This is a typical case of diffusion restriction in an early phase of
Wallerian degeneration along the left corticospinal tract secondary to a
large left putaminal hemorrhage.
Diffusion weighted (DW) imaging shows the early phase of wallerian
degeneration as hyperintense associated with decreased ADC, presumably
representing axonal and reactive astrocytic swelling [1, 2]. DW high
signals can be observed after more than 24...
This is a typical case of diffusion restriction in an early phase of
Wallerian degeneration along the left corticospinal tract secondary to a
large left putaminal hemorrhage.
Diffusion weighted (DW) imaging shows the early phase of wallerian
degeneration as hyperintense associated with decreased ADC, presumably
representing axonal and reactive astrocytic swelling [1, 2]. DW high
signals can be observed after more than 24 hours following the associated
territorial infarction or hemorrhage[3].
1. Castillo M, Mukheriji SK (1999) Early abnormalities related to
postinfarction wallerian degeneration: evaluation with MR diffusion-
weighted imaging. JCAT 23:1004–1007
2. Kang DW, Chu K, Yoon BW, Song IC, Chang KH, Roh JK
(2000) Diffusion-weighted imaging in wallerian degeneration.
J Neurol Sci 178:167–169
3. Pierpaoli C, Barnett A, Pajevic S, Chen R, Penix LR, Virta A, Basser P
(2001) Water diffusion changes in Wallerian degeneration and their
dependence on white matter architecture. Neuroimage 13:1174-1185
With interest we read the article by You et al. describing the
identification of risk factors for aneurysm rupture and providing
estimations for the likelihood of rupture in unruptured intracranial
aneurysms [1]. However, we have concerns about the study design, the
presented analysis and the conclusions regarding the predictors for
aneurysm rupture.
With interest we read the article by You et al. describing the
identification of risk factors for aneurysm rupture and providing
estimations for the likelihood of rupture in unruptured intracranial
aneurysms [1]. However, we have concerns about the study design, the
presented analysis and the conclusions regarding the predictors for
aneurysm rupture.
First, the authors used a study population which included patients
with a newly diagnosed intracranial aneurysm who were treated by surgical
or endovascular techniques. Thus, for the unruptured aneurysms the authors
considered the risk of rupture too high to leave the aneurysm untreated.
Assessing predictors for rupture of aneurysms that are considered having a
risk of rupture high enough to warrant treatment is not very relevant.
From a clinical point of view predicting the risk of rupture of aneurysms
that are not treated (and thus may rupture in the near or distant future)
is much more relevant.
Second, the authors used a nested case control design and matched for
several clinical variables. This is an efficient design for etiologic
research (identification of risk factors), but is not suitable for
prognostic research (determine the risk of rupture based on multiple
predictors). Data from a case control study nested in a cohort of known
size can be used, but only when no matching is performed. The design most
suitable to address prognostic questions is a cohort study in which all
patients with a certain condition are followed for some time to monitor
the development of the outcome [2].
Finally, and not least important, the underlying frequencies of
potential predictors according to outcome in Table 1 do not correspond
with the univariable odds ratios mentioned in Table 2. For example, we do
not understand how the crude odds ratio for female sex can be different
from 1 (odds ratio 1.4, 95% CI 0.9-2.4) whereas the authors matched on
sex. Also, we do not understand how the crude odds ratio for the
association between hypertension and aneurysm rupture could be 1.9 (95% CI
1.2-3.2) whereas hypertension was less frequent in patients with a
ruptured aneurysm (35%) compared with patients with an unruptured aneurysm
(51%).
These concerns make interpretation of the presented results extremely
difficult. The study as presented can neither provide reliable information
on predictive risk factors for rupture nor can it form a proper basis for
decisions regarding the optimal therapeutic strategy for unruptured
intracranial aneurysms.
JP Greving1, Gabriel JE Rinkel2, Ale Algra1,2.
1 Julius Center for Health Sciences and Primary Care, University
Medical Center Utrecht, The Netherlands and 2 Department of Neurology and
Neurosurgery, Rudolf Magnus Institute of Neuroscience, University Medical
Center Utrecht, Utrecht, The Netherlands.
Correspondence to: Dr JP Greving, Julius Center for Health Sciences
and Primary Care, University Medical Center Utrecht, Stratenum 6.131, PO
Box 85500, 3508 GA Utrecht, The Netherlands. j.p.greving@umcutrecht.nl
Competing interests: None.
