Being a sufferer of Benign Fasciculation Syndrome (BFS) for more than a year, I read with great interest Professor Kiernan’s editorial (1), as well as Dr. Vercueil’s personal account of his encounter with the syndrome (2). I was relieved to see how much the “clinical course” of my case corresponded to that described by Professor Kiernan and Dr. Vercueil. They surfaced during a period of stress and sleep-deprivation, and has since kept going.
As Professor Kiernan writes, anything that increases central excitability can trigger fasciculations. This includes the fear that they form part of a prodrome to motor neuron disease (MND); a defining characteristic of Fasciculation Anxiety Syndrome in Clinicians (FASICS). Even though my diagnosis of BFS have been “confirmed” by an EMG and a neurologist, I still suffer from occasional episodes of FASICS exacerbations, surfacing like a recurrent nightmare according to its own inner logic. In other words, FASICS undoubtedly shares a quality with health anxiety, namely a jumbled relationship between symptoms and belief. The opening lines of H.P Lovecraft’s “The dreams in the witch house” captures this poetically; “Whether the dreams brought on the fever, or the fever brought on the dreams, Walter Gilman did not know”.
After years of working in hospitals you will have encountered some rare cases where the etiology of a disease have followed a non-standard pathway. These can unconsciously skew your wo...
Being a sufferer of Benign Fasciculation Syndrome (BFS) for more than a year, I read with great interest Professor Kiernan’s editorial (1), as well as Dr. Vercueil’s personal account of his encounter with the syndrome (2). I was relieved to see how much the “clinical course” of my case corresponded to that described by Professor Kiernan and Dr. Vercueil. They surfaced during a period of stress and sleep-deprivation, and has since kept going.
As Professor Kiernan writes, anything that increases central excitability can trigger fasciculations. This includes the fear that they form part of a prodrome to motor neuron disease (MND); a defining characteristic of Fasciculation Anxiety Syndrome in Clinicians (FASICS). Even though my diagnosis of BFS have been “confirmed” by an EMG and a neurologist, I still suffer from occasional episodes of FASICS exacerbations, surfacing like a recurrent nightmare according to its own inner logic. In other words, FASICS undoubtedly shares a quality with health anxiety, namely a jumbled relationship between symptoms and belief. The opening lines of H.P Lovecraft’s “The dreams in the witch house” captures this poetically; “Whether the dreams brought on the fever, or the fever brought on the dreams, Walter Gilman did not know”.
After years of working in hospitals you will have encountered some rare cases where the etiology of a disease have followed a non-standard pathway. These can unconsciously skew your worldview, making you focus on the “non-Gaussian” cases and off-the scale reports. When I first noticed my twitching, I frantically started researching Pubmed and discovered to my horror that there indeed were scattered reports of patients with apparently benign fasciculations progressing to MND (3). Having no background in neurology, I was unable to discern the details and, indeed, how rare these cases actually were. Nevertheless, they became the fuel of my bouts of FASICS. I gazed too long into the abyss and the abyss gazed back into me. I became the case reports.
The protagonist of Lovecraft’s story, Walter Gilman, is a student of non-euclidean geometry. He soon discovers that his rented room is constructed according to outlandish geometrical principles. Would he have noticed this without being knowledgeable in mathematics? His preoccupation with these geometrical patterns propels him into a strange and nightmarish dimension that eventually consumes him. Our background in medicine can sometimes lure us into such parallel dimensions when we misinterpret symptoms in ourselves.
Unlike Walter Gilman, I was not lost in a parallel world. My bouts of FASICS are now rare and short-lived, but it has been a long journey. Enduring such a journey can bring a reward. Like a booty brought back from a nightmare, my FASICS have also become a powerful reminder for me that it is all too easy to fall into the trap of Foucault’s “clinical gaze”, were the patient becomes a process, rather than an individual.
As a clinical pharmacist I often meet patients that are overly worried about adverse effects of drugs. Their anxieties are often caused by a uniformed reading of the package leaflet and internet research. The existence of rare, but dramatic side effects creates an illusory scientific foundation for their fears. When counselling these patients I try to make use of statistics, but now I know that such anxiety transcends the world of figures and a wider cognitive approach should be sought. Exiting a witch house built upon a strange “geometry” of “singularities” can be quite time-consuming.
Studies on long-term outcomes are invaluable in mapping the “Gaussian space” of BFS and FASICS (4, 5), but dealing with a non-Gaussian dimension requires the human touch described by Professor Kiernan, as well as reassuring personal testimonials like Dr. Vercueil’s. This is something other sufferers of FASICS can attest to, for example Dr. Mert Erogul in his excellent Guardian long-read “The perils of being your own doctor”. What finally made him realize he was not on the path to MND was the fact that his own neurologist also suffered from benign fasciculations (6). Being lost in a witch house of case reports, you need the “anti-case reports” to show you the way out.
References:
1. Kiernan MC. Fasciculation anxiety syndrome in clinicians: FASICS. Pract Neurol. 2020;20(6):433-4.
2. Vercueil L. FASICS: fasciculation anxiety syndrome in clinicians. Pract Neurol. 2020;20(6):514-5.
3. Singh V, Gibson J, McLean B, Boggild M, Silver N, White R. Fasciculations and cramps: how benign? Report of four cases progressing to ALS. J Neurol. 2011;258(4):573-8.
4. Blexrud MD, Windebank AJ, Daube JR. Long-term follow-up of 121 patients with benign fasciculations. Ann Neurol. 1993;34(4):622-5.
5. Simon NG, Kiernan MC. Fasciculation anxiety syndrome in clinicians. J Neurol. 2013;260(7):1743-7.
6. Erogul M. The perils of being your own doctor: The Guardian; 2016 [Available from: https://www.theguardian.com/news/2016/aug/04/perils-being-your-own-docto....
We read with great interest the recent article by Drs Page & Gaillard describing neuroradiology signs and their variable utility [1].
We recently attempted to bring science to the art of descriptive radiological signs by conducting a randomized controlled trial to assess the utility of these signs for medical student radiology education [2]. We found that ‘Metaphoric Signs’ (linking radiology resemblance to an object or concept which is not actually present in an image) increased descriptive and diagnostic ability as well as students’ lesson enjoyment, when compared to teaching with normal anatomy comparators.
The importance of diagnostic accuracy of commonly used signs was similarly emphasised in our discussion. In addition to being diagnostically accurate, the success of a metaphoric sign also relies on how well it resonates with each individual learner, based on their perception of the real-world similarity alluded to in the radiology image and their prior experiences[3,4]. This is aptly exemplified by Author FG’s stronger association of the pontine appearance in osmotic demyelination with a blockbuster sci-fi trilogy than classical mythology.
An interesting observation in our study was that several students used incorrect wording for the name of the signs yet identified the correct diagnosis. Students who correctly diagnosed agenesis of the corpus callosum from a coronal MRI image containing the ‘Moose head sign’ [5] varia...
