Stiff person syndrome

[LAJ12345]

Intriguing articles. Can be confused with PD

my.clevelandclinic.org/heal...

rarediseases.org/rare-disea...

“General Discussion

Stiff-person syndrome (SPS) is a rare acquired neurological disorder characterized by progressive muscle stiffness (rigidity) and repeated episodes of painful muscle spasms. Muscular rigidity often fluctuates (i.e., grows worse and then improves) and usually occurs along with the muscle spasms. Spasms may occur randomly or be triggered by a variety of different events including a sudden noise or light physical contact. In most cases, other neurological signs or symptoms do not occur. The severity and progression of SPS varies from one person to another. If left untreated, SPS can potentially progress to cause difficulty walking and significantly impact a person’s ability to perform routine, daily tasks. Although the exact cause of SPS is unknown, it is believed to be an autoimmune disorder and sometimes occurs along with other autoimmune disorders.

Stiff-person syndrome has been described in the medical literature under many different, confusing names. Originally described as stiff-man syndrome, the name was changed to reflect that the disorder can affect individuals of any age and of either gender. In fact, most individuals with the condition are women. Stiff-person syndrome is considered by many researchers to be a spectrum of disease ranging from the involvement of just one area of the body to a widespread, rapidly progressive form that also includes involvement of the brain stem and spinal cord (PERM).”

[Smittybear7]

How do they test for it?

[LAJ12345 in reply to Smittybear7]

Diagnosis

A diagnosis of SPS is made based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation. Additional tests can be used to support a diagnosis and to rule out other conditions. Such tests include screening tests to detect the presence of antibodies against GAD-65, antibodies against amphiphysin (which are associated with paraneoplastic SPS) and an electromyography (EMG), a test that records electrical activity in skeletal (voluntary) muscles at rest and during muscle contraction. An EMG can demonstrate continuous muscle motor unit firing in stiff muscles, which is characteristic of SPS. High doses of diazepam will suppress the characteristic EMG results.

Treatment

Drugs that are considered GABA-ergic agonists therapies such as benzodiazepines, specifically diazepam and clonazepam, are used to treat muscle stiffness and episodic spasms. Affected individuals may also benefit from baclofen, usually given in addition to benzodiazepines. Other medications reported to have benefit in a small number of individuals include anti-seizure (anticonvulsant) drugs including vigabatrin, valproate, pregabalin, and gabapentin.

I was interested as rigidity is hubby’s worst symptom. And clonazapam seems the only drug he hasn’t had side effects from yet as far as I know. So I’m wondering if any on that list might also be helpful.

He doesn’t have spasms though which seem to be a symptom of this thing. Except on too high doses of levodopa.

[Smittybear7 in reply to LAJ12345]

I take Baclofen. It was prescribed bye a rehabilitation doctor and a movement specialist, neurologist. It was the help with muscle muscle cramping from sitting. I'm on 5 mg twice a day that seems to have helped. I'm on Carbidopa levodopa 25 mg to 100, I take one pill every 8 hours. I think I'm on too high a dose A lumbar spine surgeon prescribed gabapentin based on MRI results, I chose not to take that and not to have cortisone injection. The baclofen has helped somewhat, enough for now. Everything seems to have very serious side effects so I am hesitant to increase the dosage. The movement specialist thinks that I am under medicated, I think I am overmedicated due to the fact I have developed some involuntary movements. They are tolerable but concerning and embarrassing. My other neurologist thinks I should maintain the current dosage. The movement specialist suggested cutting the pills in half, taking them once every four hours instead of once every 8 hours . I'm afraid to make any adjustments for fear they will make things worse. Your husband is lucky to have you. God bless you

. I

[LAJ12345 in reply to Smittybear7]

Don’t let them make you take too much. We have been on a roller coaster over the last month with them increasing his dose and he went from pretty functional to almost completely incapacitated to the point he could not even go to the bathroom by himself and being suicidal on the higher dose, and they still wanted to increase more and “push through the dystonia”! He has instead dropped back to the original amount he was on and has almost regained his original function although his legs have weakened over the month. The clonazapam the psychiatrist prescribed has helped tremendously. I feel every time they mess with his seratonin and dopamine pathways he ends up in a bad way. But clonazapam is on the gaba pathway and seems to work. So hence my interest in this article. Dopamine has only ever made his rigidity worse. To be honest I am not convinced he needed it in the first place as the nurse only convinced him to take it to help with the rigidity.

