I enjoyed this article on this important area of non-traumatic non-aneurysmal convexity SAH. The authors seems to have not recognised the literature that points to convexity/Sulcal subarachnoid haemorrhage in the context of ipsilateral carotid artery stenosis, which in some case series make up a significant proprtion of the cases (10-50%, references below). Also I would be cautious about having dichotomoised age as a rule, but better as a guide. In the case of carotid artery stenosis this is seen in the under 60s sometimes.
Geraldes et al J Stroke Cereb Dis 23:e23-30, 2014.
Zhao et al J Int Med Res 2017 45:1870.
Renou et al 2012 Cerebrovasc Dis. 2012;34(2):147-52.
Forman et al; J Stroke Cerebrovasc Dis. 2019;28(12):104473.
I read the article by Alvar Paris et al. with great interest. The consumption of nitrous oxide (N2O) has recently surfaced as an exponentially growing form of recreational abuse, particularly among students, due to its euphoric effects and accessibility. The European Monitoring Centre for Drugs and Drug Addiction has expressed concern in a recent report about the recreational use of N2O in Europe. 1
The most common neurological complications associated with N2O consumption are toxic polyneuropathy and subacute combined degeneration of the spinal cord (N2O-SACD), which may occur concurrently— a critical aspect that should not be overlooked. Initial clinical descriptions of N2O-SACD were reported in 2015 and complicated episodic use mediated through canisters. 2 Recently, the market for N2O distribution has expanded with diverse consumption accessories. The introduction of larger cylinders facilitates the consumption of significant amounts of N2O, posing younger individuals at an elevated risk of early and severe Vitamin B12 deficiency along with systemic complications. Consequently, new complications associated with N2O intoxication have been described, and their wide-ranging effects are regularly emphasized in the literature.
Beyond psychiatric symptoms like hallucinations, cognitive impairments including Gayet-Wernicke's encephalopathy have been recently reported. 3 These deficiencies are mediated by Vitamin B1 levels, suggesting a risk of broader deficie...
I read the article by Alvar Paris et al. with great interest. The consumption of nitrous oxide (N2O) has recently surfaced as an exponentially growing form of recreational abuse, particularly among students, due to its euphoric effects and accessibility. The European Monitoring Centre for Drugs and Drug Addiction has expressed concern in a recent report about the recreational use of N2O in Europe. 1
The most common neurological complications associated with N2O consumption are toxic polyneuropathy and subacute combined degeneration of the spinal cord (N2O-SACD), which may occur concurrently— a critical aspect that should not be overlooked. Initial clinical descriptions of N2O-SACD were reported in 2015 and complicated episodic use mediated through canisters. 2 Recently, the market for N2O distribution has expanded with diverse consumption accessories. The introduction of larger cylinders facilitates the consumption of significant amounts of N2O, posing younger individuals at an elevated risk of early and severe Vitamin B12 deficiency along with systemic complications. Consequently, new complications associated with N2O intoxication have been described, and their wide-ranging effects are regularly emphasized in the literature.
Beyond psychiatric symptoms like hallucinations, cognitive impairments including Gayet-Wernicke's encephalopathy have been recently reported. 3 These deficiencies are mediated by Vitamin B1 levels, suggesting a risk of broader deficiencies beyond Vitamin B12. Physicians should be vigilant about potential cognitive and psycho-behavioral deficiencies. Neuropsychological assessments and proactive identification of cognitive sequelae are crucial.
To physical health risks, one must also consider the risks of co-addiction to psychoactive substances, as well as psychosocial risks like anxiety and depression that may complicate N2O consumption. The risk of road accidents should also be highlighted.
Radiologically, the described MRI findings appear to be related to a higher density of myelinated fibers in the fasciculus gracilis in the cervical area. This can explain the patients' sensory disorders like numbness and ataxia. However, muscle weakness and pyramidal signs often accompany these sensory disorders, pointing to a close association with pyramidal tract fibers. Frequent signal abnormalities in the lateral corticospinal tracts on axial MRI series have been noted. This emerging neuroradiological pattern aligns with recent descriptions of frequent motor disorders and warrants further analysis.
Physicians must be aware of the evolving spectrum of neurological complications associated with N2O intoxication, as well as their radiological characteristics. A surge in case incidence is anticipated due to its rapidly growing utilization.
References
1. https://www.emcdda.europa.eu/publications/rapid-communication/recreation...
