637 e-Letters

  • Factors influencing EVD related infections: unanswered questions

    I read with great interest the paper by Jamjoom et al.[1] The authors have done commendable work in establishing a national external ventricular drain related infection (ERI) rate and elucidating the factors influencing it in the largest prospective multicentre study. However, some questions have been left unanswered in this respect.
    One of the contributing factors to a low ERI rate in the study could have been the fact that majority (98.6%) of EVDs were inserted in the operating theatre. In their single centre retrospective study of 84 patients, Arabi et al[2] found that placement outside operating rooms was associated with a trend towards higher ERIs. Clark et al[3], in their retrospective review of complications of intracranial pressure monitoring in 140 trauma patients, noted that the incidence of major infectious complications (eg. clinical ventriculitis, subdural empyema, brain abscesses) was higher in the groups in which the catheter was placed in the intensive care unit. A randomised control trial would better examine the importance of this finding.
    In this study by Jamjoom et al[1], the authors found no significant difference between infected and uninfected cases with regard to the length of tunnelling. However, recent evidence although weak, points towards a preventive benefit of long tunnel EVDs over short tunnel EVDs.[4,5] Hence, a discussion about the role of tunnelling length in ERIs is felt missing in the paper.
    Korinek et al, in their stud...

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  • Leg stereotypy syndrome

    We read with great interest the research paper published recently by Lotia et al. entitled “Leg stereotypy syndrome: phenomenology and prevalence”. 1 The study brings important new information about an intriguing newly identified condition, previously designated by the same group as leg stereotypy disorder2, defined as repetitive, rhythmical, stereotypic leg movements, particularly noticeable while sitting.1,2 The authors describe the phenomenology and prevalence of leg stereotypy syndrome (LSS) by evaluating a total of 92 individuals, 57 from the general population (control group) and 35 with different movement disorders (Parkinson´s disease, restless legs syndrome, Tourette´s syndrome, and tardive dyskinesia).1 LSS was found in 7% of the control group and 17% of the movement disorders group, concluding that in terms of prevalence, this is a common condition.1 Another interesting finding was that all but one (83 %) of the patients with LSS from the movement disorders group also had a diagnosis of attention deficit hyperactivity disorder (ADHD).1 Lotia and colleagues do not believe in a relationship between ADHD and LSS1 stating in the discussion that “while certain movements or fidgetiness can be observed in individuals with anxiety or ADHD, the presence of typical stereotyped movements has not been previously described with ADHD”.1 Our group is currently studying the frequency of abnormal involuntary movements in patients with ADHD, compared a control group, and our pre...

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  • Dosing regimen of oral prednisolone and prognosis in patients with generalized myasthenia gravis

    Imai et al. examined the association between the dosing regimen of oral prednisolone (PSL) and the achievement of minimal manifestation status or better on PSL <=5 mg/day lasting >6 months in patients with generalized myasthenia gravis (1). The authors classified 590 patients into high-dose, intermediate-dose and low-dose (n=166) groups, and logistic regression analysis was applied to know the prognosis of patients in low-dose group, by splitting observational period into 1 to 3 years of treatment. The authors concluded that a low-dose PSL regimen with early combination of other treatment options was significantly associated with good prognosis. I have two concerns about their study.

    First, the dosing regimen of oral PSL should be considered with caution. Namely, the authors set the maximum dose of oral PSL in each group, and standard treatment schedule was selected after each patient was allocated. Mean daily dose of PSL does not become highest in high-dose group in the study, which happens in the study protocol. In addition, there is a possibility of higher frequency in patients with combination of other treatment options, when patients were registered into low-dose group. As the age of onset was higher and disease duration was shorter in patients with low-dose group, randomized allocation should be strictly conducted in further study.

    Second, the number of events was not enough after 1 year observation, and higher odds ratios with wide ranges of con...