We read with great interest the recent article by Drs Page & Gaillard describing neuroradiology signs and their variable utility [1].
We recently attempted to bring science to the art of descriptive radiological signs by conducting a randomized controlled trial to assess the utility of these signs for medical student radiology education [2]. We found that ‘Metaphoric Signs’ (linking radiology resemblance to an object or concept which is not actually present in an image) increased descriptive and diagnostic ability as well as students’ lesson enjoyment, when compared to teaching with normal anatomy comparators.
The importance of diagnostic accuracy of commonly used signs was similarly emphasised in our discussion. In addition to being diagnostically accurate, the success of a metaphoric sign also relies on how well it resonates with each individual learner, based on their perception of the real-world similarity alluded to in the radiology image and their prior experiences[3,4]. This is aptly exemplified by Author FG’s stronger association of the pontine appearance in osmotic demyelination with a blockbuster sci-fi trilogy than classical mythology.
An interesting observation in our study was that several students used incorrect wording for the name of the signs yet identified the correct diagnosis. Students who correctly diagnosed agenesis of the corpus callosum from a coronal MRI image containing the ‘Moose head sign’ [5] variably described a ‘Mule head’, ‘deer head’ and ‘antler head’ appearance. This may be explained by the wording of a sign having lesser importance for diagnosis than the strength of the metaphoric association identified by the learner between the radiological appearance and the real-world metaphoric resemblance, or ‘Pareidolia’.
Following on from this, we would suggest that the hoofed ruminant animal in Figure 2d of the paper by Page & Gaillard is actually stag deer (Subfamily: Cervinae, Genus: Cervus) rather than a Moose (Subfamily: Capreolinae, Genus: Alces). However, in our opinion, the coronal MRI provided (Figure 2c) appearance of agenesis of the corpus callosum truly does resemble a male deer (Stag) rather than a Moose! Therefore, the image provided may result in the strongest metaphoric association with the correct diagnosis in readers.
1. Page I, Gaillard F. Descriptive neuroradiology: beyond the hummingbird. Pract Neurol Published Online First: 27 August 2020. doi:10.1136/practneurol-2020-002526
2. Gibney B, Ghadir KH, Redmond CE et al. Pareidolia in Radiology Education: A Randomized Controlled Trial of Metaphoric Signs in Medical Student Teaching. Acad Radiol. 2020 Sep 17:S1076-6332(20)30498-0. doi: 10.1016/j.acra.2020.08.017.
3. Baker SR, Partyka L. Relative Importance of Metaphor in Radiology versus Other Medical Specialties. RadioGraphics. 2012;32:235–40. doi:10.1148/rg.321115721
4. Lakoff G. The contemporary theory of metaphor. Metaphor and Thought. 1993;:202–51. doi:10.1017/cbo9781139173865.013
5. Cherian EV, Shenoy KV, Bukelo MJ, et al. Racing car brings tear drops in the moose. BMJ Case Rep 2013;2013. doi:10.1136/bcr-2012-008165
We greatly enjoyed the excellent recent review on the diagnosis and management of primary lateral sclerosis (PLS). Turner and Talbot emphasize the importance of controlling spasticity as the most troublesome symptom, which is often associated with pain, discomfort and physical disability [1].
As the authors point out, the current standard of care (baclofen, tizanidine, benzodiazepines and botulinum toxin) is frequently complicated by side effects, difficulties in titration and a lack of well controlled clinical studies [2]. We were thus surprised that the authors omitted the option of medicinal cannabis from the discussion of this very important topic.
A study by Riva and colleagues offers clear evidence on the efficacy of the cannabinoid preparation Nabiximol for the control of spasticity in ALS and PLS patients [3], which is further supported by Meyer and colleagues providing compelling real world evidence of this effect [4].
The THC:CBD oromucosal spray (nabioximols, brand name Sativex) became approved in European Union countries in 2010. Although only approved for people with moderate to severe spasticity in multiple sclerosis, who have not responded adequately to other anti-spasticity regimes, THC:CBD is increasingly being used off-label for spasticity in patients with motor neuron diseases. In addition, the patients themselves have self-medicated with recreational cannabis for many years [5].
The multicenter, double-blind,...
We greatly enjoyed the excellent recent review on the diagnosis and management of primary lateral sclerosis (PLS). Turner and Talbot emphasize the importance of controlling spasticity as the most troublesome symptom, which is often associated with pain, discomfort and physical disability [1].
As the authors point out, the current standard of care (baclofen, tizanidine, benzodiazepines and botulinum toxin) is frequently complicated by side effects, difficulties in titration and a lack of well controlled clinical studies [2]. We were thus surprised that the authors omitted the option of medicinal cannabis from the discussion of this very important topic.
A study by Riva and colleagues offers clear evidence on the efficacy of the cannabinoid preparation Nabiximol for the control of spasticity in ALS and PLS patients [3], which is further supported by Meyer and colleagues providing compelling real world evidence of this effect [4].
The THC:CBD oromucosal spray (nabioximols, brand name Sativex) became approved in European Union countries in 2010. Although only approved for people with moderate to severe spasticity in multiple sclerosis, who have not responded adequately to other anti-spasticity regimes, THC:CBD is increasingly being used off-label for spasticity in patients with motor neuron diseases. In addition, the patients themselves have self-medicated with recreational cannabis for many years [5].
The multicenter, double-blind, randomized, placebo-controlled, phase 2 CANALS trial, Riva et al. showed that nabiximols was not only safe and well tolerated in patients with ALS and PLS, but had a significant positive effect on spasticity reflected by significant improvements in scores on the Modified Ashworth Scale, and had an additional beneficial effect on spasticity-related pain [3]. Additionally, in a subsequent retrospective mono-centric real-world cohort of ALS patients, Meyer et al. showed recently that the nabiximols treatment satisfaction was high and dosing was titrated individually. Especially, patients with moderate to severe spasticity self-reported a strong recommendation rate in comparison to patients with mild spasticity [4]. Thus the available evidence on treatment of spasticity in MND with cannabis is superior to the classical options.
Both studies have influenced the clinical practice in our Motor Neuron Disease unit and to date our experience has borne out the expectations raised by the studies.
