We would like to comment the letter of Voermans et al. [1]
Localised scleroderma is characterised clinically by the presence of
thickened, sclerotic skin lesions. However, skin changes [2] sometimes
present in a discrete manner with only indurated, hyper- and hypopigmented
areas. Thus, the clinical diagnosis is delayed.
With regard to neuromuscular manifestations of linear scleroderma, a
m...
We would like to comment the letter of Voermans et al. [1]
Localised scleroderma is characterised clinically by the presence of
thickened, sclerotic skin lesions. However, skin changes [2] sometimes
present in a discrete manner with only indurated, hyper- and hypopigmented
areas. Thus, the clinical diagnosis is delayed.
With regard to neuromuscular manifestations of linear scleroderma, a
muscle biopsy is necessary for diagnosis, especially in case of absent
skin lesions. We aggree with Voermans et al., who suggested preceding
imaging (MRI) in order to detect active sites of inflammation and thus
determine the appropriate site for the biopsy to get valuable histological
results.
Voermans et al. reported that linear scleroderma often presents
without high titer of antinuclear antibodies. This may be true and is
sometimes also our clinical experience. Nevertheless, according to the
literature [3] we recommend laboratory testing of ANA, because in absence
of sclerotic skin changes ANA may be the clue to diagnosis.
Regarding the treatment of linear scleroderma, phototherapy (UVA1)
and photochemotherapy (PUVA and bath-PUVA) are both equal antifibrotic
treatments for skin sclerosis. Both activate matrix metalloproteinases and
soften the fibrotic skin. Moreover, cutaneous immune responses are
suppressed and the fibrotic process is inhibited. [4]
Because UVA1 reaches into the subcutis only, those subtypes of localised
scleroderma with neuromuscular involvement require early and additional
systemic immunosuppressive therapy. As the most encouraging approach
according to our clinical experience and by the review of the literature,
we recommend initial high dose systemic corticosteroids (1mg/kg body
weight) in order to stop immediately the inflammation. Furthermore, in
patients with severe disease and those with extracutaneous involvement,
methotrexate should be added as a corticosteroid sparing agent and
immunosuppressive drug. [5]
Both cases [2;6] illustrate for the first time that the involvement
of deeper tissues in linear scleroderma and their clinical course may be
independent from skin involvement which may be responsible beside other
cirumstances for delay in diagnosis. Only early and immediate intervention
in this disease is promising in order to prevent the terminal stage of
fibrosis and disability.
References
[1] Voermans N.C. Striking streaks in linear scleroderma.
J. Neurol. Neurosurg. Psychiatry, 4 Mar 2009
[2] Bockle BC, Willeit J, Freund M, Sepp NT.
Neurological picture. Unexplained muscle atrophy as the unique preceding symptom of bilateral linear morphea.
J. Neurol. Neurosurg. Psychiatry 2009; 80: 310-311.
[3] Takehara K, Sato S. Localized scleroderma is an autoimmune disorder.
Rheumatology. (Oxford) 2005; 44: 274-279.
[4] Brenner M, Herzinger T, Berking C, Plewig G, Degitz K. Phototherapy and photochemotherapy of sclerosing skin diseases.
Photodermatol. Photoimmunol. Photomed. 2005; 21: 157-165.
[5] Zulian F. New developments in localized scleroderma.
Curr. Opin. Rheumatol. 2008; 20: 601-607.
[6] Voermans NC, Pillen S, de Jong EM, Creemers MC, Lammens M, van
Alfen N. Morphea profunda presenting as a neuromuscular mimic.
J. Clin. Neuromuscul. Dis. 2008; 9: 407-414.
Funding sources: None.
Conflict of interest: None declared.
This interesting study showed that up to 68% of patients complained
of significant work and social dysfunction due to stroke. This study has
many implications. The number of people who survive stroke and live with
its consequences is increasing. The case fatality rate for stroke has
declined over the past few decades,and many of people who experience a
stroke now survive.This increase in survival...
