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Biomedical subjects

F Heinen

Publications and source records attributed to F Heinen.

17 recordsLinked to original sources

Absence of transcallosal inhibition following focal magnetic stimulation in preschool children.

Focal transcranial magnetic stimulation of the hand-associated motor cortex was used to study normal healthy preschool children (n = 7; mean age, 4.6 years) and adults (n = 7; mean age, 29.4 years) under the conditions of standardized tonic voluntary contraction of small hand muscles. Callosally mediated inhibitory as well as corticospinally mediated inhibitory and excitatory motor effects were investigated. Although children had no detectable transcallosal inhibition, their corticospinally mediated postexcitatory silent period was present (mean, 140.8 +/- 30.2 msec). It was significantly shorter than in adults (mean, 192.5 +/- 32.0 msec). The motor thresholds of the cortically elicited muscle responses, measured as the lowest stimulus intensity, were significantly higher in children (mean, 89 +/- 5%) than in adults (mean, 46 +/- 6%). The corticomuscular latency of transcranially elicited motor responses revealed no difference between children and adults. These observations may reflect maturation processes in the motor system. Maturation of at least some direct corticospinal fibers occurs early in life and is followed by that of intracortical excitatory and inhibitory connections. The maturation of functionally competent callosal connections appears to occur after the age of 5 years.

Adult

Bilateral renal vein thrombosis and venous sinus thrombosis in a neonate with factor V mutation (FV Leiden).

Bilateral renal vein thrombosis and venous sinus thrombosis were diagnosed within 3 weeks of birth in a full-term neonate. Heterozygosity for a factor V mutation leading to resistance against the anticoagulatory properties of activated protein C was found. Heparin therapy led to resolution of the thrombotic manifestations. With long-term oral anticoagulation, no relapse or other thrombotic event occurred during infancy.

Anticoagulants

[Psychosomatic aspects of idiopathic spasmodic torticollis. Results of a multicenter study].

Idiopathic spasmodic torticollis (IST) is one of the most frequent dystonic movement disorders. Its classification as a focal dystonia, as well as its treatment with botulinum toxin resulted in groups of patients being regularly seen by neurologic specialists. In a multicentre study, we investigated psychosocial changes, coping and psychopathology, and their interrelations with signs, symptoms and course. 256 patients were included in the study (59.3% women, 40.7% men). The mean age was 49.1 years. Rotating torticollis occurred more often than latero-retrocollis and antero-retrocollis. A family history of IST was seen in 3.1% of the total sample. 34% of the patients had additional dystonic symptoms. Most frequently, these affected the upper extremities (13%), less often the legs. 19.1% of the patients had experienced a period of complete remission. The General Symptom Index of the SCL 90-R in 27% of the patients ranged above the double standard deviation of the normal controls, indicating a clinically significant psychopathology.

Adaptation, Psychological

Hand tremor in patients with spasmodic torticollis.

The occurrence of hand tremors in patients with spasmodic torticollis (ST) was investigated in 55 patients by clinical and quantitative measurements. Ten patients had first-order or second-order relatives affected with postural tremor. Although 40% of the patients had a medical history and clinical findings for mild postural and action tremor of the hands, only four had moderate tremor amplitudes. One patient had a severe tremor, including resting tremor, and two had mild voice tremor. A positive correlation was found between hand and head tremor. Patients with hand tremor were younger at the onset of ST than were those without. The mean amplitudes of postural tremor were only slightly higher than for the controls and much smaller than those found in classic essential tremor. The tremor caused only mild disabilities. The tremor frequencies were indistinguishable from physiologic tremor. Further analysis of the tremor records showed evidence for physiologic tremor mechanisms only. We conclude that slightly enhanced postural hand tremors are common in ST but have a low amplitude and are only rarely clinically relevant for ST patients. Although the present data support the notion of an enhanced risk of postural tremor in families of patients affected with ST, none of the criteria allowed the separation of the hand tremor of ST from other postural/action tremors and especially from enhanced physiologic tremor. Thus, the present data do not support the classification of hand tremor in ST as either "dystonic" or essential tremor.

Adolescent

Interventional neuropediatrics: treatment of dystonic and spastic muscular hyperactivity with botulinum toxin A.

