Objective: To present a unique case of a person with progressive supranuclear palsy (PSP) who developed persistent singing, and review the relevant literature.

Background: Persistent groaning and other perseverative vocalizations have been previously described in neurodegenerative conditions, including PSP. However, cases of persistent singing or humming have not been reported in PSP.

Method: The chart was reviewed for this case and a literature search was conducted using PubMed Central and Google Scholar.

Results: A 68-year-old woman presented with a 4 year history of cognitive decline and frequent falls. Evaluation showed vertical gaze palsy, square wave jerks, and Parkinsonian features supportive of PSP. At a follow-up visit 14 months later, she relied on a full-time aide, was mostly wheelchair dependent, and exhibited a novel behavior of persistent singing. The behavior occurred throughout the visit and corresponded to an existing song. She followed commands, like holding up certain fingers, while humming. When asked simple questions, like to count to ten or if she knew where she was, she answered clearly and appropriately, then immediately resumed singing. She did not respond to higher order questions like what she had for breakfast.

Exploration revealed that she began singing throughout the day a few months prior. Her song choice is usually the most recent familiar song she has heard. If she hears multiple songs in a row, she will continue the song that plays last. She reproduces entire songs. Her family plays music for her at home so that she can sing songs she enjoys. At bedtime she listens to classical music. With the absence of lyrics she stops singing, which calms her enough to fall asleep. Her family does not believe that this behavior is relaxing for her and feels that she is driven to do it.

The vocalizations seen here have an underlying awareness attached and are more organized than previously reported vocalizations. One case series has been published on dementia-associated compulsive singing in four patients with Alzheimer’s or vascular dementia. Our patient’s behavior most closely resembles those in that paper, but remains unique in its exact presentation, especially in the context of PSP.

Conclusion: This case highlights a novel occurrence of persistent singing in a patient with PSP. Further research is needed to elucidate the mechanism and significance of this phenomenon.

References: Agarwal V, Rajan J, Ramli N, et al. OFF-period purposeless groaning in parkinsonism. Neurol India. 2022;70(3):1232-1234. doi:10.4103/0028-3886.349581.

De Masi R, Orlando S, Costa MC. Dementia-associated compulsive singing (DACS): presentation of unpublished clinical cases miniseries. Int J Environ Res Public Health. 2022;19(17):10844. doi:10.3390/ijerph191710844.

Jürgens U. The neural control of vocalization in mammals: a review. J Voice. 2009;23(1):1-10. doi:10.1016/j.jvoice.2007.07.005.

Mainka T, Hidding U, Buhmann C, Haggard P, Ganos C. Voluntary inhibition of involuntary groaning in progressive supranuclear palsy. Mov Disord Clin Pract. 2018;5(3):325-326. doi:10.1002/mdc3.12598.

Merchán-del Hierro X, Rojas G, Aldinio V, et al. Moaning phenomenon and rapidly progressive dementia in anti-LGI-1 associated progressive supranuclear palsy syndrome. Tremor Other Hyperkinet Mov (N Y). 2020;10:1-4. doi:10.5334/tohm.65.

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Stamelou M, Rubio-Agusti I, Quinn N, Bhatia K. Characteristic constant groaning in late stage progressive supranuclear palsy: a case report. Parkinsonism Relat Disord. 2011;17(7):575-576. doi:10.1016/j.parkreldis.2011.04.015.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-novel-case-of-persistent-singing-in-progressive-supranuclear-palsy/