Multiple sclerosis in a family on the Faroe Islands.

BACKGROUND: John Kurtzke has proposed that multiple sclerosis (MS) on the Faroe Islands occurred as a result of the spread of a transmittable agent brought to the country during World War II. AIM: Kurtzke's theory has been opposed earlier and in this study, we present a family from the Faroe Islands containing a total of 14 family members with MS which show further inconsistencies with the theory. The present study is to our knowledge, the first description of familial incidences of MS on the Faroe Islands. METHODS: Medical histories were gathered from 12 family members and 6 of the 8 living MS cases were human leukocyte antigen (HLA)-typed. RESULTS: Seven family members had primary progressive MS (PPMS), while five had relapsing remitting MS. The HLA-DR15 allele was carried by the three cases with the most aggressive form of MS and they shared a common haplotypes. The HLA types carried by the remaining cases varied. CONCLUSION: This research questions Kurtzke's theory as three of the cases do not conform to the epidemic cohorts described. Furthermore, there appears to be a higher than usual prevalence of PPMS. The high degree of heterogeneity of the HLA types carried indicates that HLA alleles do not independently explain the risk of developing MS.

Tilt table testing in patients with suspected epilepsy.

BACKGROUND: Approximately 20-30% of patients with epilepsy are misdiagnosed and syncope often seems to be the mistaken cause. We re-evaluated patients referred to an epilepsy clinic where suspicion of neurally mediated (reflex) syncope were raised using tilt table testing (HUT). METHODS: HUT laboratory results and medical records of 120 consecutive patients were reviewed retrospectively over a period of 27 months. RESULTS: HUT was positive in 59 (49%) patients. Seventeen of 38 (45%) patients previously diagnosed with epilepsy and taking antiepileptic drugs were found to be misdiagnosed. Four of 21 patients with epilepsy (19%) had dual diagnoses of reflex syncope and epilepsy. CONCLUSION: HUT is an informative investigation when suspicions of reflex syncope are raised in patients referred to an epilepsy clinic. Reflex syncope is an important and common differential diagnosis of epilepsy.

Double-blind crossover trial of gabapentin in SPG4-linked hereditary spastic paraplegia.

Patients with hereditary spastic paraplegia (HSP) are often treated with antispastic drugs to relieve symptoms but documentation is lacking. In this study, gabapentin was tested in a double-blind crossover trial on a group of patients with HSP and linkage to the SPG4 locus. There was no difference between periods with gabapentin and placebo treatment in clinical assessment, self-reported parameters or paired transcranial magnetic stimulation evaluation of motor cortical excitability.

Adverse effects of established and new antiepileptic drugs: an attempted comparison.

Seizures are but one aspect of the negative impact epilepsy has on patients' lives. Adverse effects of antiepileptic treatment may affect the patient's quality of life to an even greater extent than the occurrence of seizures. Adverse effects of antiepileptic drugs (AEDs) are common, and because the differences in efficacy are often marginal, adverse effects may be the most important factor in choosing the best AED for the patient. The search for more efficient and less toxic agents is constantly ongoing. Current evidence suggests that the new generation of AEDs is as efficient as the established AEDs and exhibits fewer adverse effects, but the scientific evidence from randomised clinical trials comparing established and new AEDs with each other is still pending.

Utility of interictal SPECT of rCBF for focal diagnosis of the epileptogenic zone(s).

With Single Photon Emission Computed Tomography (SPECT) of regional cerebral bloodflow (rCBF) ictally and interictally, regional changes of rCBF can be detected in many cases with medically intractable complex partial seizures. Interictal SPECT shows abnormal rCBF in the epileptogenic temporal lobe in 40% to 85% of the patients. A critical survey of the methodological problems considering isotopes, scanners, data analysis and patient population is presented here as well as a few semi-quantitative studies including our own. It is concluded, that SPECT of rCBF is a useful, non-invasive method of localizing the epileptogenic zone in patients with severe partial focal epilepsy. Ictal SPECT of rCBF has a higher predictive value and is more sensitive than interictal studies for localization of the seizure focus. Interictal SPECT using a high-resolution system obtains an almost as high frequency of localization of the focus. With low resolution scanners, a minor frequency is observed. Both interictal and ictal SPECT recordings should be obtained for localization of the epileptogenic focus in presurgical cases as interictal hypoperfusion and ictal hyperperfusion demonstrated in the same focal area are highly characteristic of a seizure focus.

