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
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.
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
With respect to the article entitled ‘Montezuma’s revenge’: neurological disorders in the returning traveller, the authors make an important point about the treatment of malaria: hopefully GPs and potential travellers in the UK are always extra-ordinarily careful about malaria prophylaxis.
However, I believe it worth pointing out that the list of illnesses which may be acquired in certain countries is, unfortunately, simply wrong (Figure 1). The following infectious diseases are not found in South Africa:
1. Relapsing fever (except described in penguins![1]).
2. Plague.
3. African sleeping sickness.
Following successful treatment of the outbreak in Madagascar perhaps one should also note that the Western half of the United States is historically a region where plague is found (not mentioned by the authors)[2]. I appreciate that the list provided in the article is derived from another source, but I am sure the authors will agree on the importance of avoiding the promulgation of inaccurate information.
Finally, despite what the authors may maintain, the correct treatment, if any, of cerebral neurocysticercosis does indeed continue to remain controversial, and adequate RCTs have not been performed[3][4]. Decisions on treatment may well need to be made on a case by case basis, and it is important that neurologists should be aware of the dearth of hard evidence concerning the treatment of neurocysticercosis.
With respect to the article entitled ‘Montezuma’s revenge’: neurological disorders in the returning traveller, the authors make an important point about the treatment of malaria: hopefully GPs and potential travellers in the UK are always extra-ordinarily careful about malaria prophylaxis.
However, I believe it worth pointing out that the list of illnesses which may be acquired in certain countries is, unfortunately, simply wrong (Figure 1). The following infectious diseases are not found in South Africa:
1. Relapsing fever (except described in penguins![1]).
2. Plague.
3. African sleeping sickness.
Following successful treatment of the outbreak in Madagascar perhaps one should also note that the Western half of the United States is historically a region where plague is found (not mentioned by the authors)[2]. I appreciate that the list provided in the article is derived from another source, but I am sure the authors will agree on the importance of avoiding the promulgation of inaccurate information.
Finally, despite what the authors may maintain, the correct treatment, if any, of cerebral neurocysticercosis does indeed continue to remain controversial, and adequate RCTs have not been performed[3][4]. Decisions on treatment may well need to be made on a case by case basis, and it is important that neurologists should be aware of the dearth of hard evidence concerning the treatment of neurocysticercosis.
1 Yabsley MJ, Parsons NJ, Horne EC, et al. Novel relapsing fever Borrelia detected in African penguins (Spheniscus demersus) admitted to two rehabilitation centers in South Africa. Parasitol Res 2012;110:1125–30. doi:10.1007/s00436-011-2602-2
2 Global distribution of natural plague foci. WHO. 2016.http://www.who.int/csr/disease/plague/Plague-map-2016.pdf?ua=1
3 Carpio A, Fleury A, Romo ML, et al. Neurocysticercosis: the good, the bad, and the missing. Expert Rev Neurother 2018;18:289–301. doi:10.1080/14737175.2018.1451328
4 Singh G, Sharma R. Controversies in the treatment of seizures associated with neurocysticercosis. Epilepsy Behav 2017;76:163–7. doi:10.1016/j.yebeh.2017.05.033
We are very grateful to Dr Rota and colleagues for their interest in our guideline.
In essence, we agree that international consensus on DOAC level measurement and cut-off levels would be welcome.
The experience reported by Dr Rota and colleagues of their use of idarucizumab for emergency reversal of dabigatran prior to lumbar puncture is reassuring. Our guideline refers to the need to consult a haematologist prior to administration; however, a guideline is for guidance and we recognise that locally agreed protocols may vary. Likewise, the timings mentioned in the guideline relating to the safe reinitiation of DOACs are by necessity pragmatic. Further evidence in this area will of course be very welcome to further inform practice.