1 Turner MR, Talbot K. Primary lateral sclerosis: diagnosis and management. Pract Neurol 2020;:practneurol-2019-002300. doi:10.1136/practneurol-2019-002300
2 Ashworth NL, Satkunam LE, Deforge D. Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews Published Online First: 15 February 2012. doi:10.1002/14651858.CD004156.pub4
3 Riva N, Mora G, Sorarù G, et al. Safety and efficacy of nabiximols on spasticity symptoms in patients with motor neuron disease (CANALS): a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial. The Lancet Neurology 2019;18:155–64. doi:10.1016/S1474-4422(18)30406-X
4 Meyer T, Funke A, Münch C, et al. Real world experience of patients with amyotrophic lateral sclerosis (ALS) in the treatment of spasticity using tetrahydrocannabinol:cannabidiol (THC:CBD). BMC Neurol 2019;19:222. doi:10.1186/s12883-019-1443-y
5 Amtmann D, Weydt P, Johnson KL, et al. Survey of cannabis use in patients with amyotrophic lateral sclerosis. Am J Hosp Palliat Care 2004;21:95–104. doi:10.1177/104990910402100206
Competing interests: none
Acknowledgements: not applicable
Contributorship: SCG and PW conceived and drafted the letter, MTH edited
Funding info: not applicable
Ethical approval information: not applicable
Data sharing statement: not applicable
We thank Dr Weydt and colleagues for their interest in our review. We are aware of the subjective benefit of cannabinoids reported by some living with multiple sclerosis, and a trial of THC:CDB spray is also approved by the UK’s National Institute for Health & Care Excellence (NICE). The CANALS study mentioned, in which PLS patients were well represented (28% of treatment arm and 20% of placebo arm), was a Phase 2 study powered to consider safety and tolerability. Adverse events were reported in 76% of the treatment group versus 27% in the placebo group. A reduction in the clinician’s Modified Ashworth Spasticity scale score was noted, and in the patient’s numeric rating scale score for pain, but not their scores for spasm or spasticity. In the discussion, the authors note that muscle cramping was not alleviated in a prior randomised trial of THC in ALS (1). We agree with their call for further evaluation, through a Phase 3 study of the benefit of cannabinoids over the licensed therapies for spasticity outlined in our review.
Reference:
1. Weber M, Goldman B, Truniger S. Tetrahydrocannabinol (THC) for cramps in amyotrophic lateral sclerosis: a randomised, double-blind crossover trial. J Neurol Neurosurg Psychiatry. 2010 Oct;81(10):1135-40. doi: 10.1136/jnnp.2009.200642. PMID: 20498181.
It gives more discomfort than pleasure to comment on DaT scanning again but your editorial [1] prompted me to find out that the number of DaTscans carried out in England is increasing yearly, from 4550 in 2012/3 to 8840 in 2018/9. A trip to the dentist might have been wiser, my long-expressed opinion (based on the fundamental principles that let down 18FDopa PET) being that the DaTscan is a waste of time, radiation and money [2]. In brief it is a low resolution, inadequately sensitive, inadequately reproducible test with too many false negatives and little knowledge of confounding influences. The acronym SWEDD has, mercifully, been consigned to the dustbin[3] but we don’t have the neuropathological studies or large and long-term blinded follow-up studies, in patients and healthy individuals, despite the many tests and years since its commercial introduction, that would tell the true false positive and negative rate of the test. We can’t then confidently say how strongly a normal result argues against a clinical diagnosis of PD. Neurology is not the only specialty that uses DaTscan and indication-creep (good for the shareholder, bad for the taxpayer) means that it is also being used to distinguish Lewy Body Dementia from Alzheimer’s Dementia despite limited supportive data (4). It must also be remembered that the test measures biochemistry, not pathology; the statement in the case presentation [5] “a DaTscan was normal, with no evidence of degenerative Parkinsonism” is...
It gives more discomfort than pleasure to comment on DaT scanning again but your editorial [1] prompted me to find out that the number of DaTscans carried out in England is increasing yearly, from 4550 in 2012/3 to 8840 in 2018/9. A trip to the dentist might have been wiser, my long-expressed opinion (based on the fundamental principles that let down 18FDopa PET) being that the DaTscan is a waste of time, radiation and money [2]. In brief it is a low resolution, inadequately sensitive, inadequately reproducible test with too many false negatives and little knowledge of confounding influences. The acronym SWEDD has, mercifully, been consigned to the dustbin[3] but we don’t have the neuropathological studies or large and long-term blinded follow-up studies, in patients and healthy individuals, despite the many tests and years since its commercial introduction, that would tell the true false positive and negative rate of the test. We can’t then confidently say how strongly a normal result argues against a clinical diagnosis of PD. Neurology is not the only specialty that uses DaTscan and indication-creep (good for the shareholder, bad for the taxpayer) means that it is also being used to distinguish Lewy Body Dementia from Alzheimer’s Dementia despite limited supportive data (4). It must also be remembered that the test measures biochemistry, not pathology; the statement in the case presentation [5] “a DaTscan was normal, with no evidence of degenerative Parkinsonism” is a common but incorrect oversimplification. For drug-induced parkinsonism, is it my own oversimplification to ask why a drug that is biochemically producing parkinsonism doesn’t change the result of a biochemical test that is used to detect parkinsonism? Small studies with an unreliable test and heavy sales pitch encourage more indication-creep.
A practical neurologist’s approach is to ask if the outcome of a test will change what one does or advises and if a test can’t be trusted it shouldn’t be requested. I wonder if the £12M or more spent on the test in England in 2018 might have been more usefully used and whether the Scottish, known for their financial prudence, have a neologism for colleagues that request DaTscans?
References:
1. Araujo R, van Rumund A, Bloem BR. To scan or not to scan your Parkinson patient: that is the question! Pract Neurol 2019;19:462-4. doi: 10.1136/practneurol-2019-002225
2. Morrish PK. Controversies in Neuroimaging. in Factor SA and Weiner WJ eds. Parkinson’s disease; Diagnosis and Clinical Management. Demos Publishing (New York). 2008: 317-326
3. Erro R, Schneider SA, Stamelou M, et al. What do patients with scans without evidence of dopaminergic deficit (SWEDD) have? New evidence and continuing controversies. J Neurol Neurosurg Psychiatry 2016; 87: 319-323. doi: 10.1136/jnnp-2014-310256
4. McCleery J, Morgan S, Bradley KM et al. Dopamine transporter imaging for the diagnosis of dementia with Lewy bodies. Cochrane Database of Systematic Reviews 2015, Issue 1. Art. No.: CD010633. doi: 10.1002/14651858.CD010633.pub2
5. Wiblin I, Mitra D, Naomi W. An unusual cause of treatable parkinsonism. Pract Neurol 2019; 19; 518-20. doi: 10.1136/practneurol-2019-002339
Acknowledgement: Figures for 2018 DaTscan use kindly provided by Sheila M Dixon, Senior Analytical Manager, Performance Analysis Team, NHS England & NHS Improvement
With great interest, we read the review by Markus on personalising the secondary prevention approach to patients with stroke ¹, published in the most recent issue of Practical Neurology. Where we are presented with clinically useful and evidence-based advice for the etiological assessment of patients with acute ischemic stroke (AIS), focusing on lacunar stroke syndromes of a non-lacunar cause, and its appropriate therapeutic management. We consider the article of great importance: a must-read for all physicians who care for patients with AIS since etiological assessment is paramount to dictate the appropriate secondary prevention measures.
