Prim Care Companion CNS Disord 2023;25(2):23lr03487
To cite: Lange H. Comment on Huntington’s disease with psychotic features. Prim Care Companion CNS Disord. 2023;25(2):23lr03487.
To share: https://doi.org/10.4088/PCC.23lr03487
© 2023 Physicians Postgraduate Press, Inc.
aGeorge Huntington Institut, Münster, Germany
*Corresponding author: Herwig Lange, MD, George Huntington Institut, Wilhelm-Schickard-Straße 15, 48149, Münster, Germany ([email protected]).
See letter by Gupta and Correll and case report by Yeo et al.
To the Editor: I read with interest the report by Yeo et al1 published previously in the PCC. I have listed statements from the report with comments as follows:
(1) “It [Huntington’s disease; HD] is characterized by a triad of choreiform movements, cognitive decline, and psychiatric disturbances.”
–Some HD patients never show choreiform movements. This was first suggested by Davenport in 1915.2
(2) “A healthy individual without HD has around 11–35 CAG [cytosine-adenine-guanine] repeats, whereas an HD patient has 36 or more.”
–The normal range of CAG repeats end at 26. A number of HD patients with less than 36 CAG in HTT have been reported.3 I know of 2 HD patients with 35 CAG. The probability to manifest HD increases gradually from 0 with 26 CAG to 1 with 43 CAG in the HTT gene.
(3) “With each successive generation there is an expansion of the CAG repeat number.”
–This is incorrect. Expansion of the CAG repeat usually occurs in 60% of paternal transmission. Contraction has been documented as well.4
A very common mistake in psychiatry is to not take a family history, as was the case in this patient. This critical weakness led to delayed diagnosis and unfavorable treatment. Previously in 1972, Whittier, the physician who treated Woody Guthrie, and colleagues5 wrote about the difficulties faced by psychiatrists in the diagnosis of HD—nothing much has changed.
Relevant financial relationships: None.
Funding/support: None.
Published online: March 7, 2023.
Author Affiliations
- George Huntington Institut, Münster, Germany
- Corresponding author: Herwig Lange, MD, George Huntington Institut, Wilhelm-Schickard-Straße 15, 48149, Münster, Germany ([email protected]).
References (5)
- Yeo K, Gupta M, Correll CU. Huntington’s disease with psychotic features. Prim Care Companion CNS Disord. 2021;23(4):20l02745. PubMed CrossRef
- Davenport CB. Huntington’s chorea in relation to heredity and eugenics. Proc Natl Acad Sci U S A. 1915;1(5):283–285. PubMed CrossRef
- Savitt D, Jankovic J. Clinical phenotype in carriers of intermediate alleles in the huntingtin gene. J Neurol Sci. 2019;402:57–61. PubMed CrossRef
- Zühlke C, Riess O, Bockel B, et al. Mitotic stability and meiotic variability of the (CAG)n repeat in the Huntington disease gene. Hum Mol Genet. 1993;2(12):2063–2067. PubMed CrossRef
- Whittier JR, Heimler A, Korenyi C. The psychiatrist and Huntington’s disease (chorea). Am J Psychiatry. 1972;128(12):1546–1550. PubMed CrossRef
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