eNeurologicalScieNeurologicalSci Vol 20

September 2020

Review Article

Chronic methanol toxicity through topical and inhalational routes presenting as vision loss and restricted diffusion of the optic nerves on MRI: A case report and literature review

Christianne V. Mojica, Esteban A. Pasol, Mercedes L. Dizon, Wenceslao A. Kiat, ... Bernardo Joaquin P. Tuaño

  • Article 100258
  • https://doi.org/10.1016/j.ensci.2020.100258
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  • Abstract

    Methanol intoxication can cause irreversible neurologic sequelae if unrecognized and untreated. Ingestion is the most common form of toxicity; however, dermal and inhalational exposures likewise occur but are documented rarely. While acute intoxication is commonly encountered, chronic exposure to methanol should also be highlighted.

    We report a case of a 57-year old female presenting in the emergency room with progressive dyspnea, metabolic acidosis with high anion gap, and metabolic encephalopathy. After emergency hemodialysis, the patient complained of vision loss on both eyes. Initial non-contrast cranial magnetic resonance imaging (MRI) revealed restricted diffusion of the intraorbital segment of both optic nerves. A thorough history revealed that she was applying a clear colorless liquid bought online all over her body for alleged pruritus for more than a year.

    The syndrome of metabolic acidosis with high anion gap, metabolic encephalopathy, vision loss, and laboratory findings led us to suspect a diagnosis of chronic methanol poisoning with an acute component. The liquid in question was sent for chemical analysis and result showed that it consisted of 95.5% Methanol. This case highlights the need for high index of clinical suspicion for methanol toxicity in the absence of oral consumption, the complications of chronic form of methanol intoxication, and the uncommon radiologic finding seen in diffusion-weighted imaging (DWI).


Original Articles

Endothelial dysfunction and carotid atherosclerosis in Malawian adults: A cross-sectional study

Joseph Kamtchum-Tatuene, Gloria Mwangalika Kachingwe, Henry C. Mwandumba, Tom Solomon, Laura A. Benjamin

  • Article 100252
  • https://doi.org/10.1016/j.ensci.2020.100252
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  • Abstract

    In sub-Saharan Africa, data on prevalence, risk factors and pathobiology of carotid atherosclerosis are scarce. We aimed to investigate the relationship between biomarkers of endothelial dysfunction and carotid atherosclerosis.

    Carotid ultrasound was performed in 66 patients. Plasma concentration of ICAM-1, PAI-1, VEGF, and soluble thrombomodulin were measured by ELISA. A univariable logistic regression analysis was performed to study the relationship between carotid atherosclerosis, biomarkers of endothelial dysfunction, and various demographic and clinical parameters of the participants.

    The mean age of the participants was 58.7 years (95% CI: 54.4–63.1). Carotid atherosclerosis was diagnosed in 39.4% (95% CI: 27.6–52.2). In the univariable logistic regression, the following factors were associated with carotid atherosclerosis: age > 45 years (OR = 12.0, 95% CI: 1.4–98.8, p = .02), hypertension (OR = 3.8, 95% CI: 1.2–12.1, p = .02), and high-level of soluble thrombomodulin (OR = 3.4, 95% CI: 1.2–10.0, p = .02).

    There is an association between high levels of soluble thrombomodulin and carotid atherosclerosis in Malawian adults. Further studies with a larger sample size are needed to confirm our findings in other African populations.

Prescription pattern of anti-Parkinson's disease drugs in Japan based on a nationwide medical claims database

Masahiko Suzuki, Masaki Arai, Ayako Hayashi, Mieko Ogino

  • Article 100257
  • https://doi.org/10.1016/j.ensci.2020.100257
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  • Abstract

    Parkinson's disease (PD) treatment should follow guidelines and be tailored to each patient. Large database analyses can provide insights into prescribing patterns.

    Retrospective, cross-sectional study of patients (≥30 years) with PD diagnosis (ICD-10; schizophrenia/cerebrovascular disease excluded) using health insurance claims data (April 2008–December 2016) from the Japan Medical Data Vision database. Prescription patterns of anti-PD drugs were analysed by patient age and sex, calendar year, and overall.

