eNeurologicalScieNeurologicalSci Vol 29

December 2022

ABCs of Headache

Headache: Treatment update

Oyindamola I. Ogunlaja, Peter J. Goadsby

  • Article 100420
  • https://doi.org/10.1016/j.ensci.2022.100420
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  • Abstract

    Primary headache disorders in particular migraine are one of the most common causes of disability worldwide. Given the high burden of migraine in terms of disability, there has been an effort to develop migraine specific therapies that has led to the availability of new drugs including 5HT1F receptor agonists-ditans (lasmiditan), small molecule calcitonin gene-related peptide (CGRP) receptor antagonists-gepants: (ubrogepant, rimegepant, atogepant) and anti-CGRP monoclonal antibodies (erenumab, fremanezumab, galcanezumab and eptinezumab).

    However, some of these treatments incur a high cost and may not be a feasible option for most patients in resource limited settings. Lasmiditan and the gepants are a good option for patients with moderate-severe migraine attacks who cannot use triptans due variously to poor tolerability, or cardio- or cerebrovascular disease. For practical purposes, the new anti-CGRP monoclonal antibodies are best reserved for patients who have failed to have efficacy or had intolerable side effects from multiple traditional oral preventives.

Barriers to headache care in low- and middle-income countries

Dominique Mortel, Nfwama Kawatu, Timothy J. Steiner, Deanna Saylor

  • Article 100427
  • https://doi.org/10.1016/j.ensci.2022.100427
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  • Abstract

    Headache disorders are a common cause of disability globally and lead not only to physical disability but also to financial strain, higher rates of mental health disorders such as depression and anxiety, and reduced economic productivity which negatively impacts gross domestic product (GDP) on a national scale. While data about headache are relatively scarce in low- and middle-income countries (LMICs), those available suggest that headache disorders occur on a similar scale in LMICs as they do in high-income countries. In this manuscript, we discuss common clinical, political, economic and social barriers to headache care for people living in LMICs. These barriers, affecting every aspect of headache care, begin with community perceptions and cultural beliefs about headache, include ineffective headache care delivery systems and poor headache care training for healthcare workers, and extend through fewer available diagnostic and management tools to limited therapeutic options for headache. Finally, we review potential solutions to these barriers, including educational interventions for healthcare workers, the introduction of a tiered system for headache care provision, creation of locally contextualized diagnostic and management algorithms, and implementation of a stepped approach to headache treatment.


Original Articles

Motor cortical plasticity and its correlation with motor symptoms in Parkinson's disease

Shotaro Moriyasu, Takahiro Shimizu, Makoto Honda, Yoshikazu Ugawa, Ritsuko Hanajima

  • Article 100422
  • https://doi.org/10.1016/j.ensci.2022.100422
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  • Abstract

    The relationship between abnormal cortical plasticity and parkinsonian symptoms remains unclear in Parkinson's disease (PD).

    We studied the relationship between their symptoms and degree of Long-term potentiation (LTP)-like effects induced by quadripulse magnetic stimulation (QPS) over the primary motor cortex, which has a small inter-individual variability in humans.

    Our results suggest that motor cortical plasticity relate with mechanisms underlying bradykinesia and rigidity in the upper limb muscles. LTP induced by QPS may be used as an objective marker of parkinsonian symptoms.

Preliminary seroprevalence study of neurotropic virus antibodies in Nodding syndrome

Raquel Valdes Angues, Valerie S. Palmer, Rajarshi Mazumder, Caesar Okot, Peter S. Spencer

  • Article 100423
  • https://doi.org/10.1016/j.ensci.2022.100423
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  • Abstract

    Nodding syndrome (NS) is a mostly East African pediatric epileptiform encephalopathy of unknown etiology that shares some clinical features with measles-associated subacute sclerosing panencephalitis (SSPE) and progressive rubella panencephalitis. Two independent studies in northern Uganda identified an association between NS and prior measles infection, while an earlier study in South Sudan found an inverse association. We report preliminary serologic analyses of antibodies to measles (MV), rubella (RV), HSV-1, and CMV viruses in northern Ugandan children with NS and Household (HC) and Community (CC) Controls. Only MV-positive titers were significantly different (3-fold and > 2-fold) in NS relative to HC and HC + CC, respectively. While these results are consistent with greater prior measles infection in Ugandan persons with NS, further studies are needed to determine whether Measles virus (MV) plays any role in the etiology and pathogenesis of NS. Resolving this issue will be invaluable for the thousands of children at risk for this devastating yet often neglected condition.

