Dear Sirs,
We welcome Dr Leach's comments and a call for action from Neurologists,
regarding service provision for women of childbearing age with
neurological disorders1. We would also like to highlight that quietly, a
number of us are grasping the nettle.
Risk is inherent in clinical practice. Managing risk effectively and pro-
actively in preference to reactively minimises the likelihood of a poor
outcome. This can be...
Dear Sirs,
We welcome Dr Leach's comments and a call for action from Neurologists,
regarding service provision for women of childbearing age with
neurological disorders1. We would also like to highlight that quietly, a
number of us are grasping the nettle.
Risk is inherent in clinical practice. Managing risk effectively and pro-
actively in preference to reactively minimises the likelihood of a poor
outcome. This can be achieved by identifying those at risk and adapting
practices to avoid that risk. This has not often been more concisely put
than by Cicero who is quoted as stating that "To make a mistake is only
human; to persist in a mistake is idiotic". We agree that at the very
least, missing an opportunity to avoid a maternal death is idiotic, and
certainly tragic.
A joint approach is needed and at St George's University Hospitals NHS
Foundation Trust, Consultants in Obstetrics, Obstetric anaesthetics,
Neurology, Neurosurgery and Neuroradiology have together with excellent
support from Maternal Medicine Midwifery and Neurology Clinical Nurse
Specialists, been working collaboratively for some time.
Our Epilepsy Group run a regular clinic together with Maternal Medicine
Midwives for all women with Epilepsy delivering at St George's, and there
is a monthly joint Neurology and Obstetric clinic in maternal medicine
where women with complex (and sometimes more simple) neurological
disorders can be advised regarding pregnancy, delivery and breastfeeding.
Careful individualized plans for Neurological (including surgical and
vascular), Obstetric, Anaesthetic and midwifery led care are made for
these women.
We know from prospective data collection (risk also comes from not knowing
what you are doing) that unplanned episodes and adverse outcomes have been
avoided.
We are also aware that similar clinical services are available at other
Hospitals including University College London Hospitals, at Queen
Charlotte's and Chelsea Hospital, part of Imperial College Healthcare NHS
Trust, and has long been available at Birmingham Women's NHS foundation
Trust.
A call from the NHS England Strategic Clinical Networks was recently made
for a Neurologist advisor to join the London Maternity morbidity and
mortality expert panel. This is an opportunity to act and work together
that the clinicians at St George's will not be missing.
Yours sincerely,
Dr Dominic Paviour, Consultant Neurologist
Dr Ingrid Watt Coote, Consultant in Obstetrics and Maternal Medicine
Dr Hannah Cock, Consultant Neurologist and Epileptologist,
Ms Amanda Reeve, Clinical Nurse Specialist, Atkinson Morley Epilepsy Group
Trudy Williams, Specialist Midwife in Maternal Medicine
St George's University Hospital NHS Foundation Trust, London
References:
1. Death in pregnancy: a call for neurological action
John Paul Leach, Pract Neurol 2015;15:244-245 doi:10.1136/practneurol-2015
-001097
The outlined model of cooperative and integrative services for
pregnant women with neurological conditions is warmly welcomed (and not a
little envied!). While such services are provided the English centres
mentioned, it is sad that such high quality provision remains patchy and
incomplete. This nettle-grasping should no longer be quiet, but should be
loudly heralded and made the norm. The recently updated SIGN guideline...
The outlined model of cooperative and integrative services for
pregnant women with neurological conditions is warmly welcomed (and not a
little envied!). While such services are provided the English centres
mentioned, it is sad that such high quality provision remains patchy and
incomplete. This nettle-grasping should no longer be quiet, but should be
loudly heralded and made the norm. The recently updated SIGN guidelines
recommend a joint approach from all corners, and disparate organisations
such as the ILAE, the ABN, the Medical Royal Colleges, and the RCOG need
to come together to make such synergy routine. Only then can we ensure
that complications of epilepsy during pregnancy and the first post-partum
year follow other obstetric complications in decreasing incidence.
