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Excessive stride variability is a characteristic feature of cerebellar ataxias, even in pre-ataxic or prodromal disease stages. This study explores the relation of variability of arm swing and trunk deflection in relationship to stride length and gait speed in previously described cohorts of cerebellar disease and healthy elderly: we examined 10 patients with spinocerebellar ataxia type 14 (SCA), 12 patients with essential tremor (ET), and 67 healthy elderly (HE). Using inertial sensors, recordings of gait performance were conducted at different subjective walking speeds to delineate gait parameters and respective coefficients of variability (CoV). Comparisons across cohorts and walking speed categories revealed slower stride velocities in SCA and ET patients compared to HE, which was paralleled by reduced arm swing range of motion (RoM), peak velocity, and increased CoV of stride length, while no group differences were found for trunk deflections and their variability. Larger arm swing RoM, peak velocity, and stride length were predicted by higher gait velocity in all cohorts. Lower gait velocity predicted higher CoV values of trunk sagittal and horizontal deflections, as well as arm swing and stride length in ET and SCA patients, but not in HE. These findings highlight the role of arm movements in ataxic gait and the impact of gait velocity on variability, which are essential for defining disease manifestation and disease-related changes in longitudinal observations.

Cerebellar mutism syndrome (CMS) is a serious complication of posterior fossa surgeries affecting mainly pediatric age group. The pathophysiology is still not fully understood. It adversely affects the recovery of patients. There is no definitive and standardized management for CMS. However pharmacological therapy has been used in reported cases with variable effectiveness. We aim through this review to summarize the available evidence on pharmacological agents used to treat CMS.
A thorough systematic review until December 2022, was conducted using PubMed Central, Embase, and Web of Science, databases to identify case reports and case series of CMS patients who underwent posterior fossa surgery and received pharmacological treatment. Patients with pathologies other than posterior fossa lesions were excluded from the study.
Of 592 initial studies, 8 studies met our eligibility criteria for inclusion, with 3 more studies were added through manual search; reporting on 13 patients. The median age of 13 years (Standard deviation SD=10.60). The most frequent agent used was Bromocriptine. Other agents were fluoxetine, midazolam, zolpidem, and arpiprazole. Most patients recovered within 48 hours of initiating medical therapy. The median follow-up period was 4 months (SD=13.8). All patients showed complete recovery at the end of follow-up period.
Cerebellar mutism syndrome is reported after posterior fossa surgeries, despite attempts to identify risk factors, pathophysiology, and management of CMS, it remains a challenging condition with significant morbidity. Different Pharmacological treatments have been proposed with promising results. Further studies and formalized clinical trials are needed to evaluate available options and their effectiveness.

Hashimoto encephalopathy presents with a myriad of neuropsychiatric features in the background of elevated antithyroid antibodies and it may or may not be associated with Hashimoto thyroiditis. It is a diagnosis of exclusion. Here, we present the case of a hypothyroid woman in her 30s, with a 5-year history of chronic progressive gait ataxia along with hand and head tremor, inattention and electroencephalogram (EEG) suggestive of interictal epileptiform discharges without any clinical seizures. The patient had very high titres of anti-thyroid peroxidase antibodies >2000 IU/mL and was on very high-dose levothyroxine replacement therapy. She responded to intravenous pulse corticosteroids. Improvement was noted both clinically and on subsequent EEGs. Pure cerebellar syndrome without frank encephalopathy can also be a rare presentation of Hashimoto encephalopathy. This highlights the importance of antithyroid antibodies testing even in cases of pure cerebellar syndrome to rule out Hashimoto encephalopathy associated ataxia.

