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Malignant intracranial chordoma and sarcoma of the clivus in infancy.

Neuroradiological and clinical findings suggesting chordoma of the clivus are described in three children. They presented with torticollis as the primary and predominant symptom. Progressive deterioration with development of slurred speech, signs of intracranial pressure and further cranial nerve involvement led to more extensive neuroradiological investigation. The clinical and neuroradiological findings in these three children were similar; the prognosis was unfavourable for two of the children. The histological diagnosis in two cases was malignant chordoma; in the third case it was undifferentiated sarcoma of the clivus.

Child, Preschool↗

Microneural decompression operations in the treatment of some forms of cranial rhizopathy.

Since 1985 147 explorations of the cerebellopontine angle have been performed in patients with trigeminal neuralgia (TN), in 26 patients with hemifacial spasm (HFS), and in the craniospinal region in 22 patients with spasmodic torticollis (ST). In TN in 128 (87%) patients vascular compression of the fifth nerve was found, in 8 (5%)--compression by a tumour, but in 11 (7.5%) nerve compression was not observed. In 92 (63%) patients only microvascular decompression (MVD) was performed, but in 36 (24.5%)--MVD and partial sensory rhyzotomy (PSR). Following MVD pain was absent or insignificant in 121 (92%) after MVD and PSR in all 36 patients, recurrence was observed in 5 (5%) cases. MVD was inefficient in case of atypical TN. Two patients died. As diagnostic criteria for vascular decompression pre-operative and intra-operative recording of short latency somatosensory evoked potentials of the fifth nerve were used. In all HFS patients vascular compression of the facial nerve roots has been established. Following MVD spasm was absent in 18 (69%) patients, 6 (23%) patients had significant improvement, but in two cases (8%) the improvement was insignificant. During craniospinal exploration in ST the accessory nerve was compressed in 6 cases by artery, in 6 patients only by cross-sectional adhesions, in two by a hypertrophied dentate ligament and in 3 cases by small neurinomas of the XI th nerve. Following microdecompression of the accessory nerve (without roots transsection) 17 (77%) patients fully recovered or had significant improvement. One of the 4 patients with insignificant improvement had previously undergone stereotactic surgery, but the other had retrocollis. In one patient with neurinoma of the XI nerve recurrence was observed, no significant improvement was noted in the other two patients.

Adult↗

Congenital vertebral-jugular fistula in an infant.

Pediatric cardiologists are often consulted to evaluate continuous murmurs in children. This case report describes a child with a congenital vertebral-jugular fistula who presented with torticollis and a continuous murmur. The differential diagnosis of continuous murmurs in this setting is discussed.

Arteriovenous Malformations↗

Botulinum toxin treatment in patients with focal dystonia and hemifacial spasm. A multicenter study of the Italian Movement Disorder Group.

In six Centers belonging to the Italian Movement Disorder Study Group, the efficacy of botulinum toxin treatment was evaluated in an open collaborative study in 251 patients with focal dystonia and hemifacial spasm. The percentage of functional improvement ranged from 66% to 81% in patients with blepharospasm, from 40% to 51% in patients with spasmodic torticollis and from 73% to 81% in those with hemifacial spasm. Good results were also obtained in patients with oromandibular dystonia, laryngeal dystonia and writer's cramp. Side effects were mild and transient. Local botulinum toxin injection is the first choice symptomatic treatment in focal dystonia and hemifacial spasm.

Adult↗

A randomized, double-masked, crossover comparison of the efficacy and safety of botulinum toxin type A produced from the original bulk toxin source and current bulk toxin source for the treatment of cervical dystonia.

In 1997, the US FDA approved a new bulk toxin source (now referred to as current) for the manufacture of botulinum toxin type A (BTX-A). The current BTX-A preparation has a lower neurotoxin complex protein load than the original BTX-A preparation, which may reduce antigenic potential. The present double-masked, multicenter study compared the efficacy and safety of BTX-A (BOTOX) produced from both original and current bulk toxin sources for the treatment of cervical dystonia. Patients (N = 133) were injected with BTX-A produced from original and current bulk toxin sources using a crossover design. Adverse events were assessed at each visit. Efficacy was assessed at 2 and 6 weeks post-injection using the severity and pain-disability subscales of the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Mean BTX-A doses were comparable (original: 155 U, current: 156 U). Both BTX-A preparations produced similar, statistically significant reductions in TWSTRS severity and pain-disability scores at weeks 2 and 6 post-injection. The original and current BTX-A preparations showed no significant differences in adverse events, including both treatment-related (34%, 31%) and treatment-unrelated (27%, 32%), respectively. BTX-A produced from the original and current bulk toxin sources showed comparable efficacy and safety in the treatment of cervical dystonia; both significantly reduced dystonia severity and pain.