Therapeutic effect of botulinum toxin A was studied in a group of pediatric patients (n = 28) aged between 6 months and 18 years. The patients were diagnosed with cervical dystonia (n = 6), adductor spasm of the hip (n = 8), spastic drop foot (n = 7) and various other focal motor problems associated with spastic muscular hyperactivity (n = 7). The mean dose of botulinum toxin A (Dysport) used to inject into the affected muscle was 22 U/kg body weight. Reduced muscular hyperactivity with a significant increase in joint mobility was achieved for dystonic (p < 0.0001) as well as for spastic conditions in patients with adductor spasm (p < 0.0002). For these patients the improved joint mobility represented a significant benefit for both daily activities and nursing care. Local paresis and local hematoma were observed in 1/28 and 1/28 patients, respectively; 1/28 patients developed a secondary non-response. However, apart from these side effects, no other adverse reactions to botulinum toxin A treatment were recorded during the treatment and observation period (12-64 months). Our results suggest that botulinum toxin A represents an effective and safe therapeutic substance for the treatment of pediatric patients suffering of focal motor problems due to dystonic or spastic muscular hyperactivity.

Adolescent

Ventricular shunt infection and meningitis due to Bacillus cereus.

Non-anthrax Bacillus species are usually considered to be contaminants if found in clinical specimens. Only a few patients with systemic infections due to Bacillus cereus are reported. We present the case of a 18-month old boy with a primitive neuroectodermal tumor (PNET) in the brainstem and obstructive hydrocephalus that required an outlying and subsequently a ventriculoperitoneal drain. Following contamination at the site of entry of the external drain, shunt infection and meningitis with Bacillus cereus developed. Antibiotic treatment with vancomycin failed to eliminate the bacterium from the cerebrospinal fluid, so the shunt system had to be removed. Explantation of the shunt and addition of fosfomycin to the antibiotic regimen resulted in a complete cure of the infection.

Bacillaceae Infections

Transcranial magnetic stimulation in patients with Rett syndrome: preliminary results.

Transcranial magnetic stimulation was used to investigate Rett patients (n = 31, age 2-38 years). The results were compared to healthy control persons (n = 112, age 1 month-35 years). The peripheral conduction time showed no difference between the patients and control persons. The mean central conduction time, however, was shorter in the younger patients with Rett syndrome, and this is particularly evident in the youngest. This phenomenon could be interpreted as cortical and/or spinal hyperexcitability which occurs during the early stages of Rett syndrome.

Adolescent

Zinc deficiency in an exclusively breast-fed preterm infant.

UNLABELLED: A formerly premature, exclusively breast-fed infant with severe zinc deficiency syndrome is presented. He showed the characteristic erosive skin changes, including alopecia, as seen in acrodermatitis enteropathica. In addition, he manifested a failure to thrive and irritability. The diagnosis was confirmed by reduced serum levels of zinc (2.3 mumol/l) and alkaline phosphatase (45 U/l). We consider the reduced zinc supply in the breast milk (5.7 mumol/l) as the most likely cause of the disease. Therapy consisted of oral zinc supplements (50 mumol/kg/day) for a period of 30 weeks. Symptoms and laboratory values normalized completely and did not recur on a normal diet. CONCLUSION: A diet of breast milk can, in rare circumstances, cause insufficient zinc intake resulting in severe zinc deficiency syndrome with characteristic dermatological features. Therapy consists of temporary oral zinc supplementation at a daily dose of 50 mumol/kg.

Alkaline Phosphatase

Dystonic posture of lower extremities associated with myelomeningocele: successful treatment with botulinum A toxin in a six-month-old child.

We report on a six-month-old child with severe intermittent dystonic posture of both legs associated to a thoraco-lumbar myelomeningocele. The patient presented with a combination of progressive hypertonic knee extension and hip flexion. While the mobility of the right leg improved sufficiently after physiotherapy and splinting, satisfactory improvement of the left leg could be achieved only after local injections of botulinum A toxin, allowing for adequate functional motor development.

Botulinum Toxins

[Subgroups of torticollis spasmodicus from the psychosomatic viewpoint. Results of a cluster analysis of 144 cases. German Study Group of Dystonia Research].

The question of subgroups in idiopathic spasmodic torticollis, which has been discussed in earlier studies in order to define etiologically heterogeneous patient populations has lost some of its relevance since with the injection of botulinum toxin an effective treatment is available. However, psychosocial distress is linked with spasmodic torticollis in a substantial number of patients. In order to define criteria for psychosocial interventions in addition to the treatment with botulinum toxin, a cluster analysis was carried out to identify high-risk populations in terms of psychological and social distress. Five subgroups were defined on the basis of eight variables. Two of these five groups, one group with rotational torticollis and one with laterocollis, emerged as particularly distressed by their physical complaints, the effects of their illness on various areas of life and in terms of psychological functioning. The consistency of the subgroups was tested and statistically confirmed by analysis of variance. In a cross-validation 83.02% of the ungrouped cases were predicted correctly. The authors suggest that the evaluation of psychological and social aspects of the condition should be part of the neurological assessment in order to offer appropriate support to patients, who reveal a high degree of psychological distress.

