Dear Editors,
Carr AS, et al. Pract Neurol 2017;17:218–221. doi:10.1136/practneurol-2016-001491
In the interests of enhancing our understanding of bulbar function from the perspective of the jobbing clinical neurologist, I am writing to you regarding a publication in this month’s edition of Practical Neurology (Carling et al.). As it stands I do not think the case report does justice to the scholarship of the seven authors.
I enjoyed formally refereeing the paper on two separate occasions (enclosed), and offered a third comment (“I think the patient has a lingual nerve palsy and a tethered tongue on the left, both occurring as a complication of surgery”) by email, in the hope that it could appear in a little box as “reviewer’s comments”. I now think that I would have provided a better service to PN if I had sent this single sentence to you instead of the first review.
In the report, the authors champion the careful approach required in patients with very focal abnormalities involving the lower cranial nerves and the muscles they innervate. They describe in pictures and very useful tables—copies of which are already on my office wall—the detailed knowledge that is required to avoid misattribution in the crowded and inaccessible house of the structures involved in bulbar function.
The patient concerned had a third molar removed and postoperatively had a lingual nerve palsy. This complication is anticipated, and is included in the consent pro...
Dear Editors,
Carr AS, et al. Pract Neurol 2017;17:218–221. doi:10.1136/practneurol-2016-001491
In the interests of enhancing our understanding of bulbar function from the perspective of the jobbing clinical neurologist, I am writing to you regarding a publication in this month’s edition of Practical Neurology (Carling et al.). As it stands I do not think the case report does justice to the scholarship of the seven authors.
I enjoyed formally refereeing the paper on two separate occasions (enclosed), and offered a third comment (“I think the patient has a lingual nerve palsy and a tethered tongue on the left, both occurring as a complication of surgery”) by email, in the hope that it could appear in a little box as “reviewer’s comments”. I now think that I would have provided a better service to PN if I had sent this single sentence to you instead of the first review.
In the report, the authors champion the careful approach required in patients with very focal abnormalities involving the lower cranial nerves and the muscles they innervate. They describe in pictures and very useful tables—copies of which are already on my office wall—the detailed knowledge that is required to avoid misattribution in the crowded and inaccessible house of the structures involved in bulbar function.
The patient concerned had a third molar removed and postoperatively had a lingual nerve palsy. This complication is anticipated, and is included in the consent process. This part of the report is a valuable reminder for PN readers, but I suspect it rarely reaches neurologists as dentists and oral surgeons are so familiar with the clinical features, and the medicolegal implications.
The unusual postoperative complication in the patient described is the position of the tongue. The tongue is tilted to the left side regardless of the movements attempted, with predictable effects on relevant phoneme production and on swallowing. There has been no recovery after four years. The abnormality is striking, particularly as in complete twelfth nerve palsies—where many of the muscles of one side of the tongue can be shown to be involved clinically—electrically and radiologically the tongue is not fixed in the way described, and patients seem able to compensate relatively well with the passage of time.
The first edition of the paper attributed this to a loss of proprioception in half of the tongue, due to a loss of fibres conveying muscle stretch sense from the tongue. I questioned this in the first review, and in the second version the position of the tongue was attributed to dysfunction of styloglossus, an elevator of the tongue that fans out to insert along its lateral border. I questioned this in the second review suggesting that a more likely explanation was tethering of the tongue; the patient describes their tongue as feeling stuck, and subsequently it was suspected that there was granulomatous tissue at the site of the operation.
The authors’ point about the need for accurate diagnoses of impairments in this clinical territory is well made. This is clearly relevant in accurately identifying and naming the muscles and cranial nerve branches involved but also to distinguish pure neurological disease from temporally and anatomically adjacent structural disease. In the limbs this is more straightforward because the repertoire of signs is so broad. Observation at rest and during movement, and the testing of tone, strength, reflexes, coordination, sensation, and function is relatively straightforward, as is a basic musculoskeletal examination to look for scar tissue, contractures and joint deformities. Attempts to carry out similar tests for the jaw, larynx, pharynx and tongue are unhinged by their size and structure and the limited views, access and purchase. This congestion cramps the traditional style and sequence of neurological examination at the bedside and may leave the examiner inferring the presence of deficits on the grounds of limited evidence. In the limbs, suspected abnormalities of tone can be triangulated with strength and reflexes, and suspected weakness with inspection and passive range of movement. The signs accepted by one examiner can easily be verified or refuted by others and even quantified using validated rating scales. For the lower cranial nerves, such cross checking and shared scrutiny is more difficult. This may explain a tendency to over interpret the information that is retrieved or inferred; for the tenth nerve the gag reflex has been used in the past to inform risk assessments related to conscious level, swallowing and even mountain sickness.
With these considerations in mind, I am concerned that there may be another explanation for the findings that would further inform the approach to such cases and illustrate even more vividly the need for a special approach in cases of very localised bulbar dysfunction. Some cross checking with digital examination of the floor of the mouth, palpation of the tongue, or the results of attempts by a clinician to correct the position of the tongue may be all that is required to confirm or refute the working diagnosis.
I raise these concerns in the interests of maintaining the high standards, for authors and reviewers, of Practical Neurology.
Reviewer comments to editors (first draft)
Thanks very much for asking me to review this paper. It is a single-case report about loss of proprioception in the tongue with lingual nerve dysfunction, a common but usually transient complication of third molar removal (related to either the anaesthesia or surgery or both). There are stretch receptors in the tongue, but traditionally it has been thought that their afferent neurones travel with the hypoglossal rather than the lingual nerve.
At first I thought it was identifying something important about the tongue and in particular its proprioception, but with repeated reading I have become less certain about the interpretation of the clinical signs, and the conclusions drawn.
