The hereditary spastic paraplegias (HSPs) are a group of rare disorders with the predominant clinical feature of progressive spastic paraplegia. They are subdivided into pure and complicated forms according to whether the disorder is associated with other neurological abnormalities. We report on two unrelated female Caucasian patients with complicated HSP, aged 16 and 24 years, who showed progressive gait disturbance with spasticity and ataxia as well as cognitive impairment. Onset of symptoms was at age 3 and 10 years, respectively. MRI revealed mild diffuse non-progressive T (2)-signal alterations of cerebral white matter and thinning of the body and genu of the corpus callosum. Some similarity of clinical symptoms and MRI patterns with the phenotype of Mast syndrome prompted a mutation analysis of the SPG21 gene, encoding maspardin, which revealed a wild-type sequence in both patients. Clinical and neuroradiological features in our patients are diagnostic for complicated autosomal recessive hereditary spastic paraplegia with thin corpus callosum (HSP-TCC, SPG11). This disorder, characterized by a typical MRI pattern and a progressive spastic paraplegia that may be associated with dementia and ataxia, may have an onset in early childhood and probably is one of the more common forms of complicated HSP.
Surgical antibiotic prophylaxis (SAP) is defined as the administration of an antimicrobial agent prior to contamination in previously sterile spaces and fluid. SAP should not be confused with the therapeutic use of antibiotics. There are a growing number of studies with the goal of answering the question which patients benefit most from SAP during which specific surgical procedure. However, in the specific surgical field of head and neck surgery and otolaryngological surgery, there are only a few guidelines answering that question for specific procedures. The aim of this study was to analyze the evidence found in the literature and to develop a standard operating procedure, which specifically addresses head and neck and otolaryngeal surgical procedures.
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