[Horizontal torticollis and strabismus].
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Somatosensory evoked potentials (SEPs) of the median nerve were recorded in 40 patients with the tonic and tremulous form of torticollis and in 40 healthy volunteers. Polymyographic recordings of the activity of cervical muscles were performed in all patients with cervical dystonia to determine the dystonic and antagonistic muscles. Patient SEPs were recorded during abnormal head movement. SEPs in 20 healthy volunteers were recorded with the head in the middle position. SEPs in another 20 healthy volunteers were recorded with the head rotated 60 degrees to the right. The mean peak-to-peak amplitude values of the precentral P22/N30 and the postcentral N20/P25 complexes and their mean side-to-side ratios were calculated in the F3 (F4), C3' (C4'), and C3+ (C4+) electrode positions in all four groups. In patients with the tonic form of torticollis (group I), an apparent mean P22/N30 amplitude increase was found above the hemisphere contralateral to the direction of head deviation in both precentral electrode positions, F3(4) and C3(4)'. A statistically significant difference was observed between group I and other patient and control groups. In patients with the tremulous form of torticollis (group II), an increase in the mean P22/N30 amplitude was found above both hemispheres in both precentral electrode positions F3(4) and C3(4)'; a significant difference was found between group II and both control groups. Lateralization of the P22/N30 component was found only in patients with the tonic form of torticollis. The mean side-to-side ratio of the precentral P22/N30 component amplitude was significantly different when group I was compared with either group II or control groups. No significant difference between group II and either control group was found. No significant abnormalities in the postcentral N20/P25 component were found in either the dystonic patients or in healthy control subjects. These results might indicate a different pattern of cortex excitability in patients with tonic versus tremulous forms of torticollis and therefore may implicate different underlying pathophysiological mechanisms in these two forms of disorder.
We report nine patients who developed dystonia following head trauma. The most frequent form was hemidystonia only (six patients). One patient presented with hemidystonia plus torticollis, one with bilateral hemidystonia and one with torticollis only. Seven patients sustained a severe head injury, and two had a mild head injury. At the time of injury, six were younger than 10 years, two were adolescents, and the patient with torticollis only was an adult. Except in the patient with torticollis only, the onset of dystonia varied considerably from months to years. All patients with hemidystonia had posthemiplegic dystonia of delayed onset. Seven out of 8 patients with hemidystonia had lesions involving the contralateral caudate or putamen, as demonstrated by CT and MR. The patient with hemidystonia plus torticollis had no lesion to the basal ganglia, but a contralateral pontomesencephalic lesion. Response to medical treatment was generally poor. Functional stereotactic operations were performed in seven patients. A variety of factors may be responsible for the vascular or nonvascular posttraumatic basal ganglia lesions, which may lead to dystonia. The pathophysiology seems to be more complex than thought previously. We believe that dystonia following head injury is not as rare as is assumed. Awareness of its characteristics and optimized diagnostic procedures will lead to wider recognition of this entity.
107 patients suffering from axial dyskinesias (torticollis and torsion dystonia) were observed at our clinic over a period of 10 years. In 15 patients, stereotactic lesions involving the head-turning bundle at the caudatocapsular level, which mediates forced adversive movements, were performed. In 70% good to excellent results were confirmed. However, differences between forced adversive movements and torticollis were identified. There are striking motor and motivational similarities between torticollis and ritualized fixed action patterns of low-ranking subjects. The aetiology of axial dyskinesias, especially of torticollis, is multifactorial, long-term psychic stress being an important factor. The relationship of axial dyskinesias, especially of torticollis, to compulsive neuroses is discussed, and Mazar's 'adversive' target area is identified as a partial orbitomedial leukotomy.
