I have read all the three articles (1), (2), (3), published in Vol .77, 2006, and found them to be very good studies. It also gives a new nsight to Parkinson’s disease and seems to be very useful in our day to day practice. In this connection I would like to add a few things.
Camptocormia was first described by Brodie, in 1918. Reichel G et al, (4) proposed for new classification for cam...
I have read all the three articles (1), (2), (3), published in Vol .77, 2006, and found them to be very good studies. It also gives a new nsight to Parkinson’s disease and seems to be very useful in our day to day practice. In this connection I would like to add a few things.
Camptocormia was first described by Brodie, in 1918. Reichel G et al, (4) proposed for new classification for camptocormia , as -(a)Primary form, which is a segmental dystonia of abdominal muscles, (b) Secondary forms, where Parkinson’s disease is one of the causes.
Azher S.N. et al ,( 5) also proposed camptocormia to be a
heterogeneous disorder having multiple etiologies and needs etiological classification,which also responds poorly to levodopa therapy. D Jaldetti R et al, (6) considered camptocormia to be either a rare form of
dystonia or an extreme form of rigidity. In his study the response to levodopa was variable, even in some cases camptocormia was aggravated. Slawek J et al, (7) concluded in his study that camptocormia is probably not a form of dystonia of trunk, but may be dysfunction in other non
dopaminergic nigrostrital projection. Since then many theories have been suggested, but the exact pathogenesis of this is not yet clear. F Blotch, et al (1) have tried to link the hypothesis of focal dystonia as primary
cause,and focal myopathy as a secondary event. It seems more or less justifiable. Now if dystonia is the primary cause in camptocormia then it is worth trying botulinum toxin as a form of treatment for these camptocormia, but the exact dose, the muscles to be injected needs further
studies. Melamed et al, (8) reported that occasional patients may benefit from intramuscular botulinum toxin injection or from deep brain stimulation. However it needs further studies to explore the real pathogenetic mechanism of camptocormia in Parkinson’s disease so as to find the correct mode of treatment.
Micheli F et al, (9) in a patient of camptocormia with Parkinson’s disease reported that camptocormia improved with bilateral pallidal stimulation. Yamada K et al, (10) reported a striking alleviation of camptocormia after chronic bilateral sub-thalamic stimulation, Boulos Paul Bijjenni et al, (11), also reported that bilateral timulation of sub thalamus improves the axial symptoms of Parkinson’s disease. There is also a synergistic effect obtained when levodopa was used in conjunction
with bilateral stimulation of sub thalamic nucleus stimulation. So a comparative trial is needed to find out the best form of therapy.
Keeping these in view, patients presenting with camptocormia, should be thoroughly investigated for the presence of Parkinson’s disease. If they are found to
have Parkinsons disease, then which modes of treatment is the best for the patient on an individual basis, is the question. Whether the combination of deep brain stimulation of sub-thalamic nucleus and levodpa therapy, or only deep brain stimulation, or levodopa in
conjunction with botulinum toxin injection is better, for alleviating the axial dystonia as well as other features of Parkinson’s disease needs a controlled trial.
References
1. F Blotch, J L Houteo, S Tenzenas du Montcel, F Bonneville, F Etchepare, M L Weter, S Rivaud-Pechoux, V Hahn Barma, T Maisonobe, C Behar, J Y Lazennec, E KURYS, I Arnuf, A M Bonnet, Y Agid. Parkinson’s disease with camptocormia. J Neurol Neurosurg Pschiatry, 2006,77,1223-1228
2. A-C Lepoutre, D Devos, A Blanchard, Dauphin, V Pardessus, C A Maurage, D Ferriby, J F Hurtevent, A Cotton, A destee, L Defebvre. A specific patteren of camptocormia in Parkinson’s Disease. J Neurol Neurosurg Psychiatric,2006,77,1229-1234.
3. R Djaldetti,E Melamed. Camptocormia in Parkinson’s disease: new insights. J Neurol Neurosurg Pschiatry, 2006, 77, 1205.
4. Reichel G, Kirchhofer U, Stenner A. Camptocormia-segmental dystonia. Proposal of a new defination for an old disease. Nervenarzt 2001,April,72,(4):281-285. Abstract.
5. Azher S N,Jancovic J. Camptocormia: pathogenesis, classification, and response to therapy. Neurology, 2005, Aug 9,65,(3):355-359.
6. Djaldetti R, Mosberg-Galili R,Soroka H, Merims D, Melamed E. Camptocormia(bent spine) in patients with Parkinson’s disease-charecterization and possible pathogenesis of an unusual phenomena. Mov Disord, 1999, May, 14(3):443-447.
7. Slawek J, Derejko M, Camptocormia,a rare form of motor system disorders in Parkinson’s disease. Neurol Neurochir Pol. 2001 35(6): 1133-40 Abstract.
8. Melamed E, Djaldetti R. Cam,ptocormia in Parkinson’s disease. J Neurol.Dec,253 Suppl7,vii14-vii16.
9 .Micheli F, Cersosimo M G, Piedimonte F. Camptocormia in a patient with Parkinson’s disease: beneficial effects of pallidal deep brain stimulation. Case report, JNeurosurg.2005, Dec, 103, 6):1081-1083.
10. Yamada K, Goto S, Matsuzaki K, TamuraT, MuraseN, ShimazuH, Nagahiro S,Kuratsu J,Kaji R. Alleviation of camptocormia by bilateral subthalamic nucleus
stimulation in a patient with Parkinson’s disease. Parkinsonism Relat Disorder 2006, 12,(6):372-5,Epub2006 May 30.Abstract.
11. Boulos-Paul Bejjani,David Gervais,Isbelle Arnulf Savas
Papadopoulos,Sophie Demeret,Anne Marie Bonnet,Philippe
Cornu,PhilippeDamier,Yves Agid. Axial Parkinsonian symptoma can be improved:the role of levodopa and bilateral subthalmic stimulation. J Neurol Neurosurg Psychiatric, 2000, 68,595-600.
Leach et al (“Which electroencephalography (EEG) for epilepsy? The relative usefulness of different EEG protocols in patients with possible
epilepsy.” Journal of Neurology, Neurosurgery & Psychiatry.
2006;77:1040-2) compared the yield of 3 types of EEGs in 85 patients with epilepsy, diagnosed after two or more generalized tonic clonic seizures (GTCs). The median age of the patients was 17.9 years...
Leach et al (“Which electroencephalography (EEG) for epilepsy? The relative usefulness of different EEG protocols in patients with possible
epilepsy.” Journal of Neurology, Neurosurgery & Psychiatry.