References
[1] You SH, Kong DS, Kim JS, et al. Characteristic features of unruptured
intracranial aneurysms: Predictive risk factors for aneurysm rupture. J
Neurol Neurosurg Psychiatry 2009; 0: jnnp.2008.169573v1.
[2] Moons KGM, Royston P, Verhouwe Y et al. Prognosis and prognostic
research: what, why, and how? BMJ 2009;338:b375.
We read with interest the manuscript by Bannier et al[1] describing
weight gain at 16 months following bilateral subthalamic nucleus deep
brain stimulation (STN DBS) placement in patients with Parkinson's disease
(PD). The authors concluded that weight gain following DBS is “life-
threatening,” increases cardiovascular risk, and is more than a mere
normalization towards a baseline weight in the absenc...
We read with interest the manuscript by Bannier et al[1] describing
weight gain at 16 months following bilateral subthalamic nucleus deep
brain stimulation (STN DBS) placement in patients with Parkinson's disease
(PD). The authors concluded that weight gain following DBS is “life-
threatening,” increases cardiovascular risk, and is more than a mere
normalization towards a baseline weight in the absence of parkinsonism.
Obviously due to the short follow-up and small sample size, they were
unable to examine the rate of cardiovascular events or other surrogates
for cardiovascular health post-operatively. It is certainly difficult to
assess the impact of a 5.5 kg increase in weight on cardiovascular risk
factors. Figure 3 in their manuscript shows a negative correlation
between the preoperative weight and weight gain. Since the preoperative
weight is part of the change, this could indicate regression towards the
mean or that the subjects were relatively underweight preoperatively; it
is difficult to know what the baseline weights of these patients would
have been in the absence of advanced PD. Indeed, weight loss associated
with PD has been described in many studies[2], and it seems likely that
the patients most under their normal weight would gain the most weight
following symptom control. Additionally, it remains unclear if the weight
gain reported by Bannier et al and other authors will persist over years
in these patients as PD progresses; there is some data that this weight
gain is in fact not sustained[3]. Although obesity is an independent risk
factor for cardiovascular disease, previous studies have associated
chronic obesity rather than subacute weight gain with risk of
cardiovascular events[4, 5]. Furthermore, Bannier et al. argue that the
lack of correlation between motor improvement from DBS and weight gain in
prior studies indicates that the weight change is not related
mechanistically to relief of motor symptoms by DBS. Such a situation may
arise when there are floor or ceiling effects on one of the correlation
variables, and the large effect size and small standard deviations suggest
that nearly universal adequate motor improvement is occurring. Almost all
studies evaluating weight change after stereotactic surgery for PD are
uncontrolled, therefore an association between weight change and the
Unified Parkinson Disease Rating Scale (UPDRS) “off” change may be elusive
since the majority the DBS patients will sustain both remarkable
improvement in motor function and some degree of weight gain. If matched
controls with advanced PD and no DBS were incorporated into the
correlation analysis, they would likely show worsening of the UPDRS “off”
medications and weight loss over the same time interval, increasing the
likelihood of the demonstration of a significant correlation. While
excessive post-operative weight gain is undesirable in some patients,
future studies should examine if weight gain associated with DBS is
sustained and if it has a significant impact on cardiovascular health
before the conclusions of Bannier et al. can be drawn. The results of
such efforts have the potential to shift the benefit versus risk equations
for patients in whom this treatment may dramatically impact quality of
life.
Competing interests: None.
REFERENCES
[1] Bannier S, Montaurier C, Derost PP, et al. Overweight after deep
brain stimulation of the subthalamic nucleus in Parkinson disease: long
term follow-up.
J Neurol Neurosurg Psychiatry 2009;80(5):484-488.
[2] Chen H, Zhang SM, Hernan MA, et al. Weight loss in Parkinson's
disease.
Ann Neurol 2003;53(5):676-679.
[3] Novakova L, Ruzicka E, Jech R, et al. Increase in body weight is a non
-motor side effect of deep brain stimulation of the subthalamic nucleus in
Parkinson's disease.
Neuro Endocrinol Lett 2007;28(1):21-25.
[4] Garrison RJ, Castelli WP. Weight and thirty-year mortality of men in
the Framingham Study.
Ann Intern Med 1985;103(6(Pt 2)):1006-1009.
