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Genetic and Rare Diseases Information Center (GARD)

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Localized hypertrophic neuropathy


Other Names for this Disease
  • Onion whorl disease
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Your Question

I have localized hypertrophic neuropathy.  What are my future prospects with this disease: will I lose function?  Will it paralyze me?  Can symptoms of this disease be reduced with any treatment?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is localized hypertrophic neuropathy?

Localized hypertrophic neuropathy is a nerve condition in which nerve cells increase in number and form bundles that look like onion bulbs.  This condition affects one nerve, usually in an arm or leg.  The symptoms of this condition include muscle weakness, numbness, and decreased reflexes in the affected limb.  Symptoms slowly get worse over time.[1]  The cause of this condition is unknown, but some researchers believe it might be a type of tumor, while others believe it might be an unusual reaction to injury.[2]
Last updated: 7/9/2012

What is the prognosis of localized hypertrophic neuropathy?

Because only a few people have been diagnosed with localized hypertrophic neuropathy, there is limited information on the prognosis (meaning the future prospects or outlook) for this disease.  Prognosis depends on how quickly the symptoms worsen and whether an individual is treated.  Localized hypertrophic neuropathy is a progressive condition, which means the symptoms of muscle weakness and numbness slowly get worse over time and may lead to paralysis of the affected limb in some cases. 

One article in the scientific literature described the prognosis of localized hypertrophic neuropathy for four individuals before and after treatment.   In three individuals, treatment stabilized their symptoms, which means the weakness and numbness did not continue to get worse but stayed the same (stabilized).  The fourth individual experienced improvement in strength following treatment.  Though this article only examined four individuals, the authors state that other studies have found similar results, with all individuals' symptoms stabilizing and a few improving following treatment.[1]
Last updated: 7/9/2012

What treatment is available for localized hypertrophic neuropathy?

Because this condition is rare, there are no established guidelines for the treatment of localized hypertrophic neuropathy.  Treatment is determined based on each individual's symptoms and personal medical history.  Treatment usually consists of having surgery to remove the abnormal part of the nerve.  Some individuals do not have surgery and their symptoms are monitored over time.[1]
Last updated: 7/9/2012

References
  • Simmons Z, Mahadeen ZI, Kothari MJ, Powers S, Wise S, Towfighi J. Localized hypertrophic neuropathy: magnetic resonance imaging findings and long-term follow-up. Muscle & Nerve. 1999; 22:28-36. http://www.ncbi.nlm.nih.gov/pubmed/9883854. Accessed 7/6/2012.
  • Koszyca B, Jones N, Kneebone C, Blumbergs P. Localized hypertrophic neuropathy: a case report and review of the literature. Clinical Neuropathology. 2009; 28:54-58. http://www.ncbi.nlm.nih.gov/pubmed/19216221. Accessed 7/5/2012.