Virtual McKenzie extension exercises for low back and leg pain: a prospective pilot exploratory case series.

INTRODUCTION: Current evidence supports the inclusion of directional preference exercises for a subgroup of patients with low back (LBP) and leg pain. Recent pain neuroscience strategies have suggested that cortical restructuring associated with movement activating the body map representation in the brain might account for the observed improvement with the directional preference approach. OBJECTIVES: To explore whether or not a motor imagery directional preference approach would result in any changes in patients with LBP and leg pain. METHODS: A consecutive convenience sample of patients with LBP and leg pain were recruited at two outpatient physical therapy clinics. Measurements of LBP, leg pain, fear-avoidance beliefs (FABQ), pain catastrophizing (PCS), active lumbar flexion, and straight leg raise (SLR) were compared before and immediately after a virtual (motor imagery) directional preference exercise. RESULTS: Statistically significant differences for LBP, FABQ, PCS, active lumbar flexion, and SLR were observed, but only SLR changes met or exceeded the minimally clinically important difference (MCID). CONCLUSIONS: A brief virtual motor imagery extension treatment yielded some immediate positive shifts in patients presenting to physical therapy with LBP and leg pain. Our results indicate that randomized comparison trials are needed to determine the effect of this intervention on the short- and longer-term outcomes in patients with LBP and leg pain.

Emergency surgery in an elective setting: a case report detailing incidental diagnosis of a de Garengeot hernia.

We present a case of acute appendicitis within an incarcerated femoral hernia. This is a rare complication of the phenomenon eponymously known as a 'De Garengeot Hernia', which describes a vermiform appendix in an incarcerated femoral hernia sac. Our case is somewhat unique in the manner by which the affected patient had presented. Attending hospital for an unrelated elective surgery, an incarcerated hernia was diagnosed at time of admission. Thorough assessment in advance of the procedure and decisive action led to a satisfactory outcome. This may be the first case in literature reporting a 'De Garengeot Hernia' presenting in such a fashion.

Immediate effect of pain neuroscience education for recent onset low back pain: an exploratory single arm trial.

Study Design: A prospective, single-arm, pre-postintervention study.Objective: To determine the preliminary usefulness of providing pain neuroscience education (PNE) on improving pain and movement in patients presenting with non-chronic mechanical low back pain (LBP).Background: PNE has been shown to be an effective intervention for the treatment of chronic LBP but its usefulness in patients with non-chronic LBP has not been examined.Methods: A single group cohort pilot study was conducted. Eighty consecutive patients with LBP < 3 months completed a demographics questionnaire, leg and LBP rating (Numeric Pain Rating Scale - NPRS), disability (Oswestry Disability Index), fear-avoidance (Fear-Avoidance Beliefs Questionnaire), pain catastrophizing (Pain Catastrophizing Scale), central sensitization (Central Sensitization Inventory), pain knowledge (Revised Neurophysiology of Pain Questionnaire), risk assessment (Keele STarT Back Screening Tool), active trunk flexion and straight leg raise (SLR). Patients received a 15-minute verbal, one-on-one PNE session, followed by repeat measurement of LBP and leg pain (NPRS), trunk flexion and SLR.Results: Immediately after intervention, LBP and leg pain improved significantly (p < 0.001), but the mean change did not exceed minimal clinically important difference (MCID) of 2.0. Active trunk flexion significantly improved (p < 0.001), with the mean improvement (4.7 cm) exceeding minimal detectible change (MDC). SLR improved significantly (p = 0.002), but mean change did not exceed MDC.Conclusions: PNE may be an interesting option in the treatment of patients with non-chronic mechanical LBP. The present pilot study provides the rationale for studying larger groups of patients in controlled studies over longer periods of time.

Effects of Pain Neuroscience Education on Physician Assistant Students Understanding of Pain and Attitudes and Beliefs About Pain.

PURPOSE: Evaluate the effectiveness and efficiency of two different pain neuroscience education (PNE) lectures provided to physician assistant (PA) students. Primary outcomes explored were knowledge of pain and shift in attitudes and beliefs about chronic pain after the lecture. METHODS: A PNE lecture was provided at two separate university PA programs. One program received a two-hour PNE lecture with a case-based example. The other program received a one-hour PNE lecture without the casebased example. Measurement of change for pre and post-test pain knowledge and attitudes and beliefs about chronic pain were recorded. RESULTS: Students at both universities showed medium effect size improvements in pain knowledge following the lecture. Only students that received the longer two-hour lecture in the case-based example showed significant improvements with their attitudes and beliefs about patients with chronic pain. CONCLUSION: PA students can increase their knowledge about current pain science through lecture alone, however, case-based learning along with lecture, may be more effective in improving the attitudes and beliefs of PA students regarding patients with chronic pain.

