Mast Syndrome Outside the Amish Community: SPG21 in Europe

Background:Mast syndrome is a rare disorder belonging to the group of hereditary spastic paraplegias (HSPs). It is caused by bi-allelic mutations in the ACP33 gene, and is originally described in Old Order Amish. Outside this population, only one Japanese and one Italian family have been reported. Herein, we describe five subjects from the first three SPG21 families of German and Austrian descent.Methods:Five subjects with complicated HSP were referred to our centers. The workup consisted of neurological examination, neurophysiological and neuropsychological assessments, MRI, and genetic testing.Results:Onset varied from child- to adulthood. All patients exhibited predominant spastic para- or tetraparesis with positive pyramidal signs, pronounced cognitive impairment, ataxia, and extrapyramidal signs. Neurophysiological workup showed abnormal motor and sensory evoked potentials in all the patients. Sensorimotor axonal neuropathy was present in one patient. Imaging exhibited thin corpus callosum and global brain atrophy. Genetic testing revealed one heterozygous compound and two homozygous mutations in the ACP33 gene.Conclusion:Herein, we report the first three Austrian and two German patients with SPG21, presenting a detailed description of their clinical phenotype and disease course. Our report adds to the knowledge of this extremely rare disorder, and highlights that SPG21 must also be considered in the differential diagnosis of complicated HSP outside the Amish community.

Religious Education Policy in Public Schools During the New Order

The new order government policy about religious education in state schools gradually led to an accommodative policy. If the old order government made religious education a facultative subject, then at the beginning of the new order the government strengthened the position of religious education by removing facultative provisions, even though they were not obligatory. Until finally, through Law number 2 (1989), the government required religious education to be taught in all channels, types, and levels of education. And the right of every student to get religious lessons by their religion and is taught by religious educators. This research includes historical research and policy studies, because it relies on past data, whose steps consist of four main activities, namely heuristics, verification, interpretation, and historiography. This accommodative policy is influenced by several factors, including; the government’s determination to implement Pancasila purely and consistently after it was diverted in the old order era; the weakening of the government’s power at that time so that it required the political support of muslims as the majority population; There is an intellectual transformation of muslim thinkers and political activists towards harmonious and complementary political relations between Islam and the state so that Islamic ideas are more easily accepted; and muslims experience a process of rapid social, educational, economic and political mobilization and so that more and more people are involved in government and can influence policies in a pro-muslim direction.

The Authoritarian Temptation, and Its Radical Left Expressions

No political regime can be entirely immune from authoritarian temptations. This article focuses on the distinctive sources and dynamics that apply to post-revolutionary regimes. To prevail in bringing about radical and irreversible change they will require an effective security apparatus that overcomes the backlash that will arise from the previous order. These security requirements provide the first source of authoritarian temptation, but there are three more. Once the regime is firmly established the new rulers can choose what restraints on their conduct to accept. It is tempting to dispense with healthy channels of feedback. Moreover, even the most successful of revolutionary regimes polarise opinion between the old order and the new. And when material hardships arise loyalty may be rewarded above market rationality. In conjunction these amount to a serious set of authoritarian temptations. But there are also some countervailing considerations. A durably successful radical regime must counterbalance the requirements for unity and discipline against the need for creativity and adaptability. Initial emancipatory ambitions may be updated and renewed in order to inspire future generations and legitimise the revolutionary process. Such regimes can seesaw between authoritarian and empowering tendencies, rather than relying on repression alone to keep them in existence. Their legitimation strategy will contain three main components: i) reaffirming and updating their emancipatory origins; ii) downplaying/excusing any authoritarian “deviations”; iii) projecting future prospects for inclusionary development.

The Tsunami Moment

This chapter chronicles the populist candidate’s rise to power, which follows a pattern resembling exponential growth: it starts slowly and grows steadily until it reaches an inflection point that marks an unstoppable moment. The term tsunami describes the first moment in the dynamic theory of populism in power, when insurgent populist candidates are able to tap into the existing mass dissatisfaction with political institutions and take advantage of growing elite disarray. As they become significant contenders, their momentum increases; seen as potential winners by others, they generate bandwagon effects and fascinate the media. These aspiring populist leaders expand their appeal through different ways. Some politicians associated with the old order bet on them and offer their support. Populist candidates expand the electorate by politically activating new actors or social forces, or by mobilizing previously apathetic voters.