2006;77:1040-2) compared the yield of 3 types of EEGs in 85 patients with epilepsy, diagnosed after two or more generalized tonic clonic seizures (GTCs). The median age of the patients was 17.9 years and maximum age 35
years (interquartile range 15.7 – 22.1 years).
In the study, each patient received 3 EEGs in random order, separated by at least 7 days, prior to initiation of treatment. EEGs were read blind to protocol by a single reader. The authors report more epileptiform
abnormalities occurred in sleep deprived (SD) EEGs and conclude that SD-EEGs should be considered as a preferred protocol for “young patients presenting with epilepsy.”
This is an important study, and its emphasis on identifying a cost-effective protocol is much appreciated, but it is worth emphasizing a few points with regard to its conclusions.
First, the results of this study should not be generalized to “young patients” who are children. In two separate pediatric studies we have published of 820 EEGs performed as routine, partially sleep deprived, and fully sleep-deprived protocols, and of 2500 EEGs performed either sleep-deprived or routine protocols, we found no evidence that SD-EEGs had a higher yield.[1][2]
Second, the authors’ use of the term “Sensitivity” is not consistent with standard usage. The test haracteristic “Sensitivity,” also known as “Positive Predictive Value,” refers to the proportion of individuals “with-the-disease” who have a positive test result. The presence of the disease, the denominator of this proportion, is determined by a separate
“Gold Standard” for diagnosis. In contrast, the authors’ criteria for disease is not a based on any separate gold standard but is the sum total of individuals with epileptiform discharges on any of 3 EEGs they performed. This is post hoc. More importantly, this fails to acknowledge two forms of diagnostic misclassification: patients with epilepsies with normal EEGs and patients for whom an EEG may be “over-read” or inaccurately interpreted. The distinction between the standard definition and the authors’ definition affects (probably increases) their calculated proportions, and leads to an inflated impression of the EEG “Sensitivity.”
Rather than taking an established test characteristic and changing the definition, the authors should have used “yield” rather than “sensitivity”
in column 2, table 2.
Third, estimates should have confidence intervals, and the failure to report confidence intervals in table 2 is disappointing. In addition, the authors failed to consider or acknowledge an additional source of imprecision in any estimate of yield for EEGs, problems of inter-observer
inconsistency between EEG interpreters.[3][4][5] Thus, a good feature of this study is that there is a single reader, but the absolute yield from this reader may not apply to other EEG readers, and the study design does
not allow for assessment of the important trade-off between sensitivity and specificity in EEG.[3]
Finally, when differences are reported, an estimate is needed of the probability that a difference this large could have been observed if in fact the null hypothesis (no difference) were true. The authors’ choice of
a Chi Square Test for this purpose is questionable because the subsamples for each Chi Square test are selected on the basis of the results of the EEGs (the independent variable), not a priori. Therefore, the number of
individuals in the dependent variable columns is dependent on the numbers of subjects in the other independent variable rows. This appears to violate the requirement for Chi Square Tests that measured variables are independent. Moreover, since the population is selected on the basis of a single reader’s interpretations, the p value estimates may only apply to future studies of patients whose EEGs were read as positive by that reader(JPL).
Despite these problems, this study provides some evidence that, in young adults with clinically diagnosed, recent-onset epilepsy, SD-EEGs are somewhat more likely to show generalized or partial discharges, serving as a guide for medical decision making.
References
1. Gilbert DL, DeRoos S, Bare MA. Does sleep or sleep deprivation increase epileptiform discharges in pediatric electroencephalograms? Pediatrics. 2004;114:658-62.
2. Gilbert DL, Gartside P. Factors affecting the yield of pediatric EEGs in clinical practice. Clin Pediatr (Phila). 2002;41:25-32.
3. Gilbert DL, Sethuraman G, Kotagal U, et al. Meta-analysis of EEG test performance shows wide variation among studies. Neurology. 2003;60:564-70.
4. van Donselaar CA, Schimsheimer RJ, Geerts AT, et al. Value of the electroencephalogram in adult patients with untreated idiopathic first seizures. Archives of Neurology. 1992;49:231-37.
5. Williams GW, Luders HO, Brickner A, et al. Interobserver variability in EEG interpretation. Neurology. 1985;35:1714-19.
Transient global amnesia (TGA) is a clinical syndrome
characterized by a sudden onset of anterograde and
retrograde amnesia in the absence of other neurological
signs and symptoms, which is resolved within 24 hours.
Although the etiology of TGA remains unknown, recently
Lewis 1) suggested that a Valsalva-like action appears to
be a common triggering event among patients with TGA.
Although TGA follow...
Transient global amnesia (TGA) is a clinical syndrome
characterized by a sudden onset of anterograde and
retrograde amnesia in the absence of other neurological
signs and symptoms, which is resolved within 24 hours.
Although the etiology of TGA remains unknown, recently
Lewis 1) suggested that a Valsalva-like action appears to
be a common triggering event among patients with TGA.
Although TGA following medical procedures has not been
established, Hiraga and Matsunaga 2) reported encountering
three patients who developed TGA after upper
gastrointestinal endoscopy (UGI). However, they did not
perform single photon emission CT (SPECT). We dealt with a
case of TGA occurring immediately after UGI and performed
SPECT test during the TGA attack.
Case report. A middle aged right-handed patient underwent UGI by a veteran endoscopist. The UGI was administered under lidocaine spray anesthesia without further premedication. There was no history of migraine, epilepsy, or cerebrovascular disease. When the endoscope was being removed, the patient held their breath and strained as in the Valsalva maneuver, but no cardiac arrhythmia was noted during electrocardiographic monitoring. Just after the examination, the patient suddenly developed complete anterograde amnesia and retorograde amnesia of the recent past five or six years. Neurological examination results were normal except for severe memory impairment. Magnetic resonance imaging (MRI) of the brain, including diffusion-weighed imaging (DWI) of the brain performed during the attack, three hours after onset, showed no abnormalities. Cervical artery color duplex sonography and electroencephalography (EEG) findings were normal during the attack. The first Tc-
99m ECD SPECT study was performed during the attack, four
hours after onset, and showed hyperperfusion in the right
thalamus.
Figure 1a-b(A) First SPECT study during the transient global amnesia (TGA) attack discloses a focus of increased activity uptake in the right thalamus (arrow).
(B) Second SPECT study one month after TGA discloses decreased activity uptake in the same region as that of the previously demonstrated increased uptake.
Both hematological and hemocoagulation screening test results were normal. Twelve hours after onset, the patient had recovered completely but a memory gap for a part of the previous day remained. In the second SPECT study, conducted 1 month later, no asymmetry was detected either visually or through quantification.
