eNeurologicalScieNeurologicalSci Vol 21

December 2020

Review Articles

Kynurenine pathway in Parkinson's disease—An update

Dhivya Venkatesan, Mahalaxmi Iyer, Arul Narayanasamy, Kamalakannan Siva, Balachandar Vellingiri


Can the coronavirus infection penetrates the brain resulting in sudden anosmia followed by severe neurological disorders?

Mai M. Anwar, Ayman M. Badawi, Nadia A. Eltablawy

  • Article 100290
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  • https://doi.org/10.1016/j.ensci.2020.100290
  • Abstract

    Serious of unpredictable drawbacks of Coronavirus 2019 (COVID-19) infectious disease caused by SARS-COV-2 on the nervous system, have been widely noticed among the huge number of infected people. It was found that this type of newly revolving pandemic infection mainly infects the human respiratory tract causing mild to moderate symptoms, however, the hidden door side of COVID-19 is via penetrating the brain, revealing a huge threat especially to elderly people who are more susceptible to its severe side effects and even death to more extent. Almost 80% of COVID-19 patients suffer from severe neurological manifestations including dizziness, headache, unconscious, irritability, dysfunction in smell, and taste accompanied by muscle fatigue.

    Herein, we are trying to address the direct neuroinvasive pathway of COVID-19 into human brain cells which is mainly through the olfactory route leading to long-term neurological complications. In addition to highlighting the ability of COVID-19 infection to intensify a pre-existing AD to a more prominent severe stage. The other thing to emphasize is whether AD patients with a highly prominent activation of local immune responses are more or less exposed to getting infected with COVID-19. Along with underlying the hypothesis that the susceptibility to COVID-19 infection may lead to a future risk for neurodegenerative diseases including Alzheimer's disease (AD).

Potential biomarkers of emotional stress induced neurodegeneration

Tabassum Zafar


Original Articles

Determination of Paraneoplastic neuropathy in newly diagnosed breast tumor patients

Sayman Ceyhun, Tireli Hülya, Tuzun Erdem, Günay Gürleyik Meryem, Vardar Aker Fügen

  • Article 100265
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  • https://doi.org/10.1016/j.ensci.2020.100265
  • Abstract

    The presence of paraneoplastic neuropathy in newly diagnosed breast tumor patients will be investigated. Aim of study is conduce of early diagnosis of the disease and new biomarkers responsible for the pathogenesis to be identify.

    Paraneoplastic neuropathies can be confused with neuropathies due to non-cancerous causes both clinically and electrophysiologically. When approaching paraneoplastic neuropathies, pathological findings should be carefully reviewed and evaluated with other findigs.

    It should be remembered that an underlying breast tumor may be present in women with cancer-related neuropathic complaints.

Encephalopathy in COVID-19 patients; viral, parainfectious, or both?

Thirugnanam Umapathi, Wei Ming Jason Quek, Jia Min Yen, Hnin Su Wai Khin, ... Wai-Yung Yu

  • Article 100275
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  • https://doi.org/10.1016/j.ensci.2020.100275
  • Abstract

    We describe the clinical, laboratory and radiological features of 3 critically ill patients with COVID-19 who developed severe encephalopathy. The first patient did not regain consciousness when sedation was removed at the end of 2 weeks of intensive care. He had received treatment with convalescent plasma. His clinical examination was remarkable for intact brainstem reflexes, roving eye movements, later transient ocular flutter; and then what appeared to be slow ocular dipping. He had no coherent volitional response to the environment. The second patient recovered with measurable cognitive deficits after a prolonged period of encephalopathy. He had received combination treatment with interferon beta 1b and lopinavir/ritonavir. The third patient remained in persistent, severe agitated delirium and died 3 months into his illness. The MRI of the 3 patients showed multifocal abnormalities predominantly in the cerebral white matter, with varying involvement of the grey matter, brainstem and spinal cord. Case 1's MRI changes were consistent with acute disseminated encephalomyelitis. The patients also displayed blood markers, to varying degree, of autoimmunity and hypercoagulability. We were not able to convincingly show, from microbiological as well as immunological evaluation, if the effects of COVID-19 on these patients' nervous system were a direct consequence of the virus, proinflammatory-thrombotic state or a combination. Patient 1 responded partially to empirical, albeit delayed, therapy with intravenous immunoglobulins. Patient 2 recovered with no specific treatment. These cases illustrate the need to understand the full spectrum of encephalopathy associated with COVID-19 so as to better guide its management.