This interesting study showed that up to 68% of patients complained
of significant work and social dysfunction due to stroke. This study has
many implications. The number of people who survive stroke and live with
its consequences is increasing. The case fatality rate for stroke has
declined over the past few decades,and many of people who experience a
stroke now survive.This increase in survival has necessitated a new
approach to measuring the health outcomes associated with stroke. We
should concern more about the domains of quality of life. It has been
reported that patients' views of what constitute important outcomes are
not always identical to those of health-care professionals. This study
should remind stroke physicians to concern more about the social well
being for stroke survivors.
Rogers et al. make several points. The primary aim of our review was
to search in a systematic manner for associations between retinal
microvascular abnormalities and stroke subtypes and thus provide clues to
the microvascular pathophysiology of lacunar stroke. We aimed to include
all relevant studies (not just the large epidemiological ones), carefully
evaluate clinical and retinal features for c...
Rogers et al. make several points. The primary aim of our review was
to search in a systematic manner for associations between retinal
microvascular abnormalities and stroke subtypes and thus provide clues to
the microvascular pathophysiology of lacunar stroke. We aimed to include
all relevant studies (not just the large epidemiological ones), carefully
evaluate clinical and retinal features for comparability and we adopted as
inclusive a policy as possible to provide as much information as possible.
Rogers et al, quoting the Cochrane Handbook for Interventional
Studies, highlight the difficulties encountered when there is possible
study population duplication resulting from multiple publications from a
single cohort of patients. Observational study data require a slightly
different approach to that used for interventional studies (see http://www.equator-network.org/). We extracted and aimed to include data
from the same studies where we were interested in different subgroups.
Space restrictions did not permit us the luxury of detailing exactly what
the groups were or the difficulties involved in identifying possible
duplication. The advantage of using Forrest Plots is that the data from
each individual paper are not lost and the reader can assess the likely
effects of removing single studies. This is the recommended method for
presenting results of this type
(http://www.cochrane.org/resources/handbook/). We should emphasize that
in the context of this review the individual study point estimates are
more important than the pooled risk ratio. We re-analysed the data, as
suggested by Rogers et al, including one paper from each study and found
that the associations did not change – retinopathy was associated with
incident stroke with a summary risk ratio of 2.0 (95% CI 1.6-2.5)
(excluding possible duplicates Cheung 2007 and Wong 2002) and with
prevalent stroke - sRR of 2.9 (95% CI 2.0-4.1) (excluding Longstreth
2006).
For this review we used a wide definition of stroke (clearly
specified in our paper) to encompass the range of definitions used in the
papers. Whilst the vast majority of papers did not use the reference
standard for stroke diagnosis (indeed very few did) we considered that
there was sufficient clinical homogeneity to permit grouping of the
outcome of “stroke” and clearly discuss the limitation that this imposes
on our conclusions. In fact, we highlight the heterogeneity as well as the
lack of clarity and reliability of the diagnosis of “stroke” used in these
papers as one of the main problems affecting this area of research and a
major area for methodological improvement in future studies. If we had
excluded those which did not conform to the gold standard there would have
been virtually no data, and the exercise would have been unhelpful in
flagging the fact that better characterisation of the cerebral disorder is
needed to match the very detailed classification and characterisation of
the retinal one. In the discussion we note the heterogeneity present in
these studies and urge caution in the over-interpretation of the pooled
estimates.
Rogers et al mention the use of unadjusted and adjusted estimates.