[5] Manson JE, Willett WC, Stampfer MJ, et al. Body weight and mortality
among women.
N Engl J Med 1995;333(11):677-685.
I read with interest the article by Viegas et al. 1 about the
symptomatic, radiological and pathological involvement of the hypothalamus
in neuromyelitis optica (NMO). Their case review of a young lady presented
with spontaneous vomiting and behavioral change, confirmed the diagnosis
of NMO after the identification of aquaporin 4 antibody(AQP4 Ab).
I read with interest the article by Viegas et al. 1 about the
symptomatic, radiological and pathological involvement of the hypothalamus
in neuromyelitis optica (NMO). Their case review of a young lady presented
with spontaneous vomiting and behavioral change, confirmed the diagnosis
of NMO after the identification of aquaporin 4 antibody(AQP4 Ab).
We also have many patients presented with the similar symptoms which
later diagnosed of NMO whether by the identification of AQP4 Ab or
according to the revised NMO diagnostic criteria. 2 Some patients
presented with prolonged nausea for 1 month, or even combined with
confusion, but without significant gastroenterology abnormality. Some
resolved later spontaneously without particular treatment, but some need
high dose steroid treatment or plasma exchange.
As a neurologist in Asian country, NMO patients are more frequent
encountered and are easily being recognized when presenting with optic
neuritis or myelitis. However, NMO patients with brainstem involvements as
the case reporting are not easily being diagnosed, especially during the
first attack. We clinician should be aware of the brainstem symptoms in
young ladies who are at risk of NMO.
References
1. Viegas S, Weir A, Esiri M. et al. Symptomatic, radiological and
pathological involvement of the hypothalamus in neuromyelitis optica.
J
Neurol Neurosurg Psychiatry 2009;80:679-82
2. Wingerchuk DM, Lennon VA, Pittock SJ, et al. Revised diagnostic
criteria for neuromyelitis optica.
Neurology. 2006;66:1485-9.
In the article “Marked increase in cerebrospinal fluid glial
fibrillar acidic protein in neuromyelitis optica: an astrocytic damage
marker” J. Neurol. Neurosurg. Psychiatry 2009;80;575-577, the authors
found a significant increase in the CSF-GFAP levels during relapse in NMO
patients which were several thousand times higher than those found in
other neurological diseases (MS, OND, spinal infarct...
In the article “Marked increase in cerebrospinal fluid glial
fibrillar acidic protein in neuromyelitis optica: an astrocytic damage
marker” J. Neurol. Neurosurg. Psychiatry 2009;80;575-577, the authors
found a significant increase in the CSF-GFAP levels during relapse in NMO
patients which were several thousand times higher than those found in
other neurological diseases (MS, OND, spinal infarction and ADEM).
Although the results are very interesting, the use of the new criteria for
the diagnosis of NMO with only the inclusion of patients seropositive for
AQP4-antibody creates a bias. If the criteria for the diagnosis of NMO
described by Wingerchuk et al in 1999 (ref.1) were used, this group of
patients would be markedly diverse clinically. As described before by the
same authors, in immunopathological studies of autopsied cases of NMO, the
staining of GFAP was lost in the NMO lesions lacking AQP4
immunoreactivity. It would be expected that patients seropositive for AQP4
-antibody had abnormalities in CSF-GFAP levels. Although NMO IgG positive
antibodies in NMO patients confers a worse disease course and have a high
specificity, the sensitivity of the exam vary widely between different
populations with a tendency to be lower where the population has a
predominant African ancestry (ref.2). If seronegative patients that
fulfilled the initial (Wingerchuk et al- 1999) criteria for NMO were
included in the sample, maybe the medium level of GFAP would have
decreased considerably. I has to be clarified that these findings are
valid only for the restrict group of NMO patients that are seropositive
for AQP4-antibody and that do not represent all the spectrum of the
disease.
References
1. Wingerchuk DM, Hogancamp WF, O’Brien PC, et al. The clinical course
of
neuromyelitis optica (Devic’s syndrome).
Neurology 1999;53:1107–14.
2. Cabrera-Gómez J, Bonnan M, González-Quevedo A et al. Neuromyelitis
optica positive antibodies confer a worse course in relapsing-
neuromyelitis optica in Cuba and French West Indies.
Mult Scler. 2009
Jul;15(7):828-33.
