Botulinum toxin is effective and safe for palatal tremor: a report of five cases and a review of the literature.

Palatal tremor (formerly palatal myoclonus) is an extremely rare, but potentially treatable cause, of objective tinnitus. The tinnitus is thought to be secondary to rhythmic involuntary movements of the soft palate. Its aetiology is variable and it remains difficult to treat. Many different medical and surgical remedies have been tried but none have demonstrated reproducible success. Botulinum toxin has been used in sporadic cases and seems to produce good results. Ten patients with palatal tremor have presented to this department over the last three years. After discussion with the patients with regard to the management of this condition and possible complications, five opted for botulinum toxin therapy and five declined further intervention. Clinical diagnosis was made on the confirmation of soft palate movements synchronous with an audible clicking noise. Five patients underwent botulinum toxin injection into the insertion of the levator and tensor veli palatini muscles. Of the five that were treated with toxin, four showed complete resolution of symptoms after a course of treatment. Only one patient reported transient side effects. This would suggest that botulinum toxin is a safe and effective first line treatment for palatal tremor.

Pre-treatment clinical assessment in head and neck cancer: United Kingdom National Multidisciplinary Guidelines.

This is the official guideline endorsed by the specialty associations involved in the care of head and neck cancer patients in the UK. This paper provides recommendations on the pre-treatment clinical assessment of patients presenting with head and neck cancer. Recommendations • Comorbidity data should be collected as it is important in the analysis of survival, quality of life and functional outcomes after treatment as well as for comparing results of different treatment regimens and different centres. (R) • Patients with hypertension of over 180/110 or associated target organ damage, should have antihypertensive medication started pre-operatively as per British Hypertension Society guidelines. (R) • Rapidly correcting pre-operative hypertension with beta blockade appears to cause higher mortality due to stroke and hypotension and should not be used. (R) • Patients with poorly controlled or unstable ischaemic heart disease should be referred for cardiology assessment pre-operatively. (G) • Patients within one year of drug eluting stents should be discussed with the cardiologist who was responsible for their percutaneous coronary intervention pre-operatively with regard to cessation of antiplatelet medication due to risk of stent thrombosis. (G) • Patients with multiple recent stents should be managed in a centre with access to interventional cardiology. (G) • Surgery after myocardial infarction should be delayed if possible to reduce mortality risk. (R) • Patients with critical aortic stenosis (AS) should be considered for pre-operative intervention. (G) • Clopidogrel should be discontinued 7 days pre-operatively; warfarin should be discontinued 5 days pre-operatively. (R) • Patients with thromboembolic disease or artificial heart valves require heparin therapy to bridge peri-operative warfarin cessation, this should start 2 days after last warfarin dose. (R) • Cardiac drugs other than angotensin-converting enzyme inhibitors and angiotensin II antagonists should be continued including on the day of surgery. (R) • Angotensin-converting enzyme inhibitors and angiotensin II antagonists should be withheld on the day of surgery unless they are for the treatment of heart failure. (R) • Post-operative care in a critical care area should be considered for patients with heart failure or significant diastolic dysfunction. (R) • Patients with respiratory disease should have their peri-operative respiratory failure risk assessed and critical care booked accordingly. (G) • Patients with severe lung disease should be assessed for right heart disease pre-operatively. (G) • Patients with pulmonary hypertension and right heart failure will be at extraordinarily high risk and should have the need for surgery re-evaluated. (G) • Perioperative glucose readings should be kept within 4-12 mmol/l. (R) • Patients with a high HbA1C facing urgent surgery should have their diabetes management assessed by a diabetes specialist. (G) • Insulin-dependent diabetic patients must not omit insulin for more than one missed meal and will therefore require an insulin replacement regime. (R) • Patients taking more than 5 mg of prednisolone daily should have steroid replacement in the peri-operative period. (R) • Consider proton pump therapy for patients taking steroids in the peri-operative phase if they fit higher risk criteria. (R) • Surgery within three months of stroke carries high risk of further stroke and should be delayed if possible. (R) • Patients with rheumatoid arthritis should have flexion/extension views assessed by a senior radiologist pre-operatively. (R) • Patients at risk of post-operative cognitive dysfunction and delirium should be highlighted at pre-operative assessment. (G) • Patients with Parkinson's disease (PD) must have enteral access so drugs can be given intra-operatively. Liaison with a specialist in PD is essential. (R) • Intravenous iron should be considered for anaemia in the urgent head and neck cancer patient. (G) • Preoperative blood transfusion should be avoided where possible. (R) • Where pre-operative transfusion is essential it should be completed 24-48 hours pre-operatively. (R) • An accurate alcohol intake assessment should be completed for all patients. (G) • Patients considered to have a high level of alcohol dependency should be considered for active in-patient withdrawal at least 48 hours pre-operatively in liaison with relevant specialists. (R) • Parenteral B vitamins should be given routinely on admission to alcohol-dependent patients. (R) • Smoking cessation, commenced preferably six weeks before surgery, decreases the incidence of post-operative complications. (R) • Antibiotics are necessary for clean-contaminated head and neck surgery, but unnecessary for clean surgery. (R) • Antibiotics should be administered up to 60 minutes before skin incision, as close to the time of incision as possible. (R) • Antibiotic regimes longer than 24 hours have no additional benefit in clean-contaminated head and neck surgery. (R) • Repeat intra-operative antibiotic dosing should be considered for longer surgeries or where there is major blood loss. (R) • Local antibiotic policies should be developed and adhered to due to local resistance patterns. (G) • Individual assessment for venous thromboembolism (VTE) risk and bleeding risk should occur on admission and be reassessed throughout the patients' stay. (G) • Mechanical prophylaxis for VTE is recommended for all patients with one or more risk factors for VTE. (R) • Patients with additional risk factors of VTE and low bleeding risk should have low molecular weight heparin at prophylactic dose or unfractionated heparin if they have severe renal impairment. (R).