2. Ernst LD et al. Longitudinally Extensive Myelopathy With Novel Radiographics Features. JAMA Neurology. 2015 Nov;72(11):1370-1
3. Amir Garakani et Al. Neurologic, psychiatric, and other medical manifestations of nitrous oxide abuse: A systematic review of the case literature. Am J Addict. 2016 Aug;25(5):358-69.
The authors of this article would like to apologise for omitting a reference to The Northwick Park Integrated Care Pathway (ICP) for Management of Hemiplegic Shoulder Pain. Further information about the pathway, its tools and ongoing development, may be found here (https://www.kcl.ac.uk/cicelysaunders/resources/management-of-hemiplegic-...) and in the following 2 citations:
D. Jackson, L. Turner-Stokes, A. Khatoon, H. Stern, L. Knight & A. O'Connell (2002). Development of an integrated care pathway for the management of hemiplegic shoulder pain, Disability and Rehabilitation, 24:7, 390-398,
Jackson D, Turner-Stokes L, Williams H, Das-Gupta R. Use of an integrated care pathway: a third-round audit of the management of shoulder pain in neurological conditions. J Rehabil Med. 2003 Nov;35(6):265-70.
I was interested to read the article by Andrew Larner in Practical Neurology suggesting that listening for orbital bruits is “useful for impressing students but is not very rewarding”.
I had not heard of Charles Warlow’s challenge before.
My mentor as a trainee, Bernard Gilligan, would routinely listen to carotids and orbits and often femoral arteries as well - we saw a lot of large vessel arterial disease in those days – and I got into the habit of auscultating at least carotids and orbits.
It paid off at least once: I enclose extracts from a letter I wrote in 2001.
Thank you for referring this most interesting 30 year old lady. She has had migraines from her early twenties. Initially they were quite occasional but severe when they occurred. They tended to be triggered by exams or stress.
She moved to Australia about 3 years ago and since then has had unusual episodes of bouts of migraine. In the first of these she had migraine every day for a week or so. Since then the attacks seem to be becoming more frequent and more prolonged so that the most recent one which finished about 2 weeks ago had lasted for 3 weeks or more.
Typically at the start of a bout she will have aura symptoms consisting of numbness of the left finger, arm and face and blurred vision in the left visual field. In the early days of a bout there may be no headache following this. However then a pattern establishes with headache occurring on a virtually...
I was interested to read the article by Andrew Larner in Practical Neurology suggesting that listening for orbital bruits is “useful for impressing students but is not very rewarding”.
I had not heard of Charles Warlow’s challenge before.
My mentor as a trainee, Bernard Gilligan, would routinely listen to carotids and orbits and often femoral arteries as well - we saw a lot of large vessel arterial disease in those days – and I got into the habit of auscultating at least carotids and orbits.
It paid off at least once: I enclose extracts from a letter I wrote in 2001.
Thank you for referring this most interesting 30 year old lady. She has had migraines from her early twenties. Initially they were quite occasional but severe when they occurred. They tended to be triggered by exams or stress.
She moved to Australia about 3 years ago and since then has had unusual episodes of bouts of migraine. In the first of these she had migraine every day for a week or so. Since then the attacks seem to be becoming more frequent and more prolonged so that the most recent one which finished about 2 weeks ago had lasted for 3 weeks or more.
Typically at the start of a bout she will have aura symptoms consisting of numbness of the left finger, arm and face and blurred vision in the left visual field. In the early days of a bout there may be no headache following this. However then a pattern establishes with headache occurring on a virtually daily basis. For example in the middle of a bout typically an attack will start with visual blurring in the left field. This has a shimmering quality and would stay there for 30 to 60 minutes. The headache then comes on while the vision is still abnormal. It would be right sided always involving forehead, temple and neck. She uses Panadol and Naramig and the headache usually settles within 4 hours. Sleeping helps. After that she can function but the headache will come back again in 12 to 24 hours. Towards the end of the bout the headaches become milder but the visual episodes may still occur for a few days until they also settle.
She has been taking Inderal during the bouts but it is not clear whether this is helping at all.
She has had no other illness. In the family her mother gets migraine every week or two but there is no aura.
Blood pressure was 120/70. There was a long loud right carotid bruit which extended into diastole. There was probably in addition a soft right orbital bruit. Fundi, cranial nerves and neurological examination were normal.