While reading Pennington et al's (2015) article on Functional
Cognitive Disorder, I was struck by the similarity between their
descriptions and a patient seen in clinic (patient X), with the exception
that X's symptoms appeared over the course of a few days with no
identifiable precipitating event. Extensive physical tests and brain
imaging investigations were carried out and no organic disease was found
yet symptoms we...
While reading Pennington et al's (2015) article on Functional
Cognitive Disorder, I was struck by the similarity between their
descriptions and a patient seen in clinic (patient X), with the exception
that X's symptoms appeared over the course of a few days with no
identifiable precipitating event. Extensive physical tests and brain
imaging investigations were carried out and no organic disease was found
yet symptoms were reportedly severe and having a major impact on X's
functioning. Here I discuss X's case (with identifiable information
changed in order to respect patient confidentiality) and propose that
there may be a sub-type of functional cognitive disorder with an acute
onset.
Patient X is a 48 year old house-wife and local preacher. She
presented to clinic with her husband after being referred by her GP due to
the development of severe confusion and cognitive impairment developing
from a previously high level of functioning just a few days earlier.
Symptoms began to appear on the Sunday evening and peaked on the Tuesday,
with no change between then and the clinic assessment (Thursday). X
reported completely 'losing track of time' and reported severe memory loss
which her husband had first noticed. During the assessment, she claimed
inability to answer any of our questions herself and repeatedly looked to
her husband for answers. When asked why she could not answer the
questions, she said that she 'could not remember' and described problems
with attention, concentration and executive function. This appeared
inconsistent with her performance in daily life as she had managed to
maintain her previous social roles, although we had no objective baseline
for comparison.
X had no previous psychiatric history and no significant medical
history. She was not taking any regular medications except for the oral
contraceptive pill. She reported a normal upbringing. The only
identifiable recent stressor was that X had recently started a new
university course, part of which involved looking at different personality
types, which she said had 'confused' and 'worried' her.
It was first thought that she may have organic disease as her
symptoms came on suddenly, however she returned to psychiatry clinic
following extensive tests as no physical cause could be found. Could this
be a case of functional cognitive disorder with acute onset? Further
assessment and follow-up of patient X and others like her could unravel a
subgroup of patients who fall into this category.
Pennington and colleagues review of Functional Cognitive Disorder
(FCD) [1] is very welcome to guide practice in a complex condition that is
presenting increasingly to general and cognitive neurology clinics.
However, in our view Pennington's presumption towards specialist
assessment, investigation and surveillance is unnecessary and misses an
opportunity to provide patients with a prompt diagnosis, explanation and
reas...
Pennington and colleagues review of Functional Cognitive Disorder
(FCD) [1] is very welcome to guide practice in a complex condition that is
presenting increasingly to general and cognitive neurology clinics.
However, in our view Pennington's presumption towards specialist
assessment, investigation and surveillance is unnecessary and misses an
opportunity to provide patients with a prompt diagnosis, explanation and
reassurance and immediate treatment for the FCD and/or significant
relevant co-morbidities e.g. depression, chronic fatigue syndrome (CFS).
In particular, we cannot agree with Pennington's comment that "Functional
cognitive disorder should be diagnosed only after excluding other causes
of cognitive decline as far as possible". We believe that clinicians
should aim to make a positive "rule in" diagnosis of FCD, exactly as
proposed for other functional disorders [2] and that this can be done in
the context of the general neurology clinic.
Pennington et al rightly point out features in the history that suggest
FCD but in our view they underestimate their significant diagnostic value.
Additional positive features for FCD in our experience are a high number
of presenting symptoms, the patient's emotional response to the symptoms
being more distressing than the actual consequences of any cognitive
lapses, co-morbid mood disorders, chronic pain or fatigue and recent
relevant life events including physical illnesses such as mild traumatic
brain injury, anesthesia or chemotherapy exposure.
