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Ocular manifestations of deformational frontal plagiocephaly.

Frontal plagiocephaly can be caused by two mechanisms: craniosynostosis and external deformational pressure. Synostotic plagiocephaly is known to be associated with vertical strabismus and contralateral head tilt. Thirteen patients with deformational frontal plagiocephaly were examined to evaluate head position, ocular motility, and alignment. Nine of the patients (70%) were found to have ipsilateral torticollis, but only one patient had strabismus and this was a horizontal deviation not obviously associated with head position. The clinical features of synostotic and deformational plagiocephaly are discussed to distinguish the two conditions, which have a different treatment and outcome.

Child↗

[Long-term treatment with botulinum toxin: dosage, treatment schedules and costs].

Local injection of botulinum toxin A (BTX) is considered as the treatment of choice for spasmodic torticollis (ST), blepharospasm (BS) and, with certain limitations, for hemifacial spasm (HFS). Long-term data from 89 patients with ST, 39 patients with BS and 15 patients with HFS were evaluated and subgroups of patients who received at least three (60 ST, 17 BS, 9 HFS), six (35 ST, 10 BS, 8 HFS) and nine (26 ST) re-injections were formed. These groups were investigated in regard of increases in the dosage of BTX and in regard of shorter treatment intervals in cases of repeated treatment. Annual drug costs were calculated from these data. Patients treated at least three times were asked to fill out a questionnaire concerning satisfaction and the quality of life after BTX treatment. Furthermore, the patients' "willingness to pay" for BTX treatment was investigated. Our calculations showed neither a significant increase in the dosage of BTX nor a significant reduction in the length of treatment intervals after 3 or 6 treatment sessions. Annual drug costs for BTX for the treatment of ST were 10,542 ATS, of BS 2847 ATS and of HFS 1029 ATS. 86.1% of patients with ST, 88.9% with BS and 100% with HFS were very satisfied or satisfied with the BTX treatment. In all 3 diagnostic groups, a significant mean improvement of quality of life was measured on a visual analogue scale (p < 0.001). We conclude that the injection of botulinum toxin A is a highly effective and cost effective treatment for ST, BS and HFS, and significantly increases the quality of life of all these patients.

Adult↗

The migraine: benign paroxysmal vertigo of childhood complex.

The migraine--a benign paroxysmal vertigo of childhood complex is the most frequent etiology of pediatric dizziness, with an incidence of 34.7%. We present a series of 34 children in whom this entity was diagnosed. We describe the most important characteristics and discuss the possible etiological factors. We review the theories about a common pathophysiological origin for migraine, benign paroxysmal vertigo of childhood, and paroxysmal torticollis. Evolution of these entities confirms the idea of a common origin and a different vestibular symptomatology, depending on the age of the child experiencing dizziness.

Adolescent↗

Induced systemic candidiasis in young broiler chickens.

Systemic candidiasis was induced in broiler chickens 14 days old by intravenous injection of a suspension of viable Candida albicans cells. Injection resulted in decreased body weight, moderate mortality, swollen and reddened livers and kidneys, pancreatitis, and disturbances of the nervous system. Three types of neutral disturbances were observed: 1) extreme opisthotonus with spasmodic tremors; 2) extreme torticollis with cranial rotation of 270 degrees: and 3) extreme torticolis which resulted in the head being drawn in a medial-ventral direction. None to 50% of the inoculated birds exhibited these neural disturbances, depending on the isolant of C. albicans used. Microbiological examination of internal organs and blood revealed that C. albicans localized in the meninges of the brain. There was also a significant isolant-related effect of C. albicans on the growth rate of the inoculated birds. These easily quantitated differential effects of various isolants of C. albicans offer the prospect of correlating biochemical characteristics with virulence and obtaining information about the mechanism of pathogenicity of this microorganism.

Animals↗

[Epidural hydatid cyst of the posterior fossa].

We report a case of epidural hydatid cyst in the posterior fossa in a 5-year-old child. The disease was revealed by raised intracranial pressure with torticollis. The diagnosis was based on the brain CT scan and MRI, and confirmed surgically. The course was uneventful. Cranial epidural hydatid cysts are very rare: only 18 cases have been reported previously in the literature.

Child, Preschool↗

Botulinum toxin in otolaryngology: a review of its actions and opportunities for use.

