Navigating the clinical landscape of artery of Percheron infarction: A systematic review

Oday Atallah, Yasser F. Almealawy, Arwa Salam Alabide, Minaam Farooq, ... Amr Badary

• Article 100521
• https://doi.org/10.1016/j.ensci.2024.100521
• View PDF

• Abstract

  Infarction of the artery of Percheron (AOP) is a rare vascular condition where a single arterial branch supplies blood to the thalamic and midbrain regions, leading to neurological deficits. The challenge lies in its often-delayed diagnosis due to its rarity and diverse clinical presentations, necessitating heightened awareness among clinicians for expedited diagnosis and appropriate therapeutic interventions.

  All relevant studies involving patients diagnosed with infarction of AOP were retrieved from PubMed, Google Scholar, Web of Science, and Scopus. Only human studies that were published in full English-language reports were included. Included in the search were the terms "Artery of Percheron," "infarction," "stroke," and "demarcation". Age, gender, presenting symptoms, treatment, recovery time, and outcome of patients with AOP infarction were all recorded.

  A systematic review was conducted on a total of 530 articles, out of which 130 articles met the specified requirements. The average age is 59, with men comprising 57.7% of the population. The symptoms reported were visual disturbance in 43.9% of cases and changed mental state in 77.2% of cases. Treatment options include conservative management (85.4%), thrombolysis (11.3%), and other approaches. The optimal age range for recovery is between 41 and 50 years old.

  Our study on acute AOP infarction highlights male predominance, common comorbidities like hypertension and diabetes, and prevalent symptoms including visual disturbance and altered mental state. Early recognition is crucial, with thrombolytic therapy within the critical time window showing promising outcomes. These findings offer insights for enhanced clinical management of AOP infarction.

Evaluation of nerve fiber layer and ganglion cell complex changes in patients with migraine using optical coherence tomography

Yasmin Walha, Mona Rekik, Khadija Sonda Moalla, Sonda Kammoun, ... Amira Trigui

• Article 100525
• https://doi.org/10.1016/j.ensci.2024.100525
• View PDF

• Abstract

  To analyze changes in peripapillary retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thickness in migraine patients with and without aura compared to healthy controls and to identify factors influencing the occurrence of these anomalies.

  This is a cross-sectional case-control study including migraine patients and control subjects. All patients and controls underwent a complete ophthalmological examination, RNFL and GCC thickness measurements using a spectral domain-OCT device.

  The duration of migraine, the frequency and duration of migraine attacks, the migraine disability assessment (MIDAS) and migraine severity scale (MIGSEV) questionnaire scores were recorded.

  One hundred and twenty eyes from 60 patients (60 eyes in the migraine without aura (MWoA) group and 60 eyes in the migraine with aura (MWA) group) were included. Control group included 30 age and gender matched healthy participants (60 eyes). OCT revealed that RNFL and GCC thickness were significantly reduced in the migraine without aura (MWoA) and in the migraine with aura (MWA) groups compared to the control group and in the migraine with aura (MWA) group compared to the migraine without aura (MWoA) group. Prolonged disease duration was associated to decreased GCC thickness. RNFL and GCC thickness were correlated to disease severity, attack frequency and duration. In the multivariate study, duration of migraine and attack frequency were the main determinant factors of nasal GCC thickness. Disease severity was the main determinant of RNFL and GCC thickness, with the exception of the nasal sector.

  Our study emphasize the significant impact of both types of migraine on retinal structures. OCT would serve as a valuable biomarker in migraine.

Levodopa / opicapone as a complement to STN-DBS in clinical practice. A retrospective single-centre analysis.

