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H. Neil Simms and Christopher R. Honey


Autonomic symptoms can occur in association with the facial pain of trigeminal neuralgia (TN). The distinction between first division (V1) TN and trigeminal autonomic cephalgias, particularly short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), can be difficult. The goal in this study was to investigate the frequency of autonomic symptoms with TN and to determine their effect on surgical outcome.


The authors sent questionnaires and reviewed the records of 92 patients who underwent surgical procedures for TN to obtain visual analog scale scores for pain before and after surgery and to determine the location of the pain and the presence of autonomic symptoms.


Sixty-seven percent of patients had at least 1 autonomic symptom, and 14% had 4 or more autonomic symptoms associated with their pain. With V1 pain, the most common autonomic symptoms were conjunctival injection, ptosis, and excessive tearing. With pain involving the second division (V2), facial swelling was the most common autonomic symptom. Excessive salivation occurred most often when the pain involved the third division (V3). In patients who underwent microvascular decompression (MVD), visual analog scores for pain showed significantly greater improvement postoperatively in those who had no preoperative autonomic symptoms than in those who reported autonomic symptoms. There was also a significantly greater number of patients who were pain free postoperatively in the group without autonomic symptoms. There were 3 patients with V1 facial pain associated with conjunctival injection and tearing who, in retrospect, fulfilled all the current diagnostic criteria for SUNCT. These patients underwent MVD, during which a vessel was found to compress the trigeminal nerve. Postoperatively, the 3 patients experienced complete and long-lasting pain relief.


The presence of autonomic symptoms in TN correlated with a worse prognosis for pain relief after MVD. First division TN with autonomic symptoms can present identically to SUNCT but can respond to MVD if there is a compressive vessel. Neurosurgeons should be aware of SUNCT, especially in patients with V1 TN and autonomic symptoms, to ensure that all potential medical therapies have been tried prior to surgical treatment.

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Letter to the Editor

Deep brain stimulation interruption and suicidality

Andrew Howard, Christopher R. Honey, Trevor A. Hurwitz, Magdalena Ilcewicz-Klimek, Cindy Woo, Raymond W. Lam and Nicola Berman

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Andres M. Lozano, Peter Giacobbe, Clement Hamani, Sakina J. Rizvi, Sidney H. Kennedy, Theodore T. Kolivakis, Guy Debonnel, Abbas F. Sadikot, Raymond W. Lam, Andrew K. Howard, Magda Ilcewicz-Klimek, Christopher R. Honey and Helen S. Mayberg


Deep brain stimulation (DBS) has been recently investigated as a treatment for major depression. One of the proposed targets for this application is the subcallosal cingulate gyrus (SCG). To date, promising results after SCG DBS have been reported by a single center. In the present study the authors investigated whether these findings may be replicated at different institutions. They conducted a 3-center prospective open-label trial of SCG DBS for 12 months in patients with treatment-resistant depression.


Twenty-one patients underwent implantation of bilateral SCG electrodes. The authors examined the reduction in Hamilton Rating Scale for Depression (HRSD-17) score from baseline (RESP50).


Patients treated with SCG DBS had an RESP50 of 57% at 1 month, 48% at 6 months, and 29% at 12 months. The response rate after 12 months of DBS, however, increased to 62% when defined as a reduction in the baseline HRSD-17 of 40% or more. Reductions in depressive symptomatology were associated with amelioration in disease severity in patients who responded to surgery.


Overall, findings from this study corroborate the results of previous reports showing that outcome of SCG DBS may be replicated across centers.

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Toma Spiriev, Nora Sandu, Slavomir Kondoff, Christo Tzekov and Bernhard Schaller

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Anujan Poologaindran, Zurab Ivanishvili, Murray D. Morrison, Linda A. Rammage, Mini K. Sandhu, Nancy E. Polyhronopoulos and Christopher R. Honey

Spasmodic dysphonia (SD) is a neurological disorder of the voice where a patient's ability to speak is compromised due to involuntary contractions of the intrinsic laryngeal muscles. Since the 1980s, SD has been treated with botulinum toxin A (BTX) injections into the throat. This therapy is limited by the delayed-onset of benefits, wearing-off effects, and repeated injections required every 3 months. In a patient with essential tremor (ET) and coincident SD, the authors set out to quantify the effects of thalamic deep brain stimulation (DBS) on vocal function while investigating the underlying motor thalamic circuitry.

A 79-year-old right-handed woman with ET and coincident adductor SD was referred to our neurosurgical team. While primarily treating her limb tremor, the authors studied the effects of unilateral, thalamic DBS on vocal function using the Unified Spasmodic Dysphonia Rating Scale (USDRS) and voice-related quality of life (VRQOL). Since dystonia is increasingly being considered a multinodal network disorder, an anterior trajectory into the left thalamus was deliberately chosen such that the proximal contacts of the electrode were in the ventral oralis anterior (Voa) nucleus (pallidal outflow) and the distal contacts were in the ventral intermediate (Vim) nucleus (cerebellar outflow). In addition to assessing on/off unilateral thalamic Vim stimulation on voice, the authors experimentally assessed low-voltage unilateral Vim, Voa, or multitarget stimulation in a prospective, randomized, doubled-blinded manner. The evaluators were experienced at rating SD and were familiar with the vocal tremor of ET. A Wilcoxon signed-rank test was used to study the pre- and posttreatment effect of DBS on voice.

Unilateral left thalamic Vim stimulation (DBS on) significantly improved SD vocal dysfunction compared with no stimulation (DBS off), as measured by the USDRS (p < 0.01) and VRQOL (p < 0.01). In the experimental interrogation, both low-voltage Vim (p < 0.01) and multitarget Vim + Voa (p < 0.01) stimulation were significantly superior to low-voltage Voa stimulation.

