Masashi Hamada

Background: Dual antiplatelet therapy (DAPT) is more effective than aspirin alone in preventing early recurrences of non-cardioembolic strokes. In Japan, DAPT is often combined with argatroban, a direct thrombin inhibitor, for treating acute atherosclerotic stroke. However, the safety and effectiveness of this combination compared with those of DAPT alone remains unclear. Methods: We identified patients with acute atherothrombotic stroke treated with DAPT between April 2016 and March 2022, using the Diagnostic Procedure Combination database, a large national inpatient database in Japan. Patients were divided into two groups: those receiving DAPT plus argatroban and those receiving DAPT alone. We compared 7-day mortality, hemorrhagic complications, and readmission for stroke recurrence within 90 days between the groups using propensity-score overlap weighting analyses. Results: Of 59,983 eligible patients, 47,213 received DAPT plus argatroban, while 12,770 received DAPT alone. In the propensity-score overlap weighting analyses, no significant differences in 7-day mortality (0.2% vs 0.2%; adjusted risk ratio [aRR], 0.77; 95% confidence interval [CI], 0.49–1.21), the proportions of hemorrhagic complications (2.3% vs 2.1%; aRR, 1.12; 95% CI, 0.99–1.27), or 90-day readmission for stroke recurrence (1.2% vs 1.2%; aRR, 0.99; 95%CI, 0.84–1.17) were observed between the DAPT plus argatroban and DAPT alone groups. Conclusions: The outcomes did not differ significantly between DAPT alone and argatroban combined with DAPT for acute atherothrombotic stroke.

Purpose: Degenerative cervical myelopathy (DCM) and idiopathic normal pressure hydrocephalus (iNPH) share symptoms such as gait disturbance and urinary dysfunction, especially in the elderly. While the incidence of DCM in patients with iNPH is reported, the prevalence of iNPH in patients with DCM remains unclear. This study investigates the prevalence of iNPH in DCM patients. Methods: This retrospective cohort study included 83 patients undergoing decompression surgery for DCM between April 2018 and March 2023. Brain MRI performed within three years prior to surgery was analyzed. Patients were categorized into two groups based on the Evans Index (EI): those with EI > 0.3 were classified as the Suspected-iNPH group, and those with EI ≤ 0.3 as the Non-iNPH group. Pre- and postoperative Japanese Orthopedic Association (JOA) scores and Patient-Reported Outcomes (PROs), including the Neck Disability Index (NDI), EuroQOL-5D (EQ-5D), Core Outcome Measure Index (COMI), and Numerical Rating Scale (NRS) for pain, were compared. Suspected-iNPH cases underwent further analysis to identify disproportionately enlarged subarachnoid space hydrocephalus (DESH). Results: Thirty-one patients (37%) were classified as Suspected-iNPH, who were significantly older (p = 0.01). No significant differences were found in pre- or postoperative JOA scores, and PROs. DESH was identified in 6 patients (7%), with 2 diagnosed as probable-iNPH based on cerebrospinal fluid tap tests. Conclusions: A notable proportion of elderly DCM patients exhibit ventricular enlargement and DESH, suggesting a potential overlap with iNPH. Brain MRI should be considered for elderly DCM patients, particularly when neurological symptoms deviate from typical patterns.

Background: Myoclonus and other jerky movement disorders are hyperkinetic disorders, the diagnosis of which heavily relies on clinical neurophysiological testing. However, formal diagnostic criteria are lacking, and recently the utility and reliability of these tests have been questioned.Objective: The aim of this review was to assess the utilization of clinical neurophysiology testing to identify possible gaps and boundaries that might guide the development of new methods for a more precise diagnosis and in‐depth understanding of myoclonus.Methods: We reviewed electrophysiological features of cortical myoclonus, subcortical myoclonus (ie, myoclonus associated with dystonia, brainstem myoclonus), excessive startle reflex, spinal myoclonus (ie, spinal segmental and propriospinal myoclonus), peripheral myoclonus and mimics of myoclonus of peripheral origin (hemifacial spasm, minipolymyoclonus, myokymia), functional jerky movements, chorea, and tics.Results: Electrophysiological features that support the recognition of myoclonus subtypes, such as muscle burst duration, muscle pattern of activation, measures of cortical excitability, or movement‐related cortical potentials, have been identified. These significantly contribute to the diagnosis of jerky movement disorders, but their reliability is uncertain. Despite the significant advancements, several unresolved questions persist. Factors contributing to this include the absence of systematic neurophysiological assessment and standardized methods, alongside the limited number of patients investigated using these techniques.Conclusion: Although clinical neurophysiology remains the “gold standard” for defining and diagnosing myoclonus, our review highlighted the need to enhance the quality and reliability of neurophysiological testing in jerky movement disorders. Further studies including larger cohorts of patients recruited from different centers, employing standardized and optimized electrophysiological techniques, are warranted.

Neuronal ceroid lipofuscinosis type 2 (CLN2) is an autosomal recessive lysosomal disease caused by decreased activity of the enzyme tripeptidyl peptidase 1 (TPP1) due to pathogenic variants in the TPP1 gene. Cerliponase alfa, a recombinant proenzyme form of TPP1, has shown efficacy in preventing motor and language function decline in early-stage CLN2. However, the safety and effects of this therapy in advanced-stage CLN2 are unclear. We herein report a case of intraventricular cerliponase alfa treatment for over a year in a patient with advanced-stage CLN2. The results suggest the safety and potential efficacy of treatment at an advanced stage of CLN2.