The author proposes using the TOAST classification (Trial of Organon 10172 in Acute Stroke Treatment), arguing that classification systems that prime clinical syndromes over pathophysiological mechanisms are less useful. Nevertheless, the author omitted one classification which–partially–resolves the issue: the ASCOD (Atherosclerosis, Small-vessel, Cardiac embolism, Other, Dissection) system ². A comprehensive classification, which allows for more than one aetiology, while giving a degree of a causal relationship to the presence of each category of disease (1 potential, 2 uncertain, 3 unlikely, 0 disease not detected) including incomplete assessment (9 insufficient work-up), while considering some clinical features.
The ASCOD approach permits the identification of patients with diseases that would have been left as indeterm...
With great interest, we read the review by Markus on personalising the secondary prevention approach to patients with stroke ¹, published in the most recent issue of Practical Neurology. Where we are presented with clinically useful and evidence-based advice for the etiological assessment of patients with acute ischemic stroke (AIS), focusing on lacunar stroke syndromes of a non-lacunar cause, and its appropriate therapeutic management. We consider the article of great importance: a must-read for all physicians who care for patients with AIS since etiological assessment is paramount to dictate the appropriate secondary prevention measures.
The author proposes using the TOAST classification (Trial of Organon 10172 in Acute Stroke Treatment), arguing that classification systems that prime clinical syndromes over pathophysiological mechanisms are less useful. Nevertheless, the author omitted one classification which–partially–resolves the issue: the ASCOD (Atherosclerosis, Small-vessel, Cardiac embolism, Other, Dissection) system ². A comprehensive classification, which allows for more than one aetiology, while giving a degree of a causal relationship to the presence of each category of disease (1 potential, 2 uncertain, 3 unlikely, 0 disease not detected) including incomplete assessment (9 insufficient work-up), while considering some clinical features.
The ASCOD approach permits the identification of patients with diseases that would have been left as indeterminate with other classification systems, thus increasing the risk of stroke because of inadequate secondary prevention. Comparisons with other classification systems have shown that ASCO (the predecessor of ASCOD) allows for more than one cause of stroke, with a larger proportion of atherosclerosis and lower of indeterminate and small vessel disease with the potential to magnify cardiac embolism thus leading to overtreatment ³, but with a recurrence risk strongly tied to aetiology regardless of the classification system used ⁴.
However, both of the above mentioned-comparisons have a flaw: they used ASCO as a dichotomic variable, which is not, and is precisely the advantage of ASCOD, that allows individualizing secondary prevention approaches for the most likely aetiology without discarding other possible etiologies, thus decreasing the proportion of indeterminate strokes facilitating inclusion for clinical trials ⁵. The relevance of considering multiple etiologies this has been demonstrated by studies like AMISTAD in which concurrent intra- and extracranial atherosclerosis increased the risk of major adverse cardiovascular events ⁶, and the secondary analysis of SOCRATES, which demonstrated ticagrelor to be superior to aspirin in patients with atherosclerosis regardless of causal link ⁷.
Bottom line, the critical issue is to assess patients with AIS to detect the most likely aetiology responsible for the stroke, without losing sight of possible comorbidities and regardless of the classification system used. In our Hospital, we use ASCOD since it allows neurology residents to consider all potential etiologies, but each centre should individualize to its specific needs.
References
1. Markus H. Personalising secondary prevention: different treatments for different strokes. Practical Neurology Published Online First: 04 September 2019 doi: 10.1136/practneurol-2018-002006
2. Amarenco P, Bogousslavsky J, Caplan LR, et al. The ASCOD phenotyping of ischemic stroke (Updated ASCO Phenotyping). Cerebrovasc Dis 2013;36(1):1-5. doi: 10.1159/000352050
3. Shang W, Liu J. Stroke subtype classification: a comparative study of ASCO and modified TOAST. J Neurol Sci 2012;314(1-2):66-70. doi: 10.1016/j.jns.2011.10.029
4. Arsava EM, Helenius J, Avery R, et al. Assessment of the Predictive Validity of Etiologic Stroke Classification. JAMA Neurol 2017;74(4):419-26. doi: 10.1001/jamaneurol.2016.5815
5. Sirimarco G, Lavallee PC, Labreuche J, et al. Overlap of diseases underlying ischemic stroke: the ASCOD phenotyping. Stroke 2013;44(9):2427-33. doi: 10.1161/STROKEAHA.113.001363
6. Hoshino T, Sissani L, Labreuche J, et al. Prevalence of Systemic Atherosclerosis Burdens and Overlapping Stroke Etiologies and Their Associations With Long-term Vascular Prognosis in Stroke With Intracranial Atherosclerotic Disease. JAMA Neurol 2018;75(2):203-11. doi: 10.1001/jamaneurol.2017.3960
7. Amarenco P, Albers GW, Denison H, et al. Efficacy and safety of ticagrelor versus aspirin in acute stroke or transient ischaemic attack of atherosclerotic origin: a subgroup analysis of SOCRATES, a randomised, double-blind, controlled trial. Lancet Neurol 2017;16(4):301-10. doi: 10.1016/S1474-4422(17)30038-8
Following the publication of the “UK consensus on pregnancy in multiple sclerosis: ABN guidelines” in January 2019, new data has become available and an update is required. Whilst these updates do not change the overall recommendations in the guidelines, they add information, which we feel all neurologists should be aware of in order to provide the highest quality of information to all women with MS considering pregnancy.
(1) Interferon beta preparations in pregnancy
In September 2019, the EMA Committee for Medicinal Products for Human Use (CHMP) recommended a label change for interferon beta-1a, peginterferon beta-1a and interferon beta-1b, i.e. Avonex, Betaferon, Extavia, Plegridy and Rebif, stating that they may be considered during pregnancy if clinically indicated, and can be used during breastfeeding [1]. This decision was based on data from interferon beta registries, national registries and post-marketing experience. However, data from exposure during second and third trimesters remains limited. The duration of exposure during the first trimester is uncertain, because data were collected when interferon beta use was contraindicated during pregnancy, and it is likely that treatment was interrupted in many women when the pregnancy was detected and/or confirmed.
This supports the recommendation in the “UK consensus on pregnancy in multiple sclerosis: ABN guidelines” that these products are safe to be continued at least until...
Following the publication of the “UK consensus on pregnancy in multiple sclerosis: ABN guidelines” in January 2019, new data has become available and an update is required. Whilst these updates do not change the overall recommendations in the guidelines, they add information, which we feel all neurologists should be aware of in order to provide the highest quality of information to all women with MS considering pregnancy.
(1) Interferon beta preparations in pregnancy
In September 2019, the EMA Committee for Medicinal Products for Human Use (CHMP) recommended a label change for interferon beta-1a, peginterferon beta-1a and interferon beta-1b, i.e. Avonex, Betaferon, Extavia, Plegridy and Rebif, stating that they may be considered during pregnancy if clinically indicated, and can be used during breastfeeding [1]. This decision was based on data from interferon beta registries, national registries and post-marketing experience. However, data from exposure during second and third trimesters remains limited. The duration of exposure during the first trimester is uncertain, because data were collected when interferon beta use was contraindicated during pregnancy, and it is likely that treatment was interrupted in many women when the pregnancy was detected and/or confirmed.