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  • Response

    Ryuji Kaji MD, PhD
    Department of Neurology, Tokushima University

    I appreciate Dr Popkirov’s unique and interesting views and comments related to our paper [1]. This reminds me of the gating of sensory inputs at various levels of the nervous system, and their perception is not a passive but is a very active process. In addition to the fact that it is not possible to tickle oneself, other sensory phenomena associated with abnormal movements deserve mention. Restless legs syndrome is characterized by constant urge to move legs, and is successfully managed by dopamine agonists. Tics are also preceded by sensory symptoms. These could be examples of aberrant sensory inputs coming to the conscious level, which would normally be handled at subconscious pathways. At this moment, the exact role of the cerebellar pathway in these conditions is not clear, but should be investigated in the future aside from dystonia.

    1. Kaji R, Bhatia K, Graybiel AM. Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin? J Neurol Neurosurg Psychiatry. 2017 Oct 31. pii: jnnp-2017-316250. doi: 10.1136/jnnp-2017-316250. [Epub ahead of print]
    No conflict of interest declared

  • Classification of respiratory events in amyotrophic lateral sclerosis: diagnostic and therapeutic challenges

    Dear Editor,
    We read with great interest the article of Boentert et al.1 recently published on this journal. In their paper, the authors describe polysomnographic findings in a large series of non-ventilated patients with amyotrophic lateral sclerosis (ALS). One of the points the authors underscore is that in their patients most respiratory events during sleep were of the obstructive, and not of the central type. This finding is in agreement with what described by Kimura,2 but not by several other authors who found that most respiratory events were of the central type.3 Furthermore, the authors of this paper did not find that obstructive apneas were preferentially associated with bulbar ALS, similarly to David et al.4 but unlike what described by Santos et al.5 However, the tables in the article show that most of the respiratory events were hypopneas, whose type was not specified.
    Criteria for scoring sleep disordered breathing events have changed over time, especially as regards hypopneas. Only recently a general rule for type of hypopnea, central or obstructive has been proposed. Obstructive hypopneas are those where at least one of three criteria is met during the event: presence of snoring, flow limitation demonstrated by a flattening of the nasal pressure derived flow signal, opposing thoracic and abdominal movements. Absence of all these criteria characterizes central hypopneas.6 However, standard criteria for apneas and hypopneas fit well to patients wi...

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  • No laughing matter

    In their recent article on the pathogenesis of dystonia, Kaji and colleagues argue that aberrant cerebellar inputs can induce dystonic movement mediated by the basal ganglia.[1] In this framework, the sensory trick (geste antagoniste) leads to a realignment between predicted and actual sensory information, thus reduces (or overrides) the sensorimotor mismatch forwarded to the basal ganglia, and in turn alleviates dystonic contractions.
    A similar model of sensorimotor mismatch response has been implicated in the physiology of being tickled[2]. Specifically, it has been proposed that the inability to tickle oneself is related to a sensory attenuation mediated by the cerebellum during self-generated tactile sensation[3]. This attenuation is proportional to the precision of the sensory prediction[4]. Whether the same cerebellar processes are responsible for the alleviation of dystonia during a sensory trick would be an interesting question to explore experimentally. At the risk of straining the analogy, one could even describe the postures and movements one produces when being tickled as dystonic-like. Neurologists are reminded of this when interpreting ambiguous plantar responses in very ticklish patients -- a problem that can be avoided by employing the patient's cerebellar sensory attentuation[5].

    1. Kaji R, Bhatia K, Graybiel AM. Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin? J Neurol Neurosurg Psychiatry. 2017 Oct 31. pii: jnnp-20...

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  • “Region of interest” approach in cerebellar meta-analysis

    In our previous meta-analysis of cerebellar atrophy in seven major neurodegenerative conditions (Alzheimer’s disease (AD), amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), Huntington’s disease (HD), Parkinson’s disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP)) we investigated studies that reported grey matter (GM) loss in the cerebellum [1]. Consistent regions of atrophy were found in AD, ALS, FTD, MSA, and PSP but not HD or PD. In their comment on our meta-analysis, Sheng and Pan have argued that our method of selectively investigating studies that found cerebellar atrophy, rather than adopting a whole-brain approach, is “not optimal in a coordinate-based meta-analysis” [2]. They further cite previous whole-brain meta-analyses that did not identify clusters of cerebellar grey matter loss in patients [3-6].
    Here, we argue that our approach was justified given our aim, which was to focus on cases where cerebellar atrophy was found in the respective disease groups in order to determine 1) if such atrophy followed a consistent, robust pattern, and 2) if atrophy patterns were disease-specific or generic, where possible relating them to symptomatology.
    There are several reasons why we chose to focus on the cerebellum rather than adopting a whole-brain approach. First, the cerebellum is hardly a “region of interest” in the classical sense given its marked heterogeneity in terms of function and connectivity as we...