For the first time, the effects of high-frequency stimulation of different neural circuits in SD have been quantified. Unexpectedly, focused Voa (pallidal outflow) stimulation was inferior to Vim (cerebellar outflow) stimulation despite the classification of SD as a dystonia. While only a single case, scattered reports exist on the positive effects of thalamic DBS on dysphonia. A Phase 1 pilot trial (DEBUSSY; clinical trial no. NCT02558634, is underway at the authors' center to evaluate the safety and preliminary efficacy of DBS in SD. The authors hope that this current report stimulates neurosurgeons to investigate this new indication for DBS.

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Christopher R. Honey, Peter Gooderham, Murray Morrison and Zurab Ivanishvili

The authors describe a novel cranial neuropathy manifesting with life-threatening episodic hemilaryngopharyngeal spasm (HELPS). A 50-year-old woman presented with a 4-year history of intermittent throat contractions, escalating to life-threatening respiratory distress. Botulinum toxin injections into her right vocal cord reduced the severity of her spasms, but the episodes continued to occur. MRI demonstrated a possible neurovascular conflict involving the cranial nerve IX–X complex and the posterior inferior cerebellar artery. Microvascular decompression of the upper rootlets of the vagal nerve eliminated her HELPS without complication. The authors propose a mechanism of HELPS implicating isolated involvement of the upper motor rootlets of the vagus nerve.

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Anthony M. Kaufmann

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Christopher R. Honey, Murray D. Morrison, Manraj K. S. Heran and Baljinder S. Dhaliwal

Inducible laryngeal obstruction has been described under at least 40 different monikers, including vocal cord dysfunction, paroxysmal vocal fold motion, and irritable larynx. The etiology of this condition is believed to be laryngeal hyperactivity in response to psychological issues or acid reflux. Most patients are treated with some combination of proton pump inhibitors, speech therapy, and psychotherapy. However, a small cohort of patients remains refractory to all medical interventions. The authors describe a novel condition, hemi-laryngopharyngeal spasm (HELPS), which can cause severe episodic stridor leading to unconsciousness in association with cough. The first recognized and surgically cured patient with HELPS was reported in an earlier issue of this journal. Three additional patients have been followed up for at least a year postoperatively, and their cases are reported here.

Each patient presented with a similar pattern of episodic coughing and choking that increased in frequency, severity, and duration over years. The episodes eventually occurred while sleeping and could cause severe stridor with loss of consciousness. All three patients were initially misdiagnosed with a psychiatric illness and subjected to multiple intubations and one tracheostomy. Unilateral botulinum toxin injections in the vocal fold eased the severity of the throat contractions but not the cough. Magnetic resonance imaging showed a looping posterior inferior cerebellar artery juxtaposed to a vagus nerve in each case. Microvascular decompression (MVD) of that vessel relieved all symptoms.

The introduction of this new medical condition may help a small cohort of patients with inducible laryngeal obstructions that have not responded to the current standard treatments. Patients are asymptomatic between episodes of progressively severe coughing and choking with stridor that may lead to intubation. Severe anxiety about the unpredictable symptoms is expected and may contribute to a psychiatric misdiagnosis. Microvascular decompression for HELPS is more difficult than that for trigeminal neuralgia because the involved nerve is more susceptible to manipulation. Ultimately, the final proof that HELPS is a real and distinct syndrome will require its recognition and successful treatment by colleagues around the world.

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C. Michael Honey, Zurab Ivanishvili, Christopher R. Honey and Manraj K. S. Heran


The location of the human spinothalamic tract (STT) in the anterolateral spinal cord has been known for more than a century. The exact nature of the neuronal fiber lamination within the STT, however, remains controversial. After correlating in vivo macrostimulation-induced pain/temperature sensation during percutaneous cervical cordotomy with simultaneous CT imaging of the electrode tip location, the authors present a modern description of the somatotopy of the human cervical STT.


Twenty patients underwent CT-guided percutaneous cervical cordotomy to alleviate contralateral medication-refractory cancer pain. Patient responses to electrical stimulation (0.01–0.1 V, 50 Hz, 1 msec) were recorded and the electrode location for each response was documented with a contemporaneous CT scan. In a post hoc analysis of the data, the location for each patient’s response(s) was measured and drawn on a diagram of their cord. Positive responses were represented only when the lowest possible voltage (≤ 0.02 V) elicited a response. Negative responses were recorded if there was no clinical response at 0.1 V.


Clinically, patients did well with an average reduction in opiates of 75% at 1 week, and 67% were able to leave the palliative care unit. The size of the cervical cord varied between patients, with an average lateral extent (width) of 11 mm and a height of 9 mm. Responses from the lower limb were represented superficially (lateral) and posteriorly within the anterolateral cord. The area with responses from the upper limb was larger and surrounded those with responses from the lower limb primarily anteriorly and medially, but also posteriorly.


In this study, the somatotopic organization of the human STT was elucidated for the first time using in vivo macrostimulation and contemporaneous CT imaging during cordotomy. In this cohort of patients, the STT from the lower-limb region was located superficially and posteriorly in the anterolateral quadrant of the cervical cord, with the STT from the upper-limb region surrounding it primarily anteriorly and medially (deep) but also posteriorly. The authors discuss how the previous methods of cordotomy may have biased the earlier versions of STT lamination. They suggest that an ideal spinal cord entry site for cordotomy of either the upper- or lower-limb pain fibers is halfway between the equator and anterior pole of the cord.