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Sat, 31 May 2010 17:55:07

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Diabetic cidp

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Successful treatment of neuropathies in patients with diabetes mellitus.

Progressive peripheral neuropathy in patients with diabetes mellitus, which respond to anti-inflammatory and/or anti-immune treatment.

Twenty-one patients with diabetes mellitus to whom we gave anti-inflammatory and/or anti-immune treatment for progressiveperipheral neuropathy during the past 6 years.

MAIN OUTCOME MEASURES:

Patients were interviewed and examined at intervals before and after beginning treatment with intravenousimmunoglobulin (n = 15), prednisone (n = 13), cyclophosphamide (n = 5), plasma exchange (n = 3), and azathioprine (n = 1) (alone or in combination).

RESULTS:

Fifteen patients had evidence of axonal neuropathy by electrophysiologic studies (group A). All 15 patients had non-insulin-dependent diabetes mellitus, 10 patients had weight loss, and 13 patients had prominent involvement of thighs and/or thoracic bands consistent withdiabeticamyotrophy or mononeuropathy multiplex. Small vessel disease was seen in all 10 patients who underwent biopsy, with perivascular or vascular inflammation seen in seven patients. Six patients had demyelinatingneuropathyby electrophysiologic criteria (group B). All these patients had insulin-dependent diabetes mellitus, and no one had weight loss. The process was asymmetric in three patients and involved thoracic or abdominal regions in two patients. Onion bulbs were seen in all four patients who underwent biopsy, but no vascular inflammation or occlusion was seen. In all patients in both groups, worsening of their conditions stopped and improvement started after beginning treatment.

CONCLUSION:

Neuropathies responsive to anti-inflammatory and/or anti-immune therapy in patients with diabetes mellitus include (1) multifocal axonalneuropathycaused by inflammatory vasculopathy, predominantly in patients with non-insulin-dependent diabetes mellitus, indistinguishable from diabetic proximal neuropathy or mononeuropathy multiplex, and (2) demyelinating neuropathy indistinguishable from chronic inflammatory demyelinating polyneuropathy, predominantly in patients with insulin-dependent diabetes mellitus.

J Diabetes Complications.2014 Nov 6. pii: S1056-8727(14)00326-2. doi: 10.1016/j.jdiacomp.2014.10.012.

CIDP variants in diabetes: measuring treatment response with a small nerve fiber test.

Nevoret ML¹,Vinik AI 2

Abstract

INTRODUCTION:

Chronic inflammatory demyelinating polyneuropathy is eleven times more common among people with diabetes than the general population and is treatable with appropriate immunotherapy. Treatment response is usually measured clinically (symptomatic and functional improvement). We present a case of a patient with type 2 diabetes (T2D) and CIDP whose treatment response was measurable with the Sudoscan sudomotor function test. This test may represent a new objective evaluation of the treatment of CIDP.

CASE DESCRIPTION:

The patient is a 60year old male initially referred to our center in August 2012, at which time he was diagnosed with CIDP based on AAN electrodiagnostic criteria (NCS). Autonomic functions were significant for low heart rate variability response to expiration/inspiration (E/I), Valsalva maneuver and the ratio of the RR interval for the 30th to the 15th beat upon standing (1.08, 1.12, 1.05 respectively), and frequency analysis of the total spectral power, the standard deviation of the normal RR intervals (sdNN) and their root mean squared (rmsSD). Sudoscan electrochemical skin conductances (ESC), measuring small nerve fiber function on the palms and soles, were very low: 23 μS in the feet and 32 μS in the hands. After one cycle of intravenous immunoglobulin (IVIG: 6 doses total, 75g each) the patient had no change in symptoms of burning, numbness, shooting pains, and gait impairment. However, E/I, Valsalva, and 30:15 ratios (1.19, 1.36, 1.39 respectively) were improved, as were NCS. Sudoscan scores for feet and hands were unchanged (23 μS and 32 μS). In March 2013, the patient's autonomic functions worsened (E/I, Valsalva, and 30:15 ratios 1.1, 1.07, 1.12 respectively), but feet and hand ESC started to show improvement (35 μS and 52 μS respectively). Azathioprine was started. Eight days after a second cycle of IVIG in January 2014, the patient reported for the first time less burning, shooting pains and tingling. E/I, Valsalva, and 30:15 ratios remained low (1.03, 1.07, and not analyzable, respectively), while foot and hand ESC scores continued to improve (43 μS and 55 μS respectively).

DISCUSSION:

CIDP diagnosis and treatment response are difficult in the diabetic patient. We found that NCS and autonomic function tests did not correlate well with clinical status while numerical Sudoscan scores matched closely symptomatic changes. ESC have been found to correlate well with peripheral small fiberfunction and neuropathic symptoms in DPN. The findings in this patient warrant further investigation of the use of Sudoscan to monitor CIDP response to therapy.

Brain Nerve.2014 Feb;66(2):185-9.

A case of primaryerythromelalgiasuccessfully treated with high-doseintravenousimmunoglobulin therapy.

Kuroda T¹,Sugimoto A,Ishigaki S,Murakami H Kawamura M.

