A 77-year-old man developed diplopia, gait ataxia, and paresthesia. A clinical examination also revealed ophthalmoplegia, facial palsy, ataxia of the limbs and trunk, and reduced deep tender reflexes. Laboratory and electrophysiological studies revealed albuminocytological dissociation and demyelination. He was diagnosed as Miller-Fisher syndrome and received intravenous immunoglobulin therapy. His clinical symptoms deteriorated at 12 weeks after onset. We diagnosed acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, and which the patient recovered from following corticosteroid therapy.
Aged ; Ataxia ; Demyelinating Diseases ; Diplopia ; Extremities ; Facial Paralysis ; Gait Ataxia ; Guillain-Barre Syndrome ; Humans ; Immunization, Passive ; Miller Fisher Syndrome* ; Ophthalmoplegia ; Paresthesia ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Reflex

A 77-year-old man developed diplopia, gait ataxia, and paresthesia. A clinical examination also revealed ophthalmoplegia, facial palsy, ataxia of the limbs and trunk, and reduced deep tender reflexes. Laboratory and electrophysiological studies revealed albuminocytological dissociation and demyelination. He was diagnosed as Miller-Fisher syndrome and received intravenous immunoglobulin therapy. His clinical symptoms deteriorated at 12 weeks after onset. We diagnosed acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, and which the patient recovered from following corticosteroid therapy.
Aged ; Ataxia ; Demyelinating Diseases ; Diplopia ; Extremities ; Facial Paralysis ; Gait Ataxia ; Guillain-Barre Syndrome ; Humans ; Immunization, Passive ; Miller Fisher Syndrome* ; Ophthalmoplegia ; Paresthesia ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Reflex

No abstract available.
Polyradiculoneuropathy*

The Guillain-Barre Sydrome (GBS) is an acute inflammatory demylinating polyradiculoneuropathy of unknown etiology. It occurs frequently one to three weeks after a banal antecedent respiratory or gastrointestinal infection. Other preceding events include surgical procedures, viral exanthems and other viral illnesses, mycoplasma infections, the spirochetal infection of Lyrne disease, lymphomatous disease, adrninistration of vaccination and so on. We experienced 2 cases of typical GBS in clinical features, CSF finding and electrophysiological aspect after antecedent nckettsial infection. To our knowledge, this is the first discription of GBS associated with preceding rickettsial disease. We would like to suggest that rickettsial infection would be one of possible antecedent illness associated with GBS.
Exanthema ; Guillain-Barre Syndrome* ; Mycoplasma Infections ; Polyradiculoneuropathy ; Vaccination

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-Barre syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.
Cerebellar Ataxia ; Child* ; Dexamethasone ; Electromyography ; Guillain-Barre Syndrome ; Humans ; Immunoglobulins ; Korea ; Neural Conduction ; Polyneuropathies* ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Prednisolone ; Recurrence ; Retrospective Studies ; Seoul

BACKGROUND: Guillain-Barre syndrome (GBS) is defined as a recognizable clinical entity that is characterized by rapidly evolving symmetric limb weakness, loss of tendon reflexes, absent or mild sensory signs, and variable autonomic dysfunctions. Recently, GBS has been classified as a classical demyelinating (acute imflammatory demyelinating polyradiculoneuropathy, AIDP) and two axonal (acute motor axonal neuropathy, AMAN, and acute motor sensory axonal neuropathy, AMSAN) forms. The clinical pattern and prognosis according to type is not clear. METHODS: Forty-one patients clinically diagnosed as GBS were enrolled and classified as AIDP, AMAN, and AMSAN according to electrodiagnostic criteria. We analyzed the clinical data of each subgroup; age, sex, seasonal distribution, history of previous illness, cranial nerve involvement, respiratory involvement, and motor weakness. RESULTS: Forty-one patients with GBS were comprised of 19 patients (46.3%) with AIDP, 12 patients (29.2%) with AMAN, and 10 patients (24.3%) with AMSAN. AIDP was found more frequently in males and in winter (42.1%) while axonal forms of GBS showed neither gender nor seasonal predominance. Frequency of cranial nerve involvement was not different between the sub-groups of GBS, whereas respiratory involvement was more frequent in AMSAN (50%). Upper limbs were weaker in axonal than in demyelinating types of GBS. CONCLUSIONS: Axonal forms of GBS showed some clinical characteristics distinctive from the demyelinating forms, which might be useful in the differential diagnosis of subgroups of GBS. (J Korean Neurol Assoc 19(5):503~508, 2001)
Amantadine ; Axons ; Cranial Nerves ; Diagnosis, Differential ; Extremities ; Guillain-Barre Syndrome* ; Humans ; Male ; Polyradiculoneuropathy ; Prognosis ; Reflex, Stretch ; Seasons ; Upper Extremity