This supports the recommendation in the “UK consensus on pregnancy in multiple sclerosis: ABN guidelines” that these products are safe to be continued at least until conception, in addition to extending this advice such that women should be offered the choice of continuation during pregnancy.
(2) Fingolimod (Gilenya)
In September 2019, the MHRA issued an alert on the safety of fingolimod (Gilenya) in pregnancy [2] after data from international pregnancy registers demonstrated that exposure in pregnancy leads to an estimated additional two to three major congenital malformations per 100 livebirths compared with the general population (a two-fold increase). Reported malformations include congenital heart disease, such as tetralogy of Fallot, atrial and ventricular septal defects, and renal and musculoskeletal abnormalities [2].
If a woman of childbearing potential is started on fingolimod, the advice in the summary of product characteristics is that she must be informed of the risk of teratogenicity and provided with a patient reminder card. She must have a negative pregnancy test before receiving the first dose and at suitable intervals during treatment as appropriate. She should be regularly advised to use effective contraception during treatment and for at least two months after stopping it. Women should be advised that recurrence of disease activity, which can be substantial, has been reported in around a quarter of women after stopping fingolimod to get pregnant [4-6].
In November 2018, the license for fingolimod was extended to include its use in for children and young adults (10-17 years) with MS [3]. Due to limited treatment options in this group, fingolimod use in young women is anticipated to increase.
In our opinion, fingolimod is best avoided in women of childbearing age unless there is no other suitable treatment. Serious consideration should be given to proactively switching women of childbearing age who are taking fingolimod to an alternative DMD of at least similar efficacy well in advance of trying to conceive, and the possibility of unplanned pregnancy should be considered in all women of childbearing potential.
Work is ongoing to develop a UK MS Pregnancy Register; the above new information highlights the need for an independent register to inform practice.
Dr Ruth Dobson1,2* and Dr Peter Brex3; on behalf of all authors and the UK MS Pregnancy Register Steering Group
Acknowledgements
The following individuals and groups provided input into this update:
UK MS Pregnancy Register steering group (Dr David Rog, Dr Owen Pearson, Dr Katy Murray, Dr Stella Hughes, Dr Helen Ford, Dr Peter Brex and Dr Ruth Dobson)
UK consensus on pregnancy in multiple sclerosis: ABN guideline authors (Dr Catherine Nelson-Piercy, Dr Pooja Dassan, Megan Roberts, Prof Gavin GIovannoni)
Aoife Shields, Neuroscience Specialist Pharmacist
References
1. https://www.acnr.co.uk/2019/09/interferon-beta-treatments-receive-positi...
2. https://www.gov.uk/drug-safety-update/fingolimod-gilenya-increased-risk-...
3. https://www.ema.europa.eu/en/documents/product-information/gilenya-epar-...
4. Hemat S, Houtschens M, Vidal-Jordana A, et al. Disease activity during pregnancy after Fingolimod withdrawal due to planning a pregnancy in women with multiple sclerosis. Poster presented at: 70th American Academy of Neurology Annual Meeting; April 21–27, 2018; Los Angeles, CA.
5. Meinl I, Havla J, Hohlfeld R, Kümpfel T. Recurrence of disease activity during pregnancy after cessation of fingolimod in multiple sclerosis. Mult Scler 2018;24(7):991–914. doi:10.1177/ 1352458517731913.
6. Novi G, Ghezzi A, Pizzorno M, et al. Dramatic rebounds of MS during pregnancy following fingolimod withdrawal. Neurol Neuroimmunol Neuroinflamm 2017;4(5):e377. doi:10.1212/ NXI.0000000000000377.
We read the article “neuromythololgy” by Martin Turner and Phillip Smith ,” clinic letters revisited”1. We completely agree that the 2002 Department of Health directive that patients should be sent a copy of their clinic letters was ,” a major force in improving clinic letters”.
The article however makes no mention of the 2018 advice2 from the Academy of Medical Royal Colleges entitled ,” please write to me”. It strongly recommends that most letters be addressed directly to the patient with a copy to the GP and that English fully comprehensible to patients is used and any technical language is explained. This approach avoids much of the discussion in the article of terms like ,”this pleasant gentleman”.
We have both adopted this recommended approach since 2018. One of us starts letters,” thank you for coming this morning and explaining that you are a 43 year old garage mechanic and that you have suffered tingling in the right hand for 6 months”. The other starts,” your GP wrote to me that you have had tingling in your right hand for 6 months”.
We have carried out a preliminary audit of patients, GPs, and hospital consultants attitude to this suggested change by showing them 2 similar letters one addressed to the GP and the other to the patient. GPs quite strongly favoured letters addressed to the patient saying they received far fewer requests to clarify what the hospital doctor meant. Patients were roughly evenly divided, whilst hospital consultants st...
We read the article “neuromythololgy” by Martin Turner and Phillip Smith ,” clinic letters revisited”1. We completely agree that the 2002 Department of Health directive that patients should be sent a copy of their clinic letters was ,” a major force in improving clinic letters”.
The article however makes no mention of the 2018 advice2 from the Academy of Medical Royal Colleges entitled ,” please write to me”. It strongly recommends that most letters be addressed directly to the patient with a copy to the GP and that English fully comprehensible to patients is used and any technical language is explained. This approach avoids much of the discussion in the article of terms like ,”this pleasant gentleman”.
We have both adopted this recommended approach since 2018. One of us starts letters,” thank you for coming this morning and explaining that you are a 43 year old garage mechanic and that you have suffered tingling in the right hand for 6 months”. The other starts,” your GP wrote to me that you have had tingling in your right hand for 6 months”.
We have carried out a preliminary audit of patients, GPs, and hospital consultants attitude to this suggested change by showing them 2 similar letters one addressed to the GP and the other to the patient. GPs quite strongly favoured letters addressed to the patient saying they received far fewer requests to clarify what the hospital doctor meant. Patients were roughly evenly divided, whilst hospital consultants strongly favoured the traditional letter to the GP.
Our impression is that very few hospital doctors have actually tried the new format. We recommend colleagues give it a try.
References.
1. Turner MR, Smith PEM, Practical Neurology 2019;19:457.
2. Working group on copying letters to patients. Department of health 2002
3. Please, write to me. Writing outpatient clinic letters to patients.
Academy of Medical Royal Colleges. 2018
As I make my way to the outpatient clinic, late as usual after the morning rounds to start another busy day, an unusual walk in front of me seizes my attention: an elderly lady, with a narrow-based and short-stepped gait, decreased left-arm swing and stooped posture; unable to see her face, I assume she’s heading for the neurology outpatient clinic. Yet she passes by and I lose sight of her on the ophthalmology service. Not a patient of ours, I think to myself. That same night, while having supper at a restaurant with my soon-to-be-wife, I notice a few tables away a man on his seventies celebrating his birthday, surrounded by family. In front of the candle-sparkling birthday cake, I get a glimpse of an unusual face: elevated eyebrows, like on a permanent surprise, unable to direct his gaze to the cake—Procerus sign and vertical gaze paresis—I think to myself, while I notice the stooped posture and global slowness of movements while everyone on the table compliments him—parkinsonism also, definitely progressive supranucl…—“You’re doing that again, leave the man alone.” My train of thought gets wrecked by my fiancee, not being the first time, she knows I tend to distinguish and observe people with abnormal movements. I leave the man alone and finish my supper. -SACT.
