Intravenous immunoglobulin (IVIG) is used in treating many cases of autoimmune and inflammatory conditions thanks to its multiple anti-inflammatory and immunomodulatory properties. The clinical use of IVIG has been for the patients with primary immunodeficiencies, but lately it is expanding its usage to the realms of treating patients with neurological conditions. Both the efficacy and safety of IVIG treatment in chronic inflammatory demyelinating polyradiculoneuropathy and Guillain–Barré syndrome have been studied successfully. However, the use of IVIG treatment in other neurological conditions still remains investigational despite several successful reports. Considerable numbers of mechanisms have been suggested in order to explain the effects of IVIG, but the exact mechanisms are not understood yet. This review covers the new developments in clinical fields and the possible ways in which IVIG could help in the future.
Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Neurology ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represents an important acquired condition characterized by progressive, symmetrical, proximal and distal weakness. CIDP is characterized by sensory loss and weakness, areflexia, elevated CSF protein and electrodiagnostic evidence of multifocal demyelination with or without superimposed axonal degeneration. Some reports are made that an antecedent illness in the weeks preceding the onset of symptoms such as upper respiratory syndrome or flu-like illness, gastrointestinal syndrome etc., but intestinal pseudoobstruction as the main clinical feature in CIDP is an uncommon finding. The clinical course is variable. The condition is responsive to immunosuppressive therapy, especially prednisone and plasma exchange. We report a case of intestinal pseudoobstruction secondary to CIDP diagnosed by clinical features, electrodiagnostic study and nerve biopsy pathology.
Axons ; Biopsy ; Demyelinating Diseases ; Intestinal Pseudo-Obstruction* ; Pathology ; Plasma Exchange ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Prednisone

No abstract available.
Hepatitis C, Chronic ; Hepatitis, Chronic ; Humans ; Interferon-alpha ; Interferons ; Polyneuropathies ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogenous group of acquired peripheral neuropathies. A subset of CIDP involves predominantly distal parts of the limbs, which is similar to axonal polyneuropathy. The clinical course or response to treatment may be different in this group. We investigated the clinical course and electrodiagnostic findings of the distal CIDP. METHODS: Twenty five CIDP cases were reviewed retrospectively. Patients with proximal as well as distal involvement were grouped as typical CIDP, and patients with predominantly distal involvement as distal CIDP. We compared the clinical, laboratory and electrophysiological findings of these two groups. RESULTS: Sixteen patients had typical CIDP and nine had distal CIDP. Distal CIDP differed significantly from typical CIDP; later age of onset (p=0.049), less frequent relapses (p=0.041), more rapidly progressive to maximal disability (p =0.01), low disability score at the diagnosis (p=0.02) and after treatment (p=0.01), poor response to immunomodulating therapy (p=0.02), and infrequent conduction blocks or abnormal temporal dispersions (p<0.01). CONCLUSIONS: Distal CIDP is a distinctive variant of CIDP different from typical CIDP in clinical and electrophysiological features. Identification of the pathogenesis underlying this new entity may lead to better understanding of the heterogeneous acquired demyelinating neuropathies.
Age of Onset ; Axons ; Diagnosis ; Extremities ; Humans ; Peripheral Nervous System Diseases ; Polyneuropathies ; Polyradiculoneuropathy ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Recurrence ; Retrospective Studies

Preliminary studies have evaluated the effects of interferon beta formulations in the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) because of pathogenic similarities between CIDP and multiple sclerosis (MS). However, the efficacy of Interferon, which has been widely used for relapsing-remitting MS, is controversial in CIDP. We report here a 31year old woman with relapsing-remitting type MS treated with IFN beta-1b over 2 years who developed overt CIDP. She responded favorably to steroids. This case suggests that IFN beta-1b treatment may not prevent development of CIDP.
Female ; Humans ; Interferon-beta* ; Interferons ; Multiple Sclerosis* ; Polyneuropathies* ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Steroids

Since the clinical features were first delineated in 1975 by Dyck et al, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been clearly recognized as a separate clinical entity. The diagnosis of CIDP can be made when there are a symmetrical motor & sensory polyneuropathy with chronic progression, high CSF protein level and marked nerve conduction abnormalities of demyelinating type, which occur in the absence of associated illness. From July 1985 to June 1987, authors have observed seven cases of CIDP. Prednisone was begun at a daily dosage of 1-2mg per kg when each patient fulfilled the above described critera. Significant clinical improvement was recorded in six out of seven cases within four weeks after initiation of corticosteriod therapy. In one case dramatic relief of tingling sensation was observed within 5th day, but motor power improved gradually during the sixth to eighth weeks of daily theapy. No significant side effects of prednisone was found in all cases except for one, who suffered from compressed fracture of lumbar vertebra with minor physical trauma. Therefore it could be concluded that corticosteriod therapy seems to have beneficial effect on patients with severe degree of CIDP.
Diagnosis ; Humans ; Neural Conduction ; Polyneuropathies ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Prednisone ; Sensation ; Spine