Dear Editor,
We read the article by Dodd et al. (2018) [1] with great interest. The authors provide evidence-based recommendations for the periprocedural management of antithrombotic and anticoagulant treatment in patients who require a lumbar puncture (LP). Indeed, this is a very relevant practical point for neurologists, above all when an urgent diagnostic LP is mandatory to rule out an infectious disease of the central nervous system, or a subarachnoid hemorrhage. The recommendations on adjustment/reversal of warfarin for patients on oral anticoagulants, who require LP, are well known, i.e. long-term LP allowed if INR is < 1.4. Whilst the question of how to manage patients on Direct Oral Anticoagulants (DOACS), a relatively novel pharmacological class, is also to be answered. Dodd et al’s article [1] reports that if a non-urgent LP has to be carried out he these patients, current recommendations vary among different advisory bodies about the time lapse necessary for DOAC withdrawal before the LP, depending on the renal function. The interesting possibility of measuring the drug-specific levels, so as to estimate the anticoagulant effect of a DOAC, is also mentioned, although the authors are of the opinion that routine testing before the LP is not necessary. [1]
We believe that this is a crucial point, above all in the case of an urgent or emergent LP. Indeed, in our experience, when available, drug-specific levels can be obtained quickly and guide the cl...
Dear Editor,
We read the article by Dodd et al. (2018) [1] with great interest. The authors provide evidence-based recommendations for the periprocedural management of antithrombotic and anticoagulant treatment in patients who require a lumbar puncture (LP). Indeed, this is a very relevant practical point for neurologists, above all when an urgent diagnostic LP is mandatory to rule out an infectious disease of the central nervous system, or a subarachnoid hemorrhage. The recommendations on adjustment/reversal of warfarin for patients on oral anticoagulants, who require LP, are well known, i.e. long-term LP allowed if INR is < 1.4. Whilst the question of how to manage patients on Direct Oral Anticoagulants (DOACS), a relatively novel pharmacological class, is also to be answered. Dodd et al’s article [1] reports that if a non-urgent LP has to be carried out he these patients, current recommendations vary among different advisory bodies about the time lapse necessary for DOAC withdrawal before the LP, depending on the renal function. The interesting possibility of measuring the drug-specific levels, so as to estimate the anticoagulant effect of a DOAC, is also mentioned, although the authors are of the opinion that routine testing before the LP is not necessary. [1]
We believe that this is a crucial point, above all in the case of an urgent or emergent LP. Indeed, in our experience, when available, drug-specific levels can be obtained quickly and guide the clinical management in this clinical setting. Notably, LP may be performed immediately, which may be clinically relevant, if the drug levels are under the cut-off chosen to rule out any anticoagulant effect. However, consensus is still lacking and different advisory bodies (French expert opinion, [2] Swiss operating procedures [3]) set the lower threshold limit for the anticoagulant effect of DOACs at a range between 20-50 ng/mL for other urgent procedures, like intravenous thrombolysis.
Therefore, if there were an international, or at least European consensus on the recommended DOAC drug level cut-off to rule out any anticoagulant effect, the management of patients requiring urgent or emergent procedure, like LP or surgery, would be facilitated. Obviously, if the DOACs levels are within the therapeutical range, a delay before the LP is required, according to the recommendations.
However, a remarkably different management may be prospected if an LP is indicated in patients is on dabigatran, where emergency reversal may be achieved by idarucizumab, as Dodd et al. [1] report, recommending previous consultation with a hematologist. This is another crucial point and a clinical novelty, where an urgent/emergent LP may be allowed in dabigatran-treated patients in only a few minutes after idarucizumab administration. Although literature evidence is still scarce and only a few cases (three, considering ours) have been reported to date, in our experience an LP after dabigatran emergency reversal by idarucizumab is a safe procedure, as was demonstrated by our two personal cases, one published, [4] one in press. Therefore, we are of the opinion that idarucizumab could be safely administered to patients requiring LP, also in the absence of a haematologist, to save the time inevitably required by the consultation.
Another reversal agent, andexanet-alpha, was recently approved by the FDA and seems to be appealing for patients on direct factor Xa inhibitors (rivaroxaban, apixaban and edoxaban), as Dodd et al. [1] report. However, the time required to reverse the DOAC effect and the putative pro-thrombotic effect of andexanet-alpha make it an unlikely candidate for an emergency reversal of direct factor Xa inhibitors before an LP.