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

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

Guillain-Barré syndrome (GBS) is the most common immune-mediated polyradiculoneuropathy and it is also the most commonly reported severe adverse event following immunization in adults. To evaluate the results of clinical and laboratory features of GBS after vaccination in Korea, we analyzed the claims-based data from 2002 to 2014 using materials collected for the Advisory Committee Vaccination Injury Compensation (ACVIC) meeting including, clinical features, nerve conduction studies (NCSs), cerebrospinal fluid (CSF) profiles, treatment, and outcomes. Forty-eight compensated GBS cases (median age, 15 years; interquartile range [IQR], 13–51; male:female ratio, 1:1) of 68 suspected GBS were found following immunization and all of them with influenza immunizations with either monovalent (n = 35) or trivalent (n = 13). Among them, 30 cases fulfilled the Brighton criteria level 1–3 (62.5%). The median duration between the onset of symptoms to nadir, duration of the nadir, and total admission period were 3 (IQR, 2–7 days), 2 (IQR, 1–5 days), and 14 (IQR, 6–33 days) days, respectively. The most frequently reported symptom was quadriparesis which was present in 36 cases (75%) at nadir. CSF examination revealed albuminocytologic dissociation in 25.0% and NCS was abnormal in 61.8%. After treatment, most of them showed improvement. Clinical features were similar to typical post-infectious GBS and there were both demyelinating and axonal forms suggesting heterogeneous pathogenic mechanism. In order to improve the diagnostic certainty of post-vaccination GBS, careful documentation of clinical features and timely diagnostic work-up with follow-up studies are needed.
Adult ; Advisory Committees ; Axons ; Cerebrospinal Fluid ; Compensation and Redress ; Follow-Up Studies ; Guillain-Barre Syndrome* ; Humans ; Immunization ; Influenza, Human ; Korea* ; Neural Conduction ; Polyradiculoneuropathy ; Quadriplegia ; Vaccination

Guillain-Barre syndrome (GBS) is an autoimmune disease with an acute evolution of inflammatory demyelinating polyradiculoneuropathy. Although the precise immune mechanisms and involved antigens are uncertain, both humoral and cellular immune mechanisms are thought to be involved. Interactions between the various compartments of the immune system are governed by cytokines. Laboratory investigations have shown that immune activation can be quantified by measurement of cytokines and soluble immune activation products in serum. Interleukin-2(IL-2) is probably the best characterized among the many cytokines and soluble interleukin-2 receptor (sIL-2R) and neopterin are major immune activation products. In order to observe activities of cellular immunity of GBS, we measured serum concentrations of IL-2, sIL-2R and neopterin in 28 patients with GBS and in 22 healthy controls. Serial serum samples were drawn 2 to 25 days after motor onset of the disease, 2 to 3 days after treatment with plasmapheresis and 43-300days of follow-up. The occurences of IL-2 positive serum samples were 41.7%, 23.8% and 18.2% in each time in GBS but none in healthy controls. Initial serum sIL-2R and neopterin level were elevated in 21% and 17% of patients with GBS compared with healthy controls. After plasmapheresis, both serum sIL-2R and neopterin level were significantly elevated in GBS compared with initial serum samples and healthy controls. Thus, T-cell and macrophage activation may play a role in the pathogenesis of GBS. However, further study is needed to evaluate the effect of plasmapheresis and clinical severity on the serum concentration of IL-2, sIL-2R and neopterin in GBS.
Autoimmune Diseases ; Cytokines ; Follow-Up Studies ; Guillain-Barre Syndrome* ; Humans ; Immune System ; Immunity, Cellular ; Interleukin-2* ; Macrophage Activation ; Neopterin* ; Plasmapheresis ; Polyradiculoneuropathy ; Receptors, Interleukin-2* ; T-Lymphocytes