We thank Dr Kumar for his interest[1] in our article.[2] I agree, that our data
are not sufficient to evaluate the true risk of recurrence of cerebral
venous and sinus thrombosis (CVST) in women with inherited thrombophilic
disorders. However, we stated in our article that the risk of recurrence
is probably higher if a thrombophilia is present and that all women with
either prior cerebral or extrace...
We thank Dr Kumar for his interest[1] in our article.[2] I agree, that our data
are not sufficient to evaluate the true risk of recurrence of cerebral
venous and sinus thrombosis (CVST) in women with inherited thrombophilic
disorders. However, we stated in our article that the risk of recurrence
is probably higher if a thrombophilia is present and that all women with
either prior cerebral or extracerebral venous thromboembolism who are
pregnant or planning to conceive should be tested for thrombophilia. Our
conclusion was very cautious: "Our data do not justify a negative advice
on pregnancy in women with a history of CVST". We wanted to communicate
that further sucessful pregnancies in women with a history of CVST are
possible. Excluding the one women whose pregnancy was terminated due to a
possible teratogenic risk (because pregnancy occurred during oral
anticoagulation), there were 2 spontaneous abortions. We agree that these
complications might have been due to a thrombophilic state such as an
antiphospholipid antibody syndrome emphasizing the need for thrombophilic
testing. However, all other medical complications (seizures, gestosis)
were controllable and resulted in the birth of healthy children.
In conclusion, we agree that further prospective studies are needed to
determine the true risk of recurrence and that testing for thrombophilic
states is definately necessary. Women with a previous CVST need close
neurological and gynecologic surveillance but a history of CVST does not
exclude the possibility of further sucessful pregnancies.
References
(1) Kumar S, Alexander M, Gnanamuthu C. Risk of recurrent cerebral venous and sinus thrombosis during subsequent pregnancy and puerperium [electronic response to S Mehraein et al. Risk of recurrence of cerebral venous and sinus thrombosis during subsequent pregnancy and puerperium] jnnp.com 2003 http://jnnp.bmjjournals.com/cgi/eletters/74/6/814#53
(2) S Mehraein, H Ortwein, M Busch, M Weih, K Einhäupl, and F Masuhr. Risk of recurrence of cerebral venous and sinus thrombosis during subsequent pregnancy and puerperium. J Neurol Neurosurg Psychiatry 2003; 74:814-816.
We would like to respond to the letter written by R. Shepherd and
J. Carr concerning our paper in Journal of Neurology,
Neurosurgery and Psychiatry 2005, 76, 503-508.
Our comment that the study provided no evidence that one
treatment led to better outcomes than the other and so for the
present, therapists could use either treatment, is the natural
conclusion from our findings. However, as...
We would like to respond to the letter written by R. Shepherd and
J. Carr concerning our paper in Journal of Neurology,
Neurosurgery and Psychiatry 2005, 76, 503-508.
Our comment that the study provided no evidence that one
treatment led to better outcomes than the other and so for the
present, therapists could use either treatment, is the natural
conclusion from our findings. However, as we point out, the study
by Langhammer and Stanghelle [1] found a positive outcome for
motor relearning compared to Bobath. We expect that readers will
take a close look at the methodology and interventions of both
studies and decide for themselves which treatment to use, based
on the current evidence. There are also other studies looking at
some of the individual components of the interventions, which will
assist therapists in making decisions about their interventions.
Clearly overall there is not enough evidence at present to
categorically support one or the other of the treatment packages
that we compared.
The comment that the treatments were administered in the same
environment was a limitation which we also acknowledged in our
discussion. However, the alternative, to deliver the two treatments
in two different settings would have introduced a more serious
problem, in that it would have introduced confounding variables
connected with the different staff, resources and physical
environment in the two settings. We judged it better to have both
treatments occurring on the same ward and made efforts to ensure
patients received their allocated treatment by writing guidelines
which each group of therapists adhered to. Secondly, we
conducted an observation study which examined the content of the
treatment.[2] This study revealed that there were differences in the
treatments, so we concluded that any contamination effects were
not too severe.
In response to the observation that the physiotherapists delivering
both interventions may have similar backgrounds, we can say that
both groups did have some experience of Bobath-based (BB)
treatment. This was a problem with conducting the study in the
UK, where Bobath is the most commonly used intervention.[3] To
overcome this problem, we employed one physiotherapist who
had experience of the Movement Science-based (MSB) treatment,
and gave MSB training to another therapist.