The results obtained in a retrospective study on clinical and pharmacological aspects of 41 patients suffering craniocervical dystonia (24 with blepharospasm, 17 with torticollis) and 11 with spasm are here presented. Mean age of symptoms onset was 57.4, 43.8 and 55.8 years old respectively; this variable was comparatively higher in females than in males with torticollis. The prevalence of blepharospasm and hemifacial spasm was higher in females. A 38.7% of patients suffering blepharospasm also presented oromandibular dystonia (Meige's syndrome). Other abnormal movements less frequently associated were cephalic tremor, postural hand tremor and larynx dystonia. In three cases with blepharospasm there was family history of Parkinson's disease and in two cases with torticollis there was family history of essential tremor. The mean age of onset was lower in patients with clonic torticollis and the evolution time of symptoms was longer than in those who presented the tonic type. Clonic torticollis were less frequently associated to pain. Trihexyphenidyl (anticholinergic) was the most efficient drug in craniocervical dystonia, and clonazepam in facial hemispasm. In general, as earliest the age of onset was, as better the therapeutical response was.
34 patients with focal dystonias (13 with essential blepharospasm, 3 with Meige's syndrome, 2 with hemifacial spasm, 16 with spasmodic torticollis) were treated with botulinum type A toxin. 4 ng of botulinum type A toxin per eye were applied in the M. orbicularis oculi as first injection in the 18 patients without spasmodic torticollis. The 16 patients with idiopathic spasmodic torticollis received 10 ng botulinum toxin A in the contralateral M. sternocleidomastoideus as well as in the ipsilateral M. splenius capitis as first injection. The effect was monitored for a time period of at least 6 weeks by two subjective rating scores, a visual functional score and a global clinical impression score. Patients with blepharospasm showed a distinct improvement already after 4 days which lasted for 6 weeks. 75% of the patients with spasmodic torticollis experienced a moderate to distinct improvement after 4 days which remained stable for 6 weeks. A second injection was performed in 15 (7 blepharospasm, 8 spasmodic torticollis) patients 9-11 weeks later with a similar success. All observed side effects (weakness; stiffness of local muscles; feeling of dryness of eyes, unilateral ptosis) were mild and of transient nature. We suggest therefore botulinum type A toxin as treatment of first choice in focal dystonias.
The pathophysiology of arm tremor in patients with cervical dystonia (CD) and its relationship to other types of tremor is unclear. In the present study, we have compared the tremor in these patients with that seen in patients with essential tremor (ET) using two neurophysiological techniques: the triphasic EMG pattern accompanying ballistic wrist flexion movements; and reciprocal inhibition between forearm muscles. During ballistic wrist flexion movements, the latency of the second agonist EMG burst was later in ET than CD patients. This suggests that the mechanism of the arm tremor in CD may differ from that in ET. There was no group difference between reciprocal inhibition in patients with ET or CD. However, there was much more variability in the data from patients with CD. Because of this, we subdivided the CD patients into two groups, group A with normal levels of presynaptic inhibition and group B with reduced or absent presynaptic inhibition. A posteriori, it turned out that the patients in these two subgroups had similar clinical symptoms, but different clinical histories. The arm tremor of patients in group A started simultaneously with torticollis (mean onset age of arm tremor 40 years +/- 20.7 SD, interval between onset of arm tremor and torticollis 0 +/- 2.9 years) whereas it began much earlier (mean onset age 14 years +/- 6 SD) and preceded onset of torticollis by a longer interval (21.6 +/- 17.5 years) in patients of group B. Patients in group A also had less co-contraction in their ballistic wrist movements between the first agonist and the antagonist burst than those patients in group B. We conclude that arm tremor in patients with CD may have a mechanism different from that seen in patients with ET. Moreover, the data imply that there are two subgroups of CD patients with arm tremor, one with a late and simultaneous onset of arm tremor and torticollis (group A), and another with an early onset of arm tremor and later development of torticollis (group B). These groups do not correspond to the currently proposed clinical subdivision of 'dystonic tremor' and 'tremor associated with dystonia'.