Corpus callosotomy: seizure and psychosocial outcome. A 39-month follow-up of 20 patients.

The aim of the present study was to investigate the effect of corpus callosotomy on seizures, with emphasis on the psychosocial outcome. Data were retrospectively obtained from 20 patients (mean age 20.8 years, range 6-46). Sixteen of the operated patients took part in the 39-month (range 19-62) follow-up. Outcome measures were post-operative seizures, changes in the antiepileptic drug treatment, surgical complications including disconnection syndrome, degree of dependency according to the Barthel index, quality of life, burden on caretakers and satisfaction with the treatment. Half of the patients had a favorable seizure outcome, and of these 50% noticed an improved quality of life and were satisfied with the treatment. Four patients suffered from symptoms of cerebral disconnection syndrome which interfered with the activities of daily life. One patient died of complications 3 months after the operation. There were no significant changes in antiepileptic drug treatment, the patients' social lives or the burden of the patients on the caretakers. The conclusion is that prospective studies are needed to clarify the criteria for optimal patient selection to increase the likelihood of a positive psychosocial outcome.

Glycerol for acute ischaemic stroke.

BACKGROUND: Infarct-related oedema following ischaemic stroke is thought to be a major cause of early death. Intravenous glycerol may reduce the oedema, increase cerebral blood flow and improve cerebral metabolism. OBJECTIVES: The objective of this review was to assess the effect of glycerol in acute ischaemic stroke. SEARCH STRATEGY: We searched the Cochrane Stroke Group trials register, Medline and Embase. SELECTION CRITERIA: Randomised trials of intravenous glycerol compared with control in people with definite or presumed ischaemic stroke. Trials were included if treatment was initiated within the first four days of stroke onset. DATA COLLECTION AND ANALYSIS: Two reviewers assessed trial quality and independently extracted the data. MAIN RESULTS: Eight trials involving 649 people were included. Based on 454 patients in six trials, glycerol was associated with a decrease in deaths within 14 days of stroke onset (odds ratio of 0.58, 95% confidence interval 0.36 to 0.91). Based on 431 patients in five trials, there was a non-significant decrease in deaths within the first year of stroke onset (odds ratio of 0.82, 95% confidence interval 0.54 to 1.23). The effect of treatment on functional outcome was not clear. Haemolysis appeared to be the only adverse effect. REVIEWER'S CONCLUSIONS: There is not enough evidence to evaluate glycerol treatment for people with acute ischaemic stroke.

[Temporal lobe epilepsy--psychiatric aspects and surgical treatment]. / Temporallapsepilepsi--psykiatriske aspekter og kirurgisk behandling.

The prevalences and types of psychiatric disorders connected with temporal lobe epilepsy and surgical treatment hereof are discussed. Studies comparing psychiatric morbidity among surgically treated patients and less selected groups of patients with epilepsy, respectively, are desired. Previous follow-up studies of patients undergoing temporal lobe resection point to a high prevalence of psychiatric disorders pre- and postoperatively, in terms of personality disturbances (especially aggression) and various psychotic disorders. On the basis of older studies, postoperative psychoses are believed to appear in 7-10% of surgically treated patients. New Brain Imaging and neurophysiological techniques permit a more precise focal diagnosis. Thus more selective surgical procedures have been introduced, e.g. resection of the amygdala and hippocampus. Together with an increased awareness of the importance of psycho-social factors and the absence of severe psychiatric disturbances for a good postoperative outcome this means that results of older studies may no longer apply. Studies reflecting present day conditions are needed.

[Acupuncture analgesia. Neurochemical and neurophysiologic aspects]. / Akupunkturanalgesi. Neurokemisk og neurofysiologisk belyst.

In some parts of the world, acupuncture has been employed as a method of obtaining analgesia for thousands of years without the mechanism of its action being understood. During the past two decades, evidence has accumulated indicating that acupuncture activates an intrinsic neural network which monitors and modifies the activity of pain-transmitting neurons. The in-suppressing action is partly mediated by endogenous opioid peptides and monoamines. The system is organized at three levels of the neuroaxis: spinal cord, medulla and the midbrain. The raphe magnus nucleus and the spinal cord constitute a fundamental circuit while the PAG funnels the influences from the more rostral structures and collects information from the spinal cord. PAG initiates descending and ascending nihibition resulting in the reduction of pain. The endogenous pain-control system may be elicited by other physiological stimuli and the effect of acupuncture is scarely specific.

