What's This?
This page is to record, in a very unscientific way, a brief sketch of people with a diagnosis of CLIPPERS. The purpose is to try and get a feel for how many of us there are out there. Please send me an email with your story to wrcrum@hotmail.com. IF YOU WANT YOUR DETAILS POSTED HERE, PLEASE MAKE IT CLEAR IN YOUR EMAIL
The Cases
14. NR from the US (Added June 2024)
I was recently contacted by a patient who had an unusual, atypical and challenging journey before his CLIPPERS diagnosis. He gave me permission to share an account of his case, so here it is.
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In 2015, this active and healthy 44-year old US male with no significant health issues apart from a history of gluten intolerance started experiencing double-vision and right-side neurological issues from tip to toe. The symptoms progressed to include altered walking, and loss of fine motor-skills in his right hand. After a month, treatment with intra-venous prednisone was begun but had little effect. A month later, with symptoms worsening and preventing writing, doing up buttons, driving etc, brain-stem glioma was diagnosed based on MRI and symptoms. Dexamethasone (another corticosteroid, often used for symptom relief in glioma) was prescribed and a second diagnosis for glioma was received. Neurological symptoms started to improve.After another month, surgery and radiation were scheduled and dexamethasone was maintained due to continuing improvement in symptoms. Surgery and radiation were then postponed and MRI showed reduction in "tumour" volume. After another month the glioma diagnosis was withdrawn and, after a CLIPPERS diagnosis, prednisone was continued.
Seven months later and prednisone had been tapered down to nothing and there had been no recurrence. MRI only showed "scarring" from the earlier pseudotumor in brainstem. The patient had also actively changed their lifestyle including gluten free eating, taking Levothyroxine for thyroid, also limited sugars, drastically decreased amount of processed food, increased sleep hours/night, yoga and exercise. Eight years later the patient is symptom free.
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13. PDB from Europe (Added July 2017)
PDB was in his early sixties when symptoms began in October 2016 with a sensation of electricity in the left arm and fingers, fatigue, numbness of the right side of the body, control loss in finger movement; the doctors and osteopaths said it was due to a nerve inflammation caused by a blocked C4-C5 neck vertebra. In January this year he had an MRI of the neck which showed a white peppering. During the following weeks he got ataxia, skin sensitivity issues, poly-neuropathy, severe sonofobia, swallowing problems, neuralgia in the left side of the face, numbness and weakness in legs and feet. Symptoms varied from day to day, but there was gradual decline overall. In February he was unable to walk and admitted to hospital as an emergency. From MRI he was found to have severe inflammation on the brain stem and spinal chord, and a 2nd MRI a week later showed fast growing of the lesions. By this time he could barely speak (dysarthria), lost facial expression and eye movement, his consciousness deteriorated, he went into coma, his breathing stopped, and a few hours after being intubated, his heart stopped. Fortunately 24 hours before that they had started an IV dose of 1g of solumedrol which started to work and he woke up a day later. His fine motor skills, eye movement and facial expression improved very fast but he then had to fight an E.coli infection. After that his clinical improvement was rapid; he learned to swallow, speak, and walk again. He stayed in the neurology unit for 2 weeks, and then another 4 weeks in a rehab clinic. He was diagnosed with ADEM (Acute disseminated encephalomyelitis) when he left the hospital. At that time, he had just stopped taking steroids, however, in the 10 days he was home, he got severe gait ataxia (he already felt worse after the steroids were reduced to less than 20 mg and at that time he was without steroids for 2 weeks). Back in hospital in early May an MRI showed numerous new lesions in the cerebellum. Another 3-day flash of intravenous solumedrol resulted in a little clinical improvement, not as much as the first time. He was referred to the university hospital for a 2nd opinion. He got a 3rd flash of steroids for 5 days, to which he responded immediately. After 2 weeks of research he got his final diagnosis at the end of May - CLIPPERS. We are sure that it's the right diagnosis this time (he hasn't had a biopsy so they are still not 100% sure). He's on 50 mg of corticosteroids now, tapering to 30 mg by the end of August. He still has a lot of issues now: balance, fatigue, neuropathic pains, hiccups (daily for more then 4 months, sometimes day and night… this drives him crazy !! ). But he walks and there's a slight general improvement.
