eNeurologicalScieNeurologicalSci Vol 27

June 2022

ABCs of Headache

Migraine in the workplace

Olivia Begasse de Dhaem, Fumihiko Sakai

  • Article 100408
  • https://doi.org/10.1016/j.ensci.2022.100408
  • Download PDF
  • Abstract

    Migraine is prevalent, disabling, and peaks during people's peak productive years. The impact of migraine on people's professional lives, work productivity, and interpersonal relationships at work eventually affects everyone, has a significant detrimental effect on people with migraine, and a huge cost in terms of lost productivity. People with migraine want to work, so they do their best to work despite the varied migraine related and associated symptoms. Most of migraine-related productivity loss (89%) is due to presenteeism. People are less than half effective during a migraine attack due to the pain, migraine symptoms, attack unpredictability, migraine comorbidities, emotional impact, under-diagnosis and under-management, and the stigma. Migraine-related productivity loss may negatively affect people's career choice, job status and/or security, financial status, work relationships, mood, and confidence. Migraine is estimated to represent 16% of total US workforce presenteeism. Thankfully, there are ways to help support people with migraine in the workplace and increase their productivity such as: workplace migraine education programs, workplace migraine education and management programs, migraine-friendly work environment, migraine treatment optimization and advocacy. The example of the successful workplace migraine education and management program developed and run in collaboration between Fujitsu, the Japanase Headache Society, and the International Headache Society Global Patient Advocacy Coalition is discussed.


Review Article

Nodding syndrome: A key role for sources of nutrition?

P.S. Spencer, C. Okot, V.S. Palmer, R. Valdes Angues, R. Mazumder

  • Article 100401
  • https://doi.org/10.1016/j.ensci.2022.100401
  • Download PDF
  • Abstract

    Nodding Syndrome (NS) has occurred among severely food-stressed communities in northern Uganda and several other East African populations that, with their forced physical displacement, have resorted to nutritional support from available wild plants and fungi, some of which have neurotoxic potential. Among the latter is an agaric mushroom with an unknown content of hydrazine-generating agaritine, namely Agaricus bingensis, the unusually wide consumption of which may relate to the low serum levels of vitamin B6 in Ugandan NS subjects relative to controls. Hydrazine-related compounds induce patterns of DNA damage that promote neuropathological changes (tauopathy) reminiscent of those associated with established NS. While the cause of this childhood brain disease is unknown, we encourage increased attention to the role of malnutrition and B6 hypovitaminosis in the etiology of this devastating brain disease.


Original Articles

Recanalization treatment for acute ischemic stroke: 3-month outcome before and after implementation of routine endovascular treatment

Kati Valkonen, Nicolas Martinez-Majander, Gerli Sibolt, Marjaana Tiainen, ... Sami Curtze

  • Article 100394
  • https://doi.org/10.1016/j.ensci.2022.100394
  • Download PDF
  • Abstract

    Endovascular treatment for acute ischemic stroke has become a recommended treatment option for selected patients after several randomized controlled trials have shown the effectiveness of endovascular treatment. Due to the nature of randomized clinical trials, the generalizability to population based real life settings and the resulting benefits remain difficult to estimate.

    We included 896 consecutive patients treated with intravenous thrombolysis (IVT) within 4.5 h of stroke onset between January 2016 and December 2018, who were treated with additional endovascular treatment according to the new evidence when indicated (new-IVT-cohort). This cohort was compared to 913 intravenous thrombolysis patients treated in the 4.5 h time-window between January 2011 and December 2013 before the era of endovascular treatment (old-IVT-cohort).

Favorable benefit–risk ratio with teriflunomide treatment in relapsing-remitting multiple sclerosis: Results of the 2-year, multicenter, prospective, noninterventional TAURUS MS study in Austria

Michael Guger, Michael Matthias Ackerl, Martin Heine, Christiane Hofinger-Renner, ... Fritz Leutmezer

  • Article 100396
  • https://doi.org/10.1016/j.ensci.2022.100396
  • Download PDF
  • Abstract

    A prospective, multicenter, open-label, noninterventional study assessed the efficacy, safety, tolerability, and patient satisfaction with teriflunomide therapy over a 24-month follow-up period under real-world conditions in Austria.