Otorhinolaryngological manifestations of the mucopolysaccharidoses.

The mucopolysaccharidoses (MPS) are a family of related inherited metabolic disorders where, due to specific lysosomal enzyme deficiencies, partially degraded glycosaminoglycans (GAGs) accumulate in the body's cells. Due to the ubiquitous nature of GAGs in the body this deposition can occur in many tissue types and may interfere with cellular function. Although these conditions are rare, there is a propensity for the disease process to cause problems with the function of the ears, noses and throats of affected patients. In this review, we present an overview of the clinical manifestations of MPS in general and highlight the problems specifically presenting in the field of otorhinolaryngology.

Surgical management of medullary thyroid cancer: which guidelines should we follow?

BACKGROUND: Surgery is currently the only curative treatment for medullary thyroid cancer. Unfortunately, the surgical strategy that will offer patients at each disease stage the best chance of a biochemical cure remains unclear. The American Thyroid Association and British Thyroid Association guidelines offer different strategies. METHODS: A retrospective analysis of the surgical management of 47 patients with medullary thyroid cancer diagnosed between 1994 and 2013 was performed. Surgical management was compared with current American Thyroid Association and British Thyroid Association guidelines. Outcome was defined as the first post-operative calcitonin measurement. RESULTS: All patients with stage I-III disease achieved a post-operative biochemical cure regardless of the guidelines followed. The overall biochemical cure rate for patients with stage IVa disease was significantly reduced to 10 per cent (p < 0.01), but the biochemical cure rate for stage IVa disease patients who underwent bilateral lateral lymph node dissection was 33.3 per cent. CONCLUSION: The conservative, surveillance-driven approach recommended by the American Thyroid Association is appropriate for stage I-III disease. However, the more aggressive approach advocated by the British Thyroid Association might provide stage IVa disease patients a greater chance of achieving a biochemical cure.

Bone mineral density in geographically diverse adolescent populations.

OBJECTIVE: Measure bone mineral density (BMD) in healthy Newfoundland adolescents and determine whether BMD is comparable in geographically diverse adolescent populations. STUDY DESIGN: Lumbar spine BMD was measured by dual-energy radiograph absorptiometry in 26 healthy adolescents between ages 8 and 20 years. The age and gender of these subjects were used to predict BMD from equations derived from normative BMD data in six geographically diverse populations. The actual BMD value obtained for each subject was then compared with each of the six predicted BMD values for that adolescent using the Wilcoxon signed-ranks test. RESULTS: Actual lumbar spine BMD in Newfoundland adolescents was not significantly different from that predicted by age and gender if they were from California, Finland, France, North Carolina, and Switzerland. Only the prediction based on the Spanish population resulted in a 4% greater BMD than was actually measured in the Newfoundland adolescents. CONCLUSIONS: Lumbar spine BMD measurements for most healthy adolescent populations, as in adults, are comparable despite geographic diversity. Thus, generation of institution-specific normative BMD data may not be necessary for most adolescent populations.