Addressing neurodynamic irritability in a patient with adhesive capsulitis: a case report.

BACKGROUND: Patients with adhesive capsulitis are commonly seen by physical therapists. Pain and limited shoulder motion from adhesive capsulitis have at times been linked to neural irritation. The purpose of this case is to describe the examination and intervention of a patient with adhesive capsulitis who appeared to have a coexisting, underlying neural irritation. This paper emphasizes how the neurological component must initially be identified and addressed for a successful outcome. CASE DESCRIPTION: A 47-year-old female presented with reduced shoulder motion and function, upper extremity neural irritation, diffuse weakness, altered sensation in the involved extremity, and symptoms reproduced with upper limb neurodynamic testing. Her reduced shoulder range of motion was accompanied by limited glenohumeral glides and a report of local neck stiffness. Symptoms began several months earlier after an apparent electrical shock injury to the arm that caused symptoms and guarding of the shoulder. Intervention initially addressed the underlying neural component with spinal mobilizations while avoiding further irritation. Interventions were progressed to include mobilization and exercise to address shoulder mobility. OUTCOMES: The patient's neurodynamic irritability, distal symptoms, and neck stiffness were normalized within the first weeks of care. Subsequently, interventions were directed at the shoulder. Outcomes over an 12-week time frame included reduced pain from 10/10 to 2/10. Passive range of motion increases included flexion from 121 to 160°, abduction from 71 to 121°, and external rotation from 18 to 60°. Disability scores on Disabilities of the Arm, Shoulder, and Hand (DASH) dropped from initially 68·3 to 18·3% at discharge. She ultimately regained full upper extremity function. DISCUSSION: Therapists should be cognizant of possible neural irritation in shoulder disorders, which may contribute to conditions such as adhesive capsulitis. Identifying neural irritation is critical when determining which interventions will achieve optimal outcomes without aggravating the condition.

The effect of manual therapy and neuroplasticity education on chronic low back pain: a randomized clinical trial.

OBJECTIVE: To determine if a neuroplasticity educational explanation for a manual therapy technique will produce a different outcome compared to a traditional mechanical explanation. METHODS: Sixty-two patients with chronic low back pain (CLBP) were recruited for the study. Following consent, demographic data were obtained as well as pain ratings for low back pain (LBP) and leg pain (Numeric Pain Rating Scale), disability (Oswestry Disability Index), fear-avoidance (Fear-Avoidance-Beliefs Questionnaire), forward flexion (fingertips-to-floor), and straight leg raise (SLR) (inclinometer). Patients were then randomly allocated to receive one of two explanations (neuroplasticity or mechanical), a manual therapy technique to their lumbar spine, followed by post-intervention measurements of LBP, leg pain, forward flexion, and SLR. RESULTS: Sixty-two patients (female 35 [56.5%]), with a mean age of 60.1 years and mean duration of 9.26 years of CLBP participated in the study. There were no statistically significant interactions for LBP (p = .325), leg pain (p = .172), and trunk flexion (p = .818) between the groups, but SLR showed a significant difference in favor of the neuroplasticity explanation (p = .041). Additionally, the neuroplasticity group were 7.2 times (95% confidence interval = 1.8-28.6) more likely to improve beyond the MDC on the SLR than participants in the mechanical group. DISCUSSION: The results of this study show that a neuroplasticity explanation, compared to a traditional biomechanical explanation, resulted in a measureable difference in SLR in patients with CLBP when receiving manual therapy. Future studies need to explore if the increase in SLR correlated to changes in cortical maps of the low back.

Mortality in Dravet syndrome.

We measured the mortality rate and the rate of Sudden Unexpected Death in Epilepsy (SUDEP) in Dravet Syndrome (DS). We studied a cohort of 100 consecutively recruited, unrelated patients with DS; 87 had SCN1A mutations. Living cases had a median follow-up of 17 years. Seventeen patients died, at a median age of seven years (inter-quartile range 3-11 years) with causes of death: 10 SUDEP, four status epilepticus, two drowning and one asphyxia. The SUDEP classification included three Definite, one Definite Plus and six Probable. The Dravet-specific mortality rate/1000-person-years was 15.84 (98% CI 9.01-27.85). The Dravet-specific SUDEP rate was 9.32/1000-person-years (98% CI 4.46-19.45). The Dravet-specific SUDEP rate is the only documented syndrome-specific SUDEP rate. SUDEP in DS occurs mainly in childhood. It is also the highest SUDEP rate, considerably higher than the recent 5.1 SUDEP rate/1000-person-years for adults with refractory epilepsy.

