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PubMed2001

Prospective study of swallowing function in patients with cervical dystonia undergoing selective peripheral denervation.

OBJECTIVE: To characterise swallowing function in patients with cervical dystonia with botulinum toxin treatment failure, before and after selective peripheral denervation surgery. METHODS: Twelve patients with cervical dystonia had a thorough examination including standardised assessment for cervical dystonia, scoring of subjective dysphagia, and videofluoroscopic swallow. Videofluoroscopy was scored by consensus opinion between a speech and language therapist and an independent blinded radiologist using a validated scoring system. RESULTS: Seven patients with cervical dystonia experienced no subjective dysphagia either before or after surgery, although in all these patients there was objective videofluoroscopic evidence of underlying mild to moderate oropharyngeal dysphagia preoperatively and postoperatively. The most common finding was delayed initiation of swallow. Three other patients, also without subjective dysphagia before surgery, developed postoperative dysphagia. In these patients, videofluoroscopy showed a delayed swallow reflex before surgery, which was worse postoperatively in two. The remaining two patients had mild subjective dysphagia before surgery that improved postoperatively in one and deteriorated in the other. In the first, videofluoroscopy was normal preoperatively and postoperatively, and in the second, oral bolus preparation was moderately abnormal preoperatively and swallow initiation was delayed postoperatively. Mean subjective dysphagia scores did not change significantly. Apart from a significant improvement of tongue base retraction, videofluoroscopic scores were not significantly different after surgery. Postoperatively there was significant improvement of overall cervical dystonia severity and abnormal head rotation in the group as a whole. There was no correlation between age, duration of symptoms of cervical dystonia, preoperative or postoperative cervical dystonia severity, subjective dysphagia scores, or videofluoroscopic scores. However, in the five patients with persisting anterior sagittal head shift as part of the torticollis, tongue base retraction was less likely to improve after surgery compared with those without head shift. CONCLUSION: Surgical denervation of dystonic neck muscles, leading to improved neck posture, can also improve tongue base retraction, which is a key component of normal bolus propagation. However, delayed swallow initiation, a common feature in patients with cervical dystonia, can be further compromised by surgery, leading to subjective dysphagia. In general, selective peripheral denervation seems to be a safe procedure with no major compromise of swallowing function.

Adult↗
PubMed2005

Botulinum toxin type A therapy for blepharospasm.

BACKGROUND: Blepharospasm is a focal dystonia characterized by chronic intermittent or persistent involuntary eyelid closure due to spasmodic contractions of the orbicularis oculi muscles. Other facial and neck muscles are also frequently involved. Most cases are idiopathic and blepharospasm is generally a life-long disorder. Its severity can range from repeated frequent blinking to persistent forceful closure of the eyelids with functional blindness. Botulinum toxin type A (BtA) is the current first line therapy. OBJECTIVES: To determine whether botulinum toxin (BtA) is an effective and safe treatment for blepharospasm. SEARCH STRATEGY: We identified studies for inclusion in the review using the Cochrane Movement Disorders Group trials register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, handsearches of the Movement Disorders Journal and abstracts of international congresses on movement disorders and botulinum toxin, communication with other researchers in the field, reference lists of papers found using above search strategies, and contact with authors and drug manufacturers. SELECTION CRITERIA: Studies were eligible for inclusion in the review if they evaluated the efficacy of BtA for the treatment of blepharospasm. They must have been randomised and placebo-controlled. DATA COLLECTION AND ANALYSIS: We used a paper pro-forma to collect data from the included studies using double extraction by two independent reviewers. The two reviewers separately assessed each trial for internal validity and they settled differences between them by discussion. The outcome measures used included adverse events, improvement in symptomatic rating scales, subjective evaluation by patients and clinicians, and changes in quality of life assessments. MAIN RESULTS: We found few controlled trials. They were of short duration and enrolled small numbers of patients. Because of their poor internal validity, the characteristics of the populations studied, and the types of interventions and outcomes, none of the trials fitted our criteria for inclusion. However, all these trials found BtA to be superior to placebo as did large case-control and cohort studies, which reported that around 90% of patients benefited. AUTHORS' CONCLUSIONS: There are no high quality, randomised, controlled efficacy data to support the use of Bt for blepharospasm. Despite this, other studies suggest that BtA is highly effective and safe for treating blepharospasm and support its use. The effect size (90% of patients benefit) seen in open studies makes it very difficult and probably unethical to perform new placebo-controlled trials of efficacy of BtA for blepharospasm. Future trials should explore technical factors such as the optimum treatment intervals, different injection techniques, doses, Bt types and formulations. Other issues include service delivery, quality of life, long-term efficacy, safety, and immunogenicity.