    The analysis comprised 155,493 PD patient-years (56.1% women, mean 73.4 years). Patient number increased each year, mainly because of database expansion. L-dopa as monotherapy was the most common prescription (22.7% of patient-years); non-ergot dopamine agonists (DAs) were also common (7.6% as monotherapy, 6.8% with L-dopa). Monotherapy was prescribed for ~50% of patient-years, two drugs for 14.1%, and at least three drugs for 18.4%. Consistent with Japanese guidelines, L-dopa was mostly prescribed to older patients (≥60 years), whereas non-ergot DAs were mostly prescribed to middle-aged patients (peak at 50–69 years). Between 2008 and 2011, L-dopa prescription decreased while that of non-ergot DAs increased; this pattern reversed between 2012 and 2016.

    These results indicate that Japanese clinicians are adhering to Japanese guidelines and tailoring anti-PD treatment to individual patients.

Olfactory function deteriorates in patients with Parkinson's disease complicated with REM sleep behavior disorder

Takashi Nomura, Yuki Nomura, Masayoshi Oguri, Yasuaki Hirooka, Ritsuko Hanajima

  • Article 100261
  • https://doi.org/10.1016/j.ensci.2020.100261
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  • Abstract

    It is not concluded whether the association between olfactory dysfunction and REM sleep behavior disorder (RBD) were worsen cognitive function in patients with Parkinson's disease (PD). We sought to evaluate the impact of these symptoms in PD.

    We examined 62 patients with PD using an olfactory test (Odor Stick Identification Test for Japanese: OSIT-J) and polysomnography (PSG). We divided the patients into 3 groups: PD with clinical RBD (n = 32), PD with subclinical RBD (n = 11), and PD with normal REM sleep (n = 19). We compared their clinical backgrounds, results of OSIT-J, autonomic functions, and cognitive functions such as Montreal cognitive assessment Japanese version (MoCA-J). Some factors associated with RBD were analyzed by multiple regression.

    There were significant differences in the results of OSIT-J, and autonomic and cognitive functions between the 3 groups. There were significant differences in the total OSIT-J score between the 3 groups (PD with clinical RBD: 3.3 ± 2.2, PD with subclinical RBD: 4.0 ± 2.6, PD with normal REM sleep: 6.7 ± 3.0, p < 0.001). Patients in the group with PD with clinical RBD had a significantly lower score than those with normal REM sleep (p < 0.001). Logistic regression analysis showed that OSIT-J score was significantly associated with RBD. The PD group with clinical RBD had more patients with mild cognitive impairment than the group with normal REM sleep. Multiple regression analysis revealed that olfactory dysfunction was correlated with MoCA-J.

    Olfactory dysfunction is associated with RBD. Especially, it is important to screen olfactory function in RBD complicated patients with PD in view of cognitive impairment.

Out-patient neurological disorders in Tanzania: Experience from a private institution in Dar es Salaam

Philip B. Adebayo, Omar M. Aziz, Rose E. Mwakabatika, Mandela C. Makakala, ... Eric Aris

  • Article 100262
  • https://doi.org/10.1016/j.ensci.2020.100262
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  • Abstract

    Low and middle-income countries (LMIC) have a considerable burden of neurological disorders. Available profile of neurological disorders in our environment is biased towards neurological admissions. There is a paucity of data on out-patient neurological conditions in sub-Saharan Africa.

    To determine the frequency and demographic data of neurological illnesses being managed at the adult out-patient neurology clinic of the Aga Khan Hospital, Dar es Salaam (AKHD).

    The electronic medical records of all cases with neurological diseases who presented to the adult neurology clinic of the AKHD between January 2018, and December 2019 were retrospectively reviewed and analyzed. Neurological disorders are categorized according to the international classification of diseases version-11(ICD-11).

    Of the 1186 patients seen in a period of 2 years, there were 597 (50.4%) females and 588(49.6%) males, with median age (IQR) of 38 (30.0–52.0) and 42 (33.0–54.5) years respectively (p = 0.001). Headache disorders (27.0%); disorders of the nerve root, plexus or peripheral nerves (23.4%); epilepsy (9.3%), cerebrovascular disorders (8.9%); movement disorders (3.6%) and disorders of cognition (3.5%) were the primary neurological conditions encountered. Musculoskeletal disorders (7.5%) and mental/behavioral disorders (5.4%) were other conditions seen in the clinic.

    The pattern of neurological disorders in this cohort mirrors that of high-income countries. However, the manpower to tackle these conditions pales in comparison. Increasing the neurology workforce and paying extra attention to non-communicable disorders in SSA is advocated.