Versatile bonding and coordination modes of ditriazolylidene ligands in rhodium(iii) and iridium(iii) complexes.

Metalation of novel ditriazolium salts containing a trimethylene (-CH2CH2CH2-) or dimethylether linker (-CH2OCH2-) was probed with different rhodium(iii) and iridium(iii) precursors. When using [MCp*Cl2]2, a transmetalation protocol via a triazolylidene silver intermediate was effective, while base-assisted metalation with MCl3via sequential deprotonation of the triazolium salt with KOtBu and addition of the metal precursor afforded homoleptic complexes. The N-substituent on the triazole heterocycle directed the metalation process and led to Ctrz,Ctrz,CPh-tridentate chelating ditriazolylidene complexes for N-phenyl substituents. With ethyl substituents, only Ctrz,Ctrz-bidentate complexes were formed, while metalation with mesityl substituents was unsuccessful, presumably due to steric constraints. Through modification of the reaction conditions for the metalation step, an intermediate species was isolated that contains a Ctrz,CPh-bidentate chelate en route to the formation of the tridentate ligand system. Accordingly, Cphenyl-H bond activation occurs prior to formation of the second metal-triazolylidene bond. Stability studies with a Ctrz,Ctrz,CPh-tridentate chelating ditriazolylidene iridium complex towards DCl showed deuterium incorporation at both N-phenyl groups and indicate that Cphenyl-H bond activation is reversible while the Ctrz-Ir bond is robust. The flexible linker between the two triazolylidene donor sites provides access to both facial and meridional coordination modes.

Transfer hydrogenation with abnormal dicarbene rhodium(iii) complexes containing ancillary and modular poly-pyridine ligands.

Treatment of an abnormal dicarbene ligated rhodium(iii) dimer with 2,2'-bipyridine (bipy), 1,10-phenanthroline (phen) or 2,2':6',2''-terpyridine (terpy) results in coordination of the N-donor ligands and concomitant cleavage of the dimeric structure. Depending on the denticity of the pyridyl ligand, this situation retains one (L = terpy) or two (L = bipy, phen) flexible sites for substrate coordination. In the case of the bipy complexes, modification of the electron density at Rh, without directly affecting the steric environment about the metal centre, was achieved by the incorporation of electron-donating or electron-withdrawing substituents on the bipy backbone. The dicarbene pyridyl complexes were active in transfer hydrogenation catalysis of benzophenone at 0.15 mol% catalyst loading in a iPrOH/KOH mixture. The catalysts displayed a strong characteristic colour change (yellow to purple) after activation which allowed for visual monitoring of the status of the reaction. The colour probe and the robustness of the active catalysts proved useful for catalyst recycling. The catalytic activity sustained over five consecutive substrate batch additions and gave a maximum overall turnover number of 3100.

Initial Health Assessments and HIV Screening under the Affordable Care Act.

BACKGROUND: The Centers for Disease Control and Prevention (CDC) estimates that 156,300 (95% CI 144,100-165,900) Americans living with HIV in 2012 were unaware of their infection. To increase knowledge of HIV status, CDC guidelines seek to make HIV screening a routine part of medical care. This paper examines how routinely California primary care providers test for HIV and how providers' knowledge of California's streamlined testing requirements, use of sexual histories, and having an electronic medical record prompt for HIV testing, relate to test offers. METHODS: We surveyed all ten California health plans offered under health reform's Insurance Exchange (response rate = 50%) and 322 primary care providers to those plans (response rate = 19%) to assess use of HIV screening and risk assessments. RESULTS: Only 31.7% of 60 responding providers reported offering HIV tests to all or most new enrollees and only 8.8% offered an HIV test of blood samples all or most of the time despite the California law requiring that providers offer HIV testing of blood samples in primary care settings. Twenty-eight of the 60 providers (46.6%) were unaware that California had reduced barriers to HIV screening by eliminating the requirement for written informed consent and pre-test counseling. HIV screening of new enrollees all or most of the time was reported by 53.1% of the well-informed providers, but only 7.1% of the less informed providers, a difference of 46 percentage points (95% CI: 21.0%-66.5%). Providers who routinely obtained sexual histories were 29 percentage points (95% CI: 0.2%-54.9%) more likely to screen for HIV all or most of the time than those who did not ask sexual histories. CONCLUSION: Changing HIV screening requirements is important, but not sufficient to make HIV testing a routine part of medical care. Provider education to increase knowledge about the changed HIV testing requirements could positively impact testing rates.