The history is certainly unusual. Migraine usually doesn’t occur in bouts like this. The finding of the prominent right carotid bruit made me concerned that she may have a right cerebral arterio-venous malformation and I thought because of the crescendo nature of her episodes we ought to get this clarified quickly. I have therefore arranged for her to have an MRI brain and MRA later this afternoon.
MRI and MRA did indeed demonstrate an AVM in the right occipital lobe and this was successfully excised. The patient still gets migraines and still comes to see me.
A couple of other patients with AVMs have had orbital bruits but this was recognised after they had been diagnosed already. They also had long carotid bruits and I think that hearing a carotid bruit extending into diastole might be a reasonable basis for spending 10 seconds on listening for an orbital bruit. However, I recognise that carotid auscultation is now also going out of favour.
In their review on vision loss in giant cell arteritis (GCA), the authors point out that anterior arteritic ischaemic optic neuropathy is the most common ophthalmic manifestation of the disease, followed by central retinal artery or cilioretinal artery occlusion and posterior ischaemic optic neuropathy. We do agree that these ocular findings must be quickly diagnosed to prevent devastating visual consequences. When diagnosed, however, not much can be done to restore the visual function. We rather believe that more emphasis should be given to other ophthalmic features that can occur as the initial manifestation of GCA and, when unrecognized, can result in an unfortunate late diagnosis. These include amaurosis fugax, uveitis, anterior or posterior scleritis and ocular pain. Awareness of these uncommon ocular conditions as the presenting signs of impending visual loss in GCA should prompt clinicians to consider this diagnosis in any elderly patient presenting with ophthalmic manifestations other than permanent visual loss, especially if other signs of GCA are detectable.
I read with great interest the excellent paper by Kumta and colleagues1 and the accompanying editorial2 concerning a patient with severe Guillain-Barré syndrome (GBS) requiring mechanical ventilation, who provided his experiences during the 31-day period of locked-in syndrome. I wish to open up some insights on the reported pain.
Physical therapy, in particular the passive stretching of calf and masseter muscles, provoked him intense pain, the patient’s description being very impressive: “It felt like they were trying to tear the muscles off the bone … I’ve never known pain like it” 1. Pain due to peripheral nerve damage is basically divided into two major types: dysesthetic pain and nerve trunk pain, the distinction between them being based upon clinical observation and experience3. Reported aching characteristics in the current patient (excruciating, deep, and provoked by nerve stretch) points to nerve trunk pain.
Pain is an integral manifestation of GBS. In a series of 55 consecutive GBS patients, Moulin and colleagues analysed pain features4, which could be summarised as follows: i/ 49 (89%) patients described pain during the course of their illness; ii/ in around half of them, it was described as excruciating; and iii/ pain preceded weakness by a mean of 5.3 days in 14, and both symptoms appeared simultaneously in seven. Furthermore, in “pure” motor GBS syndromes, usually classified as acute motor axonal neuropathy (AMAN), nerve trunk pain may occur in a h...
I read with great interest the excellent paper by Kumta and colleagues1 and the accompanying editorial2 concerning a patient with severe Guillain-Barré syndrome (GBS) requiring mechanical ventilation, who provided his experiences during the 31-day period of locked-in syndrome. I wish to open up some insights on the reported pain.
Physical therapy, in particular the passive stretching of calf and masseter muscles, provoked him intense pain, the patient’s description being very impressive: “It felt like they were trying to tear the muscles off the bone … I’ve never known pain like it” 1. Pain due to peripheral nerve damage is basically divided into two major types: dysesthetic pain and nerve trunk pain, the distinction between them being based upon clinical observation and experience3. Reported aching characteristics in the current patient (excruciating, deep, and provoked by nerve stretch) points to nerve trunk pain.
Pain is an integral manifestation of GBS. In a series of 55 consecutive GBS patients, Moulin and colleagues analysed pain features4, which could be summarised as follows: i/ 49 (89%) patients described pain during the course of their illness; ii/ in around half of them, it was described as excruciating; and iii/ pain preceded weakness by a mean of 5.3 days in 14, and both symptoms appeared simultaneously in seven. Furthermore, in “pure” motor GBS syndromes, usually classified as acute motor axonal neuropathy (AMAN), nerve trunk pain may occur in a high proportion of patients5, 6. In this regard, it is worth recalling that pain was an outstanding feature in the original AMAN description7: “many patients had neck and back stiffness and pain, one father said his son seemed as though he had a rod up his spine”. The corollary is that in any locked-in GBS, each healthcare assistant should assume the high likelihood of being confronted with a patient that may be suffering from nerve trunk pain, either spontaneous or provoked.