Traumatic torticollis is an uncommon complaint in the emergency department (ED). One important cause in children is atlantoaxial rotary subluxation. Most children present with pain, torticollis ("cock-robin" position), and diminished range of motion. The onset is spontaneous and usually occurs following minor trauma. A thorough history and physical examination will eliminate the various causes of torticollis. Radiographic evaluation will demonstrate persistent asymmetry of the odontoid in its relationship to the atlas. Computed tomography, especially a dynamic study, may be needed to verify the subluxation. Treatment varies with severity and duration of the abnormality. For minor and acute cases, a soft cervical collar, rest, and analgesics may be sufficient. For more severe cases, the child may be placed on head halter traction, and for long-standing cases, halo traction or even surgical interventions may be indicated. We describe two patients with atlantoaxial rotary subluxation, who presented with torticollis, to illustrate recognition and management in the ED.
The sternomastoid "tumor" of infancy is a firm, fibrous mass, appearing at two to three weeks of age. It may or may not be associated with torticollis. Generally, the "tumor" initially grows, then stabilizes, and in about half the cases recedes spontaneously after a few months. It may leave a residual torticollis or may be associated with a facial or cranial asymmetry of a delayed torticollis. The etiology is unknown, a direct cause and effect relationship to birth trauma has been largely disproved although approximately half these children are products of breech deliveries. The treatment is controversial. Approximately half of these "tumors" will resolve spontaneously without sequelae. Progressive torticollis or development of facial asymmetry are considered indications for surgery. The purpose of this report is to acquaint the head and neck surgeon with this entity which may confront him for diagnosis and treatment.
The authors report a case of postural abnormality of the head and neck (Torticollis) in a fourteen month old infant with severe gastroesophageal reflux (GER). Disappearance of this abnormality after surgical correction of the reflux leads the authors to confirm the diagnosis of Sandifer's syndrome of which the main features are underlined. In its initial descriptions, Sandifer's syndrome was characterized by the sudden occurrence, usually during meals, of torticollis, consecutive to hiatal hernia in children. This torticollis disappears within days or weeks following surgical treatment of the hiatal hernia. It was later proved that the origin of the torticollis was not the hiatal hernia itself, but the consequent gastroesophageal reflux (GER).
A case of pseudotumour of the sternocleidomastoid muscle in a three-week old child is reported. The tumour resolved spontaneously within four months. Seventeen months later there was no sign of torticollis. This tumour is the most common cause of torticollis in newborn children. In 80-90% of the cases the tumour disappears within six months, the rest develop either a progressive or delayed torticollis, known as congenital muscular torticollis (CMT). Thus, CMT is a related condition, usually appearing at three to four years of age, and it may require surgical correction.
A protocol of combined treatment of patients with cyclotropia has been developed, including surgery and functional treatment by an original method. Combined treatment of cyclodiplopia and forced compensatory head turn (ocular torticollis) led to complete restoration of solitary vision in the entire visual field in 12 (34%) cases, in the main part of visual field in 20 (58%) cases, complete elimination of torticollis in 18 (52%) cases, and decrease of torticollis in 14 (40%) cases. Hence, the proposed treatment protocol brings about good functional results and is recommended for the treatment of patients with cyclodiplopia and torticollis.
A unique cranial asymmetry previously noted in the skeletal remains from Mokapu, O'ahu, Hawai'i, is described. The anomaly involves an indentation of one or both of the occipital condyles and facial and vault asymmetry. This examination of the asymmetry includes a search for other reported occurrences, a detailed description, and a differential diagnosis. A multiple working hypothesis approach is employed. Comparison of the osseous material with the expected clinical pictures in craniosynostosis, Kleippel-Feil syndrome, primary basilar impression, and torticollis results in the most likely explanation of congenital torticollis. A high rate of occurrence of the anomaly (1.8%) is found in the skeletal remains from the Hawaiian Islands, but it has been documented in only two instances outside Hawai'i. A survey of patients seen at The Shriner's Hospital for Crippled Children does not reveal a high rate of occurrence of torticollis in Hawaiians relative to other ethnic groups.