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Biomedical subjects

P Flachenecker

Publications and source records attributed to P Flachenecker.

31 records · Page 2Linked to original sources
PubMed1998

Selective degeneration of sudomotor fibers in Ross syndrome and successful treatment of compensatory hyperhidrosis with botulinum toxin.

We report a 5-year follow-up of a patient with Ross syndrome. A biopsy of the anhidrotic skin immunostained with protein gene product 9.5 visualized by confocal microscopy revealed selective loss of sudomotor fibers, whereas epidermal innervation remained intact, providing the first morphologic evidence of selective loss of sudomotor fibers in this syndrome. Among the different treatment strategies employed for the patient's disabling segmental hyperhidrosis, intracutaneous injection of botulinum toxin A was the most helpful.

Adaptation, Physiological↗
PubMed1998

Treatment of severe myasthenia gravis with protein A immunoadsorption and cyclophosphamide.

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction caused by antibodies against the nicotinic acetylcholine receptor (AChR-Ab). We report a 16-year-old girl with severe MG who showed a poor response to plasma exchange and tryptophan-linked polyvinylalcohol gel immunoadsorption. Further improvement of muscle strength and decline of AChR-Ab could be achieved after initiation of protein-A immunoadsorption (PA-IA). Maintenance therapy with PA-IA and intravenous pulses of cyclophosphamide resulted in a stabilisation of the disease, with a complete remission during the follow-up period of six months. We suggest that PA-IA may be valuable and safe in the management of patients with severe MG, and maintenance therapy with PA-IA and cyclophosphamide may prevent serious and potentially life-threatening relapses of the disease.

Adolescent↗
PubMed1997

Quantitative assessment of cardiovascular autonomic function in Guillain-Barre syndrome.

To study the temporal course and the relationship between autonomic failure and motor weakness in Guillain-Barré syndrome (GBS), a total of 164 autonomic function tests were serially applied for up to 1 year in 13 consecutive patients. Results were compared with those obtained in 25 healthy volunteers and 13 patients with other neurological diseases. Parasympathetic function tests comprised heart rate responses to Valsalva maneuver, deep breathing, and active change of posture, whereas sympathetic vasomotor function was assessed by blood pressure responses to handgrip and standing. At the height of the disease, subclinical autonomic involvement of both parasympathetic and sympathetic arms was revealed in the vast majority of patients. Abnormalities of autonomic function tests improved gradually over time, paralleling the recovery of motor function, but were only partially related to clinically significant autonomic dysfunction. Correlation analysis suggested that tachycardia in the context of GBS might be caused by a reduction of sympathetically mediated peripheral vascular tone rather than by vagal failure.

Adult↗
PubMed1997

Distal muscular dystrophy of Miyoshi type. Report of two cases and review of the literature.

Miyoshi myopathy (MM) is a rare distal myopathy that mainly occurs in Japan. And that is characterized by prominent involvement of the gastrocnemius muscles. Here we report two patients, brother and sister, from a German family. Onset of the disease was at the age of 20 and 22 years, respectively. In both siblings, there was an early and predominant involvement of the gastrocnemius muscles. Creatine kinase activity was elevated 37- to 95-fold above normal. Electromyography revealed fibrillations, positive sharp waves and a myopathic pattern, particularly in the distal muscles of the lower limbs. Histology of the gastrocnemius muscles showed myopathic changes consistent with muscular dystrophy. Occurrence in these two siblings but in no other family members was indicative of an autosomal-recessive inheritance. Our report indicates that MM may also be found in Germany, and that it should be considered in the differential diagnosis of distal myopathies.

Adult↗
PubMed1997

Power spectrum analysis of heart rate variability in Guillain-Barré syndrome. A longitudinal study.

Power spectrum analysis of heart rate variability was repeatedly carried out on 13 patients with Guillain-Barré syndrome for up to 1 year by Fourier analysis of regular beat-to-beat (R-R) intervals which were recorded for 5 min, converted into a continuous function by linear interpolation and resampled at 5 Hz. Low-frequency (LF) power (reflecting a mixture of parasympathetic and sympathetic activity) and high-frequency (HF) power (reflecting parasympathetic tone) were calculated by integrating the spectra from 0.04 to 0.15 Hz and from 0.15 to 0.4 Hz, respectively. At the height of the disease, the HF component was significantly decreased. The LF:HF ratio, which has been suggested to be an indicator for sympathetic activity, was increased compared with the follow-up value after 1 year. Both measures returned to normal gradually over time. Pooled-data analysis suggested that both HF and LF power were significantly related to the responses of standardized parasympathetic function tests, while the LF:HF ratio was inversely correlated with sympathetic vasomotor activity. In patients presenting with tachycardia, LF and HF power were strikingly decreased compared with patients with normal heart rates, while in patients showing vagal over-reactivity, the power of both spectral bands was significantly increased. The results suggest that spectral analysis of heart rate variability is useful for investigating the cardiovascular neural regulation in patients with Guillain-Barré syndrome. In this disorder, the sympathovagal balance is clearly shifted to sympathetic predominance at the height of the disease.

Adult↗
PubMed1996

Eyeball pressure testing in the evaluation of serious bradyarrhythmias in Guillain-Barré syndrome.

