Neurology International

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Open AccessReview

Cognitive Impairment in Neuromuscular Diseases: A Systematic Review

by Marco Orsini, Ana Carolina Andorinho de F. Ferreira, Anna Carolina Damm de Assis, Thais Magalhães, Silmar Teixeira, Victor Hugo Bastos, Victor Marinho, Thomaz Oliveira, Rossano Fiorelli, Acary Bulle Oliveira and Marcos R. G. de Freitas

Neurol. Int. 2018, 10(1), 7473; https://doi.org/10.4081/ni.2018.7473 - 04 Jul 2018

Cited by 16 | Viewed by 680

Abstract

Neuromuscular diseases are multifactorial pathologies characterized by extensive muscle fiber damage that leads to the activation of satellite cells and to the exhaustion of their pool, with consequent impairment of neurobiological aspects, such as cognition and motor control. To review the knowledge and [...] Read more.

Neuromuscular diseases are multifactorial pathologies characterized by extensive muscle fiber damage that leads to the activation of satellite cells and to the exhaustion of their pool, with consequent impairment of neurobiological aspects, such as cognition and motor control. To review the knowledge and obtain a broad view of the cognitive impairment on Neuromuscular Diseases. Cognitive impairment in neuromuscular disease was explored; a literature search up to October 2017 was conducted, including experimental studies, case reports and reviews written in English. Keywords included Cognitive Impairment, Neuromuscular Diseases, Motor Neuron Diseases, Dystrophinopathies and Mitochondrial Disorders. Several cognitive evaluation scales, neuroimaging scans, genetic analysis and laboratory applications in neuromuscular diseases, especially when it comes to the Motor Neuron Diseases, Dystrophinopathies and Mitochondrial Disorders. In addition, organisms model using rats in the genetic analysis and laboratory applications to verify the cognitive and neuromuscular impacts. Several studies indicate that congenital molecular alterations in neuromuscular diseases promote cognitive dysfunctions. Understanding these mechanisms may in the future guide the proper management of the patient, evaluation, establishment of prognosis, choice of treatment and development of innovative interventions such as gene therapy. Full article

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Open AccessArticle

Efficacy of Physical Therapy Associated with Botulinum Toxin Type A on Functional Performance in Post-Stroke Spasticity: A Randomized, Double-Blinded, Placebo-Controlled Trial

by Antonio Prazeres, Marília Lira, Paula Aguiar, Larissa Monteiro, Ítalo Vilasbôas and Ailton Melo

Neurol. Int. 2018, 10(1), 7385; https://doi.org/10.4081/ni.2018.7385 - 04 Jul 2018

Cited by 11 | Viewed by 760

Abstract

The aim was to investigate if botulinum toxin type A (BTx-A) associated with physical therapy is superior to physical therapy alone in post stroke spasticity. A randomized, double-blinded controlled trial was performed in a rehabilitation unit on Northeastern, Brazil. Patients with post stroke [...] Read more.

The aim was to investigate if botulinum toxin type A (BTx-A) associated with physical therapy is superior to physical therapy alone in post stroke spasticity. A randomized, double-blinded controlled trial was performed in a rehabilitation unit on Northeastern, Brazil. Patients with post stroke spasticity were enrolled either to BTx-A injections and a pre-defined program of physical therapy or saline injections plus physical therapy. Primary endpoint was functional performance evaluated through time up and go test, six minutes walking test and Fugl-Meyer scale for upper limb. Secondary endpoint was spasticity improvement. Confidence interval was considered at 95%. Although there was a significant decrease in upper limbs flexor tonus (P<0.05) in the BTx-A group, there was no difference regarding functional performance after 9 months of treatment. When analyzing gait speed and performance, both groups showed a significant improvement in the third month of treatment, however it was not sustained over time. Although BTx-A shows superiority to improve muscle tone, physical therapy is the cornerstone to improve function in the upper limbs of post stroke patients. Full article

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Open AccessCase Report

A Giant Cholesteatoma of the Mastoid Extending into the Foramen Magnum: A Case Report and Review of Literature

by Seidu A Richard, Li Qiang, Zhi Gang Lan, Yuekang Zhang and Chao You

Neurol. Int. 2018, 10(1), 7625; https://doi.org/10.4081/ni.2018.7625 - 04 Apr 2018

Cited by 5 | Viewed by 731

Abstract

Cholesteatomas are very rare benign, progressive lesions that have embryologic derivation and usually result in progressive exfoliation and confinement of squamous epithelium behind an intact or preciously infected tympanic membrane. To the best of our understanding no reports demonstrates the extension of cholesteatoma [...] Read more.