Lastly, Dodd et al. [1] recommend the DOAC first dose 6 hours after an atraumatic LP. We suggest the following algorhythm: in the presence of a CHADSVADSC score of >=3 administer the DOAC after 6 hours and after 12 hours with a CHADSVASC score of <3.
It is, however, encouraging that a novel clinical scenario is emerging for the management of patients on DOACs, where outstanding opportunities are provided by the availability of specific DOAC levels dosages, even if international consensus would be welcome to set a clear cut-offs for the anticoagulant effect and, above all, by the reversal agent idarucizumab, which seems to be a safe and effective tool for urgent/emergent LP in dabigatran-treated patients.
References
1. Dodd KC, Emsley HCA, Desborough MJR, Chhetri SK. Periprocedural antithrombotic management for lumbar puncture: Association of British Neurologists clinical guideline. Pract Neurol. 2018 Aug 28. pii: practneurol-2017-001820. doi: 10.1136/practneurol-2017-001820. [Epub ahead of print]
2. Touzé E, Gruel Y, Gouin-Thibault I, De Maistre E, et al. Intravenous thrombolysis for acute ischaemic stroke in patients on direct oral anticoagulants. Expert opinion of the SocieteFrancaise de NeurologieVasculaire (SFNV) and the GroupeFrancaisd'etudes sur l'Hemostase et la Thrombose (GFHT). Eur J Neurol 2018 Jan 23. doi: 10.1111/ene.13582.
3. Seiffge DJ, Traenka C, Polymeris AA, et al. Intravenous Thrombolysis in Patients with Stroke Taking Rivaroxaban Using Drug Specific Plasma Levels: Experience with a Standard Operation Procedure in Clinical Practice. J Stroke 2017; 19(3): 347-355.
4. Agosti S, Casalino L, Daffonchio A, Arena L, Celli L and Rota E. Emergency Lumbar Puncture for Suspected Meningitis after Dabigatran Reversal with Idarucizumab: A Case Report. J Clin Case Rep 2018, 8:4.
Sir,
I thoroughly enjoyed reading Dr. Allen’s excellent paper on the right way to do the ankle jerk. He is quite right in saying that ‘tendon reflexes’ are not tendon reflexes. Tapping a tendon leads to stimulation of the Golgi tendon organs, which are actually inhibitory to the alpha neurones, so no response should occur. The muscle contraction probably results from the vibrations transmitted to the intrafusal muscle fibres, leading to activation of the anterior horn cells and thus causing muscle contraction in response to the stimulus. The term “deep tendon reflex” is completely inappropriate; which deep tendons can one access? Levator palpebrae superioris? Piriformis? Gluteus medius? The tendons percussed have to be superficial so that we can get at them.
Although Dr. Allen’s method is absolutely appropriate in patients who are confined to bed, those patients who are mobile can, I suggest, be better examined if you ask them first to kneel on the seat of the chair on which they were sitting, grasping its back with their hands. They are thus unconsciously performing a Jendrassik manoeuvre, augmenting any response that their bodies might make. Their ankles, projected out behind the seat, can be tapped easily, and the response noted with equal facility.
Now in my 80s, I cannot remember whether it was Erb or Westphal or Romberg or somebody else who first suggested this method; but after 55 years in Neurology, I still find it the best way to asse...
Sir,
I thoroughly enjoyed reading Dr. Allen’s excellent paper on the right way to do the ankle jerk. He is quite right in saying that ‘tendon reflexes’ are not tendon reflexes. Tapping a tendon leads to stimulation of the Golgi tendon organs, which are actually inhibitory to the alpha neurones, so no response should occur. The muscle contraction probably results from the vibrations transmitted to the intrafusal muscle fibres, leading to activation of the anterior horn cells and thus causing muscle contraction in response to the stimulus. The term “deep tendon reflex” is completely inappropriate; which deep tendons can one access? Levator palpebrae superioris? Piriformis? Gluteus medius? The tendons percussed have to be superficial so that we can get at them.
