Purpose of Review
Tuberous sclerosis complex (TSC) and neurofibromatosis (NF) are neurocutaneous disorders often encountered by neurologists in clinical practice. This article aims to familiarize ...adult and pediatric neurologists with common features of these disorders and headache specific evaluation and management.
Recent Findings
Non-malignant intracranial tumors in TSC include cortical tubers (glioneuronal hamartomas), subependymal nodules or subependymal giant-cell astrocytomas (SEGA). Headache disorders in TSC are largely secondary and can cause headaches due to increased intracranial pressure, mass effect, obstructive hydrocephalus, or hemorrhage. Neurosurgical intervention is typically required for management of large SEGAs; however, in patients with increased surgical risk, newer treatment modalities may be offered such as neoadjuvant therapy with an mTOR inhibitor (mTORi). Newer studies indicate headache disorders are more prevalent in neurofibromatosis type 1 (NF1). Primary headache disorders can include migraine and tension-type headache, while secondary headache disorders can be due to associated neoplasms such as optic pathway gliomas or brainstem gliomas, or less commonly vasculopathies such as moyamoya syndrome. Selumetinib is an oral, small molecule mitogen-activated protein kinase (MEK) agent with antineoplastic activity which is in ongoing trials for treatment of NF1-associated pediatric low-grade gliomas.
Summary
NF1 stands out as having a higher association with primary headache disorders such as migraine. This association may be related to effects of mutation of the neurofibromin gene on pathways involved in pain and migraine genesis, however, warrants future study. Care should be taken when formulating a headache treatment plan to address comorbidities and avoid medications that may be contraindicated.
We sought to conduct a qualitative systematic review to evaluate the safety and efficacy of available treatments for pediatric patients with migraine or benign primary headache in the emergency ...department, in an effort to inform future practice.
Scopus, Medline, and PubMed databases were searched for randomized controlled trials retrospective reviews, review articles, and case studies discussing migraine or benign primary headache management that were conducted in the emergency room or outpatient acute care setting in pediatric patients (less than 18-years old). Meeting abstracts and cited references within articles were also evaluated. Multiple variables were recorded, including type of treatment, study design, dosing, primary outcome, and side effects. Therapeutic gain was calculated in studies with a placebo arm. Treatments were subjectively assessed based on methodology and number of trials for a particular therapy.
Thirty-one studies were included in the final analysis. Of these, 17 were randomized controlled trials, 9 were retrospective reviews, and 5 were prospective chart review studies. One pertained to IV fluids, 2 to nonspecific analgesic use, 5 to dopamine receptor antagonists, 2 to valproic acid, 1 to propofol, 1 to magnesium, 1 to bupivicaine, 13 to triptan medications, and 3 to dihydroergotamine (DHE). Treatments considered effective for acute migraine or benign primary headache in the analgesic category include ibuprofen, and to a lesser degree acetaminophen. Ketorolac was not compared to other NSAIDs, but was found to be less effective than prochlorperazine. Of the phenothiazines, prochlorperazine was considered most effective. Of the triptan medications, almotriptan, rizatriptan, zolmitriptan nasal spray, sumatriptan nasal spray, and combination sumatriptan/naproxen are effective agents for acute treatment. Treatments considered probably effective included IV fluids, chlorpromazine, valproate sodium, injectable sumatriptan, and IV DHE. Treatments with oral zolmitriptan showed inconsistent results, while treatments considered ineffective included isolated oral sumatriptan and oral DHE. There is insufficient evidence to comment on propofol, magnesium, and bupivicaine efficacy.
Of the available evidence, ibuprofen, prochlorperazine, and certain triptan medications are the most effective and safe agents for acute management of migraine and other benign headache disorders in the pediatric population. Additional studies in this population are needed, and should take into consideration variables such as dosing, co-administered medications, treatment duration, and length of treatment effect.
The case of a patient with a first presentation psychotic episode secondary to variant Creutzfeldt-Jakob Disease (vCJD) is presented. While psychiatric symptoms are considered a prominent feature of ...vCJD, they may precede characteristic neurological symptoms, which can delay diagnosis. The psychotic symptoms in this case differed in quality from typical psychotic presentations, which could have helped with earlier diagnosis. The patient's symptomatology suggested that errors in cognition and perception were largely contributing to his psychiatric symptoms. These errors appeared to be the result of prion destruction of relevant brain structures that may either be directly or secondarily involved in psychiatric disorders. The findings in this case can help elucidate how vCJD symptoms deviate from established guidelines for diagnosing primary psychiatric disorders.
OBJECTIVE:To review whether the incidence of catheter-associated venous thromboses was higher in patients receiving IV dihydroergotamine compared to lidocaine.
METHODS:We retrospectively reviewed all ...admissions at the University of California, San Francisco Headache Center from February 25, 2008, through October 31, 2014, for age, sex, diagnosis, aura, treatment dose, type of IV line used, days with line, superficial (SVT) or deep venous thrombosis (DVT), and pulmonary embolism (PE).
