Extracranial Trigger Site Surgery for Migraine: A Systematic Review With Meta-Analysis on Elimination of Headache Symptoms.

Introduction: The headache phase of migraine could in selected cases potentially be treated by surgical decompression of one or more "trigger sites," located at frontal, temporal, nasal, and occipital sites. This systematic review with subsequent meta-analysis aims at critically evaluating the currently available evidence for the surgical treatment of migraine headache and to determine the effect size of this treatment in a specific patient population. Methods: This study was conducted following the PRISMA guidelines. An online database search was performed. Inclusion was based on studies published between 2000 and March 2018, containing a diagnosis of migraine in compliance with the classification of the International Headache Society. The treatment must consist of one or more surgical procedures involving the extracranial nerves and/or arteries with outcome data available at minimum 6 months. Results: Eight hundred and forty-seven records were identified after duplicates were removed, 44 full text articles were assessed and 14 records were selected for inclusion. A total number of 627 patients were included in the analysis. A proportion of 0.38 of patients (random effects model, 95% CI [0.30-0.46]) experienced elimination of migraine headaches at 6-12 months follow-up. Using data from three randomized controlled trials, the calculated odds ratio for 90-100% elimination of migraine headaches is 21.46 (random effects model, 95% CI [5.64-81.58]) for patients receiving migraine surgery compared to sham or no surgery. Conclusions: Migraine surgery leads to elimination of migraine headaches in 38% of the migraine patients included in this review. However, more elaborate randomized trials are needed with transparent reporting of patient selection, medication use, and surgical procedures and implementing detailed and longer follow-up times.

The Impacts of Tumor and Tumor Associated Epilepsy on Subcortical Brain Structures and Long Distance Connectivity in Patients With Low Grade Glioma.

Low grade gliomas in cerebral cortex often cause symptoms related to higher cerebral functions such as attention, memory and executive function before treatment is initiated. Interestingly, focal tumors residing in one cortical region can lead to a diverse range of symptoms, indicating that the impact of a tumor is extended to multiple brain regions. We hypothesize that the presence of focal glioma in the cerebral cortex leads to alterations of distant subcortical areas and essential white matter tracts. In this study, we analyzed diffusion tensor imaging scans in glioma patients to study the effect of glioma on subcortical gray matter nuclei and long-distance connectivity. We found that the caudate nucleus, putamen and thalamus were affected by cortical glioma, displaying both volumetric and diffusion alterations. The cerebellar cortex contralateral to the tumor side also showed significant volume decrease. Additionally, tractography of the cortico-striatal and cortico-thalamic projections shows similar diffusion alterations. Tumor associated epilepsy might be an important contributing factor to the found alterations. Our findings indeed confirm concurrent structural and connectivity abrasions of brain areas distant from brain tumor, and provide insights into the pathogenesis of diverse neurological symptoms in glioma patients.

Two novel mutations in the gene for human alpha-mannosidase that cause alpha-mannosidosis.

Mutation analysis performed on two Italian patients with alpha-mannosidosis allowed the identification of two new mutations, IVS20-2A>G and 322-323insA. The patients were both homozygous for these mutations. The first mutation causes skipping of exon 21, whereas the second causes a frameshift introducing a stop codon at position 160 of the amino acid sequence.

Mouse beta-mannosidase: cDNA cloning, expression, and chromosomal localization.

Beta-mannosidase is an exoglycosidase involved in the degradation of N-linked oligosaccharides moieties of glycoproteins. Lack of beta-mannosidase activity leads to the lysosomal disorder beta-mannosidosis (MIM 248510). We have isolated and sequenced the gene encoding the mouse beta-mannosidase. Comparison of the deduced amino acid sequence of mouse, human, bovine, and goat beta-mannosidase showed 64%, identity, reflecting a high degree of evolutionary conservation. Analysis of a multiple tissue northern blotting revealed a major transcript of about 3.7 kb in all tissues examined. The northern analysis also demonstrates that there is differential tissue mRNA expression. The mouse beta-mannosidase gene (Bmn) was mapped to the distal end of Chromosome (Chr) 3, in a region that is homologous with a segment of human Chr 4 containing the orthologous human gene.

Gene encoding the mouse sulphamidase: cDNA cloning, structure, and chromosomal mapping.

Sulphamidase is an exoglycosidase involved in the degradation of heparan sulfate. Lack of sulphamidase activity leads to the lysosomal storage disorder Mucopolysaccharidosis type IIIA (Sanfilippo type A OMIM No. 252900). At present there are no naturally occurring small animal models of this disease that could be of fundamental importance to study the pathophysiology of the disease and to try therapeutic strategies. Cloning of the mouse gene is an important step to create a mouse model for this common mucopolysaccharidosis. We have isolated and sequenced the gene encoding mouse sulphamidase. Comparison of the deduced amino acid sequences of human and mouse sulphamidase showed 88% identity and 93% similarity. The exon-intron structure of the gene has been determined with the mouse 10-kb gene divided in 8 exons. The mouse sulphamidase gene (Sgsh) was mapped to the distal end of Chromosome (Chr) 11, in a region that is homologous with a segment of human Chr 17 containing the orthologous human gene.