Factors affecting time between symptom onset and emergency department arrival in stroke patients

Scott M. Le, Laurel A. Copeland, John E. Zeber, Jared F. Benge, ... Jennifer Rasmussen

  • Article 100285
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  • https://doi.org/10.1016/j.ensci.2020.100285
  • Abstract

    Delays in seeking care compromise diagnosis, treatment options, and outcomes in ischemic strokes. This study identified factors associated with time between stroke symptom onset and emergency department (ED) arrival at a private nonprofit medical center serving a large rural catchment area in central Texas, with the goal of identifying symptomatic, demographic, and historical factors that might influence seeking care.

    The average time from symptom onset to presentation was 15.0 h (sd = 23.2), with 43.6% of the sample presenting within 4 h of symptom onset. Results suggested that female gender (Odds Ratio [OR] = 0.70; 95% Confidence Interval [CI] 0.23–0.74), drug abuse (OR = 0.41; CI 0.23–0.74), and diabetes were significantly associated with longer time to presentation.

    A combination of demographics, stroke severity, timing, and health history contributes to delays in presenting for treatment for ischemic stroke. Stroke education concentrating on symptom recognition may benefit from a special focus on high-risk individuals as highlighted in this study.

A case series and literature review of multiple sclerosis and COVID-19: Clinical characteristics, outcomes and a brief review of immunotherapies

Saurabh Kataria, Medha Tandon, Violina Melnic, Shitiz Sriwastava

  • Article 100287
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  • https://doi.org/10.1016/j.ensci.2020.100287
  • Abstract

    In view of the emerging coronavirus pandemic, the demand for knowledge about the impact of SARS-CoV-2 on people with Multiple Sclerosis (MS) continues to grow. Patients receiving disease modifying therapy (DMT) for MS have a higher background risk of infection-related health care utilization when compared to the general population. Therefore, there is a need of evidence-based recommendations to reduce the risk of infection and also managing MS patients with SARS-CoV-2.

    This review provides a base on the clinical characteristics, outcomes and the roles of DMTs in MS patients suffering from n-cov-2. Physicians need to be vigilant about considering COVID-19 infection related relapse in the MS patients, especially in this COVID-19 pandemic era and look for pseudo-exacerbation. As most cases are found to have mild course and full recovery on DMTs, further research is needed to formulate evidence-based guidelines. This review will particularly be helpful for the researchers and registries to collect future data on MS and COVID-19.

Pattern and frequency of neurological and neurosurgical care of adult inpatients and outpatients at a tertiary referral hospital in Kenya

Jane R. von Gaudecker, Chrispine Oduor, Susan Ofner, Eren Oyungu, ... Janice Buelow

  • Article 100286
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  • https://doi.org/10.1016/j.ensci.2020.100286
  • Abstract

    To describe the patterns and burden of neurological and neurosurgical disorders at a national tertiary level referral hospital in western Kenya.

    We conducted a three-month period prevalence study. We recruited consecutive adult patients seeking neurological-neurosurgical care in both inpatient and outpatient settings at Moi Teaching and Referral Hospital.

    The findings suggest that meningitis, stroke, epilepsy, and traumatic brain injury were the most common diagnosis. More resources and efforts should be directed towards prevention, diagnosis and management of these conditions in the region.


Case Reports

A case of longitudinally extensive transverse myelitis with an isolated pontine lesion following epidural and spinal anesthesia for cesarean section

Yuki Kitazaki, Asako Ueno, Kenichiro Maeda, Rei Asano, ... Tadanori Hamano

  • Article 100264
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  • https://doi.org/10.1016/j.ensci.2020.100264
  • Abstract

    Severe neurologic complications following epidural and spinal anesthesia rarely occur. Transverse myelitis has been reported as a rare complication of epidural or spinal anesthesia. We report a case of longitudinally extensive transverse myelitis and an isolated pontine lesion, which responded to immunotherapy. The patient was a 31-year-old pregnant woman who underwent elective cesarean section under epidural and spinal anesthesia. Though the insertions of the epidural and spinal catheters were smooth, she experienced back pain and transient hearing loss during epidural anesthesia. Postoperatively, she exhibited severe motor weakness in both lower extremities, neuralgia below the level of Th10 dermatome, and urinary retention. Magnetic resonance imaging showed longitudinally extensive transverse myelitis from T6 to T10 with a ring-shaped enhanced lesion and an isolated pontine lesion. These findings on magnetic resonance imaging were suggestive of autoimmune diseases such as neuromyelitis optica. The patient was diagnosed with an immunoreactive disease triggered by epidural or spinal anesthesia and was administered high-dose methylprednisolone, which led to the improvement in clinical symptoms. Clinicians should be aware of the possibility of the development of longitudinally extensive transverse myelitis and isolated pontine lesions after cesarean section under epidural and spinal anesthesia.