12. RS from the US (Added February 2017)
At the time his symptoms first occurred in June, 2016, RS was a totally normal, strong, healthy 70 year old man. He was a bit overweight but had no health problems and took no medications. His symptoms started abruptly in with an unbalanced feeling. This was initially diagnosed by his local doctor as vertigo caused by fluid in his inner ears and was treated with a Steroid dose pack for 10 days. Thankfully his symptoms cleared up after about 2 days. However, 10 days after completing this treatment his symptoms were back and rapidly became worse with imbalance so severe he had to use a walker; slurred speech; double vision; difficulty writing; difficulty swallowing. MRI of his brain performed locally on July 29, 2016 resulted in a diagnosis of Brain Stem Glioma. The neurosurgeon said there was nothing he could do for him; the lesion was inoperable due it's size and location in his brain stem. RS was first seen at Mayo Clinic in Jacksonville, FL ( MCFL) in August 2016 and eventually after more testing (spinal tap, PET Scan, lab work) he was diagnosed with probable CLIPPERS - probable because brain biopsy was judged too risky to perform due to location of lesions in brain stem. At the end of August he had IV steroids x 3 days (1,000 mg/day) and his symptoms were totally gone by the second day. He then was prescribed oral prednisone initially of 60 mg daily which was tapered over many weeks until finally in November he was taking only 10 mg prednisone daily. Unfortunately, then his symptoms returned: imbalance, double vision, slurred speech and difficulty writing. He was admitted to MCFL for another course of IV steroids x 3 days (1,000 mg/day). Again his symptoms were completely gone by the second day and he was discharged taking prednisone 40 mg daily. In January, 2017 Methotrexate 15 mg weekly was added as the prednisone was decreased by 5 mg every 2 weeks. All was going well until February 2017 at which time he noticed that he once again had slight imbalance, double vision and difficulty writing. At this time he was taking 20 mg prednisone daily and 20 mg methotrexate weekly. His doctor was contacted; Prednisone was increased to 40 mg daily and methotrexate was increased to 25 mg weekly. The doctor wants him to follow this regimen for 1 month to give the methotrexate time to work. At this time, his symptoms are improved but not completely gone; he still has slight double vision and slight imbalance.
11. GW from the UK (Added July 2015, Updated February 2018)
GW is a 48 year old male who developed a frozen right shoulder in Spring 2014. In August he developed double vision for 3 weeks and then in late Winter he began to develop a frozen left shoulder. Double vision followed and by May 2015 he was off work with stress suspected because of hyperventilating episodes; in the following weeks it became clear this was far from the whole story. By the time he saw a neurologist he needed assistance in walking. Multiple Sclerosis was suspected but an MRI in June was not consistent with that diagnosis. Another clinician suggested CLIPPERS and blood tests seemed to rule out everything else so treatment with 1g/day of intravenous prednisolone was started following a month at 60mg/day, dropping to 50mg/day for a further month before review. His strength is definitely improving and he can now walk with a frame with improved responsive in speech, but some remaining vision problems.Update: He returned to full time work which proved challenging. Steroid dose reached a low of 7.5mg but because of problems and in spite of an MRI showing no change, the dose went back up to 20mg in mid-2017. Currently looking at starting to lower the steroid dose again, this time in conjunction with a steroid sparing agent.
10. KH from the UK (Added May 2015)
KH is a 27 year-old whose symptoms started in 2011 but who didn't get a CLIPPERS diagnosis until 2014. Initially, she experienced numbness in half of her face and then part of her tongue. After a week she developed balance problems and sensitivity to noise which she suffered for two more weeks before getting an MRI scan in hospital and being diagnosed with brain-stem inflammation. Symptoms worsened and expanded to include vomiting, double vision, bowel problems and auditory hallucinations. Her diagnosis became Multiple Sclerosis but then changed to an aggressive stage 4 tumour; consequently a brain biopsy was performed but this came back clear. Steroid treatment helped symptoms a lot but there were still relapses and eventually some suggestion that anxiety might be the cause (!) Eventually, with the help of a different doctor, CLIPPERS was diagnosed. KH is not currently on medication but suffers with some symptoms which she tries to manage through diet, exercise and lifestyle.
