Diagnosis, Treatment, and Follow-Up of Giant-Cell Arteritis: A Retrospective Multicenter Study.

BACKGROUND AND PURPOSE: Giant-cell arteritis (GCA) is the most common type of vasculitis in the elderly and is associated with high risks of visual loss and recurrence. Owing to its rarity in Asian populations, the current clinical interventions for these patients are not well known. Here we aimed to characterize the current management status of patients with GCA using Korean multicenter data. METHODS: This retrospective study analyzed medical records of patients with GCA at six Korean university hospitals from February 2009 to November 2022. GCA had originally been diagnosed based on the 1990 American College of Rheumatology (ACR) criteria, and cases were selected for inclusion in this study based on the 2022 ACR/European Alliance of Associations for Rheumatology criteria. We evaluated treatments, follow-up periods, and outcomes (relapse, remission, and adverse drug reactions) in patients with GCA with or without arteritic anterior ischemic optic neuropathy (AAION). RESULTS: This study analyzed 18 patients with a median age of 75.5 years that included 12 females (66.7%). Seven patients (38.8%) had AAION. All patients initially received prednisolone treatment, while four (22.2%) underwent adjuvant treatment with methotrexate and azathioprine during prednisolone tapering. During the median follow-up of 3.5 months (interquartile range: 2.0-23.2 months), 4 patients (22.2%) had prednisolone-related adverse reactions, 2 (11.1%) relapsed, and 13 (72.3%) dropped out. Nine patients (50.0%) experienced remission, with this being sustained in four (36.4%). CONCLUSIONS: This study observed high dropout rates and short follow-ups. Adverse effects of prednisolone were common, and relapses occurred in approximately one-tenth of Korean patients with GCA. Thus, optimizing GCA treatment necessitates regular monitoring and long-term follow-up.

SUNCT, SUNA and short-lasting unilateral neuralgiform headache attacks: Debates and an update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA. METHODS: We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases. RESULTS: Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases. CONCLUSIONS: Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.

Preventive therapy with galcanezumab for two consecutive cluster bouts in patients with episodic cluster headache: an observational multicenter study.

BACKGROUND: Cluster headache is a severe and disabling primary headache disorder. Galcanezumab is a monoclonal antibody against calcitonin gene-related peptide and a preventive therapy for episodic cluster headache. However, the approval and insurance coverage for episodic cluster headache differ in each country. Additionally, the consistency of efficacy of galcanezumab therapy has not yet been evaluated. This study aimed to assess the efficacy and safety of 240 mg of galcanezumab therapy for consecutive cluster bouts in patients with episodic cluster headache. METHODS: The study enrolled patients with episodic cluster headache who received two courses of galcanezumab therapy at three university hospitals in Republic of Korea between February 2020 and April 2022. The efficacy and safety of galcanezumab were analyzed by comparing daily headache frequency, the number of headache days, and headache intensity and adverse effects during the one-week period before and the third week after galcanezumab injection for each episode of cluster bouts. Paired t-test was used for comparing repeated data from different episodes of cluster bout. RESULTS: Sixteen patients were enrolled in this study. Fourteen patients received galcanezumab therapy for two consecutive cluster bouts. Galcanezumab was administered 24 days and 11 days after the first and second cluster bouts, respectively. The proportion of patients with 50% or more reduction in frequency of daily headache at week 3 from baseline was 86% and 64% during the first and second episodes, respectively. The proportion of patients who received transitional therapy before galcanezumab therapy was higher in the first episode of cluster bout than that in the second episode of cluster bout. No serious adverse reactions or significant differences in adverse effects between cluster bouts were noticed. Two patients received a second galcanezumab therapy during the pre-cluster period, and their cluster periods ended without typical cluster headache attacks 10-60 days after galcanezumab therapy. CONCLUSIONS: This exploratory analysis suggests that galcanezumab may be effective as a preventive therapy in subsequent cluster bouts. Patients with episodic cluster headaches who underwent galcanezumab therapy tended to receive a second round of treatment in the early stages of their next cluster bout without transitional therapy.

A case of disorder of arousal with prolonged postarousal hypersynchronous delta activity.

