VARIANTS OF AUTOIMMUNE DISEASES, - CIDP & POLYNEUROPATHY

Every autoimmune disease presents with slight variations in different patients. The variation does not mean the diseases is different or the diagnosis is different. The variation is due to the immune system selecting which place to attack, or in other words how the immune system is deceived to attack particular organs.

Lets take the case of alzheimers, in some cases just current memory is affected, in others memory just for numbers or names can be affected. Other patients will go back in time and start living 40-50 years back in their past. Some patients with alzheimers will also develop weakness, other start to have some abnormal movements while in bed or sitting. Other patients with alzheimers may lose bladder control and may also develop stiffness in their arms and legs. Many men become sexually active and develop affairs with young women, others forget to write checks. HOSTILITY AND ANGER DEVELOPS IN OTHERS DIRECTED TOWARD THE SPOUSE WHO THEY START falsely blaming as a prostitute or stealing their money. One patient started to hear noises and music and called the police to report neighbors who were quite people.

Multiple sclerosis causes variants like no ones business. Multiple sclerosis can just cause fatigue, loss of vision, weakness, numbness, hearing problems and anything you can think off.

Vasculitis presents with headaches, numb hands, cold hands and feet. Pulses are difficult to feel.

Chronic fatigue presents with tiredness all the time or after exertion. Normal sleep does not help relieve the problem. Yes this disease like all other autoimmune problems is fully reversible.

Fibromyalgia causes pain in different parts of the body, usually affects the neck muscles and the left side of body. These patients do not have a restful sleep. They may also have irritable bowel syndrome. Pain comes on after eating and none of the medical tests will show any problem.

Myofacial pain causes cracking noises in the neck and joints and knots form in the muscles.

Information on variants for all the autoimmune diseases is provided in the e-book and on CIDP is below.

Below are some reports of MS, ALS, PURE-MOTOR, PURE-SENSORY, MIXED-MOTOR SENSORY NEUROPATHY.

Much more detailed information in our autoimmune diseases e-book.

The following are direct scientific reports which may be difficult to understand for some readers. However we provide translations. In the first report a Scientist well known in CIDP circles states that the number of cases of CIDP are much more then what we think. He also states that doctors should not follow AAN (American Academy of Neurology) guidelines. The second author below states that ALS and CIDP are very similar. Recently Mayo clinic neurologist were unable to figure out a large number of Pork processing plant workers who developed CIDP. The doctors in Mayo clinic reported in press reports that CIDP could not affect the brain, spinal cord or muscles. The third report shows that eye muscle weakness can also be seen in CIDP. Vision problems can also be associated with CIDP. Only one limb can be effected in CIDP. If you have a undiagnosed problem call us. If your disease does not fit into any specific patterns then we can guide you, three will be a twenty dollar charge for this guidance.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Neurology. 2003 Apr 1;60(8 Suppl 3):S8-15.

Research criteria for defining patients with CIDP.

Sander HW, Latov N.

Peripheral Neuropathy Center, Department of Neurology, Weill Medical College of Cornell University, New York, NY 10022, USA. hws2001@med.cornell.edu

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is one of several chronic acquired demyelinating neuropathies that are considered to be autoimmune diseases. The prevalence of these illnesses may be underestimated because of limitations in clinical, serologic, and electrophysiologic diagnostic criteria. An Ad Hoc Subcommittee of the American Academy of Neurology (AAN) proposed a set of diagnostic criteria for CIDP to be used for research purposes, and several other criteria followed. Of these, the AAN electrophysiologic criteria are the most restrictive and fit only a subset of patients with CIDP.

: Rinsho Shinkeigaku. 2001 Dec;41(12):1210-3.

[New trends in neuropathy practice: clinical approach to CIDP]

Baba M.

Department of Neurological Sciences, Hirosaki University School of Medicine.