The treatment was standardised so far as could be achieved by
the process of writing treatment guidelines and agreeing to adhere
to these during treatment. The guidelines for treatment were
written collectively by the group of therapists delivering the
treatment. Each patient in the BB group received treatment based
on the same set of guidelines, and the same applied to the MSB
group. The MSB treatment did include task-oriented training, and
an emphasis on analysis of movements based on biomechanics
and increased amounts of practice. There was insufficient space to
include these guidelines in the previous papers, but these are
available to anyone who is interested and can be obtained from Dr
van Vliet (paulette.vanvliet@ntlworld.com).
We agree that the amount of treatment was small and
acknowledged this in our paper. This was a pragmatic decision
made to ensure that the treatment received by both groups was the
same, i.e. the MSB treatment was matched to the amount of BB
treatment given routinely at the time in the hospital. Resources
were not available to increase the amount of therapy in both
groups. The amount received by patients was probably typical of
UK hospitals.[4] Ongoing research indicates that in the UK,
patients receive less therapy than their European counterparts (De
Wit et al In Press). Therefore, our results are applicable to actual
working conditions in the UK. Our results indicate that with a
median 23 minutes per day, there was no difference in outcome.
We recommended in our discussion that future studies increase
the dose to see whether differences occur with more treatment.
We stand by our assertion that it is necessary to examine the effect
of the overall treatment package as well as individual components
of treatment programmes. Otherwise, the effect of combining
individual components will not be known. Another direction for
future research is to combine individual interventions for which
positive effects have been demonstrated into an overall treatment
package [5], and contrast this with current treatment. It seems to us
this would also be a useful direction.
There are considerable pragmatic difficulties to be overcome in
conducting a study such as the one we presented. Although not
ideal the methodology was designed to be as robust as possible
within the constraints of conducting research in healthcare
settings.
We have the greatest respect for the work of Professors Roberta
Shepherd and Janet Carr, whose work has transformed much of
the physiotherapy intervention for stroke patients. And we
welcome the opportunity to contribute to the continuing discussion
about the most profitable directions for future research on this
topic.
Yours sincerely,
Paulette van Vliet, Nadina Lincoln and Andrew Foxall
References
1. Langhammer B, Stanghelle JK. Bobath or Motor Relearning
Programme? A comparison of two different approaches of
physiotherapy in stroke rehabilitation: a randomized controlled
study. Clinical Rehabilitation 2000;14:361-369.
2. vanVliet PM, Lincoln NB, Robinson E. Comparison of the
content of two physiotherapy approaches for stroke. Clinical
Rehabilitation 2000;15:398-414.
3. Lennon S, Baxter D, Ashburn A. Physiotherapy based on the
Bobath concept in stroke rehabilitation: a survey within the UK.
Disability and Rehabilitation 2001;23:254-262.
4. Tinson DJ. How stroke patients spend their day: an
observational study of the treatment regime offered to patients in
hospital with movement disorders following stroke. International
Disability Studies 1989;11:45-49.
5. Weerdt WD, Feys H. Assessment of physiotherapy for patients
with stroke. The Lancet 2002;359:182-183.
Dr. De Ridder overstates reality in the title of his editorial,
"Auditory nerve compression: a forgotten treatable cause for tinnitus" and
distorts reality in the text of the commentary. Examples of distortion
include: there are no effective treatments available for most cases of
tinnitus; auditory nerve compression is a common cause of tinnitus;
carbamazepine and microvascular decompression surgery are viable treatment...
Dr. De Ridder overstates reality in the title of his editorial,
"Auditory nerve compression: a forgotten treatable cause for tinnitus" and
distorts reality in the text of the commentary. Examples of distortion
include: there are no effective treatments available for most cases of
tinnitus; auditory nerve compression is a common cause of tinnitus;
carbamazepine and microvascular decompression surgery are viable treatment
options for tinnitus. The realities are: 1) many effective, non-surgical,
non-pharmacologic management strategies are available and helpful for
patients who experience tinnitus; 2) auditory nerve compression is an
exceedingly rare cause of tinnitus; 3) therefore, microvascular
decompression surgery for tinnitus should be undertaken in a similarly
miniscule number of cases. Support for this conservative use of surgery
comes from an article written by De Ridder et al (2007): "The outcome of
operations for tinnitus, moving the blood vessel off the nerve
(microvascular decompression operations, MVD) is less successful than
microvascular decompression operations for other vascular conflict
syndromes." I agree, so let's stop recommending this surgery for
tinnitus, because it is more likely to harm patients than to help them.