Moritz A. Loeffler, Philipp Klocke, Idil Cebi, Alireza Gharabaghi, Daniel Weiss

• Article 100530
• https://doi.org/10.1016/j.ensci.2024.100530
• View PDF

• Abstract

  Deep brain stimulation of the subthalamic nucleus (STN-DBS) is a well-established treatment option in Parkinson's disease with motor and non-motor fluctuations allowing for postoperative reduction of dopaminergic medication. However, evidence is scarce on optimal medication adjustments following STN-DBS implantation. Opicapone allows for long-lasting inhibition of the catechol-O-methyltransferase (COMT) thereby enabling more constant dopaminergic stimulation compared to levodopa alone. However, especially COMT inhibitors are regularly discontinued after STN-DBS surgery. In this single-centre retrospective analysis, we aimed to analyse the clinical phenotype of patients selected for opicapone treatment following STN-DBS implantation and to define clinical determinants of patients requiring more intense dopamine-stabilising strategies after STN-DBS implantation.

  A patient cohort treated with STN-DBS + levodopa + opicapone (n = 16) was compared to an age-matched control cohort without opicapone treatment at baseline before and ≥ 5 months post-surgery. As main outcomes we assessed the MDS-UPDRS III and IV scores and reduction of the cumulative dopaminergic medication quantified by the levodopa equivalent dosages (LED).

  Whilst the MDS-UPDRS III (median [min – max]) in patients with STN-DBS as well as anatomical electrode positions did not differ significantly between the opicapone 20 [4–40] and control cohort 14 [1–44], the patients selected for opicapone treatment showed a significantly higher degree of dyskinesias already preoperatively as reflected by a UPDRS-IV A subscore of 2 [0–4] compared to controls 0 [0–4]. Postoperatively, the opicapone cohort showed stronger motor fluctuations MDS-UPDRS IV 6 [0–14] compared to the controls 0 [0−10], albeit without statistical significance. Moreover, the opicapone cohort showed significantly less reduction of dopaminergic medication (−36.4 % vs. -46.2 % in the control cohort) following STN-DBS implantation independent from the intake of dopamine agonists.

  These results indicate a clinical phenotype characterised by more motor fluctuations requiring a more stable dopamine replacement therapy to address the patients' disease biology. In these cases, levodopa + COMT inhibition by opicapone represents a therapeutic approach but determination of the potential clinical benefit requires further prospective studies.

Impact of physical activity on quality of life in patients with epilepsy in a developing country: A cross-sectional, survey-based study

Daniel San-Juan, Miguel Benjamín Cervera-Sánchez, Miguel Angel Morales-Morales, Emilio Israel Wong-Valenzuela, ... Daniela Carolina Pimentel-Saona

• Article 100533
• https://doi.org/10.1016/j.ensci.2024.100533
• View PDF

• Abstract

  Patients with epilepsy face different barriers based on ignorance and fear of themselves and others while exercising, this situation complicates attempts at exercise and promotes discrimination against patients. Physical activity has been associated with favorable results in the health of this population and can be used as a non-pharmacological treatment seeking to increase the health and improve living conditions of patients. Our study aims to explore how physical activity impacts quality-of-life perception in these patients.

  We performed a cross-sectional, survey-based study conducted at the outpatient Epilepsy Clinic in the National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez". Patients between 17 and 65 years of age and with an established diagnosis of epilepsy were considered for participation. A 31-item questionnaire was administered to all participants face-to-face or by telephone. The questions included in the survey were selected from the Global Physical Activity Questionnaire and the Quality of Life in Epilepsy Inventory to assess the level of exercise and perceived quality of life of participants. Descriptive and inferential Mann-Whitney U test statistical analysis was done using IBM SPSS Statistics 23.0 software.

  Participants' mean age was 35.69 ± 11.93 years, 45 % were male and 55 % female. Significant differences were found in the domain of perceived quality of life in the self-reported physically active participants (p = 0.0256; 95 % IC: 1.00–15.22), with a mean score of 75.78 ± 16.64 in the active group vs. 67.66 ± 15.86 in the sedentary group. In those who complied with the exercise recommendations according to the WHO, there was significant evidence of improvement in the general quality of life (p = 0.0032; 95 % IC:4.9–23.9), with a mean score of 75.22 ± 15.92 in the active group vs. 60.76 ± 17.836 in the sedentary group.