Discussing brain magnetic resonance imaging results for neonates with hypoxic-ischemic encephalopathy treated with hypothermia: A challenge for clinicians and parents

Ariel Cascio, Amaryllis Ferrand, Eric Racine, Marie St-Hilaire, ... Pia Wintermark

  • Article 100424
  • https://doi.org/10.1016/j.ensci.2022.100424
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  • Abstract

    Clinicians use brain magnetic resonance imaging (MRI) to discuss neurodevelopmental prognosis with parents of neonates with hypoxic-ischemic encephalopathy (HIE) treated with therapeutic hypothermia (TH).

    To investigate how clinicians and parents discuss these MRI results in the context of HIE and TH and how these discussions could be facilitated and more meaningful for parents.

Neurological evaluation of microcephalic children with Zika syndrome and congenital cytomegalovirus infection

Lavinia Schüler Faccini, Luciana Friedrich, Sara Kvitko de Moura, Fernanda Diffini Santa Maria, Steice da Silva Inácio de Bone

  • Article 100417
  • https://doi.org/10.1016/j.ensci.2022.100417
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  • Abstract

    The association between the virus prenatal infection by Zika virus (ZIKV) and central nervous system disorders has been well established and it has been described as the Congenital Syndrome Associated to the Zika Virus (CSZ). However, the neurological development in those patients is still an object of study. The main differential diagnosis is the Cytomegalovirus (CMV).

    This paper aims to describe the involvement of microcephalic patients affected by the congenital infection by the Zika Virus or CMV.

Decompressive hemicraniectomy for stroke by race/ethnicity in the United States

Alain Lekoubou, Cyril Tankam, Kinfe G. Bishu, Bruce Ovbiagele

  • Article 100421
  • https://doi.org/10.1016/j.ensci.2022.100421
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  • Abstract

    Racial and ethnic differences in the performance of indicated neurosurgical procedures have been reported. However, it is not clear whether there are racial or ethnic differences in the performance of decompressive hemicraniectomy (DHC) for acute ischemic stroke. This study evaluated the rate, trends, and independent association of race and ethnicity with DHC among hospitalized ischemic stroke patients in the United States.

Temporal intermittent rhythmic theta activity (TIRTA): A marker of epileptogenicity?

Jonah Fox, Niyatee Samudra, Michael Johnson, Mohammad Junaid Humayun, Bassel W. Abou-Khalil

  • Article 100433
  • https://doi.org/10.1016/j.ensci.2022.100433
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  • Abstract

    To describe a novel EEG rhythm, temporal intermittent rhythmic theta activity (TIRTA), and its potential association with epilepsy.

    In this preliminary report we suggest that TIRTA may be a novel marker of potential epileptogenicity, possibly representing a higher frequency variant of TIRDA.

Serum inflammatory and brain injury biomarkers in COVID-19 patients admitted to intensive care unit: A pilot study

Stelios Kokkoris, Elisavet Stamataki, Giorgos Emmanouil, Christina Psachoulia, ... Christina Routsi

  • Article 100434
  • https://doi.org/10.1016/j.ensci.2022.100434
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  • Abstract

    The aim of this study was to measure serum brain injury biomarkers in patients with COVID-19 admitted to intensive care unit (ICU), without evidence of brain impairment, and to determine potential correlations with systemic inflammatory markers, illness severity, and outcome.