Abstract

is a rare condition characterized by constant or paroxysmal burning pain, erythema, and the elevation of skin temperature in the extremities. Recently, the impairment of C-fiberfunction due to autoimmune system involvement is considered as the primary cause of erythromelalgia. However, a successful treatment has yet not been established. We report a case of a 39-year-old woman with primaryerythromelalgiaaccompanied by high cerebrospinal fluid protein concentration and axonalneuropathy. She received various antiepileptic and anti-inflammatory drugs, but failed to improve. She finally underwent high-doseintravenousimmunoglobulin therapy, which dramatically improved her symptoms and normalized cerebrospinal fluid protein concentration. This result demonstrates the effectiveness of high-dose intravenous immunoglobulin therapy for the treatment of primary erythromelalgia and the possibility of autoimmune system involvement.

Pediatrics. Apr 2013; 131(4): e1091-1100.

Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes

Anne Louise Oaklander, MD, PhD^a,bandMax M. Klein, PhD^a

OBJECTIVE:

We tested the hypothesis that acquired small-fiber polyneuropathy (SFPN), previously uncharacterized in children, contributes to unexplained pediatric widespread pain syndromes.

METHODS:

Forty-one consecutive patients evaluated for unexplained widespread pain

Ninety-eight percent of patients had other somatic complaints consistent with SFPN dysautonomia (90% cardiovascular, 82% gastrointestinal, and 34% urologic), 83% reported chronic fatigue, and 63% had chronic headache. Neurologic examinations identified reduced sensation in 68% and vasomotor abnormalities in 55%, including 23% with erythromelalgia. Exhaustive investigations for SFPN causality identified only history of autoimmune illnesses in 33% and serologic markers of disordered immunity in 89%. Treatment with corticosteroids and/or intravenous immune globulin objectively and subjectively benefited 80% of patients (12/15).

Electromyography (= 20) was noncontributory, with only upper-limb denervation identified in 1 patient studied during brachial plexitis. Nerve conduction studies (= 24) were noncontributory, with polyneuropathy identified in only 2 patients: one with long-standing type 1 diabetes and the other with renal failure from Goodpasture's syndrome. Five patients had borderline or isolated sensory abnormalities that might represent subclinical large-fiber involvement.

IVIG was tried in 11 patients who were unresponsive to corticosteroids or were steroid responsive but required long-duration treatment. Three had insufficient treatment for analysis (≤3 doses of 2 g/kg). Among the 8 treated with the standard regimen for autoimmune polyneuropathy (≥3 times with 2 g/kg/month), 3 (38%) did not respond and discontinued treatment, and 5 (62%) had documented significant improvement and continued treatment. Typical infusion-related symptoms responded to standard treatments. One significant adverse event (rash + deep vein thrombosis) resolved. One patient relapsed after IVIG taper and required a few additional treatments.

Mod Rheumatol.2009;19(4):437-40. doi: 10.1007/s10165-009-0180-2. Epub 2009 May 22.

Extreme efficacy ofintravenousimmunoglobulin therapy for severe burning pain in a patient with smallfiber neuropathy

Wakasugi D¹,Kawaguchi Y,Yamanaka H,Hara

Abstract

Neurological involvement occurs in approximately 20% of patients with primary Sjogren's syndrome. Although neurological symptoms can affect the peripheral nervous system and the central nervous system, the most frequent symptom is polyneuropathy.Small fiber neuropathy(SFN) is a form of painful peripheral polyneuropathy that is common in patients with diabetic neuropathy, but may also occur in toxic, infectious, or immune-mediated neuropathy. We show here a patient with Sjogren's syndrome who developed SFN and was treated withintravenousimmunoglobulin (IVIG) therapy, which was immediately and extremely effective. Because of the efficacy ofIVIGtherapy, we propose that direct immune-mediated mechanisms may be involved in the pathogenesis of SFN complicated by Sjogren's syndrome.

Eur J Neurol.2008 Dec;15(12):

Effect of intravenous immunoglobulin on cerebellar ataxia and neuropathic pain associated with celiac disease.

Souayah N¹,Chin RL, Brannagan TH, Green PH,Kokoszka A,Sanders
BACKGROUND:

Cerebellar syndrome and small fiber neuropathy may complicate celiac disease (CD) and may be resistant to a strict gluten-free diet.

RESULTS:

We report three patients with biopsy-proven CD who developed cerebellar ataxia and neuropathic pain despite strict adherence to a gluten-free diet. A small fiber neuropathy was suggested by skin biopsy findings in two patients. All patients' symptoms, including small fiber neuropathy symptoms, responded to treatment withintravenousimmunoglobulin (IVIG). Discontinuation ofIVIGin two patients resulted in worsened ataxia that reversed after resumption ofIVIG.

CONCLUSION:

Intravenous immunoglobulin may be effective in treating cerebellar ataxia and smallfiber neuropathy associated with CD, suggesting an immune pathogenesis. Further prospective, controlled studies are necessary to determine the long-term response toIVIGor other immunomodulation therapy.

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