Whilst not immediately applicable as it refers to interventional rather
than observational studies, the Cochrane Handbook states that “no general
recommendation can be made for the selection of which adjusted estimate is
preferable” and subsequently advises using the estimate from the model
that adjusted for the maximum number of co-variates”. It would have been
invidious for us to use unadjusted data (these would have produced
spuriously large apparent associations with “stroke”), when many of the
retinal findings are known to be associated with hypertension or diabetes
or other vascular risk factors. Indeed, many studies only presented
adjusted data. Rogers et al are correct to state in theory that the
conditional estimates (adjusted) are larger than population average
estimates (unadjusted) but this was not the case in this review. For
example 6 out of 8 papers studying retinopathy and incident stroke found
that the unadjusted estimates were larger than the adjusted estimates
possibly because retinal abnormalities and stroke are both co-associated
with vascular risk factors. We adopted what we felt to be the most
clinically appropriate method to avoid exaggerating the association
between retinal findings and stroke.
For the construction of the Forrest plots we did not calculate the
risk ratios ourselves from the raw data but used the point estimates and
standard errors from the ratios and 95% confidence intervals presented in
the papers.
Other factors mentioned by Rogers, such as time to or since stroke,
were often not given in the primary papers. However, this in itself should
not preclude an effort to pull together all relevant literature to take
stock of the current evidence base, identify study quality issues which
might limit the conclusions and which should be addressed in future work,
and attempt to draw conclusions as to the presence and strength of any
association with “stroke”.
Therefore, we believe that, within the confines of the existing our
systematic review has been useful. We found a paucity of papers studying
retinal changes in subtypes of ischaemic stroke and two studies which
address this deficit are now nearing publication. We found a lack of
adequate characterisation of even ischaemic versus haemorrhagic stroke,
but allowing for this methodological “noise”, certain retinal features
appeared to be associated with stroke and are certainly worthy of further
study.
References
Cheung N, Rogers S, Couper DJ et al. Is diabetic retinopathy an
independent risk factor for ischemic stroke?
Stroke 2007;38(2):398-401.
Wong TY, Klein R, Sharrett AR et al. Cerebral white matter lesions,
retinopathy, and incident clinical stroke.
Journal of the American Medical
Association 2002;288(1);67-74.
Longstreth W, Jr., Larsen EK, Klein R et al. Associations between
findings on cranial magnetic resonance imaging and retinal photography in
the elderly: the Cardiovascular Health Study.
Am J Epidemiol
2007;165(1):78-84.
De Jonghe raises an extremely important point about the difficulties
of interpreting cognitive data from patients with delirium. ‘Inattention’
is one of the core diagnostic criteria for delirium. Accordingly, the
attentional deficits with which that these patients present are likely to
compromise performance on a wide range of cognitive tasks, irrespective of
whether they also have specific deficits...
De Jonghe raises an extremely important point about the difficulties
of interpreting cognitive data from patients with delirium. ‘Inattention’
is one of the core diagnostic criteria for delirium. Accordingly, the
attentional deficits with which that these patients present are likely to
compromise performance on a wide range of cognitive tasks, irrespective of
whether they also have specific deficits in the particular cognitive
function that is being tested. As de Jonghe rightly suggests, one
challenge for delirium researchers is therefore to find ways of assessing
whether specific domains of cognitive impairment can be still be
identified over and above the effects of these core attentional
impairments.
We suggest that there are at least two ways in which one can attempt
to control for the effects of attentional deficits when assessing specific
cognitive functions. One, as de Jonghe alludes to, is to take an
independent measure of attention that can then be used as a covariate when
analysing performance on a given cognitive test. Any group differences in
performance that cannot be accounted for by performance on the attention
task(s) can then more reliably be attributed to the specific cognitive
domain being examined. However, this approach assumes that reliable and
valid measures of attention can be easily obtained, which is not
necessarily the case. The term ‘attention’ does not refer to one unitary
cognitive function. Rather, it encompasses a heterogeneous group of
cognitive operations that include the maintenance of an alert state, the
selection of targets from the environment, and the orientation of
processing resources to relevant stimuli (Posner, 2008). Any single test
of attention will therefore only capture a fraction of these abilities. As
the specific nature of the attentional deficits underlying ‘inattention’
in delirium have not yet been well circumscribed, it is difficult to
obtain a ‘key’ measure of attention that can accurately capture this
phenomenon.
