Stressor-related disorders in tuberous sclerosis.

BACKGROUND: Patients with tuberous sclerosis complex (TSC) have high rates of psychiatric comorbidity, including mood and anxiety disorders. The aim of this study is to identify patients with stressor-related disorders such as posttraumatic stress disorder (PTSD) or adjustment disorder (AD) and to describe their clinical picture in the setting of TSC. METHODS: Retrospective review of medical charts of TSC patients referred for a stressor-related disorder to a TSC psychiatric clinic. RESULTS: We identified 7 females and 2 males (3 PTSD, 6 AD), including 4 children. Two patients with severe intellectual disability presented with aggression and the remaining patients presented with avoidance. The mean duration of symptoms at the time of the study was 21 months (range: 7 to 48 months) and 7 of the 9 patients still were having trauma-related symptoms. All the patients who received an initial diagnosis of AD had their diagnosis changed to another category because their symptoms lasted >6 months. In most cases, selective serotonin reuptake inhibitors improved the symptoms. CONCLUSIONS: Stressor-related disorders in TSC frequently linger beyond 6 months and may appear with triggering events that typically are not viewed as trauma in a normal population.

Klüver-Bucy syndrome after unilateral frontotemporal resection in a child with tuberous sclerosis.

Klüver-Bucy syndrome (KBS) is a behavioral phenotype that appears most often after bilateral temporal damage. The main features of KBS are compulsion to examine objects orally, increased sexual activity, placidity, hypermetamorphosis (irresistible impulse to notice and react to everything within sight), visual agnosia, and problems with memory. It is more rarely reported in children than in adults. We present a case of KBS in a 2-year-old boy with tuberous sclerosis complex (TSC) after left frontotemporal resection for refractory epilepsy. This is the first KBS after unilateral temporal resection in a child, although it has already been reported in two adult cases. It also is the first case reported in a TSC patient.

Self-injurious behavior and tuberous sclerosis complex: frequency and possible associations in a population of 257 patients.

Self-injurious behavior (SIB) has been observed in people with tuberous sclerosis complex (TSC), although the frequency of SIB in TSC is largely unknown. SIB is associated with intellectual and developmental disabilities, but there is no single cause of SIB. We retrospectively examined the frequency of SIB in a population of 257 patients with TSC and determined possible associations with SIB. We found a 10% frequency of SIB in our TSC population. When compared with patients without psychiatric symptoms, we identified a significantly higher rate of electroencephalographic interictal spikes in the left frontal lobe and a significantly lower number of tubers in the left occipital, parietal, and posterior temporal lobes. We also found that frequency of TSC2 mutation, history of infantile spasms, history of seizures, mental retardation, and autism are significantly associated with SIB.

Psychiatric comorbid conditions in a clinic population of 241 patients with tuberous sclerosis complex.

Psychiatric symptoms were retrospectively assessed in a clinic population of 241 children and adults with tuberous sclerosis complex (TSC). Sixty-six (27%) patients had a history of mood disorder symptoms, 66 (27%) had a history of anxiety disorder symptoms, 73 (30%) had a history of attention-deficit hyperactivity disorder (ADHD) symptoms, and 68 (28%) had a history of aggressive/disruptive behavior disorder symptoms. Significant relationships were found between these symptoms and patient age, gender, genetic mutation, seizure history, surgical history, cognitive impairment, features of autism or pervasive developmental disorder, and neurological manifestations of TSC. In 43 patients seen by at least one of two affiliated psychiatrists, the most common formal diagnoses were anxiety disorders (28%), mood disorders (26%), adjustment disorders (21%), ADHD (21%), and mental disorders not otherwise specified due to general medical condition (42%). Citalopram demonstrated efficacy in treating anxiety and depression, and risperidone, in treating problematic behaviors.