Sekartaji Mask Ballet in Surakarta

ABSTRAKSendratari Topeng Sekartaji merupakan tari tradisi garapan baru yang diilhami oleh Serat Panji yang ditulis pada Jaman Jenggala Kediri abad XI-XII. Tujuan penelitian Sendratari Topeng Sekartaji adalah untuk menggali kreativitas tari di sekitar Surakarta serta menemukan bentuk dan elemen-elemen pendukungnya. Metode penelitian yang digunakan meliputi pengumpulan data dengan tekhnik observasi, wawancara dan dokumentasi. Pengolahan data dilakukan dengan tekhnik analisis bentuk dan struktur. Evaluasi validitas data dengan tekhnik trianggulasi. Penelitian menggunakan pendekatan antropologi seni yang beranggapan bahwa karya seni merupakan ekspresi karya seniman. Hasil penelitian menunjukan Sendratari Topeng Sekartaji merupakan karya tari kelompok bertema percintaan sebagai ungkapan kreativitas seni yang popular pada zaman akhir orde lama dan awal orde baru. Sendratari dalam kurun waktu, dipentaskan dengan berbagai variasi sesuai dengan pendukung, situasi dan kondisi serta waktu yang disediakan. Bentuk kreativitas seni dapat berupa frahmen, pethilan, tari tunggal dan yang paling utama adalah sendratari. Tata urutan sajian tergantung pada bentuk pertunjukanya. Gerak tari pada tokoh antagonis berupa gagahan gaya Surakarta, tokoh protagonis dengan alus luruh, sedang tari putri dengan putri luruh. Pada akhir pertunjukan selalu bermuara kepada keberhasilan hubungan cinta kasih. Kunci: sendratari, topeng, sekartaji, Surakarta.ABSTRACTThis research entitled “Sendratari Topeng Sekartaji” proposed exploring dance creativity in Surakarta and finding its formats and support elements. This research used data collection with techniques such as observation, interview and documentation as the research method. Data is processed by the technique of format and structure analysis. The technique of triangulation is used as a technique to evaluate data. This research used an art anthropological approach. It is assumed that an artwork is an expression of an artist’s work. Research results showed that Sendratari Topeng Sekartaji is a colossal dance with a romance theme. It was a representation of artwork that has been popularized since the end of the old order and early new order. During that time, sendratari has presented narratively, according to situation, condition and support elements. Art creativity manifested into fragment “pethilan”, single dance and especially “sendratari”. Its arrangement for presentation depends on its performance format. They have “gagahan” in Surakarta style for their dance moves, “alus luruh” and “putri lurah” for antagonist, protagonist, and female dance, irrespectively. The last performance always refers to romantic relationship successfully.Keywords:Sendratari Topeng; Sekartaji; Surakarta

LITERACY AND IDENTITY: FROM LIM KIM HOK, AND KHO PING HO TO THE CONTEMPORARY INDONESIAN LITERATURE

The history and progress of Indonesian literature become a piece of authentic evidence for ethnic Chinese. Both of them can exert influence on contemporary Indonesian literature at large. It can be seen in the history of Indonesian literacy, romance, martial arts stories (Cerita silat), and Chinese literary writing styles which are still used nowadays. This shows how ethnic Chinese education and Hybrid (Malay-Chinese) in Indonesia have developed rapidly. Before this country was formed into a nation-state with cultural institutions, ethnic Chinese had undergone literary expressions. Moreover, culture at that time was widely accepted for Malay nations, Java, Balinese, etc. Conversely, many identity challenges and contestation during Dutch colonial, Old Order, and New Order pressed the ethnic Chinese in various ways. In addition, this can be understood as a part of cultural exchange. Hence, Indonesian literature is currently strongly influenced by the contact of Malay and Chinese literature.

Webinar Bagi Mahasiswa Baru 2021 "Sejarah dan Pemikiran H.O.S. Cokroaminoto"

This Community Service, is carried out virtually in the form of online or webinars. This activity was organized by the Campus Academic Introduction committee, Cokroaminoto University of Yogyakarta in 2021. This webinar is a routine activity held every year, to introduce a campus environment for new students every year. This activity raised the theme "Historyand Thought of Cokroaminoto", which was carried out using a zoom platform. The purpose of this webinars to get to know more closely the famous figure as well as the nation's teacher, H.O.S. Cokroaminoto, which gave the idea of Indonesia should be independent since 1912. Cokroaminoto helped raise the first national movement organization in Indonesia, the Islamic Trading Company which was established in 1905 with the founding figure H. Samanhudi. Cokroaminoto holds the title of teacher of the nation, because he has 3 famous students: Sukarno, Musso and Kartosuwiryo, all three of whom have different ideologies: Nationalists, Communists and Religious, as well as his three disciples also managed to color the journey of this nation, even more so during the old order. In the implementation of this webinar, it appears that the participation, enthusiasm and spirit of the participants, so that this webinar activity can be carried out properly and smoothly.