Brief cognitive instruments (such as MoCA, ACE) are of limited value in
this context and have an opportunity cost in a 30 minute general neurology
consultation. Instead we suggest listening for particular features in the
patient's spontaneous speech that point towards FCD. A richly detailed
history in which multiple examples of cognitive lapses are described, and
in which events are precisely located in time, the patient's ability to
reference an earlier part of the consultation [4], and normal expressive
language (e.g. the absence of word finding pauses) are all evidence of
normal cognitive function. These can be fed back to the patient to help
explain the diagnosis, in the same way that a positive Hoover's sign can
be explained to a patient with functional lower limb weakness.
We argue that most patients with suspected FCD do not need investigations
such as brain imaging or neuropsychology. These are much more likely to
produce false positives with attendant iatrogenic harm than to shed any
light on the diagnosis, particularly in younger patients where the pre-
test probability of dementia is extremely low. The authors write that
"Cerebral atrophy that is out of keeping for the patient's age ... should
prompt further investigations". We would regard lower than expected brain
volume as a non-specific finding in a patient with typical FCD symptoms.
The temptation to perform further tests to clarify non-specific findings
from a previous test demonstrates the perils of embarking on low yield
investigations in the first place. Tests for anti-VGKC and NMDA receptor
antibodies should be reserved for patients presenting with the clinical
syndromes associated with these antibodies, which are readily
differentiated from FCD on the basis of the clinical history.
We believe that routine neuropsychological assessment for the diagnosis of
FCD is clinically unnecessary, not cost-effective and potentially
misleading. For example, a neuropsychology report stating that "early
neurodegeneration cannot be ruled out" in a case where the clinician
strongly suspects FCD may reduce patients' confidence in the diagnosis.
We would virtually never consider amyloid positron emission tomography or
cerebrospinal fluid biomarkers appropriate for patients with suspected FCD
(although they may have a role in rare cases in which a diagnosis of
Alzheimer's disease needs to be changed to FCD and this is not initially
accepted by patients, family and other healthcare professionals[5]).
These tests are abnormal only in Alzheimer's disease so cannot "rule out"
dementia in any case.
We welcome Pennington's comments about the need to develop treatments for
FCD. One of us (JAC) often recommends two books to patients that
beautifully illustrate the idiosyncrasies of normal memory; Forgetting by
Daaisma and Moonwalking with Einstein by Joshua Foer. For patients with
mild FCD, normalising their symptoms e.g. by explaining how we (the
neurologist) make many of the same mistakes can be helpful. An emerging
nosology can help practitioners select an individualised explanatory model
and prioritise treatment i.e. (1) FCD in isolation, with or without
dementia-related health anxiety, (2) FCD in the context of a psychiatric
co-morbidity (most typically depression) and (3) FCD as a feature of
another functional disorder e.g. CFS, fibromyalgia (adapted from [6]).
The stability of functional neurological diagnoses is well demonstrated
and the same likely applies to FCD, as Pennington and colleagues point
out. We therefore disagree with the suggestion that it is necessary to
follow patients up to ensure that their cognitive function remains stable.
Follow-up might of course be offered for other reasons e.g. where the
clinician needs more time to explain the diagnosis.
In summary, while welcoming Pennington and colleagues important
contribution to this emerging area of neurological practice, we urge
general neurologists to have confidence in making a positive diagnosis of
FCD, particularly in younger patients. We believe that unnecessary
specialist referral, investigation and surveillance of patients with FCD
risks delaying treatment and potentially incorrect diagnoses of pre-
dementia (e.g. MCI) or dementia itself.
