Are early myoclonic encephalopathy (EME) and the Ohtahara syndrome (EIEE) independent of each other?

BACKGROUND: Early myoclonic encephalopathy (EME) and the Ohtahara syndrome are currently listed as two separate syndromes in the classification of epilepsies. The most prominent differentiating points are the observations that patients with Ohtahara syndrome experience predominantly tonic seizures; their seizures evolve to infantile spasms and the prognosis is often worse than patients with EME. SUMMARY POINTS: We performed a literature review of published cases. Although syndromes may have distinct courses, the differentiation early on may be impossible as both myoclonus and tonic seizures may coexist. There is also an overlap in the etiologies. Tonic seizures are considered a manifestation of brainstem dysfunction and it is possible that this is more prominent in Ohtahara syndrome. To date, there are 17 autopsy cases (12 presumed to be Ohtahara cases and 5 EME). Evidence of hindbrain pathology was present in all. Tonic seizures or tonic posturing was a feature of all cases. We suggest that the two syndromes may represent a continuum and that the prominence of tonic seizures in the Ohtahara syndrome may be an indication of brainstem dysfunction which may play an important role in the subsequent transition to infantile spasms.     

Influence of asthma on quality of life and clinical characteristics of patients with nasal polyposis

Recent studies have evidenced that nasal polyposis (NP) may lead to significant limitations in physical, emotional and social aspects of life of the affected patients. The study is aimed to investigate the influence of asthma on quality of life (QoL), intensity of symptoms, endoscopic and computerized tomography (CT) sinus findings in patients with NP. The cross-sectional study included 88 adult patients with NP out of whom 35 (39.8 %) were asthmatic while 53 (60.2 %) were non-asthmatic. QoL is assessed based on Short Form-36 Health Survey (SF-36) questionnaire, while symptom intensity was presented using visual analogue scale (VAS). The objective finding is presented as endoscopic and CT score. Comparison of individual symptom intensity, total score and major symptom score failed to evidence any statistically significant difference between the groups. Minor symptom score which include intensity of headache, fetor ex ore, fatigue/malaise, dental pain, cough, pressure/fullness in the ears and fever was higher in the group with asthma (p < 0.05). Comparison of scores according to SF-36 domains, as well as summary scores for physical and mental health did not reveal statistically significant difference between the observed groups. Mean value of the endoscopic score in the group with asthma was 8.57 ± 2.22, being 8.38 ± 1.93 in the group without asthma (p > 0.05). Mean value of the CT score in the groups with and without asthma was 20.37 ± 4.34 and 17.47 ± 4.75, respectively (p < 0.01). Asthma has no influence on QoL and endoscopic findings of patients with NP, however it influences minor symptom score and CT findings.     

Complications of operative treatment of injuries of peripheral arteries

In 1991 and 1992, a total of 97 patients with 106 peripherial arterial injuries underwent surgery at the Institute for Cardiovascular Diseases, Clinical Centre of Serbia. Civilian injuries accounted for 53 (54.6%) patients (94.3% males, age range: 16-63 yr, mean: 35.2), and 44 patients had war injuries (93.2% males, age range: 19-61 yr, mean: 34.8). The injuries affected the superfitial femoral artery in 31 (29.24%); the popliteal artery in 28 (26.41%); the brachial artery in 17 (16.04%); the posterior tibial artery in 6 (5.66%); the axillary artery in 5 (4.72%); the anterior tibial artery in 5 (4.72%); the tibioperoneal trunk in 4 (3. 77%); the common femoral artery in 4 (3.77%); the external iliac artery in 2 (1.89%); the profound femoral artery in 2 (1.89%); the radial artery in 1 (0.94%); and ulnar artery in 1 (0.94%).A total of 98 reconstructive procedures were used to treat these patients. Graft interposition carried out in 50 (51.02%); by pass in 25 (25. 51%); end-to-end anastomosis in 9 (9.18%); suture in 8 (8.16%); ligation in 4 (4.08%); and patch-angioplasty in 2 (2.04%). Primary reconstruction of injured arteries was performed in 72.2% and secondary repair in 27.8% cases.Infection developed in 51 (52.57%) patients, and it was significantly (P<0.05) more common in the war injuries (70.45%) and in secondary repairs (88.89%). The presence of associated lesions (69.56%) was also correlated with a greater rate of infection.Amputation was necessary in 21 (21.65%) of our patients, and was significantly (P<0.05) more often performed after secondary (44.44%) than primary operations (12.86%) and in the presence of associated injuries (32.61%).     