Immediately following third molar removal under local anaesthetic the patient described had persistent dysarthria in association with sensory loss in a lingual nerve distribution, with what are described as pseudoathetoid movements of the tongue.
I think it is a scholarly article and I hope the authors will tolerate my questions!
Was the surgery for the removal of the left third molar unusual in any way, and in particular were any incisions required in to the gum of the third molar, and if so were they made on the buccal (standard practice) or lingual side? This would inform thinking about the possibility of additional complications.
Were tests done to demonstrate loss of proprioception in the tongue? I think this is important given their use of the term pseudoathetoid in the title of the paper.
Options include holding the tongue gently with small padded forceps and with the patient’s eyes closed testing awareness of passive tongue movements. As with other tests of lower cranial nerve dysfunction the field of view in such a crowded area is not great and the repertoire of signs limited, but if the described tongue movements are a result of proprioceptive loss, I would expect some detectable impairment of awareness of tongue position. This is of particular relevance as in day-to-day function any sensory loss in and on the tongue was apparently uncompensated for by the completely normal right side sensation (lingual and buccal) and the normal left sided sensation of the buccal mucosa, teeth, hard palate and other closely tongue-apposing structures.
Is it reasonable to compare and contrast this patient’s bizarre tongue movements with other patients who have more extensive intraoral sensory loss e.g. lateral medullary syndrome, pontine plaques and cavernomas etc.? I think we see dysarthrias commonly, and tongue deviation secondary to motor problems, but not bizarre tongue movements that we attribute to sensory loss. With such a high density of receptors in the tongue and mouth, and the relatively small area of sensory loss on just one side of her tongue and gum, it seems improbable that she would not be able to overcome a unilateral sensory problems, and relearn normal speech and swallowing.
Is there another reason why she has ended up so disabled by a unilateral sensory problem? Could it be structural or motor?
The patient describes feeling as though the tongue is stuck, something that suggests a degree of preserved proprioception, but also could suggest a structural problem. I would be interested to know if the floor of the mouth and base of tongue was palpated, digitally, on the left side to detect any persistent post operative changes, particularly any involving tongue muscles, as some of the movements of the tongue could be explained by a motor problem. I note that there was no wasting or fasciculation of the tongue and that an EMG was normal but the extrinsic muscles of the tongue may still be dysfunctional, and I would like to ask about two.
I note that the tongue at rest had a tendency to point to the right (rather than be randomly positioned in the mouth as would be expected with significant proprioceptive loss), and in figure 1 the right side seems higher than on the left. I would be interested to know if this pattern of movement could be explained by an absence of styloglossus function on the left. This muscle pulls the tongue backwards and upwards. A loss of function may explain the resting position of the tongue and the tendency of it to point to the right, as the push-me-out, pull-me-back interaction of genioglossus and styloglossus would be lost on the left, and the push-me-out action of left genioglossus would be unopposed, resulting in a tendency of the tongue to point to the right.
The hyoglossus muscle pulls the tongue down and theoretically if it was damaged or changed by surgery it could be tethering the tongue on the left, effectively pulling it down and creating the consistently asymmetrical position in the mouth.
Is there any chance that surgery could have damaged these muscles or their nerve supply?
Regarding the possibility of a motor problem they give very little information about her speech. I would be interested to know whether she was able to reliably produce alveolar and velar plosives, and whether lingual fricatives were normal. There is also no information about the nature of her swallowing problem, particularly whether there was anything to suggest, clinically or on a videofluoroscopy, inadequate apposition of the base of the tongue with the posterior pharyngeal wall.
The sequence of activation of the muscles involved in swallowing is immutable, and can be initiated voluntarily, and is not in anyway dependent on lingual nerve function therefore it is difficult to attribute her swallowing problem to sensory loss of any kind in the anterior tongue. This is another reason I would be interested to know if a motor or structural problem could underlie her symptoms, unchanged after four years.
Overall I feel that not enough evidence has been gathered to justify attributing the tongue movements to a problem with proprioception, but I would be intrigued to review more information about the clinical findings.
There are some minor points of accuracy relating to the motor innervation of palatal elevation (X and V, not IX), the relevance of inferring that a present gag reflex in a conscious adult tells us anything about swallowing function (analogous to saying that someone can walk because they have knee and ankle reflexes), or that an absent palatal or pharyngeal reflex necessarily means that there is sensory loss (in lots of normal adults palatal and pharyngeal movement is not elicited by their stimulation). There is one typo (dysphasia instead of dysphagia) and in the abstract a mistake (facial nerve instead of lingual nerve).
I have enjoyed reviewing the paper and would be interested to see a second draft.
Reviewer comments to editors (second draft)
Thank you very much for asking me to review this paper. I saw an earlier version in which a loss of proprioception/muscle stretch sense in the tongue (as a feature of a lingual nerve palsy) was thought to be contributing to the clinical picture. In this revised version it is suggested that in addition to the lingual nerve palsy there is selective involvement of styloglossus, and that this explains the abnormality of tongue movement.
I have read and reread the paper and have once again enjoyed checking and cross checking the neuroanatomy. I really enjoyed the scholarship of the authors involved in describing each individual intrinsic and extrinsic muscle of the tongue and suggesting ways in which clinicians can examine their function and I think this version is an improvement on the first.
However I still have some reservations about the central message of the report –in particular the selective involvement of styloglossus - and I feel sure that the authors would want me to challenge their conclusions.
A lingual nerve injury is very well described as a complication of third molar removal and this was clearly the case in this patient. I will not comment further on the clinical features that are clearly a result of a lingual nerve palsy.
