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Leukodystrophy, Dysmyelinating, And Spastic Paraparesis With Or Without Dystonia

Known as: FAHN, FATTY ACID HYDROXYLASE-ASSOCIATED NEURODEGENERATION, SPG35 
National Institutes of Health

Related topics

Related topics

19 relations
Abnormality of the periventricular white matterAtaxiaAtrophy/Degeneration affecting the brainstemAutosomal recessive inheritance

Broader (1)

Spastic Paraplegia, Hereditary

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2013
Highly Cited
2013
Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia
The aim of this study was to examine the impact of dystonia aetiology and duration, contracture, and age at deep brain… 
Highly Cited
2012
Highly Cited
2012
Worsening essential tremor following deep brain stimulation: disease progression versus tolerance.
A major concern regarding ventralis intermedius nucleus deep brain stimulation for essential tremor has been the loss of surgical… 
Highly Cited
2010
Highly Cited
2010
Long-term results of thalamic deep brain stimulation for essential tremor.
OBJECT Deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus (VIM) has proven to be efficacious in the… 
Highly Cited
2010
Highly Cited
2010
Mutation of FA2H underlies a complicated form of hereditary spastic paraplegia (SPG35)
Hereditary spastic paraplegia (HSP) describes a heterogeneous group of inherited neurodegenerative disorders in which the… 
Review
2008
Review
2008
Outcome predictors of pallidal stimulation in patients with primary dystonia: the role of disease duration.
Pallidal deep brain stimulation (DBS) is currently the most effective treatment for advanced, medically refractory dystonia… 
Review
2007
Review
2007
Subthalamic nucleus deep brain stimulation for severe idiopathic dystonia: impact on severity, neuropsychological status, and quality of life.
OBJECT Medically refractory dystonia has recently been treated using deep brain stimulation (DBS) targeting the globus pallidus… 
Highly Cited
2003
Highly Cited
2003
Rating scales for dystonia: A multicenter assessment
The evaluation of dystonia requires a reliable rating scale. The widely used Fahn‐Marsden Scale (F‐M) has not been sufficiently… 
Highly Cited
2003
Highly Cited
2003
Abnormalities of spatial discrimination in focal and generalized dystonia.
Sensory processing is impaired in focal hand dystonia (FHD), with most previous studies having evaluated only the symptomatic… 
Highly Cited
1998
Highly Cited
1998
Pallidotomy for generalized dystonia
We systematically evaluated the efficacy of both unilateral and bilateral stereotactic pallidotomy in eight patients with… 
Review
1985
Review
1985
The GABA-ergic system: a locus of benzodiazepine action.
"Y-Aminobutyric acid is regarded as one of the major inhibitory amino acid transmitters in the mammalian brain. Over 30 years… 
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