Albuminuria, cognition, and MRI biomarkers of cerebrovascular disease in American Indians of the Zuni Pueblo

Sephira G. Ryman, Arvind Caprihan, Gary Rosenberg, Jillian Prestopnik, ... Vallabh Shah

  • Article 100438
  • https://doi.org/10.1016/j.ensci.2022.100438
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  • Abstract

    Elevated urine albumin to creatinine ratio (UACR) is associated with cerebrovascular disease and cognitive impairment in older adults, though few studies have evaluated these relationships in midlife. This is particularly important to assess in American Indian populations, which are disproportionately impacted by diabetes and kidney disease. Additionally, evidence suggests that biomarkers may perform differently in underrepresented groups, thus, it is crucial to validate biomarkers in this unique population.

    Our pilot study validated the use of MRI biomarkers of cerebrovascular disease in this unique cohort of American Indians.

Objective perimetry and progression of multiple sclerosis

Ted Maddess, Corinne F. Carle, Emilie M.F. Rohan, Jonathan Baird-Gunning, ... Christian J. Lueck

  • Article 100430
  • https://doi.org/10.1016/j.ensci.2022.100430
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  • Abstract

    We re-examined the per-region response amplitudes and delays obtained from multifocal pupillographic objective perimetry (mfPOP) after 10 years in 44 persons living with multiple sclerosis (PwMS), both to examine which parts of the visual field had progressed in terms of response properties and to examine if the baseline data could predict the overall progression of disease.

    mfPOP may provide a rapid, convenient method of monitoring and predicting MS progression.

Verification of the association between cognitive decline and olfactory dysfunction using a DEmentia screening kit in subjects with Alzheimer's dementia, mild cognitive impairment, and normal cognitive function (DESK study): A multicenter, open-label, interventional study

Takahiro Fukumoto, Toshifumi Ezaki, Katsuya Urakami

  • Article 100439
  • https://doi.org/10.1016/j.ensci.2022.100439
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  • Abstract

    Olfactory dysfunction may be an early symptom of degenerative neurological disorders such as mild cognitive impairment (MCI), which may progress to cognitive decline and Alzheimer's disease (AD). We investigated the relationship between cognitive decline and olfactory dysfunction in healthy controls and patients with MCI or AD using the DEmentia Screening Kit (DESK), an olfactory identification assessment tool designed for Japanese populations.

    The DESK tool may discriminate between healthy individuals and those with MCI or AD, facilitating early screening for cognitive decline among Japanese patients, although the effect of age on DESK olfaction scores has not been fully explored.


Case Reports

Serial assessment of multimodality imaging in anti-leucine-rich glioma-inactivated 1 antibody encephalitis: A case report

Takafumi Wada, Hitoshi Mori, Katsuro Shindo

Acute subdural hematoma due to acute myeloid leukemia and B-cell lymphoma

Denis Babici, Pamraj Sharma, Jason DeGregorio, Brian Snelling, Khalid Hanafy

  • Article 100425
  • https://doi.org/10.1016/j.ensci.2022.100425
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  • Abstract

    Chloroma, is an rare malignant tumor characterized by the extramedullary blast proliferation of myeloid lineages that subsequently change the normal architecture of surrounding tissues. Because this is very rare disease, primary central nervous system myeloid sarcoma has not been widely reported. Myeloid sarcoma, as a group of heterogenetic diseases, presents with differential clinical and histological pictures depending upon the primary affected site. We are presenting a 77-year-old gentleman, with past medical history of chronic lymphoblastic leukemia that was not on any treatment and who presented with complaints of lethargy, disorientation, and weakness. CT scan of the head showed left-sided subdural hematoma with significant mass effect with left-to-right shift. Craniotomy and hematoma evacuation was performed and hematoma and subdural lesion was sent for pathological evaluation. Histologic examination of the hematoma revealed solid sheets of small-medium sized atypical hematopoietic cells that was imbedded in an acute and chronic hematoma. To confirm the final diagnosis, flow cytometry was performed and showed two neoplastic clones, consistent with acute myeloid leukemia with monocytic differentiation and a separate mature B-cell lymphoma. To our knowledge we are presenting the first case in the literature of the Intracranial acute myeloid leukemia/myeloid sarcoma and B-cell lymphoma that was found in the same brain lesion.