The mechanism of pain in early stages of GBS (≤ 10 days after onset) is controversial4, 5; I shall offer a brief explanation. Pathological changes in early GBS mainly consist of inflammatory oedema predominating in the region where motor and sensory roots join to form spinal nerves8-10. Such lesional predominance is correlated with less efficient blood-nerve barrier of proximal nerve trunks9. Spinal roots traverse the subarachnoid space surrounded by an elastic multicellular root sheath derived from the arachnoid and penetrate the subarachnoid angle; external to this angle, the newly formed spinal nerves possess epi-perineurium and endoneurium as in the peripheral nerve trunks (see figure 3 in reference 9). The absence of epi-perineurium in the spinal roots probably prevents their having an increase of endoneurial fluid pressure (EFP) and ischemic injury despite inflammatory changes. Perineurium is relatively inelastic and has only a limited ability to expand9. Small increases of EFP, caused by endoneurial inflammation, can be accommodated, but any increase beyond these limits, as presumably occurs in early GBS, will produce an increase in EFP leading to compromise of transperineurial microcirculation and endoneurial ischemia, which causes proximal nerve conduction failure and eventually nerve trunk pain. In other words, in early severe GBS widespread inflammation of spinal nerves might account for neuropathic pain, indistinguishable from that associated with multilevel spinal root compression. In AMAN extension of ventral root inflammatory oedema into ventral rami of spinal nerves could involve abutting dorsal rami, thus causing nerve trunk pain referred to their innervation territories, from neck to buttocks, eventually accompanied by neck and back stiffness (masterfully illustrated in figure 1 of reference 7).
Treatment of nerve trunk pain in GBS may be challenging, given that in addition to conventional non-steroidal anti-inflammatory drugs or simple analgesics, up to 74% of cases require opioids analgesics or even parental morphine9. The pathogenic role of inflammatory oedema in early GBS is the rationale for using short courses of intravenous corticosteroids; in fact, there have been at least 13 well-documented GBS patients with severe backache, unresponsive to analgesics, who exhibited rapid response to steroids9.
The touching story of the current patient teaches us how distressing may be painful sensation in a patient suffering from locked-in GBS. As timely indicated by the authors, this calls for several approaches to minimize the “living nightmare”1. Concerning nerve trunk pain such approach should comprise the following measures: i/ optimal analgesic regimens including the potential use of short courses of intravenous corticosteroids; ii/ if the patient is not completely sedated, nerve traction maneuvers should be avoided during physical therapy; and iii/ whenever possible, approach the patient asking about pain.
References
1. Kumta N, Carter A, Schuller P, et al. Locked-in Guillain-Barré syndrome: 'my living nightmare'. Pract Neurol. Epub ahead of print: 2021 Oct 29:practneurol-2021-003110. doi: 10.1136/practneurol-2021-003110.
2. Law D, Morgan M Listening to patients in intensive care. Epub ahead of print: Pract Neurol. 2021 Nov 29:practneurol-2021-003255. doi: 10.1136/practneurol-2021-003255.
3. Asbury AK, Fields HL. Pain due to peripheral nerve damage: an hypothesis. Neurology 1984; 34: 1587-90.
4. Moulin DE, Hagen N, Feasby TE, et al. Pain in Guillain-Barré syndrome. Neurology 1997; 48: 328-31.
5. Ruts L, Rico R, van Koningsveld R, et al. Pain accompanies pure motor Guillain-Barré syndrome. J Peripher Nerv Syst 2008; 13: 305-6.
6. Zhao F, Wang J, Zhang J, et al. Pain in acute motor axonal neuropathy. Muscle Nerve 2021; 64:739-43.
7. McKhann GM, Cornblath DR, Ho T, et al. Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet 1991; 338: 593-7.
8. Haymaker WE, Kernohan JW. The Landry-Guillain-Barré syndrome; a clinicopathologic report of 50 fatal cases and a critique of the literature. Medicine (Baltimore) 1949; 28: 59-141.
9. Berciano J, Sedano MJ, Pelayo-Negro AL, et al. Proximal nerve lesions in early Guillain-Barré syndrome: implications for pathogenesis and disease classification. J Neurol 2017; 264: 221-36.
