[A case of fascioliasis].

A 38-year-old rice farmer visited a hospital for abdominal pain. Computed tomography (CT) showed a liver tumor and magnetic resonance imaging (MRI) revealed a hypovascular tumor, both in segment 4. Thus, he was diagnosed with liver abscess. Ten days later, CT showed a new liver tumor in segment 8, but the size of the liver tumor in segment 4 had decreased. He was suspected with parasitic disease because of eosinophilia. Enzyme-linked immunosorbent assay showed a high level of serum Fasciola antibody. The patient was diagnosed with fascioliasis and was treated with triclabendazole. Post-treatment, CT revealed that the liver tumors had shrunk. Eosinophilia and multiple lesions were characteristic findings of parasitic disease.

Long-term outcomes and health-related quality of life in patients with autoimmune encephalitis: An observational study.

Autoimmune encephalitis (AE) subacutely causes severe and multiple symptoms; however, most patients achieve neurologically favorable outcomes. Despite the substantial recovery in motor function, persistent impairments in mental/social aspects lasting for several years have been recognized, and its potential effect on health-related quality of life (HRQOL) has been argued. To urgently evaluate the long-term effects of AE on patients' HRQOL, we investigated patient-oriented long-term outcomes and assessed the HRQOL of patients with AE. Data of patients who were diagnosed with probable/definite AE, defined by Graus AE criteria 2016, and treated at our hospital between January 2011 and October 2020 were retrospectively retrieved. Their long-term (≥2 years) outcomes, which included various sequelae and handicaps in social activities such as returning to previous work/school life through structured interview forms, were evaluated, and the HRQOL was assessed using Neuro-QOL battery. We identified 32 patients who met the Graus AE criteria 2016 and eventually enrolled 21 patients in the study. The median interval between disease onset and survey period was 63 (25-156) months, and 43% of the patients had persistent neuropsychiatric symptoms, including memory disorders, personality changes, and seizures. No more than 71% returned to their previous work/school life. Although most of the patients had global QOL within normal limits, 48% had social QOL under normal limits. Patients with sequelae were significantly less likely to return to previous work/school and had worse global/social quality of life than patients without sequelae. In conclusion, nearly half of patients with AE had social QOL under normal limits 5 years after onset. The difficulty in returning to work/school and a worse HRQOL were notable in patients with sequelae.

Lidocaine as a potential therapeutic option for super-refractory status epilepticus: A case report.

New-onset refractory status epilepticus (NORSE) is a rare and devastating condition and the prognosis is often poor, with half to two-thirds of survivors experiencing drug-resistant epilepsy, residual cognitive impairment, or functional disability, and the mortality rate is 16% to 27% for adults. We describe a patient with cryptogenic NORSE and favorable recovery from drug-resistant super-refractory SE after the use of intravenous lidocaine. The patient experienced fever and presented with refractory generalized tonic-clonic seizures. The cause was not found by performing extensive examinations, including cell surface autoantibodies and rat brain immunohistochemistry evaluations. The refractory SE with unresponsiveness to multiple anti-epileptic and prolonged sedative medications, which are necessary for prolonged mechanical ventilation, were ameliorated by additive treatment with intravenous lidocaine initiating at 1 mg/kg/h and maintaining at 2 mg/kg/h for 40 days, which led to freedom from intravenous sedative medication and mechanical ventilation. The patient was able to return to school. Lidocaine may be an optional treatment for cryptogenic NORSE.

Cerebellar Progressive Multifocal Leukoencephalopathy Mimicking Anti-Yo-Antibody-Associated Rapidly Progressive Cerebellar Syndrome.