What is less clear is the mechanism underlying the position of the tongue at rest, and during phoneme production.
The most striking, and possibly most relevant, clinical sign in this case is the depressed position, at rest, of the posterolateral aspect of the tongue. This does not correct when the subject sees her tongue in the mirror, and appears to have remained fairly static despite therapy and the passage of time (four years).
This is attributed to the selective involvement of the styloglossus muscle which (Gray’s Anatomy) “ is the shortest and smallest of the three styloid muscles and passing downwards and forwards divides into two portions, one longitudinal which enters the side of the tongue near its dorsal surface, blending with the fibres of the Longitudinalis linguae inferior in front of the Hyoglossus, and the other, oblique portion, overlaps the Hyoglossus and decussates with its fibres”.
Changes in speech production are also attributed to a problem with styloglossus (which retracts and elevates the tongue) in particular difficulty with lingual fricatives, as are changes in swallowing.
Other features of note are that before the patient attended the peripheral nerve clinic ENT surgeons had commented on a post-traumatic neuroma/granuloma at the left retromolar space and I note the patient’s comment about “a sticking of her tongue” when speaking and the pain she reported a month later around the operative area.
Conclusion
I find it difficult to believe that dysfunction of styloglossus is responsible for the problems with tongue movement in this patient for a number of reasons:
1 By the time the muscle has reached the angle of the jaw it is starting to fan out and it seems unlikely that the whole muscle could be transected during dental surgery without the surgeon noticing
2 It is a small muscle
3 MR imaging does not reveal atrophy of the tongue or any selective atrophy of styloglossus (or any other tongue muscle) and yet the disability is severe (she lost her job in telesales) and her speech did not improve with therapy or the passage of time.
4 I have not seen persistent changes in the posture of the tongue, at rest, in complete unilateral hypoglossal nerve palsies when all muscles lose their opposite number, and all of the muscular push-me-pull-you is unopposed, and unapposed.
5 For the position of the tongue to be explained by the loss of styloglossus we would have to accept that at rest the muscle is in fact contracting and elevating the base of the tongue.
My lingering concern is that the left tongue base is tethered in to the floor of the mouth posterolaterally.
Therefore I would be very interested to know if palpation of the tongue and floor of the mouth is normal and in particular if the left dorsolateral tongue can be elevated by the examiners finger or fingers in the same way as it can on the right side.
If this is the case then the report would be very valuable as it would highlight the importance of excluding structural disease before attributing changes in bulbar function to purely neurological deficits.
I hope this is helpful and I look forward to further correspondence.
Tom
We thank Prof Barker for sharing his thoughts and experience[1] on
our report of psychogenic non-epileptic seizures in Huntington's
disease[2].
We agree that neuropsychiatric manifestations are extremely common in HD
and most cases of unusual behaviours or movements would not fall into the
category of functional disorders.
Moreover we certainly do not wish to give the impression that non-
epileptic...
We thank Prof Barker for sharing his thoughts and experience[1] on
our report of psychogenic non-epileptic seizures in Huntington's
disease[2].
We agree that neuropsychiatric manifestations are extremely common in HD
and most cases of unusual behaviours or movements would not fall into the
category of functional disorders.
Moreover we certainly do not wish to give the impression that non-
epileptic attacks are highly prevalent in HD: they certainly remain
confined to a small number of patients with HD overall.
However, Prof Barker's observations from his patient cohort and the
literature are not inconsistent with our suggestion that the diagnosis is
nonetheless under-recognised. In our similarly-sized service, we have
encountered two such patients with compelling clinical and
neurophysiological evidence for non-epileptic seizures in the past year
alone. It would certainly be interesting to see what proportion of HD
patients diagnosed with seizures in a large cohort had EEG evidence to
support the diagnosis.
We could not agree more with Prof Barker, that a high index of suspicion
and low threshold for thorough investigation is necessary in all such
cases.
References
[1] Barker RA, Mason SL. Re: Psychogenic non-epileptic seizures in
early Huntington's disease. Rodrigues, Wild 16:6 452-454. Published 20
December 2016.
[2] Rodrigues FB, Wild EJ. Psychogenic non-epileptic seizures in
early Huntington's disease. Pract Neurol 2016;16:452-454
doi:10.1136/practneurol-2016-001423.
The only thing that I can disagree with in Jonathan Schott and Martin Rossor's article on the palmomental reflex in the December issue of Practical Neurology is their plea that I should stop scraping. I have found that the palmomental reflex can be made more specific for detecting brain damage by applying the orange stick or car key to the hypothenar rather than the thenar eminence. A positive palmomen...
The only thing that I can disagree with in Jonathan Schott and Martin Rossor's article on the palmomental reflex in the December issue of Practical Neurology is their plea that I should stop scraping. I have found that the palmomental reflex can be made more specific for detecting brain damage by applying the orange stick or car key to the hypothenar rather than the thenar eminence. A positive palmomental reflex sign is present if there is a mentalis twitch in at least seven out of ten successive scratches.
While I agree that the test has no role in the diagnosis of dementia or Parkinson's disease it may still have useful application in the investigation of psychosis and obsessional compulsive disorder. A positive quantitative palmomental reflex in a person under 60 would make me consider neuroimaging to look for structural cerebral abnormalities.