References
1. Pennington C, Newson M, Hayre A, et al. Functional cognitive disorder:
what is it and what to do about it? Pract Neurol 2015;15:436-444
2. Stone J. Functional neurological disorders: the neurological assessment
as treatment. Neurophysiol Clin 2014;44:363-373
3. Stone J, Reuber M, Carson A. Functional symptoms in neurology: mimics
and chameleons. Pract Neurol 2013;13:104-113
4. Jones D, Drew P, Elsey C, et al. Conversational assessment in memory
clinic encounters: interactional profiling for differentiating dementia
from functional memory disorders. Aging Ment Health 2015;
DOI:10.1080/13607863.2015.1021753
5. Coebergh JA, Wren DR, Mumford CJ. 'Undiagnosing' neurological disease:
how to do it, and when not to. Pract Neurol 2014;14:436-439
6. Stone J, Pal S, Blackburn D. Functional (Psychogenic) Cognitive
Disorders: A Perspective from the Neurology Clinic. J Alzheimers Dis
2015;48 Suppl 1:S5-S17
We welcome debate around this emerging condition. We all recognise a
situation when as clinicians we have had a strong feeling that a patient
has a functional complaint from early on in the consultation. However, the
differential diagnosis for Functional Cognitive Disorder is
neurodegenerative dementia which itself affects behaviour and personality
and could, therefore, influence many of the cues clinicians pick up on
wh...
We welcome debate around this emerging condition. We all recognise a
situation when as clinicians we have had a strong feeling that a patient
has a functional complaint from early on in the consultation. However, the
differential diagnosis for Functional Cognitive Disorder is
neurodegenerative dementia which itself affects behaviour and personality
and could, therefore, influence many of the cues clinicians pick up on
when "divining" a functional diagnosis.
Anecdotally, we have seen patients in our clinic diagnosed with a
functional disorder at other centres whom we have subsequently discovered
to have definite neurodegenerative disease. As an example, we were
referred a patient thought to have a psychiatric disorder and functional
left leg movements. After a detailed assessment including CSF biomarkers
and SPECT scanning, we suspected organic disease. His positive C9orf72
genetics have recently demonstrated he suffers from a genetic form of
Frontotemporal dementia and we believe that this altered his behaviour
such that he manifested a functional syndrome at the start.
Preceding medical events such as general anaesthesia or chemotherapy can
be triggers for health anxiety or FCD, but could also be a source of
cognitive symptoms. General anaesthesia (particularly if performed for
cardiac indications) is a risk factor for hypoxic or ischaemic brain
damage (which if focal can present with isolated cognitive deficits).
Chemotherapy agents can cause neurological complications (e.g.
methotrexate encephalopathy); their use also implies a history of
malignancy with consequent risk of tumour recurrence and direct or
indirect neurological complications.
Establishing the aetiology of cognitive complaints is the key challenge in
the memory or general neurology clinic. Large-scale research studies
suggest there is a continuum between Subjective Cognitive Impairment
(where individuals report cognitive symptoms but perform normally on
testing), MCI and dementia2. Given treatments may be effective only in the
earliest stages, identifying prodromal dementia will in the future be
increasingly important. Distinguishing incipient dementia at the Mild
Cognitive Impairment (MCI) phase from Functional Cognitive Disorder (FCD)
can be difficult, but is of vital importance to ensure patients receive
appropriate prognostic information including reassurance where necessary
and are entered into clinical trials where appropriate. This distinction
becomes particularly tricky in high-functioning individuals, and those
with co-morbid psychiatric conditions. The clinical history is very
useful, and conversational analysis has been successfully used to
differentiate dementia from FCD1. However we would counsel against solely
using the clinical history to distinguish FCD from incipient dementia at
the MCI phase. We know that anxiety, depression and abnormal behaviour may
occur in early dementia all of which might confound use of a clinical
history to differentiate incipient dementia from FCD.
Neuropsychology assessments are very useful in this context- they can
reveal patterns of deficits that are not consistent with organic
pathology, deficits out of keeping with FCD, or highlight significant
psychiatric symptoms warranting referral to psychiatry or psychology
services. Neurologists are often not trained in identifying psychiatric
disorders, therefore a structured neuropsychology assessment including
questionnaires looking for symptoms of depression or anxiety can often be
very helpful.