A 58-year-old woman with a history of systemic lupus erythematosus (SLE) who was taking prednisolone and mycophenolate mofetil presented with gait disturbances that progressively worsened over a period of 3 months. Her blood test and cerebrospinal fluid (CSF) examination results did not indicate active SLE. Initial brain magnetic resonance imaging (MRI) revealed a small spotty lesion in the left cerebellar peduncle. The clinical course was consistent with rapidly progressive cerebellar syndrome (RPCS), which sometimes involves neuronal antibodies. The line blot assay detected anti-Yo antibodies, but no malignancy was found. Immunohistological techniques using rat brain sections yielded a negative result for anti-Yo antibodies. The second MRI revealed a focal lesion and surrounding spotty lesion in the left cerebellar peduncle, which was consistent with the punctate pattern observed in progressive multifocal leukoencephalopathy (PML). The CSF JCV-DNA test indicated the presence of cerebellar PML. Immunosuppressants were reduced, and mefloquine and mirtazapine were initiated. After approximately 2 years and 1 month, the CSF JCV-DNA results became negative. Cerebellar PML may exhibit a clinical course that is consistent with RPCS. The punctate pattern should be recognized as an early manifestation of PML. The CSF JCV-DNA copy number may serve as a useful indicator of PML stabilization.

Case report: Reversible brain atrophy with low titer anti-amphiphysin antibodies related to gastric adenocarcinoma.

Amphiphysin (AMPH) autoimmunity is associated with a variety of neurological complications, including encephalitis, peripheral neuropathy, myelopathy, and cerebellar syndrome. Its diagnosis is based on clinical neurological deficits and the presence of serum anti-AMPH antibodies. Active immunotherapy, such as intravenous immunoglobulins, steroids, and other immunosuppressive therapies, has been reported to be effective in most patients. However, the extent of recovery varies depending on the case. Herein, we report the case of a 75-year-old woman with semi-rapidly progressive systemic tremors, visual hallucinations, and irritability. Upon hospitalization, she developed a mild fever and cognitive impairment. Brain magnetic resonance imaging (MRI) showed semi-rapidly progressive diffuse cerebral atrophy (DCA) over 3 months, while no clear abnormal intensities were observed. The nerve conduction study revealed sensory and motor neuropathy in the limbs. The fixed tissue-based assay (TBA) failed to detect antineuronal antibodies; however, based on commercial immunoblots, the presence of anti-AMPH antibodies was suspected. Therefore, serum immunoprecipitation was performed, which confirmed the presence of anti-AMPH antibodies. The patient also had gastric adenocarcinoma. High-dose methylprednisolone, and intravenous immunoglobulin were administered and tumor resection was performed, resulting in resolution of the cognitive impairment and improvement in the DCA on the post-treatment MRI. After immunotherapy and tumor resection, the patient's serum was analyzed using immunoprecipitation, which showed a decrease in the level of anti-AMPH antibodies. This case is noteworthy because the DCA showed improvement after immunotherapy and tumor resection. Additionally, this case demonstrates that negative TBA with positive commercial immunoblots do not necessarily indicate false positive results.

[Main Syndrome and Antibody Effects of Autoimmune Encephalitis Caused by Antineuronal Surface Antibodies: An Update].

Since the discovery of a series of antineuronal surface antibodies (NSAs), the diagnostic approach and management of patients with autoimmune encephalitis (AE) and related disorders have undergone a "paradigm shift." However, recent topics described below are also announcing the dawn of the next era in the practice of patients with AE. As the clinical spectrum of NSA-associated AE expands, some types of AE (e.g., anti-DPPX antibody-associated and anti-IgLON5 antibody-associated disorders) can be misclassified into reconsider diagnosis when using the previously published diagnostic criteria. Nobel active immunization animal models of NSA-associated disorders (e.g., anti-NMDAR encephalitis model) can remarkably emphasize the understanding of the pathophysiological effects and main syndrome induced by NSAs. Additionally, several international clinical trials (e.g., rituximab, inebilizumab, ocrelizumab, bortezomib, and rozanolixizumab) for AE treatments, including anti-NMDAR encephalitis, have been implemented. Data from these clinical trials can be used to establish the best treatment of AE.

[A case of severe alcoholic hepatitis successfully treated with granulocytapheresis].