Botulinum Toxins, Type A↗

[Initial experiences with clinical use of botulinum toxin type B].

Recently, botulinum toxin type B (BT-B) was introduced for treatment of cervical dystonia (CD). We wish to report on experience with BT-B derived from registration studies and from our own preliminary clinical experience. Botulinum toxin type B can be used successfully in CD patients with antibody-induced failure of botulinum toxin type A therapy (antibody patients). In our own 15 antibody patients, 11835 +/- 2,039 mouse units of BT-B (NeuroBloc) reduced the Toronto Western spasmodic torticollis rating scale (TWSTRS) score from 20.5 +/- 3.6 to 13.1 +/- 5.4. BT-B can also be used successfully in CD patients not previously exposed to BT-A or BT-B (de novo patients). In our own nine de novo patients, 10436 +/- 3,320 mouse units of BT-B reduced the TWSTRS score from 18.4 +/- 5.4 to 9.2 +/- 4.8. Altogether, dryness of the mouth occurred in 21 CD patients (severe 10, moderate 7, mild 4), accommodation difficulties in seven, conjunctival irritation in five, reduced sweating in four, swallowing difficulties in three, heartburn in three, constipation in three, bladder voiding difficulties in two, and dryness of nasal mucosa, head instability, and thrush in one each. BT-B produces substantially more systemic autonomic side effects than BT-A. It can be considered the therapy of choice in antibody patients. For de novo patients and for BT-A-treated CD patients, BT-B cannot be recommended currently.

Adult↗

Quantification of C2 cervical spine rotatory fixation by X-ray, MRI and CT.

Atlanto-axial rotatory displacement is known to be a cause of childhood torticollis and may as well be responsible for chronic neck pain after rear-end automobile collisions. The objective was to determine whether quantification of C2 malrotation is possible by plain radiographs in comparison to CT as the golden standard. MR imaging was evaluated as to whether it was of equal value in the detection of bony landmarks. C2 vertebra of five human cadaveric cervical spine specimens, ligamentously intact, were rotated using a Steinmann pin in steps of 5 degrees up to 15 degrees right and 15 degrees left. Plain radiographs, CT and MRI images were taken in each rotational step. Data were analyzed for quantification of C2 rotation by three independent examiners. A rotation of 5 degrees led to a spinous process deviation (SPD) from the midline of 3 mm as measured on an a.p. plain radiograph. A coefficient of rotation was calculated (1.62 degrees mm(-1)). Data analyzed by three examiners revealed a small coefficient of variation (0.03). MRI and CT measurements showed comparable results for the quantification of rotation; however, in both techniques the 15 degrees rotation was underestimated. Quantification of upper cervical spine malrotation was possible on plain radiographs using the SPD and a rotation coefficient. MRI and CT were equally successful in the assessment of C2 malrotation.

Adult↗

Botulinum toxin type B in antibody-induced botulinum toxin type A therapy failure.

Recently, it was reported that botulinum toxin type B complex (BoNT/B) (NeuroBloc(R), Elan Pharmaceuticals) can produce an adequate therapeutic response in patients with antibody induced failure of botulinum toxin type A complex (BoNT/A) therapy. We wanted to study whether this effect is transient or sustained. For this, 10 consecutive patients (6 males, 4 females, age 54.6 +/- 14.3 years, duration of illness 15.8 +/- 7.0 years) with complete BoNT/A therapy failure and BoNT/A antibody titres in excess of 10mU/ml in the mouse diaphragm assay (MDA) received BoNT/B in an initial dose of 12370 +/- 1804MU. After the first BoNT/B application the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) improved from 20.1 +/- 3.0 to 11.9 +/- 3.4. In all patients systemic anticholinergic side effects occurred. Three patients had stable continuous responses to two, three and five subsequent BoNT/B applications. Six patients showed complete secondary therapy failure to the second or third subsequent BoNT/B applications. Side effects did no longer occur. In four of them the BoNT/B doses were doubled without producing any therapeutic benefit or any side effects. In five of them MDA testing was performed and revealed BoNT/B antibody titres in excess of 1mU/ml. One patient lost half of her initial BoNT/B responsiveness indicating partial secondary BoNT/B therapy failure. This partial therapy failure was seen on two consecutive application series and has not proceeded to complete therapy failure so far. BoNT/B seems to be only temporarily effective in the majority of patients with BoNT/A antibody induced therapy failure. Whether the formation of BoNT/B antibody points to a high antigenic potency of BoNT/B, to an increased immunoreactivity in BoNT/A antibody carriers or whether it is due to the large amount of protein applied in BoNT/B therapy needs to be studied.