The authors would regard neuroimaging as an essential part of the
assessment, as recommended by NICE3. Even a patient with a 'classic' FCD
history may be harbouring structural brain pathology. To give a clinical
example, a 56 year old man presented to our general neurology clinic with
intermittent word finding difficulties during conversations and when
typing. He had a history of anxiety disorder, and his language appeared
normal during the consultation and on formal assessment. Neurological
examination was normal, and a functional cause was suspected. However on
MR imaging a large glioblastoma multiforme was found. Not performing
neuroimaging risks missing individuals with a strategic infarct,
malignancy or other structural pathology. Whilst such cases are not
common, missing such a diagnosis would have very serious consequences for
the patient.
The authors agree that investigations such as SPECT, CSF biomarkers and
autoantibodies should be used selectively, but these can be helpful in
difficult cases. We plan to explore further the additive clinical value of
each of these tests.
Discovering the aetiology of cognitive symptoms often requires us to
operate at the limits of current knowledge about how the brain guides
behaviour. We do not believe we are at the point where we can always offer
certainty to a patient. However, we find it possible to reassure patients
effectively even when we represent this uncertainty to them. If we suspect
functional cognitive disorder, we introduce the concept at the first
consultation and offer reassurance saying we will do further tests and
follow up to rule out rarities. We find this is actually quite effective
as a means of reassurance, perhaps more so than being entirely dogmatic
that there cannot be organic pathology as most patients will know it is
more or less impossible to prove a negative in neurology. Overall we feel
that whilst we might strongly suspect FCD clinically at the first
consultation, it is unwise in almost all cases to make a firm diagnosis of
FCD without appropriate investigations. Even after diagnosis and initial
management of FCD, a period of clinical follow up can be informative. It
is only such follow up that has allowed us to begin to understand critical
components of FCD and to summarise clinical features of the condition.
References
1. Elsey, C., Drew, P., Jones, D., Blackburn, D., Wakefield, S., Harkness,
K., Venneri, A. & Reuber, M. Towards diagnostic conversational
profiles of patients presenting with dementia or functional memory
disorders to memory clinics. Patient Educ. Couns. pii: S0738, (2015).
2. Garcia-ptacek, S., Cavallin, L., Kramberger, M. G., Winblad, B., Jelic,
V. & Eriksdotter, M. Subjective Cognitive Impairment Subjects in Our
Clinical Practice. 419-430 (2014). doi:10.1159/000366270
3. NICE. Dementia diagnosis and assessment. (2015).
Sir,
I read with much interest the review by Weerasinghe and Lueck on the
diagnosis of optic neuritis. The authors provide an extensive and detailed
analysis on the differential diagnoses and the management of the disease.
The importance of retinal electrophysiology is also acknowledged when
differentiating retinal disorders that can mimic optic neuritis.
Surprisingly, however, the diagnostic role of pattern-reversal Visua...
Sir,
I read with much interest the review by Weerasinghe and Lueck on the
diagnosis of optic neuritis. The authors provide an extensive and detailed
analysis on the differential diagnoses and the management of the disease.
The importance of retinal electrophysiology is also acknowledged when
differentiating retinal disorders that can mimic optic neuritis.
Surprisingly, however, the diagnostic role of pattern-reversal Visual
Evoked Potentials (VEPs) is not discussed, not even mentioned. VEPs can
assess visual pathway function at a millisecond time scale and no other
visual function measure shares this temporal sensitivity. More than 40
years ago Martin Halliday demonstrated a delay in the VEPs after an attack
of optic neuritis. Since then, a huge number of studies have provided
converging evidence about the importance of VEPs recording in optic
neuritis and Multiple Sclerosis (MS).
Doubtless, the clinical use of VEPs has changed over time and the advent
of magnetic resonance imaging (MRI) have reduced the use of VEPs in
clinical practice. However, even in the MRI era, an abnormal VEP in
unaffected eyes of patients with MS provides evidence for clinically
silent demyelinating lesions in the optic pathway. This, in turn, is
extremely useful in identifying dissemination in space and, therefore, in
establishing the diagnosis of MS.
