Neuromuskuläre Störungen
Karten zu Pact
Karten zu Pact
Set of flashcards Details
| Flashcards | 29 |
|---|---|
| Language | Deutsch |
| Category | Medical |
| Level | University |
| Created / Updated | 13.09.2014 / 25.09.2014 |
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Woraus besteht eine Motoreinheit?
- Motor neuron (im Hirnstamm oder Vorderhörner)
- Peripheral nerve (axon)
- Neuromuscular junction
- Muscle
Erkrankungen des Motoneurons? 3
Entsteht wie?
Motor neuron disease is caused by a primary degeneration of the anterior horn cell followed by axonal degeneration, and encompasses disorders such as amyotrophic lateral sclerosis, tetanus and polio
Ursachen für periphere Neuropathien?
6 Gruppen
- Infective causes: Lyme disease, Viral (CMV, Hepatitis and HIV)
- Metabolic or nutritional:
- Alcoholism
- B vitamin deficiency
- Drug induced (e.g. nitrofurantoin, isoniazide, vincristine, cisplatin, reverse transcriptase inhibitors, taxol)
- Porphyria
- Uraemia
- Diabetes
- Vasculitis
- Immune-mediated (e.g. Guillain-Barré syndrome)
- Toxic causes (Heavy metals arsenic, thallium, lead, gold), organophosphates, Hexacarbons, Shellfish (Sasatoxin)
- Prolonged critical illness, sepsis, multiple organ failure
Wann ist die Wirkung von Succinylcholin verlängert? 6
- hepatic or renal failure,
- disseminated malignancy
- malnutrition
- myocardial infarction
- pulmonary embolism
- pregnancy
Wie wirkt Botulinus-Toxin?
Symptome?
Clostridium botulinum produces neurotoxins, which interfere with the production or release of ACh.
one to three days after ingestion: nausea, vomiting, double vision, slurred speech, difficulty in swallowing and widespread paralysis. no sensory symptoms or signs!
Wo ist der Defekt im neuromuskulären System?
- Muscle tone decreased
- Clonus absent
- Muscle fasciculations present
- Muscle wasting present
- Deep tendon reflexes exaggerated
- Babinski sign
Symptome Schaden am oberen Motoneuron
Wo ist der Defekt innerhalb der Motoreinheit?
- Altered consciousness
- Altered pupillary light reactions and size and range of eye movements
- Altered facial sensation and motor function
- Altered hearing
- Altered gag, swallowing, cough
- + sensorisches Defizit?
Lower motoneuron->Hirstamm
Rückenmark
Wo liegt der Defekt in der Motoreinheit?
- Predominantly distal weakness and wasting
- Symmetrical in generalised neuropathies
- May be asymmetrical (e.g. in diabetes)
- Reduced tone, reflexes usually absent
- Autonomic dysfunction
Peripheral nerve
Wo liegt de Defekt in der Motoreinheit?
- Fatigability
- Sensory deficits absent
Motorische Endplatte
Wo liegt der Defekt in der Motoreinheit?
- May be associated with pain
- Generalised, but often predominantly proximal, weakness
- Reflexes usually preserved
- Sensory deficits absent
Muskel
Was ist ALS?
progressive, and ultimately fatal, degenerative neurological disorder
degeneration of upper motor neurons in the corticospinal tract and lower motor neurons in the brainstem and spinal cord.
Symptome ALS?
- aged 50-60.
- limb weakness
- bulbar symptoms.
- weakness and wasting with muscle fasciculations.
- Tendon reflexes are brisk (rege)
- plantar responses extensor.
- Cough and gag reflexes are reduced,
- and the tongue wasted with fasciculations.
Wie wird ALS dignostiziert?
MRI
Neurophysiologie
Was ist das Guillain-Barre-Syndorm?
Ätiologie?
Symptome?
acute demyelination of peripheral nerves occurs secondary to an endogenous or exogenous inflammatory process, with an autoimmune response to myelin antigens
Aetiologie ist unbekannt. in many cases there is a prodromal illness of some kind, usually of bacterial or viral origin.
gradual onset of weakness, often starting in the legs, gradually spreading proximally. Respiratory muscle weakness may occur. There may be muscle pain and tenderness in the early stages.
Therapie bei GBS? 3
Immungobuline
bei Rückfall evtl. Steroide
Plasmapherese
Wie beatmet man GBS-Patienten?
Intital?
wann Weaning?
The patient may initially be too weak to trigger the ventilator and therefore a controlled ventilation mode is used. Because of air hunger, higher tidal volumes and higher inspiratory flow rates may be needed to meet that demand.
Weaning may be initiated when the patient can trigger the ventilator. The optimal modes for weaning ventilatory support are pressure or volume support.
Folgen der autonomen Dysregulation?
- sinus tachycardia
- blood pressure may fluctuate spontaneously, with postural hypotension
- Autonomic features may be precipitated by tracheal suction with dramatic effect. Sinus bradycardia, and even asystole, may occur.
- Drugs with an effect on autonomic nerve function (such as metoclopramide) should be used with caution in GBS.
- Bradyarrhythmic episodes needing temporary cardiac pacing have been described.
Pathophysiologie Myastenia gravis?
autoimmune disease with an antibody which attaches to the acetylcholine receptor. This can be measured in the blood of approximately 85% of patients with the disease. A thymoma is present in about 10% of patients, and thymic hyperplasia in most younger patients.
Symptome Myastenie?
- The cardinal feature is fatigability.
- In most patients ocular symptoms are the first, In a variant of this condition, ocular symptoms are the sole manifestation.
- Limb weakness is common, and is worse after exertion.
- Bulbar involvement may lead to difficulty in speaking and swallowing.
- Respiratory fatigue leads to dyspnoea and ultimately ventilatory failure.
Diagnosestellung Myastenie? 3
CAVE?
- presence of acetylcholine receptor antibodies, which are specific for this disease, although not found in all cases.
- Anti-striated muscle antibody is also positive in some patients, particularly those with a thymoma, but is not specific.
- The diagnosis may be further supported by administration of edrophonium. Bessert sich die Symptomatik, so ist es beweisend!
edrophonium kann zu einem Kreislaufzusammenbruch führen! (unter REA-Bedingungen geben)
Postoperative Versorgung von Myasthenie-Patienten?
Nach Thymom-OP bessern sicn nur 10%
Warten oder langsames Eindosieren von Anticholinergika (Starke Speichelproduktion)
Zurückhaltende/keine Verwendung von Succinylcholin
Klinische Zeichen der cholinergen Krise?
increased salivation, colic, diarrhoea, sweating and small pupils
Wie behandelt man entzündliche Myopathien?
Steroide
evtl. Immunsuppressiva
In welchen Fällen kommt es zur einer Muskelschwäche NACH IPS-Zuweisung?
4
- Metabolic and electrolyte disorders
- Critical illness polyneuropathy
- Prolonged neuromuscular blockade
- Myopathy
Wann kommt es zu Hypokaliämie? 3
alkalosis
insulin therapy
β2 agonist therapy
Warum sagt Serum-Magensium-Spiegel wenig aus?
Das meiste ist intrazellulär
Wann tritt die CIP auf?
Definition?
IPS-Aufnethalt >7 Tage
sensorimotor axonal polyneuropathy with flaccid(Schlaff) quadriparesis and respiratory muscle weakness
Welche Substanzen verschlimmern Moypathie?
Relaxantien
Steroide
Wie zeigt sich Myopathie klinisch?
Erhöhte CK/Myoglobin
Muskelbiopsie: muscle fibre atrophy due to myosin loss,
this atrophy amounts to about 4% per day!