Reports of efficacy for the procedure claimed by Moller et al (1993) and
Dr. Jannetta were, in polite terms, overly optimistic. In his 2010 JNNP
commentary, De Ridder proclaims the importance of articles by Nam et al
(2010) and Brantberg (2010) that describe a "unique form of tinnitus in
contrast to most other kinds of tinnitus is responsive to pharmacological
(and surgical) treatment." The problem with this statement: it gives
readers (including patients and clinicians) the false impression and false
hope that carbamazepine or microvascular decompression surgery are likely
to help. The total number of patients in the study by Nam et al (2010)
plus the total in the study by Brantberg (2010) is five. Far-reaching
treatment recommendations cannot be made on the basis of five patients.
Carbamazepine is an anticonvulsant that might reduce tinnitus (or a
patient's reaction to it) in the same way that benzodiazepines or
barbiturates can in some cases. However, medications of this type - or
surgery - should not be the first considerations for tinnitus treatment.
Instead, I recommend that clinicians familiarize themselves with different
types of tinnitus management strategies that have proven effective for
hundreds of patients and present little or no risk of side effects or
catastrophic outcomes (for example, see Folmer 2002; Folmer et al, 2004;
Folmer & Carroll, 2006; Goebel et al, 2006; Henry et al, 2008; 2009;
2010).
REFERENCES
De Ridder D, Heijneman K, Haarman B, van der Loo E. Tinnitus in
vascular conflict of the eighth cranial nerve: a surgical
pathophysiological approach to ABR changes. Prog Brain Res. 2007;166:401
-411.
Folmer RL. Long-term reductions in tinnitus severity. BMC Ear Nose
and Throat Disorders 2002;2:3. http://www.biomedcentral.com/1472-6815/2/3
Folmer RL, Carroll JR. Long-term effectiveness of ear-level devices
for tinnitus. Otolaryngol Head Neck Surg.
2006;134(1):132-137.
Folmer RL, Martin WH, Shi, Y. Tinnitus: Questions to reveal the
cause, answers to provide relief. J Fam Pract. 2004;53(7):532-540.
Goebel G, Kahl M, Arnold W, Fichter M. 15-year prospective follow-up
study of behavioral therapy in a large sample of inpatients with chronic
tinnitus. Acta Otolaryngol Suppl. 2006;556:70-79.
Henry JA, Zaugg TL, Myers PJ, Kendall CJ, Michaelides EM. A triage
guide for tinnitus. J Fam Pract. 2010;59(7):389-393.
Henry JA, Zaugg TL, Myers PJ, Kendall CJ, Turbin MB. Principles and
application of educational counseling used in progressive audiologic
tinnitus management. Noise Health. 2009;11(42):33-48.
Henry JA, Zaugg TL, Myers PJ, Schechter MA. Using therapeutic sound
with progressive audiologic tinnitus management. Trends Amplif.
2008;12(3):188-209.
Moller MB, Moller AR, Jannetta PJ, Jho HD. Vascular decompression
surgery for severe tinnitus: selection criteria and results.
Laryngoscope. 1993;103(4 Pt 1):421-427.
I read with interest the abstract pertaining to your work indicating
that high intake of certain unsaturated fats are associated with lower
risk of ALS. In the analysis of your very interesting data, can you also
find a strong statistical association between high intake of saturated fat
and higher risk of ALS?
If this is true, then surely the USA would have a preponderance of
ALS cases (?)...
I read with interest the abstract pertaining to your work indicating
that high intake of certain unsaturated fats are associated with lower
risk of ALS. In the analysis of your very interesting data, can you also
find a strong statistical association between high intake of saturated fat
and higher risk of ALS?
If this is true, then surely the USA would have a preponderance of
ALS cases (?)
We read with interest the study by Knake et al. in which they investigated the efficacy and safety of intravenous levetiracetam (ivLEV) for the treatment of status epilepticus 1. Off label usage of LEV is exceedingly common and we would like to commend the authors for providing some data about the efficacy of iv LEV in the treatment of benzodiazepine refractory status epilepticus. All the patients in the...
