Biomaterial Database

Chronic inflammatory demyelinating polyneuropathy in common variable immunodeficiency. 2012

Özlem Özdemir, and Mehmet S Okan, and Sara S Kilic

Division of Pediatric Neurology, Department of Pediatrics, Medical Faculty, Uludag University, Bursa, Turkey.

Common variable immunodeficiency comprises a heterogeneous group of primary antibody deficiencies with complex clinical and immunologic phenotypes. Immune dysregulation leads to the generation of multiple autoantibodies against various antigenic targets in patients with common variable immunodeficiency. Chronic inflammatory demyelinating polyneuropathy is a heterogeneous disorder that indicates an autoimmune response against peripheral nerve myelin. We describe a 7-year-old girl with common variable immunodeficiency who developed chronic inflammatory polyneuropathy. A 5-day course of intravenous immunoglobulin (500 mg/kg/day) improved her neurologic disorder. Chronic inflammatory demyelinating polyneuropathy should be added to the broadening spectrum of neurologic complications in common variable immunodeficiency. Early detection and consequent treatment may reverse the neurologic sequelae.

UI	MeSH Term	Description	Entries
D009431	Neural Conduction	The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.	Nerve Conduction,Conduction, Nerve,Conduction, Neural,Conductions, Nerve,Conductions, Neural,Nerve Conductions,Neural Conductions
D010525	Peripheral Nerves	The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.	Endoneurium,Epineurium,Perineurium,Endoneuriums,Epineuriums,Nerve, Peripheral,Nerves, Peripheral,Perineuriums,Peripheral Nerve
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D017074	Common Variable Immunodeficiency	Heterogeneous group of immunodeficiency syndromes characterized by hypogammaglobulinemia of most isotypes, variable B-cell defects, and the presence of recurrent bacterial infections.	Immunodeficiency, Common Variable,Common Variable Hypogammaglobulinemia,Common Variable Immune Deficiency,Hypogammaglobulinemia, Acquired,Immunoglobulin Deficiency, Late-Onset,Acquired Hypogammaglobulinemia,Acquired Hypogammaglobulinemias,Common Variable Hypogammaglobulinemias,Common Variable Immunodeficiencies,Deficiencies, Late-Onset Immunoglobulin,Deficiency, Late-Onset Immunoglobulin,Hypogammaglobulinemia, Common Variable,Hypogammaglobulinemias, Acquired,Hypogammaglobulinemias, Common Variable,Immunodeficiencies, Common Variable,Immunoglobulin Deficiencies, Late-Onset,Immunoglobulin Deficiency, Late Onset,Late-Onset Immunoglobulin Deficiencies,Late-Onset Immunoglobulin Deficiency,Variable Hypogammaglobulinemia, Common,Variable Hypogammaglobulinemias, Common
D020277	Polyradiculoneuropathy, Chronic Inflammatory Demyelinating	A slowly progressive autoimmune demyelinating disease of peripheral nerves and nerve roots. Clinical manifestations include weakness and sensory loss in the extremities and enlargement of peripheral nerves. The course may be relapsing-remitting or demonstrate a step-wise progression. Protein is usually elevated in the spinal fluid and cranial nerves are typically spared. GUILLAIN-BARRE SYNDROME features a relatively rapid progression of disease which distinguishes it from this condition. (Adams et al., Principles of Neurology, 6th ed, p1337)	CIDP,Chronic Inflammatory Demyelinating Polyradiculoneuropathy,Chronic Inflammatory Polyradiculoneuropathy,Inflammatory Polyradiculopathy, Chronic,Polyneuropathy, Inflammatory Demyelinating, Chronic,Polyradiculoneuropathy, Chronic Inflammatory,Chronic Inflammatory Polyradiculoneuropathies,Chronic Inflammatory Polyradiculopathies,Chronic Inflammatory Polyradiculopathy,Inflammatory Polyradiculoneuropathies, Chronic,Inflammatory Polyradiculoneuropathy, Chronic,Inflammatory Polyradiculopathies, Chronic,Polyradiculoneuropathies, Chronic Inflammatory,Polyradiculopathies, Chronic Inflammatory,Polyradiculopathy, Chronic Inflammatory