11/296. Bilateral sternocleidomastoid tumors of infancy. The sternocleidomastoid tumor of infancy (STOI) is a relatively uncommon condition. Typically, it presents as a firm, well circumscribed mass within the sternocleidomastoid muscle (SCM) in infants 1-8 weeks of age and may be associated with torticollis. This condition must be considered in any infant with a lateral neck mass. The diagnosis can often be made clinically, but unusual presentations may present diagnostic challenges. Although bilateral involvement is rare, it does occur. The second reported case, a 2-week old female with bilateral STOIs and torticollis, is reported. Although many of the characteristics of the masses suggested the condition, the bilateral nature added uncertainty to the clinical impression, and magnetic resonance imaging (MRI) was used to confirm the diagnosis. The clinical presentation and management of the STOI are reviewed, and the unusual features of this case are discussed. ( info) |
| We report here four children (three girls, one boy) with head tremor followed longitudinally, ages 15 months to 11 years, with follow-up over 1 to 8 years. Each demonstrated onset of head tremor between the ages of 5 and 10 months. In each case head tremor was characterized by a predominant "yes-yes" or "no-no" movement of the head. In two of the children the movement was slightly skewed with chin movement toward the shoulder. Oscillations were at a frequency of about 1 to 2 Hz. They were accentuated when sitting upright without head support, increased at times of movement, and dissipated while lying flat or sleeping. The children were unable to voluntarily suppress the action and did not experience any sensation of movement. Three of the children had shuddering spells prior to onset of head tremor. Two children have developed mild dystonic posturing of the legs when intently concentrating. Their general and neurologic examinations were normal. Normal investigations included brain magnetic resonance imaging and computed tomography, urine amino acids and organic acids screening, serum lactate, erythrocyte sedimentation rate, antinuclear antibodies, and ceruloplasmin and copper levels. A family history of tremor was present in two children, maternal epilepsy in one child, and infantile shuddering occurred in the father of one child. Therapy included trials of selective and nonselective beta-adrenergic blockers, alpha-adrenergic agonists, anticholinergics, anticonvulsants, and amantadine. One child responded well to both timolol and trihexyphenidyl. A second child responded moderately to primidone. Two have not been treated. Two have had head tremor spontaneously remit. We conclude from this small series of children with head tremor that it can evolve from a prior history of shuddering spells, occurs in the context of a positive family history of tremor, and can be accompanied by the development of a mild dystonia. Therapeutic response is variable to multiple agents. Spontaneous remission occurs, suggesting a benign course. ( info) |
13/296. Refractory torticollis after a fall. Though multiple medical and psychiatric causes of torticollis have been described, cervical dystonias resulting from distant somatic dysfunctions have not. This article describes the treatment of a 62-year-old woman in whom refractory retrotorticollis of surmised pelvic etiology developed after a fall. Structurally, cervical dystonias have been addressed as problems that originate in the head and neck, but this limited view of the musculoskeletal component of torticollis may prevent physicians from directing osteopathic manipulative treatment to the underlying problem. ( info) |
14/296. Unilateral pallidal stimulation in cervical dystonia. Cervical dystonia (spasmodic torticollis) is a focal dystonia of the cervical region. Various treatment modalities have been performed with variable success rates. We present a 42-year-old woman complaining of involuntary head rotation for the last 3 years. Different medical treatments had been used for 3 years. Botulinum toxin injections resulted in temporary and moderate improvement for periods of 3-4 months. Pallidal stimulation was performed using a quadripolar electrode and a battery-operated programmable pulse generator. We conclude that a unilateral pallidal lesion or stimulation is an effective method of treatment in focal dystonia. The target must be the pallidum contralateral to the contracted sternocloidomastoid muscle. deep brain stimulation is superior to lesioning because of the capability of manipulating the stimulation parameters which can modify the pallidotomy effect. ( info) |
15/296. Integrated EMG feedback in the management of spasmodic torticollis and focal dystonia: a prospective study of 80 patients. In summary, then, without consideration of specific circuits or transmitter agents, one can conceive of a hypothetical model that involves both learning and the functional nature of the defect in torticollis and focal dystonia to describe the results obtained. The model must be further elaborated upon and tested, preferably in a quantitative manner. Naturally, the specific finding of a defective transmitter agent (e.g., GABA) such as described in parkinsonian syndrome (dopamine) or the interruption of a specific pathway that causes and improves a dyskinesia is desirable. In this chapter we have described the use of integrated EMG feedback for the treatment of focal dystonia or spasmodic torticollis. Although we have achieved significant results, it remains clear that further research in the treatment of these disorders is required. However, since this treatment does not require medication or surgery and the possibility for significant improvement is greater than 40%, it should be attempted in patients with focal dystonia or torticollis prior to other forms of therapy. SFT should be considered as a standard mode in the medical armamentarium used for the treatment of these disorders, either primarily or in conjunction with other forms of medical, surgical, and physical therapy. ( info) |