HIV tests and new diagnoses declined after california budget cuts, but reallocating funds helped reduce impact.

Historically, California supplemented federal funding of HIV prevention and testing so that Californians with HIV could become aware of their infection and obtain lifesaving treatment. However, budget deficits in 2009 led the state to eliminate its supplemental funding for HIV prevention. We analyzed the impact of California's HIV resource allocation change between state fiscal years 2009 and 2011. We found that the number of HIV tests declined 19 percent, from 66,629 to 53,760, in local health jurisdictions with high HIV burden. In low-burden jurisdictions, the number of HIV tests declined 90 percent, from 20,302 to 2,116. New diagnoses fell from 2,434 in 2009 to 2,235 in 2011 (calendar years) in high-burden jurisdictions and from 346 to 327 in low-burden ones. California's budget crunch prompted state and local programs to redirect remaining HIV funds from risk reduction education to testing activities. Thus, the impact of the budget cuts on HIV tests and new HIV diagnoses was smaller than might have been expected given the size of the cuts. As California's fiscal outlook improves, we recommend that the state restore supplemental funding for HIV prevention and testing.

Early evidence from California on transitions to a reformed health insurance system for persons living with HIV/AIDS.

BACKGROUND: Many uninsured people living with HIV/AIDS (PLWHA) will obtain managed health insurance coverage when the Affordable Care Act (ACA) is implemented in January 2014. Since 2011, California has transitioned PLWHA to Medicaid managed care (MMC) and to the Low-Income Health Program (LIHP). OBJECTIVES: To draw lessons for the ACA implementation from the transitions into MMC and the LIHP. METHODS: Surveys about clients and services provided before and after the transition to MMC and the LIHP were sent to 43 HIV service providers. Usable responses were obtained from 18 (42%). RESULTS: Although total client loads were similar in the pre- (January 2011) and posttransition periods (June 2012), many clients transitioned from fee-for-service (FFS) Medicaid to MMC. Over this period, responding agencies served 43.5% fewer PLWHA in FFS Medicaid, whereas the share of PLWHA covered by MMC rose from 16.9% to 55.5%. Managed care covered a smaller number of services than either FFS Medicaid or Ryan White sites. Ryan White providers reported that 53% of the clients they served in January 2011 had transitioned to the LIHPs. Nonetheless, they continued to provide services to many of these clients, and Ryan White caseloads did not decline. CONCLUSIONS: PLWHA enrolled in the MMC continue to depend on Ryan White sites to supply the full range of services that will allow them to take full advantage of increased access to care under ACA.

Role of the sodium channel SCN9A in genetic epilepsy with febrile seizures plus and Dravet syndrome.

Mutations of the SCN1A subunit of the sodium channel is a cause of genetic epilepsy with febrile seizures plus (GEFS(+) ) in multiplex families and accounts for 70-80% of Dravet syndrome (DS). DS cases without SCN1A mutation inherited have predicted SCN9A susceptibility variants, which may contribute to complex inheritance for these unexplained cases of DS. Compared with controls, DS cases were significantly enriched for rare SCN9A genetic variants. None of the multiplex febrile seizure or GEFS(+) families could be explained by highly penetrant SCN9A mutations.

A randomized controlled trial of flunarizine as add-on therapy and effect on cognitive outcome in children with infantile spasms.

