Subacute sclerosing panencephalitis: brain stem involvement in a peculiar pattern

The most common pattern in subacute sclerosing panencephalitis, is in the cerebral hemisphere white matter on T2-weighted images with or without atrophy. Brain-stem lesions are rare. We report brain-stem involvement in two children with subacute sclerosing panencephalitis. A peculiar pattern, with involvement of the pons with extension to both middle cerebellar peduncles and substantia nigra but sparing the pontine tegmentum, is suggested. Received: 10 January 2000/Accepted: 18 April 2000     

On-the-job training through follow-up visits to improve the quality of family planning services.

As part of a broader set of activities to strengthen family planning training and improve the quality of family planning services in Turkey, follow-up visits were performed at different family planning sites across the country in order to conduct on-the-job training. The objective of on-the-job training was to refresh and improve family planning counselling skills for all methods as well as to refresh and improve intrauterine device insertion/removal skills and also some determinants of quality care. It was also aimed at transferring up-to-date information to family planning practitioners, identifying frequently encountered problems and helping with solution approaches for problems both at the individual and programmatic levels. The results of the follow-up visits reflect issues about both the staff and the clinical facility itself in terms of conforming with the standards of the 'National Family Planning Guidelines' set forth by the Ministry of Health. The follow-up team consisted of nine members who were specially trained. They represented different sectors such as a non-governmental organization, a medical school and the Ministry of Health. The follow-up team performed 90 visits to 16 clinics in 11 provinces between 1995 and 1998. Methods used were structured observations via standard checklists, meetings with the clinic staff, self-assessment, role plays, demonstration, coaching and the provision of feedback. During this period, a total of 130 health professionals working in 16 clinics were trained on-the-job. A significant improvement was observed in the performance of the family planning practitioners and the quality of care provided in clinics. While none of the service providers were found to have a standard skill level in general counselling during the first visit, at the end of the fifth visit all were capable of providing counselling services according to the national standards. Intrauterine device insertion skills were high at the beginning of the visits, and 16 of the 17 observed service providers (94%) were assessed as conforming to the standards. At the fifth visit, all of the 42 service providers (100%) were found to be adequate. At the facility level, all 16 clinics established separate counselling rooms in the follow-up period. Additionally, the number of clinics conforming to infection prevention standards increased from two clinics in 15 at the first visit to all 16 clinics at the fifth visit. This study showed that the ultimate success of family planning programs depend on structured and well-supervised on-the-job training through follow-up visits to the sites.     

A novel metalloprotease from Vipera lebetina venom induces human endothelial cell apoptosis.

A novel endothelial cell apoptosis inducing metalloprotease (VLAIP) was found in the snake venom of Vipera lebetina. This metalloprotease is a heterodimeric glycoprotein with molecular mass of about 106 kDa. The protease hydrolyzes azocasein, fibrinogen and oxidized insulin B-chain. The enzyme readily hydrolyzes the Aalpha-chain and more slowly Bbeta-chain of fibrinogen. VLAIP does not cleave fibrin. The complete amino acid sequences of the two different monomers of VLAIP are deduced from the nucleotide sequences of cDNAs encoding these proteins. The full-length cDNA sequences of the VLAIP-A and VLAIP-B encode open reading frames of 616 and 614 amino acids that include signal peptide, propeptide and mature metalloproteinase with disintegrin-like and cysteine-rich domains. VLAIP belongs to the metalloprotease/disintegrin family of reprolysins and has high identity with the proteins that induce apoptosis of endothelial cells. Treatment of HUVEC cells with VLAIP induces changes in the attachment of cells to the substrate and causes cell death. We demonstrated that VLAIP inhibits endothelial cell adhesion to extracellular matrix proteins: fibrinogen, fibronectin, vitronectin, collagen I, and collagen IV. The induction of apoptosis by VLAIP was shown by means of a typical DNA fragmentation pattern of apoptotic cells as well as by monitoring phosphatidylserine externalization using annexin V-FITC staining and flow cytometric analysis.     

Amyloidosis in children with familial Mediterranean fever

In this survey 113 children with secondary amyloidosis due to familial Mediterranean fever are reviewed in regard to their respective histories, and physical and laboratory findings. The beneficial effects of colchicine in the treatment of this condition are evaluated. The number of children presented with amyloidosis secondary to familial Mediterranean fever was considerable. The male-female ratio was 4/3. It was observed that the number of patients with amyloidosis increased through the adolescent period, and that most of the cases demonstrated phenotype I (74.33%). Another important finding was the increase of partial thromboplastin time in 96 out of 113 cases (84.95%). All the symptoms of the periodic attacks were relieved by colchicine. A significant difference was found between the serum total protein and albumin values before and after colchicine therapy.     

Otolaryngological findings in congenital nasolacrimal duct obstruction and implications for prognosis

AIM: To investigate otolaryngological abnormalities associated with congenital nasolacrimal duct obstruction (CNLDO) and their effect on the prognosis. METHODS: 65 consecutive cases of CNLDO were followed up with routine otorhinolaryngological examination with tympanometry. RESULTS: Otitis media with effusion (OME) and uvula bifida were detected in 44.6% and 9.2% of the children, respectively. Medical treatment and probing were less effective in patients with OME (p<0. 05). CONCLUSIONS: OME and uvula bifida are significant anomalies associated with CNLDO and the former has a marked effect on the prognosis. This finding may help to determine the patients who will need further treatment after massage and probing.     

Otitic hydrocephalus: role of radiology for diagnosis

OBJECTIVE: The pathogenesis of otitic hydrocephalus remains unclear. The use of radiological examination is very important to clarify both the diagnosis and the pathogenesis. In this article the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) is discussed. PATIENTS: Two patients with the diagnosis of otitic hydrocephalus and their MRI and MRV findings are presented. RESULTS: Both of the patients presented had thrombus in their transverse sinuses pre-operatively demonstrated by MRI causing decreased flow on MRV. After the treatment blood flow through the sinus increased significantly as the symptoms subsided. CONCLUSION: MRI and MRV are valuable tools for diagnosis and follow-up of otitic hydrocephalus.     

Clinical, electrophysiological and neuropsychological findings of twenty-two children with mesial temporal sclerosis

Twenty-two patients, followed with the diagnosis of epilepsy between the ages of 8.5 and 19 years who were found to have mesial temporal sclerosis (MTS) on brain magnetic resonance imaging (MRI) were evaluated according to their clinical, electrophysiological and neuropsychological characteristics, and assessed for the significance of neuropsychological tests on lateralization. In both MTS groups, a remarkable number of patients had their intial seizure before one year of age (45.5% in the right MTS group, and 36.4% in the left MTS group). Half of the patients were seizure-free for a period of two months-9.5 years (average 3.9 years). The duration between the febrile seizure and first non-febrile seizure was 6.2 years. Eleven patients had MTS on the left temporal lobe. Interictal EEG was normal in five (22.5%) patients; 10 (45.5%) had temporal spike on the corresponding side with MTS. Six (27.3%) patients had paroxysmal activity and voltage asymmetry on cortical areas other than temporal region. The Wechsler Memory Scale (WMS), Visual Reproduction Subtest and Auditory Verbal Learning Test (AVLT) Delayed Recall and Retrieval Subtest performances were poor in the right MTS group.