10. Berciano J. Axonal degeneration in Guillain-Barré syndrome: a reappraisal. J Neurol 2021; 268: 3728-43.
Your artclce mentions phenytoin as a treatment that has been tried in open label studies. When I began as a neurologist I'd not heard of its use but was put on to it by a senior colleague. I do use carbamazepine as first line but have found phenytoin far superior to other second line agents. I draw the interested reader to the excellent article by Hellelink and Schatman (J Pain Res. 2017; 10: 1663-6 doi: 10.2147/JPR.S141896) which puts a case for phenytoin being considered first line.
Although Rowe and colleagues make it clear that the most important purpose of their review is to cover the neglected aspect of management of Progressive Supranuclear Palsy (1) they also describe the disorder’s clinical symptoms and signs and its differential diagnosis in some detail. In this section of the article they fail to mention two simple bedside tests which I have found particularly useful over the years in making me more certain about my diagnosis. In those patients where Parkinson’s disease is the main differential diagnosis sequential finger tapping for 20 seconds is frequently informative. A progressive reduction in speed and amplitude ( ‘the sequence effect’) is a sine qua non for Parkinson’s disease but is rarely found in Progressive Supranuclear Palsy where marked reduction in amplitude of finger taps without decrement and preserved speed of movement is much more usual (2). Utilisation and imitation behaviour (3, 4) and the applause sign (5), all indicative of prefrontal lobe dysfunction, are very common in fully established Richardson’s syndrome but I have also found them to be sometimes present early on particularly when abulia or behavioural abnormalities are presenting complaints. The grasp reflex on the other hand is not commonly observed
The diagnosis of Progressive Supranuclear Palsy is difficult and uncertainty is common particularly in the early stages. To retract a diagnosis of Progressive Supranuclear Palsy is tri...
Although Rowe and colleagues make it clear that the most important purpose of their review is to cover the neglected aspect of management of Progressive Supranuclear Palsy (1) they also describe the disorder’s clinical symptoms and signs and its differential diagnosis in some detail. In this section of the article they fail to mention two simple bedside tests which I have found particularly useful over the years in making me more certain about my diagnosis. In those patients where Parkinson’s disease is the main differential diagnosis sequential finger tapping for 20 seconds is frequently informative. A progressive reduction in speed and amplitude ( ‘the sequence effect’) is a sine qua non for Parkinson’s disease but is rarely found in Progressive Supranuclear Palsy where marked reduction in amplitude of finger taps without decrement and preserved speed of movement is much more usual (2). Utilisation and imitation behaviour (3, 4) and the applause sign (5), all indicative of prefrontal lobe dysfunction, are very common in fully established Richardson’s syndrome but I have also found them to be sometimes present early on particularly when abulia or behavioural abnormalities are presenting complaints. The grasp reflex on the other hand is not commonly observed
The diagnosis of Progressive Supranuclear Palsy is difficult and uncertainty is common particularly in the early stages. To retract a diagnosis of Progressive Supranuclear Palsy is tricky and there are many instances when it is prudent to temporarily withhold judgement until the clinical picture becomes clearer.
References
1. Rowe JB, Holland N, Rittman T. Progressive supranuclear palsy: diagnosis and management. Pract Neurol. 2021;21(5):376-83.
2. Ling H, Massey L, Lees A, Brown P, Day B. Hypokinesia without decrement distinguishes progressive supranuclear palsy from Parkinson's disease. Movement Disorders. 2012;27:S384-S5.
3. Lhermitte F, Pillon B, Serdaru M. Human autonomy and the frontal lobes. Part I: Imitation and utilization behavior: a neuropsychological study of 75 patients. Ann Neurol. 1986;19(4):326-34.
4. Lhermitte F. Human autonomy and the frontal lobes. Part II: Patient behavior in complex and social situations: the "environmental dependency syndrome". Ann Neurol. 1986;19(4):335-43.
5. Dubois B, Slachevsky A, Pillon B, Beato R, Villalponda JM, Litvan I. "Applause sign" helps to discriminate PSP from FTD and PD. Neurology. 2005;64(12):2132-3.
We agree with Andrew Lees on the value of the applause sign (i.e. ask a PSP patient to clap 3 times, and they typically perseverative by clapping more than 3 times) and a lack of decrement on hypokinesia tests (e.g. sequential finger tapping or in micrographic writing, without a progressive reduction in amplitude) to differentiate Progressive Supranuclear Palsy from Parkinson’s disease. Both are very useful, easy bedside tests.
