Abstract
We reported four cases of Hallervorden–Spatz disease. All four siblings (three males
and one female) in the family are affected. The first symptoms of the disease were
spastic paraparesis and optic atrophy followed by trunkal dystonia and lower motor
neurone involvement. The average age of the onset was 4.25 years. The diagnosis was
made at the ages of 17, 14, 11 and 10 years. The diagnosis was confirmed clinically,
electrophysiologically and by MRI. On MRI scans all patients demonstrated hypointense
areas in globus pallidus. There is neither specific treatment nor prenatal diagnosis.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Naming of syndromes and unethical activities: the case of Hallervorden and Spatz.Lancet. 1996; 348: 1224-1225
- Hallervorden–Spatz syndrome and brain iron metabolism.Arch Neurol. 1991; 48: 1285-1293
- Homozygosity mapping of Hallervorden–Spatz syndrome to chromosome 20p12.3-p13.Nat Genet. 1996; 14: 479-481
- Stereotactic pallidotomy in a child with Hallervorden–Spatz disease. Case report.Neurosurgery. 1999; 90: 551-554
- Optic atrophy as the first symptom in Hallervorden–Spatz syndrome.Child's Nerv Syst. 1998; 14: 135-138
- Somatosensory evoked potentials in Hallervorden–Spatz-neuroaxonal-dystrophy complex with dorsal column involvement.Clin Electroencephalogr. 1990; 21: 58-66
- Infantile neuroaxonal dystrophy.Brain. 1979; 102: 4727-4748
- Neuroaxonal dystrophy with dystonia and pallidal invomvement.Neuropediatrics. 1999; 30: 3151-3154
Article info
Publication history
Accepted:
January 10,
2001
Received in revised form:
January 10,
2001
Received:
June 19,
2000
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