It was with exceptional interest that we read “The Waiting Room”, by Araújo et al. recently published online on Practical Neurolo...
As I make my way to the outpatient clinic, late as usual after the morning rounds to start another busy day, an unusual walk in front of me seizes my attention: an elderly lady, with a narrow-based and short-stepped gait, decreased left-arm swing and stooped posture; unable to see her face, I assume she’s heading for the neurology outpatient clinic. Yet she passes by and I lose sight of her on the ophthalmology service. Not a patient of ours, I think to myself. That same night, while having supper at a restaurant with my soon-to-be-wife, I notice a few tables away a man on his seventies celebrating his birthday, surrounded by family. In front of the candle-sparkling birthday cake, I get a glimpse of an unusual face: elevated eyebrows, like on a permanent surprise, unable to direct his gaze to the cake—Procerus sign and vertical gaze paresis—I think to myself, while I notice the stooped posture and global slowness of movements while everyone on the table compliments him—parkinsonism also, definitely progressive supranucl…—“You’re doing that again, leave the man alone.” My train of thought gets wrecked by my fiancee, not being the first time, she knows I tend to distinguish and observe people with abnormal movements. I leave the man alone and finish my supper. -SACT.
It was with exceptional interest that we read “The Waiting Room”, by Araújo et al. recently published online on Practical Neurology(1), where they elaborate on the importance of beginning the neurological examination before the patient enters the doctor's office. An excellent chronicle on many signs that we might have seen but not acknowledged, in a clear and concise manner. However, while reading the article some concerns (unrelated to the quality and value of the article) were brought to our attention. How about outside the clinic or the Hospital?
Should we approach the gentleman or the lady portrayed in the first two paragraphs by author SACT? For neurologists, particularly for movement disorders specialists, patients can be found everywhere. Should neurologists be observant of the Goldwater rule (2), that prohibits psychiatrists to diagnose a subject they have not personally examined, and to communicate that diagnosis without the subjects consent? Are we then only to observe and describe just like James Parkinson did(3)? We observe as a clinical exercise, to describe phenomenology but we would never approach a person in public to invite them to our clinic, or to give an unsolicited diagnosis; that would be preposterous, we have no right to do so. In ‘Do no harm’, Henry Marsh states that neurologists are like kids looking for “strange and obscure diseases, sometimes untreatable, (...) deeply fascinating, and which they collect like rare butterflies and report in their journals(4)”. Yet we feel this description to be quite unfair because phenomenology might seize our attention, but we must strive to be like the great physician that Sir William Osler envisioned, the one who cares about “the patient who has the disease.”.
As a general neurology resident, the movement disorders field seems more and more appealing. The fact that even outside the doctor’s office we can start the diagnostic process, prompted one of the authors (SACT) to write the opening paragraphs, based on real-life experiences, in order to ask for the author’s advice on how to approach this situation; and to encourage more residents into neurology and movement disorders, because of the growing pandemic of neurodegenerative disorders like Parkinson’s disease that is calling us into action(5).
Acknowledgments: SACT would like to thank Mariana Castillo-Torres (Bachelor in Languages for Interpreting and Translation), for her kind assistance on English-language, editing and style.
Contributorship Statement: SACT contributed to the conceptual design of the article with the opening paragraphs and drafting of the manuscript. FFA contributed with the drafting of the manuscript, and a review of intellectually relevant content. IEB contributed to article conception and design, drawing from personal experiences, as well as a review for intellectually relevant content. All authors agreed on the final version of the manuscript.
References
1. Araujo R, van de Warrenburg B, Lang A, et al. The Waiting Room: neurological observations made outside the movement disorder specialist's consulting office. Pract Neurol 2019 doi: 10.1136/practneurol-2018-002110
2. Lilienfeld SO, Miller JD, Lynam DR. The Goldwater Rule: Perspectives From, and Implications for, Psychological Science. Perspect Psychol Sci 2018;13(1):3-27. doi: 10.1177/1745691617727864
3. Kempster PA, Hurwitz B, Lees AJ. A new look at James Parkinson's Essay on the Shaking Palsy. Neurology 2007;69(5):482-5. doi: 10.1212/01.wnl.0000266639.50620.d1
4. Marsh H. Do no harm: stories of life, death and brain surgery: Hachette UK 2014.
5. Dorsey ER, Bloem BR. The Parkinson Pandemic-A Call to Action. JAMA Neurol 2018;75(1):9-10. doi: 10.1001/jamaneurol.2017.3299
We thank Dr Torres and colleagues for drawing the readers’ attention to the additional value of observing patients with parkinsonian syndromes even before they enter the hospital premises. They focus specifically on the question how neurologists should act when one incidentally spots clear signs of a neurological disease among total strangers in social situations, and they emphasize just how exceptionally difficult this may be for experts in movement disorders, as these conditions can be very visible even to bystanders. Many readers will recognize how compelled one can feel to make a heartfelt recommendation to strangers to seek a neurological consultation, for example when they demonstrate clear-cut signs of Parkinson’s disease or some other neurological condition. However, we have neither acted on the impulse as this would be a serious breach of this person’s privacy. In addition, we need to realize that we are fully ignorant of the person’s context, barriers and motives that have prevented this person – who might already be aware of the signs - from seeking medical attention. We agree that one can only observe, but interfering would be out of line. Indeed, we previously observed a remarkable Parkinson-like gait disorder in Russian president Vladimir Putin and several other highly ranked Russian officials,1 just because we could not suppress our almost innate tendency to analyze movements, in this case when observing the publicly available v...
We thank Dr Torres and colleagues for drawing the readers’ attention to the additional value of observing patients with parkinsonian syndromes even before they enter the hospital premises. They focus specifically on the question how neurologists should act when one incidentally spots clear signs of a neurological disease among total strangers in social situations, and they emphasize just how exceptionally difficult this may be for experts in movement disorders, as these conditions can be very visible even to bystanders. Many readers will recognize how compelled one can feel to make a heartfelt recommendation to strangers to seek a neurological consultation, for example when they demonstrate clear-cut signs of Parkinson’s disease or some other neurological condition. However, we have neither acted on the impulse as this would be a serious breach of this person’s privacy. In addition, we need to realize that we are fully ignorant of the person’s context, barriers and motives that have prevented this person – who might already be aware of the signs - from seeking medical attention. We agree that one can only observe, but interfering would be out of line. Indeed, we previously observed a remarkable Parkinson-like gait disorder in Russian president Vladimir Putin and several other highly ranked Russian officials,1 just because we could not suppress our almost innate tendency to analyze movements, in this case when observing the publicly available video materials of these individuals. An exception could be made for patients who are already part of one’s own clinic, and where a fortunate observation of e.g. this person’s gait outside the hospital may offer additional value to what can be noted within the hospital itself. For example, cobbled stones on the street can elicit occasional stumbles that might be missed on the perfectly even hospital floors. We also find it illustrative to see how difficult it can be for patients to step outside of their car (seats are usually very low, causing great problems for patients with Parkinson’s disease or proximal leg weakness). Finally, we have seen patients manifesting freezing of gait only when they approached the revolving doors of the hospital – these can be a great challenge for people with Parkinson's.
