eNeurologicalScieNeurologicalSci Vol 31

June 2023

ABCs of Headache

Advocacy for patients with headache disorders

Riadh Gouider, Deborah Henscheid Lorenz, Audrey Craven, Wolfgang Grisold, David W. Dodick

  • Article 100466
  • https://doi.org/10.1016/j.ensci.2023.100466
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  • Abstract

    Primary headache disorders are worldwide highly prevalent and burdensome and should be therefore considered as a global public health priority. However, too many patients with primary headache disorders still do not receive satisfying care. The most likely identified reasons for such a scenario - lack of public awareness, stigma, lack of trained professionals with inadequate healthcare systems and policies - are remediable. Despite the progresses that were made in headache advocacy, these efforts have not yielded substantial improvements in research funding or access to specialty care and even standards of care. The situation is more complex in Low and Middle Income Countries (LMICs) where headache advocacy is urgently needed given the magnitude of the difficulties that patients with primary headache disorders face in accessing care. The growing emergence of coordinated, collaborative, patient-centered advocacy efforts with improved patient-clinician partnership is an opportunity to enhance progress in advocacy for a satisfying life and optimal and equitable care for people with primary headache disorders. LMICs can benefit greatly from coordinating these efforts on a global scale. The recent organization of a training program on headache diagnosis and management for healthcare professionals in Africa is a concrete example.


Review Article

A meta-analysis of post-exercise outcomes in people with amyotrophic lateral sclerosis

Cara Donohue, Giselle Carnaby, Mary Catherine Reilly, Ryan J. Colquhoun, ... Kendrea L. (Focht) Garand

  • Article 100452
  • https://doi.org/10.1016/j.ensci.2023.100452
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    To systematically evaluate post-exercise outcomes related to function and quality of life in people with ALS.

    This study provides inconclusive guidance regarding exercise regimens to maintain function and quality of life in people with ALS due to study limitations (e.g., small sample size, high attrition rate, heterogeneity in methods and participants, etc.). Future research is warranted to determine optimal treatment regimens and dosage parameters in this patient population.

Plasma exchange (PE) versus intravenous immunoglobulin (IVIG) for the treatment of Guillain-Barré syndrome (GBS) in patients with severe symptoms: A systematic review and meta-analysis

Hany A. Zaki, Haris Iftikhar, Mavia Najam, Maarij Masood, ... Eman E. Shaban

  • Article 100468
  • https://doi.org/10.1016/j.ensci.2023.100468
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  • Abstract

    Guillain- Barré syndrome (GBS) is a neuropathic condition that leads to the rapid development of impairments and is characterized by weakness and numbness or tingling sensation in the legs and arms and sometimes loss of movement and feeling in the legs, arms, upper body, and face. Currently, the cure for the disease is yet to be developed. However, treatment options such as intravenous immunoglobulin (IVIG) and plasma exchange (PE) have been used to minimize the symptoms and duration of the disease. Therefore, this systematic review and meta-analysis compared the efficacy of IVIG and PE in treating GBS patients with severe symptoms.

    Six electronic databases, including PubMed, Embase, Scopus, ScienceDirect, Medline, and Google scholar, were scoured for articles related and relevant to our research. Additionally, more studies were obtained through the reference lists of the studies retrieved from these electronic databases. Quality assessment and statistical data analysis were conducted using Review Manager software (RevMan 5.4.1).

    Our study suggests that IVIG and PE have similar curative effects. Similarly, IVIG seems easier to use and thus can be preferred for treating GBS.

Gait quality after robot therapy compared with physiotherapy in the patient with incomplete spinal cord injured: A systematic review

Isabella Fabbri, Fabio Betti, Roberto Tedeschi

  • Article 100467
  • https://doi.org/10.1016/j.ensci.2023.100467
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  • Abstract

    Spinal cord injury results in the interruption of neuronal conduction in the spinal cord, a condition that occurs in 0.1% of the world's population. This results in severe limitations in autonomy including locomotor function. Its recovery can be pursued through conventional isolated physiotherapeutic rehabilitation (overground walking training - OGT) or associated with Robot-assisted gait training - RAGT (e.g.: Lokomat ®).

    The aim of this review is to compare the effectiveness of RAGT combined with conventional physiotherapy.


Original Articles

Predictive factors for clinical outcomes in Filipino patients with watershed infarction: A single center study

Marian Irene C. Escasura, Jose C. Navarro, Stroke Data Bank Investigators

  • Article 100450
  • https://doi.org/10.1016/j.ensci.2023.100450
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  • Abstract

    Watershed infarcts denote ischemic lesions involving the distal territories of two major arteries. For years, hypotheses on its pathophysiological mechanisms have been proposed. Yet, the cause is still widely debated. This study aimed to determine the mechanism of watershed strokes and compare their clinical outcomes to acute ischemic stroke from other causes and predict the factors affecting clinical outcomes in patients with watershed infarcts.