Case Reports

A pediatric neurobehavioral treatment challenge

Lisa E. Whitley, Craig Chepke

  • Article 100244
  • https://doi.org/10.1016/j.ensci.2020.100244
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  • Abstract

    Tourette 's syndrome (TS) currently has very limited FDA approved treatment options, despite the fact that TS and other pediatric tic disorders are not uncommon and can cause significant emotional and physical distress for patients and their families. For providers who regularly see these conditions in practice this inadequacy is highly frustrating on the treatment side as there are such limited options to offer to these families, and the outcome to hope for is not highly optimistic.

    The outcome of this case presentation is an example of how health care providers can use clinical knowledge in combination with evidence-based practice to advocate for their patients and find new solutions that could be, and in this case were, life changing. It promotes providers to advocate for their patients and expand their knowledge.

    The final medication treatment utilized in this case is currently off label, however the method of finding and obtaining the treatment explained in the case additionally reveal to readers how to cautiously but judiciously make off label treatment decisions that can be important to practice and patient outcomes.

    In the course of this case the off-label medication use decision was backed by scientific literature and ongoing FDA studies as well as consulting with a collaborating psychiatrist. The subject matter and method of reasoning within this case also promote psychiatric providers use of evidence-based practice and implementation of science into active practice.

Sydenham chorea in a12- year old Saudi girl

Hatem Hamed El-shorbagy, Fahad Khamis Al Omari, Hassan Mohsen Al Ghashmari, Salem Mohsen Al Zahrani, Mohamed Abdel Kader Ghoname

  • Article 100246
  • https://doi.org/10.1016/j.ensci.2020.100246
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  • Abstract

    Sydenham chorea (SC) is the most common acquired cause of chorea in children.

    (SC) is the neurological manifestation of rheumatic fever. Rheumatic fever is still reported in Saudi Arabia, although less frequently. According to modified Jones criteria, carditis and arthritis are the major manifestations of acute rheumatic fever (ARF) in children.

    SC is often seen in isolated form; however, it can occur in association with other clinical manifestations of ARF like carditis. It has been reported that silent, mild valvular regurgitation has been found in patients with pure chorea.

    SC is characterized by abnormal body movement, associated with behavioral changes. It is an autoimmune disease that occurs following an infection with Group A beta-haemolytic streptococcal infection that is prevalent in Saudi Arabia.

    Despite recent advances in child health care services, RF continues to occur in Saudi Arabia. We describe a 12-year-old Saudi girl with SC and clinically inaudible, but echocardiographically significant mild valvular regurgitation.

    The diagnosis of Sydenham chorea should be considered in young children with choreiform movements. Doppler echocardiography may be useful in detecting silent valvular regurgitation and in deciding the duration of penicillin prophylaxis.

Large vessel stroke and COVID-19: Case report and literature review

Sumaiya Mahboob, Sri Harsha Boppana, Nicholas B. Rose, Bryce D. Beutler, Burton J. Tabaac

  • Article 100250
  • https://doi.org/10.1016/j.ensci.2020.100250
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  • Abstract

    The clinical signs of coronavirus disease-19 (COVID-19) can be heterogenous because of the diversity of potential organ involvement. We describe a 58-year-old woman who developed new-onset dysarthria and hemiplegia and was found to be COVID-19-positive. This is among the first cases of COVID-19 presenting solely with focal neurologic deficits.

Therapy-resistant dysphagia successfully treated using pharyngeal electrical stimulation in a patient with the pharyngeal-cervical-brachial variant of the Guillain-Barré syndrome

Sebastian Beirer, Wolfgang Grisold, Jennifer Dreisbach

  • Article 100255
  • https://doi.org/10.1016/j.ensci.2020.100255
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  • Abstract

    Pharyngeal electrical stimulation (PES) is a novel treatment for oropharyngeal dysphagia resulting from neurogenic causes such as stroke, prolonged intubation, tracheostomy, or multiple sclerosis, and may be effective in other medical conditions such as Guillain-Barré syndrome (GBS). A 74-year-old male patient with a pharyngeal-cervical-brachial (PCB) variant of GBS, who had been tracheotomised due to severe and persistent swallowing difficulties (dysphagia) unresponsive to traditional speech and language therapy, received PES therapy as a final treatment option. Swallow performance evaluated before and after PES using fibreoptic endoscopic evaluation of swallowing, videofluoroscopy and clinical bedside assessment, showed significant improvement in swallowing resulting in safe tracheostomy decannulation 18 days after PES.