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy with a relaps-ing or progressive course. Although the occurrence of CIDP is very rare in childhood compared to the occurrence in adulthood, it does occur in children. In childhood CIDP, the main clinical features are somewhat different from that of adults, which includes a more precipitous onset of symptoms, a high frequency of gait abnormalities, and significant neurological dysfunction. Also, the response to immune-modulating therapy is more reliable and rapid in children than in adults. The recognition of childhood CIDP is important as it may mimic hereditary neuropathies which usually pre-sent with progressive gait disturbance associated with pes cavus deformity. A careful history, clinical examination, and electrophysiological study is crucial for the accurate diagnosis of this treatable disease. Here, we report a case of CIDP in a 13-year-old Korean girl whose initial clinical presentation strongly suggested hereditary motor-sensory neuropathy.
Adolescent* ; Adult ; Child ; Congenital Abnormalities* ; Diagnosis ; Female* ; Foot Deformities* ; Gait ; Humans ; Polyneuropathies* ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating

Electrodiagnostic tests (EDX) is essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). EDX could provide information about demyelinating pathology in the peripheral nerves. According to phenotypes, CIDP could be classified several phenotypes, which has different clinical manifestations, EDX could present a different distribution pattern of demyelinating lesions. In addition, EDX could be useful markers for predicting treatment response of prognosis of CIDP.
Classification ; Diagnosis ; Electrodiagnosis ; Neural Conduction ; Pathology ; Peripheral Nerves ; Phenotype ; Polyneuropathies ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Prognosis

BACKGROUND: Multifocal motor neuropathy (MMN), Lewis-Sumner syndrome (LSS), and many chronic inflammatory demyelinating polyradiculoneuropathies (CIDPs) are representative of acquired multifocal polyneuropathy and are characterized by conduction block (CB). This retrospective study aimed to investigate the demyelinating distribution and the selective vulnerability of MMN, LSS, and CIDP with CB (CIDP-CB) in nerves. METHODS: Fifteen LSS subjects (107 nerves), 24 MMN subjects (176 nerves), and 17 CIDP-CB subjects (110 nerves) were included. Their clinical information was recorded, blood and cerebrospinal fluid tests were conducted, and nerve conductions of the median, ulnar, radial, peroneal, and tibial nerves were evaluated. CB, temporal dispersion, distal motor latency (DML), and F-wave latency were recorded, and nerve conduction velocity, terminal latency index, and modified F-wave ratio were calculated. RESULTS: CB was more likely to occur around the elbow in CIDP-CB than in MMN (78.6% vs. 6.8%, P < 0.01) but less likely to occur between the wrist and the elbow than in LSS (10.7% vs. 39.3%, P < 0.05). Tibial nerve CB was most frequently observed in MMN (47.4%, P < 0.05). CIDP-CB was characterized by a prolonged DML in all nerves, and slow motor nerve velocity of the upper limb was significant when CB nerves were excluded (P < 0.05). CONCLUSIONS We report the different distributions of segmental and diffuse demyelination of the ulnar and tibial nerves in LSS, MMN, and CIDP-CB. These distinct distributions could help in differentiating among these conditions.
Humans ; Neural Conduction ; Peripheral Nerves ; Polyneuropathies ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Retrospective Studies

Chronic inflammatory demyelinating polyneuropathy (CIDP) has been rarely reported in systemic sclerosis (SSc). We describe the clinical, electrophysiological, and pathologic findings in a 73-year-old female with established limited cutaneous SSc who later developed CIDP. The patient had progressive limb weakness, sensory loss, and slow nerve conduction velocities. Sural nerve biopsy showed evidence of demyelination and remyelination. This case demonstrates that chronic demyelinating polyneuropathy can be an unusual manifestation of SSc, presumably resulting from an immune-mediated process.
Aged ; Autoimmunity ; Biopsy ; Demyelinating Diseases ; Extremities ; Female ; Humans ; Neural Conduction ; Polyneuropathies* ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Scleroderma, Systemic* ; Sural Nerve