Neuromuskuläre Störungen

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

• 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

• hepatic or renal failure,
• disseminated malignancy
• malnutrition
• myocardial infarction
• pulmonary embolism
• pregnancy

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!

Symptome Schaden am oberen Motoneuron

Lower motoneuron->Hirstamm

Rückenmark

 Peripheral nerve

Motorische Endplatte

Muskel

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.

•  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.

MRI

Neurophysiologie

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.

Immungobuline

bei Rückfall evtl. Steroide

Plasmapherese

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.

• 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.

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.

• 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.

• 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)

Nach Thymom-OP bessern sicn nur 10%

Warten oder langsames Eindosieren von Anticholinergika (Starke Speichelproduktion)

Zurückhaltende/keine  Verwendung von Succinylcholin

increased salivation, colic, diarrhoea, sweating and small pupils

Steroide

evtl. Immunsuppressiva

• Metabolic and electrolyte disorders
• Critical illness polyneuropathy
• Prolonged neuromuscular blockade
• Myopathy

alkalosis

insulin therapy

β2 agonist therapy

Das meiste ist intrazellulär

IPS-Aufnethalt >7 Tage

sensorimotor axonal polyneuropathy with flaccid(Schlaff) quadriparesis and respiratory muscle weakness

Relaxantien

Steroide

Erhöhte CK/Myoglobin

Muskelbiopsie: muscle fibre atrophy due to myosin loss,

this atrophy amounts to about 4% per day!