The pioneers of neurology in Japan were professors Hiroshi Kawahara and Kinnosuke Miura. Kawahara published the first description of progressive bulbar palsy and wrote the first neurology textbook in Japan. Miura, on the other hand, published studies about amyotrophic lateral sclerosis, in addition to participating in the founding of the Japanese Society of Neurology. The influence of European neurology, particularly French and German, in the figures of Professor Jean-Martin Charcot and Professor Erwin Bälz, was fundamental in the consolidation of neurology in Japan.

The movement disorder known as hemifacial spasm is characterized by involuntary contractions of the muscles that are innervated by the facial nerve. The treatment of choice for this condition is botulinum toxin injections. To analyze the botulinum toxin dosage in patients undergoing treatment for hemifacial spasm during a 14-year period. A retrospective study of medical records from patients treated at the Neurology Service of Hospital Universitário Onofre Lopes, Universidade Federal do Rio Grande do Norte, from 2010 to 2024, was performed. A total of 151 patients met the inclusion criteria. The dose of botulinum toxin revealed a statistically significant increase during the first 3.46 years of follow-up. In the long-term, a trend toward dose stabilization was identified. The median latency for the onset of effect was 4 days, while the median duration of effect was 3 months. All side effects were temporary, with the most common being hemifacial weakness (17.9%) and palpebral ptosis (3.3%). Most patients presented primary hemifacial spasm (88.1%), with a neurovascular conflict identified in 24.1% of cases. The increase in botulinum toxin dosage during the first years may be explained by dosage adjustment to control hemifacial spasm with the lowest possible doses. A prolonged interval between applications may also be associated with this increase. Dose stabilization tends to be achieved over time, indicating disease control.

Parkinson's disease (PD) is a degenerative, progressive, chronic disease that mainly affects the central nervous system, caused by dopamine deficiency. One of the ways to evaluate the central nervous system is with auditory evoked potentials (AEP). To characterize the audiometric responses, and the auditory brainstem response (ABR), and cortical auditory evoked potentials (CAEP) in individuals with PD. Thirty-two patients aged between 40 and 81 of both sexes were assessed, 16 with PD (study group [SG]) and 16 without PD (control group [CG]) matched for sex and age. The subjects were assessed using pure tone audiometry, ABR with click stimuli, and CAEP using the oddball paradigm with tone burst and speech stimuli. The results were compared between the groups using a repeated measures analysis of variance (ANOVA) test. In pure-tone audiometry, significantly higher hearing thresholds were found in the SG at 6 and 8 kHz. For the ABR, no differences were observed between groups. The CAEP analysis did not find statistical differences in the latencies between the groups, however, the SG presented smaller amplitudes of P1-N1, P2-N2, and N2-P3 than the CG. The results of this study showed a significantly higher threshold in higher frequencies in PD. Although no differences were observed at the brainstem level, the decrease in amplitude of all components in patients with PD in the CAEP suggests a deficit in both automatic and attentional cortical processing of acoustic stimuli.

Tafamidis is a kinetic stabilizer that binds to the transthyretin (TTR) gene, inhibiting its dissociation. It is the only disease-modifying treatment for hereditary TTR amyloidosis with peripheral neuropathy (ATTRv-PN) available in the National Therapeutic Form (Formulário Terapêutico Nacional, FTN, in Portuguese) of the Brazilian Unified Health System (Sistema Único de Saúde, SUS, in Portuguese). To assess if the efficacy and safety of tafamidis in the Brazilian real-world experience are comparable to the results of clinical trials. We retrospectively studied all patients with ATTRv-PN evaluated at our center from September 2011 to March 2022 (data cut-off) who were initiated on tafamidis and had at least 1 follow up visit 6 months after the initiation of the drug treatment. Neurologic and functional outcomes were compared from day 1 (D1) of the tafamidis treatment (baseline) to the last follow-up. In total, 33 patients were included, 18 (55%) of whom were female. All patients were carriers of the V30M mutation of ATTRv-PN, and 20 (61%) presented early onset (EO) ATTRv-PN. At baseline, the median age of the sample was of 40 (interquartile range [IQR]: 36-68) years, the median Neuropathy Impairment Score (NIS) was of 10 (6-24) points, and the median body mass index (BMI) was of 26 (23-28) kg/m2. The mean follow-up time was of 3.4 years. At the last follow-up, the BMI, the neurological impairment, and the level of disability slightly worsened compared with baseline, while the findings of the nerve conduction studies remained stable. These same results were observed across EO and late-onset (LO) ATTRv-PN patients. A total of 25 (75.8%) patients were considered responders, and 8 (24.2%), non-responders. The efficacy and safety of tafamidis reported in clinical trials is expandable to the Brazilian real-world scenario in EO and late-onset (LO) ATTRv-PN.

Alzheimer's disease (AD), diabetic cognitive impairment (DCI), and vascular dementia (VD) are considered the most common causes of severe cognitive impairment in clinical practice. Numerous factors can influence their progression, and many studies have recently revealed that metabolic disorders play crucial roles in the progression of cognitive impairment. Mounting evidence indicate that the regulation of lipid metabolism is a major factor in maintaining brain homeostasis. Generally, abnormalities in lipid metabolism can affect amyloid-beta (Aβ) deposition, tau hyperphosphorylation, and insulin resistance through lipid metabolic signaling cascades; affect the neuronal membrane structure, neurotransmitter synthesis and release; and promote synapse growth, which can impact neural signal transmission and exacerbate disease progression in individuals with cognitive impairment, including AD, DCI, and VD. Moreover, apolipoprotein E (APOE), a key protein in lipid transport, is involved in the occurrence and development of the aforementioned diseases by regulating lipid metabolism. The present article mainly discusses how lipid metabolic disorders in the brain microenvironment are involved in regulating the progression of cognitive impairment, and it explores the regulatory effects of targeting the key lipid transport protein APOE in the context of the role of lipid metabolism in the common pathogenesis of three diseases-Aβ deposition, tau hyperphosphorylation, and insulin resistance-which will help elucidate the potential of targeting lipid metabolism for the treatment of cognitive impairment.