[Recommendations for home and long-term ventilation].
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Evidence-Based Medicine↗
Biomedical subjects
Martin Winterholler
Publications and source records attributed to Martin Winterholler.
Botulinum toxin in patients with multiple sclerosis.
Nearly all patients with multiple sclerosis (MS) will develop spasticity in the course of their disease. This symptom accounts for most of the handicap and impairment in the quality of life. Treatment with botulinum toxin will enable an efficient and safe alleviation of spasticity and the problems involved, given a realistic definition of the therapeutic target and a graded multimodal approach. Treatment may fail for a great number of reasons that require diligent analysis. Compared with other disorders resulting in spasticity as well, MS does not constitute a monophasic disorder but is influenced by many factors. Treatment of spasticity in MS must therefore be guided by its particular aspects.
Botulinum Toxins, Type A↗
Intravascular lymphomatosis mimicking disseminated encephalomyelitis and encephalomyelopathy.
Intravascular lymphomatosis is characterized by the presence of large lymphoma cells predominantly within small vessels. This report presents two patients with diagnostically misleading neurological manifestation of this disease. Case 1, a 63-year-old man, developed a sensorimotor transverse spinal cord syndrome and encephalopathy. Lumbar puncture revealed albuminocytological dissociation. Magnetic resonance imaging (MRI) showed progression of multifocal infarct-like lesions in the brain, the thoracic cord and the medullary cone. Autoimmune inflammation was suspected, and the patient received immunosuppressive therapy with immunoglobulins, steroids and azathioprine. He died 18 months after the onset of symptoms. Case 2, a 68-year-old man, showed fluctuating aphasia, disorientation, and fever for several months. Brain MRI-scan, electroencephalography (EEG) and cerebrospinal fluid (CSF) cytology were inconclusive. Premortal biopsy of lesions in liver and right suprarenal gland showed no further characterized malignancy. He died 6 months after the first occurrence of symptoms. Autopsy of both cases revealed an intravascular lymphomatosis. Tumour cells were seen disseminated in extranodal sites including heart, lung, adrenal gland, spleen, thyroid gland and brain. An intravascular lymphomatosis should be considered when a meningoencephalitic symptomatology is unclear. A biopsy of different organs including the brain and leptomeninges should not be delayed to ensure ante mortem diagnosis and to initiate chemotherapy.
Aged↗
Botulinum toxin A (Botox) and sweating-dose efficacy and comparison to other BoNT preparations.
BACKGROUND: Botulinum toxin type A (BoNT/A) is 20-50 times more effective than Botulinum toxin type B (BoNT/B) concerning the treatment of muscular hypercontractions [Sloop, R.R., Cole, B.A., Escutin, R.O., 1997. Human response to botulinum toxin injection: type B compared with type A. Neurology 49, 189-194]. Botulinum toxins block motor nerves as well as autonomic fibres [Rand, M.J., Whaler, B.C., 1965. Impairment of sympathetic transmission by botulinum toxin. Nature 206, 588-591]. OBJECTIVE: Purpose of this study was to analyse the dose dependent reduction of sweating using the BoNT/A preparation Botox and to compare the results with our earlier results analysing Dysport [Braune, C., Erbguth, F., Birklein, F., 2001. Dose thresholds and duration of the local anhidrotic effect of botulinum toxin injections: measured by sudometry. Br. J. Dermatol. 144, 111-117] and Neurobloc (BoNT/B) [Birklein, F., Eisenbarth, G., Erbguth, F., Winterholler, M., 2003. Botulinum toxin type B blocks sudomotor function effectively: a 6 month follow up. J. Invest. Dermatol. 121, 1312-1316]. METHODS: Different doses of Botox were injected subcutaneously (n=27 healthy subjects). Planimetrical analyses of the area of anhidrosis and quantitative sudomotor-axon-reflex testing (QSART) were done after 3 weeks, 3 and 6 months. RESULTS: A threshold dose of 1.25 MU Botox led to anhidrotic skin spots after 3 weeks. The duration of anhidrosis was prolonged for 3 months when 17.5 MU and for 6 months when 50 MU were injected. Anhidrotic area size decreased with time (p=0.001), indicating partial recovery at the edges. After 3 weeks, QSART had significantly decreased to 29% of baseline. With doses of 70 MU or more it decreased to zero. After 3 months, QSART had returned to 68% of baseline and after 6 months to 87%. CONCLUSIONS: Botox dose-dependently suppressed sweating. Comparison to Dysport and Neurobloc revealed a strikingly similar efficacy after 3 weeks and 3 months for all preparations. BoNT/A in general induced a more sustained anhidrosis than BoNT/B.