A 46-year-old male patient with a drinking history presented at our hospital with jaundice. He was diagnosed with moderate alcoholic hepatitis based on laboratory data. The white blood cell (WBC) counts were gradually increased and the prothrombin time was prolonged after hospitalization. Methylprednisolone (1000mg/day for 3 days) followed by oral prednisolone (40mg/day) was administered. However, the liver function was not improved and the patient progressed to severe alcoholic hepatitis. Therefore, we performed granulocytapheresis (GCAP). The WBC counts and interleukin-6 decreased and the liver function improved after 3 GCAP sessions.

Long-term effects of anti-N-methyl-d-aspartate receptor encephalitis on quality of life.

Background: Patients with anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) usually achieve neurologically favorable outcomes in the post-acute-phase. Even when motor function recovers, many patients experience numerous non-motor sequelae and cannot resume their pre-NMDARE lives even years later. Additionally, the needs of patients with NMDARE may impose a severe caregiver burden. Unfortunately, few studies have comprehensively examined patients recovering from NMDARE. We investigated the long-term effects of NMDARE on patients' quality of life (QOL). Methods: Data collected via structured self-reported questionnaires included clinical features, long-term outcomes, and QOL. These questionnaires were administered to adult members of the Japanese Anti-NMDARE Patients' Association. We used the NeuroQOL battery to assess QOL in physical, mental, and social domains. Raw NeuroQOL scores were converted to T-scores for comparison with controls. Results: Twenty-two patients completed the questionnaire. The median interval between disease onset and questionnaire response was 78 months. Forty-six percent of patients reported persistent sequelae, with only 73% able to resume prior work/school activities. Although patients' Global QOL was similar to controls, patients with NMDARE had significantly worse social QOL. Patients with worse social QOL had more frequent sequelae than those with better social QOL. Furthermore, patients with persistent sequelae had significantly worse Global QOL than those without sequelae and controls. Conclusion: Patients with NMDARE had worse social QOL than controls. Given the adverse effects of disease sequelae on QOL, treatment strategies that minimize sequelae during the acute-phase may improve patients' QOL, even years post-disease onset.

Two Patients with Spontaneous Spinal Epidural Hematoma Carrying a Good Prognosis without Surgical Operations.

(1) Introduction: Spontaneous spinal epidural hematoma (SSEH) points to hematoma within the epidural space of the spinal cord without traumatic or iatrogenic causes. (2) Case Reports: One patient showed paraplegia, numbness of both legs with acute onset, acute myelopathic signs, subsequent to back pain. Magnetic resonance imaging (MRI) showed hematoma in the posterior part of the thoracic spinal cord. Another patient showed acute numbness in the shoulder, upper part of the back, and the upper extremity on the right side after pain in the back, shoulder, and neck on the right side. Sagittal computed tomography (CT) images of the cervical bone showed a high-density area behind the spinal cord between C4 and C7. MRI analysis showed hematoma in the right diagonally posterior part of the cervical spinal cord. These 2 patients lacked traumatic or iatrogenic events, and their symptoms abated without surgical operation. (3) Conclusions: The location of hematoma correlated with symptoms in each patient. SSEH is rare but should be taken into account in patients with myelopathy or radiculopathy with acute onset subsequent to back pain. The usefulness of emergent CT scans of the spinal cord prior to MRI analysis was shown in the diagnosis of SSEH.

Clinical Features of Acute Ischemic Stroke Patients with Hypoesthesia as an Initial Symptom.

This study aimed to evaluate the clinical characteristics of acute ischemic stroke (AIS) patients who experienced hypoesthesia as the initial symptom. We retrospectively analyzed the medical records of 176 hospitalized AIS patients who met our inclusion and exclusion criteria and evaluated their clinical features and MRI findings. Among this cohort, 20 (11%) patients presented with hypoesthesia as the initial symptom. MRI scans of these 20 patients identified lesions in the thalamus or pontine tegmentum in 14 and brain lesions at other sites in 6. The 20 hypoesthesia patients had higher systolic (p = 0.031) and diastolic blood pressure (p = 0.037) on admission, and a higher rate of small-vessel occlusion (p < 0.001) than patients without hypoesthesia. The patients with hypoesthesia had a significantly shorter average hospital stay (p = 0.007) but did not differ significantly from those without hypoesthesia in National Institutes of Health Stroke Scale scores on admission (p = 0.182) or the modified Rankin Scale scores for neurologic disability on discharge (p = 0.319). In the patients with acute onset hypoesthesia, high blood pressure, and neurological deficits were more likely to be due to AIS than other causes. Since most of the lesions in AIS patients with hypoesthesia as the initial symptom were found to be small, we recommend performing MRI scans with such patients to confirm AIS.

