What is the Besinger Score?
Discussion
Myasthenia gravis (MG) is a rare disease in the pediatric population. The incidence depends on the population studied but is estimated at 1-9/1 million/year. It was first described by William Heinrich Erb in 1879. The cause is autoantibodies against components of the post-synaptic membrane of the neuromuscular junction, usually against the acetylcholine binding receptor (AChR).
It can occur at any age but is more common in the adult population. Juvenile patients (0-19 years) are divided into prepubertal (12 years) who have disease presentations similar to the adult population. In adults there are 5 grades: ocular symptoms, mild, moderate, severe and very severe.
MG symptoms include:
Ocular symptoms are common
Ptosis – uni- or bilateral, children may tilt head to see
Ophthalmoplegic facial weakness – lid twitch, strabismus
Facial/bulbar weakness
Masked facies
Chewing and swallowing problems
Speech problems
Coughing and coughing insufficiency
Respiratory symptoms
Respiratory insufficiency
Peripheral symptoms (proximal symmetrical weakness)
Exercise intolerance
Fatigability
Difficulty with climbing stairs, rising from seated position, personal hygiene
Worsening of symptoms with
Exercise
Fever or infection
Elevated temperature
Medication
Symptoms often improve with rest such as sleeping with improved symptoms at night or early in the day. Prepubertal children usually present with ocular symptoms (which can wax/wane) such as ptosis, blurred or ...
Source: PediatricEducation.org - Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
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