    Clinical outcomes between watershed infarcts and acute ischemic strokes were similar. Hemodynamic compromise in the setting of severe stenosis is the underlying mechanism for both types of watershed strokes thus, the goal of treatment is to maintain adequate perfusion. High baseline NIHSS score, increased age, female gender and underlying malignancy were all poor predictors of clinical outcomes in patients with watershed strokes.

A neurodevelopmental disorder caused by a dysfunctional CACNA1A allele

Audra A. Kramer, Daniel F. Bennett, Kristin W. Barañano, Roger A. Bannister

  • Article 100456
  • https://doi.org/10.1016/j.ensci.2023.100456
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  • Abstract

    P/Q-type Ca2+ flux into nerve terminals via CaV2.1 channels is essential for neurotransmitter release at neuromuscular junctions and nearly all central synapses. Mutations in CACNA1A, the gene encoding CaV2.1, cause a spectrum of pediatric neurological disorders. We have identified a patient harboring an autosomal-dominant de novo frameshift-causing nucleotide duplication in CACNA1A (c.5018dupG). The duplicated guanine precipitated 43 residues of altered amino acid sequence beginning with a glutamine to serine substitution in CaV2.1 at position 1674 ending with a premature stop codon (CaV2.1 p.Gln1674Serfs*43). The patient presented with episodic downbeat vertical nystagmus, hypotonia, ataxia, developmental delay and febrile seizures. In patch-clamp experiments, no Ba2+ current was observed in tsA-201 cells expressing CaV2.1 p.Gln1674Serfs*43 with β4 and α2δ-1 auxiliary subunits. The ablation of divalent flux in response to depolarization was likely attributable to the inability of CaV2.1 p.Gln1674Serfs*43 to form a complete channel pore. Our results suggest that the pathology resulting from this frameshift-inducing nucleotide duplication is a consequence of an effective haploinsufficiency.

Epidemiology of amyotrophic lateral sclerosis in the north east Tuscany in the 2018–2021 period

Sabrina Mata, Matteo Bussotti, Monica Del Mastio, Alessandro Barilaro, ... Sandro Sorbi

  • Article 100457
  • https://doi.org/10.1016/j.ensci.2023.100457
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  • Abstract

    The incidence of Amyotrophic Lateral Sclerosis (ALS) varies among different geographical areas and seems to increase over time. This study aimed to examine the epidemiologic data of ALS in the north-east Tuscany and compare the results with those of similar surveys.

    ALS epidemiological features in north-east Tuscany are in line with other Italian and European Centers. The dramatic increase of the local disease burden over the last decades probably reflects better ascertainment methods and health system.

Wide symptom variability of cerebellar infarction and comparison with pons infarction

Toralf Brüning, Tamara Beyrich, Mohamed Al-Khaled

  • Article 100459
  • https://doi.org/10.1016/j.ensci.2023.100459
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  • Abstract

    Cerebellar infarction (CI) is a serious cerebrovascular disease that may present with non-focal neurological deficits, leading to delay of clinical recognition and treatment. The aim of this study is to investigate the variability of symptoms, diagnostic outcomes and early prognosis in patients with cerebellar infarction compared with pontine infarction (PI).

    Cerebellar infarction occurs with a high variability of symptoms and should be considered when non-focal symptoms are present.

Clinico-topographic evaluation of anterior versus posterior acute ischemic stroke and correlation with early mortality-based scale prediction

Esra Demir Unal

  • Article 100458
  • https://doi.org/10.1016/j.ensci.2023.100458
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  • Abstract

    Posterior circulation ischaemic strokes (PCIs) are a clinical syndrome associated with ischemia related to stenosis, in situ thrombosis, or embolic occlusion of the posterior circulation and differ from anterior circulation ischaemic strokes (ACIs) in many aspects. In this study, ACIs and PCIs were evaluated in terms of clinico-radiological and demographic aspects, and the relevance of objective scales to early disability and mortality was investigated.

    The definition of ACIS or PCIS was classified according to the Oxfordshire Community Stroke Project (OCSP). There are mainly two groups divided into ACIs and PCIs. ACIs were included as total anterior circulation syndrome (TACS), partial anterior circulation syndrome (PACS) (right and left), and lacunar syndrome (LACS) (right and left), and PCIs were posterior circulation syndrome (POCS) (right and left). Arrival NIH Stroke Scale/Score (NIHSS) and Glasgow Coma Scale (GCS) scores were evaluated in clinical assessment and modified SOAR Score for Stroke (mSOAR) was for early mortality-based scale prediction. All data were compared, and mean, IQR (if applicable) values and ROC curve analysis were determined.

