Neurodegeneration with brain iron accumulation (NBIA) is defined as an accumulation
of iron in the basal ganglia associated with neurologic and psychiatric symptoms (
1
). Its estimated prevalence is 1:1,000,000 (
2
). Cardinal symptoms include dystonia; spasticity and/or Parkinsonian syndrome, often
associated with cognitive decline (
3
,
4
); and psychiatric disorders such as obsessive-compulsive disorder (OCD) (
3
,
5
,
6
), depression (
7
), or psychosis (
8
).To read this article in full you will need to make a payment
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References
- Genetic, clinical, and radiographic delineation of Hallervorden-Spatz syndrome.N Engl J Med. 2003; 348: 33-40
- Pathophysiology and treatment of neurodegeneration with brain iron accumulation in the pediatric population.Curr Treat Options Neurol. 2013; 15: 652-667
- The diverse phenotype and genotype of pantothenate kinase-associated neurodegeneration.Neurology. 2005; 64: 1810-1812
- Clinical heterogeneity of neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome) and pantothenate kinase-associated neurodegeneration.Mov Disord. 2004; 19: 36-42
- Complex tics, stereotypies, and compulsive behavior as clinical presentation of a juvenile progressive dystonia suggestive of Hallervorden-Spatz disease.Mov Disord. 1994; 9: 369-371
- Atypical Hallervorden-Spatz disease with preserved cognition and obtrusive obsessions and compulsions.Mov Disord. 2005; 20: 880-886
- Hallervorden-Spatz disease in psychiatric setting.J Clin Psychiatry. 1989; 50: 66-68
- Psychotic disorder in a case with Hallervorden-Spatz disease.Acta Psychiatr Scand. 2003; 108: 394-397
- Neurodegeneration with brain iron accumulation disorders overview.in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews [Internet]. University of Washington Seattle, Seattle, WA1993: 1993-2018
- The Yale-Brown Obsessive Compulsive Scale. I. Development, use, and reliability.Arch Gen Psychiatry. 1989; 46: 1006-1011
- Obsessive-compulsive disorder: advances in diagnosis and treatment.JAMA. 2017; 317: 1358-1367
- Assessment of a targeted gene panel for identification of genes associated with movement disorders.JAMA Neurol. 2018; 75: 1234-1245
- Subthalamic nucleus stimulation in severe obsessive-compulsive disorder.N Engl J Med. 2008; 359: 2121-2134
- Dystonia in neurodegeneration with brain iron accumulation: outcome of bilateral pallidal stimulation.Brain. 2010; 133: 701-712
- Three-dimensional SPACE fluid-attenuated inversion recovery at 3 T to improve subthalamic nucleus lead placement for deep brain stimulation in Parkinson’s disease: from preclinical to clinical studies.J Neurosurg. 2016; 125: 472-480
- Basal ganglia dysfunction in OCD: subthalamic neuronal activity correlates with symptoms severity and predicts high-frequency stimulation efficacy.Transl Psychiatry. 2011; 1: e5
- Network effects of deep brain stimulation.J Neurophysiol. 2015; 114: 2105-2117
Article info
Publication history
Published online: August 28, 2019
Accepted:
July 14,
2019
Received in revised form:
June 23,
2019
Identification
Copyright
© 2019 Society of Biological Psychiatry.
