ALS???

[scaredgrl85]

Please Doctor..I need help. I've been having the most unusual symptoms lately. Starting last week I've been having excruciating headaches along with on/off blurry vision. I went and saw a neurologist, he performed an upper EMG test along with an upper sensory and motor nerve conduction test which all came back normal. I then went for an MRI of the cervical spine and brain that came back normal except for a slight spasm in my neck. He said this is the cause of my headache. I wasn't having neck pain until yesterday, where it feels like my neck is very stiff. I know this can be a symptom of ALS, and sometimes spontaneously I'll get fasculations on my sides and legs but no where else. I'm a 22yr old female with no family history of nervous disorders. My question is, is ALS common in people my age? Should I ask my neurologist about ALS or am I looking too much into my symptoms? I've been very stressed since April, and my family believes this is the cause of my symptoms. Thank you so much.

[Joyce K Lee, MD]

Dear scaredgr185,

Let me answer your follow-up question, which is a good one.

Nerve conduction velocity (NCV) and electromyography (EMG) help diagnose nerve and muscle disorders. These tests, which can be used in combination, are often referred to as EMG/NCV studies. NCV is administered before EMG and measures the speed at which nerves transmit electrical signals.

During NCV, electrodes are placed on the skin over a nerve that supplies a specific muscle or muscle group. A mild, brief electrical stimulus is delivered through the electrode and the response of the muscle is detected, amplified, and displayed. The strength of the signal is also measured. Neurological conditions can cause the NCV to slow down or to be slower on one side of the body. The strength of the response also provides clues to help with diagnosis and to determine the extent of the disease.

EMG measures nerve impulses within the muscles. Tiny electrodes are placed in the muscles in the arms and legs and the electronic responses are observed using an instrument that displays movement of an electric current (oscilloscope). As muscles contract, they emit a weak electrical signal that can be detected, amplified, and tracked, providing information about how well the muscles are working. These responses are abnormal in cases of ALS.

The confirmation of ALS is facilitated by demonstrating diffuse denervation signs, decreased amplitude of compound muscle action potentials, and normal conduction velocities.
For a more detailed confirmation of ALS, more strict electrophysiologic criteria have been developed by a subcommittee of the World Federation of Neurology and are referred to as the "El Escorial" criteria for motor neuron disease.

I will not overwhelm you will all the details of this new criteria, but just to give you an idea this is what it entails:
*evidence of active and chronic denervation
*exclusion of motor neuropathies
*Signs of active denervation including fibrillation potentials and positive sharp waves
*Signs of chronic denervation include large motor unit potentials of increased duration with an increased proportion of polyphasic potentials, often of increased amplitude
*EMG signs of LMN dysfunction required to support a diagnosis of ALS should be found in at least 2 of these 4 regions of the CNS: brain stem and cervical, thoracic, and lumbosacral regions of the spinal cord.

It is true that EMG/NCS findings are more apparent with the progression of disease.
However, abnormalities even if subtle are usually detectable on EMG/NCS in the early states of the disease process.

Hope this helps,
JKL, MD

[Joyce K Lee, MD]

ar scaredgr185,

Thank you for submitting your question.
Please be informed that I cannot offer you a definitive diagnosis based on your history and symptoms.
This is solely for educational purposes and should not be substituted for a formal neurologic evaluation.

To begin, yes -- ALS is extremely uncommon in your age group.
Please allow me to offer a brief educational segment on ALS.

Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig's Disease, Maladie de Charcot or motor neurone disease) is a progressive, fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement.
Between 1 to 2 people per 100,000 develop ALS each year in the U.S. ALS most commonly strikes people between 40 and 60 years of age. Men are affected slightly more often than women.
Scientists have not found a definitive cause for ALS and the onset of the disease can be linked to a variety of risk factors.
ALS is classified into two groups, familial ALS and sporadic ALS.
1)"Familial ALS" accounts for approximately 5%-10% of all ALS cases and is caused by genetic factors. Of these approximately 10% are linked to a mutation in Superoxide dismutase (SOD1), a copper/zinc dependant dismutase that is responsible for scavenging free radicals.
2)Most of the remaining 90-95% of cases are classified as "sporadic ALS" and have no known hereditary component.
The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS.

I will stop there in terms of discussing ALS.
Again, I cannot say definitely but I think it is unlikely that you have ALS -- based on your age and your normal EMG studies.
EMG and NCV (nerve conduction) studies show distinct abnormalities in patients with ALS.

It is interesting that you mentioned your recent stress.
Stress can definitely cause many neurologic symptoms.

Before heading down the road where you begin to increase your stress by worrying about serious neurologic conditions like ALS, please make sure that you deal with your psychological stress and rule out other common causes of headaches.

Perhaps you have migraines of tension headaches.

Please continue to see your neurologist.
As an aside, you should never feel hesitant to ask your doctors questions -- when it comes to your health, no question is a bad one.

Hope this helps,
Best of luck,
JKL, MD

[scaredgrl85]

Thank you so much for your prompt response. I believe that my muscle aches are more sore than cramping. I just have one more question if you can please answer, when you state that EMG and NCV studies would show distinct abnormalities in patients with ALS, does that mean at any point in the disease , or only when symptoms become very apparent? Thanks again.

[BetteB]

A related discussion, MRI, tap, blood