Neurology Specific Literature Search   
 
[home][thesaurus]
    
Click Here to return To Results

 

Hallervorden-Spatz Syndrome and Brain Iron Metabolism
Arch Neurol 48:1285-1293, Swaiman,K.F., 1991
See this aricle in Pubmed

Article Abstract
Aberrant iron metabolism in the brain if typified by Hallervorden-Spatz syndrome.In this disorder,large amounts of iron are deposited in the globus pallidus and the pars reticulata of the substantia nigra.It is characterized by extrapyramidal dysfunction,as demonstrated by dystonia, rigidity,and choreoathetosis;onset during the first two decades of life; and progression of signs and symptoms.Corroborative findings include corticospinal tract involvement,ie,spasticity and extensor toe signs, progressive intellectual impairment,retinitis pigmentosa and optic atrophy (usually associated visual evoked response and electroretinogram abnormalities),seizures,familial occurrence,hypointense areas in the basal ganglia on magnetic resonance imaging scans(particularly in the substantia nigra),abnormal cytosomes in circulating lymphocytes,and seablue histiocytes in bone marrow.Iron function in normal brain metabolism is manifold,but high concentrations of iron in the basal ganglia area may signal a unique relationship.Data support the likelihood that iron plays a role in the modulation of dopamine binding to postsynaptic receptors.In addition,transferrin receptors and iron are also concentrated in oligodendrocytes in normal brain and,thus,may have a function in myelination.A role of iron also seems likely in oxidation and peroxication reactions involving membranes and DNA,a capability that becomes uncontrolled when protective biologic mechanisms become inadequate.
 
Related Tags
(click to filter results - removes previous filter)

Babinski sign
basal ganglia
basal ganglia,lesion of
choreoathetosis
consanguinity
dementia
dysarthria
dystonia
familial
gait disorder
genetic neurologic disorders
globus pallidus
globus pallidus,lesion of
Hallervorden Spatz disease
hyperreflexia
intellectual deficit
iron,brain
lenticular nucleus,lesion of,bilateral
mortality
movement disorder
movement disorder,extrapyramidal
MRI
MRI,abnormal
optic atrophy
pyramidal tract
pyramidal tract dysfunction
retinitis pigmentosa
review article
rigidity
sea-blue histiocytes
seizure
spasticity
speech disorder
speech disorder,childhood
substantia nigra
tremor
visual evoked response

Click Here to return To Results