We read with interest the study by Knake et al. in which they investigated the efficacy and safety of intravenous levetiracetam (ivLEV) for the treatment of status epilepticus 1. Off label usage of LEV is exceedingly common and we would like to commend the authors for providing some data about the efficacy of iv LEV in the treatment of benzodiazepine refractory status epilepticus. All the patients in the study received at least a benzodiazepine before ivLEV was given and in 16 episodes ivLEV was he last drug to be administered. While ivLEV appears to be an attractive armament in the treatment of status epilepticus due to its favorable side effect profile, data with respect to its speed of action in aborting the status is not provided. It is possible that by chance a subgroup of patients in this study had status epilepticus that was relatively easy to control and would have got aborted irrespective of the drug used after the
initial benzodiazepine infusion. In few of the other patients reported ivLEV was administered when valproic acid, phenytoin (patient No 15 a) or midazolam (patient No 12) failed to abort the status. Could it be possible
that LEV was effective due to the fact that it was either co administered or administered soon after these frontal line status epilepticus drugs?
Till further information is available on these issues it may be wise to adhere to the conventional status epilepticus abatement protocol of benzodiazepine -->phenytoin-->phenobarbital followed by either
midazolam, propofol or pentobarbital infusion for status epilepticus refractory to benzodiazepine.
Nitin K. Sethi, Prahlad K. Sethi, Josh Torgovnick, Edward Arsura
The authors report no competing interests.
References
1. Knake S, GruenerJ, Hattemer K, Klein KM, Bauer S, Oertel WH, Hamer HM, Rosenow E. Intravenous levetiracetam in the treatment of benzodiazepine refractory status epilepticus. J Neurol Neurosurg Psychiatry. 2008; 79:588-589.
Kelkar and Parry [1] regard perivascular inflammatory infiltrates as
indicative of an immune microvasculitis that contributes to nerve damage
in diabetes mellitus (DM), in particular mononeuritis multiplex. These
authors believe that the variable therapeutic responses to corticosteroids
alone or in combination with chlorambucil that they observed support the
concept of immunopathogenesis of neural damage...
Kelkar and Parry [1] regard perivascular inflammatory infiltrates as
indicative of an immune microvasculitis that contributes to nerve damage
in diabetes mellitus (DM), in particular mononeuritis multiplex. These
authors believe that the variable therapeutic responses to corticosteroids
alone or in combination with chlorambucil that they observed support the
concept of immunopathogenesis of neural damage in DM.
In sharp contrast to the relatively very short duration of DM in this
cohort,1 diabetic neuropathies tend to occur in the setting of long-
standing hyperglycaemia over decades, whether insulin-dependent or not.[2]
Secondly, diabetic mononeuropathy is characterized by a high degree of
spontaneous reversibility, usually over a several week period. In this
clinical situation, it is difficult to conclude with conviction regarding
the clinical utility of therapy with corticosteroids that, in turn, can
aggravate the diabetic metabolic state. Thirdly, significant weight loss
as seen in three of the four patients with type 2 DM1 is anomalous. Most
patients with uncomplicated type 2 DM are overweight or obese; weight gain
is the usual outcome of therapy with oral hypoglycaemic agents or insulin.
Fourthly, significantly raised erythrocyte sedimentation rate (ESR) (above
50 mm I hour) is not a feature of DM itself. Finally, the metabolic
disturbance in these patients appears to have been well controlled as seen
by near-normal HbA1C levels,[1] which feature generally does not predispose
to neuropathy. In short, the severe but early-onset neuropathy seen in
these diabetic patients1 does not appear to be linked to uncontrolled
hyperglycaemia.
Weight loss, high ESR, and a good response to corticosteroids are
characteristic features of sarcoidosis.[3] At least 25% of patients of DM
(with retinopathy) have serum elevations of angiotensin converting
enzyme.[4] Overall frequency of peripheral neuropathy in neurosarcoidosis is
18%.[5] Mononeuritis multiplex with scattered and asymmetrical sensory and
motor deficits, as described in this cohort,[1] is the most frequently seen
peripheral nerve lesion in neurosarcoidosis.6 Granulomatous T lymphocyte
infiltration in affected tissues, including peripheral nerves, is
diagnostic of sarcoidosis, but tissue evidence of disease cannot always be
obtained.[7]
The mechanism of weight loss [1] in diabetic mononeuropathy as well as
amyotrophy (an uncertain nosologic term)[2] is unknown. In the absence of
markers of vasculitis such as antinuclear antibody and rheumatoid factor,[1]
occult sarcoidosis may underlie weight loss and rapidly evolving steroid-
responsive neuropathy in DM. Corticosteroid refractory neuropathy that
resolves later spontaneously [1] is not likely to be of sarcoid origin and
indicates a miscellaneous vasculitis.
