Botulinum toxin type B: a double-blind, placebo-controlled, safety and efficacy study in cervical dystonia.

We enrolled and treated 122 patients with idiopathic cervical dystonia in a double-blind, placebo-controlled safety and efficacy study of botulinum toxin type B (BotB). Both A-responsive and A-resistant patients were enrolled. Patients received intramuscular injections of either BotB (2,500 U, 5,000 U, or 10,000 U) or placebo. The primary outcome measure of efficacy was the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-Total score at 4 weeks following study drug administration. Secondary measures of efficacy were TWSTRS-Severity, -Disability, and -Pain subscale scores, and Analog Pain Assessment, Investigator Global Assessment, Patient Global Assessment, and Sickness Impact Profile scores. Duration of effect was estimated with an intent-to-treat analysis of responders. Safety measures included clinical parameters, laboratory tests, and adverse events. The primary and most of the secondary analyses indicated a statistically significant treatment effect and a dose response. BotB is safe, well tolerated, and efficacious in the treatment of cervical dystonia at the doses tested.

Long-term outcome of iontophoresis treatment for torticollis.

In 1969, a patient with torticollis was successfully treated by a procedure called iontophoresis. Between 1958 and 1972 223 patients with torticollis were treated with this procedure. 88 of these patients (39%) were located and 56 (25% of the total, 64% of those located) returned a survey regarding their condition. 28 (13% of the total, 32% of those located, 50% of those responding) enjoyed an initial period of improvement. Of these, 16 patients (7% of the total, 18% of those located, 29% of those responding, 51% of those with initial improvement) reported sustained improvement through the time of the survey. However, 5 of these patients had additional treatment with botulinum toxin or selective denervation. We conclude that the long term effectiveness of iontophoresis treatment in the course of torticollis is minimal.

Epidemiology and outcome of cervical dystonia (spasmodic torticollis) in Rochester, Minnesota.

The natural history of cervical dystonia (spasmodic torticollis) was investigated in a population-based study in Rochester, Minnesota. Eleven new cases were identified with onset during the 20-year period 1960-1979. The overall incidence rate was 1.2 per 100,000 person-years (95% confidence interval 0.5-1.9) with a female:male ratio of age-adjusted incidence rates of 3.6:1. A unitary etiology was not apparent: injury antedated onset in four of the 11 patients, whereas six had documented thyroid disease and four had diabetes. A family history of movement disorder was recorded for only one subject. Only one of the cases would have been classified as moderate in severity; the others were mild. In follow-up through 1993, progressive disability was noted in only two patients, and two others went into remission. Three cases of intracranial aneurysm were confirmed, two of which produced fatal subarachnoid hemorrahage. A third death was due to amyotrophic lateral sclerosis.

Neurobiological correlates of learning disorders.

An anatomic pattern of (1) temporal lobe symmetry and (2) left more than right frontosylvian cortical anomalies has been documented in a small number of children with developmental disorders of reading. These alterations occur during embryogenesis and are the direct result of neither genetic factors nor perinatal stress. Underperformance in reading, writing, and other linguistic skills as well as visuo-spatial excellence may result from these changes. Related brain mechanisms may influence disorders affecting attention, arithmetic, and nonverbal learning. The suspected association between depressed mood and certain learning disorders raises the possibility of joint cause. Future work in learning disorders must define affective state precisely, specifically characterize learning disorder, and wisely apply technology to reveal anatomic, physiological, and metabolic correlates.

Spasmodic torticollis: clinical and biologic features and their implications for focal dystonia.

A previous investigation of IST in Rochester, Minnesota, from 1960 through 1979 yielded an incidence of 1.1/100,000 person-years. The rate in women was almost three times higher than that in men. Thyroid disorders, other endocrine disorders, right-hand preference, symptomatic ovarian cysts, other disorders of the female genito-reproductive system, emotional depression, cerebral aneurysm, and family history of diabetes mellitus were common. In 347 cases of IST personally evaluated (151 seen between 1969-1971, a sample of 49 seen in 1978, and 147 other cases evaluated in 1985) the female-to-male ratio was 1.6:1. The mean age at onset was 43.5 years for women and 42 years for men, and the distributions of age at onset were virtually identical for the two sexes. Thyroid disorder was prevalent in all three referral patients samples. The increased frequency of thyroid disorder was due to a high overall frequency in female patients, 28.4% (95% confidence interval, 22.3-34.4%). Thyroid disorders were 4.5 times more frequent in women with IST than in men with IST. In the control population of 61 patients with peripheral sensory neuropathy, in which sex distribution and mean age at onset of symptoms were comparable to those in IST patients, the incidence of thyroid disorder overall was only 8.2% (in women, 6.6%). Only 12 of the 347 referral IST patients were non-right-handers. Five of these 12 were female patients (2.3% of the 215 women; 95% confidence interval, 0.3-4.3%), significantly below the anticipated frequency (10%) or the observed frequency (10% and 15.16%) in two attempted control populations. Light eyes seem to be unusually common in IST patients, as are personal and family histories of essential tremor. These results may reflect shared biologic risk factors which relate age, gender or estradiol/testosterone function, cerebral anatomic and chemical lateralization, endocrinopathy including elevated thyrotropin levels, immune status, emotional depression, common cells of origin in the neural crest, race, and familial occurrence to IST and other focal dystonias and essential tremor.

Neurologic analysis of face pain.

Analysis of face pain is predicated on knowledge of the structures of the face, head, and neck that may generate this sensation. Of particular importance in this process are historical data; characteristics of the pain, as elicited in history taking, should lead to the definition of recognized syndromes that may threaten a patient's survival or function and for which appropriate intervention may bring relief. Among these syndromes are trigeminal neuralgia and neuropathy, cluster headache, cranial arteritis, nasopharyngeal carcinoma, and psychogenic face pain.