I read with interest the case report by Schulze-Bonhage et al.
documenting the termination of complex partial status epilepticus in a
patient following the intravenous administration of levetiracetam 1.
Schulze-Bonhage’s patient had seizures refractory to multiple frontline
anti-epileptic medications and lapsed into complex partial status
epilepticus when her pre-admission seizure medications were...
I read with interest the case report by Schulze-Bonhage et al.
documenting the termination of complex partial status epilepticus in a
patient following the intravenous administration of levetiracetam 1.
Schulze-Bonhage’s patient had seizures refractory to multiple frontline
anti-epileptic medications and lapsed into complex partial status
epilepticus when her pre-admission seizure medications were held during
the course of an elective admission for video EEG monitoring. This
admission was carried out as a part of epilepsy surgery work-up.
Intravenous loading of phenytoin and oral administration of lorazepam
failed to abort the status. A gratifying clinical response was achieved
within 35 minutes of intravenous levetiracetam administration.
The mechanism for the anticonvulsant effect of levetiracetam is
unique and still not fully understood. It does not seem to act through the
traditional mechanisms of ion channel blockage but rather is thought to
inhibit burst firing and propagation of seizure activity. This unique
mechanism of action may make it effective where other traditional anti-
epileptic drugs fail.
Schulze-Bonhage et al. report adds to the growing body of literature
demonstrating the effectiveness of levetiracetam in status epilepticus of
various types 2,3. Maybe it is time that conventional status epilepticus
abatement protocol of benzodiazepine->phenytoin- >phenobarbital
followed by either midazolam, propofol or pentobarbital infusion be
modified to give levetiracetam its just due.
Disclosure: None
References
1. Schulze-Bonhage A, Hefft S, Oehl B. Termination of complex partial
status epilepticus by intravenous levetiracetam.
J Neurol Neurosurg
Psychiatry 2009; 80: 931-933.
2. Möddel G, Bunten S, Dobis C, Kovac S, Dogan M, Fischera M, Dziewas
R, Schäbitz WR, Evers S, Happe S. Intravenous levetiracetam: a new
treatment alternative for refractory status epilepticus.
J Neurol
Neurosurg Psychiatry. 2009 Jun; 80(6):689-92.
3. Altenmüller DM, Kühn A, Surges R, Schulze-Bonhage A. Termination
of absence status epilepticus by low-dose intravenous levetiracetam.
Epilepsia. 2008 Jul; 49(7):1289-90.
Autoimmune diseases are caused by aberrant response of immune system
directed against triggering epitopes.(1)Coincidental occurrence of
multiple autoimmune disorders in given patient suggests either common or
similar pathogenetic mechanisms.(1)The concept of molecular mimicry hold
that an agent may share epitopic determinants with nervous system tissues
and incites immune responses.
Chronic inflammatory demyelinating po...
Autoimmune diseases are caused by aberrant response of immune system
directed against triggering epitopes.(1)Coincidental occurrence of
multiple autoimmune disorders in given patient suggests either common or
similar pathogenetic mechanisms.(1)The concept of molecular mimicry hold
that an agent may share epitopic determinants with nervous system tissues
and incites immune responses.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and
Multiple Sclerosis (MS) are acquired diseases evolving with progressions
and relapses.
Zéphir et al (2)recently reported five patients with relapsing
demyelinating disease affecting CNS and PNS and reviewed previously
described cases.
In Zephir et al (2)patients, demyelinating process started in CNS with
subsequent extension to peripheral nerves.
We experienced three patients affected by concurrent, both symptomatic,
peripheral and central demyelination. Chronology of their histories
demonstrated that bouts of symptoms initiated within PNS with extension
to CNS. Case 1 when aged 18 years, developed recurrent episodes of
unilateral complete facial palsy and distal paresthesias. On 1st
admission, deep jerks were unevokable. Brain magnetic resonance imaging
(MRI),visual evoked responses (VERs),nerve conduction were normal.Ten
years later, patient experienced hand tremor,distal extremity
numbness,imbalance.On examination, there were sustained nystagmus on
either lateral gaze, limb ataxia, positive Romberg sign, 3/5 MRC scale
distal weakness, loss of perception, areflexia. Routine laboratory tests
were normal, except for hypothyroidism due to earlier thyroiditis. CSF had
no oligoclonal IgG bands (OCIgG).Antibody assay for gangliosides, myelin
associated glycoprotein (MAG)was negative.PMP22 point
mutations,duplications,deletions and P0,connexin 32 mutations were
negative. Brain MRI was normal whereas there were multiple spinal cord
enhancing lesions fromC1 to Th7.
