Repeated greater occipital nerve injections with corticosteroids in medically intractable chronic cluster headache: a retrospective study

Introduction Current prophylactic drugs for cluster headache are associated with limited efficacy, serious side effects and poor tolerability. Greater occipital nerve injection (GON-injection) has been proven effective and safe as a single, one-time injection in episodic (ECH), and to a lesser extent, chronic cluster headache (CCH). We aim to analyse the effectiveness and safety of repeated GON-injections in medically intractable chronic cluster headache (MICCH). Methods Clinical data of all cluster headache patients who had received at least one GON-injection between 2014 and 2018 in our tertiary headache centre were retrieved from patients' medical records. Clinical history was taken as part of routine care shortly before and 6 weeks after GON-injection. Results We identified 47 MICCH patients (79 injections), and compared results with 22 non-MI CCH patients (30 injections) and 50 ECH patients (63 injections). Nineteen MICCH patients received repeated injections (32 in total, range 2-8). Rates of clinical relevant improvement to a first injection were similar in all groups (MICCH: 60%, non-MICCH 73%, ECH 76%; attack freedom: MICCH: 30%, non-MICCH 32%, ECH 43%). Furthermore, no difference in response to the first and second injection was shown between groups (all p > 0.29). Median effect duration in MICCH was 6 weeks (IQR 2.8-12 weeks). Side effects were only mild and local. Conclusion In this retrospective analysis, first and repeated GON-injections were well-tolerated and equally effective in MICCH as in non-MICCH, and ECH.Perioperative Medicine: Efficacy, Safety and Outcome (Anesthesiology/Intensive Care

Non-invasive vagus nerve stimulation for the acute treatment of episodic and chronic cluster headache: A randomized, double-blind, sham-controlled ACT2 study

Background Clinical observations and results from recent studies support the use of non-invasive vagus nerve stimulation (nVNS) for treating cluster headache (CH) attacks. This study compared nVNS with a sham device for acute treatment in patients with episodic or chronic CH (eCH, cCH). Methods After completing a 1-week run-in period, subjects were randomly assigned (1:1) to receive nVNS or sham therapy during a 2-week double-blind period. The primary efficacy endpoint was the proportion of all treated attacks that achieved pain-free status within 15 minutes after treatment initiation, without rescue treatment. Results The Full Analysis Set comprised 48 nVNS-treated (14 eCH, 34 cCH) and 44 sham-treated (13 eCH, 31 cCH) subjects. For the primary endpoint, nVNS (14%) and sham (12%) treatments were not significantly different for the total cohort. In the eCH subgroup, nVNS (48%) was superior to sham (6%;p<0.01). No significant differences between nVNS (5%) and sham (13%) were seen in the cCH subgroup. Conclusions Combing both eCH and cCH patients, nVNS was no different to sham. For the treatment of CH attacks, nVNS was superior to sham therapy in eCH but not in cCH. These results confirm and extend previous findings regarding the efficacy, safety, and tolerability of nVNS for the acute treatment of eCH

Unilateral increased visual sensitivity in cluster headache: a cross-sectional study

Background and Objectives Increased sensitivity to light and patterns is typically associated with migraine, but has also been anecdotally reported in cluster headache, leading to diagnostic confusion. We wanted to assess whether visual sensitivity is increased ictally and interictally in cluster headache.Methods We used the validated Leiden Visual Sensitivity Scale (L-VISS) questionnaire (range 0-36 points) to measure visual sensitivity in people with episodic or chronic cluster headache: (i) during attacks; (ii) in-between attacks; and in episodic cluster headache (iii) in-between bouts. The L-VISS scores were compared with the L-VISS scores obtained in a previous study in healthy controls and participants with migraine.Results Mean L-VISS scores were higher for: (i) ictal vs interictal cluster headache (episodic cluster headache: 11.9 +/- 8.0 vs. 5.2 +/- 5.5, chronic cluster headache: 13.7 +/- 8.4 vs 5.6 +/- 4.8; p < 0.001); (ii) interictal cluster headache vs controls (5.3 +/- 5.2 vs 3.6 +/- 2.8, p < 0.001); (iii) interictal chronic cluster headache vs interictal ECH in bout (5.9 +/- 0.5 vs 3.8 +/- 0.5, p = 0.009), and (iv) interictal episodic cluster headache in bout vs episodic cluster headache out-of-bout (5.2 +/- 5.5 vs. 3.7 +/- 4.3, p < 0.001). Subjective visual hypersensitivity was reported by 110/121 (91%; 9 missing) participants with cluster headache and was mostly unilateral in 70/110 (64%) and ipsilateral to the ictal pain in 69/70 (99%) participants.Conclusion Cluster headache is associated with increased ictal and interictal visual sensitivity. In contrast to migraine, this is mostly unilateral and ipsilateral on the side of the ictal pain.Paroxysmal Cerebral Disorder

