eNeurologicalScieNeurologicalSci Vol 22

March 2021

Review Article

Treatment challenges in idiopathic extracranial ICA vasospasm case report and review of the literature

Edith Graham, Karen Orjuela, Sharon Poisson, Jose Biller

  • Article 100304
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  • https://doi.org/10.1016/j.ensci.2020.100304
  • Abstract

    Idiopathic extracranial internal carotid artery (ICA) vasospasm is a rare pathological phenomenon that may lead to stroke in young patients. We report a case of an 18 year-old female with recurrent extracranial ICA vasospasm since age thirteen. We summarize published data related to this condition including all twenty-three reported cases of extracranial ICA vasospasm. We describe the various proposed pathophysiological mechanisms underlying this disorder. Various treatment modalities have been attempted but there is no known long-term effective treatment.

An overview of the digital solutions for helping people with aphasia through bibliometric analysis

Ikram Asghar, Oche A. Egaji, Mark Griffiths

  • Article 100311
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  • https://doi.org/10.1016/j.ensci.2021.100311
  • Abstract

    This paper gives insights into recent research developments in the field of digital solutions for people with aphasia and tries to analyse its impact on their rehabilitation. A bibliometric research approach is used for data collection. Relevant studies were extracted from seven major academic databases from years 2000 to 2019 inclusive. The systematic process resulted in 986 studies. The average growth in this field is 4%, which is less compared to other research areas. However, the average citations per paper is 7.27, which represents a medium level of publication quality. Scopus and Web of Science are leading databases for the number of studies (379 and 264) and quality of publications (P-Index: 49.26 and 32.85), respectively. The USA, with 42% of publications, leads this research field, followed by the UK with 15%. Both countries have national aphasia strategies in place through charities (not government level strategies), which potentially contributed to their research leadership. The results show that recent advances in digital solutions have the potential to support people with aphasia. However, further work is needed at both academic and government levels to deliver more research contributions and funding for the rehabilitation of people with aphasia.

Neuropathic pain in Mali: The current situation, comprehensive hypothesis, which therapeutic strategy for Africa?

Youssoufa Maiga, Oumar Sangho, Fatoumata Konipo, Seybou Diallo, ... Julien Nizard

  • Article 100312
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  • https://doi.org/10.1016/j.ensci.2021.100312
  • Abstract

    According to the taxonomy of the International Association for the Study of Pain (IASP 2011), neuropathic pain (NeuP) is defined as "pain caused by a lesion or disease of the somatosensory nervous system". NeuP is currently well-defined clinically, despite a high degree of etiological variation, and it has become a significant public health problem. This work aimed to study the situation regarding NeuP in current practice in Mali, as well as to analyze the therapeutic environment of the patients

    This work confirms that NeuP is encountered frequently in current practice, and its optimal management will involve specific training of carers and improvement of access to the medications recommended in this indication. In light of this issue, we revisit the debate regarding the concept of essential medications and the relevance of taking into account effective medications for the treatment of NeuP.

MRI findings in tubercular radiculomyelitis

Deepali Saxena, Denver Steven Pinto, Anisha S. Tandon, Ravi Hoisala


Original Articles

Short-latency somatosensory-evoked potentials demonstrate cortical dysfunction in patients with Angelman syndrome

Kiyoshi Egawa, Shinji Saitoh, Naoko Asahina, Hideaki Shiraishi

  • Article 100298
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  • https://doi.org/10.1016/j.ensci.2020.100298
  • Abstract

    Angelman syndrome (AS) is neurodevelopmental disorder, causal gene of which is maternally expressed UBE3A. A majority of patients results from the large deletion of relevant chromosome which includes GABAA receptor subunit genes (GABARs) as well as UBE3A (AS Del). We previously reported aberrantly desynchronized primary somatosensory response in AS Del by using magnetoencephalography. The purpose of this study is to estimate cortical and subcortical involvement in the deficit of primary somatosensory processing in AS.