    The association of PCIs with hyperlipidemia and the male gender was interpreted, and anterior infarcts were found to cause higher early clinical disability scores. The NIHSS scale was effective and reliable, especially in anterior acute strokes, but also emphasized the necessity of using the GCS assessment together in the first 24 h in the assessment of PCIs. mSOAR scale is a helpful predictor in estimating early mortality not only in ACIs but also in PCIs, similar to GCS.

Utility of optical coherence tomography in patients of central immune mediated demyelinating diseases – A prospective study

Monalisa Vegda, Samhita Panda, Kavita R. Bhatnagar

  • Article 100464
  • https://doi.org/10.1016/j.ensci.2023.100464
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  • Abstract

    Optical coherence tomography (OCT) is a non-invasive tool to measure thickness of various layers of retina. Recently, retinal nerve fibre layer (RNFL) and ganglion cell and inner plexiform layer (GCIP) thinning has been observed in OCT in patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD), This study compared OCT profile, along with visual acuity (VA), color vision (CV), contrast saturation (CS) and visual evoked potentials (VEP) in two main cohorts of MS and NMOSD and with controls, during acute episode of optic neuritis (ON), at 3 and 6 months.

    We found that changes of ON were present in 75% of MS eyes and in 45% of NMOSD patients. Of these, subclinical involvement was present in 56.25% of MS eyes and only in 5% of NMOSD eyes suggesting frequent subclinical involvement in the former. Mean RNFL was 95.23 ± 15.53 in MS and 66.14 ± 43.73 in NMOSD after 6 months of ON episode. Thinning of NQ and IQ was observed in NMOSD eyes in the immediate period after ON attack. At 6 months relative sparing of RNFL in TQ was observed in NMOSD ON eyes and MS ON showed predilection for involvement of TQ.


Case Reports

HTLV-1-associated demyelinating neuropathy: A case report and review of the literature

Keiko Tamaki, Takayasu Mishima, Yuji Tateishi, Hidekazu Mera, ... Yoshio Tsuboi

  • Article 100453
  • https://doi.org/10.1016/j.ensci.2023.100453
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  • Abstract

    A 78-year-old man developed paresthesias in the extremities. He was referred to our hospital because of positive anti-human T-cell leukemia virus type 1 (HTLV-1) antibodies in the serum and the presence of abnormal lymphocytes. He was diagnosed as chronic-type adult T-cell leukemia/lymphoma. Neurological examination revealed sensory impairment in the distal parts of the extremities with loss of deep tendon reflexes. Nerve conduction study showed motor and sensory demyelinating polyneuropathy, indicating a diagnosis of HTLV-1-associated demyelinating neuropathy. Corticosteroid therapy followed by intravenous immunoglobulin therapy improved his symptoms. Since demyelinating neuropathy associated with HTLV-1 infection is not well recognized, we here report its characteristics and clinical course through our case report and literature review.

Fulminant Guillain–Barré syndrome secondary to Campylobacter coli infection: An autopsy case report

Fumiya Kutsuna, Momoko Soeda, Aiko Hibino, Masahiro Tokuda, ... Hiroshi Iwanaga

  • Article 100454
  • https://doi.org/10.1016/j.ensci.2023.100454
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  • Abstract

    The most common infection preceding Guillain–Barré syndrome (GBS) is Campylobacter jejuni enteritis, although a few patients present with Campylobacter coli. We report a case of C. coli–induced fulminant GBS. A 61-year-old woman presented with bilateral limb weakness. Nerve conduction studies revealed a reduction of amplitude and C. coli was isolated from a fecal specimen, leading to the diagnosis of GBS. Although the patient was immediately administered immunoglobulin, her symptoms rapidly worsened and she died. Peripheral nerve autopsy revealed myelin ovoid, and infiltration of CD68-positive macrophages into nerves. More effective treatments for fulminant GBS need to be developed.

A rare challenge: A patient with progressive fibrodysplasia ossificans and acute ischemic stroke treated with mechanical thrombectomy.

Antonio Cruz-Culebras

  • Article 100460
  • https://doi.org/10.1016/j.ensci.2023.100460
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  • Abstract

    Fibrodysplasia ossificans progressiva (FOP) is a rare, disabling genetic condition characterized by congenital malformations of the great toes and progressive heterotopic ossification. Here, we briefly describe the case of a 56-year-old male with known FOP and acute ischemic stroke that underwent mechanical thrombectomy with conscious sedation. Treating physicians should be aware of special medical considerations to prevent flare-ups and inflammation that result from any tissue injuries in this disease. Mechanical thrombectomy is a challenging scenario because general anesthesia and injections should be avoided in these patients. The treatment is still preventive and supportive, but this is the first report of the procedure in a patient with FOP.