Post-Stroke Pneumonia in Real-World Practice: Background, Microbiological Examination, and Treatment.

Post-stroke pneumonia (PSP) has an impact on acute ischemic stroke (AIS). Although predictive scores for PSP have been developed, it is occasionally difficult to predict. Clarifying how PSP was treated after its onset in clinical practice is important. Admitted patients with AIS over a 2-year period were retrospectively reviewed. Of 281 patients with AIS, 24 (8.5%) developed PSP. The integer-based pneumonia risk score was higher in patients with PSP. The onset of PSP was frequently seen up to the 4th day of hospitalization. Of patients with PSP, sputum examination yielded Geckler 4 or 5 in only 8.3%. Angiotensin-converting enzyme inhibitor (ACE-I) was more frequently administered to patients with PSP; however, all these cases were started with ACE-I following PSP onset. Nasogastric tubes (NGTs) were inserted in 16 of the patients with PSP, of whom 11 were inserted following PSP onset. Multivariate analysis showed that PSP onset was a poor prognostic factor independent of the female sex, urinary tract infection, and National Institutes of Health Stroke Scale. PSP treatment would benefit from the administration of antimicrobials and ACE-I, as well as NGT insertion. To select effective agents for PSP and evaluate the indications for NGT insertion, further case studies are needed.

Leucine-rich Glioma-inactivated 1 Encephalitis Followed by Isaacs Syndrome: Alternating Presence of Pathogenic Autoantibodies to Leucine-rich Glioma-inactivated 1 and Contactin-associated Protein-like 2.

The coexistence of leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) autoantibodies in the same individual is surprisingly often observed. We herein report the first case of LGI1 encephalitis followed by Isaacs syndrome in which LGI1 and CASPR2 antibodies in the serum and cerebrospinal fluid (CSF) were measured during the entire disease course. After the resolution of limbic encephalitis, LGI1 antibodies disappeared from the CSF simultaneously with the appearance of CASPR2 antibodies in the serum. The alternating presence of these pathogenic autoantibodies along with the clinical and phenotypic alternations suggested that LGI1 encephalitis was associated with CASPR2 autoantibody production in the peripheral tissue, leading to CASPR2-associated Isaacs syndrome.

Delayed encephalopathy after COVID-19: A case series of six patients.

RATIONALE: Acute encephalopathy is a severe neurological complication of coronavirus disease 2019 (COVID-19). Most cases of acute encephalopathy associated with COVID-19 occur within several weeks of COVID-19 onset. We describe a case series of 6 patients who developed delayed encephalopathy (DE) after COVID-19. PATIENT CONCERNS AND DIAGNOSES: We evaluated patients who recovered from COVID-19 and showed acute disturbance of consciousness or focal neurological deficits without recurrence of pneumonitis. Six patients, 2 females and 4 males, with ages ranging from 65 to 83 years were included. Durations of hospitalization due to COVID-19 were between 25 and 44 days. The severity of COVID-19 was moderate in 5 and severe in 1 patient. Patients were rehospitalized for acute disturbance of consciousness concomitant with postural tremor and, abnormal behavior, hemiplegia, aphasia, or apraxia between 34 and 67 days after the onset of COVID-19. Chest computed tomography showed no exacerbation of pneumonitis. Brain magnetic resonance imaging showed no specific findings except in 1 patient with an acute lacunar infarction. Electroencephalogram demonstrated diffuse slowing in all patients. Repeat electroencephalogram after recovery from encephalopathy demonstrated normal in all patients. One of the 6 patients had cerebrospinal fluid (CSF) pleocytosis. CSF protein levels were elevated in all patients, ranging from 51 to 115 mg/dL. CSF interleukin-6 levels ranged from 2.9 to 10.9 pg/mL. The immunoglobulin index was 0.39 to 0.44. Qlim(alb) < QAlb indicating dysfunction of the blood-brain barrier was observed in all patients. Severe acute respiratory syndrome coronavirus 2 reverse transcription polymerase chain reaction of CSF was negative in all patients. Neuronal autoantibodies were absent in serum and CSF. INTERVENTIONS AND OUTCOMES: Immunotherapy including steroid pulses was administered to 3 patients; however, symptoms of encephalopathy resolved within several days in all patients, regardless of treatment with immunotherapy, and their consciousness levels were recovered fully. Notably, postural tremor remained for 2 weeks to 7 months. LESSONS: In our patients, DE after COVID-19 was characterized by symptoms of acute encephalopathy accompanied with tremor in the absence of worsening pneumonitis after the fourth week of COVID-19 onset. Our findings indicate blood-brain barrier dysfunction may contribute to the pathogenesis of DE after COVID-19.

