myelin-basic-protein and Adrenoleukodystrophy

Topics: Adrenoleukodystrophy; Antidepressive Agents, Tricyclic; Antipsychotic Agents; Autoimmunity; Benzodiazepines; Diffuse Cerebral Sclerosis of Schilder; Encephalomyelitis, Acute Disseminated; Humans; Methylprednisolone; Multiple Sclerosis; Myelin Basic Protein; Myelin Proteolipid Protein; Neuromyelitis Optica; Olanzapine; Pirenzepine; Pulse Therapy, Drug; Selective Serotonin Reuptake Inhibitors; T-Lymphocytes

We analyzed the sera of 51 patients with various phenotypes of X-linked adrenoleukodystrophy (X-ALD), 20 patients with multiple sclerosis (MS) and 22 healthy volunteers for the presence of autoantibodies specific for the recombinant extracellular immunoglobulin-like domain of human myelin oligodendrocyte glycoprotein (rhMOG(Igd)) and myelin basic protein (MBP). Anti-rhMOG(Igd) autoantibodies were significantly more frequent in X-ALD and MS patients as opposed to healthy individuals (p<0.05). Anti-MBP autoantibodies were present in about one-fourth of X-ALD and MS patients but in less than 10% of healthy individuals. Anti-rhMOG(Igd) autoantibody responses were not accompanied by increased T cell reactivity against rhMOG(Igd). These findings may have important implications for the understanding of humoral anti-myelin immunoreactivity in demyelinating diseases of the central nervous system such as X-ALD and MS.

Topics: Adolescent; Adrenoleukodystrophy; Adult; Autoantibodies; Female; Genetic Linkage; Humans; Male; Middle Aged; Multiple Sclerosis; Myelin Basic Protein; Myelin Proteins; Myelin-Associated Glycoprotein; Myelin-Oligodendrocyte Glycoprotein; Phenotype; Recombinant Proteins; X Chromosome

The childhood-onset cerebral form of adrenoleukodystrophy has a devastating neurologic prognosis. Unfortunately, there is no early method of distinguishing it from the more benign forms of adrenoleukodystrophy, such as adrenomyeloneuropathy. To evaluate the manner in which this disease entity may be reflected in the cerebrospinal fluid, we studied a consecutive series of 19 patients, all with biochemically proved adrenoleukodystrophy. total protein, immunoglobulin production, cytokine levels, and cerebrospinal fluid pressure were measured. In this single sample of cerebrospinal fluid, a significant correlation existed between clinical stage of the illness and cerebrospinal fluid myelin basic protein. No correlation existed with total protein, cytokines, or measures of immunoglobulin production.

Topics: Adolescent; Adrenoleukodystrophy; Cerebrospinal Fluid Pressure; Cerebrospinal Fluid Proteins; Child; Child, Preschool; Cytokines; Diagnosis, Differential; Female; Humans; Immunoglobulin G; Male; Myelin Basic Protein; Prognosis; Severity of Illness Index

One case of each of the following human leukodystrophies was examined immunocytochemically with antisera against myelin basic protein (MBP), myelin associated glycoprotein (MAG) and gliofibrillary acidic protein (GFA): Metachromatic leukodystrophy (MLD), connatal adrenoleukodystrophy (ALD), sudanophilic leukodystrophy of the adult (SLD) and connatal Pelizaeus-Merzbacher disease (PMD). A case of canine globoid cell leukodystrophy (GLD) was also included under the assumption that this disease was the same in the dog as in man. It was shown that the storage process in MLD and GLD did not involve MBP or MAG and that the breakdown of myelin with the formation of fat granule cells containing droplets of neutral fat in ALD and SLS proceeds in a similar way as in experimental Wallerian degeneration. In PMD, MBP is present in the vicinity of axons not surrounded by a myelin sheath demonstrable with conventional means. The globoid cells of GLD could be demonstrated to be of non-astrocytic origin.

Topics: Adolescent; Adrenoleukodystrophy; Animals; Child, Preschool; Diffuse Cerebral Sclerosis of Schilder; Dogs; Female; Glial Fibrillary Acidic Protein; Humans; Intermediate Filament Proteins; Leukodystrophy, Globoid Cell; Leukodystrophy, Metachromatic; Male; Middle Aged; Myelin Basic Protein; Myelin Proteins; Myelin-Associated Glycoprotein