Cholesteatomas are very rare benign, progressive lesions that have embryologic derivation and usually result in progressive exfoliation and confinement of squamous epithelium behind an intact or preciously infected tympanic membrane. To the best of our understanding no reports demonstrates the extension of cholesteatoma from the temporal bone into the foramen magnum. We therefore present a case of cholesteatoma extending down into the foramen magnum. We report a case of 67- year-old man with a giant cholesteatoma extending into the foramen magnum without substantial destruction of the mastoid and petrous temporal bones. The patient’s major symptoms were recurrent tinnitus in the left ear and dizziness with unilateral conductive hearing loss. A working diagnosis of cholesteatomas was made combining the symptoms and magnetic resonance imaging findings. He was then successfully operated on with very minimal postoperative complications. Cholesteatomas originating from the mastoid bone often linger with the patients for many years in a subclinical state and progress into a massive size before causing symptoms. Patients with unilateral conductive hearing loss who are otherwise asymptomatic and have a normal tympanic membrane should be suspected with a progressive cholesteatoma. Cholesteatoma should be one of the working diagnosis when an elderly patient present with unilateral conductive hearing loss that is associated with tinnitus and dizziness. Full article

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Open AccessArticle

Stroke Networks and Telemedicine: An Italian National Survey

by Laura Bernetti, Graziamaria Nuzzaco, Francesco Muscia, Alessio Gamboni, Marialuisa Zedde, Paolo Eusebi, Mauro Zampolini and Francesco Corea

Neurol. Int. 2018, 10(1), 7599; https://doi.org/10.4081/ni.2018.7599 - 04 Apr 2018

Cited by 12 | Viewed by 512

Abstract

Stroke is the leading cause of disability and death. Nowadays, clinical benefits of stroke units and thrombolysis in ischemic stroke are evidence-based. Also the benefit of endovascular treatment for acute ischemic stroke has been established. Telemedicine has been used to improve access to [...] Read more.

Stroke is the leading cause of disability and death. Nowadays, clinical benefits of stroke units and thrombolysis in ischemic stroke are evidence-based. Also the benefit of endovascular treatment for acute ischemic stroke has been established. Telemedicine has been used to improve access to care by allowing a neurologist at a remote location to interact with the patient and their family members. Prior studies have shown that the use of telemedicine for acute ischemic stroke is not only safe and effective, but it also increases the utilization of tPA, improving patient outcomes. This study aimed to investigate the diffusion of telemedicine in Italian stroke networks with an online questionnaire to assess: type of stroke care setting, Volume of thrombolysis- thrombectomy/year, access to stroke care between different hospitals, the presence of imaging sharing protocols within the network or patients dispatchment screening; type of network solutions. We have interviewed 24 Italian neurologists, working in large urban areas, from north southward, including Italian islands. In particular, these neurologists represented 14 different regions and 20 countries. A majority of neurologists replying to the survey (47.83%) worked in large general hospitals or smaller general hospitals (26%) and a smaller number of physicians (17.3%) were committed in University Hospital or (8.7%) independent foundation hospitals. The 60.87% of stroke networks involved in the survey had a low thrombolysis/year volume while the 30.43% had a thrombolysis/year volume above 100. According to the survey a local stroke network was established in 87.50% of cases. In the 45.83% of cases, the hospitals care is not homogeneous within the network. A network for the consultation of neuroimaging between hospitals is available in 33.33% of cases. Whitin those describing an active network for Teleconsult the 57.14% used personal devices, while only the 25 % use professional teleconference system, and in 25% of cases used medical devices. Our findings demonstrated a relevant diffusion of Teleconsult in Italian stroke networks. The systems adopted are mostly individual solutions not integrated in protocolled pathways. These findings may encourage a systematization of Telemedicine medical curricula to increase larger access to neurological consults. Full article

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Open AccessCase Report

Glioblastoma Multiforme Subterfuge as Acute Cerebral Hemorrhage: A Case Report and Literature Review

by Seidu A. Richard, Yunxia Ye, Hao Li, Lu Ma and Chao You

Neurol. Int. 2018, 10(1), 7558; https://doi.org/10.4081/ni.2018.7558 - 04 Apr 2018

Cited by 9 | Viewed by 478

Abstract

Hemorrhagic related Glioblastoma multiforme (GBM) are rare and characterizes with severe clinical scuffle. The etiology of this presentation although not well known is believed to be multifactorial. We present a case as well as review on the pathogenesis of evolution of the hematoma [...] Read more.