References
(1) Kelkar P, Parry GJ. Mononeuritis multiplex in diabetes mellitus:
evidence for underlying immune pathogenesis. J Neurol Neurosurg Psychiatry 2003;74:803-6.
(2) Asbury AK. Diseases of the peripheral nervous system. In
Harrison’s principles of internal medicine. 12th ed. Wilson JD Braunwald E, Isselbacher KJ, Petersdorf RG, Martin JB, Fauci AS, Root RK, Eds. New York: McGraw–Hill, Inc; 1991:2096-2107.
(3) Mayock RL, Bertrand P, Morrison CE et al. Manifestations of
sarcoidosis. Analysis of 145 patients, with a review of nine series
selected from the literature. Am J Med 1963;35:67-89.
(4) Lieberman J. Angiotensin-converting enzyme in nonpulmonary sarcoidosis.
Semin Res Med 199213:399-401.
(5) James DG, Sharma OP. Neurosarcoidosis. Proc R Soc Med 1967;60:1169-70.
(6) Oksanen V. Neurosarcoidosis. Semin Resp Med 1992;13:459-67.
(7) Gupta VK. Steroid-responsive hypercalcaemic nephropathy in diabetes
mellitus probably due to occult sarcoidosis. Nephron 1996;74:214-5.
We read with interest the article by Lo et al.[1] reporting a prospective
study showing significant neuromuscular dysfunction in anti GQ1b antibody
syndrome associated acute opthalmoparesis. Nine cases presenting with
acute opthalmopareses were assessed clinically and had stimulated single
fibre electromyography (SFEMG) of orbicular oculi at presentation. They
conclude that: 1) the abnormal SFEMG seen...
We read with interest the article by Lo et al.[1] reporting a prospective
study showing significant neuromuscular dysfunction in anti GQ1b antibody
syndrome associated acute opthalmoparesis. Nine cases presenting with
acute opthalmopareses were assessed clinically and had stimulated single
fibre electromyography (SFEMG) of orbicular oculi at presentation. They
conclude that: 1) the abnormal SFEMG seen in their cases is due to
neuromuscular junction (NMJ) transmission abnormality and; 2) the rapid
improvement seen in SFEMG jitter in anti-GQ1b antibody positive cases is
in tandem with clinical recovery.
We have concerns about the quality of methodology, presentation of
data and the author’s comprehension of SFEMG.
Firstly the SFEMG data presented is not convincingly abnormal. Authors
claim that cases 1-4 showed significantly abnormal jitter. The
illustration of case 3 shows remarkably no jitter at all. There is
possible jitter in cases 1, 2 and 4 but the illustration presented is
hardly convincing. Also the amplitudes of the SFEMG potentials in cases 1,
2 and 4 are so small that any abnormality detected would probably
represent technical/computational error rather than genuine jitter
Secondly “Everything that glitter is not gold and similarly
everything that jitter is not due to NMJ defect”. Their interpretation
that SFEMG is of value in differentiating weakness due to neuromuscular
defect from neuropathy is not true. Abnormal jitter in SFEMG is not
specific for NMJ abnormality. Jitter may also be abnormal in axonal
neuropathy, anterior horn cell disease or nerve injuries. Yes it is used
commonly to diagnose myasthenia gravis but it is not specific for
myasthenia gravis. Increased jitter is commonly seen in anterior horn cell
disease and partial nerve injury, due to abnormal conduction along newly
formed axonal sprouts (reinnervation by collateral sprouting) & is
known as ‘neurogenic’ jitter. Increased jitter may be seen in early stages
of GBS, before regeneration has been established & has been attributed
to uncertain impulse propagation along demyelinated distal axonal
branches.
