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 read your article (Neurology and clinical neurophysiology: an artificial divide. Kieran MC. Pract Neurol 2021;21:274-275) with interest.
Clinical neurophysiology is primarily a diagnostic specialty concerned with recording electrical activity from the nervous system to aid the diagnosis, classification and management of neurological disease (clinical neurophysiology curriculum: www.jrcptb.org.uk/specialties/c;inical-neurophysiology). One of the attractions of the specialty is precisely this: the breadth of the specialty and the interaction with a wide variety of specialties. Although nerve conduction studies (NCS) and electromyography (EMG) form a big part of our work, most clinical neurophysiologists, at least in the UK do a lot more. We record and report electroencephalography (EEG) studies including long term recordings and video EEG telemetry, a variety of evoked potential studies (EPs), electroretinography (ERG) studies and sleep studies, among others. The specialty is about more than NCS and EMG. Even within the area of NCS/ EMG, although a proportion of our referrals are from neurology teams, we frequently see patients referred by orthopaedic, oncology and rheumatology teams.
I suppose there could be a world in which neurologists perform NCS / EMG as well as EPs, EEGs, sleep studies on all their patients; hand surgeons and shoulder specialists perform NCS /EMG on their patients...
I read your article (Neurology and clinical neurophysiology: an artificial divide. Kieran MC. Pract Neurol 2021;21:274-275) with interest.
Clinical neurophysiology is primarily a diagnostic specialty concerned with recording electrical activity from the nervous system to aid the diagnosis, classification and management of neurological disease (clinical neurophysiology curriculum: www.jrcptb.org.uk/specialties/c;inical-neurophysiology). One of the attractions of the specialty is precisely this: the breadth of the specialty and the interaction with a wide variety of specialties. Although nerve conduction studies (NCS) and electromyography (EMG) form a big part of our work, most clinical neurophysiologists, at least in the UK do a lot more. We record and report electroencephalography (EEG) studies including long term recordings and video EEG telemetry, a variety of evoked potential studies (EPs), electroretinography (ERG) studies and sleep studies, among others. The specialty is about more than NCS and EMG. Even within the area of NCS/ EMG, although a proportion of our referrals are from neurology teams, we frequently see patients referred by orthopaedic, oncology and rheumatology teams.
I suppose there could be a world in which neurologists perform NCS / EMG as well as EPs, EEGs, sleep studies on all their patients; hand surgeons and shoulder specialists perform NCS /EMG on their patients with entrapment neuropathies and brachial plexopathies; hip surgeons assess electrodiagnostically their patients with leg weakness following hip replacement; ophthalmologists record and report electroretinographies in patients with possible degenerative retinal diseases and paediatricians record and report EEGs in children presenting with episodes likely to be seizures.
I agree that neurophysiological investigations are extensions of the clinical assessments of patients and the findings should always be interpreted within the clinical context. Clinical neurophysiologists should receive rigorous training, not only in the technical aspects of the specialty but also in the clinical areas (neurology, adult and paediatric epilepsy, ophthalmology, psychiatry etc. as well as in the art of communication with the different referring specialties). The clinical neurophysiologists and the referring clinicians need to work together and communicate clearly about the needs of the patients and the clinical and electrophysiological findings. It is in the patients’ best interests that we work together in functioning multidisciplinary teams.
I think modern medicine has a place for well-trained neurologists and competent and enthusiastic clinical neurophysiologists (and our clinical physiologist colleagues) to work side by side to deliver a quality service to our patients.
We were delighted to read the article on orbital myositis heralding herpes zoster ophthalmicus (HZO) [1], highlighting the importance of considering an underlying infectious aetiology in cases of painful complex ophthalmoplegia.
Infections are a common cause of neurological complications in our setting, although unique cases continue to remind us about broader differentials. We encountered a case of a 55-year-old female with an unremarkable past medical history who was on holiday in Mombasa but presented to a local ophthalmologist with acute-onset diplopia and a painful swollen left eye. She was commenced on oral and topical antibiotics for a presumed pre-septal orbital cellulitis, but did not improve so was transferred urgently to our tertiary regional referral centre in Nairobi. Further social and travel history were non-revealing. On physical examination her blood pressure and temperature were normal, and she had no signs of thyroid disease. On full neurological examination she only had a left lateral rectus palsy with no other signs, and mild left peri-orbital swelling with erythema.