Electrophysiology showed demyelinating polyneuropathy with proximal and
distal motor conduction block (CB.(3)Sural potential had low amplitude(3
uV,normal >6.High doses of methylprednisolone(1gr i.v daily for 3 days)
had benefit. Four months later, patient presented with vertigo,dysarthria,
blurred vision. VERs were altered. On MRI, a bulbar enhancing lesion was
found. Methylprednisolone i.v was repeated.Immune globulin was afterwards
administered (1gr /kg/ body weight)every two to three months. During
following five years, serial electrophysiology confirmed demyelination as
indicated by slowed motor velocity (within 28 and 32 m/sec),delayed F
waves,dispersed motor responses throughout. Brain and spinal cord MRI
showed dissemination, fulfilling Barkhof's criteria for MS
diagnosis.(4)The disease had no further clinical recurrences.
Case 2 since early youth experienced tingling pain in hands and feets.
Fourteen years later, patient was admitted because of myalgias and limb
distal numbness. Neurological examination revealed 3/5 MRC proximal and
distal extremity weakness,areflexia. Electrophysiology demonstrated
sensorimotor demyelinating neuropathy with CB in both median and ulnar
nerves.Muscle biopsy had neurogenic features.Laboratory tests and brain
MRI were unremakable.At age of 35, patient was admitted because of blurred
vision, imbalance,acral paresthesias. On examination, there were
tremor,limb,trunk ataxia, areflexia, distal loss of strength (2/5
MRC),impaired sensation. CSF had increased protein (50 mg/dl, normal <
45) and OCIgG bands.VERs were normal.Brain MRI showed numerous
periventricular and subcortical T2 hyperintense lesions, fulfilling
Barkhof’s criteria for dissemination.(4)
Electrophysiology confirmed ongoing demyelination in peripheral nerves.(3)
No antiganglioside nor anti-MAG antibodies were detected. Oral prednisone
was given (50 mg every other day).One year later, patient developed renal
insufficiency due to hypertension. Cognitive decline and seizures
complicated the illness, which was marked by recurrent motor deficits in
lower limbs.
Our third case,with past history of hyperthyroidism, presented after two
years of lumbar pain,distal extremity numbness,waddling gait. On
examination, extremity strength was graded 3/5 proximally, 2/5 distally on
MRC scale. Moreover,there were stepping gait,areflexia,muscular
atrophy,distal impairment of all perception modalities. Electrophysiology
revealed sensorimotor demyelinating neuropathy, without CB. Sural biopsy
showed endoneural oedema, fiber loss, epineurial T cell infiltrates.VERs,
MRI and CSF were unremarkable.Immune globulin (0,4 gr /kg body weight for
four days ) was given and repeated with benefit.Type II diabetes was
discovered three years later. Patient motor deficits relapsed twice within
five years. On latest examination,there were nystagmus, scanned speech,
trunk ataxia. Brain MRI showed multiple T2 hyperintense, enhancing white
matter lesions suggestive of MS.(4) Serial electrophysiology confirmed
peripheral nerve demyelination.
Discussion: Our patients presented stepwise recurrent and chronically
progressive demyelinating process initiated within PNS with subsequent
extension to CNS.
PNS presenting symptoms predated by ten years in case 1,by two to three
years in case 2 and 3 CNS clinical and neuroradiological changes, which
progressed in parallel. Transiently their disease responded to steroids or
immunomodulating treatment. None had antecedent infections. Antibodies to
gangliosides and MAG were absent. No connexin 32, P0, PMP 22 mutations
were found. All patients had associated thyroid disfunction: case 2
developed diabetes. Exact significance of such association,if any, is not
clear,though it may suggest susceptibility to multiple immune mediated
diseases.
Case 2 developed renal failure related to hypertension years after
neurologic onset. CIDP of our patients was diagnosed on account of
required criteria.(3) Sural biopsy confirmed diagnosis of case 3. VERs
were abnormal only in case 2. Interestingly, CSF obtained after onset of
peripheral signs showed OCIgG only in second case.