Blepharospasm↗
PubMed1978

Frontal 'oculomotor" area in alert cat. I. Eye movements and neck activity evoked by stimulation.

(1) Stimulation within cat frontal lobe elicited saccadic eye movements whose maximum velocity was significantly greater than that of normal spontaneous saccades. (2) The majority (90%) of stimulated cortical points yielded eye movements whose directions and amplitudes were independent of the position of the eye in the orbit. The direction of these eye movements depended on the site being stimulated, with a discrete and orderly representation of directions existing within the cortex. (3) A lesser number of cortical points (10%) yielded centering movements whose directions and amplitudes depended on the position of the eye in the orbit, rather than on the site being stimulated. (4) Evoked neck muscle activation frequently preceded evoked eye movements by some 15--30 msec. This timing was compatible with a coordinated head-eye orientating response. (5) On the basis of the directions, and the latencies, of evoked eye movements, the cat frontal oculomotor area could be divided into two subregions, a 'medial' and a 'lateral', (6) The 'medial' area included the mesial wall of the hemisphere with a portion of the lower lip of the cruciate sulcus, and the medial wall of the presylvian sulcus. This area yielded contraversive eye movements with shorter latencies (average 45 msec). (7) The 'lateral' area included primarily the lateral wall of the presylvian sulcus. It yielded predominantly centering eye movements, and ipsiversive movements with longer latencies (65 msec). (8) The functional characteristics of the 'medial' area, as revealed by focal stimulation, resembled those of the monkey frontal eye field.

Animals↗
PubMed1994

Hereditary distal myopathy with granulo-filamentous cytoplasmic inclusions containing desmin, dystrophin and vimentin.

A 56-year-old female and her 34-year-old daughter presented with a predominantly distal myopathy affecting the peroneal and calf muscles, neck flexors and hand muscles. Both patients and two other daughters had cardiac arrhythmias, three requiring the insertion of cardiac pacemakers. Skeletal muscle biopsies revealed a complex myopathic process with granular degeneration, rimmed vacuoles and eosinophilic cytoplasmic inclusions. Ultrastructurally, the inclusions were composed of electron dense granular material and filaments forming linear masses beneath the sarcolemma and rounded masses within the cytoplasm of the fibres. Immunohistochemistry revealed labelling of the inclusions for desmin, dystrophin and vimentin, but not for alpha-actinin, spectrin, utrophin or myosin heavy chains. This family shows a hereditary distal myopathy with some features in common with previously-reported cases in which biopsies showed cytoplasmic inclusion bodies containing desmin. This group of diseases is clinically and pathologically heterogeneous. In the present cases, the accumulation of cytoplasmic filaments may reflect a generalised disturbance of filamentous protein metabolism rather than a specific disorder of desmin.

Adult↗
PubMed1991

Protein kinase C beta-subtype-immunoreactive motor and sensory nerves in rat muscle spindle.

The localization of protein kinase C beta-subtype-like immunoreactivity (PKC-beta-LI) was studied in the muscle spindles of rat neck muscles. In the equatorial regions of muscle spindles PKC-beta-LI was detected in spiral sensory nerve endings surrounding intrafusal muscle fibers. In polar regions single PKC-beta-immunoreactive (IR) nerve fibers were found between intrafusal muscle fibers and some PKC-beta-IR motor nerve endings were seen on the surface of intrafusal fibers. These results suggest that PKC-beta may be involved in regulation of muscle contraction and tonus by modulating the sensitivity of afferent and efferent innervation of muscle spindles.

Animals↗
PubMed2003

[Clinico-radiological study of the skull deformation in the plagiocephaly without synostosis].