9. MH from Northern Europe (Added October 2013)
MH is a 22-year-old student whose symptoms started in 2003 when she was only 12 (and this of course predates the "discovery" of CLIPPERS, when these cases tended to be treated with steroids without ever being identified - BC). Her main symptoms were problems with balance and walking. She was treated with corticosteroids but the symptoms reappeared in 2008 when attempts were made to lower the dose. Since autumn 2011, when she suffered another relapse, even though the steroid dose had been the same for 2 years, she has used a walking stick or forearm crutch. Her diagnosis of CLIPPERS came in 2012 after a brain biopsy. MH is now maintained on 15mg/day Prednisolone and 50mg Azathioprine three times a day. She also gets "pulsed" corticosteroid therapy which is repeated every third month. Despite this treatment she still has problems with balance and walking, spasticity, fatigue, weakness and tingling in legs, and also bladder problems. Interestingly MH only had one brief episode of double vision but has had recent attacks of nystagmus.
8. TZ from the US (Added July 2013)
(Note from Bill Crum: this is an interesting case with a rapid onset and a variety of severe symptoms including Pseudobulbar Affect (PBA). Consequently I have included some more detail than usual about the disease progression and treatment. CLIPPERS was diagnosed eventually, at a centre with significant experience and expertise.)
An active 54 year old woman diagnosed with CLIPPERS after 2 months of tests and one relapse in two different hospitals / rehab facilities. Symptom onset in 2013 was fast:
Day 1: general malaise, headache
Day 2: difficulty walking, slurred speech, headache, nausea; stroke suspected
Day 3: right-side paralysis (including face), dizziness, nausea, short-term memory loss, catheter inserted; contrast-enhanced brain MRI shows lesions, MS and ADEM suspected
Day 5: spinal tap; Solumedrol drip for 4 days total
Day 10: improvement in rehab, continued use of catheter and no use of bowels
Day 19: deterioration with weakness, inability to walk; catheter and no use of bowels
Day 24: further deterioration, total paralysis from chest down and leg spasms
Day 30: symptoms worsening; MRI reveals new brain/spine lesions
Day 32: Solumedrol drip for 5 days, some improvement, lymphoma suspected
Day 35: transferred to rehab with tapering steroids and improvement including walking, talking, dressing, showering, continued muscle spasms
Day 59: successful catheter removal bowel function restored; continued muscle spasms
Day 66: new symptoms include emotional control issues (laughing, crying and excessive talking) CLIPPERS suspected by expert;
Day 67 - 74 MRI, spinal tap, PET scan, blood work, no brain biopsy recommended due to improved MRI from steroid use; final diagnosis CLIPPERS and PBA (pseudo bulbar affect); prescribed 50 mg prednisone every other day for at least a year along with Zoloft for PBA
Day 100+: in outpatient rehab. Fine motor coordination and speech are almost normal. Walking continues to improve with stamina and balance as main issues. Muscle spasms, mostly in the torso area. PBA issues have improved, though easily startled. Unable to drive or work.
7. PM from Canada (Updated January 2014)
A 62 year old male, diagnosed with CLIPPERS in Jan 2012. A brain biopsy in June 2012 determined he did not have lymphoma. He was then treated with tapering steroids (Dexamethasone) but has had to maintain a daily dose of 1.0mg to prevent symptoms. Day to day living is almost normal but he continues to experience right facial numbness, right hearing loss 20%, right eyesight slightly impaired 5%, balance difficulties, tiredness and the steroid "push" (which feels like a 10-20lb sack of flour being carried on the shoulders at all times). Update: steroids increased to 2.0mg / day as symptoms worsened. Update Jan 2014: Prednisolone at 7.5mg/day and now Methotrexate too. Balance improved from 6/10 to 8/10 and steroid "push" from 50lb equivalent to 10lb equivalent. Only traces of lesions in right cerebellum remain as of MRI in Dec. 2013.
