I read with interest your recent paper in BMJ Case Reports. Based on
the single case, you concluded that long-term Li treatment might cause
cortical atrophy. Without pre-Li MRI or evidence for increases in GM
following discontinuation of Li, this conclusion seems tentative. Even if
this was the case, perhaps the brain changes were not related to the
duration of use. We detected positive asso...
I read with interest your recent paper in BMJ Case Reports. Based on
the single case, you concluded that long-term Li treatment might cause
cortical atrophy. Without pre-Li MRI or evidence for increases in GM
following discontinuation of Li, this conclusion seems tentative. Even if
this was the case, perhaps the brain changes were not related to the
duration of use. We detected positive association between brain gray
matter and Li use even in patients with an average of >10 years of Li
treatment (1-3). Importantly, these patients have had regular Li
monitoring. The frequency and quality of monitoring may well determine,
whether Li would show neuroprotective effects or not, especially with long
term use.
Lithium is neurotoxic above a relatively narrow therapeutic range
and/or in combination with certain medications (haloperidol). In addition,
treatment with NSAIDs, ACE inhibitors, diuretics may yield toxic Li
levels, same as renal disease or even acute gastrointestinal problems or
dehydration.
The above-mentioned issues obviously become more likely with the
duration of treatment, especially if there is no regular monitoring and no
adjustments of the dose. Chronic and unmonitored use of Li may lead to
repeated intoxications, due to interactions with other medications,
changes in Li clearance with age, increase in rates of comorbid conditions
with age.
Based on the available literature, the long duration of treatment may
in fact be a pre-requisite for the putative neuroprotective effects to
occur - see the studies in amnestic mild cognitive impairment (4) and
Alzheimer dementia (5), as well as the pharmacoepidemiological studies
showing association between Li treatment and lower risk of dementia, but
only with repeated use (6). However, without careful and regular
monitoring, long-term Li treatment may be problematic.
Sincerely, Tomas Hajek
Reference List
1. Hajek T, Cullis J, Novak T, Kopecek M, Hoschl C, Blagdon R et al.
(2012): Hippocampal volumes in bipolar disorders: opposing effects of
illness burden and lithium treatment. Bipolar Disorders 14: 261-270.
2. Hajek T, Bauer M, Pfennig A, Cullis J, Ploch J, O'donovan C et
al. (2012): Large positive effect of lithium on prefrontal cortex N-
acetylaspartate in patients with bipolar disorder: 2-centre study. Journal
of Psychiatry and Neuroscience 37: 185-192.
3. Hajek T, Bauer M, Simhandl C, Rybakowski J, O'donovan C, Pfennig
A et al. (2013): Neuroprotective effect of lithium on hippocampal volumes
in bipolar disorder independent of long-term treatment response. Psychol
Med : 1-11.
5. Nunes MA, Viel TA, Buck HS (2013): Microdose lithium treatment
stabilized cognitive impairment in patients with Alzheimer's disease. Curr
Alzheimer Res 10: 104-107.
6. Kessing LV, Sondergard L, Forman JL, Andersen PK (2008): Lithium
treatment and risk of dementia. Arch Gen Psychiatry 65: 1331-1335.
As a clinical neurologist with interest in laterality of motor
control and consciousness, 1, 2 I read the contribution by Simonin and
colleagues and have the following clinical and neurophysiological comments
on their interpretation of the findings in their remarkable (presumably
right handed) patient. Thus, a spontaneously breathing patient with a non-
traumatic left subdural hematoma displayed bilate...
As a clinical neurologist with interest in laterality of motor
control and consciousness, 1, 2 I read the contribution by Simonin and
colleagues and have the following clinical and neurophysiological comments
on their interpretation of the findings in their remarkable (presumably
right handed) patient. Thus, a spontaneously breathing patient with a non-
traumatic left subdural hematoma displayed bilateral hemiplegia with an
ostensibly diminished level of consciousness. The EEG, however, indicated
absence of epileptiform discharges and presence of "reactive waveforms,"
indicating a wakeful state. The patient's consciousness improved
significantly after two weeks but he remained with a left hemiparesis six
months after discharge. The authors ascribed the presenting feature of the
patient, i.e. left hemiplegia ipsilateral to the lesion, to a Kernohan-
Woltman phenomenon in the absence of the same in the initial CT scans.
This interpretation, however, ignores the fact that only 50 % of Kernohan
and Woltman's patients with notching of contralateral peduncles (17/35
with supratentorial lesions) displayed the dreaded ipsilateral pyramidal
signs; raising doubt about the original belief of Kernohan and Wolman as
to the role of notching in causing the ipsilateral phenomenon they
described. 3, 4 Meanwhile, there are plenty of case reports describing
ipsilateral paralysis in subdural hematoma in the absence of any Kernohan
notch (one of those being case 1 reported by Moon et al and by the
authors). 1, 3, 4 As detailed elsewhere, 5-7 the abovementioned two
classes of exceptions make the case that ipsilateral paralysis is a
laterality indexed diaschitic phenomenon based on the withdrawal of the
excitatory signals arising from the major hemisphere and destined for the
minor for movements occurring on the nondominant side of the body (i.e.