Comprehensive investigations (including metabolic, inflammatory, auto-immune, vasculitic and infective blood tests panels) as well as magnetic resonance imaging (MRI) of the brain with venography and angiography were all normal except for modestly raised C-reactive protein of 13 mg/dL (normal range 0-5). MRI of the orbits revealed enlargement of the left lateral rectus with...
We were delighted to read the article on orbital myositis heralding herpes zoster ophthalmicus (HZO) [1], highlighting the importance of considering an underlying infectious aetiology in cases of painful complex ophthalmoplegia.
Infections are a common cause of neurological complications in our setting, although unique cases continue to remind us about broader differentials. We encountered a case of a 55-year-old female with an unremarkable past medical history who was on holiday in Mombasa but presented to a local ophthalmologist with acute-onset diplopia and a painful swollen left eye. She was commenced on oral and topical antibiotics for a presumed pre-septal orbital cellulitis, but did not improve so was transferred urgently to our tertiary regional referral centre in Nairobi. Further social and travel history were non-revealing. On physical examination her blood pressure and temperature were normal, and she had no signs of thyroid disease. On full neurological examination she only had a left lateral rectus palsy with no other signs, and mild left peri-orbital swelling with erythema.
Comprehensive investigations (including metabolic, inflammatory, auto-immune, vasculitic and infective blood tests panels) as well as magnetic resonance imaging (MRI) of the brain with venography and angiography were all normal except for modestly raised C-reactive protein of 13 mg/dL (normal range 0-5). MRI of the orbits revealed enlargement of the left lateral rectus with contrast enhancement. We diagnosed idiopathic orbital myositis (IOM) and commenced oral corticosteroids which resulted in marked improvement within 48 hours.
Our case highlights that IOM can present as orbital cellulitis [2], although the latter diagnosis is more serious and needs to be recognised and treated early. Ophthalmoplegia can be due to myositis of single or multiple extra-ocular muscles as our case and the HZO case illustrate, although there are reports of lateral rectus IOM occurring without any of the other signs of orbital inflammation [3]. In the absence of other clinical and laboratory markers to support cellulitis, causes of orbital myositis need to be considered e.g. auto-immune disease, primary infections of the muscle including neuroborreliosis [4], or post-infectious sequelae even including after COVID-19 infection [5].
We hope our case adds further diagnostic considerations to those wonderfully illustrated by our colleague when confronted with a patient complaining of acute-onset painful diplopia.
REFERENCES
[1] Chen T. Orbital myositis with herpes zoster ophthalmicus. Practical Neurology. Published Online First: 28 January 2021. doi: 10.1136/practneurol-2020-002870
[2] Lee NC, Loyal J, Berkwitt A. More Than Meets the Eye: Idiopathic Orbital Inflammation Mimicking Orbital Cellulitis. Cureus. 2021 Jan 12;13(1):e12655. doi: 10.7759/cureus.12655
[3] Wazir M, Faisal-Uddin M, Tambunan D, Jain AG. Idiopathic Lateral Rectus Myositis Without Signs of Orbital Inflammation. Cureus. 2019 Jun 7;11(6):e4859. doi: 10.7759/cureus.4859. PMID: 31410342; PMCID: PMC6684302.
[4] Mangan MS, Yildiz E. New Onset of Unilateral Orbital Myositis following Mild COVID-19 Infection. Ocular Immunology and Inflammation. Published Online First: 02 April 2021. doi: 10.1080/09273948.2021.1887282
[5] McNab AA. Orbital Myositis: A Comprehensive Review and Reclassification. Ophthalmic Plast Reconstr Surg, 2020; 36(2):109-117
Dr Gowell and his colleagues, in their detailed and vivid description of a young-onset case of frontotemporal dementia (FTD) associated with FUS pathology, have missed a trick. They point out the frontal atrophy that is evident in the patient’s brain MRI (figure 1), but have not commented on the bilateral caudate head atrophy that is also present. This is best appreciated in the third image from the left in the upper row of images (coronal, T1-weighted). This MRI characteristic is a tell-tale sign in FUS-associated FTD (ref). The differential for the presence of caudate head atrophy in brain MRI scans is not wide (also present in Huntington’s disease and neuroacathocytosis), so its presence in the context of progressive dementia with predominant frontal lobe dysfunction in the absence of movement disorder should alert the clinician to this possibility. Having a “second look” at the imaging is always worth doing!