Aseptic meningitis in Kikuchi-Fujimoto Disease - Rare manifestation of a rare disease

Masooma Hashmat, Sadaf Iftikhar, Muhammad Aemaz Ur Rehman, Aqeeb Ur Rehman, ... Muhammad Ebaad Ur Rehman

  • Article 100429
  • https://doi.org/10.1016/j.ensci.2022.100429
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  • Abstract

    Kikuchi-Fujimoto Disease (KFD) is a rare disease marked by necrotizing lymphadenitis, often presenting as unilateral cervical lymphadenopathy, along with various extranodal manifestations such as fever, skin rash, hepatosplenomegaly, and arthritis, etc. KFD is thought to be secondary to either a viral infection or an autoimmune process, however, evidence in favor of both models is scarce and non-definitive. We report a case of a young female who presented with persistent high-grade fever, bilateral cervical and axillary lymphadenopathy, and leukopenia. Excisional biopsy of affected lymph nodes revealed well-circumscribed foci of necrosis with karyorrhectic debris and scattered fibrin deposits characteristic of KFD. The patient was promptly initiated on non-steroidal anti-inflammatory drugs (NSAIDs), however, despite an early improvement in symptoms, the patient soon developed aseptic meningitis, a rare neurological complication of KFD. Intravenous followed by oral corticosteroid therapy reported a good prognosis, with no observable residual neurological deficits. Knowledge of the disease and its complications significantly helped in the avoidance of unnecessary investigations and a delay in treatment.

Case report: Near-complete cortical hearing loss caused by sequential development of bilateral putaminal hemorrhage

Yoshito Arakaki, Takeshi Yoshimoto, Hiroyuki Ishiyama, Tomotaka Tanaka, ... Masafumi Ihara

  • Article 100431
  • https://doi.org/10.1016/j.ensci.2022.100431
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  • Abstract

    Intracerebral hemorrhage with sudden hearing loss as the initial symptom is rare. A right-handed man with a history of right putaminal hemorrhage developed near-complete hearing loss and right hemiplegia and was taken to our hospital by ambulance. Non-contrast computed tomography demonstrated acute intracerebral hemorrhage in the left putamen. A region of old right putaminal hemorrhage involving the right temporal stem was also shown on fluid-attenuated inversion recovery. Standard pure-tone audiometry showed right-dominant bilateral sensorineural hearing loss. More than 2 months after onset, the bilateral sensorineural hearing loss gradually improved without interfering with daily life. Detailed history-taking indicated that the old hemorrhage in the right putamen 12 years previously had caused sudden left-dominant bilateral hearing impairment due to asymmetric but bilateral innervation from the auditory nerve. The bilateral damage to the temporal stem involving acoustic radiation resulted in temporary near-complete hearing loss after the recurrence, but the amelioration of edema in the left temporal stem may have resulted in partial recovery of the hearing loss. This patient's clinical progression suggests that the auditory tract ascends mainly on the side opposite the ear and may explain the left dominance in the level of acoustic radiation.

MOG antibody associated disease (MOGAD) presenting with extensive brain stem encephalitis: A case report

Elisabeth Olbert, Cornelia Brunner, Naela Alhani, Christian Našel, Walter Struhal

  • Article 100432
  • https://doi.org/10.1016/j.ensci.2022.100432
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  • Abstract

    Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a relatively new entity of demyelinating diseases, clinically presenting with optic neuritis, transverse myelitis, or encephalic symptoms. Typical radiological features include demyelinating cerebral and spinal lesions, cortical involvement, leptomeningeal enhancement, or tumefactive lesions. Here we present a rare case of a young patient with extensive brain stem lesion on the MRI while exhibiting nystagmus, singultus and somnolence.