Bevezetés: A cerebelláris kognitív-affektív szindróma olyan tünetegyüttest jelent, amely a motoros és végrehajtó funkciók zavarán túl a memória, a váltás-gátlás, a konceptualizálás és az érzelem és viselkedés szabályozásában is megnyilvánulhat. A szindróma felmérésére dedikált magyar nyelvű mérőeszköz eddig nem állt a hazai szakemberek rendelkezésére. Célkitűzés: A kutatás célja volt a Cerebelláris Kognitív-Affektív Szindróma Skála (CCAS-H) magyar mintán történő validációja, diagnosztikus érvényességének felmérése, valamint a teszt közzététele a szakemberek számára. Módszer: Multicentrikus kutatási elrendezésben, hat kórházi osztállyal való együttműködés keretében cerebelláris érintettségű páciensekkel és kontrollszemélyekkel történtek tesztfelvételek 2021. 07. 12. és 2023. 12. 31. között. A statisztikai elemzések során normalitástesztelést, leíró statisztikát, átlagok tesztelését, korrelációszámításokat, valamint ROC-elemzést végeztünk, Cronbach-alfa-értékeket, Cohen-féle kappát határoztunk meg. A normalitás sérülése esetén robusztus próbákat választottunk. Eredmények: 54 cerebelláris és 40 kontrollszeméllyel történt tesztfelvétel, amelyekből különböző alcsoportokat képeztünk. Mintánkban az életkor (r = –0,581***, df = 87, p<0,001) és az iskolázottság (r = 0,360***, df = 87, p<0,001) szignifikáns együttjárást mutatott az összpontszámokkal. A CCAS-H megfelelő belső konzisztenciájú (α = 0,771), teszt-reteszt vizsgálat alapján stabil (r = 0,793***, df =13, p<0,001), és megítélők közti 88,2%-os egyetértést mutatott (𝜅 = 0,779, z = 4,79, p<0,001). A fals pozitív esetek csökkentése érdekében a szindrómát előre jelző vágópontokat megemeltük, így 5 hibapontnál felmerül (szenzitivitás: 100%, specificitás: 43,48%), 6 hibapontnál valószínű (szenzitivitás: 90,91%, specificitás: 62,32%), 7 hibapontnál határozott (szenzitivitás: 81,82%, specificitás: 73,91%) a szindróma előfordulásának valószínűsége (AUC: 0,836). Megbeszélés: Az elemzések a nemzetközi ajánlásoknak megfelelően készültek. Az eredeti és más validált változatokhoz képest a magyar verzió nagyobb belső konzisztenciát mutatott. A skála stabil és megbízhatóan alkalmazható, emellett ismertetjük a teszt adta újabb kutatási kérdéseket. Következtetés: Az újonnan validált skála alkalmas mérőeszköz a szindróma mérésére. Tanulmányunkkal elérhetővé tettük a CCAS-H-t a magyar szakemberek számára. Orv Hetil. 2024; 165(20): 785–798.

The shrimp sign is characterized by a well-defined lesion in the deep cerebellar white matter, with hyperintense signal on T2- and hypointense signal on T1-weighted imaging, abutting and outlining the dentate nucleus, unilaterally or bilaterally. This sign has high sensitivity and specificity for cerebellar progressive multifocal leukoencephalopathy (PML) within the correct clinical scenario. In this article, we present a case of cerebellar PML in a woman living with human immunodeficiency virus, who was not using antiretroviral therapy, and presented the shrimp sign on brain MRI.

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患儿，男，1岁1个月，主诉\"转氨酶反复升高6月余\"。主要临床表现为整体发育迟缓，精神运动发育落后，小头畸形，肌张力低；实验室检查表现为反复肝功能异常，铜蓝蛋白降低；头颅影像学显示胼胝体薄，脑室系统扩大，脑沟裂池变深、增宽；肝脏病理表现为肝细胞轻度小泡性脂肪变性伴肝纤维化；基因检测结果提示空泡蛋白分选相关蛋白51基因变异。.

Cerebellar strokes induce coordination disorders that can affect activities of daily living. Evidence-based neurorehabilitation programs are founded on motor learning principles. The cerebellum is a key neural structure in motor learning. It is unknown whether and how well chronic cerebellar stroke individuals (CCSIs) can learn to coordinate their upper limbs through bimanual motor skill learning. The aim was to determine whether CCSIs could achieve bimanual skill learning through a serious game with the REAplan® robot and to compare CCSIs with healthy individuals (HIs). Over three consecutive days, sixteen CCSIs and eighteen HIs were trained on an asymmetric bimanual coordination task (\"CIRCUIT\" game) with the REAplan® robot, allowing quantification of speed, accuracy and coordination. The primary outcomes were the bimanual speed/accuracy trade-off (BiSAT) and bimanual coordination factor (BiCo). They were also evaluated on a bimanual REACHING task on Days 1 and 3. Correlation analyses between the robotic outcomes and clinical scale scores were computed. Throughout the sessions, BiSAT and BiCo improved during the CIRCUIT task in both HIs and CCSIs. On Day 3, HIs and CCSIs showed generalization of BiSAT, BiCo and transferred to the REACHING task. There was no significant between-group difference in progression. Four CCSIs and two HIs were categorized as \"poor learners\" according to BiSAT and/or BiCo. Increasing age correlated with reduced BiSAT but not BiCo progression. Over three days of training, HIs and CCSIs improved, retained, generalized and transferred a coordinated bimanual skill. There was no between-group difference, suggesting plastic compensation in CCSIs. Clinical trial NCT04642599 approved the 24th of November 2020.