Botulinum toxin has several important properties that make it an ideal chemical denervator. These include its high degree of specificity for the neuromuscular junction, its ability to induce temporary and reversible denervation, and its limited degree of side effects and complications. Botulinum toxin is being used safely in a wide variety of clinical settings by many different specialists. In otolaryngologic practice, it is being administered for the treatment of at least a dozen conditions, including various dysphonias, dystonias, and spasms as well as torticollis, facial nerve paralysis, and hyperkinetic facial lines. Studies have shown that botulinum toxin injections have a high rate of success in temporarily relieving symptoms.

Botulinum Toxins↗

Clinical application of botulinum toxin in otolaryngology, head and neck practice (brief review).

Botulinum toxin (Botox) is useful in controlling the symptoms of patients with movement disorders. Application of Botox serves to (1) inhibit hypertonicity, (2) enhance the action of the antagonistic muscles, and (3) avoid an impingement in order to reestablish "the balance of forces". In accordance with the principles mentioned above, Botox can be used to treat dystonias of the larynx (adductor laryngeal spasmodic dysphonia, abductor laryngeal spasmodic dysphonia), laryngeal granulomas, laryngeal joint dislocation, cricopharyngeal spasm, and posterior glottic synechiae. In addition, extra-laryngeal disorders such as blepharospasm, hemifacial spasm, oromandibular dystonia, and spasmodic torticollis respond well to Botox. The effects of Botox are reversible and have specific localized activity. Hence, Botox has served as a powerful diagnostic method in exploring the underlying mechanism of various types of dystonias and provides some therapeutic benefits before pursuing surgical options. Here we review the literature and describe our experiences with Botox, including such topics as preparing and storing Botox, identifying the target muscles under EMG-guidance, choosing an appropriate dose, and outlining the applications of Botox in Otolaryngology, Head and Neck Surgery practice.

Blepharospasm↗

[Peripheral mechanisms of the pathogenesis of cervical dystonia].

Peripheral mechanisms of pathogenesis and formation of the symptoms of cervical dystonia (spastic torticollis--ST) were investigated. By means of orthopedic diagnosis and analysis of motor stereotype, traumas, algesic syndrome in 40 patients with ST, it was found a "short leg" on the side, opposite to the rotation of the head, frequent postural loads on the head and on the neck before ST rise, and algesic and other sensory symptoms in the same region. The data of neuromyography and of evoked skin sympathetic potentials show a presence of pathologic changes in sympathetic autonomic fibers as well as in axons of accessory nerve at both sides, mainly at the left. Such alterations were mainly peripheral and were found more frequently in a group of left-sided ST. The correlations observed between a side of the rotation of a head and a side of peripheral factor, permitted to suppose that peripheral factors (the factor of a "short leg" mainly) determined frequently a side of the rotation of the head. So, ST therapy must influence the peripheral mechanisms, but not only the central ones.

Accessory Nerve↗

[Optimal medical care for children with Down syndrome and their parents].

Children with Down's syndrome have an increased risk of congenital anomalies and additional medical problems. These can be treated but are often seen as part of the syndrome and not as the cause of developmental retardation. Based on the current level of knowledge, specific medical tests and evaluations are recommended. The reasons for these guidelines, composed by the Down's syndrome Work Group of the Paediatric Association of the Netherlands, are illustrated by the case reports of five patients: in a 4-year-old boy, retardation improved after celiac disease had been diagnosed. In a 5-year-old boy, slow speech development was reversed after hearing loss was diagnosed. In a 2-year-old girl, development improved after a mitral valve stenosis had been corrected. In a 6-month-old boy, drinking problems were reversed when torticollis was diagnosed and a self-deflating drinking bottle was introduced. An adult man suffering from epilepsy and from self-mutilative rubbing and hitting of his eyes, regained his sight after cataract, myopia and atrophy bulbi were diagnosed. A well-balanced disclosure of the diagnosis, repeated medical screening, early motor training and social interventions combined with active parental participation, warrant optimal development. In cooperation with the Down's syndrome Parent Association, multidisciplinary medical teams have been established to offer and promote this care.

Adult↗

[Efficacy and tolerance of gabapentin in dystonia and hemifacial spasm: a pilot study].

INTRODUCTION: The pharmacological management of dystonia is suboptimal. Focal dystonia and hemifacial spasm can be successfully managed with botulinum toxin injections. The physiopathology of dystonia remains a mystery. The gabaergic transmission may be involved in the origin of dystonia. Gabapentin is a gabaergic agent and therefore it can be useful in dystonia. PATIENTS AND METHODS: 28 patients with different forms of dystonia and hemifacial spasm were included in this open study. They received gabapentin (mean dose 1885 mg/d). RESULTS: Gabapentin was not efficacious but a subgroup of 6 patients (3 with hemifacial spasm, 2 with posttraumatic dystonia and 1 with spasmodic torticollis) significantly improved. Gabapentin was well tolerated. Somnolence was the most commonly reported side effect. CONCLUSION: Gabapentin may be useful in some patients with dystonia and hemifacial spasm. Double blind studies are clearly needed.

