@article{ref1,
title="Management of self-injurious behavior, aggression, and psychogenic non-epileptic seizures in patients with tuberous sclerosis: a case report with a review of  literature",
journal="Curēus",
year="2020",
author="Ruxmohan, Samir and Taylor, Amanda and Lalama, Hector A. and Smith, Derrick Wayne and Gomez, Jonathan and Ortiz, Juan Fernando",
volume="12",
number="11",
pages="e11715-e11715",
abstract="Tuberous sclerosis complex (TSC) is a neurocutaneous disorder that affects multiple systems. TSC encompasses neurobehavioral abnormalities that are considered less  sensitive and specific to the disease. Autism spectrum disorder, attention deficit  disorder, anxiety, mood disorders, and self-injurious behavior (SIB) are  neurobehavioral manifestations associated with tuberous sclerosis. Among them, SIB  is poorly described and studied. We present a case report and a brief review of the  literature, which offers us insight into the pathological mechanism that explains  associated SIB in TSC patients and provides a possible multidisciplinary approach to  handle this complicated association. The case details a 21-year-old female with  tuberous sclerosis who went to the emergency department and then transfer to the  psychiatric floor due to aggressive behavior and SIB. The patient had a history of  infantile spasm in childhood and generalized tonic-clonic seizures (GTCS); the last  episode was four years ago at the age of 17. During the hospital admission, the  patient developed an apparent tonic-clonic seizure. Nevertheless,  the electroencephalogram (EEG) shows no epileptiform pattern and because of the  clinical presentation, it was concluded she had psychogenic nonepileptic seizures  (PNES). The patient's CT scan showed a stable appearance of multiple calcified  subependymal nodules and left frontal hypodensity. Mini-mental examination (MMSE)  revealed mild cognitive impairment. Patients with TSC/SIB have higher frequencies of  mental retardation, TSC2 mutations, history of infantile spasms, spike focus in the  left frontal lobe. Also, TSC/SIB patients have a higher frequency of tubers in  quadrants other than the left posterior neuroanatomical region in left occipital,  parietal, and posterior temporal lobes. Our patient had four out of five of the  risks factor for developing TSC/SIB. Almost all patients with tuberous sclerosis are  expected to develop seizures. Nevertheless, our patient was seizure-free for two  years and managed prophylactically with antiepileptic medication. PNES can also  occur in patients with tuberous sclerosis. It is essential to be attentive to  differentiate PNES from actual seizures due to their history of the high frequency  of seizures in TSC. Given the multiple systems involved in the symptomatology of  TSC, including the SIB and neurological concerns, multidisciplinary treatment  strategies must be implemented. Treatment of TSC with SIB should include  antiepileptic drugs covering seizures and managing the SIB's mood component. A  neuroleptic could be added for patients who are difficult to manage.<p /> <p>Language: en</p>",
language="en",
issn="2168-8184",
doi="10.7759/cureus.11715",
url="http://dx.doi.org/10.7759/cureus.11715"
}