The Diebold Family
The following is a somewhat re-drafted description of my experience with Opsoclonus-myoclonus. Please feel free to share it with whomever you feel appropriate. If anyone, including any member of the OMS community, has any questions, they should feel free to call me, write me, or e-mail me. I have also provided my work number below. I am an attorney with a firm in Portland, Maine, so the phone will be answered as "Thompson & Bowie."
My full name and address are:
Thomas G. Diebold
8 Meadow Way
Cape Elizabeth, Maine 04107
(207) 799-9346 (H)
(207) 774-2500 (W)
This letter is an attempt to set forth in small detail my experience as an adult with a post-viral onset of opsoclonus-myoclonus ("dancing eyes, dancing muscles"). The disorder can also be known as Kinsbourne's syndrome, or may have "ataxia" (unsteadiness) added. I have included dates below, to provide a sense of the time periods involved. In essence, I was admitted to the hospital about 2 weeks after initial onset of symptoms, remained in the hospital for 34 days, and will return to work not quite 3 months after onset. I had a full-blown set of symptoms, complete with complete, body-wide muscle spasms and tremors (the myoclonus) and eyes that constantly flashed back and forth rapidly (the opsoclonus). I did lose the ability to even sit up in bed, watch television or feed myself.
For a little background, I am 32 years old and am still recuperating as of early January, 1998. My symptoms began in mid-late October, 1997, as jumpy vision and shaking hands, after first suffering a nasty flu, or so I thought. In addition to first feeling generally lousy, my back hurt and I felt restless and itchy. Also noteworthy was that in early October, I fell down my stairs and struck the top of my head. I wasn't knocked unconscious, but I did see stars. My doctor does not believe it is related to the OMS, but I wanted to provide that history here.
The initial flu symptoms gradually transformed into more unusual symptoms that I had never experienced before. I felt like my body was wired in a fashion, like an electrical current was traveling through me. I was jumpy, unsteady, and became confused. My last day at work, October 22, 1997, I attended a hearing and was not able to take any notes at all or concentrate on what was being said. My eyes would periodically "flash" or very quickly erratically jerk from side to side. After my vision worsened, and I began to have shaking and weakness in my legs, I went to the emergency room on Sunday, October 26, 1997 where I was seen by a Physician's Assistant. He said there was nothing wrong with me, but sent me over to the big hospital in town for a CAT scan because of the head blow. The CAT scan revealed nothing. The resident in the E.R. correctly thought I had cerebellar damage based on the nystagmus problem (the erratic eye movements, or the opsoclonus) and the general trembling and unsteadiness in my hands and legs at that time (the myoclonus). Unfortunately for me, he was overridden by the physician on call, who, after conducting a brief neurological exam, diagnosed me with vestibular neuronitis (an inner ear disorder) - and told me it would get worse before it got better. I have read of this same misdiagnosis in the literature on this disorder.
Things definitely did get worse after that visit, and that week is now almost completely lost to me - I have essentially no memory of it. My wife and family tell me that I was irritable, but could speak coherently, although I did become increasingly disoriented and confused. My vision worsened as I lost the ability to read or even watch television. My family doctor prescribed valium, and despite taking it, I was not able to rest or sleep at all during this week. Interestingly, this was the first of many drug reactions for me that were not normal, especially with muscle relaxants and sleeping drugs, which typically caused the opposite of what they were meant to do. I expect this is true for many OMS patients, but would be interested in hearing more about unexpected drug reactions.
Beginning October 30, 1997, Thursday evening, things started a sharper acceleration downhill. I could barely walk at this point because, in addition unsteadiness caused by muscle shaking, I had lost my balance. Despite its clear visible symptoms, OMS also has more insidious efforts - at least it did in me. I gradually lost all balance, including the sensation of where I was physically located in relation to my surroundings. Part of my inability to walk was also because I lost the sensation of weight on my feet - I was later told by physical therapists that I was a very interesting patient for them because they could begin to predict what sensory perceptions I had lost and what supports or just simple physical contact of any form were necessary to help me. Thursday night I could not lay in bed because of incessant restless twitching and so I shambled and stumbled into the living room to sit in an easy chair. At some point in the middle of the night, I tried to get back into bed and my poor wife, who was laying awake this whole time, came out and had to help me walk into bed.