ipsilateral to the lesion). This circuitry explains the temporary nature
of the nondominant side weakness after the initial complete paralysis seen
in the patient, with the remaining weakness indicating incomplete
resolution of the initial insult within the major hemisphere. It also
explains the association between the laterality of motor control and
consciousness housed within the executive hemisphere. 7-9 Hallmarks of
lesions affecting the minor hemisphere are contralateral paralysis in the
absence of awareness of the same (anosognosia) and the conjugate deviation
of the eyes toward that hemisphere. 10 This is because sensory signals
arising from the nondominant side cannot reach consciousness before their
arrival to the major hemisphere via the posterior callosum. Bilateral
absence of sensory evoked potentials upon stimulation of the left side is
the electrodiagnostic confirmation of the above described explanation. 11
The role of callosum in underpinning dominance of the major hemisphere
over the minor (slave) hemisphere is manifested by the faster speed of the
dominant hand in bimanual simultaneous movements upon using a pencil and
paper or an etch-a-sketch device or while employing a straightedge as
described by Hall and Hartwell in Mind in 1884. 6, 7, 12
Acknowledgement: This note is dedicated to the tender memories of my
sister Farkhondeh Derakhshan
References:
1. Simonin A, Levivier M, Nistor S, Diserens K. Kernohan's notch and
misdiagnosis
of disorders of consciousness. BMJ Case Rep 2014, 17; 2014
2. Derakhshan I, Binder DK, Lyon R, Manley GT . Transcranial motor
evoked potential recording in a case of Kernohan's notch syndrome: case
report. Neurosurgery 2005; 56: E1166.
3. Moon KS, Lee JK, Joo SP, Kim TS, Jung S, Kim JH, Kim SH, Kang SS.
Kernohan's
notch phenomenon in chronic subdural hematoma: MRI findings. J Clin
Neurosci 2007;14: 989-992.
4. De Oliveira-Souza R, Benchimol M. Coma and transtentorial
herniation syndrome
due to acute non-expansive hemispheric lesion. Arq Neuropsiquiatr 1995;
53: 815-820.
5. Derakhshan I. Lateralities of motor control and the alien hand
always coincide: further observations on directionality in callosal
traffic underpinning handedness. Neurol Res 2009; 31: 258-264.
6. Derakhshan I. Right sided weakness with right subdural hematoma:
motor deafferentation of left hemisphere resulted in paralysis of the
right side. Brain Inj 2009; 23: 770-774.
7. Derakhshan I. Bimanual simultaneous movements and hemispheric
dominance:
Timing of events reveals hard-wired circuitry for action, speech, and
imagination. Psychol Res Behav Manag 2008; 1: 1-9.
8. Derakhshan I. Voluntary brain processing in disorders of
consciousness. Neurology 2009 17; 73:1712; author reply 1712-1713.
9. Derakhshan I. Laterality of motor control and consciousness share
the same hemisphere. Anesthesiology 2013;119:727-728.
10. Derakhshan I. Lateralization of cognitive functions in aphasia
after right brain amage. Yonsei Med J. 2013; 54:1070-1071.
11. Green JB, Hamilton WJ. Anosognosia for hemiplegia: somatosensory
evoked
potential studies. Neurology 1976; 26:1141-1144.
12. Willford JA, Chandler LS, Goldschmidt L, Day NL. Effects of
prenatal tobacco, alcohol and marijuana exposure on processing speed,
visual-motor coordination, and interhemispheric transfer. Neurotoxicol
Teratol 2010; 32: 580-588. (see Page 2 and Figure 1)
The present report (1) of dengue shock syndrome raises many important questions.It is estimated that at least 10% of dengue fever cases evolve to severe and eventually lethal forms of the disease and a negative serology does not rule out dengue (2). In such cases one has to rely on early clinical features to prevent complications. One such presentation that emerges is Opsoclonus Myoclonus Syndrome (3)(4) A one year old patient pr...
The present report (1) of dengue shock syndrome raises many important questions.It is estimated that at least 10% of dengue fever cases evolve to severe and eventually lethal forms of the disease and a negative serology does not rule out dengue (2). In such cases one has to rely on early clinical features to prevent complications. One such presentation that emerges is Opsoclonus Myoclonus Syndrome (3)(4) A one year old patient presented with fever with leukopenia and a platelet count of 2,41,000 and in a days time the platelets fell to 1,34,000 and the liver was palpable with a rise in SGOT and hematocrit. The child presented with a opsoclonus myoclonus kind of seizure which was diagnosed as febrile convulsion prior to derangement of the platelet count and liver enzyme. The opsoclonus myoclonus and the recent reports of association of these with dengue prompted the doctors to recheck the hematocrit, platelets and liver enzymes which were initially normal. This points out the importance of clinical features like opsoclonus myoclonus which alerts the treating doctor and it is important to recognise this syndrome as a marker of severe form of dengue and differentiate it from typical febrile convulsions which may occur in any febrile illness including dengue.
References
1) Linda Aurpibul,Punyawee Khumlue, Satja Issaranggoon na ayuthaya,Peninnah Oberdorfer. Dengue shock syndrome in an infant. BMJ Case Reports 2014
2) Osorio L(1), Ramirez M, Bonelo A, Villar LA, Parra B. Comparison of the diagnostic accuracy of commercial NS1-based diagnostic tests for early dengue infection.Virol J. 2010 Dec 6;7:361. doi: 10.1186/1743-422X-7-361.
3)Tan AH, Linn, Ramli NM, Hlaing CS, Aye AM, Sam IC, Ng CG, Goh KJ, Tan CT, Lim SY. Opsoclonus-myoclonus-ataxia syndrome associated with dengue virus infection.Parkinsonism Relat Disord. 2014 Sep 16. pii: S1353-8020(14)00324-1. doi:10.1016/j.parkreldis.2014.09.002.
4) Verma R(1), Sharma P, Garg RK, Atam V, Singh MK, Mehrotra HS.Neurological complications of dengue fever: Experience from a tertiary center of
north India.Ann Indian Acad Neurol. 2011 Oct;14(4):272-8. doi: 10.4103/0972-2327.91946.
Masters and colleagues present an instructive case study in the May
16, 2014 issue of BMJ.(1) Unfortunately, it does not teach what they
assert: namely, that "midlines should not be used for the administration
of drugs which are vesicants and...potentially vesicant in nature...."