    Highly desynchronized first-cortical SSEP components and normal latencies of subcortical components indicated cortical dysfunction rather than impairment of afferent pathways in AS Del patients, which might be attributed to GABAergic dysfunction due to loss of UBE3A function and heterozygosity of GABARs

SQSTM1L341V variant that is linked to sporadic ALS exhibits impaired association with MAP1LC3 in cultured cells

Masahisa Nozaki, Asako Otomo, Shun Mitsui, Suzuka Ono, ... Shinji Hadano

  • Article 100301
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  • https://doi.org/10.1016/j.ensci.2020.100301
  • Abstract

    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are genetically, pathologically and clinically-related progressive neurodegenerative diseases. Thus far, several SQSTM1 variations have been identified in patients with ALS and FTD. However, it remains unclear how SQSTM1 variations lead to neurodegeneration. To address this issue, we investigated the effects of ectopic expression of SQSTM1 variants, which were originally identified in Japanese and Chinese sporadic ALS patients, on the cellular viability, their intracellular distributions and the autophagic activity in cultured cells.

    Expression of SQSTM1 variants in PC12 cells exerted no observable effects on viabilities under both normal and oxidative-stressed conditions. Further, although expression of SQSTM1 variants in PC12 cells and Sqstm1-deficient mouse embryonic fibroblasts resulted in the formation of numerous granular SQSTM1-positive structures, called SQSTM1-bodies, their intracellular distributions were indistinguishable from those of wild-type SQSTM1. Nonetheless, quantitative colocalization analysis of SQSTM1-bodies with MAP1LC3 demonstrated that among ALS-linked SQSTM1 variants, L341V variant showed the significantly lower level of colocalization. However, there were no consistent effects on the autophagic activities among the variants examined.

    These results suggest that although some ALS-linked SQSTM1 variations have a discernible effect on the intracellular distribution of SQSTM1-bodies, the impacts of other variations on the cellular homeostasis are rather limited at least under transiently-expressed conditions.

Decentralized, primary-care delivered epilepsy services in Burera District, Rwanda: Service use, feasibility, and treatment

Beatha Nyirandagijimana, Alphonse Nshimyiryo, Hildegarde Mukasakindi, Jackline Odhiambo, ... Stephanie L. Smith

  • Article 100296
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  • https://doi.org/10.1016/j.ensci.2020.100296
  • Abstract

    Integrating epilepsy care into primary care settings could reduce the global burden of illness attributable to epilepsy. Since 2012, the Rwandan Ministry of Health and the international nonprofit Partners In Health have collaboratively used a multi-faceted implementation program- MESH MH—to integrate and scale-up care for epilepsy and mental disorders within rural primary care settings in Burera district, Rwanda. We here describe demographics, service use and treatment patterns for patients with epilepsy seeking care at MESH-MH supported primary care health centers.

    This was a retrospective cohort study using routinely collected data from fifteen health centers in Burera district, from January 2015 to December 2016. 286 patients with epilepsy completed 3307 visits at MESH-MH participating health centers over a two year period (Jan 1st 2015 to Dec 31st 2016). Men were over twice as likely to be diagnosed with epilepsy than women (OR 2.38, CI [1.77–3.19]), and children under 10 were thirteen times as likely to be diagnosed with epilepsy as those 10 and older (OR 13.27, CI [7.18–24.51]). Carbamazepine monotherapy was prescribed most frequently (34% of patients).

    Task-sharing of epilepsy care to primary care via implementation programs such as MESH-MH has the potential to reduce the global burden of illness attributable to epilepsy.

Paradoxical wrist flexion: A new test to detect functional weakness of the upper limb

Masahiro Sonoo

  • Article 100302
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  • https://doi.org/10.1016/j.ensci.2020.100302
  • Abstract

    Positive signs are essential for the diagnosis of functional neurological disorders (FND). The author described a new positive sign to diagnose functional weakness (FW), "paradoxical wrist flexion", and tested its validity.

    Paradoxical wrist flexion is useful for the detection of nonorganic paresis. The background theory is that an active movement is more severely affected in FW than a passive movement when maintaining a limb position.

Posterior reversible encephalopathy syndrome and COVID-19: A series of 6 cases from Lombardy, Italy

Antonio Colombo, Filippo Martinelli Boneschi, Sandro Beretta, Nereo Bresolin, ... Andrea Salmaggi

  • Article 100306
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  • https://doi.org/10.1016/j.ensci.2020.100306
  • Abstract

    Posterior reversible encephalopathy cases are increasingly being reported in patients affected by COVID-19, but the largest series so far only includes 4 patients. We present a series of 6 patients diagnosed with PRES during COVID-19 hospitalized in 5 Centers in Lombardia, Italy. 5 out of the 6 patients required intensive care assistence and seizures developed at weaning from assisted ventilation. 3 out of 6 patients underwent cerebrospinal fluid analysis which was normal in all cases, with negative PCR for Sars-CoV-2 genome search. PRES occurrence may be less rare than supposed in COVID-19 patients and a high suspicion index is warranted for prompt diagnosis and treatment.