  Physical activity is associated with a significant impact on how patients with epilepsy perceive their quality of life. In addition, it improves memory and concentration in the tasks they perform in their daily lives.

Neurobehavioral features in medication-overuse headache

Franz Riederer, Roberto Pirrotta, Chantal Martin Soelch, Andreas R. Gantenbein, ... Peter S. Sándor

• Article 100538
• https://doi.org/10.1016/j.ensci.2024.100538
• View PDF

Tiny infarction of rostral cerebellum manifested by contralesional body lateropulsion

Obay Alalousi, Mickael Bonnan

• Article 100523
• https://doi.org/10.1016/j.ensci.2024.100523
• View PDF

• Abstract

  Body lateropulsion (BLP) has been reported several times after cerebellar infarction. It is usually ipsilateral to the cerebellar infarction, particularly when limited to the rostral cerebellum. In contrast, contralesional BLP after cerebellar infarction has been reported in more caudal regions of the cerebellum (such as the nodulus or the tonsil).

  We report the case of a small infarction of the left anterior paravermis of the rostral cerebellum which resulted in bilateral symptoms: ipsilesional limb ataxia and, unexpectedly, contralesional BLP.

  Several neurological pathways were potentially involved. Both right and left dorsal spinocerebellar tracts may have been damaged by the infarction of the left anterior paravermis. On the other hand, the proximity of the infarct to the superior cerebellar peduncle may have caused damage to the vestibular pathways (fastigio-vestibular or dentato-vestibular tracts), as they exit the cerebellum by the superior cerebellar peduncle. A lesion of the cerebellum close to the superior cerebellar peduncle could result in a contralesional BLP.

Dynamic aphasia as an early sign of corticobasal degeneration: Clinico-radio-pathological correlation

Masanori Kurihara, Akira Arakawa, Aya Midori Tokumaru, Tomoyasu Matsubara, ... Yuko Saito

• Article 100526
• https://doi.org/10.1016/j.ensci.2024.100526
• View PDF

• Abstract

  A 72-year-old man presented with a 6-month history of decreased voluntary speech. Sparse speech and decreased word fluency were observed. Articulation, naming, comprehension, and repetition were preserved. Agrammatism and paraphasia were not observed. These characteristics matched those reported as dynamic aphasia. Other findings were mild behavioral symptoms, recent memory impairment, and right hemiparkinsonism. The patient's voluntary speech continued to reduce and behavioral symptoms progressed. Brain MRI including voxel-based morphometric analysis showed left-dominant white matter volume reduction in the frontal lobe including those between the left supplementary motor area (SMA)/preSMA and the frontal operculum, likely involving the frontal aslant tract (FAT). The patient became completely mute after two years from disease onset and died of aspiration pneumonia. The neuropathological diagnosis was corticobasal degeneration (CBD). This case suggests that dynamic aphasia may be the initial sign of CBD and that early involvement of left FAT may be responsible for this feature.

Paraneoplastic myelitis associated with Waldenström macroglobulinemia responsive to treatment with ibrutinib – venetoclax: A case report