A novel mitochondrial m.4414T > C MT-TM gene variant causing progressive external ophthalmoplegia and myopathy

tract We report a novel mitochondrial m.4414T>C variant in the mt-tRNAMet (MT-TM) gene in an adult patient with chronic progressive external ophthalmoplegia and myopathy whose muscle biopsy revealed focal cytochrome c oxidase (COX)-deficient and ragged red fibres. The m.4414T>C variant occurs at a strongly evolutionary conserved sequence position, disturbing a canonical base pair and disrupting the secondary and tertiary structure of the mt-tRNAMet. Definitive evidence of pathogenicity is provided by clear segregation of m.4414T>C mutant levels with COX deficiency in single muscle fibres. Interestingly, the variant is present in skeletal muscle at relatively low levels (30%) and undetectable in accessible, non-muscle tissues from the patient and her asymptomatic brother, emphasizing the continuing requirement for a diagnostic muscle biopsy as the preferred tissue for mtDNA genetic investigations of mt-tRNA variants leading to mitochondrial myopathy

Evaluation of the pharmacological treatment of migraine in children referred to the neurologist

Introduction: Migraine is a common illness in children. The burden of migraine is high; resulting in a decrease in the quality of life e.g. missing 9 schooldays a year. The guideline “headache” of the Dutch College of General Practitioners (DCGP) recommends treating migraine with rest and acetaminophen. A referral to a neurologist or paediatrician is recommended when the symptoms are worse or if prophylactic treatment is needed. An effective treatment is essential to improve the quality of life in children with migraine. The pharmacological treatment in children with migraine given by general practitioners (GPs), paediatricians and neurologists has never been evaluated in the Netherlands. Material and method: This retrospective cohort study was performed in children less than 18 years of age who were referred to an outpatient department or headache clinic of neurology at a general hospital because of migraine between January 2006 and June 2011. Patient characteristics, diagnosis and pharmacologic treatment were gathered and analysed. The data were obtained by hospital records and a questionnaire. Results: 223 patients were included in this study with a mean age of 12,8 year (range 4,3 to 17,8 years). The younger group of patients (less than 12 years of age) consisted of 41 females and 42 males and the older group (between 12 to 17 years of age) of 86 females and 54 males. Most of the patients (n=170) were referred by a GP and 46 patients by a paediatrician. Medication not listed in the DCGP guideline were used in 21,7 % of the young children and 47,9% of the older children for their migraine before consulting a specialist. After consultation with a paediatrician more patients were using triptans, antiemetics and prophylactic drugs. After consultation with a neurologist an even larger proportion of the patients were using triptans, anti-emetics and prophylactic drugs. Nausea or vomiting was present in about 72% of the children during a migraine attack. However, anti-emetic treatment was only prescribed in a minority of the patients by the GPs, paediatrician, and neurologists. Conclusion: A significant proportion of the children with migraine are using treatment not recommended in the DCGP-guideline before consulting a specialist. After consultation acute and prophylactic treatment were more often used. I would advise to include in the DCGP guideline for the treatment of headache during a migraine attack NSAIDs for both groups of children and also triptans for the children in the older group. Furthermore I would advise to include domperidone in the DCGP guideline for the treatment of nausea and vomiting during a migraine attack in both groups of children.

Cluster headache: Clinical aspects and therapy with neurostimulation

This thesis provides an insight in the clinical aspects and therapy with neurostimulation in cluster headache patients. An unique cohort of Dutch cluster headache patients (LUCA - Leiden University Cluster headache neuro-Analysis programme) has been used to analyse different clinical aspects like drug-use, chronobiology and aura symptoms in cluster headache patients. Regarding neuromodulation: a case-report about occipital nerve stimulation and pregnancy is described here and a meta-analysis of non-invasive vagal nerve stimulation as acute treatment in both episodic and chronic cluster headache. Nederlandse Hoofdpijn Vereniging (NHV)LUMC / Geneeskund

Cluster headache: Clinical aspects and therapy with neurostimulation

This thesis provides an insight in the clinical aspects and therapy with neurostimulation in cluster headache patients. An unique cohort of Dutch cluster headache patients (LUCA - Leiden University Cluster headache neuro-Analysis programme) has been used to analyse different clinical aspects like drug-use, chronobiology and aura symptoms in cluster headache patients. Regarding neuromodulation: a case-report about occipital nerve stimulation and pregnancy is described here and a meta-analysis of non-invasive vagal nerve stimulation as acute treatment in both episodic and chronic cluster headache. </p

Long Lasting Impairment of Taste and Smell as Side Effect of Lithium Carbonate in a Cluster Headache Patient

Paroxysmal Cerebral Disorder

Symptomatic Trigeminal Autonomic Cephalalgias

Paroxysmal Cerebral Disorder

Effective occipital nerve stimulation during pregnancy in a cluster headache patient

Paroxysmal Cerebral Disorder