Hemorrhagic related Glioblastoma multiforme (GBM) are rare and characterizes with severe clinical scuffle. The etiology of this presentation although not well known is believed to be multifactorial. We present a case as well as review on the pathogenesis of evolution of the hematoma into ring enhancing features of GBM on imaging studies. We present a case of 28 years old man who suddenly went into coma for 9 hours preceded with seizures that latest for 10 minutes. He had no focal neurological signs. CT-Scans images indicated acute cerebral hemorrhage near the frontal horn of the left ventricle with brain edema about the hemorrhagic lesion and MRI done a week later revealed a cerebral ring enhancing lesion. The lesion was partially resected during surgery and immunohistochemical staining confirmed GBM (WHO, grade 4). The diagnosis of intratumoral hemorrhage in GBM was very challenging at the initial stages but with time the hematoma evolved into ring enhancing images typical of GBM. It’s not every intracranial hematoma that is of pure vascular origin. Full article

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Open AccessReview

Pantothenate Kinase-Associated Neurodegeneration: Clinical Aspects, Diagnosis and Treatments

by Saeed Razmeh, Amir Hassan Habibi, Maryam Orooji, Elham Alizadeh, Karim Moradiankokhdan and Behroz Razmeh

Neurol. Int. 2018, 10(1), 7516; https://doi.org/10.4081/ni.2018.7516 - 04 Apr 2018

Cited by 13 | Viewed by 556

Abstract

Pantothenate Kinase-Associated Neurodegeneration (PKAN) is an autosomal recessive disorder characterized by a mutation in the PANK2 gene. The clinical presentation may range from only speech disorder to severe generalized dystonia, spasticity, Visual loss, dysphagia and dementia. The hallmark of this disease is eyes [...] Read more.

Pantothenate Kinase-Associated Neurodegeneration (PKAN) is an autosomal recessive disorder characterized by a mutation in the PANK2 gene. The clinical presentation may range from only speech disorder to severe generalized dystonia, spasticity, Visual loss, dysphagia and dementia. The hallmark of this disease is eyes of the tiger sign in the medial aspect of bilateral globus pallidus on T2-weighted MRI that is a hyperintense lesion surrounded by hypointensity. Common treatments for PKAN disease include anticholinergics, botulinum toxin, Oral and Intrathecal baclofen, Iron chelation drugs and surgical procedures such as ablative pallidotomy or thalamotomy, Deep brain stimulation. There are many controversies about the pathogenesis and treatment of this disease, and in recent years interesting studies have been done on PKAN disease and other similar diseases. This review summarizes the clinical presentation, etiology, imaging modalities and treatment. Full article

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Open AccessArticle

Migraine: Incorrect Self-Management for a Disabling Disease

by Giovanna Viticchi, Lorenzo Falsetti, Marco Bartolini, Laura Buratti, Alfio Ulissi, Maurizio Baldassarri, Leandro Provinciali and Mauro Silvestrini

Neurol. Int. 2018, 10(1), 7510; https://doi.org/10.4081/ni.2018.7510 - 04 Apr 2018

Cited by 10 | Viewed by 482

Abstract

Migraine is an economically and socially relevant pathology but patients’ consciousness and consequently a proper management is often unsatisfactory. In this study, we aimed to evaluate the disease awareness, the general approach and the impact on working activity in a population of headache-affected [...] Read more.

Migraine is an economically and socially relevant pathology but patients’ consciousness and consequently a proper management is often unsatisfactory. In this study, we aimed to evaluate the disease awareness, the general approach and the impact on working activity in a population of headache-affected hospital workers. During an 18-month period, hospital workers responded to a questionnaire investigating headache presence, characteristics and awareness. Specific attention was paid to define headache impact on working activities. 1774 consecutive subjects were enrolled. Headache was documented in 45.7% of the sample, migraine in 16.6%. 40.6% of the patients with headache and 50.7% of the migraneous subjects had a specialist medical evaluation. Triptans use was significantly lower than non-steroidal anti-inflammatory drugs use. 39.5% of migraneous complained of attacks during working time, 60.2% acknowledged a negative influence on work quality. 25.5% of migraneous patients reported a loss of working days attributable to the disease. Migraine is disabling for workers with negative consequences on job capacity and quality. Efforts should be made to improve disease awareness to stimulate a better diagnostic and therapeutic approach. Full article

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Open AccessEditorial

Risk Stratification in Laminopathies and Emery Dreifuss Muscular Dystrophy

by Abdallah Fayssoil

Neurol. Int. 2018, 10(1), 7468; https://doi.org/10.4081/ni.2018.7468 - 04 Apr 2018

Cited by 4 | Viewed by 406

Abstract

Laminopathies are genetic disorders due to gene mutation encoding for proteins of the nuclear envelope. Patients are at risk of conduction defect, arrhythmia, sudden death and heart failure. The authors summarize predictive factors for cardiac events reported in the literature in this group [...] Read more.