The authors misinterpret the lack of abnormal jitter in cases 5 and 9
with isolated IIIrd nerve palsy as stating that “the normal SFEMG results
support its specificity for diagnosing neuromuscular junction
abnormalities rather than abnormalities attributed to neuropathy”. Of
course one would not expect to see SFEMG abnormalities in these cases
since orbicularis oculi is supplied by the facial nerve.
References
1. Lo YL, Chan LL, Pan A, et al. Acute opthalmoparesis in anti-GQ1b
antibody syndrome: electrophysiological evidence of neuromuscular
transmission defect in orbicularis oculi. J Neurol Neurosurg Psychiatry
2004;75:436-440.
2. Rouseev R, Ashby P, Basinski A, et al. Single fibre EMG in the
frontalis muscle in ocular myasthenia: specificity and sensitivity. Muscle
Nerve 1992;15:399-403.
3. Trontelj JV, Stalberg E. Single fibre electromyography in studies
of neuromuscular function. Adv Exp Med Biol 1995;384:109-119.
4. Sanders DB, Stalberg EV. AAEM minimonograph number 25: single fibre
electromyography. Muscle nerve 1996;9:1069-1083.
5. Massey JM, Sanders DB. Single fibre EMG demonstrates reinnervation
dynamics after nerve injury. Neurology 1991;41:1150-1151.
6. Schwartz MS, Stalberg E, Schiller HH, et al. The reinnervated
motor unit in man: a single fibre EMG multieletrode investigation. J
Neurol Sci 1976;27:303-312.
7. Stalberg E, Schwartz MS, Trontelj JV. Single fibre
electromyography in various processes affecting the anterior horn cell. J
Neurol Sci 1975;24:403-415.
8. Thiele B, Stalberg E. Single fibre EMG findings in
polyneuropathies of different aetiology. J Neurol
Neurosurg Psychiatry 1975;38;881-887.
9. Kimura J. Electrodiagnosis in diseases of nerve and muscle:
principle and practice. Third edition. Oxford University Press 2001:397-
399.
I read with interest the case report by Yamamoto and colleagues on
acute onset paraplegia in a young woman, which was interpreted as a case
of acute longitudinal myelitis (1). Sudden onset without any inflammatory
prodromal symptom or sign is evocative of arterial occlusion, and the MRI
may be compatible with anterior spinal artery occlusion (2). Although
lancinating radicular pain was absent, I wond...
I read with interest the case report by Yamamoto and colleagues on
acute onset paraplegia in a young woman, which was interpreted as a case
of acute longitudinal myelitis (1). Sudden onset without any inflammatory
prodromal symptom or sign is evocative of arterial occlusion, and the MRI
may be compatible with anterior spinal artery occlusion (2). Although
lancinating radicular pain was absent, I wonder whether the authors
investigated this diagnosis, as prompt intravenous heparin treatment can
be resolutive in such cases. Procoagulant states in young women are not
rare, pregnancy and the postpartum, the contraceptive pill may play a
role.
References
1)Yamamoto T, Ito S, Hattori T. Acute longitudinal myelitis as the
initial manifestation of Sjo�gren�s syndrome. J Neurol Neurosurg
Psychiatry 2006;77:780.
2) Latronico N, Fassini P, Antonini B, Gasparotti R. A pain in the
neck. Lancet 2002; 359: 1206.
In 2005, our case report entitled 'Acute longitudinal myelitis as the
initial manifestation of Sjogren's syndrome' was published in this journal
(1). In that report, we described the case of a 31-year-old woman who
presented with acute longitudinal myelitis extending along the entire
spinal cord. She was also diagnosed with Sjogren's syndrome on the basis
of positive anti-SS-A antibody test results a...
In 2005, our case report entitled 'Acute longitudinal myelitis as the
initial manifestation of Sjogren's syndrome' was published in this journal
(1). In that report, we described the case of a 31-year-old woman who
presented with acute longitudinal myelitis extending along the entire
spinal cord. She was also diagnosed with Sjogren's syndrome on the basis
of positive anti-SS-A antibody test results and findings of lip biopsy. We
concluded that this condition was myelitis associated with Sjogren's
syndrome, considering that it was unlikely to be Devic's syndrome or
neuromyelitis optica (NMO), because of the absence of optic nerve lesions,
and on the basis of the conventional diagnostic criteria at that time.
After this paper was published, we received an e-mail from Dr. Weinshenker
who recommended that the patient be tested for the presence of NMO-IgG.