[Psychosocial consequences and surgical treatment of epilepsy. A review]. / Psykosociale konsekvenser af epilepsi og epilepsikirurgi. En oversigt.

Epilepsy is one the commonest neurological diseases. In 25% of the cases, particularly in patients with temporal foci, the seizures prove to be resistant to medicinal treatment. Several investigations have stressed that these patients have pronounced psychosocial problems which may be more important to the patient than the actual seizures. Taking into consideration the WHO definition of health as complete physical, mental and social well-being, patients with epilepsy have problems in all of these dimensions. The major problems are social isolation and employment. Surgical treatment of epilepsy has a pronounced effect on the seizures in 90% of the operations in the best series but it is still uncertain whether reduction in the number of seizures also implies better quality of life. The present data suggest improvement in the psychosocial conditions of the patients who functioned best preoperatively and who became seizure-free after operation. The importance of further longitudinal studies emphasizing the psychosocial aspects is stressed.

[Psychiatric morbidity in connection with surgical treatment of epilepsy. A short-term follow-up of patients with amygdalohippocampectomy]. / Psykiatrisk morbiditet i forbindelse med epilepsikirurgi. Korttidsopfølgning af amygdalohippokampektomerede patienter.

The aim of the study was to examine the occurrence of psychiatric disorders in epilepsy patients who had received surgical treatment, especially amygdalohippocampectomy (AHE), for the relief of medically intractable seizures. Forty-seven subjects, treated during the period 1987-1991 in the Danish epilepsy surgery programme (EPIKIR), entered a retrospective interview study. Of these, 37 had undergone AHE. Preoperative psychiatric morbidity was assessed through interview and available case notes, including a routine psychiatric interview. Postoperative psychiatric morbidity was assessed by the use of the Present State Examination. A total of six subjects (five AHE subjects) developed depressive disorders of various duration and severity after operation. In three subjects this occurred "de novo". No paranoid-hallucinatory psychoses developed within the follow-up period (a minimum of one year), and the presence of psychiatric disorders could not be associated with either lateralization of cerebral dominance of histopathological findings. Thus, depression appears to be the most frequent psychiatric problem following epilepsy surgery. Although the present study mainly deals with AHE, this finding is in accordance with the results of recent findings concerning anterior temporal lobe resection.

[Surgical treatment of epilepsy resistant to drug therapy. A review]. / Kirurgisk behandling af medikamentelt intraktabel epilepsi. En oversigt.

The prevalence of epilepsy is 7-9 cases per 1,000 population, corresponding to 440,000 individuals in Denmark. Approximately 1/4 of these individuals have seizures refractory to anticonvulsant medications and most of them have an epileptic focus in the temporal lobe. Epilepsy refractory to anticonvulsant medication is an incapacitating disease with high costs for the person and the society. The main problems are polypharmacy with side effects, suspicion of neurodegenerative consequences and a higher mortality. The modern era of epilepsy surgery began more than 100 years ago and since then, the developments in neurophysiology and neuroimaging have made it possible to demonstrate the epileptic focus with relatively high precision. As a consequence of this, the volume of the resected tissue has diminished and the operative complications become less frequent. The somatic and neuropsychological effects of a cortical resection are discrete and compensated by a general improvement in performance. Surgical treatment of epilepsy should no longer be considered as a last resort, but as a realistic treatment in cases of medication failure.

[3-year experiences with surgical treatment of epilepsy at the Hvidovre hospital]. / Tre års erfaringer med epilepsikirurgi på Hvidovre Hospital.

The results of a retrospective survey of 48 patients submitted to neurosurgery for medically intractable epilepsy are presented. Twenty-eight patients were treated with selective amygdalohippocampectomy, one with temporal lobe resection, 12 with anterior callosotomy and seven with a total callosotomy. Of the amygdalohippocampectomized patients and the one with temporal lobe resection (n = 29), 52% were seizure free, 17% experienced rare seizures, 7% had a worthwhile improvement while 24% observed no worthwhile improvement (follow-up time 6 to 36 months). Of the callosotomized patients, 11% were free from generalized seizures, 69% had a significant seizure reduction and 18% experienced no worthwhile improvement. The observed neurological complications were: one patient had hemianopia, one had superior quadrant anopia, four developed unilateral anosmia and one complete anosmia. The callosotomized patients, with two exceptions, were all mentally and physically handicapped. In the callosotomy group, two patients died, one from a intracerebral hematoma three months after the operation and another patient seven months postoperatively from unknown causes.