UNLABELLED: The aim of this study was to analyze the mechanism of the skull deformation observed in frontal and occipital plagiocephaly without synostosis. POPULATION AND METHODS: From 1987 to 2001, 96 plagiocephalies without synostosis, 72 males and 24 females, were examined. There were 72 Frontal Plagiocephalies (FP) and 24 Occipital Plagiocephalies (OP). The clinical examination of the neck muscles was performed to find out a torticolis with or without retraction. All patients underwent a 3D CT scan of the skull to exclude a craniostenosis. Cephalic Index (CI) was also calculated. RESULTS: The FP had a torticollis in 89.3% of cases associated with a muscle retraction (54.8%), a tonus asymmetry (16.6%), or in association (28.6%). The skull had a parallelepiped shape and the greater axis was oblique. The CI was 0.85. The OP had a torticollis in 50% of case associated with a retraction (28.6%), a tonus asymmetry (57.1%), or the association (14.3%). The skull had a square shape with an occipital flat and the CI was 0.95. DISCUSSION: The FP and OP are two deformations whose mechanism is different. The FP corresponds to a three plans skull asymmetry secondary to muscle traction on the base of the skull, and the OP corresponds to a posterior localized asymmetry of the skull secondary to compression. CONCLUSION: The traction and/or compression deformation start in utero or in the newborn period and the supine position is an aggravating positional factor.

Anthropometry↗
PubMed2003

Posttraumatic headache: an exploratory treatment study.

Fourteen patients with posttraumatic headache (PTHA) were treated with a comprehensive treatment package targeting headache symptoms along with associated posttraumatic stress symptoms. Treatment consisted of some or all of the following depending on headache features: thermal biofeedback, electromyography biofeedback targeting the forehead and/or neck muscles, progressive muscle relaxation, education and cognitive-behavioral therapy. Mean improvement for the treatment group was 21%, whereas mean improvement for the wait-list group was--14% indicating a worsening of headache; however, the difference between groups was not statistically significant. There was a significant between groups difference on headache-free days. Within group results were modest with 29% mean improvement by the end of treatment. The reduction in headache index was significant. Minor reductions in psychopathology, most notably anxiety, were found after treatment. This study confirmed the treatment difficulties seen in this understudied population of headache sufferers, but offered hope for symptom relief.

Adult↗
PubMed2003

Grey-scale analysis allows a quantitative evaluation of thyroid echogenicity in the patients with Hashimoto's thyroiditis.

OBJECTIVE: In the present study we have performed a grey-scale quantitative analysis of thyroid echogenicity in the patients affected by Hashimoto's thyroiditis (HT), obtaining a numerical estimate of the degree of hypoechogenicity associated with the appearance of thyroid dysfunction. MATERIALS AND METHODS: The study group included 89 patients with serum positivity for thyroglobulin (TgAb) and/or peroxidase (TPOAb) antibodies. Ultrasound (US) evaluation of thyroid gland and biochemical assay of serum thyrotropin (TSH), free-thyroxine (FT4) and free-triiodiothyronyne (FT3) were performed in all patients, and in 40 healthy subjects enrolled as control group. Thyroid echogenicity was compared with that of the surrounding neck muscles, using the grey-scale histogram analysis. The echogenicity was expressed in grey-scales (GWE). RESULTS: In HT patients, the mean of thyroid echogenicity was lower when compared to the normal thyroid (61.9 +/- 8.3 GWE vs. 71.9 +/- 3.1 GWE; P = 0.01). In all HT patients the lowest limit of thyroid echo distribution was in the echogenicity range of the surrounding muscle, the overlapping ranging between 3.4% and 95.0% (mean +/- SD 48.4 +/- 20.9%). The extension of like-muscle hypoechogenicity into the thyroid gland was significantly correlated with serum TSH values (r = 0.37; P < 0.001), serum FT4 values (r = -0.60; P < 0.001), and serum TPOAb values (r = 0.31; P = 0.004). Nobody was hypothyroid when the hypoechogenicity was less than 38.0%, whereas hypothyroidism occurred in all cases with hypoechogenicity of more than 68.9%. The receiving operating characteristic curve demonstrated that 48.3% was the best cut-off for identifying hypothyroid patients with sensitivity, specificity and diagnostic accuracy of 88.9%, 86.3% and 87.6%, respectively. CONCLUSIONS: In conclusion, the grey-scale quantitative analysis has provided a measure of thyroid hypoechogenicity associated with the appearance of hypothyroidism during the course of HT. The results of the present study would encourage the application of the computerized grey-scale analysis as complementary tool to US evaluation in the patients affected by HT.

