We studied 300 patients, 61% women, with mean age 49.7 years and mean duration of dystonia 7.8 years, to determine the demographic and clinical characteristics of cervical dystonia (CD) and its relationships to other movement disorders. Torticollis was present in 82%, laterocollis in 42%, retrocollis in 29%, and anterocollis in 25%; however, the majority (66%) had a combination of these abnormal postures. Scoliosis was present in 39%, local pain reported by 68%, and 32% had evidence of secondary cervical radiculopathy. In addition to CD, 16% of patients had oral dystonia, 12% mandibular dystonia, 10% hand/arm dystonia, and 10% had blepharospasm. Tremor was noted in 71% of patients; head-neck tremor was present in 60%, and tremor in other body regions was present in 32%. A family history of a movement disorder was present in 44% of the CD patients. Tardive dystonia was the cause in 6%; 11% had posttraumatic dystonia. Anticholinergic drugs provided moderate improvement in 33% of patients, but local intramuscular botulinum toxin injections relieved CD, local pain, or both in over 90% of all treated patients.

Botulinum toxin antibodies (ABS) may be a reason why occasionally patients do not have a response to injections with botulinum toxin type A (BTX). We tested 86 patients with cervical or oromandibular dystonia for the presence of BTX ABS; 20 were positive and 66 were negative. All patients who tested positive had no response to BTX injections on at least two consecutive treatment sessions. When compared with 22 randomly selected patients with negative BTX ABS results, the patients with positive BTX ABS tests had an earlier age at onset (mean age: 31.8 +/- 16.7 years versus 43.4 +/- 10.5; p < 0.05), higher mean dose per visit (249.2 +/- 32.5 U versus 180.8 +/- 68.7, p < 0.0005), and higher total cumulative dose (mean dose: 1,709 +/- 638 U versus 1,066 +/- 938; p < 0.01). Four out of five patients with positive ABS tests later had a response to botulinum toxin type F injections. Of 26 patients with negative BTX ABS results who were tested because of poor response on at least one visit, 21 had good response after subsequent injection and five had no effect. Except for young age at onset and higher dosages, there were no other factors that could reliably predict which patients would become immunoresistant to BTX type A injections. Treatment with alternate serotypes may offer clinical benefit to this group of patients. Absence of detectable BTX ABS may occur in patients with poor response to BTX injections because of inadequate dosage, injections of inappropriate muscles, or poor sensitivity of the BTX ABS bioassay.

(J Neurol Neurosurg Psychiatry 1999;67:646-650)

Pressure overload induces cardiac growth in the rat, which implies the hypertrophy of cardiac muscle cells and proliferation of nonmuscle cells. The cardiac cell loss observed in parallel has generally been attributed to necrosis. Using an in situ assay, we demonstrated a phase of apoptosis or programmed cell death during the first 7 d after pressure overload with a peak at day 4 while cardiac growth continued for over 30 d. The increase in apoptosis was confirmed by quantification of 180-1500-bp DNA oligonucleosomes with agarose gel electrophoresis and in situ labeling via 3 Ј -terminal deoxynucleotidyl transferase assay. While some apoptosis was observed in the basal state in nonmuscle cells, pressure overload induced apoptosis mainly in cardiomyocytes. These data suggest that cardiac hypertrophy is initiated by a wave of apoptosis of cardiomyocytes. Thus, apoptosis may be involved in the pathogenesis of heart remodeling. ( J. Clin. Invest. 1996. 97:2891-2897.) Key words: apoptosis • heart • hypertrophy • aortic stenosis • pressure overload

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