OBJECTIVE: To investigate the usefulness of eyeball pressure testing (EP) as an indicator for impending serious bradyarrhythmias in patients with Guillain-Barré syndrome (GBS) and its relationship to motor disability. BACKGROUND: Autonomic dysfunction is a common complication in GBS and accounts for a significant number of deaths. Serious bradyarrhythmias are thought to occur only in severe cases but are difficult to predict. METHODS/DESIGN: In 13 consecutive patients with GBS aged 29 to 70 years, 156 EP (6 to 19 per patient) were done serially for up to 1 year. Bilateral moderate pressure was manually applied and sustained for 25 seconds or until abnormal bradycardia developed, defined as heart rate below 40 beats per minute. Disability was graded by a score from 0 to 5 (DS). RESULTS: Four of 13 patients (DS 2/2/3-4/5) showed abnormal sensitivity to EP at least once. In two of them, vagal overreactivity could be demonstrated repeatedly, which gradually resolved within 4 and 10 days. In one patient with a rapid progressive course requiring early cardiopulmonary resuscitation, a highly abnormal EP could be recorded until 1 day after heart arrest. Another patient (DS 3-4) with abnormal EP required cardiac pacing twice because of significant bradycardia. The only other event necessitating pacing occurred in a severely disabled patient (DS 5-4) who never showed abnormal EP. CONCLUSIONS: Vagal overreactivity could be demonstrated in approximately 30% of our patients. It was not restricted to severe motor impairment and was also present in mild-to-moderately disabled patients. In this regard, EP may be a simple and useful bedside test to indicate an increased risk of developing serious bradyarrhythmias in patients with GBS.

Adult↗
PubMed1996

[EDMUS--a new European databank for multiple sclerosis. A brief introduction of ongoing and planned multicenter studies within the scope of the "European Concentrated Action for Multiple Sclerosis"].

EDMUS, the European Database for Multiple Sclerosis (MS), was established on the occasion of the first European Concerted Action for MS under the auspices of the European Community. The system is user-friendly and makes it possible to record and retrieve data relevant to MS. Within the framework of another European Concerted Action, several international multicenter trials are currently in progress. EVALUED, a validation of the EDMUS system, assesses the interrater variability; PRIMS examines the influence of pregnancy and the post-partum period on the course of MS; and PRESTIMUS investigates predictive factors in suspected MS. Further, planned studies include ERAZMUS (early azathioprine and interferon-beta treatment in MS) and AZASTOP, which is designed to evaluate the effect of stopping azathioprine treatment.

Azathioprine↗
PubMed1996

[Course of illness and prognosis of multiple sclerosis. 1: The natural illness course].

This review summarizes the natural history of multiple sclerosis (MS) in terms of disease type, survival, relapse rate and recovery as well as disability. The course of Ms is highly variable and individually difficult to predict. In epidemiological studies, the most common type of MS is the relapsing-remitting form. However, after 10-15 years of disease duration, 30-40% of patients will develop chronic progressive MS. Survival of all MS patients is only mildly affected, but markedly decreased in severely disabled patients mainly due to secondary complications. Suicide is more frequent in MS patients than in normals, especially within the first 5 years of the disease. The mean relapse rate ranges from approximately 0.5 to 0.8 relapses per year and decreases with time. Most patients will recover from relapses within 4 weeks. 50% of patients will require some form of walking aid within 15-18 years from onset. 20-40% of patients have ,,benign" MS, i.e. no or only mild disability after 10 years of MS.

Activities of Daily Living↗
PubMed1996

[Course of illness and prognosis of multiple sclerosis. 2: Predictive value of clinical and paraclinical factors].

The second part of this review summarizes the predictive value of demographic factors, the early clinical course and paraclinical methods in the prognosis of multiple sclerosis (MS). The chronic progressive course is generally thought to be associated with a worse outcome compared to relapsing-remitting MS. Moderate disability within 5 years, residual pyramidal and cerebellar deficits 6 months following an acute attack, motor, cerebellar and possibly brain stem exacerbations as well as frequent relapses were found to indicate an increased risk for developing severe disability or increased mortality. Magnetic resonance imaging (MRI), evoked potentials and cerebrospinal fluid findings were not found to be predictive in clinically definite MS, although there was a weak association of MRI findings and disability. However, these paraclinical modalities were important methods to predict the further development of clinically isolated demyelinating syndromes. In this regard, MRI was identified as the strongest predictive factor of the conversion to definite MS.

Brain↗
PubMed1995

[Multiple sclerosis and pregnancy. Overview and status of the European multicenter PRIMS study].

This work summarizes the design and state of recruitment of the European multicentre prospective PRIMS (PRegnancy In Multiple Sclerosis) project on the relationship between pregnancy and multiple sclerosis. Relapse rate, long-term effects, influence of delivery and breastfeeding as well as immunomodulation during pregnancy and puerperium are reviewed. The results suggest an increase in relapse rate during the first 3 months of puerperium with an equal or lessened risk during the 9 months of pregnancy. Overall, long-term disability seems not to be increased. The mode of delivery or breast-feeding has no adverse effects on the course of the disease. Immunological phenomena during pregnancy do not correlate to the altered course of MS.

Breast Feeding↗
PubMed1993

A new method for analysis of heart rate variability: counting statistics of 1/f fluctuations.

1/f fluctuations in heart rate are usually measured spectral analysis using Fast Fourier Transform. Here, we introduce a new method based on counting the number of QRS complexes in time intervals delta t of various length. For a 1/fb spectrum the variance of counts in delta t follows a power-law as delta t1+b. This method is applied to analyze long term fluctuations in heart rate variability in 10 healthy men. The results show periods of 1/f fluctuations with interpolated periods of white noise during night hours.

Adult↗