Vigabatrin in childhood epilepsy: comparable efficacy for generalized and partial seizures.

Thirty-one patients, aged 12.6 +/- 5.6 years, with refractory seizures for 8 +/- 4.3 years, were treated with adjunctive vigabatrin. Twenty-four percent had a > 50% reduction in seizure frequency (95% one-sided confidence interval). Generalized myoclonic, atonic, and tonic clonic and partial, with and without secondary generalization, seizures were all reduced at a mean dose of 70 +/- 38 mg/kg/day. Comparison of vigabatrin therapy duration, for partial and generalized seizure groups, utilizing Kaplan-Meier methodology showed similar survival times. Vigabatrin therapy was ineffective in the four children with tuberous sclerosis. Transient somnolence, ataxia and dizziness were the most frequent side effects. A severe aggressive agitation occurred in three patients, and necessitated discontinuation of vigabatrin in one patient. Vigabatrin was as effective in generalized as in partial seizures in this study. Clinical utility may be limited by unacceptable behavioral side effects in some patients.

Midazolam in the treatment of refractory neonatal seizures.

Midazolam, a potent short-acting benzodiazepine, is a safe and highly effective agent for the control of status epilepticus. Its efficacy in the control of neonatal seizures, however, has not been determined. Six neonates (aged 1-9 days; gestation, 30-41 weeks) developed seizures from a variety of causes. In each case, seizures persisted for > 12 h despite high-dose phenobarbital therapy with or without the addition of phenytoin. Midazolam was then administered by continuous intravenous infusion (0.1-0.4 mg/kg/h) for 1 to 3 days. Within 1 h of initiation of midazolam, seizures were controlled in all six neonates. Electroencephalographic seizures were abolished in four of six neonates; however, two neonates continued to have electrographic seizures (without clinical accompaniment) for a further 12 h. Blood pressure and pulse rate were not changed after the initiation of midazolam, and adverse reactions were not observed in any neonate. Because more than one third of all neonatal seizures are refractory to high-dose phenobarbital and phenytoin, midazolam administered by continuous intravenous infusion may be a valuable adjunctive therapy.

Zinc, copper, manganese, and iron balance of parenterally fed very low birth weight preterm infants receiving a trace element supplement.

To evaluate a pediatric trace element supplement (Ped-El, Pharmacia) 18 metabolic balance studies were completed in 13 infants (mean birth weight 909 +/- 67 g, x +/- SEM; mean gestational age 27.2 +/- 1 weeks) who received total parenteral nutrition. The supplement supplied 40 micrograms/kg/day of zinc resulting in negative retention of 226 micrograms/kg/day. Copper infused at 20 micrograms/kg/day led to a positive retention of 8 micrograms/kg/day and an increase in serum Cu (p less than 0.05) not related to Cu intakes. Manganese infused at 40 micrograms/kg/day was nearly all retained (88 +/- 16% retention). Iron infused at 120 micrograms/kg/day led to a positive retention of 93 micrograms/kg/day. Although plasma ferritin and percent transferrin saturation were elevated, only plasma Fe values were correlated with Fe intake. This trace element supplement does not appear suitable for very low birth weight preterm infants.

Clobazam for intractable pediatric epilepsy.

We report our experience with add-on clobazam therapy over a 5-year period in 63 children with refractory epilepsy. The mean duration of epilepsy was 6.7 years. Children were followed for 15 to 64 months. Of 63 children, 57 were developmentally delayed, and 54 had a symptomatic/cryptogenic epilepsy. Forty-one percent became either seizure free or had a greater than 90% reduction in seizure frequency. Seizure frequency was reduced 50% to 90% in another 24%. The average daily dose of clobazam was 0.8 mg/kg. Thirty-five percent had the medication withdrawn for persistent or unacceptable side effects or the development of tolerance (seven patients). Side effects included severe aggressive outbursts, hyperactivity, insomnia, and depression with suicidal ideation. Clobazam is a useful add-on medication for 65% of children with epilepsy. Clinical utility may be limited by behavioral side effects in some patients.