A cancer patient with a history of right-sided DVT secondary to a
PICC, undergoes a failed effort to insert a left-sided PICC--due to...
Masters and colleagues present an instructive case study in the May
16, 2014 issue of BMJ.(1) Unfortunately, it does not teach what they
assert: namely, that "midlines should not be used for the administration
of drugs which are vesicants and...potentially vesicant in nature...."
A cancer patient with a history of right-sided DVT secondary to a
PICC, undergoes a failed effort to insert a left-sided PICC--due to an
unidentified obstructing lesion. An 18cm "midline" is fashioned from a
"cut" PICC and then inserted with difficulty in the left side. This so-
called midline then exhibits evidence of insertion site injury and
sluggish blood return on aspiration, but flushes well.
Given this scenario, should one infuse oxaliplatin or any drug
through this line? Further, upon the patient indicating mild tenderness at
the commencement of the infusion, should the infusion be allowed to
continue?
Given that the patient has a propensity to form DVTs, that the
insertion of this line was traumatic and that the cut-off PICC presumably
has its tip positioned at or near the original point of obstruction--the
answer is decidedly,NO. This patient most likely has another DVT causing
retrograde pressure which predisposes to infiltration/extravasation. An
ultrasound study should have been performed prior to the line being used.
Moreover, the infusion should have been stopped at the first indication of
pain.
To conclude from this anecdote that midlines should not be used for
vesicants and potential vesicants is an extravagant reach. It ignores the
fundamental clinical errors in this case, and it implies a freedom from
risk with central lines that simply does not exist.
Equally important,the line in question was a cut-off PICC, bearing no
resemblance whatsoever to the 8-10cm midlines currently available. There
is significant published evidence, beyond mere anecdote,as to their safety
and efficacy. (2,3,4,5)
What the Masters anecdote really teaches is what we already know:
Prior to administering any drug through any line, a thorough patency and
functionality check is mandatory. When in doubt, pull it out.
References:
1. Masters B, et al. BJM Case Rep 2014. loi:10.1136/bcr-2014-204360
2. Warrington WG, et al. Outcomes of using a modified Seldinger technique
for long term intravenous therapy in hospitalized patients with difficult
venous access. JAVA 2012;17(1):24-31.
3. Dawson RB, et al. Midline Catheters: An essential tool in CLABSI
reduction. Inf Control Today 2013;17(3):42-45.
4. Caparas JV, et al. Safe administration of vancomycin through a novel
midline catheter: a randomized prospective clinical trial. J Vasc Access
2014;15(3):1-6.
5. Ireland T, et al. Two new evidence-based steps for CLABSI reduction.
Inf Control Today 2014;June:48-52.
We have read with attention the case report of Chhabra et al.
describing the presentation of pleuropericardial effusion in a case of
polymyalgia rheumatica (PMR)1. The authors described a case of an 86-year-
old woman with a one-month history of progressive dyspnea, a two-month
history of shoulder girdle pain, and significant weight loss. The report
ruled out diagnoses of elderly-onset rheumatoid arthritis (EORA), because...
We have read with attention the case report of Chhabra et al.
describing the presentation of pleuropericardial effusion in a case of
polymyalgia rheumatica (PMR)1. The authors described a case of an 86-year-
old woman with a one-month history of progressive dyspnea, a two-month
history of shoulder girdle pain, and significant weight loss. The report
ruled out diagnoses of elderly-onset rheumatoid arthritis (EORA), because
of shoulder girdle involvement, and of systemic lupus erythematosus (SLE),
due to the good response to glucocorticoid treatment. These conclusions
caused us some concern and we would like to present a few related facts.
PMR is an inflammatory disease in elderly patients characterized by
pain and morning stiffness in the cervical region and the shoulder and
pelvic girdles, and an elevated erythrocyte sedimentation rate (ERS).
Typically, neither immunological tests nor x-ray explorations show any
alterations. Therefore, the excellent response to glucocorticoids is
accepted as part of the diagnosis of PMR2. In addition, recent studies
using ultrasound (US) and magnetic resonance imaging (MRI) have shown an
almost constant presence of subacromial bursitis or bilateral bicipital
tenosynovitis, and these findings have been included in the new diagnostic
criteria for PMR3. It should be noted that PMR is inherently difficult to
diagnose due to its complex definition and the lack of specific evidence.
As such, differential diagnosis should take into account other rheumatic
and non-rheumatic diseases that may simulate a case of PMR4-6. Non-
rheumatologic causes such as infections and tumors must be ruled out, as
was done in this case report. However, the rheumatologic causes, including
EORA and elderly-onset SLE should not have been ruled out on the basis of
the available data.
On the one hand, EORA differs from young onset rheumatoid arthritis
because it affects women less frequently, has a more acute onset and is
most commonly accompanied by constitutional syndrome. It often affects
large joints, especially the shoulders, thus simulating PMR, but with less
involvement of the metacarpophalangeal joints. EORA typically shows a very
high ERS, and the proportion of patients with negative rheumatoid factor
(RF) serum levels is similar to the proportion of young patients, making
it hard to distinguish between EORA and PMR. The good response to
glucocorticoids is also a shared feature of the two diseases7-8.
Therefore, contrary to the authors' comments on the case in question, the
clinical presentation is perfectly compatible with a diagnosis of EORA. An
imaging test of the shoulder girdle would have been useful to distinguish
between EORA and PMR, and negative anticitullinated peptide antibodies
(ACPA) could help to rule out an EORA. In any case, the absence of RF or
ACPA would not rule out a diagnosis of EORA, since it would be a
seronegative form9.