Adult↗
PubMed1998

Myasthenic syndrome of snake envenomation: a clinical and neurophysiological study.

In this prospective study, 65 consecutive patients with neurological manifestations after snake envenomation, were examined in order to describe the natural history of the reversible nature of muscle weakness. Snake envenoming led to a completely reversible muscle paralysis involving the external ocular muscles with sparing of the pupils, muscles of mastication, facial muscles, palatal muscles, neck and proximal limb muscles. The deep tendon reflexes were preserved with no sensory abnormalities. The muscular weakness usually set in within an hour of envenomation and lasted up to 10 days, with fatigability lasting for 12 days. Respiratory muscle paralysis led to ventilatory failure needing ventilation in severely envenomed patients. Motor and sensory nerve conduction were normal with normal resting compound motor action potentials on electromyography. Repetitive nerve stimulation gave rise to a decremental response during high frequency stimulation. The edrophonium test gave negative results. These manifestations are due to abnormalities of neuromuscular transmission and are not typical of myasthenia gravis. As the exact pathophysiology of venom-related neurotoxicity is not known, it is suggested that the neurological manifestations of snake envenoming be designated a myasthenic syndrome. Further studies to isolate the neurotoxin and its mechanism and exact site of blocking at the neuromuscular junction would pave the way for the development of a novel long-acting neuromuscular blocking agent.

Action Potentials↗
PubMed1990

Two cases of organophosphate poisoning with development of intermediate syndrome.

Two cases of intermediate syndrome caused by organophosphorus poisoning are reported. Trichlorfon, propoxur (a carbamate pesticide) and fenthion were ingested in both attempts at suicide. After successful conventional therapy during the cholinergic phase, but before the time when the onset of delayed neuropathy might be expected, an intermediate syndrome developed. It affected the proximal limb muscles, neck flexors and respiratory muscles 2 d after pesticide ingestion. The two patients needed respiratory support. Recovery from the intermediate syndrome was complete in both patients, although one subsequently developed delayed neuropathy.

Adult↗
PubMed1994

Presenting symptoms and signs after whiplash injury: the influence of accident mechanisms.

OBJECTIVE: To assess the relationship between accident mechanisms and initial findings after whiplash injury. DESIGN: Cohort study. SETTING: Outpatient department, Department of Neurology, University of Berne, Switzerland. PATIENTS: A population-based sample of 137 consecutive patients referred by primary care physicians. Fractures or dislocations of the cervical spine, head trauma, and preexisting neurologic disorders were exclusion criteria. MAIN OUTCOME MEASURES: Patients were interviewed and examined within 7.2 days (SD, 3.9 days) after trauma. Analyzed accident features were position in the car, use of seat belt, head restraint and its point of head contact, damage to seat, head position and state of preparedness at the moment of impact, and type of collision. Analyzed symptoms were intensity and onset delay of post-traumatic head and neck pain; pain in the shoulders, back, and anterior neck; symptoms of neurologic dysfunction according to presumed origin--cranial nerve or brainstem, radicular or myelopathic; and a score of multiple symptoms. Analyzed signs were neck muscle tenderness and restricted neck movement, and signs of cranial nerve, brainstem, or radicular dysfunction. RESULTS: Passenger position in the car, use of seat belt, and the presence of a head restraint showed no significant relationship with findings. Rotated or inclined head position at the moment of impact was associated with a higher frequency of multiple symptoms (p = 0.045 and 0.008) with more severe symptoms and signs of musculoligamental cervical strain (p = 0.048 and 0.038) and of neural, particularly radicular (p = 0.031 and 0.019), damage. Unprepared occupants had a higher frequency of multiple symptoms (p = 0.031) and more severe headache (p = 0.046). Rear-end collision was associated with a higher frequency of multiple symptoms (p = 0.006), especially of cranial nerve or brainstem dysfunction (p = 0.00003). CONCLUSION: Three features of accident mechanisms were associated with more severe symptoms: an unprepared occupant; rear-end collision, with or without subsequent frontal impact; and rotated or inclined head position at the moment of impact.

Accidents, Traffic↗
PubMed1994

Serum and tissue tocopherol in sheep after intramuscular injection and(or) dietary vitamin E supplementation.

