Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1997-6-24
pubmed:abstractText
A fourteen-month-old girl, who had shown remittent fever frequently from the neonatal period, hypohidrosis, frequent change of face color and self-mutilation of the 1st and 2nd fingers of both hands and tongue in the first months of her life, developed an acute encephalopathy with generalized tonic convulsion outdoors on a sunny hot day. Generalized tonic convulsion subsided within two days, but doll's eye phenomenon, loss of pupillary reaction to light, palpebral myoclonus, and ballismus of arms and legs followed L-dopa showed some effect on the ballismus 1 month after the attack. During the hospital stay, biopsy of sural nerve was performed. Morphometric and ultrastructural studies of the sural nerve demonstrated decreased numbers of unmyelinated and small myelinated fibers. Skin biopsy of the leg revealed sweat glands with no nerve terminals, axons and Schwann cells around them. She was diagnosed as having hereditary sensory and autonomic neuropathy type IV based on the histological and clinical findings. After discharge, bone fracture was found three times without any evidence of trauma. Acute encephalopathy, probably produced in relation to the underlying neuropathy, was considered to be due to heat stroke.
pubmed:language
jpn
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0029-0831
pubmed:author
pubmed:issnType
Print
pubmed:volume
29
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
254-60
pubmed:dateRevised
2008-12-25
pubmed:meshHeading
pubmed:year
1997
pubmed:articleTitle
[A case of hereditary sensory and autonomic neuropathy type IV diagnosed following the development of acute encephalopathy due to heat stroke].
pubmed:affiliation
Department of Pediatrics, Kainan City Hospital, Wakayama.
pubmed:publicationType
Journal Article, English Abstract, Case Reports