On the other hand, the onset of SLE beyond 50 years of age is reported to
occur in 3-18% of patients. Elderly onset has a strong modifying effect on
the clinical presentation, disease course, response to treatment and
prognosis of SLE. In comparison with younger patients, late-onset SLE
sufferers often have a delayed diagnosis but less frequently present
severe manifestations. For instance, recent data suggest that pulmonary
involvement and serositis are frequent in late-onset SLE, whereas malar
rash, photosensitivity, arthritis and nephropathy occur less commonly10.
Therefore, we do not believe that SLE can be ruled out on the basis of the
data available. Firstly, the SLICC criteria for lupus11 include serositis,
synovitis (again, we would stress the need for an imaging test of the
shoulders) and positive autoimmune markers (the patient also had positive
ANAs), so we would recommend completing the immunological study with
AntiDNA, AntiSm, complement levels and antiphospholipid antibodies.
Secondly, the literature contains several references to an initial good
response to glucocorticoids in elderly-onset lupus12.
In the case that concerns us, even if the patient meets the diagnostic
criteria for PMR, the presence of pleuropericardial effusion should cast
doubt on such a diagnosis. In the literature, pleuropericardial effusion
is associated with PMR only on rare occasions13-17, whereas it is often
described in association with EORA18-19. A closer association has also
been reported between late-onset SLE and serositis, as discussed above.
We believe that the diagnosis of PMR is questionable, due to its clinical
similarities with EORA20 and the possible presentation of SLE in elderly
patients with atypical features. It would be important to verify the
presence of peripheral arthritis, to examine the shoulder girdle by US or
MRI, and to determine AntiDNA, AntiSm, antiphospholipid antibodies and
complement levels both the ACPA. Clinical follow-up would also provide
fundamental diagnostic data, since in many cases of arthritis in elderly
patients, evolution is the only key to establishing a specific diagnosis.
1. Lovely Chhabra, Ramprakash Devadoss, Karthik Gnanapandithan, David
H Spodick. Pleuropericardial effusion: an unusual presentation of
polymyalgia rheumatica. BMJ Case Reports 2014:published online 30 May
2014, doi:10.1136/bcr-2014-203881
2. Dasgupta B, Cimmino MA, Kremers HM, Schmidt WA, Schirmer M, Salvarani
C,et al. Provisional classification criteria for polymyalgia rheumatica: A
European League Against Rheumatism/American College of Rheumatology
collaborative initiative. Arthritis Rheum. 2012;64:943-54.
3. Macchioni P, Boiardi L, Catanoso M, Pazzola G, Salvarani C. Performance
of the new 2012 EULAR/ACR classification criteria for polymyalgia
rheumatica: Comparison with the previous criteria in a single-centre
study. Ann Rheum Dis.2014;73:1190-3.
4. Pease CT, Haugeberg G, Morgan AW, Montague B, Hensor EM, Bhakta BB.
Diagnosing late onset rheumatoid arthritis, polymyalgia rheumatica, and
temporal arteritis in patients presenting with polymyalgic symptoms. A
prospective longterm evaluation. J Rheumatol. 2005;32:1043-6.
5. Gonz?lez-Gay MA, Garc?a-Porr?a C, Salvarani C, Olivieri I, Hunder GG.
Polymyalgia manifestations in different conditions mimicking polymyalgia
reumatica. Clin Exp Rheumatol. 2000;18:755-9.
6. Ceccato F, U?na C, Regidor M, Rillo O, Babini S, Paira S. Enfermedades
que simulan polimialgia reum?tica. Reumatol Clin. 2011;7:156-60.
7. Oliv? Marqu?s A. Artritis reumatoide del anciano. Rev Esp Reumatol.
2003;30:66-70.
8. van Schaardenburg D, Breedveld FC. Elderly-onset rheumatoid arthritis.
Semin Arthritis Rheum. 1994;23:367-78.
9. Corrao S, Messina S, Pistone G, Calvo L, Scaglione R, Licata G. Heart
involvement in rheumatoid arthritis: Systematic review and meta-analysis.
Int J Cardiol. 2013;167:2031-8.
10. Calvo E, Becerra E, L?pez-Longo FJ, Cabrera FJ, Carre?no L, Paravisini
A, et al.Pericardial tamponade in a patient with polymyalgia rheumatica.
Clin Exp Rheumatol. 2009;1:S83-5.
11. Petri M, Orbai AM, Alarc?n GS, Gordon C et al. Derivation and
validation of the Systemic Lupus International Collaborating Clinics
classification criteria for systemic lupus erythematosus. Arthritis Rheum.
2012 Aug;64(8):2677-86.
12. Clinical expression and course in patients with late onset systemic
lupus erythematosus. Stefanidou S, Gerodimos C, Benos A, Garyfallos A et
al. Hippokratia. 2013 Apr;17(2):153-6.
13. Bohan A. Polymyalgia rheumatica and pericardial effusion. Ann Intern
Med. 1984;101:147.
14. Cobo T, Zubera-Salibarria Z, Mu?noz-S?nchez J, Santamar?a-Jauregui JM.
Polymyalgia rheumatica and pericardial effusion. Ann Intern Med.
1991;8:362-3.
15. Malone CB, McCarthy GM. Polymyalgia rheumatica as an unusual cause of
pleural and pericardial effusion. J Clin Rheumatol. 2005;11:59-60.
16. Brucato A, Brambilia G. Polymyalgia rheumatica and pericardial
tamponade. Ann Rheum Dis. 2002;61:283.
17. Balbir-Gurman A, Yigla M, Nahir AM, Braun-Moscovici Y. Rheumatoid
pleural effusion. Semin Arthritis Rheum. 2006;35:368-78.