Cisapride in functional dyspepsia: a double-blind, placebo-controlled randomized trial in general practice patients.

This trial included patients from general practice with endoscopy-negative chronic dyspepsia and epigastric pain or discomfort. Eleven eligible patients with sufficiently severe dyspeptic symptoms after a 2-week placebo run-in period were entered into a 4-week, parallel group, double-blind randomized comparison of 10 mg cisapride three times daily and matched placebo, and were subsequently evaluable. Symptoms were comparable in the two treatment groups at the start of double-blind treatment. The cisapride group had a significantly greater reduction in the frequency of daytime epigastric pain/discomfort and the frequency and severity of nocturnal pain/discomfort after 2 weeks. After 2 weeks, all six cisapride recipients were free of nocturnal pain, compared with only one of five placebo recipients. After 4 weeks of double-blind therapy, improvements in the placebo group had reduced between-treatment differences, with five of six cisapride recipients and three of five placebo recipients being free of nocturnal pain. Cisapride was well tolerated.

Radiofrequency ablation in the treatment of paediatric microcystic lymphatic malformations.

OBJECTIVE: Congenital lymphatic malformations are a challenging clinical problem. There is currently no universally accepted treatment for the management of microcystic disease. We describe the novel use of an existing technology (radiofrequency ablation, also termed Coblation) for the debulking of paediatric microcystic lymphatic malformations involving the upper aerodigestive tract. METHODS: Five children with microcystic or mixed-type lymphatic malformations were included in this retrospective case series. RESULTS: Each child had a satisfactory outcome following radiofrequency debulking, with improved oral intake and airway symptoms. No serious complications were reported. These findings constitute level IV evidence. CONCLUSION: We recommend radiofrequency ablation as a safe, viable alternative to existing techniques for the treatment of paediatric microcystic lymphatic malformations of the upper aerodigestive tract. Radiofrequency ablation achieves effective debulking of microcysts whilst avoiding excessive bleeding and thermal damage to surrounding tissues. This paper constitutes the first report of successful treatment of airway obstruction due to paediatric laryngopharyngeal microcystic disease, using radiofrequency ablation.

History of cesarean section associated with childhood onset of T1DM in Newfoundland and Labrador, Canada.

OBJECTIVES: Newfoundland and Labrador (NL) has one of the highest incidences of Type 1 diabetes mellitus (T1DM) worldwide. Rates of T1DM are increasing and the search for environmental factors that may be contributing to this increase is continuing. METHODS: This was a population-based case control design involving the linkage of data from a diabetes database with live birth registration data. 266 children aged 0-15 years with T1DM were compared to age- and gender-matched controls. Chi-square analysis and multivariate conditional logistic regression were carried out to assess maternal and infant factors (including maternal age, marital status, education, T1DM, hypertension, birth order, delivery method, gestational age, size-for-gestational-age, and birth weight). RESULTS: Cases of T1DM were more likely to be large-for-gestational-age (P = 0.024) and delivered by C-section (P = 0.009) as compared to controls. C-section delivery was associated with increased risk of T1DM (HR 1.41, P = 0.015) when birth weight and gestational age were included in the model, but not when size-for-gestational-age was included (HR 1.3, P = 0.076). CONCLUSIONS: Birth by C-section was found to be a risk factor for the development of T1DM in a region with high rates of T1DM and birth by C-section. These findings may have an impact on health practice, health care planning, and future research.

Thyroid lymphoma: acute presentation and long-term outcome.

BACKGROUND: Thyroid lymphomas are relatively uncommon. This study aimed to analyse our experience of thyroid lymphoma management and outcome. MATERIALS AND METHODS: A retrospective case note analysis of 63 patients treated in the previous 13 years was conducted. RESULTS: The five-year survival rate was 68 per cent, with most patients dying of their lymphoma. This is at odds with the British Thyroid Association statement that the prognosis of this condition is 'generally excellent'. The only presenting symptom found to be significantly associated with prognosis was dysphagia (p = 0.001). Dual modality treatment provided a significantly better outcome than single modality treatment (p = 0.014). Thyroid lymphoma can present to the head and neck surgeon 'in extremis'; however, it can respond rapidly to appropriate treatment. CONCLUSION: The outcome of thyroid lymphoma seems unrelated to the acuteness of its presentation. Thyroid surgery has no role other than for diagnosis. However, 51 per cent of the study patients underwent some form of thyroidectomy, indicating the need to implement better diagnostic pathways.