A second approach to controlling for the effects of attentional
deficits is to select cognitive tasks in which the attentional demands are
minimal. This is the approach that we deliberately took when assessing
visual perception in delirium. The perceptual tasks that we used all
allowed long exposure to the stimulus materials, contained simple
instructions that could be repeated when necessary, and did not require
speeded responses, thereby making them apparently less sensitive to the
gross attentional problems that patients with delirium typically show. Of
course, it is impossible to completely eliminate the role of attention in
these tasks. Indeed, as we explain in the discussion section of our
article, attentional processes are fundamentally involved in various
stages of the perceptual processing system. It is therefore perhaps folly
to even assume that perceptual processes ever could, or should, be
considered in true isolation of an individual’s attentional functioning.
Nevertheless, by reducing the attentional demands of the cognitive tests
in the way that we have described, we can at least be more certain that
patients’ performance on the tasks need not have been compromised by any
immediate behavioural effects of inattention, such as being unable to
constantly fixate on task material throughout the testing period.
In sum, we agree wholeheartedly with de Jonghe that it is important
that the role of attention is considered when studying cognitive processes
in delirium. We suggest that there are at least two ways by which the
effects of ‘inattention’ can be controlled for. However, perhaps
additional basic research on the fundamental neuropsychological deficits
in delirium is required before strong conclusions can be drawn regarding
the interdependencies of performance among various putative cognitive
domains.
Reference
1) Posner, MI. Measuring alertness.
Ann N Y Acad Sci 2008;1129:193-9.
With great interest we read the article by Böckle et al (1), who
report a patient with muscle atrophy preceding bilateral linear morphea.
We would like to confirm the importance of recognizing linear scleroderma
as an unusual cause of focal muscle atrophy. Recently we described a
similar case (2) and would like to comment on various aspects of Böckle’s
report.
With great interest we read the article by Böckle et al (1), who
report a patient with muscle atrophy preceding bilateral linear morphea.
We would like to confirm the importance of recognizing linear scleroderma
as an unusual cause of focal muscle atrophy. Recently we described a
similar case (2) and would like to comment on various aspects of Böckle’s
report.
Their case is unusual since the myopathy preceded the skin changes,
and because the muscle atrophy was unrelated to the site of the skin
involvement. Our patient suffered from progressive muscle atrophy while
skin changes had been in remission for years. Hence, muscle involvement in
linear scleroderma may occur independently of the sclerotic skin changes,
both in time and in location. The authors describe that EMG at onset
revealed neuropathic changes in the triceps brachii muscle. We performed
subsequent EMGs in several muscles of our patients affected limb, but
found predominantly myopathic motor units, with short-duration and low-
amplitude units and spontaneous activity (fibrillations, positive spikes,
and complex repetitive discharges). In fact, the amount of spontaneous
activity matched the clinical course and therapy effect in our patient.
The muscle biopsy in Böckle’s report displayed a terminal stage of
muscular damage. In our patient, the first biopsy of the brachioradialis
muscle showed no abnormalities while a second biopsy of the biceps brachii
muscle only revealed end-stage fibrosis. With use of muscle MRI and muscle
ultrasound, the deltoid muscle was selected as the correct site of biopsy,
and here a focal myositis was demonstrated that was decisive for the
diagnosis of linear scleroderma with deep morphea. Not only skin sclerosis
but also muscle inflammation occurs in linear streaks, and sampling error
can easily occur. Therefore, the site of muscle biopsy should be selected
carefully, preferably with the help of muscle imaging.