Use of the FilmArray® Meningitis/Encephalitis panel to detect pathogenic microorganisms in cerebrospinal fluid specimens: a single-center retrospective study.

OBJECTIVE: Meningitis and encephalitis are neurological emergencies requiring rapid diagnosis and treatment. The performance of the FilmArray® Meningitis/Encephalitis (ME) panel, a multiplex polymerase chain reaction test, and conventional methods for diagnosing meningitis and encephalitis was compared. METHODS: This retrospective study assessed 20 patients diagnosed with meningitis or encephalitis according to clinical symptoms and laboratory examination findings between January 2018 and December 2019. The results of the FilmArray® ME panel were compared with those of conventional methods. RESULTS: Pathogens were identified in 11 (55%) patients using the FilmArray® ME panel and in nine (45%) patients using conventional methods. The test identified herpes simplex virus type 1 in two patients, herpes simplex virus type 2 in one, varicella-zoster virus in four, Streptococcus pneumoniae in three, and Cryptococcus neoformans in one. Furthermore, additional pathogens were detected (n = 1, S. pneumoniae and n = 1, varicella-zoster virus). The median times to pathogen identification were 2 hours using the FilmArray® ME panel and 96 hours with conventional methods. CONCLUSIONS: The sensitivity of the FilmArray® ME panel for rapidly detecting the most common pathogens was similar to that of conventional methods. Hence, this method could decrease the time to definitive diagnosis and treatment initiation.

[Autoimmune Encephalitis Triggered by Herpes Simplex Encephalitis: Main Syndrome, Diagnosis, and Treatment].

Autoimmune disorders, such as choreoathetosis and abnormal behavior secondary to herpes simplex encephalitis (HSE), are discussed in this review. These disorders are known to develop without any evidence of HSE relapse, while they respond to immunotherapies such as intravenous corticosteroids. Recent evidence, including a prospective Spanish cohort study of HSE, revealed that autoimmune encephalitis (AE) can be triggered by HSE, which is closely related to several neuronal surface antibodies (NSAs). Anti-N-methyl-D-aspartate encephalitis (NMDARE) is the most common phenotype of AE post-HSE. Moreover, approximately 30% of cases of AE post-HSE are caused by NSAs against undetermined antigens. Thus, patients suspected of having AE post-HSE should be tested for NSAs using comprehensive techniques combining tissue-based and cultured live neuron assays. The primary syndrome of AE post-HSE is age dependent, as demonstrated in non-post-HSE onset NMDARE. Choreoathetosis is the most common symptom in infants and toddlers, while abnormal behavior and psychiatric symptoms are the most common symptoms in adolescents and adults. Regarding the treatment, current knowledge reveals that intensive immunotherapies can be used to treat AE post-HSE and lead to better outcomes although a minority of cases show recovery without immunotherapy administration.

Neurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review.