Although Dr. Allen’s method is absolutely appropriate in patients who are confined to bed, those patients who are mobile can, I suggest, be better examined if you ask them first to kneel on the seat of the chair on which they were sitting, grasping its back with their hands. They are thus unconsciously performing a Jendrassik manoeuvre, augmenting any response that their bodies might make. Their ankles, projected out behind the seat, can be tapped easily, and the response noted with equal facility.
Now in my 80s, I cannot remember whether it was Erb or Westphal or Romberg or somebody else who first suggested this method; but after 55 years in Neurology, I still find it the best way to assess the ankle jerk in mobile patients.
It is interesting that Graham Warner mentions the sound of absent ankle reflexes. I remember discussing the thud of an absent ankle with Michael Harrison, who was my greatest clinical guru, when I was his registrar at the Middlesex Hospital in London. I have also mentioned it to my students but tended to get the l look back that tells me politely that I might not come from the same planet as them. I suspect this is because learning to examine patients involves learning novel complex perceptions. Much of this is honed over many years and like all complex perceptions involves more than one sensory modality. As neurologists, we all know that appreciating a subtle flavour involves lot more than the sensory information from the tongue; it is mostly olfactory but also involves texture and temperature as well as context and expectation. This is why to many of us the discussions of wine buffs about the subtle flavours of different vintages sound like the ramblings of someone from an alien world. Thus when teaching students one has to realise that they are learning complex perceptions in unfamiliar contexts and may not be able to appreciate subtleties such as the sound of reflexes. So their “what planet is he from?” look is appropriate because perceptually they are in a different world to that of an experienced clinician. As teachers, we need to guide them to our world of complex perceptions and not all can follow, just as I would never succeed as a wine-taster (not that I don’t like...Show More
It is interesting that Graham Warner mentions the sound of absent ankle reflexes. I remember discussing the thud of an absent ankle with Michael Harrison, who was my greatest clinical guru, when I was his registrar at the Middlesex Hospital in London. I have also mentioned it to my students but tended to get the l look back that tells me politely that I might not come from the same planet as them. I suspect this is because learning to examine patients involves learning novel complex perceptions. Much of this is honed over many years and like all complex perceptions involves more than one sensory modality. As neurologists, we all know that appreciating a subtle flavour involves lot more than the sensory information from the tongue; it is mostly olfactory but also involves texture and temperature as well as context and expectation. This is why to many of us the discussions of wine buffs about the subtle flavours of different vintages sound like the ramblings of someone from an alien world. Thus when teaching students one has to realise that they are learning complex perceptions in unfamiliar contexts and may not be able to appreciate subtleties such as the sound of reflexes. So their “what planet is he from?” look is appropriate because perceptually they are in a different world to that of an experienced clinician. As teachers, we need to guide them to our world of complex perceptions and not all can follow, just as I would never succeed as a wine-taster (not that I don’t like a nice Malbec). Furthermore, many tendon hammers around these days have such hard rings that hitting one’s hand hard enough to listen for absent reflexes may prove a painful experience. Show Less
Chris Allen’s series on neurological examination offers huge relief to fellow experts who execute it incorrectly, such as the “ankle jerks”. I too use the plantar method although caution Juniors/Students to do it the right way (tendon method) when non-neurologists assess in osce’s/finals. But in teaching them how to tick the boxes I have questioned why I do what I do (self-questioning being one of the reasons I seize the opportunity to train others). I then recall as an SHO Simon Nurick (one of the people who inspired me) tellinging me to listen to the reflexes, in that when absent one hears a dull thud (rather like that of a pleural effusion, so it is interesting to learn that neurological hammers evolved from those used for respiratory examination). And it sounds more resonant when present, all akin to how taught a violin string might be, I tell them. In fact not only do I listen, as well as observe (visual observation is all that generalists teach Medical Students), but also feel. I believe this most useful when the tension in the “strings” are greatest in hypertonic such that there may be no apparent movement nor sound, but one gets tactile feed back. Hence where possible I always strike the hammer against my own hand placed carefully to achieve best transmission of the force. I suspect all “experts” draw the greater data without knowing and can so better analyse the clinical situation.
