By Elaine Wyllie, MD

It is a privilege to introduce the book “Electroencephalogram Examples and Guides,” published in August 2013. The book provides a concise and comprehensive compilation of text and EEG recordings collected to shorten the learning curve toward competence in EEG interpretation.

This book will be valuable to a wide variety of readers. For specialists preparing for EEG certification examinations, such as those offered by the AsianEpilepsyAcademy – ASEAN Neurological Association (ASEPA-ASNA) or the American Board of Clinical Neurophysiology (ABCN), it could serve as an essential guide. For the practicing neurologist, it can serve as a useful quick reference. For beginners, including EEG technologists and neurology trainees, it can be an effective teaching resource.

Throughout the book, complex concepts are simplified without the sacrifice of fine details. Facts and comparisons are given in point forms and tables. Classifications of various EEG findings are presented in easy-to-understand algorithms. EEGs from common to rare conditions are presented in a stimulating quiz format. The index enables quick reference to EEG tracings from patients with different diagnoses.

Sections 1 and 2 deal with the basics of EEG, including indications, limitations and neurophysiologic principles. Section 3 provides actual EEG recordings, starting with normal findings, artifacts and benign variants and progressing through epileptiform and non-epileptiform abnormalities. Section 4 includes a systematic step-by-step approach to EEG interpretation, and Section 5 offers a quiz for readers to practice their EEG interpretation skills. Section 6 deals with EEG evaluation of syncope and blackout spells, while Section 7 deals with evaluation of coma and altered states of consciousness. Finally,  Section 8 provides an opportunity for self-assessment of the reader’s discernment of the various points in the book.

This innovative book is recommended for anyone who seeks to learn EEG in an effective and systematic way.  That the author also made the process pleasurable is a welcome benefit indeed.

By John D. England, MD

John D. England

The Journal of the Neurological Sciences (JNS) and its publisher, Elsevier, are working together to improve the journal and to make the submission process easier for authors.

A major complaint from authors concerns the necessity to format manuscripts to fit the idiosyncratic requirements of journals.  Since many high volume journals like JNS have a high rejection rate, authors frequently must reformat their manuscripts for submission to a different journal. This is not only a hassle, but a time-consuming process for authors.

In order to simplify the submission process for authors, JNS has eliminated strict requirements for reference formatting. As of now, we will accept manuscripts with no strict requirements for reference formatting. Any style of reference formatting will be accepted as long as the style is consistent.

If the manuscript is accepted for publication, then Elsevier will change the reference formatting to fit the style for JNS. As an extension of this process, in the near future JNS and Elsevier will begin accepting entire manuscripts without strict formatting or referencing requirements.

Elsevier already has introduced this feature, which is named “Your Paper Your Way (YPYW),” for several other journals in its portfolio. It has been so well received by authors that we will shortly begin offering this service to authors of manuscripts submitted to JNS.   Taken together, these new options for manuscript formatting flexibility should make it easier for authors to submit manuscripts to JNS.

As noted in the previous issue of World Neurology, we have begun an “Editor’s Selection” of articles from JNS.  Elsevier has agreed to allow free access to selected articles for members of the World Federation of Neurology.  The process for viewing these articles is now streamlined: Click on one of the two featured articles on the World Neurology Online page, or use the “See all free JNS articles” link below the featured articles. It will take you directly to the free article page on the JNS website.  In this issue, we share these two recent articles:

The Clinical and Pathological Phenotypes of Frontotemporal Dementia with C9ORF72 Mutations. Ying Liu and others from China have written a review on the phenotypes of frontotemporal dementia associated with C9ORF72 mutations.  The expanded hexanucleotide repeat (GGGGCC) in the chromosome 9 open reading frame 72 (C9ORF72) is now recognized as one of the major causes of hereditary frontotemporal dementia (FTD). It is also the most frequent genetic cause of the ALS/FTD complex.  However, the clinical and pathological phenotypes associated with C9ORF72 mutations appear increasingly diverse, and the mechanisms of disease are not known.  See Liu Y, et al.

Journal of the Neurological Sciences 335 (2013) 26-35.  An Association Between Benzodiazepine Use and Occurrence of Benign Brain Tumors. Tomor Harnod and others analyzed data from the National Health Insurance System of Taiwan to ascertain whether there is an association between long-term benzodiazepine use and the development of brain tumors. They identified 62,186 patients who had been prescribed benzodiazepines for at least two months between Jan. 1, 2000, and Dec. 31, 2009. These patients were compared with a matched non-benzodiazepine cohort of 62,050 patients. The incidence rate for benign brain tumors was 3.33 times higher in the benzodiazepine cohort compared to the non-benzodiazepine cohort with an adjusted hazard ratio (HR) of 3.15 (95% CI = 2.37-4.20).  Additionally, the adjusted HRs for benign brain tumors increased with benzodiazepine dose. Thus, in this cohort study, the authors found a significant association of benign brain tumors with long-term benzodiazepine use. There are many possible explanations for such an association, and the authors correctly avoid the conclusion of causation. But, benzodiazepines are a commonly prescribed medication, and further studies of this important topic are warranted. See Harnod T, et al.

Journal of the Neurological Sciences 336 (2014) 8-12.