In their review, the authors also fail to mention another important
electrophysiological test, i.e. the Pattern Electroretinogram (PERG). The
PERG is generated by the activity of the retinal ganglion cells. Taken
together, PERG and VEPs are useful to identify the site(s) of dysfunction
along the retinocortical pathway. In a typical case of optic neuritis PERG
and VEP recordings might not be necessary. However, these techniques can
still retain many roles: first, to show the magnitude of conduction block
of the optic nerve fibres in the acute stage of the disease. Secondly, to
demonstrate the optic nerve conduction delay due to demyelination. Third,
to follow remyelination, which is seldom correlated to an improvement in
visual acuity, through the shortening of VEP latency. Finally, to define
the eventual retrograde degeneration of ganglion cells, which is reflected
by an amplitude reduction of the PERG N95 peak. Moreover, when diagnosing
atypical optic neuritis, these neurophysiologic techniques should be
implemented as a part of a comprehensive assessment.
Of note, the visual pathway can now be more precisely evaluated in detail
by means of new neurophysiological tests (multifocal VEPs and ERG). These
techniques provides higher sensitivity and specificity in detecting
abnormalities in visual function in optic neuritis and MS
I read with interest this work ..indeed nerve
ultrasonography has other parameters more than cross section area
measurements in detection of the pathological changes and prediction of
outcome of the treatment (medical or surgical}...the normal nerve as seen
by high resolution ultrasonography is:
1- oval shaped or round with high
ability to change its shape in response to applied pressure easi...
I read with interest this work ..indeed nerve
ultrasonography has other parameters more than cross section area
measurements in detection of the pathological changes and prediction of
outcome of the treatment (medical or surgical}...the normal nerve as seen
by high resolution ultrasonography is:
1- oval shaped or round with high
ability to change its shape in response to applied pressure easily seen by
dynamic study during flexion and extension of the limbs
2- the nerve
according to the number of fascicles has a normal fascicular pattern
3- it
has the ability to change its position according to the force applied on
it during limb movement this excursion can be calculated by the dynamic
study
4- in response to exercise it reacts in the form of increase its
cross section area and increase signals from its blood vessels
5- in
response to the applied pressure the cross section area is reduced by 20-30% with flattening of the contour so by applying all these parameters the
subtle changes can be detected first = the loss of fascicular pattern which
seen with long standing pathology can be detected and its length can be
measured = second the loss of compressibility by applied pressure [most
probably due to fibrosis and new short nerve fiber growth] can be easily
measured and the less the compressibility the poorer the outcome of
treatment third = loss of normal nerve excursion during limb movement on
the dynamic study due either intrinsic changes or surrounding fibrosis
fourth the focal nerve changes can be estimated and the change in the
volume by measuring the affected segment length and the cross section area
can calculated and used for follow up ....so the cross section area as
single criterion do not explain the recurrent cases of the operated
cubital tunnel syndrome or the poor response to the operation in
conservative cases finally to use the gray ultrasound alone the
echotexture of the nerve with applied stress compressibility test together
with the cross sectional area in rest and on dynamic stress condition
should be used in all examined cases thanks
I felt rather uncomfortable with some of the articles in the August
2006 issue of the Practical Neurology and it was not because I was reading
the journal in a sultry afternoon of Indian summer.
Andrew Chancellor was
wrong in invoking a clinical diagnosis of multiple sclerosis in his fourth
problem (Test Yourself, p260). The diagnosis of multiple sclerosis (MS)
cannot be made without objecti...
I felt rather uncomfortable with some of the articles in the August
2006 issue of the Practical Neurology and it was not because I was reading
the journal in a sultry afternoon of Indian summer.
Andrew Chancellor was
wrong in invoking a clinical diagnosis of multiple sclerosis in his fourth
problem (Test Yourself, p260). The diagnosis of multiple sclerosis (MS)
cannot be made without objective clinical findings, even if objective
paraclinical findings are in place (1). In the given case, one would only
suspect the possibility of demyelination or a "clinically vague syndrome"
(1).
