Clinical Features and Prognosis in Chinese Patients With Dipeptidyl–Peptidase–Like Protein 6 Antibody–Associated Encephalitis

Objectives:Anti-dipeptidyl–peptidase–like protein 6 (anti-DPPX) encephalitis an extremely rare type of immune-mediated encephalitis. This study aimed to analyze the electroclinical characteristics and prognosis of anti-DPPX encephalitis.Methods:Five patients (all male) with anti-DPPX encephalitis in East China from January 2016 to October 2021 was retrospective analyzed. Electroclinical features and outcomes were reviewed.Results:All five patients were male. The media age at disease onset was 32 years old with a range of 14–56 years. The main symptoms included psychiatric disturbances (2/5), amnesia (4/5), confusion (3/5), and seizures (3/5). Migrating myoclonus were identified in patient 4 with positive DPPX and contactin-associated protein-like 2 antibodies in blood. All of the patients had positive DPPX antibodies in serum. Only one of them had positive antibody in the cerebrospinal fluid. EEG showed diffuse slowing in two patients, but no epileptiform discharges were observed. Eighty percent (4/5) of the patients showed normal brain magnetic resonance imaging. After immunotherapy, improvement of neuropsychiatric symptoms from all of the patients was observed. Over a mean follow-up of 30.8 weeks, all of the patients had marked improvement in the modified Rankin Scale. To date, no tumors were not observed in any patients.Conclusions:Anti-DPPX encephalitis mainly presents as neuropsychiatric symptoms. Cooperation of DPPX antibodies and CASPR2 antibodies might have contributed to the migration of myoclonus in the patient 4. Prompt immunotherapy often results in improvement.

Comparison of transcranial doppler ultrasound indices in large and small vessel disease cerebral infarction

Background: Atherosclerotic involvement of large and small cerebral arteries leading to infarction is among the most prevalent subtypes of stroke worldwide. The hemodynamic changes due to these arterial pathologies can be studied non-invasively and in real-time by using transcranial Doppler (TCD) techniques. TCD indices of the studied arteries may guide the clinician in differentiating these two underlying arterial pathologies. Methods: A cross-sectional study of patients with small and large vessel types of cerebral infraction based on the Trial of ORG 10172 in Acute Stroke Treatment (TOAST) stroke classification was undertaken in the inpatient population of neurology service of Razi Hospital, Tabriz, Iran, from October 2018 to October 2019. After clinical diagnosis, all cases underwent TCD studies, brain magnetic resonance imaging (MRI), and brain and cervical four-vessel magnetic resonance angiography (MRA). The results of TCD indices related to major arteries of the circle of Willis were tabulated and compared between large and small vessel subtypes of cerebral infarction. Results: A statistically significant difference between right middle cerebral artery (MCA) pulsatility index (PI), left MCA PI, right internal carotid artery (ICA) PI, end-diastolic velocity (EDV), left ICA PI, left ICA EDV, left anterior cerebral artery (ACA) PI, and right vertebral artery (VA) PI measures of the two groups was seen (P < 0.05). In comparison to the large vessel group, left ACA, right VA, and bilateral MCAs and ICAs in the small-vessel stroke group demonstrated an elevated PI. Conclusion: A significant increase of PI occurs in the majority of intracranial arteries of patients with small vessel stroke. This makes PI a valuable marker for differentiating strokes with different underlying pathophysiologies.

Brain ageing in schizophrenia: evidence from 26 international cohorts via the ENIGMA Schizophrenia consortium

Schizophrenia (SZ) is associated with an increased risk of life-long cognitive impairments, age-related chronic disease, and premature mortality. We investigated evidence for advanced brain ageing in adult SZ patients, and whether this was associated with clinical characteristics in a prospective meta-analytic study conducted by the ENIGMA Schizophrenia Working Group. The study included data from 26 cohorts worldwide, with a total of 2803 SZ patients (mean age 34.2 years; range 18-72 years; 67% male) and 2598 healthy controls (mean age 33.8 years, range 18-73 years, 55% male). Brain-predicted age was individually estimated using a model trained on independent data based on 68 measures of cortical thickness and surface area, 7 subcortical volumes, lateral ventricular volumes and total intracranial volume, all derived from T1-weighted brain magnetic resonance imaging (MRI) scans. Deviations from a healthy brain ageing trajectory were assessed by the difference between brain-predicted age and chronological age (brain-predicted age difference [brain-PAD]). On average, SZ patients showed a higher brain-PAD of +3.64 years (95% CI: 3.01, 4.26; I2 = 55.28%) compared to controls, after adjusting for age and sex (Cohen's d = 0.50). Among SZ patients, brain-PAD was not associated with specific clinical characteristics (age of onset, duration of illness, symptom severity, or antipsychotic use and dose). This large-scale collaborative study suggests advanced structural brain ageing in SZ. Longitudinal studies of SZ and a range of mental and somatic health outcomes will help to further evaluate the clinical implications of increased brain-PAD and its ability to be influenced by interventions.

Susac Syndrome: an uncommon cause of impaired vision

A 35-year-old Caucasian woman presented an abrupt onset of bilateral impaired vision, and arrived to our attention two weeks later. She had a previous episode of mild dizziness. She underwent a fluorescein angiography showing branch retinal artery occlusions and a brain magnetic resonance imaging (MRI) revealing several supraand infratentorial FLAIR-hyperintense white matter lesions, two with contrast enhancement. Thrombophilic, autoimmune and infective (including Human Immunodeficiency Virus, Borrelia burgdorferi, Hepatitis B Virus, Hepatitis C Virus, Herpes Simplex Virus 1-2, Varicella Zoster Virus) screening was negative. Cerebrospinal fluid analysis showed intrathecal IgG synthesis. We suspected a Primary Central Nervous System Vasculitis, and intravenous steroids were started. Three months later a second brain MRI showed seven new lesions without contrast enhancement, and she revealed a cognitive impairment and bilateral hearing loss. Reviewing the clinical history and MRI, she fulfilled diagnostic criteria for Susac syndrome. She had two cycles of cyclophosphamide, and recovered in 6 months and then remained stable with metotrexate.