6. WS from the US (Added April 2013)
A 56 year old man who developed fatigue and problems walking in 2010. In mid-2011, when seen by a neurologist, he reported slurred speech, mal-coordination, diplopia, numbness and feeling like he was "drunk". Brain MRI showed abnormalities in the cerebellum. Subsequent, investigations including lumbar puncture, MRI (including spectroscopy) and brain biopsy found signs of brain inflammation. Monthly "pulse" treatment with steroids resulted in symptom improvement; symptoms worsened when pulse treatment was stopped leading to a CLIPPERS diagnosis. Continued problems with fatigue, gait and speech as well as sporadic diplopia, ringing in his ears and difficulty concentrating. In April 2013, his treatment was changed to tapering oral prednisolone beginning with 60mg/day.
5. BA from the US (Added April 2013)
(Note from Bill Crum: this is an interesting case with a rapid onset and a variety of severe symptoms including Pseudobulbar Affect (PBA). Consequently I have included some more detail than usual about the disease progression and treatment. CLIPPERS was diagnosed eventually, at a centre with significant experience and expertise.)
An active 54 year old woman diagnosed with CLIPPERS after 2 months of tests and one relapse in two different hospitals / rehab facilities. Symptom onset in 2013 was fast:
Day 1: general malaise, headache
Day 2: difficulty walking, slurred speech, headache, nausea; stroke suspected
Day 3: right-side paralysis (including face), dizziness, nausea, short-term memory loss, catheter inserted; contrast-enhanced brain MRI shows lesions, MS and ADEM suspected
Day 5: spinal tap; Solumedrol drip for 4 days total
Day 10: improvement in rehab, continued use of catheter and no use of bowels
Day 19: deterioration with weakness, inability to walk; catheter and no use of bowels
Day 24: further deterioration, total paralysis from chest down and leg spasms
Day 30: symptoms worsening; MRI reveals new brain/spine lesions
Day 32: Solumedrol drip for 5 days, some improvement, lymphoma suspected
Day 35: transferred to rehab with tapering steroids and improvement including walking, talking, dressing, showering, continued muscle spasms
Day 59: successful catheter removal bowel function restored; continued muscle spasms
Day 66: new symptoms include emotional control issues (laughing, crying and excessive talking) CLIPPERS suspected by expert;
Day 67 - 74 MRI, spinal tap, PET scan, blood work, no brain biopsy recommended due to improved MRI from steroid use; final diagnosis CLIPPERS and PBA (pseudo bulbar affect); prescribed 50 mg prednisone every other day for at least a year along with Zoloft for PBA
Day 100+: in outpatient rehab. Fine motor coordination and speech are almost normal. Walking continues to improve with stamina and balance as main issues. Muscle spasms, mostly in the torso area. PBA issues have improved, though easily startled. Unable to drive or work.
7. PM from Canada (Updated January 2014)
A 62 year old male, diagnosed with CLIPPERS in Jan 2012. A brain biopsy in June 2012 determined he did not have lymphoma. He was then treated with tapering steroids (Dexamethasone) but has had to maintain a daily dose of 1.0mg to prevent symptoms. Day to day living is almost normal but he continues to experience right facial numbness, right hearing loss 20%, right eyesight slightly impaired 5%, balance difficulties, tiredness and the steroid "push" (which feels like a 10-20lb sack of flour being carried on the shoulders at all times). Update: steroids increased to 2.0mg / day as symptoms worsened. Update Jan 2014: Prednisolone at 7.5mg/day and now Methotrexate too. Balance improved from 6/10 to 8/10 and steroid "push" from 50lb equivalent to 10lb equivalent. Only traces of lesions in right cerebellum remain as of MRI in Dec. 2013.