Similar caveats to those put forward by Schott and Rossor for the palmomental reflex apply to the glabellar tap (Myerson's sign). It is an accoutrement that impresses patients but is redundant in the evaluation of Parkinsonism. However, there are instances where it can be helpful. Some years ago I saw a woman of 45 who presented with physical fatigue. She had a hangdog posture, a monotonous voice, did not swing her arms when walking and had a sad face. She had seen several physicians already and carried the label of chronic fatigue syndrome. On examination she had a positive glabellar tap sign. When she returned for follow up a few months later she had developed a clear sequence effect (motor decrement) on finger tapping and I made a firm diagnosis of Parkinson's disease. Primitive reflex testing is quick and simple and may still have a place in the research screening of normal elderly people for neurological disease. I will continue to teach the palmomental reflex and the glabellar tap sign to medical students but emphasise the limitations highlighted by Schott and Rossor. Perhaps the Editors will consider a neuromythology series on useless research scales and operational criteria for the diagnosis of neurological disease when the physical signs series is completed?
We were interested to read the recent case study presented by
Rodrigues and Wild in the current edition of Practical Neurology where
they discussed "Psychogenic non-epileptic seizures in early Huntington's
disease"[1]. The authors gave an in-depth account of the clinical profile
of a 51-year-old male with early Huntington's disease (HD) who presented
with repeated episodes of repetitive, purposeless, c...
We were interested to read the recent case study presented by
Rodrigues and Wild in the current edition of Practical Neurology where
they discussed "Psychogenic non-epileptic seizures in early Huntington's
disease"[1]. The authors gave an in-depth account of the clinical profile
of a 51-year-old male with early Huntington's disease (HD) who presented
with repeated episodes of repetitive, purposeless, complex behaviours,
uncharacteristic of HD and discussed the process they followed before
diagnosing psychogenic non-epileptic seizures. Rodrigues and Wild then
went on to conclude that functional neurological symptoms and dissociative
phenomena are a common feature of early HD, particularly in the period
immediately post-diagnosis.
However, this is very different to our own clinical experience. Although
we have not prospectively undertaken a study to look specifically for
this, we have retrospectively analysed our cohort of over 700 patients
that we have seen over the last 20 years. Over this time we could only
identify two individuals who could be described as having a functional
neurological disorder. Furthermore, upon review of the literature we can
only find one case study reporting a psychogenic movement disorder in
preclinical HD [2] and a further case study of a psychogenic gait disorder
that was presented as a poster at the 2013 Sydney Movement Disorders
Conference.
Therefore, we feel that the claim by Rodrigues and Wild is somewhat
misleading -that this is a common feature of early HD. In our experience a
significant proportion of patients experience psychiatric symptoms in
early HD which can take the shape of increased anxiety and
obsessional/compulsive type behaviours and as such patients can exhibit
repetitive stereotypic actions. They do not however, commonly present with
features that would be consistent with a psychogenic disorder and it is
important that the prevalence of such symptoms is not inflated. We
acknowledge that the behaviors presented in this case study as evidenced
by the video [helpfully included in the supplementary data], are not
consistent with anxiety or OCD, but we consider this to be a relative
rarity in HD. In our opinion, an HD patient presenting with physical,
cognitive or psychiatric features that are not consistent with the common
profile of the disease should be thoroughly investigated to rule out all
possible organic causes and only reluctantly should these symptoms be
attributed to a non-organic process.
References
1. Rodrigues, F.B. and E.J. Wild. Psychogenic non-epileptic seizures
in early Huntington's disease. Pract Neurol 2016.
2. Soltan, W., et al. Abnormal gait and bradykinesia in the preclinical
phase of Huntington's disease - psychogenic movement disorder? Acta
Neuropsychiatr 2011;23(6):315-7.
In his recent Neuromythology article1 on the Babinski sign, Professor
Kiernan repeats the oft quoted recommendation regarding the best method
for testing the plantar response, which is to:
'Run a car key (figure 1; or as some have suggested, a most expensive
motor car key...) along the lateral border of the sole.'
It will not surprise many readers of Practical Neurology to learn
th...
In his recent Neuromythology article1 on the Babinski sign, Professor
Kiernan repeats the oft quoted recommendation regarding the best method
for testing the plantar response, which is to:
'Run a car key (figure 1; or as some have suggested, a most expensive
motor car key...) along the lateral border of the sole.'
It will not surprise many readers of Practical Neurology to learn
that the author of this particular recommendation was the late Professor
Henry Miller and moreover that he was proscriptive with regard to the
precise type of ignition key. In his introduction to the book 'Remembering
Henry'1, reflecting on the remarkable life and career of one of the
undoubted pioneers of modern British neurology (and prolific music
critic), the late Lord Walton of Detchant wrote:
'Books could be (and probably will be) filled with quotations from
his tongue and from his fertile pen, some few of which were hallowed for
posterity as 'Henry Millerisms' in a popular medical journal. Who can
forget such comments as: 'The best instrument for obtaining the plantar
response is the ignition key of a Bentley'; or, 'Hemiplegic multiple
sclerosis is a rarity and is to be diagnosed only by me'?'
Henry George Miller (1913-1976) became Professor of Neurology at
Newcastle University in 1964. This was the second chair in neurology to be
established in the UK (Roger Gilliat having been appointed as professor of
clinical neurology at the Institute of Neurology, Queen Square in 1962).
He is best known for his work on multiple sclerosis (publishing the first
ever therapeutic trial in 19532) and accident neurosis. Henry was
successively Dean of Medicine (1966-1968) and Vice Chancellor (1968-76) of
Newcastle University, served as Secretary-General-Treasurer of the World
Federation of Neurology and Chairman of the medical panel of the MS
Society, and held visiting chairs in Australia, Canada and the USA.
References
1. Remembering Henry. LOCK S, WINDLE H (Editors). BMJ.
The Devonshire Press. 1977.
2. Acute disseminated encephalomyelitis and acute disseminated sclerosis;
results of treatment with ACTH. MILLER HG, GIBBONS JL. BMJ.