Acetates↗

[Clinical symptoms, diagnosis and treatment of focal dystonias].

Focal dystonias are relatively rare and significantly disabling disorders. These include cervical dystonia, blepharospasm and hemifacial spasm. The spasmodic torticollis consists of tonic posturing of the head away from its neutral position or twisting of the cervical muscles. The blepharospasm is an abnormal blinking, eyelid tic or twitch resulting from any cause. The hemifacial spasm is an involuntary unilateral twitching of the facial muscle. Patients affected by focal dystonias are predominantly females, and many times psychical stress can be revealed. The pathogenesis may involve dysfunction of the basal ganglia and brain stem although the exact mechanism remains to be elucidated. The patients need to be diagnosed and treated in centers specialized in movement disorders. Although many drug treatments can be beneficial, the most effective treatment is the local Botulinum toxin injection into the affected muscles. This neurotoxin produces temporary neuromuscular blockade, which reveals the symptoms and pain. The effect of the toxin is temporary and, therefore, the injection needs to be repeated every 6-12 weeks. The most common side effects are hypersensitivity, bleeding, hematoma, ptosis, facial spasm, dysphasia or dysarthria. With the use of proper dose and injection sites these side effects can be avoided.

Anti-Dyskinesia Agents↗

[Nuchal rigidity in children: meningitis or not?].

Three children, two girls aged 4 and 2.5 years and one boy aged 8 years, presented with nuchal rigidity and symptoms such as fever, headache and nausea. Upon investigation they had: torticollis on the bases of an upper respiratory tract infection, viral meningitis and bacterial meningitis (meningococcus type C) respectively. They all recovered well after treatment. Nuchal rigidity can be caused by many illnesses other than bacterial meningitis. Lumbar puncture should be performed when meningeal irritation is suspected. In children this can be identified using the Vincent test as well as the Kernig and Brudzinski tests.

Child↗

[Multiple pathology in a Merowingian individual of a graveyard near Insheim (Rhineland-Pfalz)].

The following case report describes in detail a 57 years old (+/- 5 years) male individual from a Franconian graveyard in Insheim, Province of Rhineland-Palatinate, dating to between the 6th and 7th century A.D. The individual displays a number of unusual pathologies. The atlas shows a complete aplasia of the posterior arch, probably resulting in a torticollis. The changed static induced a massive spondylar-arthrotic degeneration of the cervical spine on the right-lateral portion. On the fronto-parietal section two fractures are visible which were caused by a sharp and violent force. The area in question measures 7 by 10 cm. It has healed in a dislocated position. The second location displays marks of a 6 cm long sword-cut which did strike the skull obtusely on the left parietal. In addition to the above the skull displays on its right side a great number of small circular hole defects which also appear on ribs, vertebrae, clavicula and the mandible. They present the typical picture of a plasmocytoma. As an expression of a secondary hyperparathyreoidism one can recognize ossifications of soft tissue on the calcaneus, tibia, fibula and patella as well as intravital loss of mandibular dentition. It is quite remarkable that the individual reached a relative old age despite of his numerous and serious physical encroachments due to the apparent and obvious intentions of his aggressive contemporaries to kill him.

Bone and Bones↗

[Neurootological diagnostic in children by means of cranio-corpo-graphy (author's transl)].

Among the different tests of the neurootological test battery, especially Cranio-Corpo-Graphy, is very useful for objectively investigating children. This method can be performed together with the Unterberger's stepping procedure. Then the blind-folded patient has to make 80-100 steps on the spot. This procedure should be completed within 1 min. For the fotographic recordings lights are installed in a helmet which is placed on the head and are also attached to both shoulders. The Polaroid-film is exposed during the whole duration of the test. Thus the body and head movements can be recorded as light tracings. For evaluating the tracings quantitatively it is necessary to superimpose in a second exposure a coordinate system. We usually take a polar coordinate system which is been produced in the hite of the head of the patient. Typical reactional patterns are used for differentiating peripheral from central or combined peripheral and central vestibular spinal lesions. Especially the Unterberger's stepping procedure is apt to be applied on the children's gaming behaviour. In cases of congenital or early children's torticollis, the amount of angular deflection of the head can be measured by means of Cranio-Corpo-Graphy.

Age Factors↗

[Transverse fetal position syndrome--a combination of congenital skeletal deformities in the newborn infant].