Adaptation, Psychological

[Encephalitis].

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Child

Clinical and polymyographic investigation of spasmodic torticollis.

Polymyographic recordings were used to identify the most dystonic muscles suitable for local injection with botulinum toxin in 100 patients with spasmodic torticollis (TS). Rotating TS (72% of the patients) was due to dystonic activity of the splenius muscle ipsilateral to and/or the sternocleidomastoid muscle contralateral to the side of chin deviation. One-third of these patients had also dystonic activation of the contralateral splenius muscle and, rarely, the contralateral trapezius muscle. Ten patients had laterocollis due to dystonic activation of all recorded muscles on one side of the neck. Nine patients had retrocollis due to activity of both splenius muscles and rarely additional activity in both trapezius muscles. The type of dystonic muscle activity was found to be tonic, phasic or tremulous. Besides the evaluation of spontaneous dystonic EMG activity further examination during the "geste antagoniste" or the muscle activity during rotating head movements can provide additional information. It is concluded that polymyography may provide a rationale for identifying the dystonic muscles underlying the different forms of TS. It may prove to be helpful for the successful therapy with botulinum toxin and may be useful in differentiating tremulous torticollis from other types of head tremor.

Adolescent

Treatment of spasmodic torticollis with local injections of botulinum toxin. One-year follow-up in 37 patients.

Thirty-seven patients with spasmodic torticollis (cervical dystonia) who received repeated local injections of botulinum toxin have been followed up for a mean period of 12.3 (10-29) months, during which time 138 treatment sessions were performed. Mean doses per muscle averaged 320 mouse units (mu; range 160-1000 mu botulinum toxin A prepared by CAMR, Porton Down, UK). Eighty-six per cent of patients experienced significant improvement of posture and 84% of those with pain had relief following the first injection. Muscular patterns of recurrent torticollis were relatively constant and in most patients efficacy was maintained with subsequent injections, while 15% of all follow-up sessions failed. Only 2 of 37 patients were consistent nonresponders; 22% and 10% of all sessions were complicated by transient dysphagia and weakness of neck muscles, respectively. It is concluded that local botulinum toxin injections can be a safe and efficaceous long-term treatment of spasmodic torticollis and that optimal doses should be between 200 and 400 mu/muscle.

Botulinum Toxins

Reciprocal inhibition of forearm flexor muscles in spasmodic torticollis.

Reciprocal inhibition between forearm extensor and flexor muscles was tested by means of an H-reflex technique in patients with spasmodic torticollis and normal controls. In both, patients and controls three different phases of reciprocal inhibition could be demonstrated with maximal inhibition at conditioning test intervals of 0 ms, 15 ms and 100 ms, respectively. However, the quantitative amount of this inhibition was different for the patients and the controls. Significantly less inhibition was found for the second and the third phase of reciprocal inhibition in the patient group. Discriminant analysis showed a clear separation between normal subjects and patients if the amount of reciprocal inhibition of the second and third phase were taken into account. We were not able to detect any side differences neither for the patients nor for the controls. The findings demonstrate a functional disturbance of motor control mechanisms of a clinically unaffected extremity in spasmodic torticollis. This is believed to reflect a bilateral disturbance most likely within the basal ganglia or their outflow. Therefore, our data support the idea, that spasmodic torticollis is associated with or even due to a generalized rather than a focal disturbance of motor control mechanisms.

Analysis of Variance

Ear click in palatal tremor: its origin and treatment with botulinum toxin.

We report the successful treatment of a rhythmic, continuing ear click in a patient with palatal tremor with local injections of botulinum toxin into the tensor veli palatini muscle. We could demonstrate that the ear click occurred during contraction of the tensor veli palatini, which opens the eustachian tube. Therefore, we believe that the clicking noise is due to the sudden breakdown of the surface tension within the eustachian tube. Our observations suggest that the ear click is due to rhythmic discharges of the trigeminal nucleus rather than the ambiguous nucleus.

Auditory Perception

Treatment of cerebral palsy with botulinum toxin A: functional benefit and reduction of disability. Three case reports.

Three patients with cerebral palsy are described suffering, respectively, of pes equinus, spasm of the m. teres major and flexion spasm of the hand, who were treated with botulinum toxin A. These patients demonstrate not only the local reduction of the muscular hyperactivity following treatment with botulinum toxin A but also the potential functional benefit resulting from such a treatment. Thus, local intramuscular injection of botulinum toxin A in children with cerebral palsy should be considered as part of a multidisciplinary treatment concept, since reduction of the disability and the functional improvements could have high impact on daily living activities.

Activities of Daily Living