We also agree that there are cases where one is unsure. A diagnosis of Progressive Supranuclear Palsy is not to be given lightly, as for any other disease. But, patients with Progressive Supranuclear Palsy have suffered disproportionately from delays and diagnostic hesitation, often long past any reasonable doubt. It does not help a patient or family to live with a wrong diagnosis, or no diagnosis, for a large part of their illness. Across many countries, the diagnosis of PSP is made at around three years from symptom onset (Murley et al, 2021; Cosseddu et al 2017; Mamarabadi et al, 2018). In other words, halfway from onset to death. This is unnecessarily long. Greater awareness and simple clinical skills can reduce the diagnostic delay by more than a year (Mamarabadi et al, 2018). Through our article, we hope our colleagues can become more confident, early and accurate in making the diagnosis of PSP as a gateway to better care.
We agree with Andrew Lees on the value of the applause sign (i.e. ask a PSP patient to clap 3 times, and they typically perseverative by clapping more than 3 times) and a lack of decrement on hypokinesia tests (e.g. sequential finger tapping or in micrographic writing, without a progressive reduction in amplitude) to differentiate Progressive Supranuclear Palsy from Parkinson’s disease. Both are very useful, easy bedside tests.
We also agree that there are cases where one is unsure. A diagnosis of Progressive Supranuclear Palsy is not to be given lightly, as for any other disease. But, patients with Progressive Supranuclear Palsy have suffered disproportionately from delays and diagnostic hesitation, often long past any reasonable doubt. It does not help a patient or family to live with a wrong diagnosis, or no diagnosis, for a large part of their illness. Across many countries, the diagnosis of PSP is made at around three years from symptom onset (Murley et al, 2021; Cosseddu et al 2017; Mamarabadi et al, 2018). In other words, halfway from onset to death. This is unnecessarily long. Greater awareness and simple clinical skills can reduce the diagnostic delay by more than a year (Mamarabadi et al, 2018). Through our article, we hope our colleagues can become more confident, early and accurate in making the diagnosis of PSP as a gateway to better care.
James Rowe, Tim Rittman and Negin Holland
References
Mamarabadi M, Razjouyan H, Golbe LI. Is the Latency from Progressive Supranuclear Palsy Onset to Diagnosis Improving? Mov Disord Clin Pract. 2018 Nov 8;5(6):603-606. doi: 10.1002/mdc3.12678. PMID: 30637280; PMCID: PMC6277372.
Murley AG, Rouse MA, Coyle-Gilchrist ITS, Jones PS, Li W, Wiggins J, Lansdall C, Vázquez Rodríguez P, Wilcox A, Patterson K, Rowe JB. Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes. J Neurol Neurosurg Psychiatry. 2021 Jul;92(7):737-744. doi: 10.1136/jnnp-2020-324903. Epub 2021 Feb 9. PMID: 33563798; PMCID: PMC8223632.
Cosseddu M, Benussi A, Gazzina S, Manes MA, Dell'Era V, Cristillo V, Turrone R, Alberici A, Borroni B. Natural history and predictors of survival in progressive supranuclear palsy. J Neurol Sci. 2017 Nov 15;382:105-107. doi: 10.1016/j.jns.2017.09.043. Epub 2017 Sep 30. PMID: 29111000.
I enjoyed this article on this important area of non-traumatic non-aneurysmal convexity SAH. The authors seems to have not recognised the literature that points to convexity/Sulcal subarachnoid haemorrhage in the context of ipsilateral carotid artery stenosis, which in some case series make up a significant proprtion of the cases (10-50%, references below). Also I would be cautious about having dichotomoised age as a rule, but better as a guide. In the case of carotid artery stenosis this is seen in the under 60s sometimes.
Geraldes et al J Stroke Cereb Dis 23:e23-30, 2014.
Zhao et al J Int Med Res 2017 45:1870.
Renou et al 2012 Cerebrovasc Dis. 2012;34(2):147-52.
Forman et al; J Stroke Cerebrovasc Dis. 2019;28(12):104473.
Amantadine is not a N-metyhl-d-aspartate (NMDA) receptor agonist as stated in the paper, as it's a NMDA antagonist.