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  • Does Cerebellar Atrophy in Neurodegeneration?

    Does Cerebellar Atrophy in Neurodegeneration?

    Li-Qin Sheng1, Ping-Lei Pan2
    1 Department of Neurology, Traditional Chinese Medicine Hospital of Kunshan, Kunshan, PR China
    2 Department of Neurology, Affiliated Yancheng Hospital, School of Medicine, Southeast University, Yancheng, PR China

    PingLei Pan, Department of Neurology, Affiliated Yancheng Hospital, School of Medicine, Southeast University, West Xindu Road 2#, Yancheng, Jiangsu Province, 224001, PR China. E-mail: panpinglei@163.com, Telephone: +8618361146977

    Coordinate-based meta-analysis is a powerful way for neuroimaging studies to identify the most consistent and replicable differences in brain activity or structure in neurodegenerative disorders. In their JNNP publication, Gellersen et al 1 conducted coordinate-based meta-analyses of 54 voxel-based morphometry (VBM) studies in Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD), behavioral variant frontotemporal dementia (bvFTD), amyotrophic lateral sclerosis (ALS), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). In this study, they solely focused on cerebellar grey matter (GM) atrophy.1 Marked cerebellar atrophy in AD, ALS, bvFTD, PSP and MSA, but not in PD or HD, was identified in the meta-analyses.1
    These findings are of interest.1 However, the procedure of the meta-analyses had a major limitation. Coordin...

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  • What is the evidence for neurological follow-up of patients with FNS?

    We thank dr. Coebergh and colleagues for their interest in our study. We agree that(a) there are many differences between the health care systems of the UK and the Netherlands, (b) the results of our study do not apply to excluded patients, and (c) the management of new neurological symptoms, relapses of previous FNS and relevant neurological and other co-morbidities remain very important in order to prevent inappropriate re-referrals and investigations of patients. However, in the absence of sound evidence from appropriate clinical studies,we disagree with the authors’ conclusion that neurological follow-up of these patients is often beneficial.
    We wish to emphasize that in our study, firstly a neurologist established the diagnosis and briefly explained the diagnosis to the patient. Secondly, the first neurologist referred the patient to a specially trained second neurologist, who scheduled half an hour to discuss the diagnosis with the patient. This approach is clearly different from immediate referral to a GP after the diagnosis.

  • Neurologists have an important role in follow up of patients with functional neurological symptoms.

    It is good to see that trials are being done to answer the critical question of how best to provide care for those patients with functional neurological symptoms (FNS). The research paper, ‘Management of patients with functional neurological symptoms: a single-centre randomised controlled trial’, by Pleizier, de Haan and Vermeulen, randomizes outpatients with functional neurological symptoms after diagnosis, to either two outpatient appointments with a neurologist, or referral back to a GP. Intriguingly, it finds no difference in outcome, that is quality of life scores, between the two groups.[1] While this study attempts to address an important question, namely the role of the neurologist in the care of patients with functional neurological disorders, we feel it has a number of problems that limit its generalizability, particularly to UK neurology practice.
    The Netherlands is a country that compared to the UK, has approximately four times as many neurologists per head of the population, and many more GPs with higher levels of job satisfaction,[2] and often have mental health nurse support in the practice itself. Neurology outpatient waiting times are shorter in the Netherlands, and in-patient neurology review happens routinely and is quicker, unlike in the UK where it may not occur at all.[3] Because of this lack of prompt neurological review in the UK, it is common for patients to receive erroneous diagnoses, often necessitating an “undiagnosis” at the eventual neu...

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