PURPOSE: Cognitive impairment is observed commonly in children with a history of infantile spasms (IS). The goal of this study was to prospectively examine the effect on cognitive outcome of a neuroprotective agent used as adjunctive therapy during treatment of the spasms. METHODS: In a randomized controlled trial, patients received a standardized therapy plus flunarizine or placebo. The standardized treatment consisted of vigabatrin as first-line therapy. Nonresponders were switched to intramuscular synthetic adrenocorticotropic hormone (sACTH depot) after 2 weeks and, if necessary, to topiramate after two additional weeks. The Vineland Adaptive Behavior Scale (VABS) and Bayley Scales of Infant Development (BSID) were used as outcome measures 24 months after the intervention. KEY FINDINGS: Sixty-eight of 101 children diagnosed over 3 years in seven centers in Canada received either adjunctive flunarizine or placebo. Sixty-five of the 68 children (96%) became spasm-free within 8 weeks and no late relapse occurred. Bayley and Vineland results were available at baseline and at 24 months in 45 children. There was no significant difference in the BSID developmental quotient between the flunarizine- and placebo-treated children at baseline (44.3 ± 35.5 vs. 30.9 ± 29.8; p = 0.18) or 24 months later (56.9 ± 33.3 vs. 46 ± 34.2; p = 0.29). However, the 10 flunarizine-treated children with no identified etiology had a better outcome than the eight controls at 24 months on both the Vineland Scale (84.1 ± 11.3 vs. 72.3 ± 9.8; p = 0.03) and the Bayley Scale (87.6 ± 14.7 vs. 69.9 ± 25.3; p = 0.07). SIGNIFICANCE: Our study failed to demonstrate a protective effect of flunarizine on cognitive outcome in a cohort of children with IS. An analysis of subgroups suggested that flunarizine may further improve cognitive outcome in children with no identified etiology.

Temporal trends in dioxin, furan and polychlorinated biphenyl concentrations in bovine milk from farms adjacent to industrial and chemical installations over a 15 year period.

The concentrations of polychlorinated dibenzo-p-dioxins, furans and polychlorinated biphenyls (PCBs) were measured in pooled bovine milk samples collected between 1991 and 2005 in County Cork, Ireland. The pooled samples were of bulk-tank milk collected from farms adjacent to industrial, chemical and pharmaceutical installations (target milk) or from rural farms distant from industrial activity (control milk). Comparing data between the first and last 3-year periods of the study revealed a 62% decrease in the mean total dioxin concentration in target milk from 1.58 to 0.60pg toxic equivalents (TEQ)/g fat. On the same basis the dioxin-like PCB concentration in target milk decreased by 80% over the study period (from 0.95pg to 0.19pg TEQ/g fat). The mean 'marker' PCB concentration in target milk from 1991 to 1993 inclusive was 3359pg/g fat. This value decreased by 75% to a mean of 849pg/g fat for the years 2003-2005 inclusive. The results of this study are consistent with low background dietary/environmental PCB contamination in both target and control herds. The total dioxin concentrations in all samples were well below the maximum tolerable limits permitted for marketable milk. The decrease in the total dioxin concentration in target and control milk samples over the study period was chiefly due to decreases in the concentration of dioxin-like PCBs, consistent with significant reductions in the concentration of PCBs in the dairy cow diet over the 15 year study period.

Electroclinical features of absence seizures in sleep.

PURPOSE: To analyze electroclinical features of absence seizures during sleep. PRINCIPAL RESULTS: 30 children with genetic generalized epilepsy had 52 paroxysms of GSW >2s during sleep. 18/52 (35%) demonstrated a clinical sign. Ictal GSW lasted an average of 6.5s. CONCLUSION: Motor manifestations are seen during GSW>2s in sleep. 72% likely represent true ictal motor features while the rest may be serendipitous sleep phenomenon.

Epilepsy with occipital features in children: factors predicting seizure outcome and neuroimaging abnormalities.

PURPOSE: The objective of this retrospective study was to identify clinical and EEG features in children with occipital epilepsy that predict MR imaging abnormalities and seizure outcome. METHODS: Patients with clinical and/or EEG features indicating occipital lobe involvement were identified from the epilepsy database at a tertiary children's hospital. The clinical and EEG features were analyzed to identify the most important predictors of abnormal MR imaging and seizure outcome. RESULTS: Sixty-six patients were identified: 21 had symptomatic epilepsy with abnormal MR imaging; 12 patients had probable symptomatic epilepsy based on an abnormal neurological exam; 33 patients had normal neurological development, normal neurological examination and normal neuroimaging. Twenty-two of these 33 patients satisfied criteria for idiopathic occipital syndromes: Panayiotopoulos syndrome (9 patients), Gastaut type idiopathic childhood occipital epilepsy (12 patients) and idiopathic photosensitive occipital epilepsy (1 patient). Eleven patients could not be classified. Early age of seizure onset and an abnormal neurological examination were significant predictors of abnormal MR imaging. None of the variables examined were strong predictors of seizure outcome. CONCLUSION: Early age of seizure onset and an abnormal neurological examination are the best predictors of abnormal MR imaging in children with epilepsy with occipital features.

The relationship of oxygen transport and cardiac index for the prevention of sickle cell crises.

