Acta neurologica Taiwanica最新文献

Background: Treatment guideline for status epilepticus (SE) specifically in patients with anti-N-methyl- D-aspartate receptor (anti-NMDAR) encephalitis is insufficient. This study aimed to clarify the determinants for the control of SE in adult patients with anti-NMDAR encephalitis.

Methods: Medical records of all patients with anti-NMDAR encephalitis hospitalized between Jan. 2010 and Sep. 2019 were analyzed for the time sequence of seizures and treatments, and antiepileptic drug (AED) regimens related to SE. The outcomes were control of SE and seizures, and the discharge score of modified Rankin Scale (mRS).

Results: All eight patients had seizures and seven (87.5%) suffered from SE which lasted for 3.6 ± 3.9 days. Five patients (71.4%) had SE earlier than using IT, whose SE was controlled by AEDs alone (n = 4) or combined with teratomas resection (n = 1). Another two patients suffered from SE after receiving IT, and one of them had SE only for 1 hour. Moreover, all SE patients received increased types and dosages of AEDs at SE end. A shorter duration of refractory SE was associated with its later occurrence after seizure onset (p = 0.005) and longer duration of AEDs use before SE (p = 0.026). All cases achieved seizure freedom after receiving AEDs and IT.

Conclusions: In these patients with anti-NMDAR encephalitis, all the SE which occurred before initiating IT was successfully controlled by AEDs alone or combined with teratoma resection, and later onset of refractory SE was associated with a shorter SE duration.

Purpose: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic cephalalgia. Since the underlying mechanism of the pathogenesis has not yet been determined, a standardized therapeutic strategy for SUNCT is unavailable. We present a case of SUNCT syndrome with successful pain relief by intravenous administration of ketamine, an N-methyl-D-aspartate receptor (NMDAR) antagonist.

Case report: A 56-year-old male patient reported severe throbbing and shooting pain in forehead, temporal and periorbital region. We confirmed conjunctival injection, lacrimation, blepharoptosis, and miosis as symptoms related to autonomic activity, and made a diagnosis of SUNCT based on ICHD-3 beta. Numerous treatments were attempted, including pregabalin, gabapentine, nonsteroidal antiinflammatory drugs, acetaminophen, steroids, antidepressants, triptans, nerve blocks, and intravenous lidocaine with unsatisfactory results. Intravenous administration of ketamine (0.4 mg/kg) for one hour, was found to relieve the severe pain.

Conclusion: Intravenous ketamine can effectively treat SUNCT syndrome. This case demonstrated that involvement of NMDAR could be one of the mechanisms of SUNCT syndrome pathogenesis and establish a therapeutic strategy for this pain syndrome.

Objective: No previous study has reported on the clinical characteristics of cryptococcal meningitis (CM) focusing solely on young adults.

Patients and methods: Ninety-nine adult patients with CM (64 men and 35 women) were enrolled, of whom 26 were classified into the young adult group (≤ 40 years) and 73 into the non-young adult group (> 40 years). The modified Rankin scale (mRS) was used to evaluate the outcomes of the survivors at the time of discharge and at 1 year of follow-up. The clinical characteristics and laboratory data between 1) the young adult CM patients with and without acquired immunecompromised syndrome and 2) the male and female young adult CM patients were compared. The prognostic factors of the young adult CM patients were also analyzed.

Results: The young adult group had a higher incidence of headache as the clinical presentation which may have been due to the higher intracranial pressure in this group. The overall mortality rate of the young adults with CM was high (38.5%, 10/26), but no significant prognostic factors were found. In followup studies of the neurologic deficits, the young adult survivors had better outcomes (mRS scores = 0-2) than the non-young adult group at discharge and 1 year after discharge.

Conclusion: The young adult CM patients had a higher incidence of headache as the clinical presentation. Although the mortality rate in the young adult CM patients was high, the survivors had better neurologic outcomes.

Ischemic stroke has been increasingly reported as a consequence of COVID-19 infection. However, the underlying etiology is not well determined. The objective of this study is to discuss association of juvenile stroke with COVID-19 infection. We analyzed 5 COVID-19 positive and stroke patients with a mean age of 41.2 years-old. Three patients developed large vessel occlusion, one small vessel occlusion and one PRES with superimposed lobar ICH, respectively. The mean initial NIHSS of our patients was 11.6. Except the one with massive cerebellar infarct, a desirable outcome occurred with a mean mRS 2.6 at discharge. The mean ESR and CRP level was elevated to 30.4 ml and 32 mg/dl. The severity of COVID-19 infection was considered mainly as mild. COVID-19 infection has the potential to induce hypercoagulability state contributing to stroke development even in the mild form of disease. Keywords: Cerebrovascular Accident, CVA, Stroke, COVID-19, Novel Coronavirus Acta.

Purpose: Case presentation of newly diagnosed systemic lupus erythematosus (SLE) presenting initially as Tolosa-Hunt syndrome (THS).

Study design: Retrospective clinical case.

Method: Case report.

Results: A healthy young man developed acute binocular diplopia within 2 days without other neurological deficits. Bilateral 6th cranial nerve palsy was observed with general reduction in the visual field test. Emergent brain magnetic resonance image (MRI) was performed, which revealed severe inflammation in the cavernous sinus, superior orbital fissure, and apex of the orbit. No cavernous thrombosis or intracranial lesion was shown in the MRI. THS was diagnosed and the patient's CN 6 palsy recovered quickly after corticosteroid treatment. However, severe anaemia was discovered during admission (Hb=6.0), so the patient was evaluated by profound laboratory tests, which revealed SLE.

