HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

Correspondence to:

BRIEF COMMUNICATIONS: G. Yoon, J. Kramer, A. Zanko, M. Guzijan, S. Lin, A. Foster-Barber, and A. L. Boxer Speech and language delay are early manifestations of juvenile-onset Huntington disease Neurology 2006; 67: 1265-1267 [Abstract] [Full text] [PDF]

Correspondence: Submit a response to this article

Correspondence published:

Speech and language delay are early manifestations of juvenile-onset Huntington disease

Peter B. Rosenberger, MD   (12 December 2006)

Reply from the Authors

Adam L. Boxer, Grace Yoon   (12 December 2006)

Speech and language delay are early manifestations of juvenile-onset Huntington disease 12 December 2006
Peter B. Rosenberger, MD 5 Whittier Place, #102A, Boston, MA 02114 Send Correspondence to journal: Re: Speech and language delay are early manifestations of juvenile-onset Huntington disease prosenberger{at}massmed.org Peter B. Rosenberger, MD	Yoon et al [1] make a worthwhile effort to specify the functional pathogenicity of a well-described disease. [1] As it stands, the report creates the impression of a specific vulnerability of speech and language to the Huntington Disease process; a conclusion not supported by the supplied data. Evidence of lesser involvement of certain non-verbal congitive processes is necessary. Of the few such tests reported (design copying, matrix reasoning) none of the scores approaches normal, nor is any history of normal non-verbal development given for any of the three cases. Considering the format of the report, it may be a work in progress. The authors should be commended and encouraged to further determine specific cognitive pathology. Reference 1. Yoon G, Kramer J, Zanko A, et al. Speech and language delay are early manifestations of juvenile-onset Huntington disease. Neurology 2006; 67: 1265-1267 Disclosure: The author reports no conflicts of interest.
Reply from the Authors 12 December 2006
Adam L. Boxer, UCSF 350 Parnassus Avenue, Suite 706, Box 1207, San Francisco, CA, 94143-1207, Grace Yoon Send Correspondence to journal: Re: Reply from the Authors aboxer{at}memory.ucsf.edu Adam L. Boxer, et al.	We thank Dr. Rosenberger for his thoughtful comments. While we speculated that the speech and language delay manifested in patients with juvenile Huntington disease may be due to the disease process itself, it was not our intent to imply a specific vulnerability of language function compared to other aspects of cognition. We agree that more detailed cognitive testing in a larger study population would be required to demonstrate such a specific vulnerability. The purpose of the manuscript was to raise awareness of speech and language delay as signs/symptoms that predate motor involvement, which has traditionally been used to define onset of disease in these patients. This information may be used, in addition to family history and with appropriate genetic counseling, to consider the possibility of a diagnosis of JHD in a child at risk. Disclosure: The authors report no conflicts of interest.