RESULTS:A peripherally inserted central catheter (PICC) or midline catheter was placed in 315 of 589 (53%) admissions. Mean age was 38 years with a range of 6 to 79 years; 121 patients (21%) were ≤18 years old. Seventy-four percent (433 of 589) of patients were female. Of 263 dihydroergotamine admissions using a PICC or midline catheter, 19 (7.2%) had either an SVT or DVT or a PE; 2 patients were diagnosed with both DVT and PE. Of 52 lidocaine admissions using a PICC or midline catheter, none had a thrombotic event (p = 0.05, Fisher exact test). Age, sex, aura, total dihydroergotamine dose, and number of days with line were not significant predictors of venous thrombosis.
CONCLUSIONS:IV dihydroergotamine treatment may be associated with an increased risk of catheter-associated venous thrombosis. A low threshold for diagnostic ultrasound investigation is appropriate because anticoagulation therapy was frequently required.
Migraine is the leading cause of years lost due to disability in individuals aged 15 to 49 years. Much has changed over the last three decades about our understanding of this complex neurological ...disorder. Various phases of migraine have been characterized and are the focus of this review. The premonitory phase involves bothersome symptoms experienced hours to days before migraine pain. Behavioral changes and functional neuroimaging studies point toward hypothalamic involvement during the premonitory and other migraine phases. Migraine aura is a disruptive, reversible neurological phenomenon that affects up to one-third of all migraineurs, and can overlap with the headache phase. The mechanism responsible for this phase is thought to be cortical spreading depolarization through the cortex. This process leads to temporary disruptions in ion homeostasis and the ensuing neuronal dysfunction. The headache phase involves activation of the trigeminocervical complex. Neuropeptides are implicated in trigeminal activation, and calcitonin gene-related peptide in particular has become a promising target of therapeutic intervention for migraine. The final phase of migraine is the postdrome, the period of time from the resolution of headache symptoms until return to baseline following a migraine. People often report neuropsychiatric, sensory, gastrointestinal, and general symptoms during this time, which can limit activity. Elucidating the neuroanatomical, chemical, and neuroimaging correlates of these migraine phases allows for an improved comprehension of the underlying changes associated with migraine symptomatology and can assist with evaluation of arising therapeutics for migraine management.
This article summarizes an updated guideline from the American Academy of Neurology and American Headache Society on the short-term treatment of migraine in children and adolescents.
OBJECTIVEThe aim of this prospective study was to survey our patients about their experience with our clinicʼs telemedicine program to better understand telemedicineʼs utility for families, and to ...improve patient satisfaction and ultimately patient care.
METHODSThis was a prospective survey study of patients and their families who had a routine telemedicine follow-up visit with the University of California San Francisco Pediatric Headache Program. The survey was administered to patients and a parent(s) following their telemedicine visit.
RESULTSFifty-one of 69 surveys (74%) were completed. All (51/51) patients and families thought that (1) telemedicine was more convenient compared to a clinic visit, (2) telemedicine caused less disruption of their daily routine, and (3) they would choose to do telemedicine again. The mean round-trip travel time from home to clinic was 6.8 hours (SD ± 8.6 hours). All participants thought telemedicine was more cost-effective than a clinic visit. Parents estimated that participating in a telemedicine visit instead of a clinic appointment saved them on average $486.
CONCLUSIONThis prospective, pediatric headache telemedicine study shows that telemedicine is convenient, perceived to be cost-effective, and patient-centered. Providing the option of telemedicine for routine pediatric headache follow-up visits results in high patient and family satisfaction.
To assess the feasibility, tolerability, and patient acceptability of single-pulse transcranial magnetic stimulation (sTMS) for migraine prevention in adolescents in an open-label pilot study.
...Migraine is common in adolescents and can be disabling. Well tolerated preventative therapies that are safe and effective are needed.
This was an open-label prospective pilot feasibility study of sTMS for migraine prevention in adolescents aged 12-17 years. Participants used sTMS twice daily in a preventative fashion, as well as additional pulses as needed acutely. A 4-week baseline run-in period (weeks 1-4) was followed by a 12-week treatment period. Feasibility was the primary outcome. Secondary outcomes included tolerability and acceptability, as well as the change in headache days, number of moderate/severe headache days, days of acute medication use, and PedMIDAS (headache disability) scores between the run-in period (weeks 1-4) and the third month of treatment (weeks 13-16).
Twenty-one participants enrolled. Nineteen completed the baseline run-in, and 12 completed the study. Using sTMS proved feasible and acceptable with overall high compliance once treatment administration was streamlined. Initially, for preventive treatment, participants were asked to give 2 pulses, wait 15 minutes, then give 2 additional pulses twice daily. This 15-minute delay proved challenging for adolescents, particularly on school days, and therefore was dropped. Study completion rate went from 4/13 (31%) to 7/8 (88%) once this change was made, P = .024. On average, participants used the device preventively between 22 and 24 days over a 28-day block. There were no serious adverse events. Two participants reported mild discomfort with device use.
sTMS appears to be a feasible, well-tolerated, and acceptable nonpharmacologic preventive treatment for migraine in adolescents. In designing future trials of sTMS for migraine prevention in adolescents, streamlined treatment administration will be essential to minimize drop-out. Efficacy needs to be assessed in a larger trial.
PDF