We describe a pattern of connatal postural deformities observed in approximately 0.6% of otherwise healthy, mature newborn infants. It comprises unilateral flattening of the skull (dolichocephalus), ipsalateral mandibular hypoplasia and torticollis, deviation of the septum nasi, pes calcaneovalgus on one and pes adductus supinatus on the other side. The deformities occur in four distinct combinations attributable to the prenatal lie of the fetus: dorsoanterior or dorsoposterior, left or right oblique lie. The deformities respond well to manipulative therapy.

Abnormalities, Multiple↗

[Cholinergizing treatment in hyperkinesis].

Hyperkineses are a clinical and pathogenetic counter-part of parkinsonism (MP). Their underlying cause is increased activity of the dopaminergic system or insufficiency of the cholinergic system. Treatment inhibiting the dopaminergic system, similarly as anticholinergic treatment is of little effectiveness in MP. A trial of substitutive treatment was undertaken activating the cholinergic system with a precursor of acetylcholine (dimethyl-amino-ethanol-deanol--Bimanol) with simultaneous inhibition of cholinesterase with prostigmin. The results of this treatment were compared with previously applied antidopaminergic treatment (Haloperidol) and with the effects of L-dopa. This treatment was given to 11 patients with Huntington's chorea (ChH), 4 with faciolingual dyskinesis (DFL), 3 with torticollis spasmodicus (TS), 3 with maladie des tics (MT) and 8 with dyskinesia following treatment with L-dopa (MP). Cholinergizing treatment gave better results than antidopaminergic treatment in TS and ChH, and worse in MT. In dyskinesia following L-dopa cholinergizing treatment gave also no effects reported by others. Differences in the results of cholinergizing and antidopaminergic treatment may indicate non-homogenous pathological mechanism of these hyperkineses. Cholinergizing treatment in hyperkineses is based on a similar principle as L-dopa treatment in MP and this approach seems to be proper but more effective preparations should be sought for.

Adult↗

[Influence of botulinum toxin treatment on previous primary headaches in patients with cranio-cervical dystonia].

INTRODUCTION: Preliminary data suggest some beneficial effects of botulinum toxin type A (BT-A) on primary headaches. OBJECTIVE: To analyze the subjective influence of BT-A on previous primary headaches of those patients receiving this treatment due to a variety of cranio-cervical dystonia (CD). PATIENTS AND METHODS: All patients receiving BT-A due to CD were interviewed with an ad hoc questionnaire on the presence or not of headaches prior to BT treatment. Diagnosis and subjective effects of BT-A on these previous headaches were recorded. RESULTS: Thirty-eight patients (28 women) were interviewed; 21 (19 women) reported a history of previous primary headache (12 migraine, 10 tension-type headache). Nine (41 %) reported sustained response ( > 50 % reduction in headache frequency) since the beginning of BT-A treatment. Patients with cervical dystonia or those receiving high doses ( > 50 U) showed the best responses. Age, gender, pain location, duration of BT-A treatment and headache diagnosis did not seem to correlate with response. CONCLUSIONS: BT-A seems to have sustained beneficial effect on primary headaches of some patients with CD, especially at doses higher than 50 U and in patients with the diagnosis of spasmodic torticollis.

Adult↗

Single fiber electromyography in normal subjects: reproducibility, variability, and technical considerations.

We performed 59 single fiber electromyography (SFEMG) examinations on 33 patients with spasmodic torticollis, but no other medical or neurological disorder. 14 patients were studied serially (3 examinations over 12 weeks). There was excessive jitter (greater than 55 microseconds) in 14 of 1180 fiber pairs (1.1%). 7 of the 14 abnormal fiber pairs were found at single sites (1 in 20 sites studied). Abnormal jitter occurred at more than 2 of 20 sites in only two patients, both of whom head increased fiber density and were more than 70 years of age. Of the 14 abnormal fibers, subtle technical problems were discovered in 4: bimodal jitter (1); borderline amplitude of single fiber potential (1); and borderline rise time (2). 6 of the 14 jittering potentials were part of a triplet or quadruplet. 4 fibers with increased jitter occurred in pairs and these were seen only in patients over 70. One patient with quiescent non-tropical sprue was excluded from consideration because of increased mean jitter before injection and confirmed during 2 subsequent studies. Therefore, SFEMG is a valid and reliable technique that must be strictly interpreted according to existing guidelines, with special consideration to studies in older patients. Abnormal jitter is rarely found in normal muscle, however, when found, it usually occurs as part of a triplet or multiplet.

Adult↗