RATIONALE: Neurosyphilis presenting as limbic encephalitis (LE) is an important differential diagnosis of autoimmune LE (ALE) defined by Graus in 2016. However, data on the clinical differences and similarities between neurosyphilis presenting as LE and ALE are limited. Herein, we report neurosyphilis presenting as ALE that fulfilled the main items of the Graus ALE criteria. Moreover, a literature review of neurosyphilis presenting as LE was performed. PATIENT CONCERNS: A 66-year-old Japanese man developed nonconvulsive status epilepticus. He presented with progressive personality change and working memory deficits within 3 months prior to admission. A hyperintense lesion localized in the bilateral medial temporal area was observed on T2-weighted fluid-attenuated inversion recovery brain magnetic resonance imaging. Cerebrospinal fluid analysis showed mild pleocytosis and the presence of oligoclonal band. However, in-house assays did not detect antineuronal antibodies. Electroencephalogram showed lateralized rhythmic delta activity in the right temporal area. The serum and cerebrospinal fluid serological and antigen tests for syphilis had positive results. DIAGNOSIS: ALE was initially suspected based on the patient's symptoms and ancillary test findings that fulfilled the Graus ALE criteria. However, based on the positive confirmatory test results for syphilis, a diagnosis of neurosyphilis was eventually made. INTERVENTION: The patient received intravenous midazolam, oral levetiracetam, and lacosamide to control nonconvulsive status epilepticus. In addition, he was treated with intravenous benzylpenicillin at a dose of 24 million units/day for 14 days. OUTCOMES: The patient's cognitive function relatively improved after antibiotic treatment. However, he presented with persistent mild working memory deficit, which was evaluated with the Wechsler Adult Intelligence Scale, 3rd edition. Therefore, on day 103 of hospitalization, he was transferred to another hospital for rehabilitation and long-term care due to limitations in performing activities of daily living. LESSONS: The present case was diagnosed with neurosyphilis presenting as ALE, but meanwhile, in most case, neurosyphilis presenting as LE developed at a slower progressive rate, and it had a broader or restricted involvement on brain MRI than ALE based on the literature review. Therefore, an appropriate differential diagnosis of LE can be obtained by identifying clinical differences between the 2 conditions.

Antitumor effects of pyrrole-imidazole polyamide modified with alkylating agent on prostate cancer cells.

Androgens and androgen receptor (AR) have a central role in prostate cancer progression by regulating its downstream signaling. Although androgen depletion therapy (ADT) is the primary treatment for most prostate cancers, they acquires resistance to ADT and become castration resistant prostate cancers (CRPC). AR complex formation with multiple transcription factors is important for enhancer activity and transcriptional regulation, which can contribute to cancer progression and resistance to ADT. We previously demonstrated that OCT1 collaborates with AR in prostate cancer, and that a pyrrole-imidazole (PI) polyamide (PIP) targeting OCT1 inhibits cell and castration-resistant tumor growth (Obinata D et al. Oncogene 2016). PIP can bind to DNA non-covalently without a drug delivery system unlike most DNA targeted therapeutics. In the present study, we developed a PIP modified with a DNA alkylating agent, chlorambucil (ChB) (OCT1-PIP-ChB). Then its effect on the growth of prostate cancer LNCaP, 22Rv1, and PC3 cells, pancreatic cancer BxPC3 cells, and colon cancer HCT116 cells, as well as non-cancerous MCF-10A epithelial cells, were analyzed. It was shown that the IC50s of OCT1-PIP-ChB for 22Rv1 and LNCaP were markedly lower compared to other cells, including non-cancerous MCF-10A cells. Comprehensive gene expression analysis of CRPC model 22Rv1 cells treated with IC50 concentrations of OCT1-PIP-ChB revealed that the gene group involved in DNA double-strand break repair was the most enriched among gene sets repressed by OCT1-PIP-ChB treatment. Importantly, in vivo study using 22Rv1 xenografts, we showed that OCT1-PIP-ChB significantly reduced tumor growth compared to the control group without showing obvious adverse effects. Thus, the PIP combined with ChB can exert a significant inhibitory effect on prostate cancer cell proliferation and castration-resistant tumor growth, suggesting a potential role as a therapeutic agent.

Intramedullary spinal cord abscess associated with right-to-left shunt via right superior vena cava draining into left atrium: A case report.