In a 28-d trial, 35 wethers weighing 35 to 60 kg were used to evaluate serum, liver, pancreas, kidney, diaphragm, heart, and neck muscle alpha-tocopherol concentrations after vitamin E administration as i.m. injections and(or) oral supplementation. Treatments (T) were as follows: 1) i.m. DL-alpha-tocopherol(DL-alpha-ol) d 1, 2) i.m. DL-alpha-tocopheryl acetate(DL-alpha-ac) d 1, 3) i.m. DL-alpha-ol d 1, 7, 14, and 21, 4) T 1 and oral supplementation, 5) T 3 and oral supplementation, 6) oral supplementation, and 7) control, no E supplementation. Supplemented wethers received 500 IU via injection and(or) 30 IU/kg of diet (acetate form). In a second 10-d trial, blood was periodically collected from 20 wethers weighing 32 to 47 kg that had been assigned to T as follows: 1) control, 2) i.m. DL-alpha-ol d 1, 3) i.m. DL-alpha-ac d 1, 4) T 2 d 1 and 7, and 5) T 3 d 1 and 7. Results from repeated measures ANOVA in both trials indicated that there were day x T effects (P < .0001) on serum alpha-tocopherol. Pretreatment serum tocopherol concentrations were .58 and .66 microgram/mL for Trials 1 and 2, respectively. In Trial 1, wethers receiving the four alcohol vitamin E forms showed a rapid increase in serum tocopherol between d 1 and 3, followed by a rapid decline to pretreatment concentrations for T 1, whereas T 3, 4, and 5 maintained higher than pretreatment concentrations after the decline.(ABSTRACT TRUNCATED AT 250 WORDS)

Absorption↗
PubMed1990

[Clinical and pathological studies on two patients with adult-onset nemaline myopathy].

Clinical and pathological findings of two patients, a 44-year-old male and a 54-year-old female, with adult-onset nemaline myopathy were described. Both patients showed normal motor development through their childhood; Patient 1 ran fast and was involved in powerful heavy labor until the age of 40, and Patient 2 was in good health until 49, when they began to have progressive muscle weakness. They had no family history of neuromuscular diseases. On neurological examination, they had moderate muscle weakness and atrophy in their limb-girdle and paravertebral muscles. Because paravertebral and neck muscles were preferentially involved, they had difficulty in holding the head straight up. They stood in a lordotic posture. They had neither high-arched palate nor facio-skeletal abnormalities which were common findings in congenital nemaline myopathy. Serum enzymes derived from muscle were normal and needle electromyography showed myogenic and neurogenic changes in both patients. On CT scan of the skeletal muscles, the paravertebral muscles were markedly decreased in density suggesting advanced fat tissue replacement in large areas. In patient 2 who was in more advanced stage, the quadriceps femoris, hamstrings and soleus muscles also showed the similar CT findings. Light microscopic examination of biopsied biceps brachii (Patient 1) and quadriceps femoris (Patient 2) demonstrated abundant rod-like structures in the majority of type 1 fibers. In both patients, there was a marked variation in fiber size. Type 1 fibers were atrophic and type 2C fibers increased in number.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

The influence of head rotation on human upright posture during balanced bilateral vibration.

During a balanced bilateral vibration of tensor fasciae latae muscles evoking no evident postural changes in a standing human the slow head turns resulted in a pronounced lateral common gravity centre displacements to the 'occipital' side. Neck influences were most prominent at intermediate tonic background and were lacking both during very weak and strong vibratory stimulation in contrast to usual tonic vibratory reflex which becomes stronger with the increase of frequency. Body sway was induced not only by actual head turns but also by illusory ones evoked by neck muscle vibration. The neck influences on standing posture are therefore present in healthy adults, but they require a definite level of tonic readiness for their manifestation and are incorporated in the whole system of body scheme mechanisms.

Electric Stimulation↗
PubMed1991

[Cervical sensitivity participates in the development of cortical coding of orientation].

Extraocular proprioceptive afferents associated with eye movements had been shown to be necessary during visual experience to allow the development of orientation selectivity in visual cortical neurons. In the present study, it has been demonstrated that cervical proprioceptive afferents were also involved in the process. Their suppression reduced the level of orientation selectivity reach after a controlled visual experience compared to the level observed in intact animals. Similarly, this suppression increased the deleterious effects of a deafferentation of eye muscles when both were associated. These results show that neck muscle proprioceptive afferents are involved in the process of development of orientation selectivity by visual cortical neurons.

Animals↗