Gray matter structural networks related to 18F-THK5351 retention in cognitively normal older adults and Alzheimer's disease patients

Yoko Shigemoto, Daichi Sone, Kyoji Okita, Norihide Maikusa, ... Hiroshi Matsuda

  • Article 100309
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  • https://doi.org/10.1016/j.ensci.2021.100309
  • Abstract

    This study aimed to examine the alterations in gray matter networks related to tau retention in Alzheimer's disease (AD) patients and cognitively normal (CN) older individuals.

    We found opposite relationships between local network properties and tau retention between amyloid-positive AD patients and amyloid-negative controls. Our findings suggest that the presence of amyloid and induced exacerbated tau retention alter the relationship of local network properties and tau retention.

Functional connectivity of the motor system in dystonia due to PKAN

Peter Stoeter, Pedro Roa, Pamela Bido, Herwin Speckter, ... Rea Rodriguez-Raecke

  • Article 100314
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  • https://doi.org/10.1016/j.ensci.2021.100314
  • Abstract

    To demonstrate deviations of functional connectivity within the motor system in dystonic patients suffering from Pantothenate Kinase Associated Neurodegeneration, a genetic and metabolic disease, which is characterized by a primary lesion in the globus pallidus.

    During resting state, correlation coefficients within the motor system were significantly lower in patients than in controls (0.025 vs. 0.133, p < 0.05). Network analysis by Network Based Statistics showed that these differences mainly affected the connectivity between a sub-network consisting of the basal ganglia and another one, the motor system-related cortical areas (p < 0.05). 6 out of 12 connections, which correlated significantly to duration of disease, were connections between both sub-networks.

    The finding of a reduced functional connectivity within the motor network, between the basal ganglia and cortical motor-related areas, fits well into the concept of a general functional disturbance of the motor system in PKAN.

Pathologic basis of the preferential thinning of thecorpus callosum in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP)

Michiaki Kinoshita, Kiyomitsu Oyanagi, Yasufumi Kondo, Keisuke Ishizawa, ... Shu-ichi Ikeda

  • Article 100310
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  • https://doi.org/10.1016/j.ensci.2021.100310
  • Abstract

    Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is an early onset dementia characterized by axonal loss in the cerebral white matter with swollen axons (spheroids). It had been reported that the preferential thinning and "focal lesions" of the corpus callosum were observed on T2-weighted MRI in ALSP patients. The present study aimed to reveal the pathologic basis of them in relation to brain lesion staging (I ~ IV: Oyanagi et al. 2017).

    Preferential thinning of corpus callosum was considered to be formed in relation to peculiar morphological alteration of microglia there in ALSP. Instead, "focal lesions" were formed in connection with the lesions in centrum semiovale.

Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access

Dilraj Sokhi, Adil Suleiman, Soraiya Manji, Juzar Hooker, Peter Mativo

  • Article 100320
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  • https://doi.org/10.1016/j.ensci.2021.100320
  • Abstract

    Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA).

    Our study is the largest case series of NMOSD from the East Africa region. Patients faced challenges of access to appropriate and affordable testing, and timely availability of a neurologist at onset, which had impacts on their functional outcomes. The majority of the seropositive cases had recent/concurrent CNS infections, suggesting triggered auto-immunity.

Practice patterns in the management of recurrent and residual non-functioning pituitary adenomas: Results from a Canada-wide survey

Graham Kasper, Nardin Samuel, Ryan Alkins, Osaama H. Khan

  • Article 100317
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  • https://doi.org/10.1016/j.ensci.2021.100317
  • Abstract

    There is no consensus regarding the management and postoperative follow-up of non-functioning pituitary adenomas (NFAs) in the setting of recurrent or residual disease. Subsequent treatment options include continued follow-up, re-resection or radiotherapy. To address this gap and better understand current practice patterns, we surveyed neurosurgeons and radiation oncologists in Canada.

    Our results identified both variations and commonalities in practice amongst Canadian neurosurgeons. Approaches deviated in the setting of residual tumor based on years of practice. This work provides a critical foundation for future studies aiming to define evidence-based best practices in the management of NFAs.

