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Neurology (Expert Forum)
Diagnosis of ALS?
This forum is for questions and support regarding neurology issues such as: Alzheimer's Disease, ALS, Autism, Brain Cancer, Cerebral Palsy, Chronic Pain, Epilepsy, Fibromyalgia, Headaches, MS, Neuralgia, Neuropathy, Parkinson's Disease, RSD, Sleep Disorders, Stroke, Traumatic Brain Injury
Diagnosis of ALS?
by blob, May 02, 2003 12:00AM
Doctor:
I was wondering if you could possibly give me some information about my brother.
About 8 years ago, he started having shrinkage of his left arm...he went for many tests and they ruled out ALS...MS...muscle inflamation., etc. He has had mri, spinal tap, blood work. Everything normal....He is taking synthroid for thyroid.
Anyways, over the years his arm shrinkage has been slowly getting worse. The only other symptom is twitching in that arm.
They are now concerend he has a rare form of ALS called Focal Motor Neuron. Can you tell me anything about this disease? Is it ALS? Will this eventually go into full blown ALS? We are having a hard time finding information on this. Thank you for your help.
I was wondering if you could possibly give me some information about my brother.
About 8 years ago, he started having shrinkage of his left arm...he went for many tests and they ruled out ALS...MS...muscle inflamation., etc. He has had mri, spinal tap, blood work. Everything normal....He is taking synthroid for thyroid.
Anyways, over the years his arm shrinkage has been slowly getting worse. The only other symptom is twitching in that arm.
They are now concerend he has a rare form of ALS called Focal Motor Neuron. Can you tell me anything about this disease? Is it ALS? Will this eventually go into full blown ALS? We are having a hard time finding information on this. Thank you for your help.
Doctor's Answer
by CCF-Neuro-M.D.-JT, May 04, 2003 12:00AM
Benign focal amyotrophy also known as monomelic (one limb) atrophy, segmental spinal muscle atrophy, or juvenile muscular atrophy is NOT the same thing as ALS and does not turn into ALS. Rather, it's a very slowly progressive lower motor neuron disease characterized by weakness and wasting that begins in one limb and for many patients never spreads beyond that. No one knows what causes it, but some people believe that it may be from recurrent trauma in the neck region compressing the spinal cord (which looks flattened on MRI and autopsy) or genetic. Usually seen in men beginning in their 20s or 30s and it's typically in the arm, although the leg can also be affected. It's a very slow process and some reports say that in about half the patients the disease never affects the other limbs. In the remainder of patients, the disease can spread to the opposite limb. And it can go to all limbs in 5% of the patients. The diagnosis is made with the help of EMG and MRI of the cervical spine to rule out other disorders. What distinguishes this from ALS are the limiting nature of the disease (only one limb) and absence of what we call upper motor neuron findings which refer to things like spasticity (increased muscle tone) and brisk reflexes. Basically while ALS is upper and lower motor neuron disease that affects all the limbs and face and swallowing/speaking muscles to some degree, benign focal amyotrophy is for the most part just lower motor neuron disease in one limb. For more info, try these sites:
www.ninds.nih.gov/health_and_medical/disorders/motor_neuron diseases.htm
www.alsa.org/index.cfm
www.pslgroup.com/als.htm
Hope that helps.
www.ninds.nih.gov/health_and_medical/disorders/motor_neuron diseases.htm
www.alsa.org/index.cfm
www.pslgroup.com/als.htm
Hope that helps.