Bastiaan R. Bloem, MD, PhD. (corresponding author)
Radboud university medical centre
Donders Institute for Brain, Cognition and Behaviour
Department of Neurology
Nijmegen, the Netherlands
bas.bloem@radboudumc.nl
Rui Araújo, MD.
Neurology Department, Centro Hospitalar e Universitário de Coimbra
Coimbra, Portugal
rmma22@gmail.com
Bart P. van de Warrenburg, MD, PhD.
Radboud university medical centre
Donders Institute for Brain, Cognition and Behaviour
Department of Neurology;
Nijmegen, the Netherlands
bart.vandewarrenburg@radboudumc.nl
Anthony E. Lang, MD, FRCPC.
Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J Safra Program in Parkinson’s Disease, Toronto Western Hospital
399 Bathurst Street, Toronto, Ontario
lang@uhnresearch.ca
Andrew J. Lees, MD, FRCP, FMedSci.
National Hospital, Queen Square
Reta Lila Weston Institute, University College London
Reference
1. Araujo R, Ferreira JJ, Antonini A, Bloem BR. "Gunslinger's gait": a new cause of unilaterally reduced arm swing. BMJ 2015;351:h6141.
To the Editors,
Being a sufferer of Benign Fasciculation Syndrome (BFS) for more than a year, I read with great interest Professor Kiernan’s editorial (1), as well as Dr. Vercueil’s personal account of his encounter with the syndrome (2). I was relieved to see how much the “clinical course” of my case corresponded to that described by Professor Kiernan and Dr. Vercueil. They surfaced during a period of stress and sleep-deprivation, and has since kept going.
As Professor Kiernan writes, anything that increases central excitability can trigger fasciculations. This includes the fear that they form part of a prodrome to motor neuron disease (MND); a defining characteristic of Fasciculation Anxiety Syndrome in Clinicians (FASICS). Even though my diagnosis of BFS have been “confirmed” by an EMG and a neurologist, I still suffer from occasional episodes of FASICS exacerbations, surfacing like a recurrent nightmare according to its own inner logic. In other words, FASICS undoubtedly shares a quality with health anxiety, namely a jumbled relationship between symptoms and belief. The opening lines of H.P Lovecraft’s “The dreams in the witch house” captures this poetically; “Whether the dreams brought on the fever, or the fever brought on the dreams, Walter Gilman did not know”.
After years of working in hospitals you will have encountered some rare cases where the etiology of a disease have followed a non-standard pathway. These can unconsciously skew your wo...
Show MoreDear Editor,
We read with great interest the recent article by Drs Page & Gaillard describing neuroradiology signs and their variable utility [1].
We recently attempted to bring science to the art of descriptive radiological signs by conducting a randomized controlled trial to assess the utility of these signs for medical student radiology education [2]. We found that ‘Metaphoric Signs’ (linking radiology resemblance to an object or concept which is not actually present in an image) increased descriptive and diagnostic ability as well as students’ lesson enjoyment, when compared to teaching with normal anatomy comparators.
The importance of diagnostic accuracy of commonly used signs was similarly emphasised in our discussion. In addition to being diagnostically accurate, the success of a metaphoric sign also relies on how well it resonates with each individual learner, based on their perception of the real-world similarity alluded to in the radiology image and their prior experiences[3,4]. This is aptly exemplified by Author FG’s stronger association of the pontine appearance in osmotic demyelination with a blockbuster sci-fi trilogy than classical mythology.
An interesting observation in our study was that several students used incorrect wording for the name of the signs yet identified the correct diagnosis. Students who correctly diagnosed agenesis of the corpus callosum from a coronal MRI image containing the ‘Moose head sign’ [5] varia...
Show MoreTo the editor
We greatly enjoyed the excellent recent review on the diagnosis and management of primary lateral sclerosis (PLS). Turner and Talbot emphasize the importance of controlling spasticity as the most troublesome symptom, which is often associated with pain, discomfort and physical disability [1].
Show MoreAs the authors point out, the current standard of care (baclofen, tizanidine, benzodiazepines and botulinum toxin) is frequently complicated by side effects, difficulties in titration and a lack of well controlled clinical studies [2]. We were thus surprised that the authors omitted the option of medicinal cannabis from the discussion of this very important topic.
A study by Riva and colleagues offers clear evidence on the efficacy of the cannabinoid preparation Nabiximol for the control of spasticity in ALS and PLS patients [3], which is further supported by Meyer and colleagues providing compelling real world evidence of this effect [4].
The THC:CBD oromucosal spray (nabioximols, brand name Sativex) became approved in European Union countries in 2010. Although only approved for people with moderate to severe spasticity in multiple sclerosis, who have not responded adequately to other anti-spasticity regimes, THC:CBD is increasingly being used off-label for spasticity in patients with motor neuron diseases. In addition, the patients themselves have self-medicated with recreational cannabis for many years [5].
The multicenter, double-blind,...
We thank Dr Weydt and colleagues for their interest in our review. We are aware of the subjective benefit of cannabinoids reported by some living with multiple sclerosis, and a trial of THC:CDB spray is also approved by the UK’s National Institute for Health & Care Excellence (NICE). The CANALS study mentioned, in which PLS patients were well represented (28% of treatment arm and 20% of placebo arm), was a Phase 2 study powered to consider safety and tolerability. Adverse events were reported in 76% of the treatment group versus 27% in the placebo group. A reduction in the clinician’s Modified Ashworth Spasticity scale score was noted, and in the patient’s numeric rating scale score for pain, but not their scores for spasm or spasticity. In the discussion, the authors note that muscle cramping was not alleviated in a prior randomised trial of THC in ALS (1). We agree with their call for further evaluation, through a Phase 3 study of the benefit of cannabinoids over the licensed therapies for spasticity outlined in our review.
Reference:
1. Weber M, Goldman B, Truniger S. Tetrahydrocannabinol (THC) for cramps in amyotrophic lateral sclerosis: a randomised, double-blind crossover trial. J Neurol Neurosurg Psychiatry. 2010 Oct;81(10):1135-40. doi: 10.1136/jnnp.2009.200642. PMID: 20498181.