    An all-comer population aged ≥18 years was followed in clinic and office-based settings. The primary objective of the study was the annualized relapse rate after 12 and 24 months of teriflunomide treatment. Patient-reported outcomes included treatment satisfaction, health-related quality of life, and fatigue, and were assessed based on the Short Form Health-36, Fatigue Severity Scale, and Treatment Satisfaction Questionnaire for Medication (TSQM)-9 questionnaires.


Utility of 18F FDG-PET in Parkinsonism in an African population

Ferzana Hassan Amod, Ahmed Iqbal Bhigjee, Nozipho Nyakale

  • Article 100399
  • https://doi.org/10.1016/j.ensci.2022.100399
  • Download PDF
  • Abstract

    Parkinson's Disease remains a diagnostic challenge. Misdiagnosis during life is approximately 25%. Diseases that resemble PD clinically, such as the Parkinsonianplus disorders usually have a poorer prognosis. A diagnostic biomarker is needed to differentiate PD from PPS. Geographical differences in PD prevalence, genetics and environmental factors may suggest a different pathogenesis of PD in Africa which may affect metabolic changes seen on 18F-FDG-PET. We investigated the utility of 18FFDG-PET in differentiating PD from PPS in a real-life clinical setting. The study was conducted at the Movement Disorder Clinic, South Africa. 81 patients with Parkinsonism had fluorine-18-labelled-fluorodeoxyglucose-PET; 53 PD and 28 PPS. Six persons living with HIV and Parkinsonism were included. Of the 22 Black African patients, 21 had PD and only one had a PPS. Image-based diagnosis was made by visual interpretation aided by statistical parametric mapping (SPM) analysis by a Nuclear Medicine Physician blinded to the clinical diagnosis. This was compared to the final clinical diagnosis made by two Movement disorder Neurologists blinded to the 18F-FDG-PET diagnosis. Patients were followed up for a median of 4 years. 18F-FDGPET diagnosis was in agreement with final clinical diagnosis in 91% of all subjects (90% PD, 93% all PPS). Our paper reports the clinically realistic sample of patients seen with Parkinsonism in Africa. The present data shows that 18F-FDG-PET can distinguish PD from PPS with good accuracy. Few Black Africans present with an Atypical Parkinsonian syndrome. The pattern of metabolism in PLH-PD is similar to PD patients without HIV.

The cross-sectional area of the vagus nerve is not reduced in Parkinson's disease patients

Laura C.J. Sijben, Werner H. Mess, Uwe Walter, A. Miranda L. Janssen, ... Marcus L.F. Janssen

  • Article 100400
  • https://doi.org/10.1016/j.ensci.2022.100400
  • Download PDF
  • Abstract

    Recent studies have revealed the importance of the gut brain axis in the development of Parkinson's disease (PD). It has also been suggested that the cross-sectional area (CSA) of the vagus nerve can be used in the diagnosis of PD. Here, we hypothesize that the CSA of the vagus nerve is decreased in PD patients compared to control participants.

    In this study we measured the CSA of the vagus nerve on both sides in 31 patients with PD and 51 healthy controls at the level of the common carotid artery using high-resolution ultrasound.

Participation of visual association areas in social processing emerges when rTPJ is inhibited

Jorge Hevia-Orozco, Azalea Reyes-Aguilar, Erick H. Pasaye, Fernando A. Barrios

  • Article 100407
  • https://doi.org/10.1016/j.ensci.2022.100407
  • Download PDF
  • Abstract

    During a social evaluation, the right temporo-parietal junction (rTPJ) plays an important role according to its contribution in making inferences about the mental states of others. However, what is the neural response if rTPJ function is inhibited during a mentalizing task? In this study, participants played the Dictator Game with two confederates: one playing cooperation (C) and other playing non-cooperation (NC) role and then they were scanned during a mentalizing test. However, we inhibited rTPJ using transcranial magnetic stimulation (TMS) after they played the game and before they were scanned. In this test, participants read negative (Neg) or positive (Pos) personal situations and then they watched confederate's pictures. Images from the TMS group were compared against controls with no TMS stimulation. After statistical comparison, we found a significantly higher activity in right and left visual association areas (BA 18) during the NCPos > NCNeg condition in the TMS group compared with the No-TMS group. Same visual association areas have been described before when participants are processing visual emotional information or when making a fast social categorization. This could reflect a neural mechanism of socio - emotional categorization that emerges after rTPJ inhibition.


