| Unexpected recovery in a newborn with severe hypomyelinating neuropathy. | |
MedLine Citation:
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PMID: 9165518 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We report the case of a severely hypotonic and weak term newborn who required ventilatory support from the time of birth. Serial neurophysiologic studies were consistent with severe demyelinating polyneuropathy. The infant's condition deteriorated over several weeks despite treatment with corticosteroids and intravenous immunoglobulin (IVIG) for presumed inflammatory demyelinating polyneuropathy. Histopathologic findings in a sural nerve biopsy, however, were similar to those previously reported in congenital hypomyelinating neuropathies. After 12 weeks of hospitalization and after discontinuation of corticosteroids, the patient began to recover and required no further ventilatory support. Remarkable improvement has continued for 18 months. This patient raises questions about the underlying mechanisms of hypomyelinating neuropathies in early infancy. |
Authors:
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B K Levy; G A Fenton; S Loaiza; G R Hayat |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pediatric neurology Volume: 16 ISSN: 0887-8994 ISO Abbreviation: Pediatr. Neurol. Publication Date: 1997 Apr |
Date Detail:
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Created Date: 1997-07-17 Completed Date: 1997-07-17 Revised Date: 2006-05-23 |
Medline Journal Info:
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Nlm Unique ID: 8508183 Medline TA: Pediatr Neurol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 245-8 Citation Subset: IM |
Affiliation:
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Department of Neurology, St. Louis University School of Medicine, Missouri, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenal Cortex Hormones
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therapeutic use Biopsy Demyelinating Diseases / pathology, physiopathology*, therapy Female Humans Immunoglobulins, Intravenous / therapeutic use Infant, Newborn Infant, Newborn, Diseases / pathology, physiopathology*, therapy Peripheral Nervous System Diseases / pathology, physiopathology*, therapy Remission, Spontaneous Sural Nerve / pathology |
| Chemical | |
Reg. No./Substance:
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0/Adrenal Cortex Hormones; 0/Immunoglobulins, Intravenous |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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