Adult↗
Successful treatment of axillary hyperhidrosis with very low doses of botulinum toxin B: a pilot study.
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Axilla↗
Yawning despite trismus in a patient with locked-in syndrome caused by a thrombosed megadolichobasilar artery.
We report a 62-year-old woman with a locked-in syndrome with bilateral masticatory spasms and persistent trismus, who was still able to yawn. A vascular malformation of the basilar artery-megadolichobasilar artery (fusiform aneurysm, vertebrobasilar dolichoectasia) was determined to be the underlying cause of this rare combination of symptoms. A thrombus in the megadolichobasilaris as well as an almost total pontine infarction were demonstrated on CT- and MRI-scans. Thus, trismus may be associated with locked-in syndrome due to megadolichobasilar artery thrombus, although yawning is still possible.
Brain Stem↗
Botulinum toxin type B blocks sudomotor function effectively: a 6 month follow up.
This study analyzes the suppression of sweat gland activity by botulinum toxin type B. We injected botulinum toxin type B (between 2 and 1000 mouse units subcutaneously) in the lateral side of both lower legs in 15 healthy volunteers. Sweat tests were carried out before botulinum toxin type B injections, and at 3 wk, 3 mo, and 6 mo. We studied focal anhidrosis by iodine-starch staining and by capacitance hygrometry after carbachol iontophoresis, according to the quantitative sudomotor axon reflex test (QSART). Iodine starch staining indicated that a threshold dose of 8 mouse units botulinum toxin type B leads to anhidrotic skin spots (>4 cm2) after 3 wk. Duration of anhidrosis was prolonged for 3 mo when 15 mouse units and for 6 mo when 125 mouse units botulinum toxin type B were injected. The size of the anhidrotic area decreased with time (p<0.001), indicating partial recovery at the edges. After 3 wk, the QSART score had significantly decreased to 18% of baseline and had decreased to zero in most subjects with doses of 62.5 mouse units or more. After 3 mo, the QSART had returned to 91% of baseline in all but one subject and, after 6 mo, recovery of sudomotor function was complete. Analysis by iodine-starch staining and QSART indicated that botulinum toxin type B suppresses sudomotor function effectively, in a concentration-dependent manner.
Botulinum Toxins↗
Burden of care in amyotrophic lateral sclerosis.
OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is a fatal disease with unique demands on patients and carers. PATIENTS AND METHODS: The total burden of care and burden components in 37 ALS carers were measured using validated questionnaires. Furthermore, influencing factors (functional impairment of the patient, additional carers, participation in support groups) were assessed. RESULTS: The mean total burden of care for ALS was low compared with dementia, mixed neuropsychiatric and internal diseases, but was correlated with functional impairment (P = 0.003). The main burden components were 'personal and social restrictions' and 'physical and emotional problems'. Problem behaviour of the patients was low in general, but was higher in carers participating in support groups (P = 0.002). Carers supported by additional carers had higher strain. CONCLUSION: The low burden of ALS carers may be caused by the low incidence of problem behaviour in ALS patients. However, if problem behaviour exists, carers participate more often in support groups, indicating the need for assistance. The burden of care increases with the functional impairment. Support for the carers has to start sooner.