18. Mjaavatten MD, Bykerk VP. Early rheumatoid arthritis: The performance
of the 2010 ACR/EULAR criteria for diagnosing RA. Best Pract Res Clin
Rheumatol. 2013;27:451-66.
19. Laurent Arnaud, Alexis Mathian, Jaques Boddaert, Zahir Amoura. Late-
onset systemic lupus erythematosus: epidemiology, diagnosis and treatment.
Department of Internal Medicine, French Reference Center for Systemic
Lupus Erythematosus, Groupe Hospitalier Piti?-Salp?tri?re, Paris, France.
Drugs & Aging 03/2012; 29(3):181-9.
20. Melania Mart?nez-Morillo, Samantha Rodr?guez-Muguruza, Anne Riveros-
Frutos, Alejandro Oliv?. Rheumatic polymyalgia with pleuropericardial
effusion: An uncommon association. Reumatol Clin. 2014; 11(2): 123-124.
This is a very interesting and unusual case. In the absence of a
biopsy-proven diagnosis, and in the absence of Autoantibodies, would
Fabry's Disease enter the differential diagnosis ? The skin lesions in
Fabry's may not be present initially and usually present later in the
disease evolution.
I would like to congratulate the authors on the elaborate description
of the MRI findings in their patient. Whereas, these findings are
interesting, the more important question is what do they represent and
what did the patient have? Neurological injury from myriad disorders,
acute and chronic, can leave various intricate traces on the brain
parenchyma. All of these can be given elaborate and idiosyncratic names.
However...
I would like to congratulate the authors on the elaborate description
of the MRI findings in their patient. Whereas, these findings are
interesting, the more important question is what do they represent and
what did the patient have? Neurological injury from myriad disorders,
acute and chronic, can leave various intricate traces on the brain
parenchyma. All of these can be given elaborate and idiosyncratic names.
However it is in light of the clinical utility in diagnosing disorders
that these signs need to be interpreted in. I would like to know the
clinical use, sensitivity and specificity of the sign/s described by these
authors here.
The patient in the case report had two levels of protein C with values of 60.8% and 64.5%. The first value was apparently unreliable because it was tested during the acute thrombotic episode. The accuracy of the second value is also questionable even though it was tested during heparin bridging. How long heparin bridging was done before the repeat testing was not mentioned in the case report. Ideally, patients should be off warfar...
The patient in the case report had two levels of protein C with values of 60.8% and 64.5%. The first value was apparently unreliable because it was tested during the acute thrombotic episode. The accuracy of the second value is also questionable even though it was tested during heparin bridging. How long heparin bridging was done before the repeat testing was not mentioned in the case report. Ideally, patients should be off warfarin for 2-3 weeks before protein C activity testing is performed.
In addition, it is difficult to establish a normal range for protein C due to a variety of factors. Levels less than 55%, however, are likely to reflect deficiency, whereas those between 55% and 70% are considered borderline and may be consistent with a deficiency state or the lower end of the normal distribution.
Moreover, acquired deficiency of protein C such as liver disease, protein-losing enteropathy, and malnutrition needs to be ruled out, especially in a patient with HIV.
In light of the facts mentioned above, it is doubtful that the patient had protein C deficiency unequivocally.
Last but not the least, a statement in the "Learning Points" may need correction. Protein C deficiency is not only limited to venous thrombosis but also associated with arterial thrombosis.
References:
1. Hoffman R. Hematology : basic principles and practice. 6th ed. Philadelphia, PA: Saunders/Elsevier; 2013.
2. Tajima K, Yamamoto H, Yamamoto M, Kato Y, Kato T. Adult-onset arterial thrombosis in a pedigree of homozygous and heterozygous protein C deficiency. Thrombosis research. Jan 2013;131(1):102-104.
3. Bovill EG, Bauer KA, Dickerman JD, et al. The clinical spectrum of heterozygous protein C deficiency in a large New England kindred. Blood 1989;73:712-717.
4. Mahmoodi BK, Brouwer JL, Veeger NJ, van der Meer J. Hereditary deficiency of protein C or protein S confers increased risk of arterial thromboembolic events at a young age: results from a large family cohort study. Circulation 2008;118:1659-1667.
5. American Society of Hematology self-assessment program. 5th ed. 2013.
Marappa Ganeshan and colleagues provide an intriguing case of acute
compartment syndrome occurring after operative management of distal radius
fracture under regional anesthesia with brachial plexus blockade (i.e.
axillary block) as an outpatient surgical procedure.1 The patient, though
comfortable in the immediate postoperative period, presented to the
emergency department approximately 24 hours after discharge with an...
Marappa Ganeshan and colleagues provide an intriguing case of acute
compartment syndrome occurring after operative management of distal radius
fracture under regional anesthesia with brachial plexus blockade (i.e.
axillary block) as an outpatient surgical procedure.1 The patient, though
comfortable in the immediate postoperative period, presented to the
emergency department approximately 24 hours after discharge with an
inability to feel his fingers, absent active movements of the fingers and
wrist, and painful passive movements. He was diagnosed with acute
compartment syndrome and appropriately underwent forearm fasciotomies.
The authors conclude that peripheral regional blocks are "not necessarily
the safest option" in the management of traumatic fractures given their
potential to mask symptoms of acute compartment syndrome and delay
diagnosis and treatment. This may not be the case.
