Late-Life Obsessive-Compulsive Disorder and Huntington's Disease

L.G. is a 72-year-old man with a past medical history significant for hypothyroidism and diminished hearing subsequent to a right mastoidectomy as a boy. Personal and family history for either neurologic or psychiatric diseases was denied, and the patient had never abused alcohol or illicit drugs. About 20 months prior to his current presentation he experienced an episode of vertigo, and the clinical examination by a neurologist, MRI of the brain, carotid duplex studies, and an EEG were all normal. A few months after that episode of vertigo, the patient's wife reported a change in her husband's personality marked by extreme anxiety and agitation, which appeared to have developed after an exterminator had been called to the home to place some mousetraps. Subsequently, the patient developed obsessive thoughts about contamination and fears that his grandchildren would find the traps and be poisoned. He began washing his hands repetitively to the point where they became raw and abraded. The patient was also noted to be more forgetful, had difficulty making decisions and managing the bills, and was verbally abusive, which was a change from his usual behavior.

In the year following his initial neurologic evaluation, the patient was assessed by a second neurologist and a psychiatrist, both of whom attempted to treat the psychiatric symptoms with a variety of medications, including venlafaxine, clonazepam, paroxetine, fluvoxamine, alprazolam, and lorazepam. Because of the patient's resistance to taking medications, none of these were tried for an adequate amount of time to assess efficacy. The patient continued to worsen and developed checking rituals whereby he spent hours at night reviewing the home security system and making certain that all locks were in place prior to going to bed. About 6 months before my assessment, he underwent neuropsychological testing at another institution, and the findings were significant for memory impairment, executive dysfunction, attention deficits, and a mild anomia. With his behavioral changes and lack of insight, it was concluded that the patient was suffering from a frontotemporal dementia.

With the exception of a 1-week trial of risperidone 6 months prior to my evaluation, which the patient claimed caused him to become more agitated, he remained untreated. The neuropsychiatric evaluation in my office was notable for several features, including irritability, argumentativeness, and circumstantial speech. The patient was perseverative in his denial of any difficulties and demonstrated only mild short-term memory problems on cognitive examination. His neurologic examination was remarkable for decreased hearing to whisper on the right, shoulder shrugs (left>right), mild choreiform movements of his hands, akathisia, and a wide-based gait.

Because of the combined presence of a movement disorder, obsessive-compulsive features, and a history of dementia documented by neuropsychologic testing, the possibility of a basal ganglia lesion was entertained. An MRI was repeated with and without gadolinium and was significant only for periventricular white matter changes. Laboratory data including B₁₂, folate, rapid plasma reagent, and thyroid function tests were all within normal limits. Genetic testing was then performed to assess for HD. The result was positive, with 42 CAG trinucleotide repeats (<26 being normal) consistent with the HD allele. On further review of the family history, it was ascertained that the patient's deceased father had a wide-based gait similar to that of the patient and that his personality was also considered “odd.” Because of the patient's lack of insight, he refused to believe the diagnosis and would not accept medication to treat his symptoms, and he was lost to follow-up.

This case serves to remind the clinician that despite a lack of family history and radiologic findings (no evidence of caudate atrophy) and the first manifestation of symptoms at an advanced age, which epidemiologically is not typical for either HD or OCD,^5,6 HD should be considered in a patient with this constellation of symptoms. Furthermore, although psychiatric symptoms such as depression can often herald the presentation of HD, OCD symptoms are not very common in this disorder. Therefore, for a patient in this age group with late-onset HD to have manifested OCD symptoms at the initial presentation of this disorder is particularly striking, and to my knowledge not previously reported.