16/296. Spontaneous atlantoaxial rotatory fixation in old age after cerebral infarction: case report. STUDY DESIGN: Case report of spontaneous Fielding and Hawkins Type I atlantoaxial rotatory fixation in a 78-year-old man after hemiplegia and homonymous hemianopsia caused by cerebral infarction. OBJECTIVES: To describe a case of spontaneous atlantoaxial rotatory fixation in old age and review previous adult cases of atlantoaxial rotatory fixation without fracture. SUMMARY OF BACKGROUND DATA: Atlantoaxial rotatory fixation in adults is a relatively rare finding and is mainly caused by trauma. To the author's knowledge, there has been no previous report of spontaneous atlantoaxial rotatory fixation in old age. methods: The patient's head was fixed in a 40 degrees left-rotated position. Left hemiplegia and homonymous left-side hemianopsia developed due to cerebral infarction. Computed tomography of the cervical spine clearly showed rotatory fixation of the atlas on the axis. RESULTS: Successful reduction was obtained after 1 day of skull traction. CONCLUSIONS: It was hypothesized that repeated left-rotational stress due to homonymous hemianopsia loaded to the atlantoaxial joint caused abnormal laxity of the joint. ( info) |
17/296. Rotary atlanto-axial subluxation with torticollis following central-venous catheter insertion. Atlanto-axial subluxation with torticollis is an uncommon condition that occurs in children usually as a result of pharyngeal infection, minor trauma, or neck surgery. Passive motion of the head and neck during general anesthesia is probably another etiologic factor. torticollis is the most common presenting physical finding. pain may or may not be present, but is commonly present with passive neck motion. Neurologic sequelae are uncommon. Our case illustrates this condition as a complication of central venous catheter (CVC) insertion in a child under general anesthesia. The surgeon should suspect this pathology when a child presents with torticollis following CVC placement. Precautions should be taken in the operating room to avoid aggressive rotation and extension of the child's neck while under general anesthesia whether or not cervical inflammation is present. Special attention to head and neck positioning should be taken in patients with Down's syndrome since they are at increased risk for atlanto-axial subluxation. The prognosis is excellent when diagnosed early. A delay in diagnosis can result in the need for surgical intervention. ( info) |
18/296. Spasmodic torticollis due to neurovascular compression of the spinal accessory nerve by the anteroinferior cerebellar artery: case report. OBJECTIVE AND IMPORTANCE: Spasmodic torticollis is a neuromuscular disorder characterized by uncontrollable clonic and intermittently tonic spasm of the neck muscles. We report a case of spasmodic torticollis attributable to neurovascular compression of the right XIth cranial nerve by the right anteroinferior cerebellar artery (AICA). CLINICAL PRESENTATION: A 72-year-old man with a 2-year history of right spasmodic torticollis underwent magnetic resonance imaging, which demonstrated compression of the right XIth cranial nerve by an abnormal descending loop of the right AICA. INTERVENTION: The patient underwent microvascular decompression surgery. During surgery, it was confirmed that an abnormal loop of the right AICA was compressing the right accessory nerve. Compression was released by the interposition of muscle between the artery and the nerve. CONCLUSION: The patient's postoperative course was uneventful, and his symptoms were fully relieved at the 2-year follow-up examination. This is the first reported case of spasmodic torticollis attributable to compression by the AICA; usually, the blood vessels involved are the vertebral artery and the posteroinferior cerebellar artery. ( info) |
19/296. A Yorkshire family with adult-onset cranio-cervical primary torsion dystonia. Although a family history is described in approximately 20% of patients, large families with adult-onset craniocervical primary (idiopathic) torsion dystonia (PTD) are rare. We report a new British family with cranio-cervical dystonia. Seventeen members of the family were examined. Five cases were diagnosed as definite PTD and one as probable PTD. Mean age at onset was 29 years (range, 19-40 yrs). The phenotype was characterized by adult-onset cranio-cervical dystonia in all affected cases. A few cases had additional voice tremor and/or postural arm tremor. The GAG deletion in the DYT1 gene was excluded in the index case. Linkage analysis was performed between the disease and several marker loci spanning DYT6 and DYT7 regions, and haplotypes were reconstructed in all subjects. Although linkage analysis was not completely informative, reconstructed haplotypes excluded linkage between the disease and either DYT6 or DYT7. This report confirms that familial cranio-cervical dystonia is genetically heterogeneous, and further studies of other PTD families with similar clinical features are needed to identify other new genes. ( info) |
| We report a case of a child with the stigmata of von Recklinghausen's disease and a plexiform neurofibroma arising from the penile shaft. ( info) |