[LAJ12345 in reply to Smittybear7]

I think it might be a good idea to try that cutting and spreading . See the graph below. That might keep the levels between the too high and too low point. But bear in mind you likely have a good portion of your natural cycle underlying it all Have you tried clonazapam? He is taking 1/4 x 0.5 mg tablet morning, afternoon, then a half before bed so 1 tablet a day and it has helped a lot.

[LAJ12345 in reply to Smittybear7]

What time do you take your dose? The madopar capsule was 50/12.5 so half as much as your 100/25 and still too much. Plus he burns through it too quickly and runs out and is shaky and frozen. I don’t think it is too little. It is too much at once but too far apart.

We have just been given the tablet we can cut so once the tail of his overdosing peters out I am going to cut in half and give every 2 hours to see if that keeps him in the comfort zone. But give the capsule at full strength first thing as it is off a lower base to push him up to the zone.

[Smittybear7 in reply to LAJ12345]

The carbidopa-levodopa 6 a.m. 2 p.m. 10 p.m. the Baclofen 10 in the morning and 10 at night

[LAJ12345 in reply to Smittybear7]

Have any of the c/l doses worse side effects than others?

[Smittybear7 in reply to LAJ12345]

I started on Carbidopa levodopa in October of 2021. Started at 1/2 pill three times a day the move to one pill three times a day then move to one and a half a.m. 1 in the afternoon 1 at night. As the dosage increase I started to have involuntary movements of shoulder shrug ,lip quivering a little more intense tremor, sometimes my mouth pulling to the left. it fluctuates from day to day .stress exacerbates it. Some days I have very little.

[LAJ12345 in reply to Smittybear7]

Sounds to me like you have gone past the point of enough . Do you find the night dose helps with sleep or makes it worse? Hubby found it wakes him up when it peaks and makes him feel like his feet are burning. I don’t think he needs the night one as he is better without it and wakes up fine and able to move.

[LAJ12345 in reply to Smittybear7]

Why did you increase it?

[Smittybear7 in reply to LAJ12345]

The carbidopa-levodopa is 25 to 100 mg

[LAJ12345 in reply to Smittybear7]

Yes, that is double the smallest madopar

[Smittybear7 in reply to LAJ12345]

Who helps you decide the dosage?

[LAJ12345 in reply to Smittybear7]

We took the pd nurse’s advice a few weeks ago to increase the dose from 7,11, 3 x 50/12.5. to

7am 100/25,

11,3, 7 x 50/12.5

11pm long acting madopar

Then we tried the HBS long acting x 5 per day and that was when it completely disabled him and he went through hell every night so now we work on trial and error with their approval once we finally convinced her he was on too much not too little. They seem to confuse dystonia from overdose with freezing stiffness when it wears off.

I had him every hour fill out a sheet ticking a box for rigidity, anxiety, freezing, shaking if it was occurring and got a clear picture that rigidity was occurring about 90 minutes after each dose, getting worse every dose during the day and getting worse each subsequent day. And also 3 1/2 hours after every dose he was freezing and shaking. It was a clear cycle. It seemed clear to me that it was too much but running out too quickly.

He has a dna analysis that says his ddc gene is blocked that converts levodopa to dopamine but his beta hydroxylase is quick to turn dopamine to noradrenaline. So it seems like what ever dopamine he gets is burned through quickly I think if I am reading it correctly. Still trying to get my head around the actual process going on.

Dna

[CaseyInsights in reply to Smittybear7]

The movement specialist suggested cutting the pills in half, taking them once every four hours instead of once every 8 hours .

This seem like a very good suggestion to me. The suggestion is that you take the same amount of medication just spread it out. This is to eliminate any spikes in Levodopa. Think it through: do not allow fear to freeze you up

🥶

[LAJ12345 in reply to CaseyInsights]

I agree, it makes sense not to have the high peaks and troughs. Constant levels are the key .Clonazapam helps with the fear aspect.