Including the report by Marappa Ganeshan and colleagues, there are
now 8 cases in the published literature regarding acute compartment
syndrome in the setting of regional anesthesia with peripheral nerve
blockade.1-7 Contrary to the authors' statement, there is no compelling
association between peripheral nerve blocks and the incidence of extremity
compartment syndromes. For example, in a study directly cited by the
authors, Hyder et al. described a case of lower extremity compartment
syndrome in the setting of intramedullary nailing for tibial fracture in
which the patient received a single-injection peripheral nerve block in
the femoral nerve distribution.4 This patient ultimately developed acute
compartment syndrome and muscle necrosis in the anterior tibial
compartment, the diagnosis of which was delayed due to peripheral nerve
blockade. However, the authors fail to explain how blockade of the
femoral nerve could mask pain in a compartment clearly innervated by
branches of the sciatic nerve (i.e. peroneal nerve). Moreover, the
authors fail to explain how single injection of bupivacaine (with expected
duration of 12-18 hours) could delay clinical diagnosis of compartment
syndrome by more than 48 hours.
In all remaining reports, there is no direct implication of
peripheral nerve blockade delaying diagnosis or masking symptoms of
compartment syndrome. In fact, in four cases clinicians were only alerted
to compartment ischemia by the development of new onset breakthrough pain
despite adequate sensory and/or motor blockade.2 3 6 This has led some to
suggest that peripheral nerve blockade in patients at risk for
compartmental ischemia is not only safe but may be useful.2 For example,
in addition to alerting clinicians to escalating break-through pain
requirements, regional blockade may provide the theoretical benefit of
increased blood flow and oxygen delivery to affected tissues.
While Marappa Ganeshan and colleagues provide a compelling case of
missed ischemia in the setting of peripheral nerve blockade, certain
details require further clarification. First, the concentration, volume,
and type of local anesthetic utilized for axillary nerve blockade should
be specified. This has significant implications for anticipated duration
of blockade and the timing of return of upper extremity sensory and motor
function. In addition, it must be stressed that the patient underwent
closed fracture reduction with percutaneous wire fixation approximately 4
weeks prior to open reduction and internal fixation under regional
anesthesia. In the interim, he was maintained in a plaster cast and
experienced escalating pain in the setting of persistent fracture segment
displacement. The presence of fracture, bony displacement, soft-tissue
manipulation, and external compression all significantly increase the risk
for the development of compartment ischemia.8 It is quite possible that
intracompartmental pressures may have been escalating prior to his
procedure, rather than developing acutely under the guise of blocked
sensory nerves.
Regardless of whether peripheral nerve blockade is used or not,
clinical vigilance by trained medical personnel remains the sine qua non
in the management of extremity fracture. This vigilance must be increased
in those with significant risk factors for compartment ischemia, such as
the gentleman described in this case report. Hence, the most important
discussion point in this case may be the decision to perform it as an
outpatient procedure.
References:
1. Marappa Ganeshan R, Mamoowala N, Ward M, Sochart D. Acute compartment
syndrome risk in fracture fixation with regional blocks. BMJ Case Rep
2015.
2. Kucera TJ, Boezaart AP. Regional anesthesia does not consistently block
ischemic pain: two further cases and a review of the literature. Pain Med
2014;15(2):316-9.
3. Walker BJ, Noonan KJ, Bosenberg AT. Evolving compartment syndrome not
masked by a continuous peripheral nerve block: evidence-based case
management. Reg Anesth Pain Med 2012;37(4):393-7.
4. Hyder N, Kessler S, Jennings AG, De Boer PG. Compartment syndrome in
tibial shaft fracture missed because of a local nerve block. J Bone Joint
Surg Br 1996;78(3):499-500.
5. Noopuri B, Shashane S, Getty C. Acute compartment sydrome following
revisional arthroplasty of the forefoot: the dangers of anke-block. Foot
Ankle Int 2000;21:680-2.
6. Cometa MA, Esch AT, Boezaart AP. Did continuous femoral and sciatic
nerve block obscure the diagnosis or delay the treatment of acute lower
leg compartment syndrome? A case report. Pain Med 2011;12(5):823-8.
7. Uzel AP, Steinmann G. Thigh compartment syndrome after intramedullary
femoral nailing: possible femoral nerve block influence on diagnosis
timing. Orthop Traumatol Surg Res 2009;95(4):309-13.
8. von Keudell AG, Weaver MJ, Appelton PT, Bae DS, Dyer GS, Heng M, et al.
Diagnosis and treatment of acute extremity compartment syndrome. Lancet
2015;386(10000):1299-310.
We read with interest the article by Braithwaite et al. This article has helped us in diagnosis and management of a patient here in Mumbai, India.Dengue Eye Disease has become more common and has been investigated extensively with many diagnostic modalities (1 to 33) and has been suspected to have 10 percent prevalence in some outbreaks of dengue . Dengue could present to an eye surgeon first as subconjunctival haemorrhage and ec...
