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What is Tensillon Test???

Definition

Tensilon is the trade name for edrophonium chloride. The Tensilon test is an injection of edrophonium chloride used to diagnosis myasthenia gravis (MG).

Purpose

Tensilon blocks the action of an enzyme, acetylcholinesterase, an important part of the system regulating neuromuscular transmission. To stimulate a muscle, a nerve cell (neuron) releases the chemical acetylcholine. To prevent prolonged muscle response to a single nerve signal, acetylcholine is broken down by acetylcholinesterase after the muscle is stimulated.

In myasthenia gravis, there are too few receptors for acetylcholine on the muscle. The acetylcholine is broken down before it can fully stimulate this reduced number of receptors, and, as a result, the muscle is weak. By blocking the action of acetylcholinesterase, Tensilon prolongs the muscle stimulation, and temporarily improves strength. Increased strength following an injection of Tensilon strongly suggests a dignosis of MG. The Tensilon test is most effective when easily observed weakness is present, and is less useful for vague or fluctuating complaints.

Precautions

The Tensilon test may cause heart rhythm abnormalities, especially in those patients with preexisting conditions.

Description

The Tensilon test involves the intravenous injection of a small amount of Tensilon. The needle is left in place. If no adverse reaction is observed within 30 seconds, an additional volume is injected. Results are apparent within one minute.

Preparation

Before the test, the patient must stop taking all drugs that can inhibit acetylcholinesterase. The referring physician can advise on specific drugs the patient is taking.

Aftercare

The effects of Tensilon subside quickly, and are completely gone after 30-60 minutes. No aftercare is needed.

Risks

Atrial fibrillation and bradycardia are possible in sensitive individuals. The administering physician must have appropriate resuscitative equipment available.

Normal results

In a patient without MG, the Tensilon test will not produce an obvious increase in a previously weak muscle. Some subjective feelings of increased strength are possible but not significant.

Abnormal results

An obvious increase in strength in weakened muscles strongly suggests the diagnosis of myasthenia gravis. The effect comes on very rapidly, and fades within minutes.

Ibnu Shaukani

Myasthenia Gravis

A 35 year old woman presents to the clinic with a chief complaint of double vision. She reports intermittent and progressive worsening double vision for approximately 2months, rarely at first, but now every day. She works as a computer programmer and the symptoms increase the longer she stares at the computer screen. She had also noted a drooping of her eyelids, which seems to worsen with prolonged working at the screen. Both symptoms subside with rest. She is generally fatigued but has noted no other weakness or neurological symptoms. Her medical history is unremarkable. Physical examination is notable only for the neurological findings. Cranial nerve examination discloses impaired lateral movement of the right eye and bilateral ptosis, which worsen with repetitive eye movements. Motor, sensory and reflex examinations are otherwise unremarkable.

what is the likely diagnosis?

Myasthenia Gravis

what is the pathogenesis of this disease

An autoimmune disease where the body produce atibody towards the post synaptic Ach receptors.Also,the antibodies also attack the acethylcholine(Ach) at the synatic cleft.The net effects are decrease number of ffuncctional Ach receptor and functional Ach

explain the pathophysiology of each signs and symptoms.

patient comes to the hospital with the complaints of fatigue, ptosis and also double vision.Destruction of the receptors cause diminished transmission of the nerve impulse across the neuromuscular junction. Muscle depolarization is not achieved, therefore, muscle weakness develop.The signs and symptoms of MG depends on which muscle involve:

• extra ocular muslces :double vision
• levator palpebral superioris:ptosis
• upper esophagus :dysphagia
• palatalmuscle :dysathria
• diaphragm :respiratoryfailure

Usually,fatigue develop due to repetitive stimulation or prolonge use.This occur due to decrease number of available neurotrasmitter and the numbers of functional Ach receptor.

what are the investigations and the significant of it?

• TensillonTest

admistration of cholinesterase inhibitor will cause improvement of muscle power.This will increase the number of Ach in the synaptic cleft.

• antibody towards the Ach receptor
• chest Xray

autoimmune disease may associate with thymoma or thymic hyperplasia.

what treatments should be considered

• cholinesteraseinhibitor
• plasmapheresis,corticosteroid
• thymectomy

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