Sleep-deprived residents and rapid picture naming performance using the Mobile Universal Lexicon Evaluation System (MULES) test

Jenna Conway, Luke Moretti, Rachel Nolan-Kenney, Omar Akhand, ... Laura J. Balcer

  • Article 100323
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  • https://doi.org/10.1016/j.ensci.2021.100323
  • Abstract

    The Mobile Universal Lexicon Evaluation System (MULES) is a rapid picture naming task that captures extensive brain networks involving neurocognitive, afferent/efferent visual, and language pathways. Many of the factors captured by MULES may be abnormal in sleep-deprived residents. This study investigates the effect of sleep deprivation in post-call residents on MULES performance.

    The MULES is a novel test for effects of sleep deprivation on neurocognition and vision pathways. Sleep deprivation significantly worsens MULES performance. Subjective sleepiness may also affect MULES performance. MULES may serve as a useful performance assessment tool for sleep deprivation in residents.

Detect it so you can treat it: A case series and proposed checklist to detect neurotoxicity in checkpoint therapy

Saskia Bolz, Thivyah Ramakrishnan, Michael Fleischer, Elisabeth Livingstone, ... Tim Hagenacker

  • Article 100324
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  • https://doi.org/10.1016/j.ensci.2021.100324
  • Abstract

    Checkpoint inhibitors show impressive and durable responses in various cancer types and provide new avenues for cancer immunotherapy. However, these drugs have a variety of adverse events. Common autoimmune-related adverse effects include fatigue, hepatitis, skin rash, endocrine deficiencies, and colitis. Neurotoxicity has been reported, but its incidence and course remain unclear.

    Checkpoint inhibitor therapy remains a success story; however, neurological immune-related adverse events may cause severe life-threatening conditions. We propose a guide for the early detection of neurological adverse events during routine clinical treatment to prevent more severe courses of checkpoint inhibitor-induced neurotoxicity.

Case Reports

D-dimer level elevation can aid in detection of asymptomatic COVID-19 presenting with acute cerebral infarction

Takeru Umemura, Hirohisa Kondo, Hirotsugu Ohta, Koichiro Futatsuya, ... Junkoh Yamamoto

  • Article 100294
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  • https://doi.org/10.1016/j.ensci.2020.100294
  • Abstract

    Coronavirus disease 2019 (COVID-19) mainly manifests as a respiratory syndrome, besides causing other complications. Severe COVID-19 may also present with coagulopathy, leading to venous thrombosis and cerebral infarction. Generally, acute stroke is a secondary complication in patients displaying respiratory syndromes. Here, we present a case of acute stroke in an 84-year-old female patient who did not manifest any respiratory symptoms. The patient had no cough or fever before the stroke onset; nevertheless, COVID-19 PCR test was positive. The patient also had markedly elevated serum D-dimer levels. Our findings suggest that coagulopathy can occur even in a patient with asymptomatic COVID-19 infection, and to our knowledge, this is the first report of such a case. We concluded that elevated D-dimer levels can serve as an additional COVID-19 screening tool in stroke patients.

A first presentation of multiple sclerosis with concurrent COVID-19 infection

Lisa Moore, Malik Ghannam, Georgios Manousakis

  • Article 100299
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  • https://doi.org/10.1016/j.ensci.2020.100299
  • Abstract

    Infection from SARS-CoV-2 virus has developed into a worldwide pandemic. Potential neurological complications include meningitis, encephalitis, Guillain-Barre syndrome, cerebrovascular disease, seizures, and demyelinating disease. In this paper, we describe a case of newly diagnosed multiple sclerosis co-occurring with active COVID-19 infection.

Inadvertent intrathecal administration of daunomycin resulting in fatality: Case report and therapeutic considerations

Sauson Soldozy, Anant Patel, Kurt Yaeger, Daniel Felbaum, ... M. Nathan Nair

  • Article 100297
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  • https://doi.org/10.1016/j.ensci.2020.100297
  • Abstract

    Daunomycin is a chemotherapeutic agent of the anthracycline family that is administered intravenously, most commonly in combination therapy. The authors report the first known adult case of inadvertently administered daunomycin directly into the human central nervous system and the neurologic manifestations and therapeutic interventions that followed.