Hence, we collected her serum sample on 22 July 2004, and sent it for NMO-
IgG testing to the Neuroimmunology Laboratory, Mayo Clinic; however, the
results of the NMO-IgG test were negative. Thereafter, the patient further
experienced 4 episodes of myelitis but none of optic neuritis. Her brain
magnetic resonance (MR) image obtained during the first episode was
normal, but that obtained during the second episode showed periventricular
lesions with gadolinium enhancement.
Recently, we developed an enzyme-linked immunosorbent assay (ELISA) for
detecting anti-aquaporin-4 antibody (2), and we measured this antibody in
the same serum sample that was used for testing NMO-IgG; the results of
the ELISA were strongly positive. Moreover, the extended disease entity
'NMO spectrum disorder' was proposed by Wingerchuk et al. in 2007 (3),
including idiopathic single or recurrent episodes of longitudinally
extensive myelitis. Eventually, we revised the diagnosis of 'myelitis
associated with Sjogren's syndrome' as 'neuromyelitis optica spectrum
disorder'. This was supported by the fact that the serum sample that had
been negative for NMO-IgG in the original test at the Neuroimmunology
Laboratory was found to be positive for NMO-IgG/anti-aquaporin-4 antibody
in a retest using their novel and improved assays by Dr. Pittock.
There are many reports concerning the involvement of the central nervous
system (CNS), including myelitis associated with Sjogren's syndrome (4).
However, the association between CNS involvement, especially
longitudinally extensive myelitis, and Sjogren's syndrome remains
controversial. Firstly, most of the reports were published before the
discovery of NMO-IgG or anti-aquaporin-4 antibody. Secondly, NMO-IgG or
anti-aquaporin-4 antibody test results should be carefully interpreted.
Many techniques were used for the detection of these antibodies, but the
sensitivities of these techniques were thought to differ. Further,
sequential analysis of anti-aquaporin-4 antibody titres in the same
patient revealed dynamic changes in the antibody levels related to the
disease activity and treatments. A negative result in 1 test does not
exclude the possibility of a positive result in another test. Lastly, it
has been known that not a few NMO patients concurrently present with anti-
SS-A antibody in their serum or Sjogren's syndrome but without any disease
activity of Sjogren's syndrome. Moreover, it has been reported that NMO-
IgG is detected only in patients with NMO spectrum disorder, but not in
Sjogren's syndrome patients who do not have optic neuritis or myelitis
(5).
From these points, it is necessary to carefully rule out NMO in order to
evaluate CNS involvement associated with Sjogren's syndrome. Further
investigation is needed to clarify whether longitudinal myelitis in
patients with Sjogren's syndrome is a condition caused by Sjogren's
syndrome or neuromyelitis optica spectrum disorder, and whether 'myelitis
associated with Sjogren's syndrome' truly exists.
P.S. We thank Dr. Weinshenker for his helpful advice, and Dr. Pittock for
confirmation of the positivity of NMO-IgG/anti-aquaporin-4 antibody by
novel and improved assays.
References
1. Yamamoto T, Ito S, Hattori T. Acute longitudinal myelitis as the
initial manifestation of Sjogren's syndrome. J Neurol Neurosurg Psychiatry
2006;77:780.
2. Hayakawa S, Mori M, Okuta A, et al. Neuromyelitis optica and anti-
aquaporin-4 antibodies measured by an enzyme-linked immunosorbent assay. J
Neuroimmunol 2008;196:181-7.
3. Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of
neuromyelitis optica. Lancet Neurol 2007;6:805-15.
4. Rabadi MH, Kundi S, Brett D, et al. Primary Sjogren syndrome
presenting as neuromyelitis optica. J Neurol Neurosurg Psychiatry
2010;81:213-4.
5. Pittock SJ, Lennon VA, de Seze J, et al. Neuromyelitis optica and
non-organ-specific autoimmunity. Arch Neurol 2008;65:78-83.
Thank you very much for sending the eLetter regarding our
retrospective case "benzodiazepine refractory status epilepticus". We agree, that standard protocols should be used as the rule as long as levetiracetam is not approved for the treatment of status epilepticus (SE) and
as long as there is no prospective randomized study demonstrating the efficacy of levetiracetam in SE. In the pa...