Sathya Narasimhan, Kristin M. Galetta, Jorge J. Castillo, Shamik Bhattacharyya

• Article 100527
• https://doi.org/10.1016/j.ensci.2024.100527
• View PDF

• Abstract

  Waldenström macroglobulinemia (WM) is a B-cell lymphoproliferative malignancy characterized by IgM paraproteinemia and presence of lymphoplasmacytic cells in the bone marrow. Isolated longitudinally extensive transverse myelitis (LETM) is a rare manifestation of WM. We report a rare case of paraneoplastic LETM in a 68-year-old male with treatment-naïve WM (MYD88 L265P mutation in bone marrow aspirate), who responded to ibrutinib and venetoclax therapy. Our patient presented with a two-month history of unsteadiness, tingling, and numbness in both hands and feet, that progressed to bilateral leg and arm weakness. Based on radiographic findings, a diagnosis of paraneoplastic LETM was made and he was treated acutely with IV methylprednisolone followed by a quick oral prednisone taper. However, he subsequently relapsed and symptomatically worsened while on rituximab therapy. Accounting for worsening anemia, our patient was enrolled in a Phase II trial evaluating the effects of ibrutinib-venetoclax therapy in treatment naïve WM. After three months of study therapy, he had a complete response of myelopathy symptoms and MRI lesions. Our observation of sustained disease response in this patient may support a role for concurrent BTK and BCL2 inhibition in paraneoplastic myelitis associated with B-cell lymphoproliferative disorders. However, this observation needs to be validated in larger cohort studies and potentially in clinical trials if further data are supportive.

Internal carotid artery dissection in a patient with Parkinson's disease after COVID-19 infection

Takanobu Okubo, Hidehiro Ishikawa, Keita Matsuura, Asako Tamura, ... Akihiro Shindo

• Article 100529
• https://doi.org/10.1016/j.ensci.2024.100529
• View PDF

Double trouble: The intersection of neurobrucellosis and systemic lupus erythematosus in a 24-year-old female

Hashim Talib Hashim, Aws Murad Frhood, Ahmed Abdulhussain Shahatta, Ahmed Dheyaa Al-Obaidi, Arshed Shakir Kadim

• Article 100531
• https://doi.org/10.1016/j.ensci.2024.100531
• View PDF

• Abstract

  Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organs, while brucellosis is a zoonotic infection prevalent in endemic areas. Neurobrucellosis, a severe complication of brucellosis, can mimic or coexist with autoimmune conditions like SLE, complicating diagnosis and treatment. This case report highlights the diagnostic challenges and management strategies for such overlapping diseases.

  In this case report, we present a 24-year-old female who initially presented with symptoms that were misleadingly attributed to a simple vaginal ulcer. Her clinical course evolved to include migratory polyarthralgia, nocturnal fevers, and significant weight loss, eventually culminating in severe headaches and dizziness that led to a diagnosis of meningitis. Comprehensive diagnostic work-up, including the presence of a malar rash, positive anti-dsDNA antibodies, and elevated inflammatory markers, pointed towards SLE. However, the identification of bacterial infection markers in the cerebrospinal fluid (CSF) and her history of animal contact in an endemic area led to the diagnosis of neurobrucellosis. The patient's remarkable response to doxycycline and rifampicin confirmed the infectious etiology, highlighting the complex interplay between these two diseases.

  The intersection of SLE and neurobrucellosis in this patient underscores the importance of a multidisciplinary approach to diagnosis and treatment, ensuring that both the autoimmune and infectious aspects of the patient's condition are adequately addressed. This case contributes valuable insights into the management of such rare and complex presentations, emphasizing the need for vigilance and adaptability in clinical practice.

De novo myasthenia gravis in a patient with malignant melanoma after concurrent SARS-CoV-2 vaccination and immune checkpoint inhibitor therapy: Case report and literature review

• Mohadese Shahin, Pedram Fadavi, Mohammad Mostafa Ansari Ramandi, Soroush Shahrokh, Farzad Taghizadeh-Hesary
  • Article 100534
  • https://doi.org/10.1016/j.ensci.2024.100534
  • View PDF

Thalamic deep brain stimulation for postural tremor caused by hyperthermia-induced cerebellar dysfunction: A case report

• Mitsuyoshi Tamura, Shigeki Hirano, Yoshihisa Kitayama, Marie Morooka, ... Satoshi Kuwabara
  • Article 100536
  • https://doi.org/10.1016/j.ensci.2024.100536
  • View PDF

  • Abstract

    The efficacy of deep brain stimulation (DBS) in treating tremor symptoms in cerebellar disorders remains unclear.

