Orthostatic headache and bilateral abducens palsy secondary to spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is a well-documented syndrome characterized typically by a benign, self-limited course. Patients typically present with postural or exertional headaches that can be temporarily relieved by lying in a supine or recumbent position. A 35-year-old Caucasian male suffered orthostatic headache that developed to a bilateral abducens palsy. We ordered relative rest and the patient improved and completely recovered after 3 months. Although SIH is considered as a benign and self limited process it could also be associated with disabling complications. We should be aware of the possible complications and inform our patients. SIH can present with headache and bilateral abducens palsy even when the headache is improving

Osseointegrated total hip replacement connected to a lower limb prosthesis : a proof-of-concept study with three cases

Background- Osseointegrated implants are a suitable alternative for prosthetic attachment in individuals with a transfemoral amputation, who are unable to wear a socket. However, the small bone-implant contact area, reduced muscular leverage, and osteoporosis contraindicate osseointegrated implant use in transfemoral amputees with osteoporosis and a short residuum. We report on the feasibility of combining total hip replacement (THR) with an osseointegrated implant for prosthetic attachment. Methods- We retrospectively reviewed the cases of three transfemoral amputees who underwent osseointegration with THR between 2013 and 2014. In a two-stage procedure, a custom-made femoral prosthesis was connected to a THR with a modular revision stem and a stoma was created. Clinical outcomes were assessed at baseline and 1.5–2.5-year follow-up using standard measures of health-related quality of life, ambulation, and activity levels including the Short Form-36 (SF-36), Questionnaire for Transfemoral Amputees (Q-TFA), Timed Up and Go test, and 6-min walk test. Results- Patient age ranged from 35 to 65 years. There were no major adverse events, but there was one case of superficial infection. All patients showed improved Q-TFA and SF-36 scores. Two patients who were wheelchair-bound at baseline became community ambulators, and the third patient exhibited improved ambulation. Conclusions- This study demonstrated the feasibility of combining a THR with an osseointegrated implant in transfemoral amputees.7 page(s

Integrated analysis of transcriptomic and proteomic alterations in mouse models of ALS/FTD identify early metabolic adaptions with similarities to mitochondrial dysfunction disorders

Objective: Sporadic and familial amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disease that results in loss of motor neurons and, in some patients, associates with frontotemporal dementia (FTD). Apart from the accumulation of proteinaceous deposits, emerging literature indicates that aberrant mitochondrial bioenergetics may contribute to the onset and progression of ALS/FTD. Here we sought to investigate the pathophysiological signatures of mitochondrial dysfunction associated with ALS/FTD. Methods: By means of label-free mass spectrometry (MS) and mRNA sequencing (mRNA-seq), we report pre-symptomatic changes in the cortices of TDP-43 and FUS mutant mouse models. Using tissues from transgenic mouse models of mitochondrial diseases as a reference, we performed comparative analyses and extracted unique and common mitochondrial signatures that revealed neuroprotective compensatory mechanisms in response to early damage. Results: In this regard, upregulation of both Acyl-CoA Synthetase Long-Chain Family Member 3 (ACSL3) and mitochondrial tyrosyl-tRNA synthetase 2 (YARS2) were the most representative change in pre-symptomatic ALS/FTD tissues, suggesting that fatty acid beta-oxidation and mitochondrial protein translation are mechanisms of adaptation in response to ALS/FTD pathology. Conclusions: Together, our unbiased integrative analyses unveil novel molecular components that may influence mitochondrial homeostasis in the earliest phase of ALS