The patient in Böckle’s report was referred to the outpatients
department for autoimmune disease only when blood analysis revealed a high
titer of antinuclear antibodies, six years after onset of the muscle
weakness of his left triceps. There, he presented with an indurated area
of the back of his right hand and forearm, and skin biopsy was indicative
for linear scleroderma. This course suggests that neurologists may not be
trained to be alert to the dermal changes that were the clue to this
diagnosis. Furthermore, linear scleroderma often presents without high
titer of antinuclear antibodies
Böckle et al report good effect of treatment with balneophototherapy
and methylprednisolon. As our patient did not improve with oral prednisone
and low-dose methotrexate, we had to resort to higher dose subcutaneous
methotrexate in combination with UVA1 phototherapy. This was well
tolerated and resulted in considerable improvement of skin sclerosis and
reduction of contractures. Muscle strength and mass has not improved yet;
this may indicate that the deep morphea has already resulted in end-stage
myopathy.
Linear scleroderma is a striking cause of focal myositis causing
atrophy and weakness, Neurologists should consider this diagnosis in
patients with segmental muscle weakness and atrophy, also when skin
lesions are minimal or not progressive. Serious disability may be
prevented with appropriate immunosuppressive treatment.
References
(1) Böckle BC, Willeit J, Freund M, Sepp NT. Unexplained muscle atrophy as the unique preceding symptom of bilateral linear morphea.
J Neurol Neurosurg
Psychiatry 2009;80:310-1.
(2) Voermans NC, Pillen S, de Jong EM, Creemers MC, Lammens M, van Alfen N Morphea profunda presenting as a
neuromuscular mimic.
J Clin Neuromuscul Dis 2008;9:407-14.
I read with interest the article by Yogarajah et al 1. Working in a
comprehensive epilepsy center of a tertiary referral hospital in the
United States, I found the cost benefit analysis of long term monitoring
(LTM) either by video telemetry (VT) or inpatient ambulatory
electroencephalography (aEEG) useful information indeed. I agree with the
authors that one of the greatest utility of LTM lies in...
I read with interest the article by Yogarajah et al 1. Working in a
comprehensive epilepsy center of a tertiary referral hospital in the
United States, I found the cost benefit analysis of long term monitoring
(LTM) either by video telemetry (VT) or inpatient ambulatory
electroencephalography (aEEG) useful information indeed. I agree with the
authors that one of the greatest utility of LTM lies in its ability to
distinguish epileptic disorders from non-epileptic events (NEE).
Distinguishing between these two groups of patients early on helps to cut
down costs and improves quality of life issues.
In my experience, I have also found LTM especially beneficial to rule
out non-convulsive seizures in patients admitted to the intensive care
unit with depressed levels of sensorium and in clarifying the diagnosis in
patients with infrequent paroxysmal events. In our center we do not do
inpatient aEEG studies and the authors demonstration that inpatient aEEG
is as effective as VT in distinguishing between NEE, focal and generalized
epilepsy is very important as it can significantly help reduce cost of
care.
The above information though is not being taught to residents and
others in training who frequently order LTM in patients in whom the
diagnosis may be clarified by a routine 30 minutes EEG study. Cutting
costs is a priority in today’s managed care systems and the most effective
way would be by educating the neurology community in better identifying
those patients who shall truly benefit from LTM.
References
1. Yogarajah M, Powell HW, Heaney D, Smith SJ, Duncan JS, Sisodiya
SM. Long term monitoring in refractory epilepsy: the Gowers Unit
experience.
J Neurol Neurosurg Psychiatry. 2009; 80(3):305-10.
Brown et al. (2009) examined visual perceptual deficits in delirium.
Neuropsychology of Delirium has not been studied extensively. Though the
concept of delirium goes back a very long time, it is not referred to in
the authorative handbook Fundamentals of Human Neuropsychology (Kolb &
Whishaw, 2003). Why this apparent lack of neuropsychological interest in
delirium? Perhaps because delirium is o...
Brown et al. (2009) examined visual perceptual deficits in delirium.