I’m confident too that they like me may even apply varying non-standard force...
Chris Allen’s series on neurological examination offers huge relief to fellow experts who execute it incorrectly, such as the “ankle jerks”. I too use the plantar method although caution Juniors/Students to do it the right way (tendon method) when non-neurologists assess in osce’s/finals. But in teaching them how to tick the boxes I have questioned why I do what I do (self-questioning being one of the reasons I seize the opportunity to train others). I then recall as an SHO Simon Nurick (one of the people who inspired me) tellinging me to listen to the reflexes, in that when absent one hears a dull thud (rather like that of a pleural effusion, so it is interesting to learn that neurological hammers evolved from those used for respiratory examination). And it sounds more resonant when present, all akin to how taught a violin string might be, I tell them. In fact not only do I listen, as well as observe (visual observation is all that generalists teach Medical Students), but also feel. I believe this most useful when the tension in the “strings” are greatest in hypertonic such that there may be no apparent movement nor sound, but one gets tactile feed back. Hence where possible I always strike the hammer against my own hand placed carefully to achieve best transmission of the force. I suspect all “experts” draw the greater data without knowing and can so better analyse the clinical situation.
I’m confident too that they like me may even apply varying non-standard forces to gain more data points and look for the degree of spread I do to judge the UMN flavour of things.
Eponymous names are not allowed, and I don’t know if SN’s teaching was something handed down, so tendon jerk sounds cannot be labelled Nurick’s sign, but in the world of evidenced practice we can still recognise experience, wisdom and “eminence”??
Graham Warner
Dear Editor,
Thomas Brown provided a helpful recent review of pure autonomic failure (PAF) in this journal1. However, there is one very important point which I would wish to add. The diagnosis of this rare condition should be made with extreme caution in the early years of presentation. The first case in the review had a diagnosis of PAF made just two years after symptoms began. In fact, other commoner causes of primary autonomic failure may declare themselves many years after the symptoms first begin, and I have seen a number of such cases during my time at the autonomic unit in Queen Square. A rough rule of thumb used to be that at least five years should pass before making a definitive diagnosis of PAF2. More recent evidence suggests that even longer may be required. A third of cases already with symptoms suggestive of PAF for an average of 5 years were found to have an alternative diagnosis with subsequent follow up for a further four years3. Alternative diagnoses included other synucleinopathies such as Parkinson’s disease, dementia with Lewy bodies or multiple system atrophy.
For many years attempts have been made to find reliable ways to distinguish PAF from other causes of primary autonomic failure. PAF is seen classically as a post-ganglionic disorder whilst multiple system atrophy is seen as a pre-ganglionic form of primary autonomic failure. Utilising this pathophysiological difference, a variety of differentiating tests have been attempted....
Dear Editor,
Thomas Brown provided a helpful recent review of pure autonomic failure (PAF) in this journal1. However, there is one very important point which I would wish to add. The diagnosis of this rare condition should be made with extreme caution in the early years of presentation. The first case in the review had a diagnosis of PAF made just two years after symptoms began. In fact, other commoner causes of primary autonomic failure may declare themselves many years after the symptoms first begin, and I have seen a number of such cases during my time at the autonomic unit in Queen Square. A rough rule of thumb used to be that at least five years should pass before making a definitive diagnosis of PAF2. More recent evidence suggests that even longer may be required. A third of cases already with symptoms suggestive of PAF for an average of 5 years were found to have an alternative diagnosis with subsequent follow up for a further four years3. Alternative diagnoses included other synucleinopathies such as Parkinson’s disease, dementia with Lewy bodies or multiple system atrophy.