Adult↗
[Chronic cough in muscular dystrophy].
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Bronchoscopy↗
Sudomotor testing predicts the presence of neutralizing botulinum A toxin antibodies.
The increasing number of patients being treated with botulinum toxin A complex (BoNT/A) has led to a higher incidence of neutralizing anti-BoNT/A antibodies (ABAs). Because BoNT/A is known to inhibit sweating, here we report sudometry as a possibility for predicting the presence of ABA. Sixteen patients suffering from spasmodic torticollis were selected: in 2 patients, BoNT/A treatment continued to be effective, in 9 patients, the treatment effect was impaired, and in 5 patients, secondary treatment failure developed. BoNT/A (100 mouse units, Dysport; Ipsen Pharma, Berkshire, United Kingdom) was injected subcutaneously into the lateral calves. Sweating was visualized with iodine starch staining. In addition, quantitative sudomotor axon reflex testing was performed at the injection site. Individual ABA titers were determined with a mouse bioassay. Results of sudometry significantly correlated with the BoNT/A treatment success. The quantitative sudomotor axon reflex testing was 0.58 +/- 0.63 fraction of the normal mean in patients with treatment failure, 0.18 +/- 0.13 fraction of the normal mean in those who responded partially, and 0 in responders (p < 0.01). Accordingly, the areas of the anhidrotic skin after subcutaneous injections were 4.5 +/- 10.3 cm(2), 32.7 +/- 16.5 cm(2), and 62 cm(2) (p < 0.01). Discrimination analysis indicated that the presence of ABA (6 ABA-positive and 10 ABA-negative) could be predicted correctly in all patients from the results of sudometry. Therefore, sudometry is a useful tool for identifying patients with neutralizing ABAs and might be helpful for identifying reasons for BoNT/A treatment failure.
Adult↗
Accidental pneumothorax from a nasogastric tube in a patient with severe hemineglect: a case report.
Nasogastric tubes are frequently used for nutrition of patients with neurologic diseases. We report an instance of inadvertant placement of a standard nasogastric tube into the left pleural space in a patient with right parietotemporal intracerebral hemorrhage and severe hemineglect on the left side. The 2 confirmatory maneuvers-aspiration of fluid and auscultating the abdomen on insufflating air-were false-positive. We conclude that only radiologic confirmation of the position of nasogastric tubes and the awareness of the associated dangers will help minimize the occurrence of such events in patients with disorders of perception or altered consciousness.
Aged↗
Subjective experience and coping in ALS.
OBJECTIVE: Amyotrophic lateral sclerosis is a rapidly progressive and fatal disease which has no known cure and limited symptomatic treatment. While coping strategies in more common diseases are widely assessed, coping is poorly understood in ALS. METHODS: We examined 41 ALS patients using a standardised interview, a validated coping self-rating questionnaire and a self-rating depression scale. The evaluation was repeated after six months. RESULTS: "Loss of speech", "loss of mobility" and "the poor prognosis" were the most frequent answers in the standardised interview to questions regarding the worst aspect of the disease. Pain was seldom mentioned. "Family members" were most helpful in coping with the disease, followed by "unspecific mechanisms" and "technical aids". None of our patients expressed a wish for assisted suicide. In comparison with other fatal diseases, patients with ALS had similar rankings in the coping mechanism of "rumination", but lower rankings in "search for social integration", "defence of fear", "search for information and communication". In contrast,* "search for hold in the religion" was of high importance for our ALS patients. In the follow-up examination the importance of "search for information and communication" increased. CONCLUSION: The results emphasise the importance of "loss of speech" and the importance of the caring family as well as the availability of technical aids in ALS. Coping in ALS seems to be based mainly on "rumination" and *"hold in the religion", but the increasing importance of "search for information" indicates that the sustained offer of information is essential.
Adaptation, Psychological↗