[111BANDIT in reply to CaseyInsights]

My husband had the same experience. The symptoms largely went away when he started on time release pills rather than the full dose 3times per day.

[LAJ12345 in reply to 111BANDIT]

The time release made hubby very bad but probably because it was too strong for him so the constant level was way too high.

[Smittybear7 in reply to CaseyInsights]

Thanks for your advice. The pills are so small that they are hard to cut exactly in half with the pill cutter.

[LAJ12345 in reply to Smittybear7]

Probably doesn’t matter if it’s not that exact so long as you take both halves in the same time period as the original. We have to cut a quetiapine into 4 pieces and it is about 5mm diameter! Fiddly for sure😊. It’s a shame they don’t make these tablets in smaller doses for sensitive folk like hubby. I’m sure he isn’t the only one.

[CaseyInsights in reply to Smittybear7]

Yes it is difficult to cut them evenly. I break them, but they are never equal. I use the larger portion in the morning. I have had no problems

Current Dosage

Morning: 1.5 C/L 25/100

Lunch: 1.5 C/L 25/100

Evening: 1 DopaBoost.

Do not forget the P5P (active B6) on evenings. Use 100 mg each day

[Smittybear7 in reply to LAJ12345]

I also had an EMG test nothing was determined to be a major problem. They did find one area the may have caused the nerves to be irritated but did not indicate a major concern. I also have tremors, muscle tightness, involuntary movement what's your car two and a half to three hours after Carbidopa levodopa, then seemed to quiet down or disappear until two and a half hours after the next dose

[LAJ12345 in reply to Smittybear7]

This is very similar to hubby’s reaction to madopar. There seems to be dystonia at the top and bottom of a cycle of medication. And worse at night.

My current theory is his natural dopamine cycle is a bit sub par but still functioning to a degree. He is fine first thing in the morning and until an hour or so after taking his first dose then the cycles get worse as the day goes on. I put that down to the normal dopamine cycle peaking at midnight and being at a low at mid day. So the daytime doses are at a low point of his cycle and anything in the evening pushes him too high and he has a bad night.

Below is my feeling about what seems to be happening. Based on watching him. He is having last dose at 3 and is ok in the night. We are waiting to see if that lasts as he has a long tail of too high dose underlying his cycle at the moment so that should taper off and he might need to take a part dose later in the day. At least now they have given us a tablet we can cut up so we can add a tiny bit more at a time.

My current theory

[Smittybear7 in reply to LAJ12345]

What dose of clonazepam is he taking? How bad was his rigidity and spasms?

[LAJ12345 in reply to Smittybear7]

He is taking 1/4 x 0.5 mg tablet morning, afternoon, then a half before bed so 1 tablet a day and it has helped a lot.Before starting on the levodopa it was annoying but not painful . It has made his right hand not work well. On the later higher dose of madopar it was painful and totally disabling . He could not urinate without my assistance to hold “it”. His muscles were spasming visibly. That sounded like the rigid person symptoms above, and makes me wonder if their problem is too much dopamine.

[Smittybear7 in reply to LAJ12345]

It's frustrating because this too hard to try to figure out the right amount.

[LAJ12345 in reply to Smittybear7]

Once we figured out that his doses were making him worse as the day went on we think he is making his own over night and that is why the later in the day ones are worst. Plus they are added to the top of the previous pills tail as it takes a while for the last of each pill to leave the system .

So to us a stronger one first thing , makes sense, then another half strength 4 hours later, and 4 hours after that to cover the dip midday in the natural cycle, then if he was to take more any later it would take into account that there was the tail of the previous 3 doses plus his natural dopamine cycle so it would be at a longer interval and and a smaller still dose. Eg 25 mg at 10pm. But he is not needing that at the moment.

[Sydney75 in reply to LAJ12345]

My HWP sometimes get myoclonic leg Jerk, thought it was RLS. Klonopin does calm this down when it happens.