By Raymond Voltz

Raymond Voltz

Two recent international publications have put the spotlight on the situation of worldwide provision of palliative care. These are the “World Cancer Report,” published by the International Association of Research in Cancer (IARC) and the “Global Atlas of Palliative Care at the End of Life,” published by the WHO in collaboration with the World Wide Palliative Care Alliance. The “World Cancer Report” reminds us that the war on cancer has not been won and that still much remains to be done when treatment fails.

“The Global Atlas of Palliative Care at the End of Life” highlights the need for more palliative care: It estimates that 19.2 million people worldwide require palliative care with about 38 percent of patients dying from cardiovascular diseases, 34 percent of cancer and 10 percent of COPD. In the remainder, specific diseases such as multiple sclerosis (MS) and Parkinson’s are mentioned, and although less important on the global scale, patients with MMD/ALS. The atlas highlights the worldwide skewed distribution of palliative care services and access to palliative care depending on income and geographical regions worldwide. They conclude that palliative care is a human right, and that therefore each country should take improvement of palliative care up as a national strategy.

In some countries, it starts at the beginning: the access to opioids. A few weeks ago, a well-known Russian military committed suicide, stating in his final letter, that he did so because of an unbearable pain due to cancer and that he was not able to get opioids. Some countries are proud of their drug protection programs, which also means that they protect cancer patients from getting the right drugs at the right time. Here, a sensible political balance has to be reached. Opioids, of course, are important for the treatment of pain as well as the treatment of dyspnea. Provision of multiprofessional high-skilled palliative care follows those basic tenets.

So what does this mean for neurology? Cancer patients, cardiovascular patients and specific neurological diseases are treated within the responsibility of neurologists worldwide. As the atlas also states, their estimates only refer to patients at the end of their lives. The concept of early integration of palliative care has now fully reached the oncological world with the ASCO stating that all cancer patients should have access to palliative care. So, the need for palliative care is even greater than stated in the atlas, which the authors fully acknowledge. So where is the relevant interest in neurology? Do all neurologists worldwide know what palliative care structures already exist in their neighborhoods? Do they refer refer patients there early enough for common management of patients and families? Where is the topic of palliative care in the training of neurologists worldwide? Where are the neurologists who help establishing palliative care structures in their region? Where are the ones who advocate for a national palliative care strategy including all relevant patient groups? And finally, where is the interest of moving the field forward by valid research? Still, many neurologists think this in an unscientific topic not relevant to them, and that is a pity — mainly for many patients worldwide suffering not only from devastating neurological diseases but also from clear palliative care mismanagement by their neurologists.

Nir Giladi

By Mark Hallett, MD

Management of patients with Parkinson’s disease has progressed well. Levodopa and the dopamine agonists are effective, and when complications arise such as dyskinesias and fluctuations, deep brain stimulation (DBS) can be effective. However, another significant problem is now emerging. That is gait difficulties later in the course of Parkinson’s disease that are not responsive to either the dopaminergic therapy or DBS. Patients can look pretty good in many ways, but will have trouble relatively isolated to balance and gait.

One interesting feature of the gait difficulty is freezing of gait (FOG). With FOG, there is a failure to move forward despite intent to do so.  FOG can occur at gait onset or in the middle of walking, particularly in some settings such as walking through a doorway or turning. At times, there can be off-freezing that is dopa responsive; the problematic situation is on-freezing. FOG also occurs in other Parkinsonian conditions such as progressive supranuclear palsy.

There have been two prior workshops devoted to the topic, but recently there was an international conference Feb. 5-7, organized by Professors Nir Giladi and Jeff Hausdorff at the Dead Sea. The conference was sponsored by TelAvivUniversity, the TelAvivMedicalCenter and the International Parkinson and Movement Disorder Society. More than 160 attendees came from 20 countries.

Jeff Hausdorff

Freezing also can occur with upper extremity movements or speech, but gait is more commonly affected and more debilitating. The topic is now under intense scrutiny. Gait is a complex movement requiring integrated activity of all parts of the brain and spinal cord with both balancing and stepping. This is likely why gait is more commonly affected than other movements.

There are a number of theories as to the etiology, and, as freezing is likely to be multifactorial, many of these may well be relevant in different patients. One type of problem is the deterioration of motor control capabilities, such as loss of the internal drive for movement, difficulties with multitasking and difficulties in carrying out automatic movements. Lack of gait symmetry is often correlated with freezing.

Another interesting motor control problem in Parkinson’s disease is the sequence effect, the progressive decline of movement amplitude in a sequence of what should be similar movements. Step length often gradually declines prior to a freeze, so this seems important at least in some circumstances. Cognitive problems, importantly loss of executive function, appear relevant. There is clearly a role of environment factors, including the path that needs to be traversed; the narrow doorway, for example.

There was considerable discussion of therapy. In the end, given the multiplicity of causes, therapy may have to be individualized.  Certainly, there should be care to optimize dopaminergic therapy. Physical therapy can play a role. Much discussion focused on the value of DBS of the pedunculopontine nucleus (PPN). The PPN sits in a complex region at the junction of the midbrain and the pons and appears to be a part of, or at least close to, the mesencephalic locomotor region. The literature is really still sparse, and it is not clear that the DBS electrodes are actually in the PPN in all patients, but some patients do appear improved.

Gait freezing remains an important clinical phenomenon, a fascinating set of problems for physiology and a challenge for treatment. Giladi and Hausdorff have been leaders in all aspects, and the attendees were grateful to them for organizing a useful conference that will set the direction of research in the next few years.