OTHER PEOPLE’S STORIES
of PDN/MGUS-associated neuropathy.
In the past 6 yearsI have been contacted by several men and women who have been diagnosed with this neuropathy. Their stories vary considerably although there are common factors and experiences.
Several have readily agreedthat a brief account of their personal story may appear on this page, believing that this may be of help to others.
LM of Vienna, Virginia, USA was diagnosed with anti-MAG IgM-associated neuropathy in late 2002, at the age of 53. Early in 2004 the symptoms in his left foot became more pronounced so that he now walks best with help from an orthotic device – a Walk-on brace made by Otto Bock. Also early in 2004 he underwent a series of 4 weekly treatments with rituximab/rituxan: this produced a very modest improvement in strength. He takes 2,100mg of neurontin a day to alleviate pain. LM regularly visits the neurology department of the Baltimore John Hopkins Hospital where, says LM, Dr David Cornblatt and his team specialise in PN patients.
Now 50 years of age, TM of Richmond, Virginia, USAtells his story: I was diagnosed in 1998. I had pulled into my driveway, missed the brake completely and hit the garage. A Peripheral Neuropathy was diagnosed and the MGUS was found 2 months later. My IgM, of the non-anti MAG type, was at a significant level. I realised that I’d had the PN symptoms for years, probably since 1993, but had tried to ignore them. By 1998 I had little strength in my hands, had difficulty in holding objects, could fall in a dark room because of poor balance sense and became readily fatigued. Mowing the lawn could knock me out for a day. After diagnosis I endured 16 months of plasmapheresis treatments and then 4 different chemotherapies. Improvement was slight and after treatments were stopped my IgM levels increased. My oncologist had nothing left to offer me. Then in 2002 I read on the internet about a study involving MGUS and Rituxan. My oncologist eagerly pursued this when I showed it to her. 4 Rituxan programs (every 6 months) have been a blessing. My IgM levels have stabilized and although I still exhibit ataxia the severity has dropped. I have gained in strength and stamina and can function very well now, in spite of my sometimes "drunken sailor walk". Often I consider myself to be lucky compared with some PN sufferers who cannot obtain treatment because little is known about the cause of their neuropathy.TM has managed to continue working. He is married with 2 children.
PL, a lady who lives near London (UK) and is 84 years of age, first became aware of symptoms in her feet in the summer of 1995 that were only confirmed as being a peripheral neuropathy 3 years later. In between she had been encouraged by a doctor to wear ‘trainers’, to join in water aerobics and to join a gym circuit and then told that she had arthritis in her knees. At one stage she was accused of time wasting! Then in early 1998 a hospital department found that there really was a problem with the nerves in her feet and lower legs and she was referred to another hospital. Various tests showed the peripheral neuropathy and the presence in her blood of an excess of one protein, an M-protein or paraprotein without ever being informed of which one. In January 1999 a neurologist explained the neuropathy to her – CIDP associated with a paraprotein – and she had several plasma exchange treatments in the following months.
There has been no improvement since then though and really "everything is worse in fact", a slow progression. In mid-2004 her neurologist was concerned about the deterioration in her feet and arranged for her to have 5 days of IViG treatment. PL did not experience any improvement from this.
She has frequent balance problems and much tightness of her foot muscles, accompanied by numbness or at other times by burning sensations. Splints are worn and she benefits from using a zimmer frame. Her enjoyment of gardening, with buckets, tools, mats and plastic bags attached to the zimmer frame is one consolation. She adds," Perhaps my frustration is down to my age! I’d like to knock a few years off and this neuropathy with them."
LB of Florida, USA, now 62 years of age, was diagnosed with anti-MAG IgM-associated neuropathy. The neuropathy had progressively developed, with very mild symptoms,
over a dozen years or so from 1990 when he was 48. "Symptoms that were really more noticeable in retrospect than they were at the time", writes LB.
He continues: By the mid-1990’s these symptoms became more pronounced and pain began to develop, constant burning pain as well as numbness in both feet, gradually extending up the lower legs. Further symptoms included increased "intention tremors" in both hands, imbalance when on my feet and rather tipsy gait. By 2000 and up to now the symptoms have become quite pronounced so that I have progressively developed a significant functional disability. Activities such as using a knife and fork, shaving with a safety razor, using a touch-tone telephone and handwriting have become more difficult due to the tremor. Worst of all is the extraordinary searing pain in the bottom of both feet, qualitatively and quantitatively different from the burning pain in my extremities.
TB, who lives in Stratford-upon-Avon, England and is 57 years of age, has experienced typical symptoms of peripheral neuropathy since 1994. They progressed in his hands and feet, severely restricting his mobility and dexterity whilst trying to maintain full-time employment and bring up teenagers. The symptoms had baffled his doctors and local specialists and only resulted in several incorrect diagnoses and treatments.
Frustrated he did some research and then ‘negotiated’ his way to a prominent UK specialist in peripheral nerve inflammatory, demyelinating disorders. Following a typical battery of tests, and some initial inconclusive results, TB was eventually diagnosed with CIDP in 1997. Sometime later raised levels of IgM kappa were identified but whether this was the underlying cause of the neuropathy seemed uncertain but it seems that he is a typical case of MGUS-associated neuropathy or CIDP associated with a paraprotein.