    In PCB GBS, we present the potential benefit of PES for the treatment of persistent dysphagia and faster tracheostomy decannulation. PES was safe and may be beneficial in other neurologic disorders, where traditional dysphagia therapies have proved unsuccessful.

Selective aphasia and focal hypoperfusion in a bilingual patient with HaNDL syndrome

Antonio Cruz-Culebras, Rocio Vera

  • Article 100259
  • https://doi.org/10.1016/j.ensci.2020.100259
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  • Abstract

    The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) is a rare disease (1) characterized by a benign, self-limited headache syndrome accompanied by neurological deficits (isolated aphasia can be seen in nearly 22% of these patients (2)).

    Differential diagnosis between acute ischemic stroke and HaNDL syndrome has to be made in order to decide whether to perform a lumbar puncture or start reperfusion treatment early. CT perfusion have proved to be useful for differential diagnosis (3).

    We present a case of a HaNDL patient referred to the Emergency Department as a stroke in the context of acute onset of selective aphasia (Spanish) in a bilingual patient (French-Spanish). Urgent CT perfusion during the episode revealed increased mean transit time (MTT) with normal Cerebral Blood Flow (CBF) in posterior language areas. The case provides information on a HaNDL attack and its pathophysiology with hemodynamic changes in the acute period during the episode and the benign condition of the illness.

Cerebral embolism accompanied by remarkable diffusion-weighted imaging reversal in a 10 month-old infant with congenital heart disease

Hajime Ikenouchi, Manabu Inoue, Kaori Miwa, Kenichiro Yi, ... Masatoshi Koga


Letters to the Editor

Prothrombotic state induced by COVID-19 infection as trigger for stroke in young patients: A dangerous association.

Francesco Cavallieri, Alessandro Marti, Antonio Fasano, Annalisa Dalla Salda, ... Marialuisa Zedde

Squamous cell carcinoma of the scalp causing cortical venous thrombosis and Intraparenchymal hematoma

Mihir Gupta, Anudeep Yekula, Varun Sagi, Aditya Mittal, Marc S. Schwartz

Acute management of a stroke-like episode in MELAS syndrome: What should we know?

Tirso González-Pinto González, Javier Almeida Velasco, Ana Moreno Estébanez, Garazi Agirre Beitia, ... Amaia Martínez Arroyo

Increased serum alkaline phosphatase and early neurological deterioration in patients with atherothrombotic brain infarction attributable to intracranial atherosclerosis

Toshiyuki Uehara, Koji Yoshida, Hideo Terasawa, Hirotaka Shimizu, Yasushi Kita

  • Article 100253
  • https://doi.org/10.1016/j.ensci.2020.100253
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  • Abstract

    The purpose of this study was to determine whether increased alkaline phosphatase (ALP) was associated with early neurological deterioration (END) in patients with atherothrombotic brain infarction (ATBI) attributable to intracranial atherosclerosis (ICAS) or not.

    We analyzed data derived from 70 patients (47 men; mean age, 72.4 ± 12.8 years) with symptomatic ICAS who were admitted within 3 days of ATBI onset between April 2013 and December 2018. We defined END as an increase of ≥2 in the National Institutes of Health Stroke Scale scores during the first 72 h of hospitalization.

    Eleven (15.7%) patients had END. Serum ALP levels on admission were significantly higher among patients with, than without END (median [interquartile range], 296 [233–338] vs. 216 [187–262] U/L, p = .0081).

    Increased serum ALP levels on admission may be able to predict developing END in patients with symptomatic ICAS.

Cerebral venous sinus thrombosis might be under-diagnosed in the COVID-19 era

Banafsheh Shakibajahromi, Afshin Borhani-Haghighi, Sara Haseli, Ashkan Mowla

A thromboembolic mechanism in bow hunter's stroke: Importance of hemodynamic evaluation by ultrasonography during head rotation

Nobuyuki Eura, Kozue Saito, Hironori Shimizu, Yasuhiro Takeshima, ... Kazuma Sugie

Acute hypothermia in Covid 19: A case report

Nicolas Allard, Ari Maruani, Corina Cret, Alain Ameri