    Accidental IT injection of daunomycin is a neurosurgical emergency and warrants prompt intervention. Symptoms can mimic other medical conditions, making it imperative an accurate diagnosis is made so that appropriate therapies are implemented. At this time, therapies include rapid removal of the chemotherapeutic agent from the IT compartment by aspiration and irrigation; however, it is unclear if neuroprotective agents may provide added benefit.

Postoperative cerebral air embolism with delayed abnormal brain MRI findings

Yuwa Oka, Koji Tsuzaki, Mayu Kamei, Akihiro Kikuya, Toshiaki Hamano

  • Article 100305
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  • https://doi.org/10.1016/j.ensci.2020.100305
  • Abstract

    Cerebral air embolism (CAE) is a rare but well-known complication resulting from invasive medical procedures; however, previous studies have not examined the postoperative longitudinal MRI changes in CAE. In particular, the likelihood that such changes may be observed after an initial delay when using magnetic resonance imaging (MRI) has not been explored. We herein report a case of CAE with no MRI abnormalities 4 h after a pulmonary vein isolation (PVI) procedure and where the first abnormality was found 22 h after the procedure.

    A 65-year-old man underwent PVI for paroxysmal atrial fibrillation and showed no signs of recovery from anesthesia after the procedure; thus, he was transferred to our emergency department for further examination. Neurological examination revealed conjugate eye deviation to the right and quadriplegia. Although initial computed tomography (CT) and MRI revealed no abnormalities, CAE was suspected, and a high-concentration oxygen treatment was administered. MRI performed 22 h after the procedure revealed restricted diffusion affecting the cortical areas. At the same day, he was transferred for hyperbaric‑oxygen chamber treatment. After 7 days of treatment, the patient recovered clinically and neurologically. He regained consciousness and was able to communicate.

    As suggested by this case, CT and MRI findings may fail to reveal CAE abnormalities initially. In such cases, as urgent treatment is necessary, it is important to consider diagnosing CAE based on the patient's history and administering a high concentration of oxygen. Finally, to reach a correct diagnosis, repeated brain MRI should be considered for patients with suspected CAE.

Emboli stroke following migration of carotid foreign body: A case report

Che-Cheng Chang, Han-Cheng Wang

  • Article 100313
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  • https://doi.org/10.1016/j.ensci.2021.100313
  • Abstract

    Foreign body embolization can cause intracranial artery occlusion with ischemic stroke. Reported etiologies include post cerebrovascular interventions, migration of esophageal foreign body and neck trauma. We reported a case with punctured wound at left neck, X-ray and computed tomography revealed a foreign body located in the carotid region. The patient eventually developed stroke symptoms in the next day after operation. Non-contrast brain Computer tomography at that time revealed that porcelain fragment located at the suprasellar area, and infarction of the left anterior basal ganglion. Our patient is the first reported case having an embolic stroke secondary to distal migration of a foreign body from the carotid artery after neck trauma. We call attention to this rare neurologic complication of neck trauma with foreign body retention. Appropriate and prompt identification of concurrent vascular injuries with retention of foreign body is strongly advised in neck trauma patients.

"Semantic variant primary progressive aphasia" due to comorbidity of Lewy body disease and a previous cerebral venous infarction in the left anterior temporal lobe: A case report

Kazuo Kakinuma, Wataru Narita, Toru Baba, Osamu Iizuka, ... Kyoko Suzuki

  • Article 100318
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  • https://doi.org/10.1016/j.ensci.2021.100318
  • Abstract

    Primary progressive aphasia (PPA) is a neurological syndrome characterized by progressive language impairment. Various neurodegenerative disorders cause PPA. Dementia with Lewy bodies (DLB) is one known cause of PPA, and little is known about this association. Almost all published cases of PPA associated with DLB are the logopenic variant of PPA. Here, we describe the novel case of a patient with DLB presenting clinical features of the semantic variant PPA (svPPA). A 75-year-old woman was referred to our hospital with a 2-year history of progressive anomia and amnesia. Two months before admission, she had been experiencing visual hallucinations, and at the age of 60 years, she had venous infarction in the left temporal lobe, which she recovered from without any residual symptoms. Upon admission to our hospital, she displayed anomia, impaired single-word comprehension, and surface dyslexia with preserved repetition and speech production. These symptoms met the criteria for the diagnosis of svPPA. 123I-ioflupane single-photon emission computed tomography and 123I-meta-iodobenzylguanidine myocardial scintigraphy indicated DLB. Thus, she was administered donepezil, and this dramatically improved her symptoms. We hypothesize that the combination of DLB with the previous asymptomatic venous thrombosis in the left temporal lobe may have contributed to the "svPPA" in this patient. In conclusion, we show that PPA associated with DLB could be treated with donepezil, and we suggest that donepezil should be pursued as a treatment option for PPA.

