HD Lighthouse Contributing Editor's Comment: More attention needs to be devoted to Juvenile Huntington's Disease -- from diagnosis to the understanding of the natural course of the disease to the treatment of symptoms to inclusion in clinical trials. While JHD advocates like our own HDL friend Gordon are working with international HD organizations like Euro-HD to improve knowledge and care, the Lighthouse will be making a special effort to find and post the information that is currently available.

The article summarized below reports on the cases of children with JHD who became symptomatic before age 10. Their CAG counts ranged between 93 and 120. In all three cases, language delay preceded speech impairment and motor dysfunction. None of the three were able to speak in two or three word phrases until after age three but weren't diagnosed until motor abnormalities became evident. The families felt that their children had benefited from diagnosis and the subsequent individualized education plans developed for them; all experienced significant improvement in speech.

The authors point out that clinical diagnosis of Huntington's Disease is generally done when motor symptoms appear. However with JHD, just as with adult HD, other symptoms may present first.

-- Marsha Miller, Ph.D.
Posted to the HDL: 30 Oct 2006

Speech and Language Delay are Early Manifestations of Juvenile-Onset Huntington Disease

G. Yoon, MD, FRCP(C), J. Kramer, PsyD, A. Zanko, MS, M. Guzijan, MS, S. Lin, MSW, A. Foster-Barber, MD, PhD and A. L. Boxer, MD, PhD

The neurocognitive features of juvenile-onset Huntington disease (HD) are not well understood. We present three patients with onset of HD symptoms before age 10 years in whom speech delay was the first symptom. Speech delay predated motor symptoms by at least 2 years, and language function was consistently impaired on formal testing. Screening for speech delay is particularly important in children with a family history of HD. # # #

Source: Neurology 2006;67:1265-1267

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