Our recent study showed that the overall prevalence of CIDP was estimated as 2.2 per 100,000 population in Aomori Prefecture, in Northan Honshu of Japan. In our series of more than 80 cases with CIDP, a chronic acquired inflammatory demyelinating polyneuropathy, nearly 30% showed clear laterality of weakness, and electrophysiologic laterality or multifocality was apparent in almost all cases. Nearly 90% of patients were able to walk without walking aids or other assistance. Sixty% showed distal dominant muscular weakness. In 12 patients with age of onset under 15, pes cavus deformity was seen in 5. Two thirds complained numbness in the extremities during progressive phase. Four cases initially showed severe sensory ataxia associated with motor conduction block. It should be, thus, reminded that clinical spectrum of CIDP is enormously wide: chronic acquired demyelinating multiple mononeuropathy showing asymmetric involvement (Lewis-Summer syndrome) should be put on one side of the clinical presentation of CIDP. Multifocal motor neuropathy (MMN) is, on the other hand, an unique syndrome mimicking amyotrophic lateral sclerosis (ALS). There may be, however, true association syndrome of CIDP and ALS presenting both peripheral nerve demyelination and pyramidal sign with progressive bulbar involvement. Recently, several atypical varieties of CIDP showing only one-limb involvement, upper limb weakness rather than lower limb power loss, or proximal weakness, etc ... have been reported in the literature. To realize such clinical variations of chronic acquired demyelinating neuropathy is important for early diagnosis and commencement of treatment of CIDP. Clinical guideline for suspicion of CIDP could be useful for general physicians and neurologists unfamiliar to peripheral neuropathies

{From the above article it is clear that some patients who have ALS can be a variant of CIDP. Since CIDP is treatable with IVIG, thus all patients with ALS should consider getting a trial of IVIG as a diagnostic tool.}

Isolated unilateral adduction deficit and ptosis as the presenting features of chronic inflammatory demyelinating polyradiculoneuropathy.

Pieh C, Rossillion B, Heritier-Barras AC, Chofflon M, Landis T, Safran AB., Geneva Switzerland.

A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) presented with an isolated unilateral adduction deficit and ptosis. Investigations were negative until the onset of limb weakness and fatigue 2 years later. At that time, electroneuromyography, cerebrospinal fluid examination, and magnetic resonance imaging confirmed the diagnosis of CIDP. Thus, ophthalmic signs can precede extremity and bulbar signs with a long latency in CIDP.

Intravenous immunoglobulin as first treatment in diabetics with concomitant distal symmetric axonal polyneuropathy and CIDP.

Cocito D, Ciaramitaro P, Isoardo G, Barbero P, Migliaretti G, Pipieri A, Proto G, Quadri R, Bergamasco B, Durelli L. Turin, Italy.

The authors investigated the impact of IVIg as first line treatment of diabetic patients suffering from chronic inflammatory demyelinating polyneuropathy (CIDP) concomitant with distal symmetric axonal polyneuropathy. Nine patients with these clinical and electrophysiological features were treated with IVIg (0.4 g/Kg/day for 5 days). Clinical and electrophysiological evaluations were performed before and after treatment. Following IVIg treatment there was no significant improvement in clinical deficit. However, there was a significant and persistent decrease in the Rankin scale score and an improvement in the demyelinating feature on nerve conduction studies. Our findings suggest that IVIg had small but detectable beneficial effects on diabetic patients with CIDP and a high degree of axonal damage.

Arch Neurol. 2002 May;59(5):758-65.

Demyelinating neuropathy in diabetes mellitus.

Sharma KR, Cross J, Farronay O, Ayyar DR, Shebert RT, Bradley WG.

Department of Neurology, University of Miami School of Medicine (M740), 1150 NW 14th St, Room 603, Miami, FL 33136, USA.

CONCLUSIONS: Demyelinating neuropathy meeting the electrophysiologic criteria for CIDP occurred in both types of DM, and its occurrence was significantly higher in diabetic than in nondiabetic patients.

J Neuroophthalmol. 1999 Mar;19(1):67-9.

Optic atrophy and chronic acquired polyneuropathy.

Lee AG, Galetta SL, Lepore FE, Appel SH.

Department of Ophthalmology, Baylor College of Medicine, Houston, Texas 77030, USA.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, multifocal disorder usually defined as limited to the peripheral nervous system. Multifocal motor neuropathy, an acquired demyelinating neuropathy with conduction block affecting motor neurons only, may be a pathogenically distinct syndrome or a predominantly motor variant of chronic inflammatory demyelinating polyneuropathy. Central nervous system demyelination including optic neuropathy has been reported uncommonly previously in these entities. We report two cases and review the literature on the possible association of optic neuropathy and chronic acquired polyneuropathy.

Neuromuscul Disord. 2000 Aug;10(6):398-406.

Childhood chronic inflammatory demyelinating polyneuropathy: clinical course and long-term outcome.

Ryan MM, Grattan-Smith PJ, Procopis PG, Morgan G, Ouvrier RA.