1953;2(4850):1345-1348
This sensitive review is very welcome, as in our experience, most
patients with functional disorders are told by specialists what they do
not have. I would be much more inclined to refer patients if this kind of
approach was more common. It is one reason why relatively few of the
patients we see in primary care are referred for specialist attention.
Specialists are seeing only the tip of the primary care functional
iceb...
This sensitive review is very welcome, as in our experience, most
patients with functional disorders are told by specialists what they do
not have. I would be much more inclined to refer patients if this kind of
approach was more common. It is one reason why relatively few of the
patients we see in primary care are referred for specialist attention.
Specialists are seeing only the tip of the primary care functional
iceberg. Referral rates vary widely depending variously on levels of
experience, resilience, tolerance of uncertainty and access to peer or
specialist support.
One advantage GPs have over our specialist colleagues is that we get
to know our patients over many years - which is how long it may take to
discover the biography behind their functional symptoms. A second
advantage we have is a knowledge of their family and social context within
which they experience their symptoms enabling us to take a systemic
approach. We have more in common with Oliver Sacks- who rediscovered the
importance of narratives divulged slowly, and used to visit his patients
at home like his father, a London GP, than we do with our younger
neurological colleagues.
It's worth remembering that a clinical history is not narrative and
biography is not psychology. Stone warns against framing functional
neurological symptoms in psychological terms, which I would agree with -
mental illness, if it co-exists ought to be seen as a co-morbidity rather
than a primary cause. But Stone's approach is still biomedical and lacks
the systemic approach that time, continuity and contextual factors
illuminate. I recently invited older GPs to talk to trainees about their
favourite patients. Many described patients who were struck with their
functional symptoms for years, decades even - in a state of narrative
chaos, before something changed and they found some kind of peace with
themselves and were much better able to live with their symptoms. In
narrative medicine this is a shift from chaos to quest, where making sense
takes over from searching for a cure. There are many ways of making sense
of functional symptoms and my main concern about Stone's approach is that
usually it's most honest to admit we just don't (yet) know.
Title: Romberg's test has stood up to the test of time and should
remain standing
(Neuromythology: Letter to the Editor)
I was Interested to read Professor Martin Turner's article entitled
Romberg's test no longer stands up. In the article Professor Turner
describes the test as "the process of standing unsupported with the eyes
closed and feet together for 30 s" and asserts that "the positive result
is the p...
Title: Romberg's test has stood up to the test of time and should
remain standing
(Neuromythology: Letter to the Editor)
I was Interested to read Professor Martin Turner's article entitled
Romberg's test no longer stands up. In the article Professor Turner
describes the test as "the process of standing unsupported with the eyes
closed and feet together for 30 s" and asserts that "the positive result
is the patient falling to the floor" which he gives as one of his reasons
for abandoning the test. He further states that the test is "neither
highly sensitive nor specific for its original purported purpose".
Furthermore, along with the authors of previous reviews of the test which
he quotes,1,2 he denies Romberg the credit of having linked the positivity
of the test to a dorsal column lesion. I note that Professor Turner
does not reference the 2nd Edition Romberg's Manual, the original source
description3 of the test in and his description of the test or sign does
not corresponded with the original. Romberg3 describes his sign twice in
the Manual. In the second description, at the top of page 396 of Volume
2, Romberg states "If he [the patient with Tabes Dorsalis] is ordered to
close his eyes while in the erect posture, he at once commences to totter
and swing from side to side; the insecurity of his gait also exhibits
itself more in the dark". In the earlier description in Volume 1, on
pages 226-227, he says "I have observed that anaesthesia of the muscles
alone without loss of tactile power, invariably accompanies tabes
dorsalis. A simple experiment suffices to determine the fact. If the
patient is told to shut his eyes while in the erect posture, he
immediately begins to move from side to side, and the oscillations soon
attain such a pitch that unless supported he falls to the ground."
Note firstly that there are arguably two parts to Romberg's test. The
first part, requires no time interval, on the contrary, a positive
Romberg's test is demonstrated by an increased sway that begins "at once"
or "immediately" after the eyes are closed. Furthermore, according to his
second description of the sign, a fall to the floor is not required and
only the now never-undertaken second part of the test, requiring darkness,
is potentially dangerous. Romberg's claim for specificity and
sensitivity is that "it invariably accompanies tabes dorsalis" and "it is
a symptom which I have not observed in other paralyses, nor in
uncomplicated amuarosis; since then [since he first said that he described
the sign 10 years previously], I have found it in a considerable number of
patients [with Tabes Dorsalis] .... and in no case have I found it
wanting." Romberg also states that "some patients mention the
circumstance [increased unsteadiness in the dark, while standing without
support] without being asked about it". It is also interesting that a
little later on in the same description, on page 397 of Volume 2, Romberg
says that as it progresses, "the necessity of employing the eyes becomes
more and more urgent; if he [the patient with Tabes Dorsalis] closes his
eyes, even while sitting, his body begins to sway to and fro" and he "is
no longer able to recognize the position of his own limbs and cannot tell
whether the right leg is crossed over the left, or the reverse". Since
this description of the disease was prior to the discovery of the
causative spirochaete, it quite possibly that Romberg (as well as other
19th century Neurologists) may have included other causes dorsal column
damage and large fibre neuropathy / neuronopathy and among his cases.