I was not already in the hospital at this point because I was scheduled for an appointment with a neurologist the next day, Friday, October 31, 1997. By Friday morning I had lost the ability to walk and was essentially not competent mentally. I can remember dragging myself on my elbows and knees to the bathroom to take a bath (I couldn't stand up for a shower) while my wife was downstairs for a moment rather than waiting for her - I was not a good patient and did not have a real awareness of what was going on with me. I do have a clear recollection of laying on the bed while my wife readied us for the trip to the neurologist and looking up at my brother while twitching spasmodically all over my body. I remember clearly his look of shock, but otherwise I could not really see around me because of the opsoclonus.
After looking at me for about 30 seconds and conducting a brief neurological exam, the neurologist had me admitted to Maine Medical Center, where I spent the next 34 days. I did have seizures, and brain swelling, and was totally incapacitated by full-body myoclonus (or body-wide tremors and shaking), but once I began to recover, recovery was quick. Essentially, in the space of a week to 10 days, I went from lying helpless in bed to walking slowly in the hallways. I was discharged on December 4, 1997, and have now been home for almost 2 weeks, and I feel a little stronger and less unstable each day. The eyes have improved and I can now read books for a time, and while the hands shake some, I can write this message. I hope to return to work sometime in January, 1998. (Post-script - I have been released for part-time work beginning January 12, 1998 and the symptoms continue to recede).
By Friday, October 31, 1997 and for the next several days, I was essentially in state of continual muscle spasm and body-wide tremor. I was given morphine in an attempt to relax the muscles movements and am told that did help. My eyes were flashing back and forth so much I couldn't even identify people coming into room. Sleep deprivation, which has been building since the week before I was admitted into the hospital, became a huge issue. The morphine was the only thing that provided any relief. The first week in the hospital, my condition actually worsened, and I still felt a burning tingling spreading in the areas where the tremors and muscle spasms were worse. Because my situation was somewhat acute, I was primed with essentially every treatment that is in the literature: steroids, IVIg, deprecote, muscle relexants, and other drugs I do not know of or recall. I did have seizures and delusions, and swelling of the brain, so things were very unpleasant for a period, more so for my family.
While I mentioned earlier drug reactions, I should elaborate more specifically on one particular episode. I was given an anti-seizure drug called deprecote, which initially helped. However, I became increasingly disoriented and delusional (with a severe paranoia thrown in), which culminated in several seizures over the course of a couple of days. I can still remember the particular delusions I was having, which essentially dealt with my imagining things falling on me or me falling onto the ceiling or something. Sensory and visual perceptions was very impaired at this point. Anyway, I had one especially unpleasant episode in which I, lost in a delusion about the blood-treatment machine falling on me and my wife, actually swung at her in an panicked attempt to get her out of range. Because of this and other concerns about my condition, I was given an EEG. This revealed toxic levels of deprecote in my brain. Apparently, my system, instead of eliminating the deprecote like most people, was retaining and storing it, so the deprecote level reached dangerously high levels. I do not know if this is my personal reaction, or if opsoclonus-myoclonus effects drug reactions.
Things began to improve for me about three weeks into my hospital stay once I started receiving pheresis treatments with what is called the Column A, or Protein A, or even Prosorba. I had 2 treatments with the general pheresis, and then 13 with the Column. Because I failed to respond to any other treatment, and because my spinal taps were still showing that the antibodies in question were still be produced the third week in the hospital, I was embarked on an aggressive and prolonged course of treatment with the pheresis. Initially, these treatments were extremely difficult to get through, and I went only 1/2 with the first Column treatment, but gradually I tolerated them better and better as I got stronger. I think this treatment should be tried more widely, as I had no response to steroids, deprecote, IVIgG and some others. Pheresis treatment is very much like dialysis, and essentially involves filtering the blood through a machine to replace plasma or filter out the antibodies caused the muscle shaking and eye movements.