Department of Neurology, The Royal Alexandra Hospital for Children, Sydney, Australia. ryan_mo@a1.tch.harvard.edu

We reviewed the clinical history, electrophysiologic and pathologic findings, and response to therapy of 16 children with chronic inflammatory demyelinating polyneuropathy. The majority presented with lower limb weakness. Sensory loss was uncommon. The illness was monophasic in seven children, relapsing in six, and three had a slowly progressive course. All patients were treated with immunosuppressive agents. In 11, the initial treatment was prednisolone. All had at least a short-term response but five went on to develop a relapsing course. Intravenous immunoglobulin was the initial treatment in four patients. Three responded rapidly, with treatment being stopped after a maximum of 5 months. In resistant chronic inflammatory demyelinating neuropathy, in addition to prednisolone and immunoglobulin, plasma exchange, azathioprine, cyclosporine, methotrexate, cyclophosphamide and pulse methylprednisolone were tried at different times in different patients. On serial neurophysiologic testing slowing of nerve conduction persisted for long periods after clinical recovery. Follow-up was for an average of 10 years. When last seen 14 patients were asymptomatic, two having mild residual deficits. Childhood chronic inflammatory demyelinating neuropathy responds to conventional treatment and generally has a favourable long-term outcome.

Neurology. 2000 Jan 11;54(1):26-32.

ONE LIMB NEUROPATHY AS CIDP OR MMF

Van den Berg-Vos RM, Van den Berg LH, Franssen H, Vermeulen M, Witkamp TD, Jansen GH, van Es HW, Kerkhoff H, Wokke JH.

Department of Neurology, Rudolf Magnus Institute for Neurosciences, Utrecht, The Netherlands.

BACKGROUND: Several patients have been reported with an asymmetric sensory or sensorimotor demyelinating neuropathy not fulfilling the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy or multifocal motor neuropathy. OBJECTIVE: To present the clinical, electrophysiologic, radiologic, and pathologic features of six patients with an asymmetric sensory or sensorimotor demyelinating neuropathy. RESULTS: All six patients were initially affected in only one limb; in four patients the neuropathy progressed to other limbs in an asymmetric fashion during several years. On electrophysiologic examination, evidence of multifocal demyelination and conduction block in motor and sensory nerves was found in all patients. MRI of the brachial plexus revealed swollen nerves and an increased signal intensity on T2-weighted imaging in four patients. A biopsy sample taken from the brachial plexus of one patient revealed evidence of inflammation. All patients showed a beneficial response to IV immunoglobulin treatment. Thirty-four similar patients have been reported previously, many of whom were initially diagnosed as having various other (nontreatable) diseases. CONCLUSIONS: The authors propose calling this neuropathy "multifocal inflammatory demyelinating neuropathy" and considering it as a distinct clinical entity to facilitate early diagnosis of this treatable disorder.

Pain Med. 2002 Jun;3(2):119-27.

IVIG in the treatment of chronic pain syndromes.

Goebel A, Netal S, Schedel R, Sprotte G.

Klinik fur Anaesthesiologie, University Wurzburg, Wurzburg, Germany.

Objective. To examine the use of intravenous immunoglobulin (IVIG) in chronic pain. Design. A prospective multiple-dose, open-label cohort study in 130 consecutive patients who suffered from 12 chronic pain syndromes. The largest symptom groups were (number of patients): Fibromyalgia (48); Spinal pain (20); Complex regional pain syndrome (CRPS, 11); Peripheral neuropathic pain (12); and Atypical odontalgia or atypical facial pain (11). All patients had insufficient pain relief with established treatments. Pain relief was recorded using average pain intensity values as documented in standardized diaries. A specific treatment protocol was developed, and patients were enrolled over a 36-month period. Results. Overall, 20% of patients had>70% pain relief and 27.7% of patients reported relief between 25% and 70%. Six patients (4.6%) had moderately increased pain levels for a duration of up to 9 weeks. Good relief, of more than 70%, was found in all major symptom groups. Patients with pain of short duration (<2 years) reported high relief rates (33.8% of patients in this group reported relief openface>70%). No serious adverse events were reported. Conclusions. IVIG may be effective in patients suffering from chronic pain. Controlled studies are needed to evaluate the efficacy of IVIG in these patients. Patients with a good response to IVIG may be models for the study of neuroimmune interactions in chronic pain.

PMID: 15102158 [PubMed - in process]

: Drugs. 2003;63(3):275-87.

Management of chronic inflammatory demyelinating polyradiculoneuropathy.

Hughes RA.