Thank you very much for sending the eLetter regarding our
retrospective case "benzodiazepine refractory status epilepticus". We agree, that standard protocols should be used as the rule as long as levetiracetam is not approved for the treatment of status epilepticus (SE) and
as long as there is no prospective randomized study demonstrating the efficacy of levetiracetam in SE. In the patients reported, usually a concomitant disease (such as liver failiure etc.) guided us away from using phenytoin or phenobarbitol (see table 1 in the article). As all
patients received at least one other antiepileptic drug, we cannot conclude if one antiepileptic drug or their combination was effective. This point was discussed in the paper. However, we thought it might be important to share our clinical experience with those who may experience similar situations in clinical practice and we hope that our results will spur interest in the conduction of a large, prospective trial.
Dear Editor
We thank Dr Kumar for his interest[1] in our article.[2] I agree, that our data are not sufficient to evaluate the true risk of recurrence of cerebral venous and sinus thrombosis (CVST) in women with inherited thrombophilic disorders. However, we stated in our article that the risk of recurrence is probably higher if a thrombophilia is present and that all women with either prior cerebral or extrace...
Dear Editor,
We would like to respond to the letter written by R. Shepherd and J. Carr concerning our paper in Journal of Neurology, Neurosurgery and Psychiatry 2005, 76, 503-508.
Our comment that the study provided no evidence that one treatment led to better outcomes than the other and so for the present, therapists could use either treatment, is the natural conclusion from our findings. However, as...
Dr. De Ridder overstates reality in the title of his editorial, "Auditory nerve compression: a forgotten treatable cause for tinnitus" and distorts reality in the text of the commentary. Examples of distortion include: there are no effective treatments available for most cases of tinnitus; auditory nerve compression is a common cause of tinnitus; carbamazepine and microvascular decompression surgery are viable treatment...
Dear Editor,
I read with interest the abstract pertaining to your work indicating that high intake of certain unsaturated fats are associated with lower risk of ALS. In the analysis of your very interesting data, can you also find a strong statistical association between high intake of saturated fat and higher risk of ALS?
If this is true, then surely the USA would have a preponderance of ALS cases (?)...
Dear Editor
We read with interest the study by Knake et al. in which they investigated the efficacy and safety of intravenous levetiracetam (ivLEV) for the treatment of status epilepticus 1. Off label usage of LEV is exceedingly common and we would like to commend the authors for providing some data about the efficacy of iv LEV in the treatment of benzodiazepine refractory status epilepticus. All the patients in the...
Dear Editor
Kelkar and Parry [1] regard perivascular inflammatory infiltrates as indicative of an immune microvasculitis that contributes to nerve damage in diabetes mellitus (DM), in particular mononeuritis multiplex. These authors believe that the variable therapeutic responses to corticosteroids alone or in combination with chlorambucil that they observed support the concept of immunopathogenesis of neural damage...
Dear Editor,
We read with interest the article by Lo et al.[1] reporting a prospective study showing significant neuromuscular dysfunction in anti GQ1b antibody syndrome associated acute opthalmoparesis. Nine cases presenting with acute opthalmopareses were assessed clinically and had stimulated single fibre electromyography (SFEMG) of orbicular oculi at presentation. They conclude that: 1) the abnormal SFEMG seen...
Dear Editor,
I read with interest the case report by Yamamoto and colleagues on acute onset paraplegia in a young woman, which was interpreted as a case of acute longitudinal myelitis (1). Sudden onset without any inflammatory prodromal symptom or sign is evocative of arterial occlusion, and the MRI may be compatible with anterior spinal artery occlusion (2). Although lancinating radicular pain was absent, I wond...
Dear Editor,
In 2005, our case report entitled 'Acute longitudinal myelitis as the initial manifestation of Sjogren's syndrome' was published in this journal (1). In that report, we described the case of a 31-year-old woman who presented with acute longitudinal myelitis extending along the entire spinal cord. She was also diagnosed with Sjogren's syndrome on the basis of positive anti-SS-A antibody test results a...
Dear Editor, Dear Dr. Sethi,
Thank you very much for sending the eLetter regarding our retrospective case "benzodiazepine refractory status epilepticus". We agree, that standard protocols should be used as the rule as long as levetiracetam is not approved for the treatment of status epilepticus (SE) and as long as there is no prospective randomized study demonstrating the efficacy of levetiracetam in SE. In the pa...
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