We should be aware of the inherent pitfall of overdiagnosis of MS
and its consequences. In their commentary, Gold and Hohfield (2) avoided
the key issue of selection of MS patients for monoclonal antibody based
interventions in clinical practice. Given the unknown risks of long-term
therapy, the benefit would seem less certain in patients with low
disability and relatively stable disease (3). However, nearly two-thirds
of recruited patients in natalizumab trials (1801 and 1802) had low
disability (Kurtzke’s EDSS score 0-2.5), only a minority had serious
clinical relapses during treatment (7 and 13% in the treated and placebo
arms respectively in the monotherapy trial), and absolute reduction of
disease progression was rather modest (between 6 and 12%). I am uncertain
if the trial data can be extrapolated to the general population of the MS
clinic notwithstanding the regulatory approval of the drug.
My disappointment did not get any better with the review of ulnar
neuropathy by John Stewart (4). Worldwide, probably the commonest cause of
ulnar neuropathy -and its disabling serious motor complication-is leprosy,
not habitual elbow leaning. Leprosy only appears at the tail of Table 2,
with no mention about its clinical diagnosis and management, and the fact
that leprosy is possibly the only example of peripheral (ulnar) nerve
abscess. A picture of Hansen, rather than the sculpture of Rodin would
have been more appropriate, but I have enclosed a picture (courtesy: World
Health Organisation) of an ulnar
neuropathic hand of leprosy after reconstruction surgery. Maybe the
editor would commission an article on this topic in the future to rectify
the omission. As one of the junior colleagues has written it, I would
prefer not commenting on some of the misconceptions regarding the immunity
of Guillain-Barre syndrome (GBS)(5).
Passive transfer of anti-ganglioside
antibodies has never capitulated the nerve pathology of GBS and anti-GQ1b
antibody does not explain the known pathology of cerebellar ataxia in
Miller Fisher syndrome. It may also be important to draw the attention
of the readers to the not-so-infrequent findings of liver function test
abnormalities and the commonly overlooked association of GBS with acute
glomerulonephritis(6-10). Plasma exchange or human intravenous
immunoglobulin should be started in an eligible patient once the diagnosis
is clinically secure and treatment must not be delayed for peripheral
nerve electrophysiology (Figure 3, p214). The risk of relapsing GBS is
also higher in treated patients but the relapses respond well to
treatment.
Rather than writing individual responses, I thought it might not be
inappropriate to write a jobbing neurologist’s feedback to the editor and
I hope to be excused if my response falls outside the rules of the book.
Figure
References
1. Polman CH, Reingold SC, Edan G, et al. Diagnostic criteria for
multiple sclerosis: 2005 revisions to the "McDonald criteria". Ann Neurol
2005; 58: 840-6.
2. Gold R, Hohlfield R. Multipe sclerosis therapy: new agents carry
new risks. Practical Neurology 2006; 6: 248-51.
3. Chaudhuri A. Lessons for clinical trials from natalizumab in
multiple sclerosis. BMJ 2006; 332: 416-419.
4. Stewart J. Ulnar neuropathies: where, why and what to do. Practical
Neurology 2006; 6: 218-229.
5. Pritchard J. What’s new in Guillain-Barre syndrome? Practical
Neurology 2006; 6: 218-217.
6. Behan PO, Lowen stein LM, Stilmant M, Sax DS. Landry-Guillain-
Barre-Strohl syndrome and immune-complex nephritis. Lancet 1973; 1: 850-4.
7. Talamo TS, Borochovitz D. Membranous glomerulonephritis associated
with the Guillain-Barre syndrome. Am J Clin Pathol 1982; 78: 563-6.
8. Bettinelli A, Giani M, Rossi L, et al. Ex novo episodes of acute
glomerulonephritis and Guillain-Barre syndrome: a case report. Clin
Nephrol 1989; 31: 269-73.
9. Olbricht CJ, Stark E, Helmchen U, Sculze M, Brunkhorst R, Koch KM.
Glomerulonephritis associated with inflammatory demyelinating
polyradiculoneuropathy: a case report and review of the literature.