Case Reports

Thymoma-associated anti-LGI1 encephalitis and myasthenia gravis: A unique combination with autoantibodies

Akane Satake, Takamura Nagasaka, Takafumi Kurita, Hiroaki Murata, ... Yoshihisa Takiyama

  • Article 100395
  • https://doi.org/10.1016/j.ensci.2022.100395
  • Download PDF
  • Abstract

    We report a 77-year-old woman with a thymoma, anti-LGI1antibody associated encephalitis (LGI1 encephalitis), and MG accompanied by positive anti-acetylcholine receptor antibodies (AchR Ab) and anti-titin antibodies (titin Ab). She was treated with thymomectomy followed by immunosuppressive therapy, which resulted in immediate amelioration of motor weakness and gradual improvement of cognitive impairment over the next two years. LGI1 Ab were positive at two months after thymomectomy, followed by negative conversion demonstrated on 1 year examination. The AchR Ab level had gradually decreased but titin Ab was positive on re-examination after two years, although the cognition and motor impairment symptoms had been alleviated. In patients with suspected autoimmune encephalitis, the detection of several autoantibodies including LGI1 and thymomas provides useful information for making an accurate diagnosis.

Gradenigo's syndrome presenting as IX and X cranial nerve palsy without clinically apparent ear infection: A case report and review of literature

Safia Bano, Ahmad Nawaz, Abyaz Asmar, Muhammad Aemaz Ur Rehman, ... Hamid Ali

  • Article 100397
  • https://doi.org/10.1016/j.ensci.2022.100397
  • Download PDF
  • Abstract

    Gradenigo's syndrome (GS) is a triad (otorrhea, abducens nerve palsy, and pain in the trigeminal nerve distribution) of clinical findings that are caused by contiguous spread of petrous apicitis to the nearby neurovascular structures. Petrous apicitis is usually secondary to otitis media but atypical etiologies and absence of the classical triad pose a diagnostic challenge for physicians. We report a rare case of GS in an afebrile 55-year-old male who presented with unilateral headache, dysphagia and hoarseness (IX and X cranial nerve involvement), and diplopia with lateral gaze palsy (VI nerve involvement) in the absence of trigeminal neuralgia or a history of otitis media. Magnetic Resonance Imaging (MRI) revealed hyperintense lesions in the right petrous apex indicating petrous apicitis, the hallmark of GS. Prompt initiation of broad-spectrum antibiotics led to a marked improvement in dysphagia and voice quality on the 4th post-admission day, and complete resolution of symptoms by the end of the fourth week. This shows that GS can present even in the absence of clinically apparent ear infection and cranial nerve palsies may not be limited to the V and VI nerve in all cases. Physicians should be aware of such atypical manifestations as prompt radiological assessment followed by early antibiotics can prevent life-threatening complications from developing.

Cerebral venous sinus thrombosis as the initial presentation of essential thrombocythemia - A case report and literature review

Qudsum Yousaf, Haseeb Amad Khan, Fateen Ata, Adeel Ahmad Khan, ... Zohaib Yousaf

  • Article 100398
  • https://doi.org/10.1016/j.ensci.2022.100398
  • Download PDF
  • Abstract

    Myeloproliferative disorders (MPD) are associated with vascular thrombosis. Common sites for thrombosis are large arteries; however, less commonly, cerebral venous sinus thrombosis (CVST) has also been reported. It is rare to have CVST as an initial presentation of MPD. We discuss a male patient in whose presentation due to CVST led to the diagnosis of essential thrombocythemia (ET). Furthermore, we performed a literature review to evaluate the association of CVST with ET.