MRI abnormalities in all fullfilled Barkhof’s criteria for
dissemination.(4)
Concurrent CIDP and CNS demyelination may progress either overtly or
clinically silent.(2)
Myelin P1 expressed in peripheral nerves is identical to central myelin
basic protein; moreover cross reactivity between cryptic antigenic targets
in CNS and PNS might be crucial.(1-2)
Zephyr et al (2) on account of clinical and laboratory data consider their
patients affected by new demyelinating entity distinct from classical MS
and CIDP:none of their cases had CB or abnormal temporal dispersion
although fulfilled criteria for CIDP.(3)
None of Zephyr et al patients (2) had CSF OCIgG bands, one had initial
pleiocytosis, three hyperproteinorrachia.
It is known that MS patients may lack oligoclonal bands.(5)
Among the 100 CIDP described by Bouchard et al (5) two out of five with
symptomatic CNS involvement had OCIgG bands , three MRI features of MS.
Overall neurologic features of reported cases (2,5) raise issue as to
whether they represent overlap version or distinct variant of CIDP and MS.
Regardless which mechanism may underlie their disease, patients with
concurrent CNS and PNS demyelination seem to share rather similar
immunopathogenesis suggesting a spectrum of dysimmune attacks against
myelin.(1, 2,5 )
References
1. Koller H,Schoeter M, Kieseier BC, Hartung HP . Cronic inflammatory
demyelinating polyneuropathy-update on pathogenesis , diagnostic criteria
and therapy. Curr Opin Neurol 2005;18: 273-8.
2. Zéphir H, Stojkovic T, Latour P et al Relapsing demyelinating disease
affecting both the central and peripheral nervous systems. J Neurol
Neurosurg Psychiatry 2008;79:1032-39.
3. Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task
Force. Research criteria for diagnosis of chronic inflammatory
demyelinating polyneuropathy. Neurology 1991 ;41: 617-18
4. Barkhof F, Filippi M, Miller DH.Comparaison of MRI imaging criteria at
first presentation to predict conversion to clinically definite multiple
sclerosis.Brain 1997;120:2059-69.
5. Bouchard C, Lacroix C, Plante’ V et al .Clinicopathologic findings and
prognosis of chronic inflammatory demyelinating polyneuropathy.Neurology
1999:52: 498-503.
I came across the article of Dr. Sarikcioglu and Dr. Sindel (1) and I
would like to add a few additional items on the contributions of Professor
Pierre Mollaret to science and in particular to Neurology. Dr. Mollaret
was a Professor of General Pathology who dedicated most of his time to the
study of both the treatment and the epidemiology of infectious diseases.
Nevertheless he had a real interest in...
I came across the article of Dr. Sarikcioglu and Dr. Sindel (1) and I
would like to add a few additional items on the contributions of Professor
Pierre Mollaret to science and in particular to Neurology. Dr. Mollaret
was a Professor of General Pathology who dedicated most of his time to the
study of both the treatment and the epidemiology of infectious diseases.
Nevertheless he had a real interest in neurological diseases as a result
of his training in La Salpetrière with Professor Georges Guillain. Even
then he clearly had an inclination for infectious diseases as is shown by
his work on the neurological sequelae of von Economo’s encephalitis
lethargica (2).
On the other hand, the need to provide respiratory assistance to victims
of poliomyelitis led Professor Mollaret to promote one of the first
reanimation facilities in France in the Claude Bernard Hospital. The
maintenance of respiratory assistance at all costs in patients in coma
enabled Professor Mollaret, together with Marcel Goulon, to describe brain
death, a stage beyond coma (“le coma depassé”) (3), the frontier between
life and death, as he himself repeatedly referred to it. It is probably
his greatest contribution to neurology and medicine in general but Dr.
Sarikcioglu and Dr. Sindel (1) did not refer to it.
As for the famous Guillain-Mollaret triangle usually drawn between the
dentate nucleus, red nucleus, inferior olivary nucleus and back to the
dentate nucleus, I would like to point out that in fact it does not exit
as there is not a direct connection between the inferior olivary nucleus
and the contralateral dentate nucleus.
Palatal myoclonus (or rather, tremor) is the main clinical syndrome due to
lesions in the dentato-olivary pathway. The late Professor Jean Lapresle
carried out the most important anatomical work demonstrating the strict
topological relationship in the dentato-olivary pathway. Professor
Lapresle contributed several articles on the subject (4, 5), written with
a Benedictine neuropathological meticulousness.