Neuropsychology of Delirium has not been studied extensively. Though the
concept of delirium goes back a very long time, it is not referred to in
the authorative handbook Fundamentals of Human Neuropsychology (Kolb &
Whishaw, 2003). Why this apparent lack of neuropsychological interest in
delirium? Perhaps because delirium is often under diagnosed and patients
are not referred for neuropsychological examination. Secondly, because of
the transient nature of delirium clinicians may choose to refer patients
not until after the they have recovered. Thirdly, and perhaps most
importantly, patients are simply too impaired to take the test, e.g. while
some delirious patients would doze off during neuropsychological testing
because of a disturbance of consciousness, others may restlessly fight to
get of the hospital bed. Evidently, in these instances floor effects on
standard neuropsychological tests are to be expected, to say the least.
The study by Brown et al. is important because the investigators were
able to examine delirious patients. This is a crucial first step for
gaining a better understanding of the neuropsychological deficits in
delirium. Focus of the study was on visual perceptual impairments. As
patients were able to take the test, they probably did not have severe
delirium. Control patients and dementia patients outperformed delirium
patients on visual perceptual task, even after controlling for memory
deficits and general cognitive impairment.
However, authors did not control for core symptoms of delirium, i.e.
attention deficits. This point can not be stressed enough - Attention is a
conditio sine qua non, without attention no episodic memory, no coherent
thought, and also no adequate visual perception? The Brown et al. study
did not include tests of attention. It is possible that attention deficits
acted as confounders of results. Had there been tests of attention
included, the effects of delirium on different cognitive functions could
have been disentangled, and validity of visual perceptual impairments as
independent cognitive symptoms of delirium could have been established.
Hopefully, future studies will include measures for both visual perceptual
as attention deficits in the neuropsychological models of delirium.
We read with interest the summary and subsequent commentary on the
Cochrane review of immunosuppressant drugs for myasthenia gravis (MG)
(Hart et al.2009, Celani et al, 2009). We would like to express concerns
regarding the practical implications of these reviews.
Cochrane reports are not appropriate in guiding clinical practice
where highly effective treatments predated the use of randomi...
We read with interest the summary and subsequent commentary on the
Cochrane review of immunosuppressant drugs for myasthenia gravis (MG)
(Hart et al.2009, Celani et al, 2009). We would like to express concerns
regarding the practical implications of these reviews.
Cochrane reports are not appropriate in guiding clinical practice
where highly effective treatments predated the use of randomised
controlled trials (RCTs) to test efficacy. For example corticosteroids are
routinely used and are very effective in producing remission in the
majority of patients with MG. No RCTs against placebo have been performed
and comparison now would be unethical and unnecessary.
With rare diseases, recruitment for RCTs can be very difficult and
all the studies included in this analysis had difficulty recruiting
adequate numbers. Only one subsequent study referred to by Celani
eventually managed to get sufficient numbers. However they had to add a
further 18 centers across 10 new countries and this would not be
affordable with an investigator led study.Thus clinical practice has to
work with the best evidence and summarising a review with a statement that
‘bigger, better designed, longer studies are needed’ may not be helpful.
The major issue however in this review is the selection of the
outcome used for meta-analysis i.e. improvement in weakness at 6 months.
Of note only three of the six studies selected for inclusion recorded this
outcome, thus leading to the inevitable conclusion of lack of evidence of
efficacy from three studies. Assessing subtle difference in timing of
early responses of immunosuppressive agents that are known to take months
to work would require much larger cohorts and this question is not of
prime importance in clinical practice. The main reason to use a second
immunosuppressive agent in clinical practice is as a steroid-sparer and it
is established that azathioprine is effective in this respect (Palace et
al, 1998)1 .
We are concerned that the conclusion, indicating that limited
evidence from RCTs shows no benefit from azathioprine, is misleading and
might be interpreted to suggest that azathioprine should not be used in
the treatment of MG.
References
1. Palace J, Newsom-Davis J, Lecky B. A randomized double-blind trial
of prednisolone alone or with azathioprine in myasthenia gravis.
Neurology
1998 50 1778-1783.