[Preoperative EEG in medically intractable epilepsy]. / Praeoperativt eeg ved medicinsk intraktabel epilepsi.

The aim of the preoperative neurophysiological investigations is to identify the primary epileptogenic focus and its relation to functional cortical areas. The investigations include interictal and ictal extracranial (scalp) electroencephalography (EEG) and invasive methods (depth, subdural, foramen ovale electrodes and peroperative electrocorticography). If an epileptic focus is located in the anterior part of the temporal lobe by the use of scalp EEG, this is normally regarded as sufficient for an anterior lobectomy. However, because of poor spatial resolution by scalp EEG, it is difficult to separate mesial from lateral foci, identify the exact extent of posterior temporal or extra-temporal foci, identify the primary focus in patients with bilateral abnormalities and identify cases with minor scalp EEG-changes. As invasive EEG shows higher spatial resolution and gives an opportunity to evaluate functional areas, invasive EEG has significant advantages in these patients. Use of invasive EEG bears a slightly increased risk and discomfort to the patient, but is necessary in the presurgical evaluation of some patients suffering from medically intractable epilepsy.

Low level laser treatment of chondromalacia patellae.

A randomized, double blind study of low level laser treatment of chondromalacia of the patella in 40 patients has been undertaken. Chondromalacia was established by arthroscopy and was related to subjective symptoms. Eight treatments were given during five weeks by a physiotherapist with the use of a GAAS pulsed laser, real or sham. Assessment of the location, quality and intensity of pain, and the influence on mood, gait, sleep, work, and sports was made before and after treatment including an eight to twelve week evaluation. No statistical difference was found between real and sham use of low level laser for the symptoms of chondromalacia of the patella.

Acupuncture in the treatment of Graves' ophthalmopathy. A blinded randomized study.

Seventeen patients with Graves' ophthalmopathy, all euthyroid for at least one year, were included in a blinded trial to test the effect of acupuncture twice a week during two months on the eye disease, assessed by an ophthalmologist and computed tomography of the eye muscle volume. No significant change was found in eye muscle volume, Hertel measure, palpebral aperture, intraocular pressure, Hess chart, nor was there any statistically significant improvement of the irritative conjunctival symptoms.

No effect of low power laser in lateral epicondylitis.

Thirty-six patients with lateral epicondylitis of the elbow (19 women, 17 men, median age 48 yrs) were treated either with active laser or placebo, 18 patients in each group. The active laser was a GA-AL-AS 30 mW/830 nm low power laser (LPL). The study design was double blind and randomized. The treatment session consisted of eight treatments, two per week. Patients were irradiated on tender points on the lateral epicondyle and in the forearm extensors. Output power was 3,6 J/point. A follow up was performed by telephone, 10 weeks after the last treatment. No difference between laser and placebo was found on lateral elbow pain (Mann Whitney test, 95% confidence limits). We conclude that low power laser offers no advantage over placebo in the treatment of musculoskeletal pain as lateral epicondylitis. Further studies with low power laser treatment of musculoskeletal pain seem useless.

Epilepsy and pregnancy: lamotrigine as main drug used.

OBJECTIVES: To study the risk of teratogenicity in infants of women with epilepsy. MATERIAL AND METHODS: Prospective data from 1996 to 2000 comprised 147 pregnancies. The most frequent antiepileptic drugs (AEDs) used were lamotrigine (LTG) 35% (n = 51), oxcarbazepine (OXC) 25% (n = 37) and valproate (VPA) 20% (n = 30). Seventy-four per cent (n = 109) received monotherapy. Folic acid supplementation was taken during first trimester by 118 patients (80%). RESULTS: The overall risk of malformations among newborns in the AED-exposed group was 3.1% (n = 4). Two children were born with multiple malformations (VPA monotherapy), two children had ventricular septal defects (one OXC monotherapy, and one OXC and LTG). The risk of malformations was 2.0% in women treated with LTG and 6.7% in women treated with VPA (NS). CONCLUSION: Despite the small number of cases in the study these data indicate that treatment with LTG during pregnancy might be relatively safe. Larger prospective studies are needed to obtain adequate power for statistical analysis.