Basilar invagination: A mimicker of bulbar-onset amyotrophic lateral sclerosis

Jason Seng Hong Tan, Shirley Lee, Fu Liong Hiew

  • Article 100321
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  • https://doi.org/10.1016/j.ensci.2021.100321
  • Abstract

    Amyotrophic lateral sclerosis (ALS) is characterized by progressive onset motor deficits with heterogenous presentations ranging from dysarthria to foot drop. Approximately 20% of the patients present with focal bulbar symptoms, in which some may remain restricted to bulbar region (isolated bulbar palsy), and the remaining eventually spreads to involve other body regions (classical ALS). Without accompanying upper and lower motor neurons signs elsewhere, differential diagnoses for isolated bulbar symptoms are extensive, include ALS variants as well as potentially treatable mimics. Therefore, it is important to take heed on every possible aetiology that may disrupt the hypoglossal nucleus, nerve, or lingual muscle itself. Herein, we illustrated a rare presentation of Group A basilar invagination, which mimicked bulbar-onset ALS.

Chemoradiation induced multiple sclerosis-like demyelination

Alexandra Borges, Daniela Garcez, Cátia Pedro, João Passos

  • Article 100315
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  • https://doi.org/10.1016/j.ensci.2021.100315
  • Abstract

    We report the case of a 28-year-old man, diagnosed with a non-secreting, non-metastatic suprasellar germinoma treated with chemoradiation who developed, four months after completion of radiation therapy, multiple discrete demyelinating lesions mimicking multiple sclerosis (MS). The patient had no previous diagnosis of MS and the neuroimaging studies performed both at the time of diagnosis and after chemotherapy, pre-irradiation, showed no evidence of white matter lesions. He remained asymptomatic, with no focal neurological deficits. Biochemical analysis of the CSF was positive for the intrathecal synthesis of IgG with oligoclonal bands. Follow-up MRI six months later showed a spontaneous decrease in lesion size and resolution of associated inflammatory signs, with lesions remaining stable in number. We discuss the potential origin of these white matter lesions, which may correspond to MS-like late-delayed demyelination secondary to chemoradiation therapy, in a previously predisposed patient.

Pneumomediastinum while using mechanical insufflation-exsufflation after recovery from riluzole-induced interstitial lung disease

Yuji Saitoh, Masayuki Miyazaki, Nobuaki Arai, Yuji Takahashi

  • Article 100326
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  • https://doi.org/10.1016/j.ensci.2021.100326
  • Abstract

    We, herein, report a 61-year-old male patient with amyotrophic lateral sclerosis (ALS) complicated pneumomediastinum while using mechanical insufflation-exsufflation (MI-E) after recovery from riluzole (RZ)-induced interstitial lung disease (RZ-ILD). After the treatment of RZ-ILD, he required non-invasive mechanical ventilation (NIV) at minimal pressure settings and MI-E to manage ALS-related breathing and airway-clearance issues, respectively. After a while, he developed progressive worsening dyspnoea, and chest computed tomography revealed extensive pneumomediastinum that had spread to the area surrounding the oesophagus, the retrosternal space, and the pericardial space. He was treated with immediate discontinuation of MI-E; however, he had to keep using NIV to support his severe respiratory muscle involvement. Pneumomediastinum gradually reduced in size and no recurrence of pneumomediastinum occurred. The clinical course of our patient suggests that excessive coughing associated with MI-E combined with his previous RZ-ILD, which potentially renders his lungs vulnerable to airway pressure, may have been the aetiological factors for secondary pneumomediastinum, i.e. barotrauma. Clinicians should be aware of the risk of pneumomediastinum while using MI-E in patients with ALS, who have other pre-existing risk factors for pneumomediastinum, such as drug-induced ILD in our case.

