Category: Huntington's Disease
Objective: Huntington’s disease (HD) is a rare neurodegenerative disorder inherited by an autosomal dominant fashion which is most commonly characterised by choreic movement disorders. However, cognitive decline and psychiatric manifestations including mania, obsessive compulsive disorder and various forms of psychosis are also reported. Since the preceding psychopathology in HD is still unclear, we here presented a case of HD with trichotillomania (TTM), described as a stereotypic movement of repetitive and excessive hair pulling, to keep in mind as a rare initial symptom of HD.
Background: A-48 year old woman presented to our hospital with involuntary abnormal movements at her limbs that started 2 years ago.
Method: Her neurological examination revealed choreic movements at the limbs. She had lack of empathy with mild social disinhibition and cognitive decline. Mild executive dysfunction. On physical examination she had bald patches on her scalp, and wearing a hijab to hide the patches. These stereotypic behaviours began approximately 4 years ago, and worsened with heightening anxiety symptoms and psychosocial stress. Since then she was diagnosed as TTM and treated with trifluoperazine, and fluoxetine without any significant benefit. She had hypothyroidism which was under treatment with levothyroxine 75 mcg od, per oral. Since her father had HD, her self-referred genetic testing revealed CAG repeat expansion of 43 in the huntingtin gene. Laboratory investigations were unremarkable otherwise.
Results: Risperidone 0.5 mg/day po was started for TTM and chorea, and titrated up to 3 mg/day at 3 weeks. TTM and choreic movements showed moderate improvement at the 4th week. However her cognitive decline and social disinhibition showed unremarkable and insufficient response. She is still under dose titration with close follow-ups.
Conclusion: TTM is an obsessive-compulsive disorder characterized by uncontrollable, self-inflicted hair pulling resulting in noticeable hair loss. Since it is a very rare psychiatric manifestation of HD, we presented this case to keep TTM in mind as a possible preceding symptom of HD, and to emphasize the potential positive effect of risperidone in the treatment of TTM and choreic movements in HD.
References: 1. Oravecz R, Štuhec M. Trichotillomania successfully treated with risperidone and naltrexone: a geriatric case report. J Am Med Dir Assoc . 2014 Apr;15(4):301-2. doi: 10.1016/j.jamda.2014.01.007.
2. Howard AK, Krishnamoorthy A, Leavitt BR, Raymond LA, Weissman CR. Treatment of Huntington Disease and Comorbid Trichotillomania With Aripiprazole. J Neuropsychiatry Clin Neurosci. 2015 Summer;27(3):e211-2. doi: 10.1176/appi.neuropsych.14090232.
To cite this abstract in AMA style:
Y. Degirmenci, N. Bicak. A case of trichotillomania as the preceding symptom of Huntington’ s diseaseand the effect of risperidone [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/a-case-of-trichotillomania-as-the-preceding-symptom-of-huntington-s-diseaseand-the-effect-of-risperidone/. Accessed November 21, 2024.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/a-case-of-trichotillomania-as-the-preceding-symptom-of-huntington-s-diseaseand-the-effect-of-risperidone/