Department of Clinical Neurosciences, Guy's, King's and St Thomas' School of Medicine, London, UK. richard.a.hughes@kcl.ac.uk

This review briefly describes current concepts concerning the nosological status, pathogenesis and management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CIDP is an uncommon variable disorder of unknown but probably autoimmune aetiology. The commonest form of CIDP causes more or less symmetrical progressive or relapsing weakness affecting proximal and distal muscles. Against this background the review describes the short-term responses to corticosteroids, intravenous immunoglobulin (IVIg) and plasma exchange that have been confirmed in randomised trials. In the absence of better evidence about long-term efficacy, corticosteroids or IVIg are usually favoured because of convenience. Benefit following introduction of azathioprine, cyclophosphamide, cyclosporin, other immunosuppressive agents, and interferon-beta and -alpha has been reported but randomised trials are needed to confirm these benefits. In patients with pure motor CIDP and multifocal motor neuropathy, corticosteroids may cause worsening and IVIg is more likely to be effective. General measures to rehabilitate patients and manage symptoms, including foot drop, weak hands, fatigue and pain, are important.

PMID: 12534332 [PubMed - indexed for MEDLINE]

Neurology. 1997 Feb;48(2):321-8.

Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy.

Gorson KC, Allam G, Ropper AH.

Neurology Service, St. Elizabeth's Medical Center, Boston, MA 02135, USA.

We report the clinical and EMG details of 67 consecutive patients with strictly defined chronic inflammatory demyelinating polyneuropathy (CIDP) during a 4-year period and compare responses to treatment in patients with idiopathic CIDP (CIDP-I) and CIDP with monoclonal gammopathy of uncertain significance (CIDP-MGUS). Patients were examined an average of 28 months after first symptoms. There were several variant presentations that still conformed to the clinical and electrophysiologic definitions of CIDP, including a pure motor syndrome (10%), sensory ataxic variant (12%), mononeuritis multiplex pattern (9%), paraparetic pattern (4%), and relapsing acute Guillain-Barre syndrome (16%). Pain was more frequent than in previous studies (42%). Conduction block was the commonest EMG abnormality (detected in at least one nerve in 73% of patients), but only 31% had a pure demyelinating neuropathy and the majority had some degree of axonal change. Patients with CIDP-MGUS had less severe weakness, greater imbalance, leg ataxia, vibration loss in the hands, and absent median and ulnar sensory potentials, but were as likely as CIDP-I patients to respond to plasma exchange. Seventeen of 44 patients (39%) with idiopathic CIDP improved for at least 2 months with an initial therapy. Although the response rates among plasma exchange, IVIG, and steroids were similar, functional improvement (Rankin score) was greatest with plasma exchange. Of 26 patients who failed to respond to an initial therapy, 9 (35%) benefited from an alternative treatment, and of the 11 who required a third modality 3 (27%) improved. Overall, 66% responded to one of the three main therapies for CIDP.

PMID: 9040714 [PubMed - indexed for MEDLINE]

Diabetes Metab. 2001 Apr;27(2 Pt 1):155-8.

An unusual neuropathy in a diabetic patient: evidence for intravenous immunoglobin-induced effective therapy.

Romedenne P, Mukendi R, Stasse P, Indekeu P, Buysschaert M, Colin IM.

Department of Internal Medicine, CHR-St Joseph Medical Center, Mons, Belgium.

We report the case of a 68-year old type-2 diabetic male patient who was admitted to hospital for progressive weakness in the right lower limb. Although his metabolic control was good, he lost more than 20 kg of weight. Despite intensive physio- and vitaminotherapy, his neurological condition kept on degrading with a severe amyotrophy and pain of the right thigh. He was unable to walk and to stand alone. Besides a yet known sensitive polyneuropathy, the electrophysiological study revealed an obvious motor involvement with signs of demyelination and axonal degeneration. Combined with the albuminocytologic dissociation observed in the cerebrospinal fluid, these specific clinical and electrophysiological features led us to postulate a diagnosis of inflammatory neuropathy. The patient underwent a treatment by methylprednisolone and immunoglobins that rapidly induced a striking improvement of his neurological condition. This case report illustrates that rare forms of neuropathy such as inflammatory neuropathies close to chronic inflammatory demyelinating polyneuropathy (CIDP) can occur in diabetic patients and superimpose on the more commonly described forms of neuropathies. It recalls the importance of recognizing CIDP-like neuropathies because unlike other forms of neuropathy, inflammatory neuropathies are perfectly curable.

: (the following study shows that AAN guidelines for CIDP fail to help in diagnosing patients); The spectrum of chronic inflammatory demyelinating polyneuropathy.

Rotta FT, Sussman AT, Bradley WG, Ram Ayyar D, Sharma KR, Shebert RT.

Department of Neurology, University of Miami School of Medicine, PO Box 016960, Miami, FL, USA.

Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients; whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum; of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN; research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at; least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these; inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these,; 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly; asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement,; and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the; acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than; would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.

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