References:
- Vanle B, Olcott W, Jimenez J, Bashmi L, Danovitch I, IsHak WW. NMDA antagonists for treating the non-motor symptoms in Parkinson's disease. Transl Psychiatry. 2018 Jun 15;8(1):117. doi: 10.1038/s41398-018-0162-2. PMID: 29907742; PMCID: PMC6003962.,
- Kornhuber J, Weller M, Schoppmeyer K, Riederer P. Amantadine and memantine are NMDA receptor antagonists with neuroprotective properties. J Neural Transm Suppl. 1994;43:91-104. PMID: 7884411.
Note from the editors:
an erratum will be published
I read the article by Alvar Paris et al. with great interest. The consumption of nitrous oxide (N2O) has recently surfaced as an exponentially growing form of recreational abuse, particularly among students, due to its euphoric effects and accessibility. The European Monitoring Centre for Drugs and Drug Addiction has expressed concern in a recent report about the recreational use of N2O in Europe. 1
Show MoreThe most common neurological complications associated with N2O consumption are toxic polyneuropathy and subacute combined degeneration of the spinal cord (N2O-SACD), which may occur concurrently— a critical aspect that should not be overlooked. Initial clinical descriptions of N2O-SACD were reported in 2015 and complicated episodic use mediated through canisters. 2 Recently, the market for N2O distribution has expanded with diverse consumption accessories. The introduction of larger cylinders facilitates the consumption of significant amounts of N2O, posing younger individuals at an elevated risk of early and severe Vitamin B12 deficiency along with systemic complications. Consequently, new complications associated with N2O intoxication have been described, and their wide-ranging effects are regularly emphasized in the literature.
Beyond psychiatric symptoms like hallucinations, cognitive impairments including Gayet-Wernicke's encephalopathy have been recently reported. 3 These deficiencies are mediated by Vitamin B1 levels, suggesting a risk of broader deficie...
The authors of this article would like to apologise for omitting a reference to The Northwick Park Integrated Care Pathway (ICP) for Management of Hemiplegic Shoulder Pain. Further information about the pathway, its tools and ongoing development, may be found here (https://www.kcl.ac.uk/cicelysaunders/resources/management-of-hemiplegic-...) and in the following 2 citations:
D. Jackson, L. Turner-Stokes, A. Khatoon, H. Stern, L. Knight & A. O'Connell (2002). Development of an integrated care pathway for the management of hemiplegic shoulder pain, Disability and Rehabilitation, 24:7, 390-398,
Jackson D, Turner-Stokes L, Williams H, Das-Gupta R. Use of an integrated care pathway: a third-round audit of the management of shoulder pain in neurological conditions. J Rehabil Med. 2003 Nov;35(6):265-70.
I was interested to read the article by Andrew Larner in Practical Neurology suggesting that listening for orbital bruits is “useful for impressing students but is not very rewarding”.
I had not heard of Charles Warlow’s challenge before.
My mentor as a trainee, Bernard Gilligan, would routinely listen to carotids and orbits and often femoral arteries as well - we saw a lot of large vessel arterial disease in those days – and I got into the habit of auscultating at least carotids and orbits.
It paid off at least once: I enclose extracts from a letter I wrote in 2001.
Thank you for referring this most interesting 30 year old lady. She has had migraines from her early twenties. Initially they were quite occasional but severe when they occurred. They tended to be triggered by exams or stress.
She moved to Australia about 3 years ago and since then has had unusual episodes of bouts of migraine. In the first of these she had migraine every day for a week or so. Since then the attacks seem to be becoming more frequent and more prolonged so that the most recent one which finished about 2 weeks ago had lasted for 3 weeks or more.
Typically at the start of a bout she will have aura symptoms consisting of numbness of the left finger, arm and face and blurred vision in the left visual field. In the early days of a bout there may be no headache following this. However then a pattern establishes with headache occurring on a virtually...
Show MoreIn their review on vision loss in giant cell arteritis (GCA), the authors point out that anterior arteritic ischaemic optic neuropathy is the most common ophthalmic manifestation of the disease, followed by central retinal artery or cilioretinal artery occlusion and posterior ischaemic optic neuropathy. We do agree that these ocular findings must be quickly diagnosed to prevent devastating visual consequences. When diagnosed, however, not much can be done to restore the visual function. We rather believe that more emphasis should be given to other ophthalmic features that can occur as the initial manifestation of GCA and, when unrecognized, can result in an unfortunate late diagnosis. These include amaurosis fugax, uveitis, anterior or posterior scleritis and ocular pain. Awareness of these uncommon ocular conditions as the presenting signs of impending visual loss in GCA should prompt clinicians to consider this diagnosis in any elderly patient presenting with ophthalmic manifestations other than permanent visual loss, especially if other signs of GCA are detectable.