Nephron 1993; 64: 139-41.
10. Emsley HC, Molloy J. Inflammatory demyelinating polyneuropathy
associated with membranous glomerulonephritis and thrombocytopenia. Clin
Neurol Neurosurg 2002; 105: 23-6.
I very much enjoyed Dr Stewart’s informative article on ulnar
neuropathies in the August issue of the journal,1 which included images of
Rodin’s The Thinker to demonstrate that elbow flexion may increase the
likelihood of nerve damage.
May I suggest the painting of Saint Bartholomew of 1661 (see fig) by
Rembrandt van Rijn (1606–69) in the J Paul Getty Museum, Los Angeles, as a
possible exam...
I very much enjoyed Dr Stewart’s informative article on ulnar
neuropathies in the August issue of the journal,1 which included images of
Rodin’s The Thinker to demonstrate that elbow flexion may increase the
likelihood of nerve damage.
May I suggest the painting of Saint Bartholomew of 1661 (see fig) by
Rembrandt van Rijn (1606–69) in the J Paul Getty Museum, Los Angeles, as a
possible example of ulnar neuropathy? Wasting of the first dorsal
interosseous muscle seems evident in the apostle’s left hand.
In the differential diagnosis of ulnar neuropathy, camptodactyly (bent
little finger) might also be included. Although there are no motor or
sensory signs, patients may sometimes be referred for neurophysiological
studies to exclude ulnar neuropathy because of the clawed hand posture.2
REFERENCES
1. Stewart J. Ulnar neuropathies: where, why, and what to do? Practical
Neurology 2006;6:218–29.
2. Larner AJ. Camptodactyly in a neurology outpatient clinic. Int J Clin
Pract 2001;55:592–5.
I must thank Dr Smith for his entertaining and informative case study of van Gogh. (1) It pains me to ask for caution to be used when interpreting his findings, (particularly as I love a good pun for a title).
Arnold and Loftus in 1991 calculated that one must drink 182 litres of absinthe to induce xanthopsia. (2,3,4) After that lethal load, one can only speculate that it may be difficult for Mr V...
I must thank Dr Smith for his entertaining and informative case study of van Gogh. (1) It pains me to ask for caution to be used when interpreting his findings, (particularly as I love a good pun for a title).
Arnold and Loftus in 1991 calculated that one must drink 182 litres of absinthe to induce xanthopsia. (2,3,4) After that lethal load, one can only speculate that it may be difficult for Mr VG to find his muse until the affects of green liquid had thoroughly worn off.
Arnold and Loftus do make a good case for acute intermittent porphyria. (5) However as mentioned by Dr Smith, the presence of digitalis in not one but two portraits of his physician, Gachet, just seems too
perfect to ignore.
Rhys Thomas
Medical SHO, Gloucestershire Royal Hospital
Competing Interests: I have enjoyed a glass of absinthe in Prague - but it did not cloud my judgement.
References
1. Smith PEM, Absinthe attacks, Practical Neurology 2006;6:376-381
2. Wolf P, Creativity and chronic disease Vincent van Gogh (1853-1890) West J Med. 2001 November; 175(5): 348
3. Arnold WN and Loftus LS, Xanthopsia and van Gogh's yellow palette,Eye. 1991;5 (Pt 5):503-10
4. Arnold W, The Illness of Vincent Van Gogh, Journal of the History of the Neurosciences, 2004, Vol 14: No 1, pp 22-43
5. Loftus LS and Arnold WN, Vincent van Gogh's illness: acute intermittent porphyria? BMJ. 1991 December 21; 303(6817): 1589–1591
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I must thank Dr Smith for his entertaining and informative case study of van Gogh. (1) It pains me to ask for caution to be used when interpreting his findings, (particularly as I love a good pun for a title).
Arnold and Loftus in 1991 calculated that one must drink 182 litres of absinthe to induce xanthopsia. (2,3,4) After that lethal load, one can only speculate that it may be difficult for Mr V...
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