We read with interest the article by Braithwaite et al. This article has helped us in diagnosis and management of a patient here in Mumbai, India.Dengue Eye Disease has become more common and has been investigated extensively with many diagnostic modalities (1 to 33) and has been suspected to have 10 percent prevalence in some outbreaks of dengue . Dengue could present to an eye surgeon first as subconjunctival haemorrhage and ecchymosis (17) , or some patients may be suffering from dengue eye disease without symptoms .A subtle foveolitis may be missed without OCT.Dengue may also cause symptomatic retinochoroiditis, vasculitis, choroidal neovascularisation (19) secondary to immune damage , central retinal artery occlusion (14) , AION, frosted branch angitis (11) and even panophthalmitis (23) , bilateral vitreous haemorrhage (31) , bilateral stellar neuroretinitis , bilateral choroidal effusion (33) and may be a suspect ethology even for bilateral acute angle closure glaucoma (28) and also oculomotor paralysis (26) . We used iPad softwares to examine severely ill dengue patients at bedside. We used it to identify optic neuropathy and foveolitis in one patient and confirmed it on the basis of this present article. A 32 year old female patient was admitted with dengue and a routine examination was sought for eye check up at bedside because of the patient complaining of "funny vision " . The patient was tested with iPad based softwares encompassing everything from visual acuity, contranst sensitivity , colour sensitivity, Amslers, Stero vision , bedside iPad based visual field testing in order to give patients a state of the art management (4) The diagnosis of optic neuropathy was confirmed on OCT following dengue related micropsia which was diagnosed and monitored with Amslers test. The patient was observed to see if spontaneous resolution takes place as reported in some cases (2) . OCT was repeated when the Amsler was reported to be worse and the macular thickness was documented to be increased and so the patient was given steroids following which the micropsia resolved and the macular thickness reduced .Recently we have seen quite a few cases of Dengue with visual involvement including a patient who lost both eyes to dengue panophthalmitis like that reported earlier (23) and we would suggest that not asking for an eye check if a patient reports distortion or "funny vision" or reports the " Seet Quek Lim triad" of flashes of light,floaters and blurring of vision(29) , may now be construed as being negligence if patient loses vision eventually because dengue vision loss can be irreversible and may be seen in upto 10 percent of cases and severe cases may include cerebral venous thrombosis (13) which may be picked up by papillodema which can be diagnosed by a fundoscopy and very severe cases may proceed to even proptosis and globe rupture secondary to Dengue as seen by Nagaraj et al (18)
References:-
1) Juanarita, Jaafar et al. "Dengue Related Maculopathy and Foveolitis." Asian Pacific Journal of Tropical Biomedicine 2.9 (2012): 755-756.
2) Luk F, O, Chan C, K, Lai T, Y, A Case of Dengue Maculopathy with Spontaneous Recovery. Case Rep Ophthalmol 2013;4:28-33
3) Chan, David P.L. et al. "Ophthalmic Complications of Dengue." Emerging Infectious Diseases 12.2 (2006): 285-289.
4) Gan VC. Dengue: Moving from Current Standard of Care to State-of-the-Art Treatment. Current Treatment Options in Infectious Diseases. 2014;6(3):208-226. doi:10.1007/s40506-014-0025-1.
5)Ng AW, Teoh SC. Dengue eye disease. Surv Ophthalmol. 2015
Mar-Apr;60(2):106-14. doi: 10.1016/j.survophthal.2014.07.003. Epub 2014 Aug 12.
6) Teoh SC, Chee CK, Laude A, Goh KY, Barkham T, Ang BS; Eye Institute Dengue-related Ophthalmic Complications Workgroup. Optical coherence tomographypatterns as predictors of visual outcome in dengue-related maculopathy. Retina. 2010 Mar;30(3):390-8. doi: 10.1097/IAE.0b013e3181bd2fc6.
7) Su DH, Bacsal K, Chee SP, Flores JV, Lim WK, Cheng BC, Jap AH; Dengue Maculopathy Study Group. Prevalence of dengue maculopathy in patients hospitalized for dengue fever. Ophthalmology. 2007 Sep;114(9):1743-7.
8) Chee E, Sims JL, Jap A, Tan BH, Oh H, Chee SP. Comparison of prevalence of dengue maculopathy during two epidemics with differing predominant serotypes. Am J Ophthalmol. 2009 Dec;148(6):910-3. doi: 10.1016/j.ajo.2009.06.030.
9) Mendes TS, Sobrinho EF, Rosa AA, dos Anjos LM, da Costa GM, Souza Gda S, Gomes BD, Saito CA, da Silva Filho M, Silveira LC. Dengue maculopathy: visual electrophysiology and optical coherence tomography. Doc Ophthalmol. 2009 Oct;119(2):145-55. doi: 10.1007/s10633-009-9178-5.
10) Tan MH, Tan PE, Wong EN, Chen FK. Structure and function correlation in a patient with dengue-associated maculopathy. Clin Experiment Ophthalmol. 2014Jul;42(5):504-7. doi: 10.1111/ceo.12269.
11) Rani PK, Chhablani J, Bhargava A. Frosted Branch Angiitis in a Patient Co Infected With Dengue Hemorrhagic Fever and Malaria. JAMA Ophthalmol. 2015 Jun;133(6):e1568. doi: 10.1001/jamaophthalmol.2015.68.
12) Lim WK, Mathur R, Koh A, Yeoh R, Chee SP. Ocular manifestations of dengue fever. Ophthalmology. 2004 Nov;111(11):2057-64. PubMed PMID: 15522372.
13) Vasanthi N, Vairamon PM, Gowtham T, Das AK. Unusual Presentation of Dengue Fever-Cerebral Venous Thrombosis. J Clin Diagn Res. 2015 Jun;9(6):OD09-10. doi: 10.7860/JCDR/2015/13132.6068.
14) Sadiq N, Naqaish T, Arif A, Mohammad K, Jalis M. Central retinal artery occlusion secondary to dengue fever. J Ayub Med Coll Abbottabad. 2014 Jan-Mar;26(1):98-9.
15) Kanungo S, Shukla D, Kim R. Branch retinal artery occlusion secondary to dengue fever. Indian J Ophthalmol. 2008 Jan-Feb;56(1):73-4.
16)Gupta S, Das D. Subhyaloid haemorrhage in dengue fever. J Indian Med Assoc. 2013 Sep;111(9):623-4.
17) Jain S, Goswami A, Singh N, Kaur S. Bilateral eyelid ecchymosis and sub conjunctival haemorrhage manifesting as presenting feature in a case of dengue haemorrhagic fever. Trop Doct. 2014 Dec 24. pii: 0049475514565429.
18) Nagaraj KB, Jayadev C, Yajmaan S, Prakash S. An unusual ocular emergency in severe dengue. Middle East Afr J Ophthalmol. 2014 Oct-Dec;21(4):347-9. doi:10.4103/0974-9233.142276.