6. WS from the US (Added April 2013)
A 56 year old man who developed fatigue and problems walking in 2010. In mid-2011, when seen by a neurologist, he reported slurred speech, mal-coordination, diplopia, numbness and feeling like he was "drunk". Brain MRI showed abnormalities in the cerebellum. Subsequent, investigations including lumbar puncture, MRI (including spectroscopy) and brain biopsy found signs of brain inflammation. Monthly "pulse" treatment with steroids resulted in symptom improvement; symptoms worsened when pulse treatment was stopped leading to a CLIPPERS diagnosis. Continued problems with fatigue, gait and speech as well as sporadic diplopia, ringing in his ears and difficulty concentrating. In April 2013, his treatment was changed to tapering oral prednisolone beginning with 60mg/day.
5. BA from the US (Added April 2013)
A 52 year old female who, in 2012, had an acute onset of vomiting and problems standing. A lesion on the brainstem was found and she had left-side paralysis and problems with swallowing and seeing. Eventually, after being severely ill, she was diagnosed with CLIPPERS following a brain biopsy, and treated with steroids and globulin. She has improved on this treatment but still has serious residual problems with left-side movement, swallowing, and perception of temperature and pain. She can now walk (with assistance in case of balance lapses) and with her left arm strapped up.
4. GK from Germany
Healthy male aged 64 who first had symptoms in December 2011: tiredness, numbness in left temple, tinnitus. Cortisone treatment in Feb. 2012 but then headache, diplopia (medium-to-long distance), dizziness, fatigue, lack of concentration, odor-sensitivity. Extensive investigation for encephalitis in late March 2012 followed by 500mg Prednisolone / day for 5 days then a decreasing dose from 100mg down to 2.5mg. Headache, diplopia and weakness recurred in June 2012 and, following a diagnosis of CLIPPERS, a second high-dose Prednisolone treatment combined with Azathioprine followed. After drug treatment was stopped at the end of August 2012 another relapse followed and the diagnosis of CLIPPERS was confirmed. Another high-dose Prednisolone treatement followed and now planning on 5mg Prednisolone a day and 200mg Azathioprine in 2013.
3. KVG from Canada
Male aged 31. No previous health issues. Initial symptoms in July 2010 of ear ache, and dizziness (lightheaded not vertigo). Followed by diplopia, right side hemiparesis, paresthesia, ataxia, and extreme vomiting. Brain biopsy in Jan 2011. Initial diagnoses with CNS Vasculitis. Treatment - Solu Medrol 1g/day x5, then decreased from 75mg Prednisone, and Cyclophosphamide IV. Symptoms improved. March 2012, attempted to come off Prednisone, and stay on Cell Cept, but led to a flare up, symptoms worsened. Solu Medrol 1g/day x3, then back up to 60mg Prednisone. Symptoms improve. Flare up led to re-diagnoses - CLIPPERS. Currently 40mg Prednisone, 150mg Cyclophosphamide. Slowly reducing Prednisone and planning on starting Methotrexate to replace Cyclophosphamide. Symptoms continue to improve very slowly, but suffer from severe dizziness and balance issues.
2. BG from the US.
Male aged 59 with no history of neurological or cardiac problems. Initial symptoms were vertigo and sinus headache in September 2011. Subsequently double vision, paresthesia, numbness, speech problems. Initial treatment 3 days of 1g/day Prednisolone then a decreasing dose starting at 60mg/day. Now also taking Methotrexate. Some residual transient double vision. (Last updated 17th May 2012)
1. BC from the UK.
Male
aged 45 with no previous history of illness. Symptoms appeared June
2011. Worsening diplopia followed over the next couple of months by
worsening ataxia and paresthesia. Diagnosed with CLIPPERS September
2011. Treated with 1mg/day x 5 days IV Prednisolone then 60mg/day
decreasing dose of Prednisolone. Symptoms mostly resolved by November
2011. Currently stable on 100mg twice-daily Azathioprine and reducing Prednisolone. Since June 2012 has been taking 100mg Azathioprine twice-daily only with no recurrence of CLIPPERS symptoms to date. In 2016 the doctors became alarmed at a low neutrophil (white-blood-cell) count and the Azathioprine dose was changed to 100mg (AM) and 50mg (PM). Despite this BC has remained well since the first CLIPPERS attack. (Last updated February 2017)
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