Macro-EMG potentials (MEP)s and twitch contractions (spike-triggered-averaging) of single motor units (MUs) have been recorded in the first dorsal interosseus muscle (FDI) of 10 control subjects and 20 patients with amyotrophic lateral sclerosis (ALS). MUs over the full range of voluntary recruitment thresholds were studied. Patients with slightly affected FDIs (5) mainly showed MUs with enlarged MEPs and increased twitch forces. In contrast, the patients with more severely affected FDIs (15) revealed decreased twitch forces, especially in the MUs with higher thresholds. The corresponding MEPs could be enlarged as well as normal. It appears that MU sprouting and the resulting increase of twitch force can compensate for the loss of motoneuron in early stages of ALS. In more advanced stages, however, a decline of the force of the surviving MUs, especially of those with higher thresholds, seems to contribute to the progressive muscle weakness, in addition to the corticospinal degeneration and the reduction in the number of motoneurons.

To study the mechanism of attacks in familial hypokalemic paralysis, we recorded resting membrane potentials, action potentials, current-voltage relationships, and isometric forces in intercostal muscle fibers from three patients. In normal extracellular medium, the resting potential was reduced, but membrane conductance was not different from control. Excitability was reduced and the action potentials had no overshoot. On exposure to a 1-mM potassium solution, with or without insulin, the cells depolarized to about -50 mV, and became inexcitable. Over the tested membrane potential range from -120 to -40 mV, the slope conductance in the 1-mM potassium solution was not different from that of control fibers in a 1-mM potassium solution. In particular, the potassium component conductance was not reduced. Depolarized fibers could not be completely repolarized by returning to a 3.5-mM potassium solution. An experimentally induced transient shift of the chloride equilibrium potential to a highly negative value caused stable repolarization. Paralysis could also be induced by replacement of extracellular chloride with an impermanent anion, a treatment which causes myotonia in healthy fibers. It was concluded that the basic defects are a reduced excitability and an increased sodium conductance, and that these defects are aggravated on reduction of the extracellular potassium concentration.

To study the mechanism of periodic paralysis, we investigated the properties of intact muscle fibers biopsied from a patient who had adynamia episodica hereditaria with electromyographic signs of myotonia. When the potassium concentration in the extracellular medium, [K],, was 3.5 mmol/l, force of contraction, membrane resting potential, and intracellular sodium activity were normal, but depolarizing voltage clamp steps revealed the existence of an abnormal inward current. This current was activated at membrane potentials less negative than -80 mV, reached a maximum within 50 msec, and was not inactivated with time. The inward current was completely and reversibly blocked by tetrodotoxin, which indicates that it was carried by sodium ions. In a solution containing 9 mmol/l potassium, normal muscle would depolarize to -63 mV and yet be capable of developing full tetanic force upon stimulation. The muscle from the patient depolarized to -57 mV and became inexcitable, i.e., it was paralyzed. A contracture did not develop. Lowering of the extracellular pH did not influence the resting potential, but it effectively antagonized or prevented the paralytic effect of high [K], by changing the inactivation characteristics of the sodium channels. Hydrochlorothiazide, which had a therapeutic effect on the patient, did not prevent paralysis in vitro. An abnormal rise of the intracellular sodium activity was recorded when the extracellular potassium concentration was raised to 10 mmol/l.

No abstract

As any patient may require rehabilitation and physical therapies, all physicians need to acquire at least a basic knowledge of Physical and Rehabilitation Medicine (PRM). In 2005 PRM teaching was implemented in all phases of the curriculum for medical students in Germany. The curriculum includes, among others, the following topics: principles of rehabilitation; the model of the International Classification of Functioning, Disability and Health (ICF); principles and effects of physiotherapy and occupational therapy; indications and contraindications for PRM interventions. Teaching of PRM topics is implemented from the first week in all phases of the curriculum, as: (i) lectures in the module "Introduction to Medicine (Propaedeuticum)"; (ii) a cross-sectional course entitled "Rehabilitation, Physical Medicine and Naturopathic Treatment (RPMN)"; (iii) single lectures on PRM in other fields; (iv) elective mandatory courses on the social model of rehabilitation, balneology, and others; and (v) the option to choose PRM as a subject for practical training. All modules are evaluated regularly by the students. Global ratings of the module "Propaedeuticum" were good, and of the cross-sectional course "RPMN" very good. The advanced part of the practical training was rated highly by the students. In conclusion, the implementation of teaching of PRM and other rehabilitation topics in undergraduate medical education is a successful concept that fulfils the criteria for education in medical school set out by the American Association of Academic Physiatrists.

