ABSTRACT
Children with the opsoclonus-myoclonus syndrome (OMS) usually respond to corticotropin (adrenocorticotrophic hormone, ACTH) treatment but the mechanism of benefit is unknown. We previously showed that both cerebrospinal fluid (CSF) homovanillic acid (HVA) and 5-hydroxyindole-acetic acid (5-HIAA) concentrations are low in pediatric OMS. In this study, we measured levels of CSF Dopa, catecholamines, deaminated metabolites of catecholamines, as well as HVA and 5-HIAA in eight patients before and during treatment with ACTH. All the children were ACTH-responsive with 50-70% improvement in multiple clinical features of OMS. ACTH treatment reduced the HVA concentration in every child by a mean of 21% (p < 0.001). Treatment with ACTH was associated with significant correlations between dopaminergic markers such as HVA, dihydroxyphenylacetic acid (DOPAC), and Dopa. There were no significant changes in the CSF concentrations of the noradrenergic markers norepinephrine (NE) and dihydroxyphenylglycol (DHPG), or the serotonergic marker 5-HIAA. The only child with a marked inflammatory pattern in CSF, which was reversed by ACTH, was atypical for a large increase in NE and decrease in 5-HIAA during ACTH treatment. Beneficial effects of ACTH in OMS are not associated with normalization of HVA or 5-HIAA levels. The pattern of decreased HVA and unchanged DOPAC levels could reflect decreased extraneuronal uptake of catecholamines (which steroids inhibit) or decreased 0-methylation of catecholamines in nonneuronal cells.
Subject(s)
Adrenocorticotropic Hormone/administration & dosage , Myoclonus/drug therapy , Neurotransmitter Agents/cerebrospinal fluid , Ocular Motility Disorders/drug therapy , 3,4-Dihydroxyphenylacetic Acid/cerebrospinal fluid , Catecholamines/cerebrospinal fluid , Child, Preschool , Dihydroxyphenylalanine/cerebrospinal fluid , Female , Homovanillic Acid/cerebrospinal fluid , Humans , Hydroxyindoleacetic Acid/cerebrospinal fluid , Infant , Male , Myoclonus/cerebrospinal fluid , Ocular Motility Disorders/cerebrospinal fluid , Reference ValuesABSTRACT
The authors report a 7-year-old girl who developed neck pain and stiffness over a four-day period. There was no fever, trauma, systemic illness or headache. Physical examination demonstrated subtle neurologic deficits indicative of cervical cord compression. CAT scan and subsequent Magnetic Resonance Imaging (MRI) of the cervical spine demonstrated a spinal epidural hematoma, which was evacuated surgically. Post-operative angiography failed to demonstrate a vascular abnormality. The child recovered without neurologic deficit. MRI proved to be a sensitive tool in identifying the nature and extent of this lesion, and may be considered in lieu of myelography.
Subject(s)
Hematoma, Epidural, Cranial/diagnosis , Magnetic Resonance Imaging , Child , Female , Hematoma, Epidural, Cranial/physiopathology , Hematoma, Epidural, Cranial/surgery , HumansABSTRACT
Dopamine release into the extracellular space was measured with in vivo electrochemical detection in the ipsilateral and contralateral striata in Mongolian gerbils that suffered a stroke after acute unilateral carotid artery ligations. A sevenfold increase in the dopamine signal occurred within 15 minutes of carotid ligation in the ischemic side, while the unlesioned side had no significant change. Increased extracellular levels of dopamine persisted throughout the 3-hour recording period. Pretreatment with alpha-methyl-p-tyrosine 6 hours prior to recording significantly attenuated the signal increase. This study is the first direct demonstration of the marked, continuous dopamine release that occurs during acute cerebral ischemia.
Subject(s)
Cerebrovascular Disorders/metabolism , Corpus Striatum/metabolism , Dopamine/metabolism , Animals , Carotid Arteries , Cerebrovascular Disorders/pathology , Gerbillinae , Ligation , Methyltyrosines/pharmacology , alpha-MethyltyrosineABSTRACT
The cell surface antigen distribution on traumatic neuroma Schwann cells and neurofibroma Schwann-like cells was characterized using monoclonal antibodies that define melanoma-associated antigens. Immunofluorescence staining of cultured cells, immunoprecipitation of radioiodinated antigens from cells placed in short-term cultures, and immunoperoxidase staining of frozen tissue sections revealed most of the melanoma-associated antigens tested on traumatic neuroma and neurofibroma Schwann cells and on fetal and adult femoral nerve. The cross-reactivity of the antibodies with neural cells may reflect the common neural crest embryological origin of Schwann cells and melanocytes. Cell sorter analysis of neurofibroma cells using a monoclonal antibody directed against the melanoma nerve growth factor receptor resulted in cell cultures highly enriched for Schwann-like cells which may bear the genetic defect responsible for neurofibromatosis. The antigen detected by this monoclonal antibody is the neurofibroma nerve growth factor receptor and the antibody was a potent inhibitor of nerve growth factor binding to neurofibroma cells.
Subject(s)
Antigens, Neoplasm/analysis , Melanoma/immunology , Neurofibroma/immunology , Schwann Cells/immunology , Antibodies, Monoclonal/immunology , Cell Separation , Cross Reactions , Flow Cytometry , Fluorescent Antibody Technique , Glycoproteins/immunology , Humans , Immunoenzyme Techniques , Neuroma/immunology , Proteoglycans/immunology , Receptors, Cell Surface/immunology , Receptors, Nerve Growth FactorABSTRACT
We describe the use of a panel of monoclonal antibodies, directed against leukocyte surface antigens to characterize CSF mononuclear cells with regard to malignancy when cytopathology was inconclusive. Cytocentrifuged preparations from three patients in which traditional modalities had not yielded a diagnosis were studied, utilizing a panel of antibodies for B and T cell antigens. All three patients were found to have B cell lymphoma of the CNS. Rapid institution of the appropriate therapy resulted in marked improvement of CNS symptoms in each case. Our results indicate that in patients with CNS disease and CSF pleocytosis of undefined nature, this technique may provide rapid and precise diagnostic information.
Subject(s)
Central Nervous System Diseases/diagnosis , Cerebrospinal Fluid/cytology , Lymphoma/diagnosis , Nervous System Neoplasms/diagnosis , Adolescent , Aged , B-Lymphocytes , Central Nervous System Diseases/genetics , Fluorescent Antibody Technique , Humans , Lymphoma/genetics , Male , Middle Aged , Nervous System Neoplasms/genetics , PhenotypeABSTRACT
Extrapyramidal symptoms are extremely rare in multiple sclerosis. We examined a patient with a ten year history of multiple sclerosis, who developed choreoathetoid movements of both upper extremities. To our knowledge this is the second case report of this association.