Findings for the second brain MRI, also conducted 1 month
later, were normal, too.
Discussion.
Our patient met all the proposed criteria for TGA. The
amnesia of our patient was not produced by anesthesia,
because the patient did not receive any sedation or premedication except for lidocaine spray. Furthermore DWI MRI of our patient did not show hyperintensity, thus making an ischemic origin unlikely. Although the occurrence of TGA after UGI is rare, two other reports of TGA following UGI 3-4) have been published, and both cases were from Japan as in the study by Hiraga and Matsunaga 2). These cases did not receive any sedation, because UGI is usually administered without sedation in Japan. TGA may be overlooked in some cases in Western countries because the transient nature of the event and concomitant sedation may obscure TGA occurrence. Hiraga and Matsunaga 2) proposed that autonomic dysfunction or emotional stress might be a possible cause of TGA. In addition, Valsalva-like action without sedation may also have been a triggering factor in our case as suggested by Lewis.
While none of the previously reported cases were
examined by SPECT after UGI, our SPECT findings support the notion that TGA can occur after UGI. SPECT performed during TGA of our patient showed increased activity uptake in the right thalamus. Spontaneous temporal lobe seizures are unlikely to have occurred because EEG during the episode was normal and no epileptic seizures were observed.�@Although SPECT findings have shown hypoperfusion in memory relevant brain structures in almost all patients with TGA, some studies reported transient hyperperfusion in the left medial temporal lobe during TGA attack 5).
Although TGA is a rare complication of medical procedures, physicians should be aware of its potential occurence.
References
1. Lewis SL. Aetiology of transient global amnesia. Lancet
1998;352:397-99.
2. Hiraga A, Matsunaga T. Trasient global amnesia after
gastroscopy. J Neurol Neurosurg Psychiatry 2006;77:995-996.
3. Joshita Y, Mineo E, Kihira K, Sato K, Kasano T, Yoshida
Y, Ido K, Kimura K, Yoshida M. Rare hazard of gastrointestinal endoscopy. A case of transient global
amnesia. Gastroenterol. Endosc 1990;32:589-92. (in
Japanese)
4. Sawada Y, Kamihira M, Hirakawa R, Yoshida Y, Imawari M.
A case of transient global amnesia precipitated by
gastrointestinal endoscopy. Progress of Digestive endoscopy 2002;60:44-46. (in Japanese)
5. Matsuda H, Higashi S, Tsuji S, Sumiya H, Miyauchi T,
Hisada K, Yamashita J. High resolution Tc-99m HMPAO SPECT
in a patient with transient global amnesia. Clin Nucl Med
1993;18: 46-49.
I have read your article(1) with interest ,which is also a very good study .However, in this connection I would like to mention that ,in your study the mean time of recording the EEG is almost double in sleep deprivation EEG than that of routine EEG. Whether this long duration of recording in sleep deprived cases has given much more abnormal EEG than that of others and whether sleep deprived EEG should b...
I have read your article(1) with interest ,which is also a very good study .However, in this connection I would like to mention that ,in your study the mean time of recording the EEG is almost double in sleep deprivation EEG than that of routine EEG. Whether this long duration of recording in sleep deprived cases has given much more abnormal EEG than that of others and whether sleep deprived EEG should be taken for longer duration in all cases, to get a higher yield is the question. Also whether sleep deprived EEG should be a routine procedure in young adults with newly diagnosed epilepsy?
Although many studies have shown that the yield of abnormal EEG is profound in patients suspected of having epilepsy after a sleep deprived EEG, but it is to be noted that all night sleep deprivation prior to EEG recording causes much inconvenience to the patient, as well as to the family members especially in pediatric cases. Degen R et al (2) in a study of EEG of 190 patients reported the activation rate of epileptic activity in 52.6% (without sleep deprivation) and 53.2%(with sleep deprivation) and
also noted no real differences between the two methods in classifying the epileptic discharges. Kubicki S et al (3) reported electroencephalographic activity indicative of seizure disorder in 53.6%in patients suspected to have epilepsy, when a short term sleep EEG recorded following a partial sleep deprivation during the previous night. Evangeline et al(4) have found melatonin induced sleep EEG was as informative as sleep deprived EEG in pediatric groups. Melatonin also do not alter the microstructure of
sleep. Milstein et al (5) reported melatonin induced sleep recording to be safe and reliable in adults.
Considering all these factors, I think it is better to carry out a study in adult patients to prove that partial sleep deprivation during previous night followed by a melatonin induced sleep recording can yield the same information as that of a total sleep deprivation recording. It is then that a protocol for EEG recording with total sleep deprivation in newly diagnosed epileptic patients is to be considered.
REFERENCES
1 JP Leach, LJ Stephen, C Salveta, M J Brodie--Which
electroencephalography(EEG)for Epilepsy? The relative usefulness of different EEG protocols in patients with possible epilepsy.J Neurol Neurosurg Psychiatry, 2006, 77, 1040-1042.
2 Degen R, DegenH E, Reker M---Sleep EEG or without sleep deprivation? Does sleep deprivation activate more epileptic activity in patients suffering from different types of epilepsy? Eur Neurol.1987, 26(1):51-9 Abstract PubMed.
3 Kubicki S, Scheuler W,Wittenbecher H---Short term sleep EEG recordings after partial sleep deprivation as a phenomena: an evaluation of 719 EEG recordings.Epilepsy Res Suppl. 1991,2:217-30.Abstract PubMed
4 Evangeline Wassmer,Paul F B Carter, Gina Welsh, Stefano Seri, William P Whitehouse--Melatonin is useful for recording sleep EEG a prospective audit of outcome,--Developmental Medicine&Child Neurology,2001,43,735-738
In response to our article, Spengos et al. suggested that we should evaluate the circadian variation of stroke onset separately for the aetiologically different subtypes of ischaemic stroke. Stroke diagnostic criteria of the registry in our study are based essentially on MONICA
manual version 1.1, which classifies cerebral infarction (CIF) into that due to cerebral thrombosis (TMB), embolic brain infarction...
In response to our article, Spengos et al. suggested that we should evaluate the circadian variation of stroke onset separately for the aetiologically different subtypes of ischaemic stroke. Stroke diagnostic criteria of the registry in our study are based essentially on MONICA
manual version 1.1, which classifies cerebral infarction (CIF) into that due to cerebral thrombosis (TMB), embolic brain infarction (EMB) and other or unknown aetiology.[1][2] Unfortunately, it was impossible to separate lacunar stroke (LACS) and atherosclerotic stroke, and both were
categorised as TMB in the registry.
