well known problem caused by streptomycin, kanamycin, capreomycin and viomycin. The use of streptomycin in pregnant mothers is associated with congenital deafness in newborns in certain cases. The aminoglycoside antibiotics are also associated with flaccid paralysis following neuromuscular blockade. Adverse reactions to cycloserine are mainly dose-related with neurological and psychiatric syndromes noted in up to 50% of patients. Recent data indicate that isoniazid, rifampicin, ethambutol, pyrazinamide, streptomycin, kanamycin, ethionamide, and cycloserine appear in measurable quantities in the cerebrospinal fluid. Five of these compounds (isoniazid, rifampicin, ethambutol, kanamycin, cycloserine) pass to some degree through non-inflamed meninges. Other than discontinuation of the therapeutic regimen and general supportive measures, very few methods are described in the literature for treatment of acute intoxications with antituberculosis drugs.
Intramuscular injections of botulinum neurotoxin type A cause reversible chemodenervation and subsequent paralysis by blocking the presynaptic release of acetylcholine. Botulinum toxin type A has emerged as the most effective form of symptomatic treatment for abnormabilities in muscle movement (blepharospasm, hemifacial spasm, torticollis) and has been approved for use in these conditions. First results in the treatment of patients suffering from oromandibular dystonia, myogenic craniomandibular dysfunction and recurrent dislocation of the temporomandibular joint are presented. In most cases, therapeutic effects occurred within 1-6 days post-injection. Muscular hyperfunction was reliably reduced and involuntary activity patterns gradually ceased. No severe side effects of the local injections were noted.
In all craniofacial deformities the differential diagnosis between synostotic and non-synostotic conditions must be made. While the first group is usually subject to intracranial surgical intervention, the treatment of non-synostotic deformities is the subject of controversial discussion. Often associated with premature birth, restrictive intrauterine environment or torticollis, these conditions; also defined as positional deformations, can lead to severe plagiocephalic head shapes. Dynamic orthotic cranioplasty is a conservative method of treating these deformities in early childhood by means of an individually fabricated orthotic head band. The principle is that dynamic pressure is applied to prominent parts while leaving space for growth in depressed areas. In accordance with this principle, various deformities can be addressed. Through clinical, anthropometric and radiographic evaluation it has been shown that dynamic orthotic cranioplasty can correct positional deformation of the cranial vault, skull base and upper face with no relapse following treatment. It must be emphasised that the method is simple, easy to handle and very effective when treatment starts within the first 6 months of life. Therefore, its potential should be recognised by paediatricians and craniofacial surgeons.
From anatomic and functional aspects the stomatognathic system and the upper cervical spine are closely interlinked. Together with complex neuromuscular relationships, this gives rise to an important field of cooperation between orthodontics and orthopedics. The literature appeals for close interdisciplinary cooperation for patients with syndromes and for those with torticollis and scoliosis. Since orthopedic points of contact are obvious in these special cases, orthopedic aspects are now being taken increasingly into account in farther-reaching studies. With the rising popularity of manual medicine, these aspects are being recognized from the orthopedic point of view too in terms of functional correlations and are being increasingly debated at international congresses and in the literature. Although relevant publications were initially confined to studies of moderate scientific interest or case reports, potential correlations have undergone scientific investigation in recent interdisciplinary studies. Despite the many clinical studies, no unequivocal recommendation can be given for basic conditions under which an orthopedist is bound to be consulted on patients with orthodontic findings. This literature review is aimed at providing an introduction to this still hotly debated issue.
