Pathological hair-pulling or trichotillomania, which is commonly associated with anxiety and depression, obsessive-compulsive disorder, and neurodevelopmental disorders, has been rarely associated with dementing illnesses. Investigators have not clarified the neural correlates and treatment of trichotillomania in dementia. We report a patient who developed an early-onset cognitive decline with genetic, cerebrospinal fluid biomarker and structural and functional neuroimaging studies consistent with Alzheimer’s disease. Eight years into her disease, she developed severe, repetitive hair-pulling behavior leading to marked hair loss, along with other repetitive and “frontal” behaviors. Selective serotonin reuptake inhibitors (SSRIs) were ineffective in controlling her hair-pulling behavior, which subsequently responded to quetiapine 150 mg/day. This patient and a review of the literature suggest that trichotillomania may be a compulsive-related symptom in dementias of different etiologies as they involve frontal areas and release primitive grooming behavior from frontostriatal dysfunction. Dopamine blockade, rather than SSRIs, may be effective in managing trichotillomania in dementia.
Trichotillomania is characterized by an overwhelming urge to pull out one’s own hair with resultant noticeable hair loss. Although trichotillomania is a distinct diagnostic entity [
Clinicians may be unware that trichotillomania can also be a neuropsychiatric symptom of dementia. Although the literature on this is sparse [
A 54-year-old, left-handed woman had a five-year history of a very insidious onset and gradually progressive decline in memory and cognition. Her husband reported early word-finding difficulty with increasing use of nonspecific words when she was not able to find the correct words. The patient also had impaired orientation, memory and new learning, auditory comprehension, and visuospatial abilities. The patient still retained the ability to participate in her activities of daily living, but she was unable to do them on her own. Her past medical history was negative for other diseases or toxic exposures, and there was no history of dementia in her family. On examination, the patient scored 9/30 on Mini-Mental State Examination (MMSE), and she had marked difficulty with language fluency, comprehension, and confrontational naming. Her declarative, episodic memory was significantly impaired. She could not copy visuospatial figures, search for dots and words, or perform executive tasks. Examination of cranial nerves, gait and coordination, motor, sensory, and reflexes did not reveal abnormalities.
The results of laboratory investigations were unremarkable except for the presence of an apolipoprotein (APOE)
(a) T1 axial magnetic resonance imaging of brain shows predominant cerebral atrophy in the parietal lobes; (b) fluorodeoxyglucose positron emission tomography axial view showing bilateral parietal hypometabolism and hypometabolism of the posterior cingulate cortex.
Eight years after the onset of her disease, she developed restless, agitated behavior, and repetitive, compulsive acts. The most prominent was severe, constant, and uncontrollable hair-pulling, which eventually resulted in her pulling out most of the hair on her scalp. Despite multiple attempts and trials of interventions and medications, her hair-pulling continued for the next two years. Her hair-pulling was constant during waking hours, unless restrained or distracted, and involved primarily her scalp, but also her eyebrows, arms, and other parts of her body. Once pulled she did not play with or eat the hair. Simultaneously, she developed rubbing and picking of her fingers to excoriation, hand-biting, and picking up lint from floor and shaping it into balls. The finger excoriation involved cutting at her thumb and other fingers with her fingernails until the affected fingertips bled. Her examination now included behaviors often found with frontal brain disease: palilalia or multiple repetitions of her own sparse utterances; logoclonia or repetition of end syllables; and upper extremity grasp reflexes often to the extent of “groping.”
Because of her dementia, she could not communicate a reason for her trichotillomania. Her husband reported that her insight regarding her illness was impaired, but she had obvious irritability and tension. The patient would become agitated when her husband attempted to stop her repetitive behaviors, but there was no evidence of relief after she successfully pulled out her hair. The trichotillomania and compulsive-like behaviors were not temporally associated with intercurrent illnesses or changes in medication. Trials of fluoxetine and sertraline (up to 150 mg/day for two or more weeks) were ineffective in decreasing her constant hair-pulling or her skin excoriation (per her husband’s records of the length of waking time she spent on hair-pulling). The patient continued to worsen her repetitive behaviors; therefore, most of her medications were stopped and she was started on quetiapine at 25 mg/day, which was gradually titrated up to 150 mg/day. On this dose, both her hair-pulling and skin-picking decreased to intermittent episodes occurring during the week, and, most significantly, she was more easily diverted from these acts when they occurred. The patient has remained in this improved state for over a year of follow-up.
This patient had profound trichotillomania in the context of an early-onset (<65 years of age) dementia with evidence for Alzheimer’s disease (AD) on cerebrospinal biomarker studies and neuroimaging. The presence of an APOE
Our understanding of the nature of trichotillomania is incomplete, and its occurrence in dementia may offer an avenue for understanding this behavior. Explanations for trichotillomania have ranged from an impulse control disorder such as kleptomania, a stereotypic self-injurious behavior as in neurodevelopmental disorders, a behavior that is part of the obsessive-compulsive disorder spectrum, or a purely body focused repetitive behavior (BFRB) along with skin-picking [
The accompanying compulsive-like behaviors along with palilalia, logoclonia, grasp reflexes, and impairment of insight suggest frontal systems involvement with the progression of the dementia. When these compulsive-like behaviors occur near onset of the dementia, it may indicate behavioral variant frontotemporal dementia (bvFTD) and, when late, an extension of a dementia such as AD to frontal regions. Nearly 80 percent of bvFTD patients with proven frontal pathology have had compulsive-like behaviors, and neuroimaging and neuropathological studies indicate that compulsive-like behaviors in bvFTD patients result from damage to the frontal lobe and to the basal ganglia, particularly the caudate nuclei [
Studies show that patients with trichotillomania have frontostriatal involvement and dysfunction. Compared to controls, patients with trichotillomania may have decreased volume of the left inferior frontal gyrus and left putamen volume [
The neurochemical etiology of trichotillomania may involve different neurotransmitters. First, an imbalance of serotonin may play a role in repetitive self-injurious behaviors in both Cornelia de Lange syndrome, Lesch-Nyhan syndrome, and OCD. Some studies have shown that selective serotonin reuptake inhibitors (SSRIs), including fluoxetine, sertraline, escitalopram, and clomipramine, have successfully reduced the severity of compulsive-like behaviors in some patients with dementia [
In conclusion, trichotillomania can be a manifestation of compulsive-like, grooming behavior in dementias of different etiologies as they spread to involve frontal areas, with release of a frontal-striatal inhibitory pathway resulting in excessive primitive grooming behavior. Several medications can successfully treat trichotillomania. In dementia, if SSRIs are not effective, antipsychotic dopamine antagonists like quetiapine may reduce the incidence of trichotillomania [
The authors declare that there is no conflict of interests.
This work was supported by the Nation Institutes of Health [NIA R01AG050967].