Serial MRI Features of Canine GM1 Gangliosidosis: A Possible Imaging Biomarker for Diagnosis and Progression of the Disease

GM1 gangliosidosis is a fatal neurodegenerative lysosomal storage disease caused by an autosomal recessively inherited deficiency of β-galactosidase activity. Effective therapies need to be developed to treat the disease. In Shiba Inu dogs, one of the canine GM1 gangliosidosis models, neurological signs of the disease, including ataxia, start at approximately 5 months of age and progress until the terminal stage at 12 to 15 months of age. In the present study, serial MR images were taken of an affected dog from a model colony of GM1 gangliosidosis and 4 sporadic clinical cases demonstrating the same mutation in order to characterize the MRI features of this canine GM1 gangliosidosis. By 2 months of age at the latest and persisting until the terminal stage of the disease, the MR findings consistently displayed diffuse hyperintensity in the white matter of the entire cerebrum on T2-weighted images. In addition, brain atrophy manifested at 9 months of age and progressed thereafter. Although a definitive diagnosis depends on biochemical and genetic analyses, these MR characteristics could serve as a diagnostic marker in suspect animals with or without neurological signs. Furthermore, serial changes in MR images could be used as a biomarker to noninvasively monitor the efficacy of newly developed therapeutic strategies

ハイヨウセイ キンイシュク ニ エイキョウ オ アタエル インシ ト ソノ エイキョウ

ベッドレストによる廃用性筋萎縮を予防し, 身体機能の低下を最小限に留める方法・手段を考え知ることは, 理学療法士という対象者の身体機能の向上を目的とする専門職に就く上で必須である. ベッドレストに伴う筋萎縮の発生機序・原因を, 内分泌系, 栄養管理, 筋線維の解剖学的・生理学的知識, 筋を支配する神経系の廃用といった多様な観点から理解し, 筋萎縮予防策を想起することとした. 筋線維における解剖学的・生理学的知識を整理しつつ, 筋を支配する神経系の廃用予防, 内分泌系に対するアプローチ, 筋萎縮と栄養面との関係性, 有効なトレーニング方法について, 各書籍・過去の先行研究に基づき再考した. 結果として, ベッドレスト患者の筋力低下に対してアプローチする際には, 筋力増強訓練の方法・負荷量, 対象者の栄養状態・睡眠状態を加味し, 内分泌による筋タンパクの異化・同化の相互性を考慮する必要性が示唆された. 【I はじめに】 筋力増強・筋肥大に関するトレーニング方法はメディア・書籍等で多く紹介されている.Physical Therapists are professional who are improvement of the functional handicap. Then it is indispensable to know the method and means which prevents a bed-rest patient\u27s disuse muscle atrophy and stops less the physical function as low as we can. We tried to understand a cause and a process with internal secretion system, nutrition, anatomy and physiology of muscle, atrophy of nervous system. Also we suggested how prevent the muscle atrophy and training menu with the past books and go ahead of research. Lastly, let us comment on the way of the approach to less muscle. We suggested that it is necessary to consider a method of muscle training, power of resistance, patient\u27s state of nutritional and sleep. In addition, we have to reflect on a correlation of dissimilation and assimilation of muscle protein by internal secretion system

Head Tilting Elicited by Head Turning in Three Dogs with Hypoplastic Cerebellar Nodulus and Ventral Uvula

The nodulus and ventral uvula (NU) of the cerebellum play a major role in vestibular function in humans and experimental animals; however, there is almost no information about NU function in the veterinary clinical literature. In this report, we describe three canine cases diagnosed with presumptive NU hypoplasia. Of them, one adult dog presented with cervical intervertebral disk disease, and two juvenile dogs presented with signs of central vestibular disease. Interestingly, an unusual and possibly overlooked neurological sign that we called positioning head tilt was observed in these dogs. The dogs were able to turn freely in any direction at will. The head was in a level position when static or when the dog walked in a straight line. However, the head was tilted to the opposite side when the dog turned. Veterinary clinicians should be aware of this neurological sign, which has not been reported previously, and its application in lesion localization in dogs

Impact of vehicle electrification on global supply chains and emission transfer

Numerous countries plan to promote increased levels of vehicle electrification. This study demonstrates that, when considering the life cycle of automobiles, higher levels of vehicle electrification over the next 30 years in Japan would generate higher carbon emissions, preventing the country from meeting carbon reduction and neutrality targets in 2030 and 2050, respectively. In 2020, 2030, and 2050, domestic emissions could decrease to 92.5, 72.9, and 49.6 Mt, respectively, while emission transfers to other countries could reach 12.1 Mt (11.5% of the global carbon footprint), 10.4 Mt (12.5%), and 8.7 Mt (14.9%). The results indicate that even if the widespread use of alternative fuel vehicles could reduce domestic emissions, such emissions might be transferred to foreign countries, and blanket promotion of automobile electrification should be avoided. Instead, governments and the automotive industry should take responsibility for global and whole life-cycle emissions of vehicles, not only domestic tank-to-wheel emissions. These results provide baseline information for use in the recommendation measures and international rules to be adopted by the automobile industry stakeholders and policymakers

Positioning head tilt observed in two cats with myasthenia gravis

Case series summary A 1-year-old castrated male domestic shorthair cat was suspected with myasthenia gravis (MG) based on neurological examination, complete blood count (CBC), serum biochemistry, radiography and electrophysiological examination. In addition, a 9-year-old spayed female domestic shorthair cat was diagnosed with MG based on neurological examination, CBC, serum biochemistry, radiography, ultrasonography and increased acetylcholine receptor antibody titre. Positioning head tilt (PHT) was observed at the time of diagnosis in both cats. Relevance and novel information Although the pathophysiology of PHT in cats is not fully understood, the mechanism for PHT in cats with MG may be similar to that of cats with hypokalaemic myopathy, supporting our hypothesis that muscle spindle dysfunction causes PHT

Case of a miniature dachshund with a primitive neuroectodermal tumor confined to the forebrain region treated with a combination of surgery and chemotherapy

A miniature dachshund aged 9 years and 7 months with a history of polyuria/polydipsia and depression was referred. General physical and neurological examinations revealed no obvious abnormalities. MRI of the brain revealed a large space-occupying lesion in the left frontal lobe. This was surgically removed and pathologically diagnosed as a primitive neuroectodermal tumor (PNET). Although the clinical signs had been improved, follow-up MRI revealed recurrence of the tumor. Lomustine was administered, but 1 year after surgery, the dog exhibited cluster seizures and died. This is the first reported case of a dog with PNET confined to the forebrain region treated by surgical resection in combination with chemotherapy, as observed by repeated follow-up MRI