Botulinumtoxin (BTX) is a neurotoxin produced from Clostridium botulinum under anaerobic conditions and is responsible for botulism, a notifiable, bacterial form of food poisoning. The first case of botulism is believed to have occurred in 1735. An epidemic in Southern Germany in 1793 claimed the death of over the half of those patients who had become ill through eating uncooked blood sausages. The term "pharmakon" is Greek and implicates that a drug originates from poison (potion, remedy). Theophrastus Bombast von Hohenheim known as Paracelsus (1493/94-1541) first described this duality with his dictum "alle ding sind gift und nichts on gift; alein die dosis macht das ein ding kein gift ist" (only the dose makes a remedy poisonous). In Baden-Württemberg in 1817, the poet and physician Dr. Justinus Christian Kerner described the symptoms of botulism, so that at this time botulism was also called Kerner disease. Until the turn of the century the reason for poisoning was not known. Van Ermengem succeeded in isolating the anaerobic bacterium causing botulism, but the specific mechanism of BTX was only established after the second World War. In the late seventies the ophthalmologist Dr. Alan Scott used BTX the first time in the treatment of strabismus. The drug was then used in the treatment of several muscle spasticities such as, for example, torticollis or hemifacial spasm. Only recently BTX has been successfully used for focal hyperhidrosis. We review the history of botulinum toxin from its discovery in the nineteenth century and the research into its effect in the middle of the 20th century up to its clinical use at the present time.
Lesions of the spinal accessory nerve are usually iatrogenic, especially after lymph node extirpation on the neck. Between 1994 and 2003, 31 patients were operated on in the Neurosurgical Department of the University of Ulm for iatrogenic lesions of the XIth cranial nerve. Of 31 patients, 22 had undergone a previous lymph node extirpation, 2 had been injured during a selective peripheral denervation for spasmodic torticollis, and the other 7 patients by different causes. The neurosurgical intervention was performed 0-19 months after trauma (mean 7.2 months). All patients showed paresis/atrophy of the trapezius muscle, and the abduction of the shoulder was markedly reduced. Additional neck and/or shoulder pain was present in 29 of 31 cases. In seven cases, the nerve was compressed by scar tissue and subsequently treated by external neurolysis. Ten patients underwent an end-to-end anastomosis; autologous sural nerve grafting was necessary in 13 cases. After a mean follow-up of 12.6 months, 7 of 31 patients completely recovered. Of 31 patients, 19 experienced partial relief of pain and weakness. Only five patients remained unchanged. The clinical findings after autologous nerve grafting, end-to-end reconstruction, or external neurolysis did not show any significant differences. Microsurgical reconstruction of iatrogenic injury of the spinal accessory nerve is very promising if the interval between trauma and surgical revision is less than 6 months. Up to 12 months, partial recovery can be achieved. Outcome after longer delay is unsatisfactory.
RATIONALE: Typical neuroleptic medications are still administered to as many as 40% of patients receiving antipsychotic treatment in the US. Intermittent administration or interruption of long-term neuroleptic medication for schizophrenia may increase the incidence of human tardive dyskinesias, and similarly may produce increasingly marked motor side-effects, parkinsonism, and other behavioral pathologies in non-human primates. OBJECTIVES AND METHODS: Given these similarities, we addressed the issue of prolonged and intermittent typical neuroleptic treatment and dopaminergic function during a 5-year, multi-phase study with social colonies of Cebus apella monkeys. In the previously reported phase 1, we examined the effects of 48 weeks of exposure to, followed by withdrawal from, fluphenazine decanoate (FPZ). Phase 3 reported here examined the effects of 18 weeks of re-exposure to FPZ in these same monkeys, 91 weeks after discontinuation of their phase 1 FPZ treatment. RESULTS: Analysis of blood plasma FPZ indicated levels of 0.22+/-0.08 ng/ml for the six injections during the re-exposure period (n=54), comparable to the 0.24+/-0.07 ng/ml levels measured during our original treatment with this dose. Acute dyskinesias and dystonias increased by 300% upon re-exposure to FPZ; 15 of 18 FPZ-treated animals exhibited oral-buccal dyskinesias and all exhibited torticollis or retrocollis. Retreatment with FPZ was also associated with highly significant reductions in Self- and Environment-Directed Behavior and Directed Affiliation, effects similar to those seen during the original phase 1 FPZ treatment. Although FPZ re-treatment was associated with a significant reduction in Directed Aggression (an effect that was more robust than that observed during phase 1), in phase 3, we again observed an increase in Directed Aggression during early drug discontinuation when animals were in a stress-inducing situation. CONCLUSIONS: These results both support our phase 1 conclusion that typical neuroleptic medications may contribute to negative symptoms of schizophrenia and provide additional evidence for the possibility of increased aggression in stressful situations when medication is discontinued. Additionally, the results indicate that intermittent treatment with typical neuroleptics may dramatically increase the incidence of dystonias and dyskinesias.