The registry categorises CIF into TMB, which accounted for 47.3% of CIF cases (3,582/7,575), EMB which accounted for 15.6% (1,181/7,575) and other or unknown aetiology, which accounted for 37.1% (2,812/7,575).
The percentages of cases of CIF in which stroke onset occurred while the patient was asleep were 16.5%, 13.5% and 11.7%, respectively. The percentage of cases occurring while sleeping was lower among patients with EMB than among those with TMB (P<0.05). The percentages of cases
occurring during sleep in both TMB and EMB were greater than those of intracerebral haemorrhage (ICH) and subarachnoidal haemorrhage (SAH) (P<0.005 and <0.001, respectively). The time-specific onset for
twelve 2-h periods for separated CIF categories were calculated in the same way as in our article.[2] The cases in which TMB occurred during sleep showed a higher single peak than other categorised CIF during the period from 06:00 to 07:59.
The cases in which EMB occurred during sleep showed a single but lower peak than that of TMB.
Cases of other or unknown aetiology showed a single peak, which was higher than that of EMB but lower than that of TMB.
The cases of TMB, EMB and other in which onset occurred in the waking state showed two similar peaks; a higher peak in the morning and a lower peak in the afternoon. TMB, which accounted for 47.3% of cases of CIF, showed a marked influence on circadian rhythm of whole CIF onset. Spengos et al.
reported that onset of LACS was associated with stroke during sleep (SDS).[3] LACS may have accounted for the high percentage of TMB in our study, and influenced circadian variation of onset of the whole CIF, especially onset during sleep or status in sleep.
Spengos et al. expressed doubt about the relation between stroke onset of CIF and variation in blood pressure (BP) according to lack of information of BP on admission. Our study not only suggested that BP dropped during sleep, but also that various changes during sleep, such as the drop in BP, respiratory disorders during sleep and haemostatic
functions, are risk factors and triggers for onset of CIF.[2]
The results of our study suggested that sleep or status in sleep was a risk factor for CIF but not for ICH or SAH, because all cases of stroke onset during sleep and with unknown situation occurred equally between midnight and 06:00, and circadian rhythm lost its nadir during the night in CIF, but not in ICH or SAH.[2] Spengos et al. also expressed doubt about our assumption of an equal distribution of SDS between midnight and 06:00. The purpose of this method, which was intentionally biased for
circadian variation in all onset situations from midnight to early morning, was not to evaluate circadian variation, but to evaluate the relation between sleep or status in sleep and the risk of occurrence of stroke in each subtype.
In the registry, SDS of CIF in which onset time was registered between midnight and early morning accounted for most cases, and the number of SDS cases of CIF in the other time zone was markedly lower. Many of the cases
of SDS in which onset occurred during the daytime or was unspecified were supposed to be unconscious and to have been found to have had a stroke late as the patients lived alone or due to the absence of housemates.
However, these cases were also exposed to a risk of SDS. We feel that it is appropriate to redistribute SDS during sleeping hours for evaluation of the risk of SDS in our study.
In their recent study, Spengos et al. suggested that there is a relation between bimodal variation of stroke onset and siesta.[3] Bimodal variation of stroke onset has been reported in the UK and Japan, both countries in which siesta is uncommon.[2][4] Therefore, factors other than
siesta may be involved. We supposed that BP variation, which showed bimodal variation, is responsible for the bimodal circadian rhythm.[2][5] In our study, we showed that the evening peak from 18:00 to 19:59 is higher than the morning peak of 08:00 to 09:59 among patients with ICH and SAH in which stroke onset occurred during waking hours, whereas CIF showed a higher morning peak.[2] In addition, the reasons for these differences in peak height between ischaemic and hemorrhagic stroke were discussed
with regard to haemostatic functions. The reader�fs suggestion that we did not underline these results is mistaken.
As noted by Spengos et al., it is very important to evaluate circadian variation of CIF onset and risk factors and triggers by subtype for aetiological differences. Such studies will provide important information regarding risk factors and triggers of stroke onset.
References
1. World Health Organization MONICA Project. Event Registration Data Component, MONICA Manual Version 1.1. Document for meeting of MONICA Principal Investigators, 1986.
2. Omama S, Yoshida Y,Ogawa A, et al. Differences in circadian variation of cerebral infarction, intracerebral haemorrhage and subarachnoid haemorrhage by situation at onset. J Neurol Neurosurg Psychiatry published 17 August 2006, 10.1136/jnnp.2006.090373
3. Spengos K, Vemmos K, Tsivgoulis G, et al. Two-peak circadian distribution of stroke onset in Greek patients. A hospital based study. Cerebrovasc Dis 2003; 15: 70-77.
4. Wroe SJ, Sandercock P, Bamford J, et al. Diurnal variation in incidence of stroke: Oxfordshire community stroke project. BMJ 1992;18:155-157.
5. Stergiou GS, Vermmos KN, Pliarchopoulou KM, et al. Parallel morning and evening surge in stroke onset, blood pressure, and physical activity. Stroke 2002;33:1480-1486.
Samarasekera et al. reported four patients with non-
paraneoplastic acute limbic encephalitis (ALE), who had negative testing for voltage-gated potassium channel auto-antibodies[1]. We wish to report a patient with a previously unreported association of ALE with
hemophagocytic syndrome (HPS) and discuss the possible pathophysiology.
A 9-year-old boy, the second child of healthy non-consanguin...
Samarasekera et al. reported four patients with non-
paraneoplastic acute limbic encephalitis (ALE), who had negative testing for voltage-gated potassium channel auto-antibodies[1]. We wish to report a patient with a previously unreported association of ALE with
hemophagocytic syndrome (HPS) and discuss the possible pathophysiology.