I read with great interest the excellent paper by Kumta and colleagues1 and the accompanying editorial2 concerning a patient with severe Guillain-Barré syndrome (GBS) requiring mechanical ventilation, who provided his experiences during the 31-day period of locked-in syndrome. I wish to open up some insights on the reported pain.
Show MorePhysical therapy, in particular the passive stretching of calf and masseter muscles, provoked him intense pain, the patient’s description being very impressive: “It felt like they were trying to tear the muscles off the bone … I’ve never known pain like it” 1. Pain due to peripheral nerve damage is basically divided into two major types: dysesthetic pain and nerve trunk pain, the distinction between them being based upon clinical observation and experience3. Reported aching characteristics in the current patient (excruciating, deep, and provoked by nerve stretch) points to nerve trunk pain.
Pain is an integral manifestation of GBS. In a series of 55 consecutive GBS patients, Moulin and colleagues analysed pain features4, which could be summarised as follows: i/ 49 (89%) patients described pain during the course of their illness; ii/ in around half of them, it was described as excruciating; and iii/ pain preceded weakness by a mean of 5.3 days in 14, and both symptoms appeared simultaneously in seven. Furthermore, in “pure” motor GBS syndromes, usually classified as acute motor axonal neuropathy (AMAN), nerve trunk pain may occur in a h...
Your artclce mentions phenytoin as a treatment that has been tried in open label studies. When I began as a neurologist I'd not heard of its use but was put on to it by a senior colleague. I do use carbamazepine as first line but have found phenytoin far superior to other second line agents. I draw the interested reader to the excellent article by Hellelink and Schatman (J Pain Res. 2017; 10: 1663-6 doi: 10.2147/JPR.S141896) which puts a case for phenytoin being considered first line.
To the editor
Although Rowe and colleagues make it clear that the most important purpose of their review is to cover the neglected aspect of management of Progressive Supranuclear Palsy (1) they also describe the disorder’s clinical symptoms and signs and its differential diagnosis in some detail. In this section of the article they fail to mention two simple bedside tests which I have found particularly useful over the years in making me more certain about my diagnosis. In those patients where Parkinson’s disease is the main differential diagnosis sequential finger tapping for 20 seconds is frequently informative. A progressive reduction in speed and amplitude ( ‘the sequence effect’) is a sine qua non for Parkinson’s disease but is rarely found in Progressive Supranuclear Palsy where marked reduction in amplitude of finger taps without decrement and preserved speed of movement is much more usual (2). Utilisation and imitation behaviour (3, 4) and the applause sign (5), all indicative of prefrontal lobe dysfunction, are very common in fully established Richardson’s syndrome but I have also found them to be sometimes present early on particularly when abulia or behavioural abnormalities are presenting complaints. The grasp reflex on the other hand is not commonly observed
Show MoreThe diagnosis of Progressive Supranuclear Palsy is difficult and uncertainty is common particularly in the early stages. To retract a diagnosis of Progressive Supranuclear Palsy is tri...
To the Editor,
We agree with Andrew Lees on the value of the applause sign (i.e. ask a PSP patient to clap 3 times, and they typically perseverative by clapping more than 3 times) and a lack of decrement on hypokinesia tests (e.g. sequential finger tapping or in micrographic writing, without a progressive reduction in amplitude) to differentiate Progressive Supranuclear Palsy from Parkinson’s disease. Both are very useful, easy bedside tests.
We also agree that there are cases where one is unsure. A diagnosis of Progressive Supranuclear Palsy is not to be given lightly, as for any other disease. But, patients with Progressive Supranuclear Palsy have suffered disproportionately from delays and diagnostic hesitation, often long past any reasonable doubt. It does not help a patient or family to live with a wrong diagnosis, or no diagnosis, for a large part of their illness. Across many countries, the diagnosis of PSP is made at around three years from symptom onset (Murley et al, 2021; Cosseddu et al 2017; Mamarabadi et al, 2018). In other words, halfway from onset to death. This is unnecessarily long. Greater awareness and simple clinical skills can reduce the diagnostic delay by more than a year (Mamarabadi et al, 2018). Through our article, we hope our colleagues can become more confident, early and accurate in making the diagnosis of PSP as a gateway to better care.
James Rowe, Tim Rittman and Negin Holland
References
Mamarabadi M, R...
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