We enrolled six patients suffering from refractory chronic cluster headache in a pilot trial of neurostimulation of the ipsilateral ventroposterior hypothalamus using the stereotactic coordinates published previously. After the varying durations needed to determine optimal stimulation parameters and a mean follow-up of 14.5 months, the clinical outcome is excellent in three patients (two are pain-free; one has fewer than three attacks per month), but unsatisfactory in one patient, who only has had transient remissions. Mean voltage is 3.28 V, diplopia being the major factor limiting its increase. When the stimulator was switched off in one pain-free patient, attacks resumed after 3 months until it was turned on again. In one patient the implantation procedure had to be interrupted because of a panic attack with autonomic disturbances. Another patient died from an intracerebral haemorrhage that developed along the lead tract several hours after surgery; there were no other vascular changes on post-mortem examination. After 1 month, the hypothalamic stimulation induced resistance against the attack-triggering agent nitroglycerin and tended to increase pain thresholds at extracephalic, but not at cephalic, sites. It had no detectable effect on neurohypophyseal hormones or melatonin excretion. We conclude that hypothalamic stimulation has remarkable efficacy in most, but not all, patients with treatment-resistant chronic cluster headache. Its efficacy is not due to a simple analgesic effect or to hormonal changes. Intracerebral haemorrhage cannot be neglected in the risk evaluation of the procedure. Whether it might be more prevalent than in deep-brain stimulation for movement disorders remains to be determined.

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The authors report the case of a 21-year-old woman who presented with headaches, frequent sensations of loss of equilibrium, and intermittent strabismus. A tectal arteriovenous malformation (AVM) was diagnosed based on magnetic resonance (MR) imaging findings. The AVM drained toward the straight sinus and was associated with a tonsillar prolapse (Chiari malformation Type I [CM-I]) and cervical syringomyelia. The tectal AVM was embolized with N-butyl cyanoacrylate, and disconnection of about 80% of the lesion was obtained. All clinical symptoms resolved after embolization, and radiosurgery was proposed to treat the malformation remnant. A control MR image confirmed the regression of the tonsillar prolapse and the disappearance of the syrinx. This report emphasizes that CM-I and syringomyelia may be acquired and related to hydrovenous disorders.

Purpose Electrical stimulation of the sub-thalamic nucleus (STN-DBS) is well established to alleviate motor fluctuations in advanced Parkinson’s disease but little is known about its very long-term efficacy. Methods We followed over 12 years 15 parkinsonian patients having undergone STN-DBS and compared them to a matched group of 14 patients with best medical drug therapy. All had been considered as good candidates for surgery. They were allocated to each group depending on their own decision. Results After 12 years, mortality rates were similar in both groups. In the DBS group, best “on” UPDRS III scores (on medications, on stimulation) remained significantly better and dyskinesia shorter and weaker than in the drug-treated group (on medication only). Yet, looking at independent life and quality of life (QoL) evaluated with PDQ39, no significant difference could be observed between groups at the end of follow-up, probably due to development of dopa- and stimulation-resistant motor and non-motor symptoms like falls, freezing, dementia, apathy and depression, the latter two more frequent in the DBS group. Conclusion Drug- and DBS-resistant symptoms and signs occur more often after long disease evolution and in elder patients. It might be why differences in QoL between both groups no longer existed after twelve years as, compared to other studies, our patients were older at inclusion.