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

A 24-year-old male developed bulbar palsy, ophthalmoplegia, ptosis, and shoulder weakness bilaterally 2 weeks after he had experienced an upper respiratory infection. The electrodiagnostic study demonstrated axonal polyradiculoneuropathy. The repetitive nerve stimulation study (RNS) showed no significant decrement of the compound muscle action potentials (CMAPs). The videofluoroscopic swallowing study (VFSS) showed severe impairment of the pharyngeal phase of swallowing. He was diagnosed as having the pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. The patient's dysphagia was not improved for 3 months. A follow up RNS showed a significant decrement of the CMAPs. Pyridostigmine bromide was tried to improve the dysphagia. The patient showed immediate improvement of his dysphagia on the VFSS after the trial with pyridostigmine bromide. Pyridostigmine bromide was given before each meal for 8 days and he showed continuous improvement of his dysphagia. The follow up VFSS after 3 months showed complete recovery of dysphagia.
Action Potentials ; Axons ; Bulbar Palsy, Progressive ; Deglutition ; Deglutition Disorders ; Follow-Up Studies ; Guillain-Barre Syndrome ; Humans ; Male ; Meals ; Muscles ; Ophthalmoplegia ; Polyradiculoneuropathy ; Pyridostigmine Bromide ; Shoulder ; Young Adult

Neuropsychiatric manifestations in patients with systemic lupus erythematosus are fairly common, with a prevalence of 37~95%. Among 19 neuropsychiatric manifestations, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is quite rare, and is characterized by progressive, symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes. Generally, plasma exchange and intravenous immunoglobulin are the main treatment modalities. Here, we report a case of AIDP in a 29-year-old SLE patient, who was fully recovered with a treatment of high-dose glucocorticoid and immunosuppressive agents. Ours case suggests that AIDP should be treated differently in SLE patients to avoid disastrous results.
Adult ; Central Nervous System ; Guillain-Barre Syndrome* ; Humans ; Immunoglobulins ; Immunosuppressive Agents ; Lupus Erythematosus, Systemic* ; Muscle Weakness ; Plasma Exchange ; Polyradiculoneuropathy ; Prevalence ; Reflex, Stretch