Conclusion: With painful ophthalmoplegia, cavernous sinus syndrome is highly suspected. THS is one of the differential diagnoses for cavernous sinus syndrome. THS is a rare disease, recognized by the National Organization for Rare Disorders, and characterized by inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex under image study. The inflammatory changes are mostly idiopathic, but secondary causes such as sarcoidosis or other autoimmune diseases need to be ruled out. Physicians should be aware of possible underlying conditions, such as immunosuppressed status as in SLE, as the true cause of THS.

Purpose: Encephalitis secondary to human herpesvirus 6 (HHV-6) infection is frequently encountered in immunocompromised patients; in contrast, HHV-6 encephalitis in immunocompetent patients is rare. There are only 3 reports of status epilepticus due to HHV-6 encephalitis in immunocompetent adults. In the present study, a case of refractory status epilepticus secondary to HHV-6 encephalitis was reported in an immunocompetent female.

Case report: We report a case of a previously healthy 46-year-old female who presented with a one-week history of back pain, fever and generalized tonic-clonic seizures that progressed to status epilepticus. The video electroencephalography showed epileptiform discharges on both frontotemporal regions. Neuroimaging showed hyperintensities on the bilateral insula and temporal lobes. The cerebrospinal fluid showed elevated pressure and was positive for HHV-6. She was given ganciclovir and a total of eleven antiepileptic drugs. Despite these medications, she developed refractory status epilepticus and eventually succumbed due to multiple medical complications.

Conclusion: This case highlights HHV-6 encephalitis as an important diagnostic consideration in patients presenting with refractory status epilepticus, regardless of immune status.

Objective: Health-related quality of life (HRQoL) has become an important outcome for neurosurgical patients underwent surgery. The authors utilized the European Organization for Research and Treatment of Cancer Quality of Life Core Questionnaire (EORTC QLQ C-30) to assess the quality of life of spheno-orbital meningiomas patients after surgery in one national central referral hospital in Indonesia.

Methods: In this cross-sectional study, medical record-based data of 40 spheno-orbital meningioma patients who underwent surgery at Dr. Cipto Mangunkusumo General Hospital, Jakarta, Indonesia from October-December 2016 were analyzed. Differences between pre-and postsurgical EORTC scores was examined using the Student's t test.

Results: There was a statistically significant increase of EORTC QLQ-C30 scores in global health status, role functioning, emotional functioning, cognitive functioning, and social functioning variables, and statistically significant decrease for clinical symptoms variables.

Conclusion: Evidently, there was improvement in HRQoL in spheno-orbital meningiomas patients after surgery, in whom EORTC QLQ-C30 can be used to assess the HRQoL prior to and after surgical procedures.

Central pontine myelinolysis (CPM) is a neurological disorder characterized by demyelination on the bottom of pons. CPM is known to be the most common clinical presentation of osmotic demyelination syndrome. Osmotic stress formed by rapid correction of hyponatremia in glia cells is thought to be important in pathogenesis. Oligodendroglias are more sensitive to dehydration and volume changes as they are tightly aligned in the pontine. Chronic alcohol use is a rare cause of osmotic demyelination. In chronic alcoholics, central pontine myelinolysis may be asymptomatic or mild symptoms may develop. We presented the case to emphasize that chronic alcoholism is a rare cause of central pontine myelinosis. Keywords: myelinolysis, central pontine, alcoholism.

Background: Rituximab has been increasingly prescribed in the treatment of multiple sclerosis (MS) over recent years. Tumefactive demyelinating lesions can occur at the onset or over the course of MS. Another major cause of these lesions is the side effects of drugs such as natalizumab or fingolimod. This study is a case report of a young MS patient who suffered from tumefactive lesions following the injection of rituximab.

Case presentation: The patient was an 18-year-old man with MS who developed double vision, imbalance, and quadriparesis symptoms followed by a decrease in his consciousness two days after administration of rituximab. Tumefactive lesions were observed in the patient's brain magnetic resonance imaging (MRI).

Conclusion: Rituximab should be considered as a potential cause of tumefactive demyelinating lesions in patients with MS.

Objective: Chronic inflammatory processes involving the vascular wall may induce atherosclerosis. Immune-inflammatory processes proceed throughout all stages of acute stroke. We investigated the association of three immune-inflammatory markers, namely systemic immune-inflammation index (SII), neutrophil-to-lymphocyte ratio (NLR), and neutrophil count (NC), with prehospital delay and clinical features in patients with acute ischemic stroke.

Methods: We retrospectively enrolled 2543 inpatients admitted within 4 days of symptom onset from May 2010 to February 2020. Patients were stratified into three groups: Group A, comprising 161 patients with tissue plasminogen activator (tPA) treatment; Group B, comprising 415 patients who were eligible for tPA treatment; and Group C, comprising all 2543 patients.

Results: The levels of all three immune-inflammatory markers had positive linear correlations with onsetto- emergency room time, initial National Institutes of Health Stroke Scale (NIHSS) scores, and discharge modified Rankin Scale scores. In Group B, levels of follow-up, but not initial, immuneinflammatory markers were higher in patients with unfavorable outcomes. Common significant predictors of in-hospital complications and unfavorable outcomes were age > 72 years, female sex, NIHSS > 4, diabetes mellitus, and all three immune-inflammatory markers. When combined with other predictors, NC > 7.2 × 103/mL achieved optimal predictive performance (0.794) for in-hospital complications, and SII > 651, NLR > 2.9, and NC > 7.2 × 103/mL had equal predictive performance up to 0.859 for unfavorable outcomes.

Conclusions: Immune-inflammatory markers dynamically increased from symptom onset of acute ischemic stroke in patients eligible for thrombolytic therapy. Higher levels of immune-inflammatory markers suggest more in-hospital complications and unfavorable short-term outcomes.