High-dose immunoglobulin-dependent chronic inflammatory demyelinating polyneuropathy successfully managed with subcutaneous immunoglobulin using pharmacokinetic analysis

Satomi Hiya, Satoru Fujiwara, Fumiaki Tanaka, Nobuo Kohara, Michi Kawamoto

  • Article 100404
  • https://doi.org/10.1016/j.ensci.2022.100404
  • Download PDF
  • Abstract

    Immunoglobulin G therapy for chronic inflammatory demyelinating polyneuropathy (CIDP) often requires individual dose adjustments because of the heterogeneity of pathogenesis and varying catabolic rates. However, currently available pharmacokinetic studies of immunoglobulin G therapy do not consider individual differences. We conducted a pharmacokinetic study of both intravenous immunoglobulin and subcutaneous immunoglobulin in a single patient with CIDP who was dependent on high-dose immunoglobulin treatment. This patient—a 77-year-old man with symmetrical limb weakness, diffuse demyelination determined by a nerve conduction study, and lacking autoantibodies—was treated with intravenous immunoglobulin and experienced severe fluctuations in symptoms. We transitioned him to subcutaneous immunoglobulin: his serum immunoglobulin G levels stabilised and he experienced symptomatic relief. Monitoring of serum immunoglobulin G concentrations revealed volatile changes following intravenous immunoglobulin administration which stabilised following subcutaneous immunoglobulin treatment. This suggests that subcutaneous immunoglobulin is a preferable long-term treatment option, especially for high-dose immunoglobulin-dependent patients with CIDP.

Rare pitfall in the magnetic resonance imaging of status epilepticus

Mustafa Al-Chalabi, Silvi Bajrami, Nurose Karim, Ajaz Sheikh

  • Article 100405
  • https://doi.org/10.1016/j.ensci.2022.100405
  • Download PDF
  • Abstract

    Brain MRI in Status Epilepticus (SE) is often helpful in diagnosis, lateralization and localization of the seizure focus. MRI changes in SE include predominantly ipsilateral diffusion weighted imaging (DWI) changes in the hippocampus and pulvinar or similar changes involving basal ganglia, thalamus, cerebellum, brain stem and external capsule (Chatzikonstantinou et al., 2011 [1]). These changes are thought to be due to transient vasogenic and cytotoxic edema due to either transient damage or breakdown of blood brain barrier, proportional to the frequency and duration of the epileptic activity (Amato et al., 2001 [2]). Such changes may also be reflected on T2- weighted and T2-Fluid-Attenuated Inversion Recovery (FLAIR) sequences of MRI.

    Herein, we present a case of a transient FLAIR cerebrospinal fluid (CSF) hyperintensity on the second MRI brain in a patient with focal status epilepticus. This imaging finding led to diagnostic confusion and was initially thought to represent subarachnoid hemorrhage. However, lumbar puncture, brain computed tomography (CT), and a follow-up brain MRI ruled out that possibility and other CSF pathologies. We concluded that the transient FLAIR changes in the second brain MRI were related to a rare imaging pitfall caused by Gadolinium enhancement of CSF on the FLAIR sequence, popularly referred to as hyperintense acute reperfusion marker (HARM).


Letters to the Editor

An autopsy case of TAFRO syndrome with multiple cerebral infarctions caused by small vessel pathology

Chihiro Matsuoka, Hisashi Takahashi, Rei Yasuda, Shinji Ashida, ... Toshiki Mizuno

Autoimmune glial fibrillary acidic protein astrocytopathy with delayed abnormal magnetic resonance imaging findings

Shohei Imanaka, Yuwa Oka, Akio Kimura, Takayoshi Shimohata, Sadayuki Matsumoto

Seronegative neuromyelitis optica spectrum disorder in primary familial brain calcification with PDGFB variant

Masahiro Biyajima, Yuya Kobayashi, Kiyoshi Nakafuji, Rie Watanabe, ... Hiroyuki Yahikozawa