    A 47-year-old woman presented with neck and arm tremor and ataxic speech/gait after four days of >40 °C fever due to septic shock attributed to lithiasis-pyelonephritis. Left ventral intermediate nucleus thalamus DBS alleviated contralateral postural arm tremor, although the action tremor and terminal oscillation remained unchanged.

    To our knowledge, this is the first report of thalamic DBS for hyperthermia-induced cerebellar dysfunction. Patients with postural tremor resulting from cerebellar damage can benefit from thalamic DBS, leading to improved activities of daily living.

     

Evaluation of the gold cost criteria as a diagnostic criteria of amyotrophic lateral sclerosis

• Ramez M. Odat, Omar Alomari, Anas Elgenidy
  • Article 100524
  • https://doi.org/10.1016/j.ensci.2024.100524
  • View PDF

Vascular parkinsonism showing dopamine transporter scan findings mimicking those of Parkinson's disease

• Hirotaka Sakuramoto, Hiroaki Fujita, Keitaro Ogaki, Keisuke Suzuki
  • Article 100528
  • https://doi.org/10.1016/j.ensci.2024.100528
  • View PDF

  • Abstract

    Dopamine transporter (DAT) scan is important in the diagnosis of Parkinson's disease (PD). We herein report a patient with vascular parkinsonism (VP) who showed dopamine transporter (DAT) scan findings which mimicked those typically seen in patients with Parkinson's disease (PD). DAT scan findings in patients with VP are characterized by a lower striatal asymmetry index than in patients with PD and decreased uptake in the area consistent with old cerebral infarction or hemorrhage. However, we should be aware that, as in our patient, VP patients with asymmetric basal ganglia lesions, may show increased striatal asymmetry index and asymmetric DAT scan findings.

World neurology updates: Other primary headache disorder – Treatment

• Sina Marzoughi, Peter J. Goadsby
  • Article 100535
  • https://doi.org/10.1016/j.ensci.2024.100535
  • View PDF

IIH, SIH and headache: Diagnosis and treatment update

• Shuu-Jiun Wang
  • Article 100532
  • https://doi.org/10.1016/j.ensci.2024.100532
  • View PDF

  • Abstract

    Idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH) are two major secondary headache disorders resulting from abnormal intracranial pressure (ICP). This short communication outlines the pathophysiology, diagnostic criteria, and management strategies for IIH and SIH. IIH predominantly affects obese women of childbearing age and presents with daily headaches, visual disturbances, and papilledema. It is characterized by elevated cerebrospinal fluid (CSF) pressure, with diagnosis supported by imaging and lumbar puncture. Treatment includes weight reduction, medications, and surgical interventions in refractory cases. SIH, conversely, is caused by spontaneous spinal CSF leaks and presents with acute orthostatic headaches. Diagnosis is supported by neuroimaging and lumbar puncture, revealing low CSF pressure. Treatment includes supported care, (targeted) epidural blood patch, with surgical repair considered in refractory cases. Advances in imaging and treatment have significantly improved outcomes for both conditions.

Myasthenia gravis: The evolving therapeutic landscape

• Gil I. Wolfe, Jonathan E. Hanson, Nicholas J. Silvestri
  • Article 100541
  • https://doi.org/10.1016/j.ensci.2024.100541
  • View PDF

  • Abstract

    Pharmacological options in the management of generalized myasthenia gravis (gMG) have expanded rapidly in the last 7 years. There are now several complement inhibitors and neonatal Fc receptor antagonists on the market in many countries for patient management, following the successful completion of Phase 3 studies. In open-label extensions, these agents have proven to be effective over the longer term extending several years, with benefits such as reduction of corticosteroid requirements being observed. In the communication below, we will briefly summarize recent pharmacologic advancements in the management of gMG and outline how these agents are currently being used and may be used in the future.