For many years attempts have been made to find reliable ways to distinguish PAF from other causes of primary autonomic failure. PAF is seen classically as a post-ganglionic disorder whilst multiple system atrophy is seen as a pre-ganglionic form of primary autonomic failure. Utilising this pathophysiological difference, a variety of differentiating tests have been attempted. One of the more promising has been the use of functional imaging to demonstrate the cardiac sympathetic the denervation one would expect with the post-ganglionic involvement of sympathetic nerves in PAF in contrast to multiple system atrophy. However this does not distinguish PAF from Parkinson’s disease reliably. Whilst some differences in the haemodynamic responses to stimuli such as water drinking may exist between PAF and other conditions4, as yet there is not a single test which can with certainty isolate true cases of PAF from its potential mimics. These conditions can differ significantly in terms of management and prognosis. Therefore whilst a working diagnosis of PAF may be tentatively considered early in the clinical course, ongoing surveillance over many years would be an important practical suggestion in such cases.
REFERENCES
1 Brown TP. Pure autonomic failure. Pract Neurol. 2017 Oct;17(5):341-348
2 Freeman R. Pure Autonomic Failure: An immaculate misconception? Neurology 2004;63;953-954
3 Kaufmann H et al. Natural history of pure autonomic failure: A United States prospective cohort. Ann Neurol. 2017 Feb;81(2):287-297
4 Young TM, Mathias CJ. The effects of water ingestion on orthostatic hypotension in two groups of chronic autonomic failure: multiple system atrophy and pure autonomic failure. J Neurol Neurosurg Psychiatry. 2004 Dec;75(12):1737-41.
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...
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...
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 MoreTo the Editor
With respect to the article entitled ‘Montezuma’s revenge’: neurological disorders in the returning traveller, the authors make an important point about the treatment of malaria: hopefully GPs and potential travellers in the UK are always extra-ordinarily careful about malaria prophylaxis.
However, I believe it worth pointing out that the list of illnesses which may be acquired in certain countries is, unfortunately, simply wrong (Figure 1). The following infectious diseases are not found in South Africa:
1. Relapsing fever (except described in penguins![1]).
2. Plague.
3. African sleeping sickness.
Following successful treatment of the outbreak in Madagascar perhaps one should also note that the Western half of the United States is historically a region where plague is found (not mentioned by the authors)[2]. I appreciate that the list provided in the article is derived from another source, but I am sure the authors will agree on the importance of avoiding the promulgation of inaccurate information.
Finally, despite what the authors may maintain, the correct treatment, if any, of cerebral neurocysticercosis does indeed continue to remain controversial, and adequate RCTs have not been performed[3][4]. Decisions on treatment may well need to be made on a case by case basis, and it is important that neurologists should be aware of the dearth of hard evidence concerning the treatment of neurocysticercosis.
1 Yab...
Show MoreWe are very grateful to Dr Rota and colleagues for their interest in our guideline.
In essence, we agree that international consensus on DOAC level measurement and cut-off levels would be welcome.
The experience reported by Dr Rota and colleagues of their use of idarucizumab for emergency reversal of dabigatran prior to lumbar puncture is reassuring. Our guideline refers to the need to consult a haematologist prior to administration; however, a guideline is for guidance and we recognise that locally agreed protocols may vary. Likewise, the timings mentioned in the guideline relating to the safe reinitiation of DOACs are by necessity pragmatic. Further evidence in this area will of course be very welcome to further inform practice.
Dear Editor,
Show MoreWe read the article by Dodd et al. (2018) [1] with great interest. The authors provide evidence-based recommendations for the periprocedural management of antithrombotic and anticoagulant treatment in patients who require a lumbar puncture (LP). Indeed, this is a very relevant practical point for neurologists, above all when an urgent diagnostic LP is mandatory to rule out an infectious disease of the central nervous system, or a subarachnoid hemorrhage. The recommendations on adjustment/reversal of warfarin for patients on oral anticoagulants, who require LP, are well known, i.e. long-term LP allowed if INR is < 1.4. Whilst the question of how to manage patients on Direct Oral Anticoagulants (DOACS), a relatively novel pharmacological class, is also to be answered. Dodd et al’s article [1] reports that if a non-urgent LP has to be carried out he these patients, current recommendations vary among different advisory bodies about the time lapse necessary for DOAC withdrawal before the LP, depending on the renal function. The interesting possibility of measuring the drug-specific levels, so as to estimate the anticoagulant effect of a DOAC, is also mentioned, although the authors are of the opinion that routine testing before the LP is not necessary. [1]
We believe that this is a crucial point, above all in the case of an urgent or emergent LP. Indeed, in our experience, when available, drug-specific levels can be obtained quickly and guide the cl...