A 9-year-old boy, the second child of healthy non-consanguineous parents, was well until he showed fever (day 0 of illness). On day 3, he had leukopenia; white blood cells (WBC) count 1,400/micro l, and a mild
consciousness disturbance. On day 4, the leukopenia had worsened; WBC count 900/micro l, and thrombocytopenia appeared; platelet count 79,000/micro l. The blood chemistry showed some abnormalities; aminotransferase 75 IU/l, ferritin 1,320 ng/ml and soluble interleukin
(IL)-2 receptor 1,930 (reference range 124-466) U/ml. Polymerase chain reaction, for cytomegalovirus, Epstein-Barr virus, herpes simplex virus, human herpes virus 6, 7 and varicella-zoster virus, using peripheral blood or cerebro-spinal fluid (CSF), were all negative. Bone marrow aspiration revealed hemophagocytosis but no malignancy. On day 6, he had a transient loss of consciousness, and he had convulsions afterwards. Then, brain computed tomography and magnetic resonance imaging (MRI) did not detect any abnormalities. Methylprednisolone pulse (30 mg/kg/day for 3 days) and gamma globulin (400 mg/kg/day for 3 days) therapies were started on that
day. To control seizures, midazolam and phenobarbital were administered. On day 11, awake EEG showed no occipital theta or alpha activity with high
voltage slow wave. On day 12, peripheral blood natural killer (NK) cell activity was 7 (reference range 18-40)%. Consciousness disturbance was slowly improving and midazolam and phenobarbital were gradually replaced
by carbamazepine. On day 17, brain MRI showed bilateral claustral lesions. Perforin expressions in cytotoxic T and NK cells were not reduced. On day 24, brain MRI showed the widened lesions, involving bilateral claustrum and hippocampi. On day 26, the hematology and blood
chemistry showed normal results, including ferritin. Cytokines, including IL-2, 4, 6, 10, interferon-gamma and tumor necrosis factor-alpha, were examined with serum and CSF, all resulted within normal limits. On day
30, soluble IL-2 receptor and NK cell activity were 674 U/ml and 44 %, respectively. On day 33, forward and backward digit span length were 4 and 2, respectively. On day 34, brain MRI showed that bilateral claustral
lesions were reduced. On day 44, Wechsler Intelligence Scale for Children Third Edition showed borderline mentality, verbal IQ=81, performance IQ=75 and full-Scale IQ=76. Follow up brain MRI on day 124, showed no lesions.
The examinations with auto-antibodies against the glutamate receptors (GluR) delta 2 and epsilon 2 were prompted, using CSF on day 26, serum on day 26 and 124. Their results were non-specific, anti-GluR epsilon 2 IgG
was weakly positive in CSF on day26 and anti-GluR epsilon 2 IgM was positive in serum on day 124.
Our case is compatible with HPS, except ALE[2]. Although HPS often accompanies CNS involvement, ALE associated with HPS has not been reported. We hypothesize two possible pathological mechanisms, that seem to be polar opposites. One plausible explanation is immunological augmentation, which could result in an auto-antibody production. And the other is immunological compromise, which could result in viral infection. The increased soluble IL-2 receptor could result from T-cell activation, which seems to comprise the former possibility. On the other hand,
reduced NK cell activity seems to comprise the latter. The data from our patient are inconclusive. Further work is required to establish the immunological basis for ALE.
References
[1]Samarasekera SR, Vincent A, Welch JL, Jackson M, Nichols P, Griffiths TD. The course and outcome of acute limbic encephalitis with negative voltage-gated potassium channel antibodies. J Neurol Neurosurg Psychiatry (in print).
[2]Imashuku S, Hyakuna N, Funabiki T, Ikuta K, Sako M, Iwai A, et al.Low natural killer activity and central nervous system disease as a high-risk prognostic indicator in young patients with hemophagocytic lymphohistiocytosis. Cancer 2002;94:3023~31.
I read the case reports of Gotkine et al 1 with great interest and definitely agree with their suggestion that an increase in sympathetic activity may reverse the pain in cluster headache.
We saw already in the late 1960´s that spontaneous attacks of cluster headache are not seldom preceded by a shift of the vegetative tone in a parasympathetic direction. Furthermore, the attacks are fairly often associated...
I read the case reports of Gotkine et al 1 with great interest and definitely agree with their suggestion that an increase in sympathetic activity may reverse the pain in cluster headache.
We saw already in the late 1960´s that spontaneous attacks of cluster headache are not seldom preceded by a shift of the vegetative tone in a parasympathetic direction. Furthermore, the attacks are fairly often associated with symptoms indicative of increased parasympathetic activity,
e.g. bradycardia. Two of our patients noticed that heavy physical exercise had a positive effect on spontaneous headache attacks. We therefore suggested that an increased tone of the sympathetic nervous system might have a favourable influence on the headache. We investigated 2 the effect on the pain of a rise in blood pressure induced by constant prolonged physical exercise on a cycle ergometer or intravenous infusion of noradrenaline in 11 male cluster headache sufferers. Both procedures started 15 min after administration of nitroglycerin and lasted for 60 min. It was found that both maximal intensity and duration of provoked headache attacks were significantly reduced compared with control attacks observed following nitroglycerin administration. In one patient noradrenaline was successfully induced during a spontaneous attack. Our
results are in some way similar to the most interesting observations by Gotkine et al. The activity of the sympathetic nervous system relative to attacks of cluster headache definitely deserves further studies.
References:
1.Gotkine M, Steiner I, Biran I. Now dear, I have a headache! Immediate improvement of cluster headache after sexual activity. J Neurol Neurosurg Psychiat 2006; 77: 1296.
2.Ekbom K, Lindahl J. Effect of induced rise of blood pressure on pain in cluster headache. Acta Neurol Scand 1970; 46: 585-600.
Karl E.Ekbom
Karolinska University Hospital
In their paper on unilateral motor ‘deficits’, Young and colleagues [1] report a less-studied aspect of migraine pathophysiology. In contrast to the experience of this tertiary headache-care centre, my own experience
of managing migraine patients between 1976-2006 suggests that a vague upper limb motor involvement is an uncommon feature, not associated with objective weakness or functional limitation or...
In their paper on unilateral motor ‘deficits’, Young and colleagues [1] report a less-studied aspect of migraine pathophysiology. In contrast to the experience of this tertiary headache-care centre, my own experience
of managing migraine patients between 1976-2006 suggests that a vague upper limb motor involvement is an uncommon feature, not associated with objective weakness or functional limitation or disability of the affected limb(s) including writing or typing or walking, not accompanied by any feature of an upper-motor neuron (UMN) deficit, not neurologically lateralizing in relation to the headache or the aura and generally ipsilateral to the headache, not requiring any additional therapy specifically directed at the motor symptom, generally unlinked to the presence or absence of visual aura symptoms, and not present regularly or invariably in repetitive attacks. I have never encountered a patient of
migraine with scintillating scotoma and unilateral UMN weakness; I am not aware that migrainous scintillating scotoma has ever been reported with hemiplegic migraine, whether spontaneous or familial. Even with the first-
attack of typical migraine with upper limb symptoms not consistent with an UMN deficit, there is little justification to seek neurological imaging studies either in the field or in research. I have rarely encountered non-
paretic subjective hemiplegic weakness in association with migraine attacks; also, such patients do not manifest neurological deficit(s). While Young et al. underscore that all their patients had objective evidence of arm and leg weakness [1], weakness in the absence of alteration of deep tendon reflexes or clasp-knife rigidity or extensor
plantar reflex should always arouse suspicion of behavioural alteration. To equate giveway weakness without tonic rigidity to ‘clasp-knife weakness’ with UMN rigidity is fundamentally incorrect and speculative.