Romberg3, correlating the clinical features with the pathology, mentions
on the same page as his sign, that post-mortem findings "almost without
exception show the existence of partial atrophy of the spinal cord" and
that "the contents of the cords of the cauda equina have often been found
to have disappeared to such an extent that nothing but the empty
neurilemmatous sheaths seemed to remain". He further noted that "it is of
especial interest to observe that the posterior, sensory roots are
occasionally alone affected in conjunction with the posterior columns of
the spinal cord, the anterior motor columns and nerves retaining their
normal structure". To my mind, that counts as linking the positivity of
the test to a dorsal column lesion and indeed to a lesion of the sensory
roots and / or nerves which Professor Turner, incidentally, did not
mention as a cause of a positive Romberg's sign.
Sir William Gowers4 in the 1888 American Edition of his textbook cited
Romberg and his test as did the legendary Jean-Martin Charcot5 in his
Tuesday Lectures. It is interesting that Gowers4, referring to Romberg's
test states that "the effect of closure of the eyes is greatest when
sensation in the soles of the feet is defective, but does not depend on
this loss; it may be marked when sensation on the soles of the feet is
perfect". Although the current method of joint position testing in the
Hallux, referred to by Professor Turner, seems unlikely to have been
employed at the time of Gowers, it is certainly my experience that dorsal
column function or peripheral nerve proprioceptive sensory function can be
sufficiently impaired to cause "positive Rombergism" when concurrent
testing of joint position sense in the hallux on both sides is apparently
within normal limits. By way of explanation of this personal observation,
I would suggest that proprioception is likely to be a more complex
predominantly unconscious function of the nervous system, rather than the
simple transmission of the position of the distal phalanx of the hallux to
consciousness. Therefore, I disagree with Professor Turner's assertion
that simply testing joint position test in the Hallux is a substitute for
Romberg's test.
Before dismissing a test proposed by the one of the greatest Neurologists
of the 19th century who was author of the first systematic textbook of
Neurology ever written and who made numerous other seminal contributions
to our subject, I think we should be a little more circumspect. Wilson6
in his classic 1940 textbook also considered Romberg's sign useful and it
is considered an important part of the neurological examination in most
modern textbooks of Neurology. In my view, Romberg's Test is a very
useful part of the neurological examination, because it so beautifully
illustrates and confirms to the clinician and student alike, the degree to
which loss of proprioception contributes to ataxia and impaired balance in
any particular case (whichever of the limited number of underlying
pathophysiologies is responsible). A positive Romberg's test therefore
helps the clinician to attribute ataxia and / or impaired balance to
pathology in the dorsal column of the spinal cord, or in the peripheral
large nerve fibres destined for the dorsal columns, rather than to
pathology in the cerebellum. It is not dangerous and has stood the test
of time. I believe Romberg's test should remain part of the routine
neurological examination taught to all medical students and used by all
Neurologists. I believe that Professor Turner should stand down on this
issue and that Professor Romberg and his test remain upstanding.
References
1. Lanska DJ, Goetz CG
Romberg's sign. Development, adoption, and adaptation in the 19th century
Neurology 2000;55:1201-6.
doi:10.1212/WNL.55.8.1201
2. Pearce JM
Romberg and His Sign.
Eur Neurol 2005;53:210-13
doi:10.1159/000086732
3. Romberg MH
A Manual of The Nervous Diseases of Man 2nd Edition 1851 Volumes 1&2 226-
227,396-397.
Translated and edited by Sieveking EH, London: Sydenham Society
Reprint: Alabama: Gryphon Editions Ltd 1983:
4. Gowers WR
A Manual of Diseases of the Nervous System. American Edition P.289
Philadelphia: P.Blakiston, Son & Co 1888
Reprint: Alabama: Gryphon Editions Ltd 1983:
5. Charcot JM
Charcot the Clinician. The Tuesday Lectures
Excerpted and translated case presentations by Goetz CG P143
Raven Press New York 1897
6. Kinnier Wilson SA
Neurology Volume 1 P.494
London: Edward Arnold & Co 1947
"Provenance and peer review. Not commissioned, Externally peer
reviewed. This paper was reviewed by Martin Tuner, Oxford, UK."
I am in general agreement with the notion expressed in Professor
Hawkes' editorial that many patients in
neurology outpatient clinics do not need examination. Certainly, the full
neurological examination has a rather Zen-like quality: it takes a great
deal of work to master it and then one finds one
doesn't really need it. However, I would like
to express a number of disagreements with some of what Professor Hawkes
wr...
I am in general agreement with the notion expressed in Professor
Hawkes' editorial that many patients in
neurology outpatient clinics do not need examination. Certainly, the full
neurological examination has a rather Zen-like quality: it takes a great
deal of work to master it and then one finds one
doesn't really need it. However, I would like
to express a number of disagreements with some of what Professor Hawkes
wrote.
The examination is, of course, not purely diagnostic. I have listed
a number of other features in Box 1.
Fundamentally, Professor Hawkes appears to suggest that the
examination can be replaced by investigation. It could be argued that this
replaces one unnecessary activity (and a relatively cheap one) with
another unnecessary activity (and a much more expensive one). Waiting
times for individuals to be seen in outpatient clinics have been reduced
in recent times. However, there are often significant delays for
necessary investigations, such as MR Imaging and Neurophysiology. One
reason for this is that many arguably unnecessary investigations are
undertaken, particularly imaging for headache, neck pain and back ache.
The majority of patients in general neurology outpatient clinics can in
fact be dealt with by a careful history taking.
On a more specific point, Professor Hawkes states that
"if your patient has a
headache...he or she is going to need a brain
scan". This is an extremely contentious point.