An adult nemaline myopathy patient with respiratory and heart failure harboring a novel NEB variant

Masahiro Ohara, Yoshihiko Saito, Mutsufusa Watanabe, Saneyuki Mizutani, ... Hiroto Fujigasaki

  • Article 100268
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  • https://doi.org/10.1016/j.ensci.2020.100268
  • Abstract

    Nemaline myopathy is a heterogeneous disorder of skeletal muscle, and histologically characterized by the presence of nemaline bodies in muscle fibers. Patients with typical congenital form of nemaline myopathy initially present with proximal but later also distal muscle weakness, mostly involving facial and respiratory muscle. Cardiac involvement has been rarely observed especially in nebulin-related nemaline myopathy and there have been only two reports about nebulin-related nemaline myopathy patients with cardiac involvement. We present here the case of a 65-year-old woman manifesting slowly progressive distal myopathy with respiratory and heart failure. She harbored two variants in the nebulin gene, c.20131C > T (p.Arg6711Trp) and c.674C > T (p.Pro225Leu), and one of them, c.674C > T, was a novel variant. In this report, we discuss the pathogenicity of the novel variant and its association with clinical phenotypes including cardiac involvement.

Postcentral gyrus infarction with spared proprioceptive sensation

Daisuke Tsukui, Yugo Ishihara, Norito Kokubun, Koichi Hirata, Keisuke Suzuki

  • Article 100267
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  • https://doi.org/10.1016/j.ensci.2020.100267
  • Abstract

    We here report a patient with postcentral gyrus infarction whose touch and pain sensations in the right forearm and hand were impaired but proprioceptive sensation was spared. We observed the clinicoradiological correlation between sensory impairment of tactile and pain sensation with spared proprioceptive sensation and the posterior postcentral gyrus lesion, which may be important in understanding the function of human primary sensory cortex.

Peripheral neuropathy in systemic vasculitis and other autoimmune diseases – a report of five cases emphasizing the importance of etiologic characterization

Rita Rodrigues, Mariana Branco, Renata Silva, Luís Ruano, ... Catarina Santos

  • Article 100272
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  • https://doi.org/10.1016/j.ensci.2020.100272
  • Abstract

    Peripheral neuropathies may present in the context of systemic vasculitis and other autoimmune diseases. The etiologic characterization is crucial to define the treatment and prognosis in secondary vasculitis. The purpose of this study is to describe the pathway of etiologic investigation including the role of nerve biopsy.

    This series reveals the etiologic and phenotypic diversity of peripheral neuropathies related with systemic vasculitis. The therapeutic approach and prognosis were distinct in each patient, emphasizing the importance of a prompt diagnosis and appropriate treatment.

Post COVID-19 syndrome associated with orthostatic cerebral hypoperfusion syndrome, small fiber neuropathy and benefit of immunotherapy: a case report

Peter Novak

  • Article 100276
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  • https://doi.org/10.1016/j.ensci.2020.100276
  • Abstract

    Coronavirus disease (COVID-19) is a novel highly contagious infectious disease caused by the coronavirus SARS-CoV2. The virus affects the human respiratory and other systems, and presents mostly as acute respiratory syndrome with fever, fatigue, dry cough, myalgia and dyspnea. The clinical manifestations vary from no symptoms to multiple organ failure. Majority of patients fully recover. Several postinfectious presumably autoimmune complications of COVID-19 affecting the brain or peripheral large nerve fibers have been reported. This report describes a post COVID-19 patient who developed chronic fatigue, orthostatic dizziness and brain fog consistent with orthostatic hypoperfusion syndrome (OCHOS), a form of orthostatic intolerance, and painful small fiber neuropathy (SFN). Initially, the patient was diagnosed with.

    OCHOS (detected by the tilt test with transcranial Doppler monitoring) and SFN (confirmed by skin biopsy), and both OCHOS/SFN were attributed to Post Treatment Lyme Disease Syndrome of presumed autoimmune etiology. Patient recovered on symptomatic therapy. COVID-19 triggered exacerbation of OCHOS/SFN responded to immunotherapy with intravenous immunoglobulins. This case suggests that post COVID-19 syndrome may present as an autoimmune OCHOS/SFN and that early immunotherapy may be effective. Further studies are necessary to confirm the link between OCHOS/SFN and COVID-19 disease as well as to confirm the benefit of immunotherapy.

