Characteristics of Aggression in HHUGS (Hypothalamic Hamartoma with Uncontrolled Gelastic Seizures).
Abstract number :
2.184
Submission category :
Year :
2001
Submission ID :
2313
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
K. Liow, MD, Neurophysiology, University of Kansas Medical School-Wichita, Wichita, KS; A. Weissenberger, BA, NIMH, NIH, Bethesda, MD; M. Lynn Dell, MD, NIMH, NIH, Bethesda, MD; C. Fratelli, PhD, CC, NIH, Bethesda, MD; A. Zametkin, MD, NIMH, NIH, Bethesda
RATIONALE: The syndrome of HHUGS (Hypothalamic Hamartoma with Uncontrolled Gelastic Seizures) is characterized by gelastic (laughing) seizures, congenital malformation of hypothalamic hamartoma (HH), cognitive deterioration and associated behavioral problems. Although interictal rage has often been described in this syndrome, no systemic study has been done to characterize it.
METHODS: Twelve children with Magnetic Resonance Imaging(MRI) evidence of HH and neurophysiologic evidence of gelastic seizures were systemically interviewed using the Vitello Aggression Scale. A sibling similar in age was also interviewed using same testings regimen. Affective aggression characteristics tested using the following criteria: (1)non-profitable damaging of own property; (2)completely out of control when aggressive; (3)exposes self to physical harm when aggressive; (4)is aggressive without a purpose; (5)aggression is unplanned; (6)is agressive in fornt of other people; (7)fights with stronger children; (8)expresses remorse after aggression. Predatory aggression characteristics tested using the following: (1)can control own behavior when aggressive; (2)very careful to protect self when aggressive; (3)tries to get something from being aggressive; fights with weaker children.
RESULTS: 11 of the 12 affected children (91.6%) completed the interview. Majority of affected children presented with affective aggressive characteristics (18.2% with 7 of 8 characteristics, 27.3% with 6 of 8, another 18.2 % with 5 of 8, 9% with 4 of 8 and 27% do not present with affective aggression characteristics). Only 1 patient (9%) present with all 4 predatory aggression characteristics, 18.2% with 2 of 4. 3 patients (27.2%) did not show any affective or predatory aggression characteristics. All control subjects showed no characteristics of affective or predatory aggression.
CONCLUSIONS: Results show that the rage or aggression in HHUGS are of the affective type, indicating that it is an impulsive and sudden reponse and not premeditated or motivated by ill will toward victim as in predatory aggression. The study further supports the theory that a relationship exists between hypothalamus and aggression.
Support: NINDS-NIH
NIMH-NIH