Schmidt et al have performed an interesting study using longitudinal
brain imaging to monitor progress of dementia and potential response to
drugs aiming to modify the disease (1). The authors describe that patients
were taking a number of concomitant drugs and in the paper document that
the study groups were well-balanced for concomitant use of low-dose
neuroleptics, antidepressants and sedatives/...
Schmidt et al have performed an interesting study using longitudinal
brain imaging to monitor progress of dementia and potential response to
drugs aiming to modify the disease (1). The authors describe that patients
were taking a number of concomitant drugs and in the paper document that
the study groups were well-balanced for concomitant use of low-dose
neuroleptics, antidepressants and sedatives/ hypnotics. They do not
mention whether the study groups were as well balanced for their use of
diuretics and laxatives.
This is relevant, as most research using longitudinal brain imaging
assumes that brain volume is constant over short periods of time.
However, small studies using young healthy volunteers have demonstrated
that brain size may change transiently with hydration status (2) and
volume changes of between 0.55% and 0.72% have been demonstrated (3).
Considering the size of the volume changes seen in the study by Schmidt et
al, such degrees of change could represent a significant source of error.
Elderly people have impaired thirst sensation and those with early or
established dementia may be at greater risk of acute or chronic
dehydration at home or in hospital. The authors state that the study was
done to facilitate the design of a larger trial looking at the effect of
memantine. If volumetric imaging is to be used as a surrogate marker, it
may be very important to balance case-mix, not only for the drugs they
mentioned, but also for drugs that provoke dehydration, in view of the
potential degree of volumentric change that dehydration may provoke.
References
1. Schmidt R, Ropele S, Pendl B et al. Longitudinal multimodal
imaging in mild to moderate Alzheimer disease: a pilot study with
memantine.
Neurol Neurosurg Psychiatry 2008;79:1312–1317
2. Dickson JM, Weavers HM, Mitchell N et al. The effects of
dehydration on brain volume – preliminary results.
Int J Sports Medicine
2005; 26(6):481-5.
3. Duning T, Kloska S, Steinsträter O, Kugel H et al. Dehydration
confounds the assessment of brain atrophy.
Neurology 2005;64:548–550.
Mattes and coworkers report an interesting case of Pourfour du Petit
syndrome which is very useful because the ‘opposite of Horner syndrome’ is
largely unknown [1]. The authors describe that the classical signs of
Pourfour du Petit syndrome are mydriasis, lid retraction, exophthalmos,
sweating and paleness of the affected side.
However, it is probably a misconception that exophthalmos is a clinical
s...
Mattes and coworkers report an interesting case of Pourfour du Petit
syndrome which is very useful because the ‘opposite of Horner syndrome’ is
largely unknown [1]. The authors describe that the classical signs of
Pourfour du Petit syndrome are mydriasis, lid retraction, exophthalmos,
sweating and paleness of the affected side.
However, it is probably a misconception that exophthalmos is a clinical
sign of human Pourfour du Petit syndrome. Firstly, enophthalmos (the
opposite of exophthalmos) is not present in human Horner syndrome [2-5].
Secondly, there are no pathophysiological mechanisms to explain
exophthalmos in Pourfour du Petit syndrome. Thirdly, there was no evidence
of exophthalmos in the presented case [1].
Therefore we believe that exophthalmos is not present in human Pourfour du
Petit syndrome.
REFERENCES
1. Mattes D, Mayer M, Feichtinger M, Lindner S. Neurological picture.
A case of Pourfour du Petit syndrome following tumour surgery of the
mandible.
J Neurol Neurosurg Psychiatry 2009;80:69.
2. van der Wiel HL, van Gijn J. No enophthalmos in Horner's syndrome.
J Neurol Neurosurg Psychiatry 1987;50:498-9.
3. Loewenfeld IE. The Pupil: Anatomy, physiology, and clinical
applications,
Volume 1. Boston: Butterworth-Heinemann; 1999, p. 1139.