Hemorrhagic stroke and COVID-19 infection: Coincidence or causality?

Pedro Fraiman, Mariana Freire, Manuel Moreira-Neto, Clecio Godeiro-Junior

  • Article 100274
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  • https://doi.org/10.1016/j.ensci.2020.100274
  • Abstract

    Amyloid Protein Precursor gene duplication is a rare cause of early-onset Alzheimer's disease that can be associated with Cerebral Amyloid Angiopathy. This condition predisposes cerebrovascular events, specifically, intracerebral hemorrhagic stroke. This report describes a case of first-time intracerebral hemorrhage in a patient with APP gene duplication during SARS-CoV-2 infection, a typically pro-thrombotic and pro-inflammatory condition, as a possible trigger for this condition.

Antiganglioside antibodies and paraneoplastic neuromuscular junction disorder?

Noushin Jazebi, Chilvana Patel, Xiang Fang

Neuroretinitis in a young woman

Edward Margolin, Trishal Jeeva-Patel

Postprandial cerebral infarction resolved by extracranial-intracranial bypass surgery

Hajime Ikenouchi, Takeshi Yoshimoto, Eika Hamano, Satoshi Saito, ... Masafumi Ihara

  • Article 100283
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  • https://doi.org/10.1016/j.ensci.2020.100283
  • Abstract

    A 51-year-old man with type 2 diabetes mellitus was admitted with a 2-month history of repeated episodes of transient aphasia and right hemiparesis after food intake. His blood pressure (BP) fell when the neurological deficits developed. The fall in BP after each meal was confirmed by 24-h ambulatory blood pressure monitoring (ABPM), which established the diagnosis of postprandial hypotension (PPH). Diffusion-weighted magnetic resonance imaging of the brain showed multiple high-intensity lesions at the borderzone between the anterior and middle cerebral artery (MCA) territories in the left hemisphere. Digital subtraction angiography showed tapered occlusion at the origin of the left internal carotid artery (ICA). Despite sufficient antiplatelet therapy and medication for PPH, the transient symptoms remained. Positron emission tomography scanning using H215O showed decreased cerebral blood flow with increased oxygen extraction fraction in the left MCA territory. As the symptomatic left ICA occlusion was intractable, an extracranial-intracranial (EC-IC) bypass surgery was conducted without any perioperative complications. Although PPH remained, cerebrovascular ischemic events including repeated transient ischemic attack disappeared for 2 months after surgery. The coincidence of stroke with ABPM-proved transient hypotension suggested that the brain infarcts were caused by hemodynamic changes related to PPH co-existent with the chronic left ICA occlusion. ABPM is useful in evaluating hemodynamic infarcts associated with BP fluctuation, and should be considered for patients with chronic ICA occlusion. In addition, EC-IC bypass may be a treatment option for symptomatic chronic ICA occlusion due to PPH.

Severe lymphopenia after subcutaneous cladribine in a patient with multiple sclerosis: To vaccinate or not?

M. Mateo-Casas, S. Reyes, S. De Trane, F. Edwards, ... G. Giovannoni

  • Article 100279
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  • https://doi.org/10.1016/j.ensci.2020.100279
  • Abstract

    To describe a fatal case of influenza A pneumonia in a patient with severe lymphopenia after receiving subcutaneous cladribine to treat her multiple sclerosis (MS).

    Cladribine-related lymphopenia is usually mild to moderate, however severe lymphopenia may occur. People with MS, especially those who are immunosuppressed, should be offered the inactivated influenza vaccine annually.

A case of type 1 facioscapulohumeral muscular dystrophy (FSHD) with restrictive ventilatory defect and congestive heart failure

Nobutoshi Morimoto, Mizuki Morimoto, Yoshiaki Takahashi, Motonori Takamiya, ... Koji Abe

  • Article 100284
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  • https://doi.org/10.1016/j.ensci.2020.100284
  • Abstract

    Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant muscle disease characterized by asymmetric involvement of muscles in the face, upper extremity, trunk, and lower extremity regions, with variable severity. It was recently reported that restrictive respiratory involvement is more frequent and severe than previously recognized, while cardiac dysfunction other than arrhythmia is still considered extremely rare in FSHD. [Case report] A 59-year-old man presenting with marked muscle atrophy in the trunk and asymmetrical muscle atrophy in the legs was hospitalized because of dyspnea and edema in the face and limbs. Shortness of breath with body movement started from approximately 40 years of age. Muscle biopsy revealed myopathic change with mild to moderate variation in fiber size. The diagnosis of FSHD was made by D4Z4 contraction to three repeats on genetic testing. A pulmonary function test revealed a decline of forced vital capacity (FVC) and a preserved FEV1/FVC indicating restrictive ventilatory defect (RVD). Ultrasonic echocardiogram (UCG) showed diffuse left ventricular hypokinesis, ventricular septum thickening, pericardial effusion, and decreased ejection fraction (LVEF 30%). [Conclusion] Although restrictive ventilatory defect and congestive heart failure are uncommon in FSHD, respiratory and cardiac evaluation may be necessary in patients with FSHD.

Bilateral parkinsonism in a patient with infarcts involving the unilateral basal ganglia

Shiroh Miura, Masayuki Ochi, Hirofumi Ochi, Michiya Igase, ... Yasumasa Ohyagi

  • Article 100291
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  • https://doi.org/10.1016/j.ensci.2020.100291
  • Abstract

    We describe a 61-year-old woman with bilateral parkinsonism caused by unilateral infarction limited to the territory of the lenticulostriate arteries. Although dopamine transporter imaging with single-photon emission computed tomography (DaTSPECT) demonstrated reduced putaminal tracer binding concordant with the size and location of the vascular lesion, the specific binding ratio was within the normal range. Five months after onset, the patient was free from parkinsonism without the use of any antiparkinsonian agents. When patients show bilateral parkinsonism, it is important to consider infarction of the lenticulostriate arteries. Additionally, DaTSPECT might be useful for predicting the prognosis of parkinsonism caused by infarction.


Letters to the Editor

Reorganization of multiple sclerosis health care system in Clinical Centre of Montenegro during the COVID-19 pandemic

Jevto Erakovic, Dragica Milikic, Ljiljana Radulovic, Slavisa Perunicic, ... Milovan Roganovic

  • Article 100263
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  • https://doi.org/10.1016/j.ensci.2020.100263
  • Abstract

    The COVID-19 epidemic has brought a number of changes to health systems, including the provision of health services to patients with multiple sclerosis (MS). Work in the Clinic for Neurology of the Clinical Center of Montenegro (CN-CCM) with MS patients continued as much as possible during the epidemic. The administration of the already started disease-modifying therapy (DMT) continued, and the introduction of the new one was postponed until the moment when the epidemic started to slow down. During the epidemic period, plasmapheresis treatment was performed in CN-CCM without any complications. New ways of communications (special e-mail address and phone line) with MS patients during epidemic were realized. During epidemic, smaller number of relapses were reported compared to same period in 2019. There were not MS patients in Montenegro suffering from SARS-Cov2 virus infection.

JC virus granule cell neuronopathy associated with Ruxolitinib: A case report and review of the literature

Kentaro Nakayama, Masataka Nakamura, Akiko Konishi, Satoshi Kaneko, ... Hirofumi Kusaka

Autosomal dominant cerebellar ataxia, deafness, and narcolepsy with amenorrhea, subclinical optic atrophy, and electroencephalographic abnormality: A case report

Kai-Chieh Chang, Yih-Chih Kuo, Hsueh-Wen Hsueh, Ni-Chung Lee, ... Chi-Chao Chao

Impact of COVID-19 on the clinical care of Ethiopian PD patients: A glimpse into the burden

Biniyam A. Ayele, Yared Zenebe, Hanna Demissie, Abenet Tafesse, ... Ali Shalash

Optimising therapeutic strategies for acute stroke-like lesions in MELAS

Josef Finsterer

Successful basilar artery dilatation in pure bilateral cerebral peduncular infarctions using balloon angioplasty

Yutaro Kikuchi, Naoko Miyamoto, Yuki Urushida, Chisato Shimizu, ... Mikio Shoji

COVID-19 and stroke: Red flags for secondary movement disorders?

Daniella Balduino Victorino, Marcia Guimarães-Marques, Mariana Nejm, Fulvio Alexandre Scorza, Carla Alessandra Scorza



Corrigendum to 'Association of sickle cell trait with measures of cognitive function and dementia in African Americans' eNeurologicalSci, Vol. 16 (2019), 100,201

Nemin Chen, Christina Caruso, Alvaro Alonso, Vimal K. Derebail, ... Hyacinth I. Hyacinth