During my training in Paris in the seventies of the past century, I was
able to meet Professor Mollaret, already retired, who regularly attended
the sessions of the Societé Française de Neurologie, always elegantly
dressed and wearing a bowtie. I was also fortunate enough to have
Professors Goulon and Lapresle as my “patrons” in Garches and Bicêtre
respectively, and this is a small homage of gratitude to their memory.
References
1. Sarikcioglu L, Sindel M. Pierre Mollaret (1898-1987) and his
legacy to science.
J Neurol Neurosur Psychiatry 2007;78: 1129-1135
2. Guillain G, Mollaret P. Les sequelles de l’encéphalite épidémique.
Étude clinique et thérapeutique. Paris, G. Doin, 1932.
3. Mollaret P, Goulon M. Le coma depassé. Memoire préliminaire.
Rev Neurol
(Paris) 1959 ;101 :3-15.
4. Lapresle J, Ben Hamida M. The dentato-olivary pathway. Somatotopic
relationship between the dentate nucleus and the contralateral inferior
olive.
Arch Neurol 1970;22:135-143
5. Lapresle J. Rhythmic palatal myoclonus and the dentato-olivary pathway.
J Neurol 1979;220:223-230
we are indebted with Dr. Josef Finsterer and Dr. Marlies Frank
because they give us the chance to explain some points of our paper. We
will reply to their concerns as requested.
We followed the most restrictive diagnostic criteria of the AAN
because we needed to extremely select our patients. Our neurophysiological
criteria are slightly more restrictive than the AAN criteria and we have
r...
we are indebted with Dr. Josef Finsterer and Dr. Marlies Frank
because they give us the chance to explain some points of our paper. We
will reply to their concerns as requested.
We followed the most restrictive diagnostic criteria of the AAN
because we needed to extremely select our patients. Our neurophysiological
criteria are slightly more restrictive than the AAN criteria and we have
reported it in the methods. We thought it was valuable.
In the Results section we have pointed out that our patients had no
sensory disturbances at first examination "on clinical grounds". It does
not exclude a neurophysiological or pathological involvement of the
sensory nerves as documented.
We have admitted that NIS is much more sensitive to changes in motor
than in sensory disturbances, but nevertheless improvement in motor
function is significant. SAP reduction is prevalent in diabetic patients
with sensory symptoms. We speculated that sensory disturbances are related
to superimposed diabetic sensory polyneuropathy and not to worsening of
the CIDP because of the concomitant improvement of motor function. It is
well known that even in patients with long term excellent glycaemic
control the incidence of diabetic neuropathy remains elevated (Martini CL
et al, Diabetes Care 2006, 29, 340-344).
Obviously we do not know why these 2 patients did not respond to IVIg
therapy. Our established criteria to classify patients as treatment
responders or non-responders are clearly pointed out in the methods
section. We evaluated the response to the first course of IVIg. Non-
responders remained unchanged or worsened. This choice could be
inappropriate but we established it before the beginning of the study.
Patients classified as treatment responders were treated again in case of
relapse. Relapse was defined as worsening after improvement with an
increase of 4 points or more in the NIS score (see methods - treatment and
outcome).
No patient had renal insufficiency at baseline (see results: "only
one patient complained of other complication of diabetes-retinopathy") and
no patient developed it during IVIg therapy.
Neurophysiological studies of the ulnar nerve were carried out. There
is an overlap between ulnar and median nerve data.
Last, we have no clear explanation for our male preponderance.
It is interesting to note that, Figure 3 Shows that Mean Memory
factor score for Neurofibrillary tangles(NFT) score-5 is 40.1,30.3 and
13.0 for plaques in CA1, plaques in subiculum and plaques in entorhinal
cortex respectively which is less compared to the memory scores of higher
NFT scores like 5 to 15 and >15. But this can be cautiously
interpreted as memory scoring is not directly related t...
It is interesting to note that, Figure 3 Shows that Mean Memory
factor score for Neurofibrillary tangles(NFT) score-5 is 40.1,30.3 and
13.0 for plaques in CA1, plaques in subiculum and plaques in entorhinal
cortex respectively which is less compared to the memory scores of higher
NFT scores like 5 to 15 and >15. But this can be cautiously
interpreted as memory scoring is not directly related the Plaques density
in the specific anatomical regions because of uneven distribution of data.