Since pathophysiology of migraine is itself poorly comprehended, it is particularly important to distinguish negative phenomena in terms of neurological/paretic or non-paretic ‘deficits’ or ‘accompaniments’. Also, facial weakness in 17% (subjective) or ~8% (objective) of migraine with unilateral motor symptoms (MUMS) patients [1] can be misleading if suggested by direct inquiry in questionnaires or clinical interview in the absence of definitive clinical signs of unilateral facial paresis. In the 2 patients with signs of ‘weakness in the face’, Young et al. [1] do not describe any of the classical clinical signs of facial paresis, unilateral or bilateral. Furthermore, monocular and hemianopic visual symptoms, as
described by Young et al. in 50% and 18% of patients, respectively, are unlikely to reflect brain visual cortical involvement.[2] The general impression among neurologists devoted to migraine research that the
migrainous scintillating scotoma reflects visual cortical involvement is based on belief rather than clinical evidence. There has not been one clearly documented case of bilateral migrainous scintillating scotoma in a
homonymous hemianopic distribution. Migrainous scintillating scotoma is usually monocular or uniocular – hemianopic -- in distribution [2][3].
Over a 6-week period in 2003, I myself experienced and recorded migraine headaches with hemianopic scintillating scotoma; all attacks of scintillating scotoma were strictly monocular, i.e., confined to the left eye. Determination of uniocular or binocular distribution of migrainous scintillating scotoma or other visual aura is an important but neglected difficult-to-evaluate aspect of evaluation of migraine patients.[2][3]. If the migrainous scintillating scotoma is indeed monocular or uniocular in
most cases, a central brain origin of the phenomenon related to CSD can be virtually excluded.[2] Positive migraine aura has never been shown to consistently lateralize neurologically with the side of headache,
unilateral, bilateral or side-shifting. More importantly, Young et al. [1] seek to attribute giveway motor weakness in migraine patients to cutaneous allodynia or to cortical spreading depression (CSD); both phenomena are linked to migraine pathophysiology by serendipity, a series of loosely-linked pathophysiological assumptions, and fragmentary evidences [2][4][5]. Additionally, migraine disability scores (MIDAS) – being ‘housebound’ or ‘losing’ jobs [1] -- cannot strictly substitute as a measure for functional disability due to motor weakness. Finally, while labeling MUMS patients as ‘super-migraineurs’ and underscoring ‘more extreme forms of disability’, Young et al. [1] ignore that their own cohort of MUMS patients with more cutaneous allodynia and more cluster headache-like autonomic activation had markedly shorter (>50%) duration of episodic headaches (Table 3).
Cutaneous allodynia is not a specific or pathognomonic feature of migraine [5]. Cutaneous allodynia is a common non-specific feature of pain neurophysiology in health as well as in disease [6][7][8][9][10]. Second, pain levels are not significantly different between migraine attacks with allodynia or without allodynia [11]. Third, on different occasions, the same migraine patient may manifest allodynic or non-allodynic attacks [5] just like MUMS patients might manifest headache both with or without
weakness. [1] Fourth, the suggested role of the parasympathetic nervous system in occurrence of cutaneous allodynia in some migraine patients, and, its presumed link with pathogenetic central and peripheral neuronal
sensitization [12,13] is conceptually striking but counter-intuitive [4]. While donepezil – a parasympathomimetic agent -- offers significant prophylaxis to migraine patients comparable to propranolol (40 mg b.i.d),
evidence for a potent antinociceptive effect of nicotine [14] and the rationale for use of parasympathomimetic nicotine agonists as analgesics is rapidly evolving [15][16][17]. Nicotine readily crosses the blood-brain
barrier (BBB) and releases acetylcholine as well as arginine vasopressin, β-endorphin, nor-epinephrine, dopamine, serotonin and adrenocorticotropic hormone [17]. With marked shortening of headache duration in migraine patients with allodynia in this cohort [1], an adaptive role for allodynia appears biologically plausible. [17]
Young et al. also invoke cortical neuronal excitation and depression as a possible mechanism for march of motor symptoms in some migraine patients. [1] CSD is a classic example of the seduction of common-sense by an attractive hypothesis [2][17]18]. A large number of factors raise
caution regarding the role of CSD as a pathogenetic mechanism in migraine or in the ischaemic human brain: (i) The distinction between physiology of the normal and the injured human brain must be maintained. As reviewed by
Fabricius et al. [19], CSD in the intact normo-perfused brain does not lead to cell-death [20]. Basically, the brain of migraine patients does not mirror the pattern(s) of the injured human brain. A belief persist that the BBB might be disrupted during migraine attacks but disruption of BBB due to vasogenic œdema is a rare and transient occurrence. In addition, it is commonly known that vasogenic edema affects mainly the white matter; also, lack of contrast enhancement in some cases argues
against BBB breakdown and suggests cytotoxic œdema.[21] (ii) Influence of CSD on brain functioning is basically not deleterious. CSD by itself does not affect ATP levels, mitochondrial aconitase activity, or induce neuronal injury.[22] Conversely, a large and growing body of evidence indicates that CSD is biologically adaptive or neuroprotective. CSD influences the expression of many (to date, over 40) genes associated with inflammation and induces a long-lasting ischemic tolerance that results in
smaller subsequent infarcts and stimulates persistent neurogenesis [23][24][25][26][27][28]. (iii) The association of recurrent peri-infarct depolarisations (PIDs) in the injured brain of experimental animals with
larger final infarct volume [19] is circumstantial, without elucidation of the cause-effect conundrum. We have no idea how CSDs or PIDs might induce further tissue damage in brain-injured humans or experimental animals.
Transient reductions in tissue perfusion or pO2 or decline in dialysate glucose [19] are as likely, if not more likely, to reflect the effect of repetitive cycles of PID/CSD and neuronal recovery. Recovery from spreading depression is an oxidative, energy-dependent process [29].
