Management of Huntington's Disease Symptoms

Mood Disturbance Management

The American Academy of Neurology recommends aggressive treatment of psychiatric manifestations in Huntington's disease, as they significantly impact quality of life and are major drivers of emergency department visits, hospital admissions, and transfer to residential care 1
Selective serotonin reuptake inhibitors (SSRIs) or low-dose amitriptyline should be considered for mood symptoms, as these have demonstrated benefit in functional neuropsychiatric disorders, according to the International Neuropsychiatric Association 2
Cognitive Behavioral Therapy (CBT) strategies may be useful for addressing maladaptive beliefs, self-reported sensations, and avoidance behaviors that commonly accompany mood disturbances in Huntington's disease, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry 2
Psychiatric consultation is warranted given that neuropsychiatric dysfunction increases morbidity, care burden, and resource utilization in dementia syndromes, as stated by the Alzheimer's Association 1

The American Academy of Neurology suggests that comprehensive neuropsychological testing should focus on specific cognitive domains vulnerable in Huntington's disease, although the exact testing strategy may vary depending on the patient's condition 3
Testing should occur at baseline and repeated at 1-year, 2-year, and 4-5 year intervals to track emerging cognitive deficits, adapting the assessment battery as higher-order cognitive skills evolve, as recommended by the Ca-A Cancer Journal for Clinicians 3
Adjustment of compensatory strategies and support services is necessary to address progressive deterioration in attention, executive function, and visuospatial abilities, according to the Journal of Neurology, Neurosurgery and Psychiatry 2

A comprehensive interdisciplinary team approach is essential for managing the devastating triad of motor dysfunction, cognitive decline, and psychiatric disturbances in Huntington's disease, as stated by the Alzheimer's Association 1
The team should include a dementia subspecialist, movement disorder specialist, neuropsychologist, psychiatrist, speech and occupational therapists, as recommended by the International Parkinson and Movement Disorder Society 4
Psychiatric symptoms should not be neglected, as they are major drivers of morbidity and institutionalization, often more so than motor symptoms, according to the Alzheimer's Association 1

The American College of Radiology recommends genetic testing for CAG repeat expansion in the huntingtin gene on chromosome 4p16.3 as the definitive diagnostic test for Huntington's disease, with ≥40 CAG repeats confirming the diagnosis and providing 100% specificity 5, 6
Genetic counseling is mandatory before testing, particularly given the autosomal dominant inheritance with complete penetrance for alleles ≥40 repeats 5, 6
The American College of Radiology suggests that MRI brain without IV contrast is the optimal initial imaging modality, though it may be normal early in disease course 7, 8, 9
MRI findings in established HD include progressive marked atrophy of the neostriatum, particularly the caudate nucleus head with enlargement of frontal horns of lateral ventricles, and abnormal T2 signal in the caudate and putamen 8, 9

The American College of Radiology states that cerebrovascular disease, infectious etiologies, autoimmune conditions, metabolic disorders, and drug-induced syndromes should be considered in the differential diagnosis of chorea 5, 6, 10
The strength of evidence for tetrabenazine in treating chorea in HD is high, with a statistically significant reduction in Total Chorea Score compared to placebo, although the exact reduction is not specified in the provided text 10
Speech therapy is beneficial for orofacial chorea affecting speech and swallowing, with moderate strength of evidence 10, 11
Occupational therapy assists with activities of daily living impacted by choreiform movements, with moderate strength of evidence 10, 11
The average age of onset for HD is 35-45 years, with disease progression leading to death within 15-20 years of onset 5
Juvenile HD (onset <20 years) occurs in ~5% of cases, presents with rigidity, spasticity, and intellectual decline rather than chorea, and often has CAG repeats exceeding 55 6