It gives more discomfort than pleasure to comment on DaT scanning again but your editorial [1] prompted me to find out that the number of DaTscans carried out in England is increasing yearly, from 4550 in 2012/3 to 8840 in 2018/9. A trip to the dentist might have been wiser, my long-expressed opinion (based on the fundamental principles that let down 18FDopa PET) being that the DaTscan is a waste of time, radiation and money [2]. In brief it is a low resolution, inadequately sensitive, inadequately reproducible test with too many false negatives and little knowledge of confounding influences. The acronym SWEDD has, mercifully, been consigned to the dustbin[3] but we don’t have the neuropathological studies or large and long-term blinded follow-up studies, in patients and healthy individuals, despite the many tests and years since its commercial introduction, that would tell the true false positive and negative rate of the test. We can’t then confidently say how strongly a normal result argues against a clinical diagnosis of PD. Neurology is not the only specialty that uses DaTscan and indication-creep (good for the shareholder, bad for the taxpayer) means that it is also being used to distinguish Lewy Body Dementia from Alzheimer’s Dementia despite limited supportive data (4). It must also be remembered that the test measures biochemistry, not pathology; the statement in the case presentation [5] “a DaTscan was normal, with no evidence of degenerative Parkinsonism” is...
Show MoreWith great interest, we read the review by Markus on personalising the secondary prevention approach to patients with stroke ¹, published in the most recent issue of Practical Neurology. Where we are presented with clinically useful and evidence-based advice for the etiological assessment of patients with acute ischemic stroke (AIS), focusing on lacunar stroke syndromes of a non-lacunar cause, and its appropriate therapeutic management. We consider the article of great importance: a must-read for all physicians who care for patients with AIS since etiological assessment is paramount to dictate the appropriate secondary prevention measures.
The author proposes using the TOAST classification (Trial of Organon 10172 in Acute Stroke Treatment), arguing that classification systems that prime clinical syndromes over pathophysiological mechanisms are less useful. Nevertheless, the author omitted one classification which–partially–resolves the issue: the ASCOD (Atherosclerosis, Small-vessel, Cardiac embolism, Other, Dissection) system ². A comprehensive classification, which allows for more than one aetiology, while giving a degree of a causal relationship to the presence of each category of disease (1 potential, 2 uncertain, 3 unlikely, 0 disease not detected) including incomplete assessment (9 insufficient work-up), while considering some clinical features.
The ASCOD approach permits the identification of patients with diseases that would have been left as indeterm...
Show MoreDear Editors,
Following the publication of the “UK consensus on pregnancy in multiple sclerosis: ABN guidelines” in January 2019, new data has become available and an update is required. Whilst these updates do not change the overall recommendations in the guidelines, they add information, which we feel all neurologists should be aware of in order to provide the highest quality of information to all women with MS considering pregnancy.
(1) Interferon beta preparations in pregnancy
In September 2019, the EMA Committee for Medicinal Products for Human Use (CHMP) recommended a label change for interferon beta-1a, peginterferon beta-1a and interferon beta-1b, i.e. Avonex, Betaferon, Extavia, Plegridy and Rebif, stating that they may be considered during pregnancy if clinically indicated, and can be used during breastfeeding [1]. This decision was based on data from interferon beta registries, national registries and post-marketing experience. However, data from exposure during second and third trimesters remains limited. The duration of exposure during the first trimester is uncertain, because data were collected when interferon beta use was contraindicated during pregnancy, and it is likely that treatment was interrupted in many women when the pregnancy was detected and/or confirmed.
This supports the recommendation in the “UK consensus on pregnancy in multiple sclerosis: ABN guidelines” that these products are safe to be continued at least until...
Show MoreWe read the article “neuromythololgy” by Martin Turner and Phillip Smith ,” clinic letters revisited”1. We completely agree that the 2002 Department of Health directive that patients should be sent a copy of their clinic letters was ,” a major force in improving clinic letters”.
The article however makes no mention of the 2018 advice2 from the Academy of Medical Royal Colleges entitled ,” please write to me”. It strongly recommends that most letters be addressed directly to the patient with a copy to the GP and that English fully comprehensible to patients is used and any technical language is explained. This approach avoids much of the discussion in the article of terms like ,”this pleasant gentleman”.
We have both adopted this recommended approach since 2018. One of us starts letters,” thank you for coming this morning and explaining that you are a 43 year old garage mechanic and that you have suffered tingling in the right hand for 6 months”. The other starts,” your GP wrote to me that you have had tingling in your right hand for 6 months”.
We have carried out a preliminary audit of patients, GPs, and hospital consultants attitude to this suggested change by showing them 2 similar letters one addressed to the GP and the other to the patient. GPs quite strongly favoured letters addressed to the patient saying they received far fewer requests to clarify what the hospital doctor meant. Patients were roughly evenly divided, whilst hospital consultants st...
Show MoreAs I make my way to the outpatient clinic, late as usual after the morning rounds to start another busy day, an unusual walk in front of me seizes my attention: an elderly lady, with a narrow-based and short-stepped gait, decreased left-arm swing and stooped posture; unable to see her face, I assume she’s heading for the neurology outpatient clinic. Yet she passes by and I lose sight of her on the ophthalmology service. Not a patient of ours, I think to myself. That same night, while having supper at a restaurant with my soon-to-be-wife, I notice a few tables away a man on his seventies celebrating his birthday, surrounded by family. In front of the candle-sparkling birthday cake, I get a glimpse of an unusual face: elevated eyebrows, like on a permanent surprise, unable to direct his gaze to the cake—Procerus sign and vertical gaze paresis—I think to myself, while I notice the stooped posture and global slowness of movements while everyone on the table compliments him—parkinsonism also, definitely progressive supranucl…—“You’re doing that again, leave the man alone.” My train of thought gets wrecked by my fiancee, not being the first time, she knows I tend to distinguish and observe people with abnormal movements. I leave the man alone and finish my supper. -SACT.
It was with exceptional interest that we read “The Waiting Room”, by Araújo et al. recently published online on Practical Neurolo...
Show MoreWe thank Dr Torres and colleagues for drawing the readers’ attention to the additional value of observing patients with parkinsonian syndromes even before they enter the hospital premises. They focus specifically on the question how neurologists should act when one incidentally spots clear signs of a neurological disease among total strangers in social situations, and they emphasize just how exceptionally difficult this may be for experts in movement disorders, as these conditions can be very visible even to bystanders. Many readers will recognize how compelled one can feel to make a heartfelt recommendation to strangers to seek a neurological consultation, for example when they demonstrate clear-cut signs of Parkinson’s disease or some other neurological condition. However, we have neither acted on the impulse as this would be a serious breach of this person’s privacy. In addition, we need to realize that we are fully ignorant of the person’s context, barriers and motives that have prevented this person – who might already be aware of the signs - from seeking medical attention. We agree that one can only observe, but interfering would be out of line. Indeed, we previously observed a remarkable Parkinson-like gait disorder in Russian president Vladimir Putin and several other highly ranked Russian officials,1 just because we could not suppress our almost innate tendency to analyze movements, in this case when observing the publicly available v...
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