RATIONALE: Intramedullary spinal cord abscess (ISCA) is a rare but treatable bacterial infection of the central nervous system, and the etiology in no less than 40% of the cases is cryptogenic. Although a few cases of ISCA in individuals with a right-to-left shunt (RL shunt) have been reported, only few arguments focused on the association between RL shunt and ISCA have been provoked. The right superior vena cava (RSVC) draining into the left atrium (LA) is an uncommon systemic venous anomaly that results in an RL shunt, and this anomaly causes several types of neurological complication such as stroke or brain abscess. We report the first case of ISCA associated with RSVC-LA RL shunt. PATIENT CONCERNS: A 36-year-old man developed progressive paraparesis, dysuria, and spontaneous pain in the lumbar region and lower extremities. Spinal magnetic resonance imaging revealed an intramedullary lesion extended from Th12 to L2 with ring-shaped gadolinium enhancement. Cerebrospinal fluid (CSF) study exhibited a marked pleocytosis, and CSF culture grew Streptococcus intermedius. Cardiovascular computed tomography angiography identified RSVC-LA RL shunt, which caused transient acute cardiac syndrome due to air embolus. DIAGNOSES: The patient was diagnosed with ISCA associated with an RSVC-LA RL shunt. INTERVENTIONS: The patient was treated with a combination of intravenous administration of meropenem and vancomycin in a daily dose of 6 and 2.5 g, respectively, followed by intravenous administration of ampicillin in a daily dose of 750 mg. The intravenous antibiotic therapy was continued for 37 days. OUTCOMES: A favorable neurological outcome was obtained by the intravenous antibiotic therapy, and recurrence of infection was prevented by continuous oral antibiotic therapy for 18 months. LESSONS: With a literature review of ISCA associated with RL shunt, we insist that screening for RSVC-LA is beneficial to patients who are diagnosed with cryptogenic ISCA as its identification leads to appropriate preventive therapy.

Ocular flutter as the presenting manifestation of autoimmune glial fibrillary acidic protein astrocytopathy.

A 39-year-old man exhibited ocular flutter and cerebellar ataxia following a subacute disturbance of consciousness and partial seizure. He was diagnosed with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy by tissue- and cell-based antibody assays. Brain single-photon emission computed tomography detected a significant increase in blood flow in the fastigial nucleus, a critical region for eye saccade control. Immunotherapies diminished the ocular flutter and reduced hyperperfusion in the fastigial nucleus. This case suggests that autoimmune GFAP astrocytopathy can cause ocular flutter and provides strong imaging evidence supporting the hypothesis that ocular flutter is caused by hyperactivity or disinhibition of the fastigial nucleus.

[Anti-NMDAR Encephalitis with Poor Recovery on Steroid Pulse and IVIg: Practical Approach to Intensive Immunotherapy].

Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is a well-defined autoimmune encephalitis that is responsive to early intensive immunotherapy. Recent international consensus regarding treatment of NMDARE provides a practical treatment algorithm for immunotherapy escalation, while considering a patient's age, disease severity, and other background information. First-line immunotherapy, which includes an intravenous (IV) corticosteroid pulse with the addition of either intravenous immunoglobulins (IVIg) or plasma exchange, should be offered to all NMDARE-diagnosed patients as soon as possible. In cases where insufficient improvement follows a repeat of the first-line combination therapy (assessed on day 14 after the initial treatment), second-line immunotherapy comprising rituximab or an IV cyclophosphamide pulse (IVCPA) is considered. Per the recent expert consensus, rituximab is preferred to IVCPA as the second-line drug of choice, although the use of either drug in the treatment of NMDARE is off-label. Most patients show gradual improvement in the first few weeks following the introduction of second-line therapy, although repeated and alternating use of both drugs is often required. Some patients, whose NMDARE was refractory to the aforementioned therapies, have also been successfully treated with tocilizumab or bortezomib. Moreover, several international clinical trials involving rituximab, inebilizumab, bortezomib, and rozanolixizumab in the treatment of autoimmune encephalitis (AE, which includes NMDARE) are being conducted to establish high-grade evidence for the treatment of AE.