Disorder of arousal (DOA) is a form of non-rapid eye movement sleep parasomnia caused by partial or incomplete arousal from deep sleep. Most previous studies of patients with DOA analyzed prearousal hypersynchronous delta activity (HSDA), but few studies have described postarousal HSDA. Herein, we report a 23-year-old man with a history of abrupt arousal during sleep and confused behavior and speech since he was 14 years old. During video electroencephalography monitoring, he had 9 arousal events of getting up, sitting on the bed, looking around, or simple arousal, including eyes open, looking at the ceiling, or head flexion. During all arousal events, the postarousal electroencephalography pattern was prolonged HSDA for approximately 40 seconds. The patient was treated unsuccessfully for more than 2 years with an antiseizure medication (lacosamide); eventually, he responded to clonazepam that was administered for the possibility of DOA. Prolonged rhythmic HSDA without spatiotemporal evolution can appear as a postarousal electroencephalography pattern of DOA. When diagnosing DOA, it is important to recognize that postarousal HSDA can appear as a characteristic electroencephalography pattern of DOA. CITATION: Kang M, Shin D, Lee HC, Provini F, Jung KY. A case of disorder of arousal with prolonged postarousal hypersynchronous delta activity. J Clin Sleep Med. 2023;19(7):1365-1368.

Adult-onset rapidly worsening progressive myoclonic epilepsy caused by a novel variant in DHDDS.

Progressive myoclonic epilepsy (PME) is a heterogeneous neurogenetic disorder manifesting as progressive myoclonus, seizure, and ataxia. We report a case of PME caused by a novel DHDDS variant. Additionally, by reviewing the literature on DHDDS mutations, we compared the phenotype of our patient with previously reported phenotypes. We identified DHDDS (c.638G>A, p. Ser213Asn) as a likely pathogenic variant. The literature review revealed 15 PME patients with DHDDS mutations from 13 unrelated families. According to previous studies, late-onset patients tend to have a slow-progressive disease course. Although his myoclonus and ataxia were adult onset, our patient experienced rapid disease aggravation.

Different imaging patterns of PCNSL and IVL: a case report.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare, malignant, non-Hodgkin's lymphoma of the brain, leptomeninges, and rarely the spinal cord. PCNSL has characteristic magnetic resonance imaging (MRI) findings, and effective treatment strategies are available. It is characterized predominately by neurological symptoms, which are caused by tumor infiltration into the nervous system as well as ischemia. Chemotherapy is an effective treatment, if started prior to the ischemic damage. CASE PRESENTATION: A 62-year-old male patient with PCNSL presented with altered mental status. The initial brain MRI revealed high signal intensity on the T2-weighted images (T2WIs) of the putamen area of the right basal ganglia, and the clinical symptoms improved after steroid administration. However, the symptoms were later deteriorated, we considered the possibility of autoimmune encephalitis and, consequently, conducted an immunomodulatory therapy. In a follow-up brain MRI, enlargement lesions of T2WI in basal ganglia and pons were simultaneously enhanced. Subsequently, the patient's mental status deteriorated to a semi-coma and PCNSL was diagnosed after a surgical biopsy. Chemotherapy was started immediately; however, the patient died. CONCLUSIONS: Effective treatments are available for PCNSL and intravascular lymphoma; thus, their prognosis is generally good if they are diagnosed early. Herein, we report the case of a patient suspected with autoimmune encephalitis after brain MRI and treated with immunomodulation therapy. However, PCNSL was confirmed by a surgical biopsy. It is, therefore recommended to consider lymphoma in patients with neurological symptoms that are difficult to localize and rapidly progressive enhancing lesions showing a mass effect on brain MRI.

UV-triggerable alginate beads decorated with basic proteinoid-coumarin conjugates.

UV-triggerable bead was prepared by coating the surface of alginate bead with a photo-responsive proteinoid. Epoxypropoxy coumarin (EPC) was covalently attached to a basic proteinoid composed of lysine and serine (PLS), and the PLS-EPC conjugate was used as a photo-responsive coat of the bead. EPC residues of PLS-EPC conjugate on the bead surface were photo-cross-linked upon the irradiation of λ = 365 nm, and then FITC-dextran was loaded as a fluorescence dye in the bead coated with photo-cross-linked PLS-EPC conjugate. Upon the irradiation of λ = 254 nm, the degree of release from the bead was enhanced possibly because the cross-linked network of PLS-EPC conjugate coating layer is photo-cleaved. The UV-triggerable bead developed in the present study could be applied to a UV-responsive drug carrier.

The incidence of tuberculosis after a measles outbreak.

Among 53,974 cases of measles that occurred during the 2000-2001 outbreak in Korea, the incidence of tuberculosis following measles was 47 cases per 214,949.6 person-years, which was significantly lower than that in the general population (standardized incidence ratio, 0.73; 95% confidence interval, 0.54-0.96). In conclusion, we did not find a positive relationship between measles and tuberculosis.