Tuesday, 1 March 2016

Negative inspiratory force myasthenia gravis

Negative inspiratory force myasthenia gravis

Advances in critical care have improved the mortality rate associated with myasthenic crisis. Guillain-Barre Syndrome (GBS) and Myasthenia Gravis (MG) are common causes of acute weakness. Myasthenic Crisis - PubMed Central (PMC) Myasthenic crisis is a complication of myasthenia gravis characterized by worsening of muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. Negative inspiratory force (NIF) should be documented and trended a NIF of -cmHor less is an indication for endotracheal intubation.


Indication for mechanical ventilation includes FVC mLkg or less (normal mLkg) and NIF cm H2O or less (normal cm H2O). Myasthenia gravis is disease of the neuromuscular junction, in which autoantibodies are directed against nicotinic acetylcholine receptors (AChR) located on the postsynaptic end plate. Placing a patient on NIPPV may be an option in those with a diminished but not dangerously weak respiratory apparatus.


Flaccid paralysis resulting from cholinergic crisis can be distinguished from myasthenia gravis by the use of the drug edrophonium, which worsens the paralysis caused by cholinergic crisis, but strengthens the muscle in the case of myasthenia gravis. Respiratory muscle strength can be assessed by measuring the maximal inspiratory pressure (MIP or PImax and the maximal expiratory pressure (MEP or PEmax). REFERENCES - UpToDate Although data are limite the proportion of patients with myasthenia gravis who experience at least one myasthenic crisis may be as high as to percent and the annual risk of myasthenic crisis among patients with myasthenia gravis is approximately to percent 3.

UpToDate

Myasthenia gravis - WikEM

Myasthenia gravis myasthenic crisis - EMCrit Project Mar 0 20Negative Inspiratory Force (NIF) This is the largest amount of negative pressure that the patient is able to exert when inhaling (video above). The MIP reflects the strength of the diaphragm and other inspiratory muscles, while the MEP reflects the strength of the abdominal muscles and other expiratory muscles. There is no role for this test in myasthenia gravis (more on this below). About of these patients may develop respiratory failure requiring intubation, so a major concern is determining who requires ICU-level. The disease can manifest as a combination of weakness in ocular, bulbar, and most importantly respiratory muscles.


Myasthenia gravis - Investigations BMJ Best Practice Serial measurements of forced vital capacity (FVC) and negative inspiratory force (NIF) are taken. Mar 1 20Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) are direct measures of respiratory muscle strength and may be more sensitive in detecting early respiratory muscle dysfunction compared with spirometry, but MIP and MEP are not usually performed on all patients referred for PFTs. (with pictures) Jan 1 20Understand your pain in the front of the neck symptoms, including causes common questions.

REFERENCES - UpToDate

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Myasthenic Crisis - PubMed Central (PMC)

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