Posteromedial Hypothalamic Deep Brain Stimulation for Refractory Aggressiveness in a Patient With Weaver Syndrome: Clinical, Technical Report and Operative Video

Abstract

BACKGROUND AND IMPORTANCE Deep brain stimulation of the posteromedial hypothalamus (PMH DBS) appears to be an effective treatment for drug-resistant aggressiveness. Weaver syndrome (WS) is a rare genetic disorder in which patients develop some degree of intellectual disability and rarely severe behavioral alterations that may benefit from this procedure. CLINICAL PRESENTATION We present the case of a 26-yr-old man diagnosed with WS presenting with uncontrollable self and heteroaggressiveness and disruptive behavior refractory to pharmacological treatment and under severe physical and mechanical restraining measures. The patient was successfully treated with bilateral PMH DBS resulting in affective improvement, greater tolerance for signs of affection, regularization in his sleep pattern and appetite disturbances at 12-mo follow-up. A detailed description and video of the procedure are presented, and a review of the clinical characteristics of WS and the utility and benefits of PMH DBS for refractory aggressiveness are reviewed. CONCLUSION To our knowledge, this is the first case of refractory aggressiveness described in WS as well as the first patient with WS successfully treated with PMH DBS.

Posterior Hypothalamic Region Deep Brain Stimulation for the Treatment of Aggression Disorders in Patients with Intellectual Disability: A Systematic Review

“…Therefore, 33 studies were eligible for full-text screening. After exclusion of 23 studies (4 included overlapping populations, 13 had insufficient data, 2 dealt with irrelevant topics, 4 resulted in conference abstract), a total of 10 studies met the inclusion criteria and were included in our study [12][13][14][15][16][17][18][19][20][21]. All 10 studies were retrospective case series or case reports and published between 2008 and 2022 (Table 1).…”

“…Electrodes placement was assisted by intraoperative neurophysiological microrecording in 6 studies [13][14][15][16][17][18]; five of them also performed intraoperative stimulation The modified Newcastle-Ottawa Scale was employed to assess the quality of each paper using a predefined set of 5 criteria that were tailored to the purposes of our study. The items are scored in response to each question as negative (0) or positive (1).…”

Atypical Extracellular Action Potentials from Posteromedial Hypothalamus in Anesthetized Humans

“…The notch filter was off. PMH region was identified by MER and response to electrical stimulation [15,23]. After the PMH was identified, a quadripolar DBS electrode was implanted, with a programmable stimulator placed in an abdominal or pectoral location.…”

Commentary: Posteromedial Hypothalamic Deep Brain Stimulation for Refractory Aggressiveness in a Patient With Weaver Syndrome: Clinical, Technical Report, and Operative Video

“…he authors 1 have provided a detailed description on treating drug-resistant aggressiveness in patients with Weaver syndrome. This operative case study is a significant contribution to the existing yet limited body of literature on stimulation of the posteromedial hypothalamus (PMH) as it provides a detailed contemporary description of the surgical technique along with its pearls and pitfalls.…”