Sir,
I thoroughly enjoyed reading Dr. Allen’s excellent paper on the right way to do the ankle jerk. He is quite right in saying that ‘tendon reflexes’ are not tendon reflexes. Tapping a tendon leads to stimulation of the Golgi tendon organs, which are actually inhibitory to the alpha neurones, so no response should occur. The muscle contraction probably results from the vibrations transmitted to the intrafusal muscle fibres, leading to activation of the anterior horn cells and thus causing muscle contraction in response to the stimulus. The term “deep tendon reflex” is completely inappropriate; which deep tendons can one access? Levator palpebrae superioris? Piriformis? Gluteus medius? The tendons percussed have to be superficial so that we can get at them.
Although Dr. Allen’s method is absolutely appropriate in patients who are confined to bed, those patients who are mobile can, I suggest, be better examined if you ask them first to kneel on the seat of the chair on which they were sitting, grasping its back with their hands. They are thus unconsciously performing a Jendrassik manoeuvre, augmenting any response that their bodies might make. Their ankles, projected out behind the seat, can be tapped easily, and the response noted with equal facility.
Now in my 80s, I cannot remember whether it was Erb or Westphal or Romberg or somebody else who first suggested this method; but after 55 years in Neurology, I still find it the best way to asse...
Show MoreChris Allen’s series on neurological examination offers huge relief to fellow experts who execute it incorrectly, such as the “ankle jerks”. I too use the plantar method although caution Juniors/Students to do it the right way (tendon method) when non-neurologists assess in osce’s/finals. But in teaching them how to tick the boxes I have questioned why I do what I do (self-questioning being one of the reasons I seize the opportunity to train others). I then recall as an SHO Simon Nurick (one of the people who inspired me) tellinging me to listen to the reflexes, in that when absent one hears a dull thud (rather like that of a pleural effusion, so it is interesting to learn that neurological hammers evolved from those used for respiratory examination). And it sounds more resonant when present, all akin to how taught a violin string might be, I tell them. In fact not only do I listen, as well as observe (visual observation is all that generalists teach Medical Students), but also feel. I believe this most useful when the tension in the “strings” are greatest in hypertonic such that there may be no apparent movement nor sound, but one gets tactile feed back. Hence where possible I always strike the hammer against my own hand placed carefully to achieve best transmission of the force. I suspect all “experts” draw the greater data without knowing and can so better analyse the clinical situation.
Show MoreI’m confident too that they like me may even apply varying non-standard force...
Dear Editor,
Show MoreThomas Brown provided a helpful recent review of pure autonomic failure (PAF) in this journal1. However, there is one very important point which I would wish to add. The diagnosis of this rare condition should be made with extreme caution in the early years of presentation. The first case in the review had a diagnosis of PAF made just two years after symptoms began. In fact, other commoner causes of primary autonomic failure may declare themselves many years after the symptoms first begin, and I have seen a number of such cases during my time at the autonomic unit in Queen Square. A rough rule of thumb used to be that at least five years should pass before making a definitive diagnosis of PAF2. More recent evidence suggests that even longer may be required. A third of cases already with symptoms suggestive of PAF for an average of 5 years were found to have an alternative diagnosis with subsequent follow up for a further four years3. Alternative diagnoses included other synucleinopathies such as Parkinson’s disease, dementia with Lewy bodies or multiple system atrophy.
For many years attempts have been made to find reliable ways to distinguish PAF from other causes of primary autonomic failure. PAF is seen classically as a post-ganglionic disorder whilst multiple system atrophy is seen as a pre-ganglionic form of primary autonomic failure. Utilising this pathophysiological difference, a variety of differentiating tests have been attempted....
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