Bilateral Optic Neuritis Caused by Meningococcal Meningoencephalitis

In this article, we described a case of pathogen-induced bilateral optic neuritis accompanying meningococcal meningoencephalitis in an adolescent male. A 15-year-old boy presented to our emergency room due to progressive severe headache, stiff neck, diffuse extremity pain, fever, and nausea concerning meningoencephalitis. Intravenous ceftriaxone, metamizole, and ondansetron were started immediately. Due to acute autonomic dysregulation and development of petechiae, he was transferred to the pediatric intensive care unit. Next morning, the patient developed severe visual impairment. Investigations revealed elevated C-reactive protein, procalcitonin and leucocyte count, decreased platelet count, and clotting activation. Cerebrospinal fluid (CSF) analysis revealed increased leucocyte count, protein, and decreased glucose concentration with pathological CSF cytology. Brain magnetic resonance imaging revealed an increased contrast enhancement in the optic nerve sheath, consistent with acute bilateral optic neuritis. He was started on high-dose intravenous pulse methylprednisolone therapy. After treatment with ceftriaxone und pulse steroids, the patient was discharged in good condition without any visual sequel.

Immune Checkpoint Inhibitor-Induced Limbic Encephalitis during Treatment with Atezolizumab in a Patient with Small-Cell Lung Cancer: A Case Report and Review of the Literature

Paraneoplastic neurological syndrome (PNS) is associated with malignancies, including small-cell lung cancer. Recently, PNS cases among patients with small-cell lung cancer (SCLC) induced by immune checkpoint inhibitors have increased. We herein report a 66-year-old man with SCLC who developed disorientation, dysphagia, and gait disturbance after three courses of treatment with atezolizumab. Brain magnetic resonance imaging revealed a high-intensity area in the bilateral temporal lobes. Blood test results were positive for anti-Hu and anti-Zic4 antibodies, which led to the diagnosis of limbic encephalitis as PNS. Some symptoms improved with intravenous administration of steroids and immunoglobulins.

Acute to Subacute Spinal Cord Infarction Mimicking Acute Multiple Sclerosis: Usefulness of Diffusion-Weighted MRI for Diagnosis

Background: Spinal cord infarction (SCI) is difficult to diagnose because of its rarity, unknown etiology, and unestablished diagnostic criteria. Additionally, the timeline of SCI has not been studied in detail, as few studies using diffusion-weighted image (DWI) sequences of the spine of a small target population have been previously conducted. Case Study: A 56-year-old male with underlying arrhythmia suddenly developed visual field defects on the right side, pain in the left upper extremity, and a tingling sensation in the left hand. Brain Magnetic resonance imaging (MRI) revealed acute to subacute stages of multifocal brain infarction. On additional cervical spinal MRI, it showed atypical MRI findings of SCI, considered late acute to early subacute phase, which were similar to those seen in the acute phase of multiple sclerosis (MS). Additional DWI revealed restricted diffusion. From these findings, it could be inferred that the patient’s SCI occurred at the same time as the multifocal brain infarctions caused by atrial fibrillation. Conclusion: A DWI sequence of spine MRI could be helpful in the diagnosis of acute to subacute phase SCI and in differentiating with acute MS.

Prevalence, distribution, and clinical associations of suspected postictal changes on brain magnetic resonance imaging in epileptic dogs

OBJECTIVE To determine the prevalence of presumed postictal changes (PC) on brain MRI in epileptic dogs, describe their distribution, and recognize possible correlations with different epilepsy features. ANIMALS 540 client-owned dogs with epilepsy and a complete medical record that underwent brain MRI at 4 veterinary referral hospitals between 2016 and 2019. PROCEDURES Data were collected regarding signalment, seizure type, seizure severity, time between last seizure and MRI, and etiological classification of epilepsy. Postictal changes were considered when solitary or multiple intraparenchymal hyperintense lesions were observed on T2-weighted and fluid-attenuated inversion recovery images and were hypointense or isointense on T1-weighted sequences, which were not confined to a vascular territory and showed no to mild mass effect and no to mild contrast enhancement. RESULTS Sixty-seven dogs (12.4%) showed MRI features consistent with PC. The most common brain sites affected were the piriform lobe, hippocampus, temporal neocortex, and cingulate gyrus. Dogs having suffered cluster seizures or status epilepticus were associated with a higher probability of occurrence of PC, compared to dogs with self-limiting seizures (OR 2.39; 95% confidence interval, 1.33 to 4.30). Suspected PC were detected both in dogs with idiopathic epilepsy and in those with structural epilepsy. Dogs with unknown-origin epilepsy were more likely to have presumed PC than were dogs with structural (OR 0.15; 95% confidence interval, 0.06 to 0.33) or idiopathic epilepsy (OR 0.42; 95% confidence interval, 0.20 to 0.87). Time between last seizure and MRI was significantly shorter in dogs with PC. CLINICAL RELEVANCE MRI lesions consistent with PC were common in epileptic dogs, and the brain distribution of these lesions varied. Occurrence of cluster seizures or status epilepticus, diagnosis of unknown origin epilepsy, and lower time from last seizure to MRI are predictors of suspected PC.