Object: Erethism describes severe cases of unprovoked aggressive behavior, usually associated with some degree of mental impairment and gross brain damage. The etiology can be epileptic, postencephalitic, or posttraumatic, or the condition can be caused by brain malformations or perinatal insults. Erethism is often refractory to medication, and patients must often be interned in institutions, where they are managed with major restraining measures. The hypothalamus is a crucial group of nuclei that coordinate behavioral and autonomic responses and play a central role in the control of aggressive behavior. Deep brain stimulation (DBS) of the posteromedial hypothalamus (PMH) has been proposed as a treatment for resistant erethism, although experience with this treatment around the world is scarce. The objective of this study was to examine the long-term outcome of PMH DBS in 6 patients with severe erethism treated at the authors' institution. Methods: Medical records of 6 patients treated with PMH DBS for intractable aggressiveness were reviewed. The therapeutic effect on behavior was assessed by the Inventory for Client and Agency Planning preoperatively and at the last follow-up visit. Results: Two patients died during the follow-up period due to causes unrelated to the neurosurgical treatment. Five of 6 patients experienced a significant reduction in aggressiveness (the mean Inventory for Client and Agency Planning general aggressiveness score was −47 at baseline and −25 at the last follow-up; mean follow-up 3.5 years). Similar responses were obtained with low- and high-frequency stimulation. In 4 cases, the patients' sleep patterns became more regular, and in 1 case, binge eating and polydipsia ceased. One of the 3 patients who had epilepsy noticed a 30% reduction in seizure frequency. Another patient experienced a marked sympathetic response with high-frequency stimulation during the first stimulation trial, but this subsided when stimulation was set at low frequency. A worsening of a previous headache was noted by 1 patient. There were no other side effects. Conclusions: In this case series, 5 of 6 patients with pathological aggressiveness had a reduction of their outbursts of violence after PMH DBS, without significant adverse effects. Prospective controlled studies with a larger number of patients are needed to confirm these results.

Tremor is an important cause of disability in patients with multiple sclerosis (MS). Deep brain stimulation (DBS) in the ventral intermediate nucleus (VIM) of the thalamus is said to be beneficial for MS tremor. To assess the long-term efficacy of VIM DBS for MS disabling tumor. We treated 10 patients (4 men and 6 women) with advanced MS-related medication-resistant tremor with DBS at the VIM thalamic nucleus. DBS was unilateral in 9 patients and bilateral in 1 patient in 2 stages. Contralateral arm tremor was assessed with the Fahn-Tolosa-Marin tremor rating scale. At 1 year, 5 of 10 patients (5 of 11 hemispheres) had a reduction in tremor scores with stimulation compared with baseline; in 3 patients, the reduction was > 50%. After 36 months, 3 patients continued benefiting from stimulation, 2 having > 50% improvement. Of the 6 symptomatic sides that did not benefit at 1 year, 3 failed to have even initial benefit, and 3 had a transient improvement lasting < 1 year. One patient stopped using stimulation because of a lack of improvement at 5 months after surgery and was lost to follow-up. Approximately one-half of the patients derived some benefit from VIM DBS 1 year after surgery, but this benefit reached a > 50% reduction in only 30% of the patients. This level of improvement may be related to the variability of the demyelinating lesions and the superimposition of ataxia in the MS patients. Developing better treatments for MS tremor continues to be a challenge.

Case Report from JAMA Neurology — Bilateral Pallidal Stimulation for X-Linked Dystonia Parkinsonism