eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Hallervorden-Spatz Disease: Follow-up

Author: Philip A Hanna, MD, Associate Professor, Department of Neuroscience, Seton Hall University School of Graduate Medical Education; Residency Program Director, New Jersey Neuroscience Institute, JFK Medical Center; Neurology Director, Huntington's Disease Unit, JFK Hartwyck-Cedarbrook
Coauthor(s): Brian L Gerhardstein, MD, PhD, Staff Physician, Department of Neurology, New Jersey Neuroscience Institute, JFK Medical Center; Neeta Garg, MD, DM, Assistant Professor, Department of Neurology, State University of New York at Buffalo
Contributor Information and Disclosures

Updated: Dec 1, 2009

Follow-up

Further Inpatient Care

Admission for supportive care is occasionally necessary.

Further Outpatient Care

Referral to a neurologist, particularly a movement disorders specialist, is helpful. Rehabilitation physicians often are consulted to coordinate therapy regimens.

Inpatient & Outpatient Medications

See Medical Care and Medication.

Transfer

Transfer is seldom necessary.

Prognosis

The clinical course is variable. In most patients, the disease has a progressive course extending over several years, leading to death in early childhood. Some patients experience rapid deterioration of function secondary to dystonia, rigidity, dysphagia, and respiratory compromise and die within 1-2 years of disease onset. Other patients undergo a slower progression or even plateau for many years and may continue to function into the third decade of life.12

Patient Education

The Web site of the NBIA Disorders Association (formerly Hallervorden-Spatz Syndrome Association) is helpful.

Miscellaneous

Medicolegal Pitfalls

Referral to a neurologist, particularly a movement disorders specialist, is helpful.

 


More on Hallervorden-Spatz Disease

Overview: Hallervorden-Spatz Disease
Differential Diagnoses & Workup: Hallervorden-Spatz Disease
Treatment & Medication: Hallervorden-Spatz Disease
Follow-up: Hallervorden-Spatz Disease
Multimedia: Hallervorden-Spatz Disease
References
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References

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  30. Johnson MA, Kuo YM, Westaway SK, et al. Mitochondrial localization of human PANK2 and hypotheses of secondary iron accumulation in pantothenate kinase-associated neurodegeneration. Ann N Y Acad Sci. Mar 2004;1012:282-98. [Medline].

  31. Kotzbauer PT, Truax AC, Trojanowski JQ, Lee VM. Altered neuronal mitochondrial coenzyme a synthesis in neurodegeneration with brain iron accumulation caused by abnormal processing, stability, and catalytic activity of mutant pantothenate kinase 2. J Neurosci. Jan 19 2005;25(3):689-98. [Medline].

  32. Kuo YM, Duncan JL, Westaway SK, et al. Deficiency of pantothenate kinase 2 (Pank2) in mice leads to retinal degeneration and azoospermia. Hum Mol Genet. Jan 1 2005;14(1):49-57. [Medline].

  33. Wakabayashi K, Fukushima T, Koide R, et al. Juvenile-onset generalized neuroaxonal dystrophy (Hallervorden-Spatz disease) with diffuse neurofibrillary and lewy body pathology. Acta Neuropathol (Berl). Mar 2000;99(3):331-6. [Medline].

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Further Reading

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Keywords

HSD, neurodegeneration with brain iron accumulation type 1, NBIA-1, Hallervorden-Spatz disease, progressive extrapyramidal dysfunction, dementia, PANK2 gene, pantothenate kinase-associated neurodegeneration, PKAN

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Contributor Information and Disclosures

Author

Philip A Hanna, MD, Associate Professor, Department of Neuroscience, Seton Hall University School of Graduate Medical Education; Residency Program Director, New Jersey Neuroscience Institute, JFK Medical Center; Neurology Director, Huntington's Disease Unit, JFK Hartwyck-Cedarbrook
Philip A Hanna, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Movement Disorders Society
Disclosure: Nothing to disclose.

Coauthor(s)

Brian L Gerhardstein, MD, PhD, Staff Physician, Department of Neurology, New Jersey Neuroscience Institute, JFK Medical Center
Disclosure: Nothing to disclose.

Neeta Garg, MD, DM, Assistant Professor, Department of Neurology, State University of New York at Buffalo
Neeta Garg, MD, DM is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Medical Editor

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison
Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Nestor Galvez-Jimenez, MD, MSc, MHA, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida
Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

 
 
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