    Brain stem affection in MOGAD is rare. However, in patients presenting with an unclear brain stem encephalitis the possibility of MOGAD should be considered and tested using MOG antibodies. In case of a positive testing treatment with steroids and immunoglobulins seems recommendable.

Cerebral syphilitic gumma mimicking a brain tumor that enlarged temporarily after commencing antibiotic treatment

Yoshiaki Takahashi, Nobutoshi Morimoto, Mizuki Morimoto, Shunsuke Mori, ... Toru Yamashita

  • Article 100436
  • https://doi.org/10.1016/j.ensci.2022.100436
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  • Abstract

    In this case report, we describe a 60-year-old man who presented with headaches for 1 year and mild confusion for 3 weeks and was initially diagnosed as having a cerebral tumor on the basis of finding a round lesion in the right lenticular nucleus with ring enhancement on gadolinium-enhanced T1-weighted brain magnetic resonance imaging. However, the discovery of positive serology for Treponema pallidum infection on routine tests on admission prompted analysis of cerebrospinal fluid, which was also positive on Treponema pallidum hemagglutination (TPHA), rapid plasma reagin (RPR), and treponemal antibody absorption (FTA-ABS) tests. Thus, he was diagnosed as having an intracranial syphilitic gumma. After commencing treatment with penicillin G, the lesion temporarily increased in size, but subsequently resolved completely with continuing antibiotic treatment. In the present era of increasing prevalence of syphilitic infection and because they are eminently treatable, syphilitic gummas should be included in the differential diagnosis of apparent brain tumors. Additionally, temporary enlargement of a probable gumma after instituting antibiotic treatment should not prompt cessation or change of the antibiotics.

Impact of blood flow volume in determining the destination of intracardiac thrombi using computational fluid dynamics

Yorito Hattori, Naoki Tagawa, Masanori Nakamura, Masafumi Ihara

  • Article 100437
  • https://doi.org/10.1016/j.ensci.2022.100437
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  • Abstract

    Atrial fibrillation (AF) induces cardioembolic stroke due to intracardiac fibrin thrombus formation. Although it is well established that a cardioembolic stroke affects the anterior circulation more frequently than it affects the posterior circulation, the destination where the thrombi migrate when cardioembolic stroke occurs in each patient remains unclear. We present a critical case wherein a bilateral internal carotid artery (ICA) territory infarction was diagnosed in a patient with AF who apparently developed nearly simultaneous occlusion in the ICAs bilaterally. A 92-year-old woman with AF who appeared to have developed bilateral occluded common carotid artery (CCA)–ICAs almost simultaneously presented after the sudden onset of coma and quadriplegia and was diagnosed with bilateral ICA territory infarction. The patient died at 4 days after the onset due to the huge infarction. The blood flow in the aorta and the major branches of the aortic arch were examined using computational fluid dynamics (CFD) based on contrast-enhanced computed tomography angiography, which revealed that the right and left CCAs covered larger flow volumes than the other aortic arch branches, suggesting that the intracardiac thrombi migrated into the bilateral CCA–ICAs in the patient. The study findings imply that the fluid dynamic factors of major branches from the aortic arch can be one of the decisive factors for intracardiac thrombus distribution. CFD could simulate patient-specific hemodynamics and may be useful to investigate the susceptibility of the aortic arch branches to occlusion by AF-induced intracardiac emboli.


Letters to the Editor

Remission of idiopathic retroperitoneal fibrosis by ofatumumab as a disease-modifying therapy for multiple sclerosis

Akira Hanazono, Yui Sanpei, Hinako Shimada, Keita Yasuda, ... Masashiro Sugawara

Usefulness of arterial spin labeling in identifying status epilepticus secondary to acquired thrombotic thrombocytopenic purpura

Tomoya Shibahara, Keiji Sakamoto, Fumitaka Yoshino, Mikiaki Matsuoka, ... Hiroshi Nakane