19) Veloso CE, Schmidt-Erfurth U, Nehemy MB. Choroidal neovascularization induced by immunogenic alteration of the retinal pigment epithelium in dengue Fever. Case Rep Ophthalmol. 2015 Jan 17;6(1):18-23. doi: 10.1159/000371791.
20) Rhee TK, Han JI. Use of optical coherence tomography to evaluate visual acuity and visual field changes in dengue fever. Korean J Ophthalmol. 2014
Feb;28(1):96-9. doi: 10.3341/kjo.2014.28.1.96.
21) Yamamoto K, Takahashi H, Kanno M, Noda Y, Fujino Y. Changes in parafoveal retinal thickness and subfoveal choroidal thickness in a patient with dengue fever-associated maculopathy. J Ophthalmic Inflamm Infect. 2013 Oct 31;3(1):63.doi: 10.1186/1869-5760-3-63.
22) Koh YT, Sanjay S. Characteristics and Ophthalmic Manifestations of the Classic Dengue Fever Epidemic in Singapore (2005-2006). Asia Pac J Ophthalmol (Phila).2013 Mar-Apr;2(2):99-103. doi: 10.1097/APO.0b013e31828a1917.
23) Saranappa S B S, Sowbhagya HN. Panophthalmitis in dengue fever. Indian Pediatr. 2012 Sep;49(9):760.
24) Chhavvi N, Venkatesh C, Soundararajan P, Gunasekaran D. Unusual ocular manifestations of dengue fever in a young girl. Indian J Pediatr. 2013 Jun;80(6):522-3. doi: 10.1007/s12098-012-0871-0.
25) Tabbara K. Dengue retinochoroiditis. Ann Saudi Med. 2012 Sep-Oct;32(5):530-3. doi: 10.5144/0256-4947.2012.30.4.1105.
26) Donnio A, B?ral L, Olindo S, Cabie A, Merle H. [Dengue, a new etiology in oculomotor paralysis]. Can J Ophthalmol. 2010 Apr;45(2):183-4. doi: 10.1139/i09-207.
27) Sanjay S, Wagle AM, Au Eong KG. Dengue optic neuropathy. Ophthalmology. 2009Jan;116(1):170; author reply 170. doi: 10.1016/j.ophtha.2008.08.015.
28) Pierre Filho Pde T, Carvalho Filho JP, Pierre ET. Bilateral acute angle closure glaucoma in a patient with dengue fever: case report. Arq Bras Oftalmol. 2008 Mar-Apr;71(2):265-8.
29) Seet RC, Quek AM, Lim EC. Symptoms and risk factors of ocular complications following dengue infection. J Clin Virol. 2007 Feb;38(2):101-5.
30) Chia A, Luu CD, Mathur R, Cheng B, Chee SP. Electrophysiological findings in patients with dengue-related maculopathy. Arch Ophthalmol. 2006 Oct;124(10):1421-6.
31) Sanjay S, Au Eong KG. Bilateral vitreous haemorrhage associated with dengue fever. Eye (Lond). 2007 Jan;21(1):144-5.
32) de Amorim Garcia CA, Gomes AH, de Oliveira AG. Bilateral stellar neuroretinitis in a patient with dengue fever. Eye (Lond). 2006
Dec;20(12):1382-3.
33) Cruz-Villegas V, Berrocal AM, Davis JL. Bilateral choroidal effusions associated with dengue fever. Retina. 2003 Aug;23(4):576-8.
Dear Dr. Evrensel,
I read with interest your recent paper in BMJ Case Reports. Based on the single case, you concluded that long-term Li treatment might cause cortical atrophy. Without pre-Li MRI or evidence for increases in GM following discontinuation of Li, this conclusion seems tentative. Even if this was the case, perhaps the brain changes were not related to the duration of use. We detected positive asso...
Dear Sir:
As a clinical neurologist with interest in laterality of motor control and consciousness, 1, 2 I read the contribution by Simonin and colleagues and have the following clinical and neurophysiological comments on their interpretation of the findings in their remarkable (presumably right handed) patient. Thus, a spontaneously breathing patient with a non- traumatic left subdural hematoma displayed bilate...
Masters and colleagues present an instructive case study in the May 16, 2014 issue of BMJ.(1) Unfortunately, it does not teach what they assert: namely, that "midlines should not be used for the administration of drugs which are vesicants and...potentially vesicant in nature...."
A cancer patient with a history of right-sided DVT secondary to a PICC, undergoes a failed effort to insert a left-sided PICC--due to...
We have read with attention the case report of Chhabra et al. describing the presentation of pleuropericardial effusion in a case of polymyalgia rheumatica (PMR)1. The authors described a case of an 86-year- old woman with a one-month history of progressive dyspnea, a two-month history of shoulder girdle pain, and significant weight loss. The report ruled out diagnoses of elderly-onset rheumatoid arthritis (EORA), because...
This is a very interesting and unusual case. In the absence of a biopsy-proven diagnosis, and in the absence of Autoantibodies, would Fabry's Disease enter the differential diagnosis ? The skin lesions in Fabry's may not be present initially and usually present later in the disease evolution.
Conflict of Interest:
None declared
I would like to congratulate the authors on the elaborate description of the MRI findings in their patient. Whereas, these findings are interesting, the more important question is what do they represent and what did the patient have? Neurological injury from myriad disorders, acute and chronic, can leave various intricate traces on the brain parenchyma. All of these can be given elaborate and idiosyncratic names. However...
Marappa Ganeshan and colleagues provide an intriguing case of acute compartment syndrome occurring after operative management of distal radius fracture under regional anesthesia with brachial plexus blockade (i.e. axillary block) as an outpatient surgical procedure.1 The patient, though comfortable in the immediate postoperative period, presented to the emergency department approximately 24 hours after discharge with an...
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