Therapeutic plasma exchange for persistent encephalopathy associated with Covid-19

Chakrapani Ranganathan, Shelley D. Fusinski, Imad M. Obeid, Khaled M. Ismail, ... Sean M. Dawes

  • Article 100327
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  • https://doi.org/10.1016/j.ensci.2021.100327
  • Abstract

    Patients infected with COVID-19 virus, show a highly variable symptomatology which can include central nervous system (CNS) dysfunction. One of the most disabling CNS manifestations is persistent severe encephalopathy seen for weeks after the resolution of the acute viral pneumonia and associated acute systemic illnesses. The precise pathophysiology of this persistent Post COVID Encephalopathy is unknown but may involve direct viral invasion of microvascular endothelium, microvascular thrombosis, toxic neuronal effects of inflammatory products, vasoactive pathology at arteriolar level or leptomeningeal inflammation. Currently, there are no established specific treatments for Post COVID -19 encephalopathy. We present a case series of three patients that underwent Therapeutic Plasma Exchange (TPE) with salinized albumin that suggests a positive therapeutic effect. We believe that the results warrant further evaluation for the role of TPE with a prospective randomized trial in persistent Post COVID -19 encephalopathy syndrome.


Letters to the Editor

Recurrent infections caused by different species of Neisseria bacteria in a patient with complement seven deficiency

Michiyo Masuda, Mutsumi Iijima, Yusuke Ainoda, Yuji Hirai, ... Kazuo Kitagawa

Significance of an epidermal growth factor receptor mutation in cerebrospinal fluid for leptomeningeal metastasis and successful treatment with osimertinib: A case report and literature review

Takeo Sakurai, Akimasa Tuchida, Hiroshi Nishida

Levator palpebrae superioris myositis: An uncommon cause of ptosis

Karen Hei-tung Lai, William C.Y. Leung, Sum Lung Wong, Grace Ho, Richard Shek-kwan Chang

Kampō medicine and Muro disease (Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex): Postscript and Historical Footnote

Peter S. Spencer, Valerie S. Palmer, Tameko Kihira, Sohei Yoshida, ... Yoshiro Yase

  • Article 100308
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  • https://doi.org/10.1016/j.ensci.2020.100308
  • Abstract

    Western Pacific Amyotrophic Lateral Sclerosis and Parkinsonism-dementia Complex (ALS/PDC) is a disappearing neurodegenerative disease in three former high-incidence foci of the U.S. territory of Guam, Papua-Indonesia (New Guinea) and Kii Peninsula, Honshu Island, Japan (Muro disease). We report additional data that associate medicinal use of cycad seed to Muro disease in the southern Kozagawa focus of ALS/PDC. In the other two ALS/PDC-affected populations, cycad seed was used as a traditional topical medicine in New Guinea and Guam and, additionally, for food on Guam.

Idiopathic basal ganglia calcification may cause pathological conditions resembling Parkinson's disease

Yoshihiko Nakazato, Kei Ikeda, Kazumichi Ota, Naotoshi Tamura, Toshimasa Yamamoto

Multiple sclerosis, B cell therapy, and the COVID-19 vaccine

Eric J. Seachrist

Telephone Screening of Cognitive Status (TICS) in severe COVID-19 patients: Utility in the era of social isolation

Ana Beatriz Soldati, Cristina Almeida, Marco Lima, Abelardo Araujo, ... Marcus Tulius T. Silva

Impact of COVID-19 on Future Ischemic Stroke Incidence

Aisha Elfasi, Franklin D. Echevarria, Robert Rodriguez, Yoram A. Roman Casul, ... Alexis N. Simpkins

  • Article 100325
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  • https://doi.org/10.1016/j.ensci.2021.100325
  • Abstract

    With the ever-expanding population of patients infected with SARS-CoV-2, we are learning more about the immediate and long-term clinical manifestations of coronavirus disease 2019 (COVID-19). Ischemic stroke (IS) is now one of the well-documented additional clinical manifestations of COVID-19. Most COVID-19 related IS cases have been categorized as cryptogenic or embolic stroke of undetermined source (ESUS), which are most often suspected to have an undiagnosed cardioembolic source. COVID-19 is known to also cause cardiac dysfunction, heart failure, and atrial arrhythmias (AA), but the long-term impact of this cardiac dysfunction on stroke incidence is unknown. With millions afflicted with COVID-19 and the ever-rising infection rate, it is important to consider the potential long-term impact of COVID-19 on future IS incidence. Accomplishing these goals will require novel strategies that allow for diagnosis, data capture, and prediction of future IS risk using tools that are adaptable to the evolving clinical challenges in patient care delivery and research.