Ref.
Josephs KA, Whitwell JL, Parisi JE, et al. Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Eur J Neurol 2010;17(7):969-975.
Comment on: Nathoo N, Naik S, Rempel J, et al. Superficial siderosis treated with dural tear repair and deferiprone. Pract Neurol 2021;21(1):71-72.
Dear Colleagues
Concerning “superficial siderosis” I would like to emphasize that we have to distinguish two different types regarding localization and pathophysiology: 1) The infratentorial type that was described in the case report [1]. Patients present with a triad of sensorineural hearing loss, ataxia and myelopathy [2]. Treatment is based on staunching recurrent bleeding and elimination of toxic iron deposits with deferiprone. The latter is not without some risk: neutropenia with sepsis [3]. – 2) The supratentorial type is a manifestation of cerebral amyloid angiopathy in the vast majority of cases [4], and there is no known cure. Cortical superficial siderosis – of the supratentorial type – is the cause of transient focal neurological episodes that can mimic transient ischaemic attacks, migraine auras or simple partial seizures [5].
Conflicts of interest and ethics
The author declares that there are no conflicts of interest, in particular none with a manufacturer of pharmaceutical products...
Comment on: Nathoo N, Naik S, Rempel J, et al. Superficial siderosis treated with dural tear repair and deferiprone. Pract Neurol 2021;21(1):71-72.
Dear Colleagues
Concerning “superficial siderosis” I would like to emphasize that we have to distinguish two different types regarding localization and pathophysiology: 1) The infratentorial type that was described in the case report [1]. Patients present with a triad of sensorineural hearing loss, ataxia and myelopathy [2]. Treatment is based on staunching recurrent bleeding and elimination of toxic iron deposits with deferiprone. The latter is not without some risk: neutropenia with sepsis [3]. – 2) The supratentorial type is a manifestation of cerebral amyloid angiopathy in the vast majority of cases [4], and there is no known cure. Cortical superficial siderosis – of the supratentorial type – is the cause of transient focal neurological episodes that can mimic transient ischaemic attacks, migraine auras or simple partial seizures [5].
Conflicts of interest and ethics
The author declares that there are no conflicts of interest, in particular none with a manufacturer of pharmaceutical products. This manuscript is not under review by another journal, and it is based on previously published work, so no new studies in humans or animals were necessary.
References
1) Nathoo N, Naik S, Rempel J, et al. Superficial siderosis treated with dural tear repair and deferiprone. Pract Neurol 2021;21(1):71-72.
2) Wilson D, Chatterjee F, Farmer SF et al. Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway. Ann Neurol 2017;81(3):333-343.
3) Sammaraiee Y, Banerjee G, Farmer S et al. Risks associated with oral deferiprone in the treatment of infratentorial superficial siderosis. J Neurol 2020;267(1):239-243.
4) Lummel N, Wollenweber FA, Demaerel P et al. Clinical spectrum, underlying etiologies and radiological characteristics of cortical superficial siderosis. J Neurol 2015;262(6):1455-1462.
5) Vales-Montero M, Garcia-Pastor A, Iglesias-Mohedano AM et al. Cerebral amyloid angiopathy-related transient focal neurological episodes: a transient ischemic attack mimic with an increased risk of intracranial hemorrhage. J Neurol Sci 2019;406:116452.
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...
Show MoreI read your article (Neurology and clinical neurophysiology: an artificial divide. Kieran MC. Pract Neurol 2021;21:274-275) with interest.