[LAJ12345 in reply to Sydney75]

Makes you wonder if some people might have this or something similar to this and have been misdiagnosed. It says this is rare but maybe all these conditions are a continuum and merge into each other. Interestingly some of the same drugs can be used to treat either this or PD. So if he has some of these symptoms it makes you wonder if those are the drugs that might help.

Klonopin and clonazapam are the same thing. They work on the GABA side of things. Interestingly sublingual GABA has helped hubby in the past when he had mild anxiety.

[MarionP]

This can also happen with certain antipsychotics, which as you probably know are intentionally directed toward reducing the amount of, or the effect of, dopamine, when such is the intentional goal of treatment, such as for schizophrenia. I have seen them also used in cases of PD, usually done by a neurologist. But beware because when you use a dopamine antagonist or blocker such as the antipsychotics, for the purpose of PD it really needs to be done in a very very small dose, much less than typically used for antipsychotics.

Especially a medication called geodon, nasty stuff I have never liked it used with mental illness, schizophrenia, etc, psychotic disorders, because wow when it goes wrong that side effect is nasty stiffens up your throat, your throat muscles going to spasm and they stay that way, stiffens up your neck you can't do anything about it and they have to give an antihistamine called cogentin to try and reduce that effect until the medication itself wears off. I don't know why some shrinks have used it after finding out those side effects because seeing it is a horror show. But the idea I'm bringing up here is that it is a side effect of reducing/ blocking dopamine, and sometimes you will see such dopamine blockers involved in diseases with chorioathetoid or seizure movement issues and really your prescriber needs to be exceedingly careful in their use.

[LAJ12345 in reply to MarionP]

What a nightmare that sounds. The dose of levodopa they made hubby increase to made his neck and throat seize up and he had a bit of panic about that, and had all his muscles literally crawling under his skin, and they said (without even seeing him) he was under medicated and just needed to get over the threshold and it would come right!

[MarionP in reply to LAJ12345]

Well you can question them more deliberately about that or you can maybe they'd like to try it themselves first, or just tell them it is too frightening for now and to back off that medication and try to find something else. You can also try hydrating more, spasming up can also happen anytime and just needs maybe some more water or tea drunk.

[LAJ12345 in reply to MarionP]

Yes, we had a talk about it and I finally convinced them he was getting too much dopamine , not insufficient . I had him plot symptoms every hour and the rigidity and spasms were always at peak dopamine. I make him drink plenty of drinks through the day . 2x litre water plus cups of tea and coffee. What is in tea that is useful? Quinine ie in tonic water?

[MarionP in reply to LAJ12345]

Any hydration, not tea specifically at all, I just happened to drink a lot of tea. As far as quinine, the doctor passed it on but said he knew of no actual evidence to support it, just been hearing it among his colleagues. So it may be placebo or there may be something about it, quinine is an interesting chemical, and since I like it, I just buy tonic water and mix it in with orange juice and I get the same as something we can't get here in the states anymore anyway I fell 8n love with in the 70s, Schweppes Bitter Orange. Tastes great with diet tonic water too.

[Smittybear7 in reply to MarionP]

I've also heard about tonic water and muscle spasms

[MarionP in reply to Smittybear7]

That's a relief, thought I might get another ad hominem attack from neuro-whatsis.

[Smittybear7 in reply to MarionP]

?????

[MarionP in reply to Smittybear7]

Exactly my reaction.

[Rather]

The muscles in both my legs and the lower torso will sometimes momentarily stiffen and stretch out in a type of spasm, when I stand up or while l'm in bed and stretch my legs. I tried to describe it to my nuerologist and he said it was not PD related because it happens onboth sides of my body. I am not yet taking any meds. Any insights?

[MarionP in reply to Rather]

Sometimes it's a natural aging effect, my own doctor has even suggested drinking more quinine, in any event more water and more tea. It has something to do with the gradual age-related decay of muscles in general and something about adding more water sometimes relieves that.

[LAJ12345 in reply to Rather]

Are you getting enough magnesium?

[Rainin14 in reply to Rather]

regular yellow mustard for spasms🤗😇😊

[LAJ12345 in reply to Rainin14]

Interesting.

[MarionP in reply to Rainin14]

Indeed? Interesting. Really? What's behind that, interesting.