Hyperaemia, rather than oligaemia, is a more prominent feature of CSD. Massive depolarizations of neurons and glia characteristic of CSD or slow potential change (SPC) with dramatic changes in intra- and extra-cellular
ion concentrations are likely to induce a repetitive neuronal ‘functional silence’ that overall lowers the metabolic requirement of the injured tissue. (iv) In unanaesthetized rats, CSD does not induce aversion as an
immediate or delayed reaction [30]. Conversely, 4-8 minutes after electrophoretic injection of potassium ions into occipital cortices, aggressive and stereotyped eating, drinking, and exploratory behavior were elicited by unilateral and bilateral spreading depression [31]. (v) After cortical recovery from CSD, a prolonged hypesthesia persists [32]. Hypesthesia is a typical feature of CSD. (vi) CSD blocks significantly the hyperthermic influence of prostaglandin E1 and E.coli endotoxin [33]. (vi)
In cats, furosemide inhibits CSD [34] while valproate does not [35]; while furosemide does not prevent migraine, valproate can offer prophylaxis. [36] Also, dihydroergotamine, acetylsalicylic acid, and metoprolol do not affect CSD in the cat. [35] (vii) CSD-augmented matrix metalloproteases (MMP) upregulation may have a pathogenetic role in vascular permeability changes in migraine, besides stroke and trauma [37][38][39]. While nitric oxide (NO) has been implicated in MMP activation and NO increases within brain tissue during CSD, NO is essential for recovery of ionic homeostasis[40][41]. Remarkably, neuronal NO aggravates cortical hyperemia observed during CSD in rabbits [42]. Paradoxically, the NO-donor nitroglycerin generally rapidly reverses migraine aura while serving as the best experimental human model for migraine headache; [43] nitroglycerin, however, enhances CSD-induced NO release.[40] (viii) Nifedipine and
isoproterenol do not freely cross the intact BBB but can instantaneously abort migraine aura.[43]. (ix) While chronic daily administration of migraine prophylactic drugs -- topiramate, valproate, propranolol, amitriptyline, and methysergide -- dose-dependently suppressed CSD frequency by 40 to 80% and increased the cathodal stimulation threshold in rats [44], intriguingly, noradrenergic agonists (norepinephrine and
clonidine) also reversibly block migration of CSD in anaesthetized adult rats.[45] Suppression of CSD by both noradrenergic agonists and antagonists in rat confounds any rational or definitive hypothesis – including the biobehavioral model of migraine that envisions a central key pathogenetic role intrinsic noradrenergic activation [46] -- or conclusion about the pathogenetic role of CSD in migraine patients. (x) While both neurogenic inflammation and stimulated c-fos (early immediate response gene) expression within postsynaptic brain stem neurons of the trigeminal nucleus caudalis following trigeminovascular stimulation are blocked by
sumatriptan and ergot alkaloids [47][48], c-fos expression in the trigeminal caudal nucleus following KCL application appear to be a non-specific response to hyperosmolar KCl/NaCl milieu rather than to CSD. [49]
(xi) Single neuron activity in the trigemino-cervical caudal nucleus remains unchanged during and after CSD in cats [50] and rats [51] following single or repetitive waves of CSD. CSD also did not alter ipsilateral dural plasma extravasation or alter the release of calcitonin
gene-related peptide and prostaglandin E2 from the dura in an in vitro model.[51] Experimental studies in animals with experiments performed in anaesthetetized animals or slice preparations are limited by species differences with humans; additionally, CSD has been produced by artificial
physical or chemical stimuli, thereby limiting validity of extrapolation of the conclusions to human migraine. Up to now no appropriate animal model exits for human migraine; consequently, results of animal experiments might be applied to human migraine models only with a great
deal of circumspection. (xii) Red-green checkerboard visually triggered headache in migraine patients -- in contrast to the initial pronounced cerebral hyperaemia seen in experimental CSD -- is accompanied by a
spreading suppression of initial neuronal activation and increased occipital cortex oxygenation.[52] Visual cortical stimulation by retinal stimuli is not specific to migraine patients. As demonstrated by Leão’s experiments more than 5 decades ago, retinal stimulation with brief
flashes of light can also elicit spreading cortical silence and depression.[53][54] (xiii) Bowyer et al. have shown spreading depression-like propagating direct current magnetoencephalographic (DC MEG) signal during spontaneous aura with fortification spectra but bilateral DC MEG
signals following checkerboard stimulation.[55] CSD-like or equivalent phenomenon in migraine patients must be viewed in a broad clinical context rather than as conclusive evidences for a pathogenetic role for CSD. (xiv)
As atenolol or nadolol or verapamil – drugs that do not readily cross the intact BBB or critically influence brain neuronal function -- prevent migraine, a pathogenetic role for CSD or for primary brain neuronal dysfunction in migraine is considerably attenuated if not eliminated.
[5][17][18][46] (xv) Neither the headache nor the aura represents the true beginning of a migraine attack; onset of migraine attacks lies in the ‘pre-prodromal’ phase while the prodromal phase itself might last several
hours or a few days. [2][5][17][56][57]. Neuro-physiologically, CSD cannot support the characteristic clinical feature of delayed or post-stress onset of migraine attacks in a wide variety of situations and
circumstances. (xvi) Majority of migraine patients do not develop aura; CSD does not appear relevant to this large subset.[45] (xvii) Contrary to the general perception, neuro-anatomically migraine is not a pan-trigeminal disorder; a predominant or selective involvement of the
ophthalmic division of the trigeminal nerve (V1) is suggested by neuroanatomical distribution of only V1 fibres to the upper cervical segments, occurrence of photophobia, and absence of typical migraine headache or aura in the cohort of patients having undergone enucleation or evisceration of the eye.[2][58] Dental extraction (upper or lower jaw) with intense stimulation of maxillary or mandibular divisions of the trigeminal nerve is only rarely associated with migraine headache.[59] CSD cannot rationalize selective involvement of V1 nerve fibres.[2] (xviii) If CSD in migraine patients is indeed the outcome of enhanced brain neuronal excitability, the ability of amitriptyline – a potentially pro-convulsive agent – to prevent migraine creates a conceptual impasse.[2][17] (xix) Migraine has characteristic periods of onset (exacerbations) and offset
(remissions). The basis for propensity for the migraine patient to develop CSD or become resistant to occurrence of CSD is completely unknown. The theory that hypomagnesemia or brain magnesium depletion might aggravate cortical neuronal excitability and precipitate CSD [60] is neither specific to migraine patients nor consistent with known patterns of both distribution of magnesium in cerebrospinal fluid and walls of cranial
blood vessels as well as the pharmacokinetics of exogenously administered magnesium.[17] Oral or intravenously administered magnesium does not readily cross the intact BBB.[17]
Leão’s chance discovery in 1944 of “marked, enduring reduction of the “spontaneous” electrical activity of the cortex” has struck a chord of consensus among most neuro-physiologists and neurologists. More than half a century later, we are no closer to unraveling the biological purpose of the electrophysiological phenomenon. Occurrence of CSD in the compromised human brain has been established; whether the nature of the phenomenon is
pathogenetic or adaptive continues to seem elusive. To neurologists in pursuit of migraine mechanisms, it is nothing short of gospel truth that migrainous scintillating scotomata originate at the level of the visual brain cortex. In migraine pathophysiology, the entire concept of CSD, however, has been hitherto maintained by default.[18] Scientific equipoise, nevertheless, mandates careful consideration of facts on the other side of a conceptual divide. That a fundamental physiological process such as CSD will exert diametrically opposite biological influences in the normo-oxygenated human brain (protective/boon) or in the injured compromised human brain (pathogenetic/bane) is a highly unlikely proposition bordering on irrational sceticism. Recently, a plausible mechanistic hypothesis based on retinal spreading depression has been advanced to explain the phenomenon of monocular or uniocular hemianopic
migrainous scintillating scotoma.[2][18]
The pseudo- or neurologically non-lateralizing ‘weakness’ of MUMS without functional motor deficit or accompaniments of UMN impairment very likely represents an aberrant behavioural response to recurrent cycles of pain-related arousal. As a new term in the vast, inexhaustible lexicon of migraine, giveway weakness arouses both curiosity and hope. Nevertheless, to link MUMS to cutaneous allodynia or to CSD [1] is to stray further into the formidable maze of migraine.
