* ? Discrete syndrome vs ALS variant
+ Slowly progressive
+ Legs before arms
+ Most commonly in 5th decade
+ No family history
o Motor dysfunction
+ Corticospinal ± corticobulbar tract dysfunction
# Spasticity: Legs > Arms
# Tendon reflexes: Brisk
# Plantar reflex: Normal or upgoing
# Pseudobulbar signs: Especially with disease onset > 45 years
+ No lower motor neuron change
o Sensory: Normal
o Frontal lobe dysfunction: Mild
+ > 3 years; Up to 3 decades
o Bladder function: Normal until late in disease
o Magnetic stimulation: Absent or prolonged cortical motor evoked latencies
o MRI: Focal atrophy of precentral gyrus
o PET scan: Reduced glucose consumption in pericentral region
o Central motor conduction times: Prolonged
o Normal: Serum; CSF; EMG; Spinal cord imaging; Serum CK; CSF protein
* Disease association: ? Breast cnacer
o Corticospinal tract: Axonal loss
o Normal: Anterior horn cells ± Betz cells
* Differential diagnosis
o Structural disorders: Spinal; Foramen magnum; Hydrocephalus
o Hereditary spinal disorders
PLS: Recessive, Juvenile onset9
l Alsin ; Chromosome 2q33; Recessive
* Gene mutations
o PLS: Deletion in exon 9
o ALS2: Mutation in another region of alsin gene
o Familial spastic paraparesis, infantile onset
o Onset: Childhood
o Spasticity: Bulbar; Extremities
o Gaze paresis
o Normal: Cognition; Sensation
o Central motor conduction times: Delayed or Unrecordable
o EMG: No denervation
I can't recall anyone here saying they were diagnosed with it. The preceding was taken from notes on the George Washington University Neurological Website St. Louis Mi.
Sorry Ruby and Dana. PLS is Primary Lateral Sclerosis. In my post I just posted the clinical notes. I should have just picked out the pertinent information. I did say at the bottom of my post that I haven't heard of anyone on site that had PLS.
Four neurologists and no confirmation of a particular disease is a bit unnerving but looking at the bright side depending on what your symptoms are now you should live long as they say 3 years to 30 years is the life span with the disease. ALS is 2 or 3 to 5 depending on whose information you are using.
Most of the motor neuron diseases are hard to pinpoint with ALS being the hardest to diagnose. They usually say you don't have anything else so it must be ALS. Have you tried going to Yahoo Groups PLS. I'm not sure if there is one there but there is one for most of the common ailments and the not so common ones as well. Good luck and welcome to the site.
Hi ricturn, sorry to take so long to answer.
I started playing around joining groups a couple of weeks ago.
This is one is good but I would also suggest you join Yahoo.com/group/PLS-Friends.
I am surprised that you have been diagnosed with PLS if you had it a short time when diagnosed.
My symptoms started in Jan./03. Jan./04 I was dignosed with ALS. Three months later and another Electromyogram, he said "no" as it was not progressing fast enough. Now they say they are "leaning" towards PLS. I gather from what other support members say it takes over 5 years before you can definitely be diagnosed with PLS. In between I guess we are in limbo waiting to see if it is going to change to ALS?
My symptoms started in my right leg. Tripping, limping, etc. - gradually getting worse. The leg atrophied (in the first 3 mons.) so that is giving the Drs. a problem. It is now just like a dead weight I drag around. My right arm does not swing when I walk and I am starting to loose my balance. My problems are nothing when I read about other members. So I try to live for today and try not to think about tomorrow!........ruby from Vancouver
My name is Marlo, i was originally diagnosted with ALS but when i was sent for a second opinion, the doctor send it could be PLS. So either way i have a terminal illness. So don't think your in this alone,, cause your not. There are many of us out there. Take care, we'll talk again. Marlo
My father-inlaw has been diagnosed for the past 15 years or so. PLS is as not as terminal as ALS is, and much much, much, more rare. You have a 1 in 30,000 chance of getting ALS. 1 in 10,000,000 chance with PLS. It's a disease of exclusion, as there is not one test for it. All other diseases must be ruled out.
My father-in-law is in a wheelchair, and only his immediate family can understand him when he talks. He has complete use of his mind (sharpest guy I ever met) and is able to type emails, role his own chair, and work on his model train set. He is also able to feed himself to an extent. He even authored an autobiography. It took 7 years for him to be diagnosed - the Mayo Clinic couldn't figure it out. It was some doctor in Michigan. I think the progression of the disease has taken it's course, as he is not getting worse. What does affect him is his advancing age (72). Unrelated medical issues seem to creep in as well. There was a scare 3 years ago when he got a rare virus he caught in Arizona. His weakened condition made it tough, but he got through it.
It seems to me that he has strength in his muscles (what a handshake!), they are just so cramped up, he can move them slowly and slightly. He drools, as he has little facial muscle control. He takes 2-3 pills a day of various medications.
His care taker is also his wife, who has been a saint over the last 15 years providing 24 hour care. She is having joint problems, probably as a result of the care taking over the years. She has had a knee replacement and been diagnosed with advanced carpul tunnel in both wrists.
He was a marine in the 1950's, and is Sicillian - so he views himself as a tough guy. Without question this trait has helped him cope, but for a guy who was very active and physical, there's no doubt it's been more than difficult. He's adjusted well, however. His condition has had an interesting side affect - he's a bit more emotional than ever. He laughs more, as well as cries without being able to "stop it". It can be fun to see such emotion coming from him, especially since he's "so tough".
Don't know if this will help anyone, but I thought I would share this with people. He's an amazing man, really, and unfortunately this disease has made it hard for him. It's the only thing that can keep him down. His spirit is strong.
Thanks Stu. A lot of times we receive a diagnosis and all the doctors will say is get your affairs in order. It is heatening to hear that some of us can live otside "the envelope" as they say and still continue to lead productive lives with an illness. Too many want to put you in a space and say Oh you can't do that you're disabled. Proof of what some people can do is good news to hear. Thanks again. Al.