Adult↗

Botulinum toxin type B de novo therapy of cervical dystonia: frequency of antibody induced therapy failure.

Botulinum toxin induced therapy failure type B antibody (BT-B, BT-B-AB) has so far only been reported after previous formation of antibodies against botulinum toxin type A (BT-A, BTA- AB). We wanted to explore the risk of BT-B-AB-induced therapy failure in patients who were exposed to botulinum toxin for the first time. For this purpose we followed nine patients with cervical dystonia receiving BT-B (NeuroBloc/Myo- Bloc, Elan Pharmaceuticals) in a dose of 11435 +/- 2977MU during 4.9 +/- 3.0 injection series. All patients showed a satisfactory initial therapeutic response as documented by a Toronto Western Spasmodic Torticollis Rating Scale score reduction from 17.7 +/- 9.4 to 5.3 +/- 4.8 and an overall subjective improvement of 56.1 +/- 28.3%. Seven patients experienced systemic anticholinergic side effects. Five patients had stable therapeutic responses over subsequent injection series. Four patients experienced complete therapy failure with BT-B-AB titres in excess of 10 mU/ml on the mouse diaphragm assay. Doubling the last effective BT-B dose produced neither therapeutic effects nor side effects. Subsequent applications of botulinum toxin type A produced a continued therapeutic response in one patient and complete therapy failure in the other.Despite the small sample size a frequency of 44 % indicates a high risk for BT-B-AB-induced complete therapy failure. The high amount of neurotoxin administered when NeuroBloc/MyoBloc is used might be a contributory factor. Further prospective comparative studies are necessary to monitor the frequency and time course of BT-B-AB formation.

Adult↗

Patterns of postural asymmetry in infants: a standardized video-based analysis.

Cervical rotation deficit (CRD) and trunk convexity (TC) constitute the diagnosis of infantile postural asymmetry (IPA), which is often associated with further asymmetric features. However, very little data on the entire symptom complex are currently available. The aim of this study was to analyse the entire clinical spectrum of IPA based on a standardized video documentation. Forty-five infants (27 male) with an asymmetry score of > or = 12 points (scale: 4-24) at a median post-term age of 10 weeks (range: 6-16) were selected from two previously studies using predefined criteria. CRD and TC as reactive movements to an orienting head turn in the prone and supine position were assessed from video recordings by three independent observers. Plagiocephaly, oblique body position and asymmetric foot position were descriptively assessed by consent of the same observers. Hip dysplasia data were derived from sonography charts. The assessment of the reactive movements showed a "scoliosis" pattern in sox infants, a "torticollis" pattern in nine infants, a "mixed prone" pattern in 13 infants and a "mixed" pattern in 26 infants. Side agreement in the prone and supine position of TC and CRD was seen in 27 infants, with a left-sided convexity and left-sided head rotation deficit in two-thirds of the infants. Plagiocephaly was present in 27 infants, oblique body position in 13 infants, hip dysplasia in 4 infants and calcaneus foot in 11 infants. In conclusion, infantile asymmetry pattern analysis showed that morphological and functional anomalies are intricately linked and that infants with only a single apparent sign of asymmetry have actually a much more generalized disturbance.

Clubfoot↗

Delayed closed reduction of rotatory atlantoaxial dislocation in an adult.

We report a case of rotatory atlantoaxial dislocation due to a rugby injury in an adult. The patient presented with torticollis 4 weeks after the injury. The neurological evaluation was normal. Reduction proved difficult to obtain and required 10 days of skull traction followed by gentle manipulation. After reduction, dynamic cervical radiographs showed no instability and magnetic resonance imaging (MRI) confirmed that the transverse ligament was intact. After 6 weeks of immobilization in a Minerva jacket, a dynamic rotatory computed tomography (CT) scan confirmed that the atlantoaxial joint was stable. Such cases of atlantoaxial joint dislocation in adults treated by traction after a considerable delay are rare. In our patient, demonstration by MRI that the transverse ligament was intact led to the decision to use conservative therapy, which proved successful.

Adult↗

Transient tetraplegia and vegetative dysfunction depending on cervical positioning caused by a cervical spinal chordoma.

A 39-year-old man with acquired torticollis suffering from cervicobrachialgia and neurological deficits is presented. Due to a change in head position a transient reproducible tetraplegia and severe vegetative dysfunctions were caused. The origin of this uncommon serious combination of symptoms and signs was a chordoma of the upper cervical spine. After surgical decompression the patient was free of neurological deficit and pain. Review of the literature did not reveal any similar case.

