Deciphering the Neuropsychiatric Landscape of Huntington’s Disease: Insights and Implications—A Case Report

Huntington’s disease (HD) is an autosomal dominant neurodegenerative illness with a specific phenomenology that includes chorea, incoordination, dystonia, behavioral difficulties, and cognitive decline. The most frequent cause of inherited chorea manifesting in adults is HD, which affects approximately 5 per 100,000 to 12 per 100,000 people. This case report describes the case of a 42-year-old woman from Assam who had been experiencing progressive and gradual onset choreiform movements of both upper limbs, affecting the fingers and face for the last two years, along with an unsteady gait and slurred speech and lack of personal care and hygiene. She also developed psychotic manifestations in the form of erotomanic delusions along with decreased sleep, restlessness, and disorganized behavior. A positive family history of dementia in their maternal grandmother and movement disorder in their paternal grandmother, along with the history of the death of her elder brother at birth, was present. The patient was admitted considering HD as a provisional diagnosis, and treatment with tablets tetrabenazine, olanzapine, risperidone, and lorazepam for her behavioral and movement symptoms was initiated. Her magnetic resonance imaging (MRI) brain report showed caudate atrophy. HD repeat expansion analysis that revealed full penetrance expansion of approximately 46 coronary angiogram (CAG) repeats in either allele was confirmatory of HD. There was an improvement in her symptoms seen in two follow-up visits to the outpatient department (OPD) after discharge except for reduced sleep; hence, melatonin 10 mg was added for it. Middle-aged patients presenting to psychiatrists with choreiform movement and psychiatric symptoms along with a positive family history should raise the suspicion of HD.

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Journal
Academic Bulletin of Mental Health
Published
2024-10-14
DOI
https://doi.org/10.25259/abmh_17_2024
Primary Topic
Genetic Neurodegenerative Diseases
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article
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article

Deciphering the Neuropsychiatric Landscape of Huntington’s Disease: Insights and Implications—A Case Report

Purna Kashyap, Deepanjali Medhi
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Academic Bulletin of Mental Health
Genetic Neurodegenerative Diseases
article

Deciphering the Neuropsychiatric Landscape of Huntington’s Disease: Insights and Implications—A Case Report

Purna Kashyap, Deepanjali Medhi
article en

Abstract

Huntington’s disease (HD) is an autosomal dominant neurodegenerative illness with a specific phenomenology that includes chorea, incoordination, dystonia, behavioral difficulties, and cognitive decline. The most frequent cause of inherited chorea manifesting in adults is HD, which affects approximately 5 per 100,000 to 12 per 100,000 people. This case report describes the case of a 42-year-old woman from Assam who had been experiencing progressive and gradual onset choreiform movements of both upper limbs, affecting the fingers and face for the last two years, along with an unsteady gait and slurred speech and lack of personal care and hygiene. She also developed psychotic manifestations in the form of erotomanic delusions along with decreased sleep, restlessness, and disorganized behavior. A positive family history of dementia in their maternal grandmother and movement disorder in their paternal grandmother, along with the history of the death of her elder brother at birth, was present. The patient was admitted considering HD as a provisional diagnosis, and treatment with tablets tetrabenazine, olanzapine, risperidone, and lorazepam for her behavioral and movement symptoms was initiated. Her magnetic resonance imaging (MRI) brain report showed caudate atrophy. HD repeat expansion analysis that revealed full penetrance expansion of approximately 46 coronary angiogram (CAG) repeats in either allele was confirmatory of HD. There was an improvement in her symptoms seen in two follow-up visits to the outpatient department (OPD) after discharge except for reduced sleep; hence, melatonin 10 mg was added for it. Middle-aged patients presenting to psychiatrists with choreiform movement and psychiatric symptoms along with a positive family history should raise the suspicion of HD.

Academic Bulletin of Mental HealthVol. 3
Gauhati Medical College and Hospital (IN)
Good health and well-being
Openalex Percentile: Top 81%
Genetic Neurodegenerative Diseases
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Deciphering the Neuropsychiatric Landscape of Huntington’s Disease: Insights and Implications—A Case Report — Purna Kashyap, Deepanjali Medhi · Academic Bulletin of Mental Health (2024) | TGRS Research Map | TGRS