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Isaac's Syndrome

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by

Akua Boateng

on 9 January 2014

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Transcript of Isaac's Syndrome

Isaac's Syndrome is also know as
acquired
Neuromyotonia or continuous muscle fiber activity syndrome.
It is a rare neuromuscular disorder.
In 1961 a South African physician Hyam Isaac was first to describe it.
Background Info.
What is Isaac's Syndrome?
The Causes of Isaac's Syndrome
Do YOU have it?
(Symptoms)
Now What (Treatment and Prognosis)
Stats N' Pics
The other name for Isaac's Syndrome, Neuromyotonia means nerve (neuro) and muscle stiffness (myotonia).
It is characterized by the rapid and abnormal bursts of nerve impulses from the motor neurons of the peripheral nerves.
The nerve impulses cause continuous muscle activity such as twitching or cramping.
Mechanics
There is no definite cause of Isaac's Syndrome.
Evidence shows that there are factors which
may
be a cause but it is substantial.

There are three common factors: Acquired, Paraneoplastic, Hereditary.
Not Exactly...
Patients who have acquired Isaac's Disease do not literally contract it because it is a contagious virus.
Acquired Isaac's disease is usually as a result of exposure to toxins such as gold and mercury or radiation.
The most common explanation is that an autoimmune reaction occured in which antibodies bind to the potassium channels on peripheral nerves.
The autoimmune condition is the most common cause resulting in 80% of patients.
How it's "gotten"
Paraneoplastic means the consequence of the presence of cancer in the body, but it does not have to be local.
Paraneoplastic instances occurred when tumors of the lung and thymus gland (thymoma) or lymphoma where present in the patient's body.
Para...WHAT!
This is the least common form of causes of Isaac's Syndrome. Some even repudiate the idea that it is a plausible cause.
Hereditary just means that the patient is genetically predisposed to the condition.
Hereditary
This form involves prolonged, involuntary, muscle activity which manifest as myokymia (myo = muscle and kyma = wave).
Myokymia is the persistent undulating movement (wave) in the skin that lies above the affected muscles
It is also describe as continuous contracting or twitching muscles
THE FIRST LIST
The other form of symptoms is slow relaxation.

It is the muscles failing to relax after intense activity (myotonia, neuromyotonia).
Myotonia and Neuromyotonia involve stiffness in muscle which cause immense pain.
Stiffness occurs more frequently in adolescence than adulthood.
THE SECOND LIST
Temporary muscle weakness
Tongue and pharyngeal muscles are sometimes affected, leading to swallowing difficulties and hoarseness
Muscle cramps
Slowed movement
Increased sweating
Slurred speech
The Misfit List
The main way to diagnose is by electromyography (EMG) where doctors record a persons electrical activity and detect abnormalities.
Because of the disease's rareness treatment is very experimental and is not long lasting,
Usually anticonvulsants are given to stop the involuntary muscle contractions also to relieve the stiffness.
Immunosuppressive therapy is used for those who are thought to have the auto-immune form
Diagnosis and Treatment
The prognosis of Issac's syndrome is not quite developed.
The treatments are short term but they do alleviate some pain.
Prognosis
Most individuals experience symptoms before age 40
During ages 16 to 20 the sole symptom is muscle stiffness and cramping
Onset is between ages 15 and 60
Affects less than 200,000 people in the US population
Statistics
Isaac's Syndrome
Complex Information...
The symptoms (especially the stiffness) are most prominent in the calves, legs, trunk, and sometimes the face and neck, but can also affect other body parts.
Approximately one third of people with Isaac's syndrome experience numbness and tingling in the skin, but they do not experience muscle pain.
Weight loss may occur.
Isaac's syndrome also effects the brain.
-personality changes, hallucinations, mood swings and sleeping disorders. (Morvan syndrome)
Bibliography
http://www.ninds.nih.gov/disorders/isaacs_syndrome/isaacs_syndrome.htm
http://www.princeton.edu/~achaney/tmve/wiki100k/docs/Neuromyotonia.html
http://www.socialstyrelsen.se/rarediseases/isaacssyndrome#anchor_1
http://www.riversideonline.com/health_reference/Nervous-System/AN00824.cfm
Full transcript