CLIPPERS?

[Katherine]

Hi

I am an Australian woman with significant documented neurological symptoms - unsteady gait, hyperreflexia, swallowing difficulties, a feeling of strangulation when singing, reading out loud, or public speaking. I have facial drooping (left-sided) and a range of symptoms relating to autonomic dysfunction (bowel and temperature dysregulation). I can no longer write with my right hand, I walk with a cane, am immensely fatigued and suffer from rigid muscles in my right arm, left thigh and left trunk.

I have lots of small brain lesions - none typical of MS - the long-time suspicion of my neurologist. After my failure to develop the requisite brain lesions, my neurologist determined that my condition was not MS and not autoimmune in nature, but neuro degenerative and possibly either Multiple System Atrophy (MSA) or Corticobasal Ganglionic Degeneration (CBGD), both of which are unfortunately fatal.

With the recent discovery of a sarcoid lesion on my arm and a consequent diagnosis of sarcoidosis, my neurologist is excitedly considering the possibility of neurosarcoidosis. (He's excited because neurosarcoidosis can be treated; whereas MSA and CBGD cannot.) However, to date investigations have revealed no evidence of systemic sarcoidosis, which is almost unheard of with neurosarcoidosis. He says my brain lesions may well be sarcoid lesions. The sarcoidosis Specialist is less certain, but is considering treating me with Methotrexate and steroids to see what happens.

I'd not heard of CLIPPERS until New Year's Eve when (having been left at home alone while my family attended a fireworks display), I was idly comparing my MRI lesions with others on the internet. Lo and behold, I came across CLIPPERS and thought that mine looked remarkably similar. Nonetheless, I can't claim to have ever undertaken a whit of MRI interpretation training, so I could be very wrong.

My symptoms commenced about three years ago at the age of 47. My decline has been steady since then, however I actively parent my 7-year old and undertake some consultancy work from home. I spend my afternoons in bed until 3pm and retire for the night at 8pm most nights.

So... there's the background. Here's my questions - (1) Does this sound anything like CLIPPERS?; (2) Is my onset perhaps a bit slow for the 'typical' case?

Thanks all and Happy New Year.

Regards
Katherine

[BillCrum]

Hi Katherine,

Welcome to the CLIPPERS forum.

As you may have gathered, it's very hard to be specific about CLIPPERS. My own symptom onset was over a matter of 3-4 months before I got treated, but you may well get other people who had symptoms for longer.

The other aspect is time to treatment, in that one of the key CLIPPERS criteria is that it Responds to Steroids. So while having an improvement after steroids, doesn't guarantee a CLIPPERS diagnosis, NOT having an improvement is evidence against CLIPPERS. Of course as we lack a definitive test for CLIPPERS at the moment, it is hard to be too specific, but the steroid effect is pretty central to the whole thing (as far as I understand it anyway).

Interesting also that you mention neurosarcoidosis. My doctors also considered that as a possible diagnosis before they found out about CLIPPERS and they went through a stage of looking for evidence of sarcoidosis outside the brain. I was told it was very rare to have sarcoidosis purely in the brain, but then getting CLIPPERS is also very rare so go figure.

Hopefully some other people here may comment on how long they had symptoms before treatment. I know that my symptoms (mostly double vision, balance disorders, coordination problems, numbness) are not the only ones CLIPPERS sufferers experience.

Best Wishes

-Bill

[Katherine]

Thanks for the reply Bill.

And good on you for providing this service to the CLIPPERS community. It is a truly astonishing resource, with solid advice delivered in such a clear and lighthearted way.

I know first hand the angst of suffering from a nameless rare neurological disease and the overwhelming joy one imagines will be felt on gaining a name for the condition. I guess though that without websites such as yours - in cases like CLIPPERS - that joy could well be short-lived as one is confronted with a dirth of information.

Thanks again Bill.

Cheers
Katherine