Show MoreClinical neurophysiology is primarily a diagnostic specialty concerned with recording electrical activity from the nervous system to aid the diagnosis, classification and management of neurological disease (clinical neurophysiology curriculum: www.jrcptb.org.uk/specialties/c;inical-neurophysiology). One of the attractions of the specialty is precisely this: the breadth of the specialty and the interaction with a wide variety of specialties. Although nerve conduction studies (NCS) and electromyography (EMG) form a big part of our work, most clinical neurophysiologists, at least in the UK do a lot more. We record and report electroencephalography (EEG) studies including long term recordings and video EEG telemetry, a variety of evoked potential studies (EPs), electroretinography (ERG) studies and sleep studies, among others. The specialty is about more than NCS and EMG. Even within the area of NCS/ EMG, although a proportion of our referrals are from neurology teams, we frequently see patients referred by orthopaedic, oncology and rheumatology teams.
I suppose there could be a world in which neurologists perform NCS / EMG as well as EPs, EEGs, sleep studies on all their patients; hand surgeons and shoulder specialists perform NCS /EMG on their patients...
We were delighted to read the article on orbital myositis heralding herpes zoster ophthalmicus (HZO) [1], highlighting the importance of considering an underlying infectious aetiology in cases of painful complex ophthalmoplegia.
Infections are a common cause of neurological complications in our setting, although unique cases continue to remind us about broader differentials. We encountered a case of a 55-year-old female with an unremarkable past medical history who was on holiday in Mombasa but presented to a local ophthalmologist with acute-onset diplopia and a painful swollen left eye. She was commenced on oral and topical antibiotics for a presumed pre-septal orbital cellulitis, but did not improve so was transferred urgently to our tertiary regional referral centre in Nairobi. Further social and travel history were non-revealing. On physical examination her blood pressure and temperature were normal, and she had no signs of thyroid disease. On full neurological examination she only had a left lateral rectus palsy with no other signs, and mild left peri-orbital swelling with erythema.
Comprehensive investigations (including metabolic, inflammatory, auto-immune, vasculitic and infective blood tests panels) as well as magnetic resonance imaging (MRI) of the brain with venography and angiography were all normal except for modestly raised C-reactive protein of 13 mg/dL (normal range 0-5). MRI of the orbits revealed enlargement of the left lateral rectus with...
Show MoreDr Gowell and his colleagues, in their detailed and vivid description of a young-onset case of frontotemporal dementia (FTD) associated with FUS pathology, have missed a trick. They point out the frontal atrophy that is evident in the patient’s brain MRI (figure 1), but have not commented on the bilateral caudate head atrophy that is also present. This is best appreciated in the third image from the left in the upper row of images (coronal, T1-weighted). This MRI characteristic is a tell-tale sign in FUS-associated FTD (ref). The differential for the presence of caudate head atrophy in brain MRI scans is not wide (also present in Huntington’s disease and neuroacathocytosis), so its presence in the context of progressive dementia with predominant frontal lobe dysfunction in the absence of movement disorder should alert the clinician to this possibility. Having a “second look” at the imaging is always worth doing!
Ref.
Josephs KA, Whitwell JL, Parisi JE, et al. Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Eur J Neurol 2010;17(7):969-975.
Comment on: Nathoo N, Naik S, Rempel J, et al. Superficial siderosis treated with dural tear repair and deferiprone. Pract Neurol 2021;21(1):71-72.
Dear Colleagues
Concerning “superficial siderosis” I would like to emphasize that we have to distinguish two different types regarding localization and pathophysiology: 1) The infratentorial type that was described in the case report [1]. Patients present with a triad of sensorineural hearing loss, ataxia and myelopathy [2]. Treatment is based on staunching recurrent bleeding and elimination of toxic iron deposits with deferiprone. The latter is not without some risk: neutropenia with sepsis [3]. – 2) The supratentorial type is a manifestation of cerebral amyloid angiopathy in the vast majority of cases [4], and there is no known cure. Cortical superficial siderosis – of the supratentorial type – is the cause of transient focal neurological episodes that can mimic transient ischaemic attacks, migraine auras or simple partial seizures [5].
Yours sincerely,
Daniel Eschle, MD, MSc
Consultant Neurologist
Kantonsspital Uri, 6460 Altdorf (Switzerland), Telephone: ++41 41 875 51 51
E-Mail: deschle@hotmail.com or daniel.eschle@ksuri.ch
Conflicts of interest and ethics
Show MoreThe author declares that there are no conflicts of interest, in particular none with a manufacturer of pharmaceutical products...
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