Laminopathies are genetic disorders due to gene mutation encoding for proteins of the nuclear envelope. Patients are at risk of conduction defect, arrhythmia, sudden death and heart failure. The authors summarize predictive factors for cardiac events reported in the literature in this group of disease. Full article

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Open AccessEditorial

Glioma Research: Early Detection of a Downward Trend in Publication Productivity?

by Michael A. Meyer

Neurol. Int. 2018, 10(1), 7562; https://doi.org/10.4081/ni.2018.7562 - 30 Mar 2018

Cited by 1 | Viewed by 354

Abstract

Global publication productivity for brain tumors was examined on a annual basis and compared to other neurologic disorders including stroke, epilepsy and Alzheimer’s disease. An early downward trend is suggested for 2016; possible links to fluctuations in funding is discussed. Full article

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Open AccessArticle

Multilingual Website and Cyberconsultations for Oromandibular Dystonia

by Kazuya Yoshida

Neurol. Int. 2018, 10(1), 7536; https://doi.org/10.4081/ni.2018.7536 - 30 Mar 2018

Cited by 18 | Viewed by 455

Abstract

Oromandibular dystonia is a focal dystonia that manifests as involuntary masticatory and/or tongue muscle contractions. This movement disorder is frequently misdiagnosed as a temporomandibular disorder. Hence, it would be useful to establish a method that makes it possible for patients with the condition [...] Read more.

Oromandibular dystonia is a focal dystonia that manifests as involuntary masticatory and/or tongue muscle contractions. This movement disorder is frequently misdiagnosed as a temporomandibular disorder. Hence, it would be useful to establish a method that makes it possible for patients with the condition to find appropriate medical institutions by themselves. The author produced a website Involuntary movements of the stomatognathic region (https://sites. google.com/site/oromandibulardystoniaenglish/) for patients with oromandibular dystonia, which is available in twenty languages. It has been viewed more than 1,000,000 times by individuals from all over the world. The visitors to the site have completed questionnaires and/or sent images or videos of their involuntary movements over the internet. Cyberconsultations (remote diagnosis) were also performed via Skype™. Approximately 1000 patients with involuntary stomatognathic movements visited our department. Only 12.5% of the patients had previously been diagnosed with or were suspected to have dystonia. The findings of this study suggest that the multilingual website has contributed to increasing awareness of oromandibular dystonia and that the provision of basic telemedicine via the internet can aid the diagnosis and treatment of oromandibular dystonia. Full article

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Open AccessArticle

Measuring Stroke Patients’ Exercise Preferences Using a Discrete Choice Experiment

by Wolfgang Geidl, Katja Knocke, Wilfried Schupp and Klaus Pfeifer

Neurol. Int. 2018, 10(1), 6993; https://doi.org/10.4081/ni.2018.6993 - 30 Mar 2018

Cited by 13 | Viewed by 503

Abstract

Physical activity post stroke improves health, yet physical inactivity is highly prevalent. Tailored exercise programs considering physical activity preferences are a promising approach to promote physical activity. Therefore, this study seeks to measure exercise preferences of stroke survivors. Stroke survivors conducted a discrete [...] Read more.

Physical activity post stroke improves health, yet physical inactivity is highly prevalent. Tailored exercise programs considering physical activity preferences are a promising approach to promote physical activity. Therefore, this study seeks to measure exercise preferences of stroke survivors. Stroke survivors conducted a discrete choice experiment (DCE). DCE was presented in a face-to-face interview where patients had to choose eight times between two different exercise programs. Exercise programs differed by characteristics, with the six attributes under consideration being social situation, location, type of exercise, intensity, frequency, and duration. Utilities of the exercise attributes were estimated with a logit choice model. Stroke survivors (n=103, mean age: 67, SD=13.0; 60% male) show significant differences in the rated utilities of the exercise attributes (P<0.001). Participants had strong preferences for light and moderate intense physical activity and favored shorter exercise sessions. Stroke survivors have remarkable exercise preferences especially for intensity and duration of exercise. Results contribute to the tailoring of physical activity programs after stroke thereby facilitating maintenance of physical activity. Full article

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Neurol. Int., Volume 10, Issue 1 (March 2018) – 11 articles

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