4. Thompson HS, Miller NR. Disorders of pupillary function,
accommodation, and lacrimation. In: Miller NR, Newman NJ, editors.
Walsh
and Hoyt's Clinical Neuro-ophthalmology, Volume1, 5th ed. Baltimore:
Williams and Wilkins; 1998, pp. 961-1040.
5. Daroff R. Enophthalmos is not present in Horner syndrome.
PLoS Med
2005;2:e120.
Dear Editor,
We would like to comment the letter of Voermans et al. [1]
Localised scleroderma is characterised clinically by the presence of thickened, sclerotic skin lesions. However, skin changes [2] sometimes present in a discrete manner with only indurated, hyper- and hypopigmented areas. Thus, the clinical diagnosis is delayed. With regard to neuromuscular manifestations of linear scleroderma, a m...
Dear Editor,
This interesting study showed that up to 68% of patients complained of significant work and social dysfunction due to stroke. This study has many implications. The number of people who survive stroke and live with its consequences is increasing. The case fatality rate for stroke has declined over the past few decades,and many of people who experience a stroke now survive.This increase in survival...
Dear Editor,
Rogers et al. make several points. The primary aim of our review was to search in a systematic manner for associations between retinal microvascular abnormalities and stroke subtypes and thus provide clues to the microvascular pathophysiology of lacunar stroke. We aimed to include all relevant studies (not just the large epidemiological ones), carefully evaluate clinical and retinal features for c...
Dear Editor,
De Jonghe raises an extremely important point about the difficulties of interpreting cognitive data from patients with delirium. ‘Inattention’ is one of the core diagnostic criteria for delirium. Accordingly, the attentional deficits with which that these patients present are likely to compromise performance on a wide range of cognitive tasks, irrespective of whether they also have specific deficits...
Dear Editor,
With great interest we read the article by Böckle et al (1), who report a patient with muscle atrophy preceding bilateral linear morphea. We would like to confirm the importance of recognizing linear scleroderma as an unusual cause of focal muscle atrophy. Recently we described a similar case (2) and would like to comment on various aspects of Böckle’s report.
Their case is unusual since t...
Dear Editor,
I read with interest the article by Yogarajah et al 1. Working in a comprehensive epilepsy center of a tertiary referral hospital in the United States, I found the cost benefit analysis of long term monitoring (LTM) either by video telemetry (VT) or inpatient ambulatory electroencephalography (aEEG) useful information indeed. I agree with the authors that one of the greatest utility of LTM lies in...
Dear Editor,
Brown et al. (2009) examined visual perceptual deficits in delirium. Neuropsychology of Delirium has not been studied extensively. Though the concept of delirium goes back a very long time, it is not referred to in the authorative handbook Fundamentals of Human Neuropsychology (Kolb & Whishaw, 2003). Why this apparent lack of neuropsychological interest in delirium? Perhaps because delirium is o...
Dear Editor,
We read with interest the summary and subsequent commentary on the Cochrane review of immunosuppressant drugs for myasthenia gravis (MG) (Hart et al.2009, Celani et al, 2009). We would like to express concerns regarding the practical implications of these reviews.
Cochrane reports are not appropriate in guiding clinical practice where highly effective treatments predated the use of randomi...
Dear Editor,
Schmidt et al have performed an interesting study using longitudinal brain imaging to monitor progress of dementia and potential response to drugs aiming to modify the disease (1). The authors describe that patients were taking a number of concomitant drugs and in the paper document that the study groups were well-balanced for concomitant use of low-dose neuroleptics, antidepressants and sedatives/...
Dear Editor,
Mattes and coworkers report an interesting case of Pourfour du Petit syndrome which is very useful because the ‘opposite of Horner syndrome’ is largely unknown [1]. The authors describe that the classical signs of Pourfour du Petit syndrome are mydriasis, lid retraction, exophthalmos, sweating and paleness of the affected side. However, it is probably a misconception that exophthalmos is a clinical s...
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