Neurocognitive Disorders, Delirium and Neuropsychiatry
Background: Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease that is characterized by motor disturbances, cognitive impairment, and psychiatric symptoms. Psychotic symptoms occur in 3%-11% of patients with HD (Van Duijn et al, 2014). Neuropsychiatric symptoms can be challenging to treat, are distressing to patients, are burdensome to caregivers and families, and may be predictive of residential care needs.
Case: We present a case of a 57-year-old male with a diagnosis of Huntington's disease, who presented to the medical hospital with auditory and visual hallucinations, paranoid delusions, impulsivity, depressive symptoms, and suicidal ideation. Patient was managed by the Consultation Liaison Psychiatry Service, where multiple medication trials (Quetiapine, Olanzapine, Valproic acid, Escitalopram, Trazodone, Clonazepam) were attempted without benefit. He worsened and began refusing food and fluid intake with accompanying catatonic symptoms (Bush Francis Catatonia Rating Scale Score=6; stupor- 1, mutism-2, staring-1, withdrawal-2). Electroconvulsive therapy (ECT) was provided in 14 acute phase treatments resulting in significant improvement in psychosis, catatonia (Bush Francis Catatonia Rating Scale Score=0), depression, and overall functioning. The patient was discharged to a long-term care facility.
He presented again to the medical hospital after a year, with sepsis and during the prolonged admission, began exhibiting worsening irritability, auditory hallucinations and catatonic symptoms (Bush Francis Catatonia Rating Scale Score=10; stupor- 1, mutism-2, posturing-3, staring-1, withdrawal-3). ECT was again provided in 22 acute phase treatments with improvement in catatonia (Bush Francis Catatonia Rating Scale Score=0), psychosis and mood.
Discussion: Psychotic symptoms in HD are thought to be associated with greater cognitive decline, functional deficits, increased incidence of behavioral disturbances, and increased risk for suicide (Connors et al, 2020). This case supports that additional consideration should be given to the use of ECT as an adjunct in treatment resistant cases as well as in cases with wider psychiatric manifestations such as mood and psychotic symptoms. ECT may control these symptoms more effectively and reduce overall polypharmacy (Mowafi et al, 2021), as was evident in this case.
Conclusion: There is limited evidence with regards to treatment of psychotic and depressive symptoms in HD. Antipsychotics and antidepressants can be useful but other than what is reported in a few case series or case reports, there are no randomized controlled trials to guide treatment choices. ECT can simplify polypharmacy and allow better symptom control in patients with debilitating psychiatric manifestations of HD.
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2. Connors MH, Teixeira-Pinto A, Loy CT. Psychosis and longitudinal outcomes in Huntington disease: the COHORT Study. J Neurol Neurosurg Psychiatry. 2020 Jan;91(1):15-20. doi: 10.1136/jnnp-2019-320646. Epub 2019 Oct 13. PMID: 31611263
3. Mowafi W, Millard J. Electroconvulsive therapy for severe depression, psychosis and chorea in a patient with Huntington's disease: case report and review of the literature. BJPsych Bull. 2021 Apr;45(2):97-104. doi: 10.1192/bjb.2020.51. PMID: 32513333; PMCID: PMC8111993.
Jisha Lovin Kuriakose, MD– Consultation Liaison Psychiatrist, Long Island Jewish Medical Center- Northwell Heath, New Hyde Park, NY, United States
Joanna Drucker, PMHNP-BC– Consult Liaison Nurse Practitioner, Northwell Health, New Hyde Park, NY, United States
Humaira Shoaib, MD– Consultation-Liaison Psychiatrist, Long Island Jewish Medical Center-Northwell Health, New Hyde Park, NY, United States
Shamik Mukherji, MD– Consultation - Liaison Psychiatrist, Long Island Jewish Medical Center/Northwell Health, Garden City, NY, United States
Georgios Petrides, MD– Director, Division of ECT, Zucker Hillside Hospital, Northwell Health, New Hyde Park, NY, United States
Sohag Sanghani, MD, MPH, FAPA– Director, ECT service, Zucker Hillside Hospital, Glen Oaks, NY, United States