This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus.
The aim of this study was to clarify the morphological characteristics of the communicating branch between the spinal accessory nerve and the anterior root of the first cervical nerve (McKenzie branch), a structure severed during intradural selective rhizotomy for treatment of spasmodic torticollis. The right and left sides of 50 upper cervical segments of spinal cords were dissected and examined under a surgical microscope. Seven of the total 100 samples were found to have the McKenzie branch. Two types of McKenzie branch, which run close to the vertebral artery, were also identified, which might be helpful for improving the surgical results of intradural selective rhizotomy.
BACKGROUND: The pre- and postsurgical results after mono- and bilateral transposition surgery for abducens nerve palsy are reported. PATIENTS AND METHODS: In 13 eyes from 11 patients with 6th nerve paralysis, transposition surgery modified after Kaufmann was performed after a median duration of 13 months (range 6-28 months). The actual angle of strabismus, the capability of abduction and the field of binocular single vision with the tangent screen according to Harms were measured prior to surgery, immediately after surgery (1-3 weeks) and in the later postsurgical period (9-12 months). RESULTS: Of the patients 7 achieved fusion with binocular single vision, 5 of these patients had to accept an ocular torticollis of 10 degrees (range 5-15 degrees) and 4 patients had a tendency to exclusion. Of the 11 patients, 9 were treated successfully with one single surgical procedure 2 of which had bilateral abducens nerve palsy and 2 patients needed 2 further operations. The median preoperative convergent angle in the primary position was reduced from 28 degrees (range 16 degrees-60 degrees) to a median angle of 0 degree, sometimes only latent (range D 7 degrees-C 4 degrees) in the late postoperative period. The median postoperative abduction over the midline was 10 degrees (range 3 degrees-25 degrees). CONCLUSIONS: Bilateral 6th nerve paralysis was treated effectively with simultaneous transposition surgery in both eyes. In most cases an additional weakening of the medial rectus muscle was not necessary. An initial overcorrection with exotropia in the early postoperative period is desired and is a predictive factor for a good late postoperative result.
INTRODUCTION: An increasing number of infants presenting with posterior cranial asymmetry are referred to the paediatric neurosurgeon for clinical evaluation. Most of these cranial dysmorphisms cannot be due to lambdoidal sinostosis, since they originate from pre- (intrauterine constraint) or postnatal (prematurity, torticollis, sleeping position) predisposing factors that have deformed the cranial vault. In the literature there is broad consensus about the conservative management of this nonsynostotic plagiocephaly by means of "positional therapy" or "orthoplasty moulding" with cranial helmets or bands. Some reports can be found about the effectiveness of so-called dynamic orthotic cranioplasty (DOC) to improve the cranial asymmetry. TECHNICAL NOTE: In order to simplify the manufacturing procedure and improve the modulation of the corrective forces, we have designed a new orthotic device (thermoplastic orthotic device, TOD) using thermoplastic materials that can be moulded directly on the infant's head. CONCLUSION: With this technique it is no longer necessary to take any cranial impressions and the orthotic device can be easily remodelled, ensuring perfect adaptability of the device to the cranial vault throughout the entire treatment. Moreover, the superior flexibility of this orthosis allows modulation of the corrective forces.