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Hand held dynamometry measures the strength of the long finger flexor muscles and is therefore eminently suitable to the study of myotonic dystrophy and other distal myopathies. The dynamometer used in this study is available for approximately £135 and weighs less than one kilogam. In our experience, patients found it easy to use. We acknowledge that this technique may not be suitable for some severely affe...
Hand held dynamometry measures the strength of the long finger flexor muscles and is therefore eminently suitable to the study of myotonic dystrophy and other distal myopathies. The dynamometer used in this study is available for approximately £135 and weighs less than one kilogam. In our experience, patients found it easy to use. We acknowledge that this technique may not be suitable for some severely affected patients, and in this group measurement of pinch grip would be useful. We agree that a
Dr RG Whittaker, Dr E Ferenczi, Dr D Hilton-Jones.
I read with interest the article by Whittaker et al. I
completely agree that the MRC scale is unsuitable for
detecting the small changes in strength seen in a slowly
progressive disease such as myotonic dystrophy (MD).
However, I also wonder what strength a hand-held dynamometers measure. MD is a disease that affects limb
muscles in the distal especially in hand muscles. Hand-held dynamometers may...
I read with interest the article by Whittaker et al. I
completely agree that the MRC scale is unsuitable for
detecting the small changes in strength seen in a slowly
progressive disease such as myotonic dystrophy (MD).
However, I also wonder what strength a hand-held dynamometers measure. MD is a disease that affects limb
muscles in the distal especially in hand muscles. Hand-held dynamometers may be heavy to hold for MD patients and
expensive. In our clinics, we use pinch meters to evaluate
the small changes in strength in MD disease course. I
consider that an optimal study to correlate results of
strength measured by hand-held dynamometers and pinch
meters is useful for developing standardizing method to
evaluate strength changes in MD patients.
Dear Editor,
I have read all the three articles (1), (2), (3), published in Vol .77, 2006, and found them to be very good studies. It also gives a new nsight to Parkinson’s disease and seems to be very useful in our day to day practice. In this connection I would like to add a few things.
Camptocormia was first described by Brodie, in 1918. Reichel G et al, (4) proposed for new classification for cam...
Dear Editor,
Leach et al (“Which electroencephalography (EEG) for epilepsy? The relative usefulness of different EEG protocols in patients with possible epilepsy.” Journal of Neurology, Neurosurgery & Psychiatry. 2006;77:1040-2) compared the yield of 3 types of EEGs in 85 patients with epilepsy, diagnosed after two or more generalized tonic clonic seizures (GTCs). The median age of the patients was 17.9 years...
Dear Editor,
Transient global amnesia (TGA) is a clinical syndrome characterized by a sudden onset of anterograde and retrograde amnesia in the absence of other neurological signs and symptoms, which is resolved within 24 hours. Although the etiology of TGA remains unknown, recently Lewis 1) suggested that a Valsalva-like action appears to be a common triggering event among patients with TGA. Although TGA follow...
Dear Editor,
I have read your article(1) with interest ,which is also a very good study .However, in this connection I would like to mention that ,in your study the mean time of recording the EEG is almost double in sleep deprivation EEG than that of routine EEG. Whether this long duration of recording in sleep deprived cases has given much more abnormal EEG than that of others and whether sleep deprived EEG should b...
Dear editor
In response to our article, Spengos et al. suggested that we should evaluate the circadian variation of stroke onset separately for the aetiologically different subtypes of ischaemic stroke. Stroke diagnostic criteria of the registry in our study are based essentially on MONICA manual version 1.1, which classifies cerebral infarction (CIF) into that due to cerebral thrombosis (TMB), embolic brain infarction...
Dear Editor,
Samarasekera et al. reported four patients with non- paraneoplastic acute limbic encephalitis (ALE), who had negative testing for voltage-gated potassium channel auto-antibodies[1]. We wish to report a patient with a previously unreported association of ALE with hemophagocytic syndrome (HPS) and discuss the possible pathophysiology.
A 9-year-old boy, the second child of healthy non-consanguin...
Dear Editor,
I read the case reports of Gotkine et al 1 with great interest and definitely agree with their suggestion that an increase in sympathetic activity may reverse the pain in cluster headache. We saw already in the late 1960´s that spontaneous attacks of cluster headache are not seldom preceded by a shift of the vegetative tone in a parasympathetic direction. Furthermore, the attacks are fairly often associated...
Dear Editor,
In their paper on unilateral motor ‘deficits’, Young and colleagues [1] report a less-studied aspect of migraine pathophysiology. In contrast to the experience of this tertiary headache-care centre, my own experience of managing migraine patients between 1976-2006 suggests that a vague upper limb motor involvement is an uncommon feature, not associated with objective weakness or functional limitation or...
Dear Editor,
Hand held dynamometry measures the strength of the long finger flexor muscles and is therefore eminently suitable to the study of myotonic dystrophy and other distal myopathies. The dynamometer used in this study is available for approximately £135 and weighs less than one kilogam. In our experience, patients found it easy to use. We acknowledge that this technique may not be suitable for some severely affe...
Dear Editor
I read with interest the article by Whittaker et al. I completely agree that the MRC scale is unsuitable for detecting the small changes in strength seen in a slowly progressive disease such as myotonic dystrophy (MD). However, I also wonder what strength a hand-held dynamometers measure. MD is a disease that affects limb muscles in the distal especially in hand muscles. Hand-held dynamometers may...
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