Indeed, Professor Hawkes does imply that this
"need" is for
reassurance rather than for specific diagnostic purposes and, of course,
the majority of causes of headache have normal scans, therefore this
investigation is indeed not diagnostic. He states that no matter how much
one reassures the patient that it is not a serious form of headache, they
will still want a scan. On a purely anecdotal basis, it does seem as
though the desire for brain scans in patients with headaches does vary
greatly, not only from patient to patient but also according to which
clinician they see. One important point is that simple reassurance of a
non-serious cause is not always sufficient: the patient may require a
positive diagnosis. If they accept that diagnosis, they may not wish for a
scan; it is to be hoped that such acceptance depends not simply on the
"charm" of the doctor
(as Professor Hawkes puts it) but also on their perceived competence and
the way they explain the diagnosis.
Professor Hawkes does not deal with the issue of incidental abnormalities.
There are publications indicating that brain scanning in cases of headache
uncovers incidental abnormalities in a proportion of patients, causing
additional anxiety and greater use of medical time 1,2,3. As a taxpayer,
I might feel somewhat aggrieved if my financial support for the headache
patient with extends over dismaying a series of cost events: a general
practice consultation, a neurological consultation, a high-tech
investigation, further investigation because of doubtful abnormalities on
the first test and, finally, additional consultation time to deal with the
uncovered incidental abnormalities. Professor Hawkes states that patients
may wish to pay for a scan "in the private
sector". (As an aside here, all NHS
consultations are indeed private. I think Professor Hawkes is referring
here to the commercial sector.) If indeed a patient requires a medically
unnecessary scan, then it might be reasonable for them to pay for it - providing that all subsequent matters arising
from the scan, including reassurance about abnormal results, are also
dealt with in the commercial sector.
As a final point, Professor Hawkes discusses the issues of
"video clinics". I
can understand that this is a potential way of dealing with certain
problems. However, there is an irreducible human element in the medical
consultation and face-to-face consultations can be extremely important,
allowing interaction, assessment and reassurance that is simply not
possible through remote video or audio connection.
References
1. Computed Tomography in the headache patient: Is routine evaluation
really necessary? Mitchell et al Headache 1993 33:82-86
2. Incidental Findings in magnetic resonance imaging of the brains of
healthy young men. Weber & Kopf J Neurol Sci 2006 240:81-84
3. Incidental findings on brain magnetic resonance imaging: systematic
review and meta-analysis. Morris et al BMJ In Press
Box 1
Non-diagnostic aspects of the examination
o To give the clinician time to think
o To help establish doctor/patient relationship
o To allow the patient to volunteer aspects of the history not given
during direct enquiry
o To demonstrate to the patient features which are important in their
management
o Non specific therapeutic effect of examination
Sometimes it is necessary to make a point with overemphasis and we
believe that was done in the letter by Chris Hawkes. We agree that it is
essential to talk to a patient and to watch them and observe how they
speak, what they are saying and what they are doing with their body as
they walk in and out of a room. However, to exclude the examination is a
form of conceit. The only reason that a very senior neurologist can
o...
Sometimes it is necessary to make a point with overemphasis and we
believe that was done in the letter by Chris Hawkes. We agree that it is
essential to talk to a patient and to watch them and observe how they
speak, what they are saying and what they are doing with their body as
they walk in and out of a room. However, to exclude the examination is a
form of conceit. The only reason that a very senior neurologist can
observe so much is from a long period of diligently examining all patients
and slowly learning shortcuts by pattern recognition. But you
can't teach neurology that way. Students must
first be taught the discipline of a routine in order to learn which part
of the routine can be discarded in individual patients. When a TIA is
caused by atrial fibrillation a finger on the pulse will lead you to the
ECG as the first test. A stethoscope on the neck will suggest that a
Doppler should happen immediately. When resources are limited (which
occurs in much more than half the world) it is important to direct your
tests sensibly rather than blindly ordering a battery. If a student
can't distinguish between an upper and lower
motor lesion then the differential of a paralysed limb trebles and an EMG
as well as an MRI needs to be done routinely. We are not against spending
more time talking to a patient, even if it means less time examining, but
don't eliminate the examination.
It's like throwing the baby out with the
bathwater. When in doubt the most cost effective investigation is to
retake the history and to examine the patient. We
mustn't forget that an essential part of any
consultation is to gain the trust and respect of the patient; if a patient
doesn't trust the consultant they are unlikely
to accept the diagnosis and treatment plan. We have all seen patients for
second and third opinions when on completing the examination they
complement you on how thorough you have been and inform you that their
previous consultants hadn't bothered to examine
them. The "laying on of
hands" is as essential to
today's consultation as it was in the past. In
today's litigious environment not examining a
patient would be welcomed by the opposing legal team with glee. We doubt
any expert witness would support the notion of not examining a patient as
standard clinical practice. In the new era of revalidation admitting to
your peers that you do not examine your patients would be inviting an
early retirement or a change in career.
In the referring doctor vs consultant neurosurgeon interaction(1),
there is, sometimes, a reversal of roles with the result that it is the
patient(often elderly) who is confronted with the reality that he or she
is not suitable candidate for acceptance by the neurosurgeon. For a
succesful referral to take place the referring doctor(often a harassed
trainee) has to make the patient marketable(2) to tertiary care, using...