The epidemiology of clinical neonatal seizures in Newfoundland: a population-based study.

OBJECTIVE: To study the incidence, clinical features, etiologic distribution, and day of seizure onset by etiology in neonates with seizures. DESIGN: Prospective, population-based study involving all the obstetric and neonatal units across the province of Newfoundland, Canada. All units were given educational sessions on neonatal seizure symptomatology. SUBJECTS: Detailed questionnaires were prospectively collected for all infants with probable neonatal seizures for a period of 5 years. RESULTS: The incidence rate was 2. 6 per 1000 live births, 2.00 for term neonates, 11.1 for preterm neonates, and 13.5 for infants weighing <2500 g at birth. Seizures lasting 30 minutes or longer were present in 5%, and the neonatal death rate among infants with seizures was 9%. Hypoxic-ischemic encephalopathy was the presumed cause in 40%, infections in 20%, and metabolic abnormalities in 19%. CONCLUSIONS: Clinical neonatal seizures occur 6 times more often in preterm infants than in term infants. Hypoxic-ischemic encephalopathy continues to be a major marker of the likelihood of seizures.

The role of vigabatrin in childhood seizure disorders: results from a clinical audit.

PURPOSE: The emergence of visual field defects attributed to vigabatrin (VGB) treatment and intramyelinic edema in animal experiments has raised concerns about its future role in the treatment of childhood seizures. METHODS: We evaluated our experience with this antiepileptic agent with retrospective analysis of database and chart audit. RESULTS: Of 73 patients, 43 girls and 33 boys were treated with VGB over a 7-year period. The mean age of patients at the introduction of VGB was 87 months (range, 5-257 months). In 12 of 73 cases, VGB was used as monotherapy; in 61 of 73 cases, it was used as an add-on drug. Seizure types included secondarily generalized seizures (21), mixed seizures (21), partial seizures (18), and generalized seizures (13). Seizure etiology was idiopathic/cryptogenic in 22 patients, symptomatic in 50, and undetermined in a single patient. The mean duration of therapy was 16 months (median, 10 months; range, 1-144 months). VGB was effective in 30 (seven seizure free, 23 with >90% reduction in seizures), partially effective in four (50-90% reduction in seizures), and ineffective in 38 (<50% reduction in seizures). Nearly 50% of patients with infantile spasms responded to VGB. All patients underwent ophthalmic evaluation; two (16%) of 12 patients who could undergo static threshold perimetry were demonstrated to have the characteristic visual field constriction. CONCLUSIONS: VGB is effective in producing a significant reduction in seizure frequency in nearly half the patients with childhood seizures, including refractory epilepsy. Despite emerging concerns regarding visual side effects, this drug retains an important role in the medical management of childhood epilepsy.

Effect of carbamazepine and valproate on bone mineral density.

OBJECTIVE: To examine the effect of carbamazepine and valproate monotherapy on bone mineral density in children. METHODS: Axial (second, third, and fourth lumbar vertebrae) and appendicular (distal third of radius) bone mineral density was measured by dual-energy x-ray absorptiometry in 27 healthy children and 26 children with uncomplicated idiopathic epilepsy treated with either carbamazepine (n = 13) or valproate (n = 13) for more than 18 months. Control subjects and patients were similar with respect to age, race (all white), and geographic area, and had no dietary restrictions, neurologic impairment, or physical handicaps. RESULTS: Subjects were seizure-free for more than 6 months on a regimen of carbamazepine or valproate therapy, and had mean serum trough levels of 6.88 +/- 2 micrograms/ml and 72.04 +/- 45.6 micrograms/ml, respectively. Dietary calcium intake was similar in control and treated groups. After correction for gender and age, children treated with valproate had a 14% (p = 0.003) and 10% (p = 0.005) reduction in bone mineral density at the axial and appendicular sites, respectively. The reduction in bone mineral density increased with the duration of valproate therapy. Carbamazepine did not significantly reduce bone mineral density. CONCLUSION: Valproate montherapy, but not carbamazepine therapy, significantly reduces axial and appendicular bone mineral density in children with idiopathic epilepsy and may increase their risk of osteoporotic fractures.