Emergency Treatment Myastheniccholinergic Crises

- Identify and treat precipitating cause, e.g. infection, drug interaction or overdose

- Sit patient at 45 clear airway, give nasal 02 and if overt respiratory failure - intubate and ventilate for as long as required.

Myasthenic crisis Cholinergic crisis

- IV neostigmine 8-12 mg/24 hrs - Withdraw all anticholinesterases

- sc. atropine 0.5 mg tds - Monitor respiratory function (vital capacity)

- Prednisilone 100 mg daily - Wean from ventilation when appropriate

- Consider plasmapheresis or IVIG - Re-introduce oral anti-cholinesterases in

- Change IV to oral anticholinesterases low dose and gradually increase when able to swallow

NEONATAL form of myasthenia gravis: this develops in a number of infants of myasthenic mothers.

- Suggested by poor crying/sucking and floppy limbs.

- Presents within 48 hours of birth and may persist until the end of 3rd month.

- Caused by passive transplacental passage of IgG (acetylcholine receptor antibodies).

- Treatment with anticholinesterases is required until spontaneous recovery occurs. Remission occurs following exchange transfusion.

This disorder may occur in infants even when their mother has been in remission for many years. CONGENITAL form of myasthenia gravis

This usually commences in infancy and persists through adult life. Receptor antibodies are not found and the disease may result from structural abnormalities of the receptors themselves. (A number of such disorders have 468 been identified.) Thymectomy is contraindicated in this disorder.

section v

MULTIFOCAL NEUROLOGICAL DISEASE

AND ITS MANAGEMENT

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