Also with similar observational results were present as shown for
Neurophil Threads(NT) in CA1, Subiculum and entorhinal cortex for severity
scoring. This may be going in contrast with the authors opinion “..
amyloid plaques in either region of the hippocampal formation,
particularly in the entorhinal cortex, were associated with memory
function in cross sectional and longitudinal analyses.”
It is not clear whether the dominant or non-dominant cerebral lobe is
evaluated for histopathological sections. This may be one of the major
limiting point because the pathogenesis, interpretation of cognitive
function results and progression of the cognitive decline are cerebral
hemispheres specific.
It is also interesting to note the important observation by the
authors that, the analyses relating Braak stage or counts of plaques, NFT
and NT in the frontal and parietal lobes with cognitive performance, none
of the neuropathological measures was associated with memory or any other
cognitive domain.
This is a typical case of diffusion restriction in an early phase of Wallerian degeneration along the left corticospinal tract secondary to a large left putaminal hemorrhage. Diffusion weighted (DW) imaging shows the early phase of wallerian degeneration as hyperintense associated with decreased ADC, presumably representing axonal and reactive astrocytic swelling [1, 2]. DW high signals can be observed after more than 24...
Dear Editor,
With interest we read the article by You et al. describing the identification of risk factors for aneurysm rupture and providing estimations for the likelihood of rupture in unruptured intracranial aneurysms [1]. However, we have concerns about the study design, the presented analysis and the conclusions regarding the predictors for aneurysm rupture.
First, the authors used a study popul...
Dear Editor,
We read with interest the manuscript by Bannier et al[1] describing weight gain at 16 months following bilateral subthalamic nucleus deep brain stimulation (STN DBS) placement in patients with Parkinson's disease (PD). The authors concluded that weight gain following DBS is “life- threatening,” increases cardiovascular risk, and is more than a mere normalization towards a baseline weight in the absenc...
Dear Editor,
I read with interest the article by Viegas et al. 1 about the symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica (NMO). Their case review of a young lady presented with spontaneous vomiting and behavioral change, confirmed the diagnosis of NMO after the identification of aquaporin 4 antibody(AQP4 Ab).
We also have many patients presented with...
Dear Editor,
In the article “Marked increase in cerebrospinal fluid glial fibrillar acidic protein in neuromyelitis optica: an astrocytic damage marker” J. Neurol. Neurosurg. Psychiatry 2009;80;575-577, the authors found a significant increase in the CSF-GFAP levels during relapse in NMO patients which were several thousand times higher than those found in other neurological diseases (MS, OND, spinal infarct...
Dear Editor,
I read with interest the case report by Schulze-Bonhage et al. documenting the termination of complex partial status epilepticus in a patient following the intravenous administration of levetiracetam 1. Schulze-Bonhage’s patient had seizures refractory to multiple frontline anti-epileptic medications and lapsed into complex partial status epilepticus when her pre-admission seizure medications were...
Autoimmune diseases are caused by aberrant response of immune system directed against triggering epitopes.(1)Coincidental occurrence of multiple autoimmune disorders in given patient suggests either common or similar pathogenetic mechanisms.(1)The concept of molecular mimicry hold that an agent may share epitopic determinants with nervous system tissues and incites immune responses. Chronic inflammatory demyelinating po...
Dear Editor,
I came across the article of Dr. Sarikcioglu and Dr. Sindel (1) and I would like to add a few additional items on the contributions of Professor Pierre Mollaret to science and in particular to Neurology. Dr. Mollaret was a Professor of General Pathology who dedicated most of his time to the study of both the treatment and the epidemiology of infectious diseases. Nevertheless he had a real interest in...
Dear Editor,
we are indebted with Dr. Josef Finsterer and Dr. Marlies Frank because they give us the chance to explain some points of our paper. We will reply to their concerns as requested.
We followed the most restrictive diagnostic criteria of the AAN because we needed to extremely select our patients. Our neurophysiological criteria are slightly more restrictive than the AAN criteria and we have r...
Dear editor
It is interesting to note that, Figure 3 Shows that Mean Memory factor score for Neurofibrillary tangles(NFT) score-5 is 40.1,30.3 and 13.0 for plaques in CA1, plaques in subiculum and plaques in entorhinal cortex respectively which is less compared to the memory scores of higher NFT scores like 5 to 15 and >15. But this can be cautiously interpreted as memory scoring is not directly related t...
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