In the referring doctor vs consultant neurosurgeon interaction(1),
there is, sometimes, a reversal of roles with the result that it is the
patient(often elderly) who is confronted with the reality that he or she
is not suitable candidate for acceptance by the neurosurgeon. For a
succesful referral to take place the referring doctor(often a harassed
trainee) has to make the patient marketable(2) to tertiary care, using
"streetwise" strategies and wiles best borrowed from Sir Alan Sugar's
"Apprentice" programme(3). In the clinical context less unsavoury
strategies include an aptitude for lateral thinking, exemplified by
acting(by requesting computerised tomography) on the hitherto unvalidated
suspicion that chronic treatment with antiplatelet agents(even without
coprescription of warfarin) might be a risk factor for subdural
haematoma(SDH) in patients with head injury(4). That is the easy bit. The
real challenge is to convince tertiary care that, notwithstanding evidence
from potentially outdated observational studies suggesting certain care
pathways for elderly patients with SDH(5), even a 75 year old SDH patient
with either pupillary dilatation or extensor motor responses might be a
suitable candidate for neurosurgical intervention. On a more serious note,
although I have used the Editorial(1) as a pretext, the two observational
studies(4)(5) that I have mentioned seriously need to be followed up by
well designed prospective studies so as to enhance the clinical quality
and intellectual rigour of management of SDH.
References
(1) Haines SJ
How to choose your neurosurgeon
Practical Neurology 2010;10:2-3
(2) Nagus P., Bridges J., Braithwaite J
Selling patients
BMJ 2009;339:19-26
(3) Harding J
Show business
The Times June 13, 2007, pages 4-5
(4) Major J., Reed MJ
A restrospective review of patients with head injury with coexistent
anticoagulant and antiplatelet use admitted from a UK emergency department
Emerg Med J 2009;26:871-6
(5) Jamjoom A., Nelson R., Stranjalis G et al
Outcome following surgical evacuation of traumatic intracranial haematomas
in the elderly
British Journal of Surgery 1992;6:27-32
Dear Editors,
Show MoreCarr AS, et al. Pract Neurol 2017;17:218–221. doi:10.1136/practneurol-2016-001491
In the interests of enhancing our understanding of bulbar function from the perspective of the jobbing clinical neurologist, I am writing to you regarding a publication in this month’s edition of Practical Neurology (Carling et al.). As it stands I do not think the case report does justice to the scholarship of the seven authors.
I enjoyed formally refereeing the paper on two separate occasions (enclosed), and offered a third comment (“I think the patient has a lingual nerve palsy and a tethered tongue on the left, both occurring as a complication of surgery”) by email, in the hope that it could appear in a little box as “reviewer’s comments”. I now think that I would have provided a better service to PN if I had sent this single sentence to you instead of the first review.
In the report, the authors champion the careful approach required in patients with very focal abnormalities involving the lower cranial nerves and the muscles they innervate. They describe in pictures and very useful tables—copies of which are already on my office wall—the detailed knowledge that is required to avoid misattribution in the crowded and inaccessible house of the structures involved in bulbar function.
The patient concerned had a third molar removed and postoperatively had a lingual nerve palsy. This complication is anticipated, and is included in the consent pro...
Dear Editor,
We thank Prof Barker for sharing his thoughts and experience[1] on our report of psychogenic non-epileptic seizures in Huntington's disease[2]. We agree that neuropsychiatric manifestations are extremely common in HD and most cases of unusual behaviours or movements would not fall into the category of functional disorders. Moreover we certainly do not wish to give the impression that non- epileptic...
Dear Editor,
The only thing that I can disagree with in Jonathan Schott and Martin Rossor's article on the palmomental reflex in the December issue of Practical Neurology is their plea that I should stop scraping. I have found that the palmomental reflex can be made more specific for detecting brain damage by applying the orange stick or car key to the hypothenar rather than the thenar eminence. A positive palmomen...
Dear Editor,
We were interested to read the recent case study presented by Rodrigues and Wild in the current edition of Practical Neurology where they discussed "Psychogenic non-epileptic seizures in early Huntington's disease"[1]. The authors gave an in-depth account of the clinical profile of a 51-year-old male with early Huntington's disease (HD) who presented with repeated episodes of repetitive, purposeless, c...
Dear Editor,
In his recent Neuromythology article1 on the Babinski sign, Professor Kiernan repeats the oft quoted recommendation regarding the best method for testing the plantar response, which is to:
'Run a car key (figure 1; or as some have suggested, a most expensive motor car key...) along the lateral border of the sole.'
It will not surprise many readers of Practical Neurology to learn th...
This sensitive review is very welcome, as in our experience, most patients with functional disorders are told by specialists what they do not have. I would be much more inclined to refer patients if this kind of approach was more common. It is one reason why relatively few of the patients we see in primary care are referred for specialist attention. Specialists are seeing only the tip of the primary care functional iceb...
Title: Romberg's test has stood up to the test of time and should remain standing (Neuromythology: Letter to the Editor)
I was Interested to read Professor Martin Turner's article entitled Romberg's test no longer stands up. In the article Professor Turner describes the test as "the process of standing unsupported with the eyes closed and feet together for 30 s" and asserts that "the positive result is the p...
I am in general agreement with the notion expressed in Professor Hawkes' editorial that many patients in neurology outpatient clinics do not need examination. Certainly, the full neurological examination has a rather Zen-like quality: it takes a great deal of work to master it and then one finds one doesn't really need it. However, I would like to express a number of disagreements with some of what Professor Hawkes wr...
Sometimes it is necessary to make a point with overemphasis and we believe that was done in the letter by Chris Hawkes. We agree that it is essential to talk to a patient and to watch them and observe how they speak, what they are saying and what they are doing with their body as they walk in and out of a room. However, to exclude the examination is a form of conceit. The only reason that a very senior neurologist can o...
In the referring doctor vs consultant neurosurgeon interaction(1), there is, sometimes, a reversal of roles with the result that it is the patient(often elderly) who is confronted with the reality that he or she is not suitable candidate for acceptance by the neurosurgeon. For a succesful referral to take place the referring doctor(often a harassed trainee) has to make the patient marketable(2) to tertiary care, using...
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