PubMed HealthSearch

Biomedical subjects

R Stell

Publications and source records attributed to R Stell.

13 recordsLinked to original sources

Botulinum toxin treatment of spasmodic torticollis.

We reviewed the efficacy and adverse effects of repeated botulinum toxin injections into hyperactive neck muscles of 107 successive patients with spasmodic torticollis. They received 510 injection treatments over a median period of 15 months (range 3-42 months). One patient failed to benefit at all, but 101 (95%) patients reported considerable (moderate or excellent) benefit from at least one treatment. On a global subjective response rating, 93% of 429 treatments resulted in some improvement and 76% in moderate or excellent improvement. Pain reduction followed 89% of 190 treatments with moderate or excellent reduction after 66%. Median duration of benefit was 9 weeks. All torticollis types responded equally well and injections into two (or more) involved neck muscles were more effective than injection into a single muscle. The most frequent adverse effect was dysphagia, occurring after 44% of all treatments, but this was severe after only 2%. Antibodies to botulinum toxin were detected in the serum of three out of the five patients in whom loss of treatment efficacy occurred. We conclude that botulinum toxin treatment is the most effective available therapy for spasmodic torticollis and practical advice is provided for anyone wishing to set up the technique.

Adult

Cervico-ocular function in patients with spasmodic torticollis.

The cervico-ocular (COR) and active and passive vestibulo-ocular reflexes (VOR) were measured in seven patients with spasmodic torticollis (ST) and six normal controls. The COR was found to be weak or absent in both groups. The VOR gain was similar in the two groups but five patients had a significant asymmetry of the response. There was no evidence of abnormal cervico-vestibular interaction during active head rotation. The study suggests that the VOR asymmetry frequently found in ST cannot be explained on the basis of an abnormal cervical input.

Adult

Ataxia telangiectasia presenting as an extrapyramidal movement disorder and ocular motor apraxia without overt telangiectasia.

Ataxia telangiectasia may present with few, if any, of its typical extraneurological manifestations, but the combination of an extrapyramidal movement disorder, ocular motor apraxia with head thrusting and cerebellar incoordination is characteristic. In this sporadic case there was no overt immune dysfunction, oculocutaneous telangiectasia were inconspicuous and the neurological presentation was atypical with dystonia predominating over cerebellar incoordination. The uncontrollable and disabling involuntary movements, which have not to our knowledge been described in ataxia telangiectasia before, showed a partial response to moderately large doses of benzhexol, but were refractory to all other medications. Treatment in the future is to be with increasing doses of benzhexol until the dystonia is controlled or larger doses cannot be tolerated.

Adult

Saccadic function in spasmodic torticollis.

Twelve patients with idiopathic spasmodic torticollis were compared with 19 normal controls on tests of saccadic eye movements thought to depend upon normal basal ganglia function. The patients were able to make random, predictive, remembered, and self-paced saccades equally as well as control subjects. This suggests that those parts of the basal ganglia which may be damaged in spasmodic torticollis, are separate from pathways responsible for the normal initiation and execution of saccades.

Adult

Ataxia telangiectasia: a reappraisal of the ocular motor features and their value in the diagnosis of atypical cases.

The eye movements of four patients with ataxia telangiectasia (AT), three of whom had an unusual neurological presentation, were studied. All had striking abnormalities of saccadic generation with markedly hypometric saccades, increased saccadic latency, but normal saccadic velocity. Three patients used head thrusts to aid refixation. In addition, there was absence of smooth pursuit and optokinetic nystagmus, and hyperactive vestibular responses. Two of the four patients had, in addition, periodic alternating nystagmus. This combination of an ocular motor apraxia with superadded cerebellar ocular motor abnormalities, and possibly periodic alternating nystagmus, should strongly suggest the diagnosis of AT, even if the clinical syndrome is otherwise atypical.

Adult

Vestibulo-ocular abnormalities in spasmodic torticollis before and after botulinum toxin injections.

In order to establish whether vestibular abnormalities often found in spasmodic torticollis are secondary to the abnormal head posture, the vestibulo-ocular reflex (VOR) was studied in eight patients before and after correction of head posture with botulinum toxin. Eye movements were recorded in the dark during sinusoidal and velocity step rotation. Four patients showed a significantly asymmetric response, with the slow phase of the VOR more active ipsilateral to the torticollis (chin). Despite significant improvement of the head posture in all patients for up to 10 weeks following treatment, no correction of the vestibular asymmetry occurred. This suggests that the VOR abnormalities are not caused by the head posture itself. We interpret the findings as evidence of primary involvement of the vestibular system in torticollis and we postulate a widespread derangement of the sensory-motor mechanisms controlling head posture in this disease.

Adult

An exteroceptive reflex in the sternocleidomastoid muscle produced by electrical stimulation of the supraorbital nerve in normal subjects and patients with spasmodic torticollis.

We examined suppression of EMG activity in the contracting sternocleidomastoid muscles, produced by electrical stimulation of the supraorbital nerve in 10 normal subjects and 9 patients with spasmodic torticollis. This exteroceptive reflex in the sternocleidomastoid muscle consisted of 2 or 3 phases: (1) an early, small, and unstable phase of facilitation, followed by (2) a period of suppression beginning 35 msec after the stimulus, lasting for 35 msec with a reduction in EMG activity to approximately 40% of the prestimulus level, and (3) a further phase of facilitation at a latency of 70 msec, with duration 35 msec and an increase in EMG activity to approximately 35% above prestimulus levels. The latency and duration of the suppressive phase of this reflex were similar to the exteroceptive suppression of EMG activity in the masseter muscle after supraorbital nerve stimulation (masseter silent period). In patients with spasmodic torticollis, the depth of this exteroceptive suppression in the sternocleidomastoid muscles was less than that observed in an age-matched cohort of normal subjects, although the latency and duration were normal. In contrast, exteroceptive suppression in the masseter muscle was normal. These findings suggest abnormal function of inhibitory interneuronal networks between the 5th cranial nerve and the motor neurons of the spinal accessory and upper cervical nerves which mediate exteroceptive suppression in the sternocleidomastoid muscle in patients with spasmodic torticollis.

Adult

Botulinum toxin in spasmodic torticollis.

Ten patients with spasmodic torticollis were treated by injection of a total dose of 30 ng of botulinum toxin type A into the affected sternomastoid and posterior cervical muscles. Nine patients reported improvement in head position and control, which was confirmed in seven cases by clinical assessment and "blind" videotape ratings before and 6 weeks after injection. Five patients who had pain reported relief. Seven patients had mild transient dysphagia after injection; two who were given a more concentrated solution of the toxin developed more severe dysphagia, but this also recovered. Other minor transient side effects included weakness of the voice and local pain. The beneficial effects of botulinum toxin injections lasted some 2 to 3 months. A slight reduction in the total dose of toxin injected avoided the main side effects, and this method of treatment appears to offer successful control of head position and pain in the majority of patients with torticollis.

Adult

A double-blind trial of hyperbaric oxygen in the treatment of multiple sclerosis.

A randomized double-blind trial of hyperbaric oxygen (2 atmospheres absolute for 20 sessions of 90 minutes, over a period of one month) demonstrated no advantage over a placebo gas mixture in the treatment of multiple sclerosis. Of 41 patients completing the trial, 21 had been treated with oxygen, eight of whom reported improvement (two confirmed objectively). Of 20 patients in the placebo group, seven claimed improvement (four confirmed objectively). Even when patients with mild disease were considered separately, or when functional systems were assessed individually, no benefit could be shown to result from hyperbaric oxygen therapy.

Adult