BACKGROUND: Grisel's syndrome is a uni- or bilateral subluxation of the atlas on the axis associated with an inflammatory condition of the head or neck. This uncommon entity usually affects children. The condition seems to be secondary to hyperemia and laxity of the atlanto-axial joints. PATIENTS AND METHODS: We report clinical and neuroimaging findings, treatment modalities, and outcomes in 4 children with atlanto-axial rotatory subluxation (AARS) associated with a previous upper respiratory tract infection treated at our Unit during the last 7 years. RESULTS: All children presented with neck pain, head tilt, and reduced neck motion. In 3 patients, the condition was secondary to otitis media and in the remaining patient to viral pharyngitis. The diagnosis was established by clinical signs and symptoms and confirmed by neuroimaging studies, especially by computerized tomography and 3-D reconstructions. Early detection and treatment resulted in a good outcome in all patients. None of the patients required surgical treatment. CONCLUSIONS: Grisel's syndrome must be suspected in children with painful torticollis associated with an upper tract respiratory infection. The best results are obtained with early conservative treatment, which prevents chronic changes leading to persistent neck pain and deformity, namely atlanto-axial rotatory fixation (AARF). We emphasize the fact that the diagnosis of Grisel's syndrome is mainly clinical, while the diagnostic confirmation of AARF can only be attained by special neuroimaging techniques.
CASE REPORT: A case of spontaneous hemorrhage into an intradural extramedullary neurenteric cyst at the C4-C6 level in a 6-year-old boy is reported. The child presented with sudden neck pain, torticollis, and myelopathy. The cyst was of high signal intensity on both T1- and T2-weighted magnetic resonance images, suggesting hemorrhage into the cyst. The combination of CT and MR provided useful preoperative information regarding the nature and the location of the cyst. A posterior approach via osteoplastic laminotomy was used for this anteriorly localized cyst. The use of intraoperative ultrasound was helpful in identifying and puncturing the cyst to minimize the spinal cord manipulation during the excision of the cyst wall. DISCUSSION: We report the first documented case of hemorrhage into a spinal neurenteric cyst that has been confirmed surgically and histopathologically.
OBJECTS: Clinical symptoms and signs of increased intracranial pressure (ICP) may be nonspecific and unreliable, or even entirely absent, in hydrocephalic infants and children. Even with a radiological examination, it is often difficult to distinguish between "arrested hydrocephalus" and slowly progressive hydrocephalus requiring treatment. METHODS: We present two cases with unusual and initially misleading clinical manifestations of increased ICP. In both cases, the disturbed cerebrospinal fluid (CSF) dynamics, i.e. raised ICP, were not recognised until demonstrated by a long-term ICP monitoring. In a 5-month-old boy with normal head circumference and normal psychomotor development, the sudden onset of episodes of torticollis and screaming were the only symptoms. No pathology underlying the developing hydrocephalus and the raised ICP could be established, but the boy's condition improved after a shunt operation. In the other case, symptoms and signs consisted primarily in a slowly progressive dilatation of the facial veins with onset at the age of 2-3 years. As the girl also presented a congenital subvalvular aortic stenosis, the venous congestion was initially thought to reflect a vena cava superior syndrome. Further radiological examinations, however, revealed an extensive sinus thrombosis underlying the raised ICP. The girl underwent shunt insertion, and the venous congestion was reduced. CONCLUSIONS: The cases illustrate that though clinical and radiological findings may be very doubtful, or unusual for increased ICP, direct diagnostic long-term ICP monitoring should always be contemplated. Only ICP monitoring can reveal with certainty whether disturbed CSF dynamics are involved, requiring a CSF diverting operation to treat and stabilise the condition.