Childhood ADHD is strongly associated with a broad range of psychiatric disorders during adolescence: a population-based birth cohort study

Background: To evaluate associations between attention‐deficit/hyperactivity disorder (ADHD) and comorbid psychiatric disorders using research‐identified incident cases of ADHD and population‐based controls. Methods: Subjects included a birth cohort of all children born 1976–1982 remaining in Rochester, MN after age five (n = 5,718). Among them we identified 379 ADHD incident cases and 758 age‐gender matched non‐ADHD controls, passively followed to age 19 years. All psychiatric diagnoses were identified and abstracted, but only those confirmed by qualified medical professionals were included in the analysis. For each psychiatric disorder, cumulative incidence rates for subjects with and without ADHD were estimated using the Kaplan–Meier method. Corresponding hazard ratios (HR) were estimated using Cox models adjusted for gender and mother’s age and education at the subject’s birth. The association between ADHD and the likelihood of having an internalizing or externalizing disorder was summarized by estimating odds ratios (OR). Results: Attention‐deficit/hyperactivity disorder was associated with a significantly increased risk of adjustment disorders (HR = 3.88), conduct/oppositional defiant disorder (HR = 9.54), mood disorders (HR = 3.67), anxiety disorders (HR = 2.94), tic disorders (HR = 6.53), eating disorders (HR = 5.68), personality disorders (HR = 5.80), and substance‐related disorders (HR = 4.03). When psychiatric comorbidities were classified on the internalization‐externalization dimension, ADHD was strongly associated with coexisting internalizing/externalizing (OR = 10.6), or externalizing‐only (OR = 10.0) disorders. Conclusion: This population‐based study confirms that children with ADHD are at significantly increased risk for a wide range of psychiatric disorders. Besides treating the ADHD, clinicians should identify and provide appropriate treatment for psychiatric comorbidities.     

Endoscopic supraglottic laryngectomy: a proposal for a classification by the working committee on nomenclature, European Laryngological Society

In July 1999, the European Laryngological Society (ELS) has accepted a proposal for the classification of different laryngeal endoscopic cordectomies. This is actually a common classification system used as a tool for surgical training, documentation and comparison of results. The same harmonization work is deemed necessary for the treatment of supraglottic lesions. The ELS is proposing a classification of the different laryngeal endoscopic supraglottic partial laryngectomies. This classification comprises four types of supraglottic laryngectomies: Type I, limited excision of small size superficial lesions of the free edge of the epiglottis, the ary-epiglottic fold, the arytenoid, or the ventricular fold or any other part of the supraglottis; Type II, medial supraglottic laryngectomy without resection of the pre-epiglottic space, suitable for T1 lesions of either the suprahyoid or the infrahyoid laryngeal surface of the epiglottis (Type IIa, superior hemi-epiglottectomy or Type IIb, total epiglottectomy, respectively); Type III, medial supraglottic laryngectomy with resection of the pre-epiglottic space, suitable for T1–T2 tumors of the infrahyoid endolaryngeal epiglottis without (Type IIIa) or with (Type IIIb) extension to the ventricular fold, necessitating its excision; finally, Type IV, lateral supraglottic laryngectomy, suitable for tumors of the threefolds’ region, which may include the ventricular fold (Type IVa) or the arytenoid (Type IVb), when involved. As in the cases of endoscopic cordectomies, these operations are similarly classified according to the surgical approach used and the degree of resection completed in order to facilitate their use in daily clinical practice.     

Autism: a review of the state of the science for pediatric primary health care clinicians

Autism is a complex neurodevelopmental disorder characterized by impaired reciprocal social interaction, impaired communication, and restricted, repetitive, or stereotyped behaviors. Autism seems to affect more children than was previously believed, although this phenomenon may be due to broadening of the diagnostic criteria and increased awareness of the condition. Recent research has clearly indicated the importance of early identification, since early intensive treatment is associated with better long-term outcome. There are many controversies and competing theories about the etiology and treatment of autism, often leaving families confused about the best course of treatment and intervention. Pediatric primary health care clinicians have an important role in both the early identification and ongoing management of children with autism. It is, therefore, essential that primary care clinicians have up-to-date information about the science of autism.     

Performance on the Operant Test Battery in young children exposed to procedures requiring general anaesthesia: the MASK study

Background

It is not known whether the neurotoxicity produced by anaesthetics administered to young animals can also occur in children. Exposure of infant macaques to ketamine impairs performance in selected domains of the Operant Test Battery (OTB), which can also be administered to children. This study determined whether a similar pattern of results on the OTB is found in children exposed to procedures requiring general anaesthesia before age 3 yr.