Successful treatment with subcutaneous ofatumumab in an adolescent patient with refractory myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD)

Satomi Hiya, Hajime Yoshimura, Michi Kawamoto

  • Article 100461
  • https://doi.org/10.1016/j.ensci.2023.100461
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  • Abstract

    Preventing relapse of myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD) with steroids and immunosuppressants is sometimes difficult. There is no standard treatment for refractory cases. We present the case of a 17-year-old female patient with longitudinally extensive myelitis, asymptomatic bilateral optic neuritis, and positive serum MOG-IgG. While taking steroids and several immunosuppressants during the following 14 months, she suffered from two symptomatic relapses in the cerebrum and spinal cord, and multiple asymptomatic relapses in the cerebrum. The patient was negative for MOG-IgG at the second relapse of myelitis. Subcutaneous ofatumumab has suppressed relapse for 13 months. Ofatumumab can be considered a therapeutic option for refractory MOGAD.

Battling the Eagle's sharp beak, Eagle syndrome; a case report

Muhammad Ayhan Amir, Ibad Ur Rehman, Umar Riaz, Hadia Zaheer Lone, ... Hashim Talib Hashim

  • Article 100462
  • https://doi.org/10.1016/j.ensci.2023.100462
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  • Abstract

    Eagle Syndrome is a pain syndrome of rare and unwonted incidence. Forbearer has an elongated styloid process or a calcified stylohyoid ligament, suppressing glossopharyngeal nerve leading to a mélange of symptoms including sporadic cervicofacial pain, headache, and foreign body sensation. Here we present case of a 65 year old military man of south Asian origin, who presented with complaints of sudden episodes of blackouts for past five years and pain in neck while turning head to left for past two months.

    Patient's ultrasound Doppler showed marked narrowing of proximal left internal carotid artery with approximate diametric stenosis of 70% according to The North American Symptomatic Carotid Endarterectomy Trial (NASCET).Further studies of MRI Brain was done,revealing small Foci of restricted diffusion along Territory of Left MCA along with age related Microangiopathic cerebral changes. CT Scan of neck was also done which showed Abnormal elongation of bilateral styloid process more on the left side.

    The case was discussed in a Multidisciplinary Team Meeting comprising ENT surgeon, vascular surgeon and surgical excision was planned through trans cervical approach. Surgery was successful as seen by post op and follow up scans.

Claustrum sparing sign in seronegative limbic encephalitis

Abeer Sabry Safan, Mohammad Al-Termanini, Mohamed Abdelhady, Yasir Osman, ... Ahmed Lutfe Abdussalam

  • Article 100465
  • https://doi.org/10.1016/j.ensci.2023.100465
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  • Abstract

    Limbic encephalitis (LE) is a rare variant of autoimmune encephalitis. It often manifests with subacute neuropsychiatric symptoms of agitation, delusions, variable seizure semiology, and short-term memory loss. Seronegative limbic encephalitis can pose a diagnostic conundrum owing to its inadequately understood pathophysiology.

    We report a rare case of a young male with subacute neuropsychiatric manifestations of delusions, agitations and seizures. He was diagnosed with seronegative limbic encephalitis (SNLE). Brain MRI demonstrated bilateral Claustrum sparing sign. An EEG showed continuous left-sided epileptiform discharges in periodic to predominantly left middle temporal. Patient condition gradually improved with pulsed methylprednisolone, intravenous immunoglobulins and anti-seizure medications.


Letters to the Editor

Response-monitored dual anti-platelet treatment in a patient with spontaneous post-partum four-vessel cervical artery dissectio

Ina Nørgaard, Jakob Stensballe, Götz Benndorf, Thomas Truelsen

  • Article 100451
  • https://doi.org/10.1016/j.ensci.2023.100451
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Relapse of neuromyelitis optica spectrum disorder after BNT162b2 mRNA Covid-19 vaccination

Qi Wang, Jin-Ju Kang, Yutong Bai, Sun-Young Oh

  • Article 100455
  • https://doi.org/10.1016/j.ensci.2023.100455
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Spinocerebellar Ataxia type 17 presenting with progressive myoclonic epilepsy

Apisit Boongird, Pichet Termsarasab, Teeratorn Pulkes

  • Article 100463
  • https://doi.org/10.1016/j.ensci.2023.100463
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Beta-propeller protein-associated neurodegeneration: A clinical update with a case report

Moustafa A. Mansour, Yehia Moawad, Hassan Ali

  • Article 100469
  • https://doi.org/10.1016/j.ensci.2023.100469
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