Adult↗

The role of the parafascicular complex (CM-Pf) of the human thalamus in the neuronal mechanisms of selective attention.

The reactions of 93 neurons in the parafascicular complex (CM-Pf) of the human thalamus were studied by microelectrode recording during stereotaxic neurosurgical operations in patients with spastic torticollis. High reactivity was demonstrated for two previously classified types of neurons with identical irregular (type A) and bursting Ca2+ -dependent (type B) activities in response to presentation of relevant verbal stimuli evoking selective attention in humans. Concordant changes in the network activity of A and B neurons were observed, in the form of linked activatory-inhibitory patterns of responses and the appearance, at the moment of presentation of an imperative morpheme of the command stimulus, of rapidly occurring intercellular interactions consisting of local synchronization with simultaneously developing rhythmic oscillatory (3-4 Hz) activity. Data are presented on the existence of a direct connection between these neuronal rearrangements and activation of selective attention, providing evidence for the involvement of the thalamic parafascicular complex (CM-Pf) in the mechanisms of selective attention and processing of relevant verbal information during the preparative period of voluntary actions.

Acoustic Stimulation↗

Absence of spontaneous head tilt in superior oblique muscle palsy.

We used the head-tilt test--based on an imbalance between the function of a paretic superior oblique muscle and its homonymous superior rectus muscle, rather than on an anomaly of cycloversion-to test three patients with superior oblique muscle palsies. Spontaneous ocular torticollis was absent if a patient had poor vision in one eye or if his vertical fusional amplitudes were of such magnitude that he was able to overcome his vertical deviation in all positions of gaze. These latter instances, though rare, may be more common than reports indicate.

Adult↗

Rotatory atlantoaxial subluxation following pharyngoplasty.

Atlantoaxial rotatory subluxation often presents in childhood as a stiff, painful neck with associated torticollis. It can occur spontaneously following minor trauma or an inflammatory process in the cervical tissues. Failure to recognise the condition may result in a fixed rotatory subluxation which can be difficult to treat. The authors report on two cases of atlantoaxial rotatory subluxation which occurred following pharyngoplasty and discuss the clinical presentation and management of these cases. The literature on this subject is briefly reviewed.

Atlanto-Axial Joint↗

The cranio-facio-cervical scoliosis complex.

Six patients are presented with facial asymmetry including vertical orbital dystopia in association with torticollis. All six have also had other abnormalities. Some degree of hemifacial microsomia was present in five of the six, a so-called plagiocephalic or rhomboid skull with normal cranial sutures in four of the six, and cervical spine anomalies in four of the six. Other deformities have also been noted. The condition is best named the cranio-facio-cervical scoliosis complex. The implications for treatment of the facial asymmetry, in particular the unequal levels of the orbits in the established deformity, are discussed and a surgical plan which has been used in four patients described.

Abnormalities, Multiple↗

Increased striatal 18F-dopa uptake and normal glucose metabolism in idiopathic dystonia syndrome.

Striatal 18F-Dopa uptake and glucose metabolism were studied by positron emission tomography with 6-L-[18F]fluorodopa and [18F]fluorodeoxyglucose, respectively, in 8 patients with idiopathic dystonia. Patients with abnormal findings on the brain CT and MRI were excluded from this study. The clinical diagnosis consisted of torsion dystonia in 3 patients, focal dystonia limited in the arm in 3 and cervical dystonia (spasmodic torticollis) in 2. The 18F-Dopa uptake, corrected by nonspecific retention in the cerebellum, at 120 min post-administration was evaluated, and increased 18F-Dopa uptake in the putamen and in the caudate head was observed in the patients with idiopathic dystonia compared to the normal controls. The striatal glucose metabolism in the patients with idiopathic dystonia showed no difference with the normal controls. These findings suggest that pathogenetic mechanism of idiopathic dystonia involves increased presynaptic activity of the dopaminergic system in the striatum.

Adult↗

Haloperidol and cognitive shifting.

In this study haloperidol appeared to affect the performance on a selected category of cognitive tasks considered to represent shifting aptitude. A pretest--post-test design was used with two groups of subjects: 17 patients suffering from idiopathic spasmodic torticollis, and 17 controls who were matched for age and intelligence. The results are discussed in relation to previous findings on haloperidol and cognition, shifting disorder in Parkinson's disease and changes in behavioural organization found in animals with an experimentally induced dopaminergic hypoactivity.

Attention↗