Here, we report a mutation screening by single-stranded conformational analysis of the astroglial human brain glutamate transporter (HBGT) II complementary DNA in patients with amyotrophic lateral sclerosis. The conformational analysis data indicate a lack of sequence variations in the HBGT II coding region in 6 patients with amyotrophic lateral sclerosis and the same number of nonneurological control subjects. In both groups, three variants of the HBGT II 5' untranslated region were isolated. We have no evidence that the reported complementary DNA variants are disease specific.

To investigate the pathomechanism of paramyotonic stiffness, the mechanogram of isometric finger force and the electromyogram of the flexor digitorum muscle were simultaneously recorded in five unrelated paramyotonia congenita patients. Cooling of the forearm provoked "spontaneous" electrical activity, but the accompanying force was less than 5% of the maximal voluntary isometric contraction amplitude. The relaxation of maximal voluntary contractions executed in the cold had a normal first phase and a very slow second phase. The force amplitude at the beginning of the slow phase was up to 80% of the maximal contraction amplitude; the duration of the slow phase was up to several minutes. It was concluded that the slowed muscle relaxation is more important as a factor contributing to paramyotonic stiffness than spontaneous force generation. Involuntary electrical activity recorded during the slow relaxation phase was too low to account for the force. Intercostal muscle biopsies obtained from four patients showed similar phases of slow relaxation when stimulated to give isometric twitches or tetani in the cold. Extracellular recording with electrodes designed to pick up all activity from the small bundles clearly showed that the slow relaxation phase was not caused by spontaneous action potentials. One possible explanation for the slowed relaxation is a long-lasting depolarization-induced contracture of the muscle fibers following activation in the cold.

Zusammenfassung Einleitung: Zur physiotherapeutischen Behandlung der Parkinson-Erkrankung wurde im letzten Jahrzehnt eine steigende Zahl randomisiert kontrollierter Studien mit innovativen neuen Ans?tzen publiziert. Dies belegen auch aktuelle Metaanalysen, die eine zunehmend breitere Basis f?r die Bewertung ihrer klinischen Wirkungen bilden. Allerdings weisen diese Analysen einige Unterschiede im Hinblick auf die Studienauswahl sowie die Bewertung der klinischen Effekte auf. Die vorliegende ?bersicht hat daher zum Ziel, eine aktualisierte Zusammenfassung der Evidenz der Wirkungen der einzelnen Verfahren und ihrer klinischen Anwendung zu liefern. Methode: Eine elektronische Suche nach Metaanalysen, die zwischen Januar 2000 und M?rz 2014 erschienen sind, wurde in den Datenbanken MEDLINE, PubMed und Google Scholar durchgef?hrt. Ergebnisse: Es konnten 8 Metaanalysen identifiziert werden. Mit Ia Evidenz ist die Wirkung f?r die Trainings- und Tanztherapie sowie die Behandlung mit externen Reizen (Cues) belegt. Auch die Laufbandtherapie erreichte Ia Niveau, allerdings findet sich eine abweichende Bewertung in 2 Metaanalysen mit unterschiedlichen Auswahlkriterien f?r die einbezogenen Studien. Die Wirkung von Tai-Chi bzw. Qigong ?bungen und der LSVT-BIG-Therapie ist auf Ib Niveau belegt, positive Wirkungen eines repetitiven Trainings von Ausfallschritten werden auf IIb Niveau berichtet. Keine nachhaltigen Wirkungen konnten bei der Ganzk?rper-Vibrationstherapie nachgewiesen werden. Nur auf 3 Studien st?tzen sich Befunde ?ber positive Effekte der konventionellen Physiotherapie in Bezug auf die Flexibilit?t sowie motorische und ADL Funktionen. Die Effektst?rken wurden insgesamt als gering bis moderat mit Werte bis 0,56 eingesch?tzt. Nahezu alle behandelten Patienten befanden sich in einem noch wenig fortgeschritten Stadium ihrer Erkrankung (entsprechend Hoehn und Yahr Stufe 2?3). Grunds?tzliche Probleme f?r eine Bewertung waren variable Inhalte, Intensit?ten und eine unterschiedliche Dauer der gepr?ften Behandlungen sowie der Einschluss von Kontrollgruppen mit aber auch ohne eine Therapie. Schlussfolgerung: Die neuen physiotherapeutischen Konzepte bieten einen vielversprechenden neuen Ansatz f?r die Behandlung von Parkinson Patienten. Die besten therapeutischen Wirkungen sind bei der Bradykinese und posturalen Instabilit?t zu erwarten, sodass die gepr?ften Methoden als komplement?re Therapieformen zur Behandlung von Symptomen anzusehen sind, die nicht ausreichend durch eine medikament?se oder operative Behandlung zu bessern sind. Anhand der bisher publizierten Studien l?sst sich noch kein optimales, einheitliches Physiotherapiekonzept angeben.