While there were many extremely unpleasant aspects to this condition, families can help by being there at night if the OMS victim has the vertigo problem, which he or she probably will if the opsoclonus, or eye movements, is bad enough. If so, be there and touch them, hold them when they experience it. By using the term "vertigo" (which is the best term I could think of to describe this), I am attempting to describe a sensation to disorientation as to where one is in relation to objects around you - and falling toward them. After the incredible itchy restlessness subsided a little, night-time became extremely difficult because of this problem for me. I would lay awake all night clutching desperately to the railing of the hospital bed because my body completely and absolutely felt as though it was going fly into the ceiling, and that looked to be about 10 stories away. This went on while I was wide-awake. I couldn't close my eyes because of a strong spinning/twisting sensation would overwhelm me, and if I opened my eyes, I would have these perception problems. This was very unpleasant.
Family members may see the patient with opsoclonus-myoclonus suddenly accelerate their body movements at night as they go into the vertigo attack, and they may even start rolling off the bed or grabbing at things around them to stabilize themselves - that's what I did. I definitely noticed that if people touched me, it abated the symptoms, especially the vertigo, and enabled me to get at least a little sleep. I also noticed that things improved once padding was put around my bed, which served to cut down on my field of vision, so there could be less distortion to get lost in. One of the nurses administering the column A pheresis noticed that contact helped abate the muscle jerks, and held my hand during the 2.5 hours of treatment to stop the tremors from shaking the needles lose in my arms when my family wasn't around to do it.
I think families are also important in educating the nursing staff, and doctors, about the care a patient with opsoclonus-myoclonus needs. I believe it took a little while for the nursing staff to realize how incapacitated I was, including not even being able to feed myself. Further, because of the endless thrashing about, I was pulling the IV out of my arm, one time with the needle still stuck in. I was in a stupor at this point and didn't realize that I had blood all over me. Moreover, because of the stupor, and my inability to see and manipulate objects to my side (my field of vision and functioning capacity was limited to directly in front of me), I do not think I could have hit the button to summon the nurses myself. Fortunately my brother was there, as he was all-day, all-night for over three weeks, and he alerted the nursing staff. There were also a couple of episodes when, lost in a delusion, I tried to get up and roll out of bed. My brother would essentially tackle me and hold me until I became aware of myself again, or buzz for the nursing staff. Because of this problem, I was moved to an observation room in the hospital. Nights were the worst time for me, and mornings were difficult. When the symptoms were full-bore, I needed to be watched at night.
There is no particular pain associated with myoclonus-opsoclonus, but I did experience about four nights of excruciating pain because of what I understand to be a bladder spasm. My muscles were in a state of near constant contraction, and my bladder followed suite. I could not describe what was happening or what the pain was, so it took a while to figure out what was happening.
Family members should also pay attention to the calories being consumed by a patient with opsoclonus-myoclonus. Despite the fact the muscle tremoring is involuntary, many calories are being burned off as a result. I lost quite a bit of weight and in particular my legs really atrophied. I had nausea as a result of one of the drugs and just wasn't interested in eating. I was told that I was a couple of days aware from having a feeding tube inserted. My family constantly badgered me to eat and went out and got whatever I was interested in eating. I was given a green light to eat whatever I wanted, whenever I wanted. My family helped quite a bit in getting me to eat when I did not want to, and I think that made a difference. Make sure the opsoclonus-myoclonus patient eats well. Bowel movements, while an unpleasant topic, are also important and family members should be aware whether those processes are functioning properly.
Finally, I should mention that, as with most neurological disorders, there are emotional issues too. For me, I lapsed into depression and was very weepy. My doctors told me that emotional lability (or the inability to control emotional swings) was common and expected. Some may become violent or very irritable, others might be sad. I could almost feel that I did not have emotional control, but that came back as my symptoms improved.
As I write this, some two and half months after onset, I am almost well. If you saw me on the street, you might think I was completely normal. I am still weak, and my myoclonic symptoms (or the muscle jerks), manifest in a minor way if I do not sleep the night before, or I overexert myself, or I am startled, or if I get cold and start to shiver. I am reading a lot, but get a little dizzy or fuzzy if I focus too much. I have told that things should be normal in about six months. On the whole, I feel fortunate, and I am very much aware that I am lucky my condition was not related to a cancer, but was instead a post-viral attack.Again, please feel free to contact me if there are any questions. Please also excuse grammatical or typographical errors. I think that other experiences and comments should be combined with this in one document and distributed.
January 8, 1997
Updated June 12, 1999