Dystonia can occasionally be found in idiopathic Parkinson's disease. It is very uncommon in untreated patients and is more frequently seen as a complication of its treatment. In this review, the various types of dystonia occurring in PD, the differential diagnosis with other parkinsonian syndromes associated with dystonia and treatments available are revised. Dystonia unrelated to treatment can be typical (blepharospasm, torticollis), atypical (parkinsonian writer's cramp, camptocormia, anismus), or occurring in earlyonset Parkinson disease (the so-called kinesigenic foot dystonia, considered a hallmark of early-onset Parkinson's disease). Early and prominent dystonia in untreated patients with parkinsonism should raise the suspicion of other entities other than Parkinson's disease, such as progressive supranuclear palsy, multiple system atrophy or corticobasal degeneration. In patients on chronic dopaminergic treatment, peak-dose dystonia, diphasic dystonia and off-dystonia can be seen. The later constitutes the major dystonic feature of chronic levodopa therapy, and a wide variety of strategies are available to manage this complication. Among them, deep brain stimulation of the subthalamic nucleus has proved to be the most effective one. Dystonic reactions (mainly involving oculomotor cranial nerves and limbs) in operated patients (especially carriers of deep brain stimulation (DBS) devices) are increasingly being reported, constituting a new type of dystonia in patients with Parkinson's disease: dystonia linked to surgical treatment.
The clinical, pathophysiological and genetic features of some of the paroxysmal movement disorders are reviewed. Paroxysmal kinesigenic choreoathetosis/dyskinesias (PKC/PKD) is a condition in which brief and frequent dyskinetic attacks are provoked by sudden movement. PKC is more common in men and can be idiopathic (commonly familial) or due to a variety of causes. The pathophysiology of PKC is uncertain but it could be an ion-channel disorder. Antiepileptic drugs particularly carbamazepine are very helpful in a large proportion of cases. Paroxysmal exercise induced dystonia (PED) is a rare disorder manifesting as episodes of dystonia mostly affecting the feet induced by continuous exercise like walking or running. Although the initial cases were familial, there is a higher proportion of sporadic cases. The pathophysiology of PED is unknown and antiepileptic drugs are generally unhelpful. In paroxysmal dystonic choreoathetosis/non-kinesigenic dyskinesias (PDC/PNKD) the attacks are of long duration and induced by variety of factors including coffee, tea, alcohol and fatigue but not by sudden movement. PDC can be idiopathic (familial or sporadic) or symptomatic due to a variety of causes. The gene for familial PDC has been linked in 2 families to chromosome 2 q close to a cluster of ion channel genes again suggesting that this disorder may also be a channelopathy. Other paroxysmal disorders include paroxysmal nocturnal dyskinesia, a form of frontal lobe epilepsy in some cases which may be familial with autosomal dominant inheritance (ADNFLE). The gene for ADNFLE in one family has been found to be a mutation in the neuronal acetylcholine receptor gene (CHRNA4) on chromosome 20q. Tonic spasms in multiple sclerosis and Sandiffers syndrome producing intermittent torticollis in infants and children are other paroxysmal movement disorders.
Dysphagia is a common symptom in various neurological disorders affecting pharyngeal functions. Cricopharyngeal dysfunction is one of the major findings in these patients. The most effective treatment for restoring normal swallowing function in persistent cricopharyngeal dysfunction is cricopharyngeal myotomy, especially when mechanical obstruction or a well-localized neuromuscular dysfunction, such as a cricopharyngeal muscle spasm, is present. However, when there is a more diffuse neurological disorder present the results of surgery are more disappointing. In unclear cases, or in patients with temporary problems, no good method other than swallowing training, bougienage, and tube feeding are available. During the past decade, botulinum toxin has been found to be of therapeutic value in the treatment of a variety of neurological disorders associated with inappropriate muscular contractions such as torticollis and spasmodic dysphonia. Recently, injections of botulinum toxin in patients with cricopharyngeal muscle dysfunction have been reported to result in marked relief of dysphagia. In this article we describe our experiences with botulinum toxin injections to treat four patients suffering from deglutition problems and cricopharyngeal dysphagia of different origins. Botulinum toxin was injected into the cricopharyngeus muscle that was identified by endoscopy under general anesthesia. In this study, no major side effects were observed. Three patients obtained a significant improvement of esophageal symptoms after the first injection. The treatment had limited effect in one patient who had reflux disease and only slight cricopharyngeus dysfunction.