The formation of deoxyhemoglobin S (deoxy-Hb S) polymers is the key triggering event for the complex pathophysiologic manifestations of sickle cell anemia (SCA). This polymer formation is associated with a marked right-shifted oxyhemoglobin dissociation curve (decreased affinity, increased P50), which results in a decrease in arterial oxygen saturation (SaO2. There is a delay period ("delay time") from the formation of deoxy-Hb S to polymerization that is markedly sensitive (to the power of 30-40) to the concentration and solubility changes of deoxy-Hb S. Deoxy-Hb S polymer formation leads to sickle cell vaso-occlusion, a unique characteristic of SCA. This theoretical study, which views SCA as a disease of oxygen transport, provides a novel framework to suggest that a small to modest increase in cardiac index (by decreasing the P50 and thus increasing the SaO2) could change the distribution of the delay times (sec) such that the balance between occlusion and opening of microcirculatory vessels is shifted favoring the opening of these vessels, therefore disfavoring vaso-occlusion. Our approach integrates a mathematical model of oxygen transport in SCA with: (1) the expression relating the solubility of deoxy-Hb S to SaO2, and (2) the kinetic expression relating the delay time to the solubility of deoxy-Hb S.

Glucose transporter-1 deficiency syndrome: the expanding clinical and genetic spectrum of a treatable disorder.

Glucose transporter-1 deficiency syndrome is caused by mutations in the SLC2A1 gene in the majority of patients and results in impaired glucose transport into the brain. From 2004-2008, 132 requests for mutational analysis of the SLC2A1 gene were studied by automated Sanger sequencing and multiplex ligation-dependent probe amplification. Mutations in the SLC2A1 gene were detected in 54 patients (41%) and subsequently in three clinically affected family members. In these 57 patients we identified 49 different mutations, including six multiple exon deletions, six known mutations and 37 novel mutations (13 missense, five nonsense, 13 frame shift, four splice site and two translation initiation mutations). Clinical data were retrospectively collected from referring physicians by means of a questionnaire. Three different phenotypes were recognized: (i) the classical phenotype (84%), subdivided into early-onset (<2 years) (65%) and late-onset (18%); (ii) a non-classical phenotype, with mental retardation and movement disorder, without epilepsy (15%); and (iii) one adult case of glucose transporter-1 deficiency syndrome with minimal symptoms. Recognizing glucose transporter-1 deficiency syndrome is important, since a ketogenic diet was effective in most of the patients with epilepsy (86%) and also reduced movement disorders in 48% of the patients with a classical phenotype and 71% of the patients with a non-classical phenotype. The average delay in diagnosing classical glucose transporter-1 deficiency syndrome was 6.6 years (range 1 month-16 years). Cerebrospinal fluid glucose was below 2.5 mmol/l (range 0.9-2.4 mmol/l) in all patients and cerebrospinal fluid : blood glucose ratio was below 0.50 in all but one patient (range 0.19-0.52). Cerebrospinal fluid lactate was low to normal in all patients. Our relatively large series of 57 patients with glucose transporter-1 deficiency syndrome allowed us to identify correlations between genotype, phenotype and biochemical data. Type of mutation was related to the severity of mental retardation and the presence of complex movement disorders. Cerebrospinal fluid : blood glucose ratio was related to type of mutation and phenotype. In conclusion, a substantial number of the patients with glucose transporter-1 deficiency syndrome do not have epilepsy. Our study demonstrates that a lumbar puncture provides the diagnostic clue to glucose transporter-1 deficiency syndrome and can thereby dramatically reduce diagnostic delay to allow early start of the ketogenic diet.

Medical management of Lennox-Gastaut syndrome.

Lennox-Gastaut syndrome occurs in 3% of children with epilepsy and is characterized by multiple seizure types, slow spike-and-wave discharges and a poor prognosis for seizure control and cognitive development. Although randomized controlled trials of adjunctive felbamate, lamotrigine, topiramate and rufinamide have demonstrated a > or =50% reduction in seizure frequency, very few children achieve complete seizure control and a Cochrane review of the treatment of Lennox-Gastaut syndrome concluded that the optimum treatment was uncertain and that no drug has been shown to be highly efficacious. Valproate, lamotrigine and topiramate were considered recently by expert panels in the US and Europe to be the first-line drugs. The ketogenic diet may be more effective than antiepileptic drugs and should be considered early in treatment. An improvement in the management of Lennox-Gastaut syndrome requires a better understanding of the pathophysiology of this disorder and the development of animal models in which to test new compounds.