Horizontalism in the implementation of land redistribution program in Cipari Cilacap Indonesia

This study explains the dynamics of non-state actors’ involvement in implementing a land redistribution program in Cipari, Cilacap, Indonesia. The urgency of this study is the massive involvement of non-state actors in the implementation of land redistribution programs while lacking analysis found for the dimension of horizontalism in the implementation of public policies, especially related to the land redistribution program as a part of land reform policy programs in Indonesia. This study focuses on the involvement of non-state actors, the role of Street-Level Bureaucrats (SLBs), and the relationship between SLBs and Local Farmers Organizations (LFO). This study implemented a qualitative approach with a case study strategy. The key informants consisted of village heads, village officials, LFO administrators, and administrators of LFO advocate organizations. We performed observation, in-depth interviews, and documentation reviews for data collection and implemented thematic data analysis. Three main findings include the non-state actors’ involvements since the colonial era (pre-independence), the old order, the new order, to post-reformation, the role of SLBs as state actors dominating the implementation of the land redistribution program, and the negotiation relationship between SLBs and LFO showing the SLBs as the winner.

The History of Moslem Intellectuals: A Case Study at UIn Syarif Hidayatullah Jakarta

This study aimed to explain that the intellectual community who studied at and was born from the state Institute of Islamic Studies Syarif Hidayatullah (IAIN Jakarta) in the 1970s to 1985 were the second-generation intellectuals who had their own characteristics. As the study was library research, the data collection was done by examining deeply the written documents or literatures that were relevant with the problems being investigated. The obtained data were then qualitatively analyzed by comparing and connecting the existing variables; and inferences were drawn from the findings to get an ideal concept of building a good national and state life based on the Islamic substantial values. The results of the study showed that second generation intellectuals became international intellectuals as Islamic historians in Southeast Asia, theologians, political or socio-economic analysts, and activists of Islamic movements. The study also pointed out that it was not only the first generation that made the second generation successful in their career and studies, but many factors on and off campus contributed significantly, and affect the next generations continuously from time to time. Evidently, in their development, discussion activities of intra-or extra campus organizations, talked not only about Islam but also political issues, such as the old order government policies. The study concluded that the second generation of IAIN Jakarta Intellectual community played many roles in Islamic studies in national and international level. It suggested that studies on the same topis with different points of views are still necessary to conduct.

Traditional Farming Lifestyle in Old Older Mennonites Modulates Human Milk Composition

BackgroundIn addition to farming exposures in childhood, maternal farming exposures provide strong protection against allergic disease in their children; however, the effect of farming lifestyle on human milk (HM) composition is unknown.ObjectiveThis study aims to characterize the maternal immune effects of Old Order Mennonite (OOM) traditional farming lifestyle when compared with Rochester (ROC) families at higher risk for asthma and allergic diseases using HM as a proxy.MethodsHM samples collected at median 2 months of lactation from 52 OOM and 29 ROC mothers were assayed for IgA1 and IgA2 antibodies, cytokines, endotoxin, HM oligosaccharides (HMOs), and targeted fatty acid (FA) metabolites. Development of early childhood atopic diseases in children by 3 years of age was assessed. In addition to group comparisons, systems level network analysis was performed to identify communities of multiple HM factors in ROC and OOM lifestyle.ResultsHM contains IgA1 and IgA2 antibodies broadly recognizing food, inhalant, and bacterial antigens. OOM HM has significantly higher levels of IgA to peanut, ovalbumin, dust mites, and Streptococcus equii as well TGF-β2, and IFN-λ3. A strong correlation occurred between maternal antibiotic use and levels of several HMOs. Path-based analysis of HMOs shows lower activity in the path involving lactoneohexaose (LNH) in the OOM as well as higher levels of lacto-N-neotetraose (LNnT) and two long-chain FAs C-18OH (stearic acid) and C-23OH (tricosanoic acid) compared with Rochester HM. OOM and Rochester milk formed five different clusters, e.g., butyrate production was associated with Prevotellaceae, Veillonellaceae, and Micrococcaceae cluster. Development of atopic disease in early childhood was more common in Rochester and associated with lower levels of total IgA, IgA2 to dust mite, as well as of TSLP.ConclusionTraditional, agrarian lifestyle, and antibiotic use are strong regulators of maternally derived immune and metabolic factors, which may have downstream implications for postnatal developmental programming of infant’s gut microbiome and immune system.