Symptoms have been relieved by Immunoglobulin infusion since 1997, initially at the London National Hospital for Nervous Diseases but later nearer home in Birmingham. The IViG treatment is currently administered over a two day period each month. Benefits vary considerably following each treatment but mobility and sensation have certainly improved. The downside is that the side effects of the IViG appear to be worsening and an intolerance developing. Gabapentin (neurontin) is taken at a daily dose of 1800mg to relieve burning pain.
EP, now 60,
first became aware of tingling sensations in the bottom of his feet in 1990. For years the symptoms were not terrible and being a runner he thought that he was suffering from tendonitis and muscle soreness. In 2000 the tingling turned to numbness and burning sensations. His doctor sent him to a neurologist whose tests included the serum test by electropheresis. This confirmed the presence of extra amount of a protein "that shouldn’t be there." This turned out to be the IgG type monoclonal gammopathy. So it was confirmed that EP has PDN or MGUS-associated neuropathy. In 2002 he reported that despite the tingling, numbness and cramps/tightness he otherwise felt well, possibly due to years of running.
Now, in late 2004, he is aware that the numbness in his feet has spread slowly and he states that he has given up pavement running, but more due to other runners' problems! Instead he does 30 minutes daily treadmill exercise, which he feels is vital to help counter the neuropathy problems, for as long as the disorder allows.
SZ reports that he is a 36 year old North Carolina (USA) resident of oriental background. He had experienced peripheral neuropathy symptoms for 2 years before they became more severe. Then he was diagnosed with CIDP associated with IgG MGUS.
He had prednisone treatment for 18 months, although this was only effective at high doses. A switch to IVIG treatment accompanied by prednisone was made in August 2003, initially for 2 monthly treatments. When prednisone was withdrawn he needed monthly IVIG.
In March 2004 he had a series of 4 weekly infusions of Rituxan, but did not improve. It was then backed up with IVIG in mid-May since when he has improved considerably.
HS, now 83,was diagnosed as having IgG MGUS (kappa chain) in mid-1997. A bone marrow biopsy excluded the possibility of any malignancies. Various cytotoxic drugs were tried without benefit.
Yet in retrospect he recalls that in his 60’s he had loss of balance whilst showering, became more clumsy and tripped easily. His doctor noted proprioception and sent him to a neurologist who confirmed some ataxia and apparent polyneuropathy. Meanwhile the ataxia and clumsiness increased and after several neurologists’ consultations he was diagnosed in 1994 as having "polyneuropathy, idiopathic, with sensory ataxia."
Eventually he saw a famous neurologist. By late 1998 additional diagnosis included polyneuropathy findings including severe loss of lower limb function, advanced demyelination and loss of large muscle fiber. For 6 weeks in mid-1999 he had 12 successive plasmapheresis treatments but with no discernible benefit, although he experienced apparent side effects of shingles on his vocal cords. A further unhelpful plasmapheresis course in mid-2000 was followed a year later by a course of tacrolimus and azathioprine as immunotherapies. Untoward reactions led to cessation of this treatment. Mid 2003 low dose Toparimate was tried for possible stimulus of new nerve fibres, again without benefit.
"It seems that that the paraprotein process is ‘arrested’ but still ‘smolders’ at low level", HS reports. There is considerable muscle atrophy in the lower extremities and much decreased flexion in feet and ankles as well as prehensility in both hands. Ankle to calf orthotics on both legs were ordered to counter HS’s "foot drop".
Yet he still does aerobics and weight lifting for an hour or more every day, drives a car, goes bathing and works in the garden!
ADDED FEBRUARY 2005.
JW of NSW, Australia is a still active farmer. He was first aware of peripheral neuropathy type numbness symptoms [numb finger tips and at tips of his toes] in 1987. When these developed to the "glove and stocking stage and with weaknesses in his hands he chose to go to his local doctor about the problems. He was referred to a neurologist but this did not lead to a helpful diagnostic experience. As he weakened he was fortunate to be put in contact with a neurologist experienced in the "inflammatory, demyelinating" disorders.
Speedy testing led to a diagnosis of demyelination but with little axonal damage. Although his broad diagnosis is that of CIDP, immunologists at 2 of Sydney's major university teaching hospitals are convinced that paraproteinaemia is the cause. So CIDP associated with paraproteinaemia or PDN as the diagnosis appears to be the best statement.
JW began a regime of prednisone and plasmapheresis treatment, which needed adjustment from time to time. For 9 years he also had Imuran (azothiaprin) treatment but was relieved to have this withdrawn. Skin cancers developed and had to be cut out. Plasmapheresis treatment is now at approximately monthly intervals. He has been fortunate to have avoided major side effects from the prednisone, his neurologist attributing this to his working life style! he does have a list of minor ones, although snapped ligaments/muscles would not appear on everyone's list of minor side-effects!!!!!!!!!
Careful checks of his paraprotein levels over almost 18 years have not shown any worries about developing to a malignant disorder such as multiple myeloma.
JW still does a day's work on the farm in spite of "some weakness, numbness and shakiness". Taking his time with all jobs "is the best way to keep away from accidents" he says. He enjoys his family life and they regard their 8 grandchildren as a blessing. JW and his wife are active worshippers and members of their local Baptist church.