Long-Term Outcome of West Syndrome: A Study of Adults with a History of Infantile Spasms

Summary: To our knowledge, ours is the first study to evaluate the outcome of infantile spasms (IS) in adult patients. We analyzed 214 children born between 1960 and 1976 who had been followed for 20–35 years or until death at 3 months to 30 years of age. Mortality was 31% (67 of 214 patients). Thirty-six of the surviving patients (24%) had normal (25 patients) or only slightly impaired (11 patients) intelligence as assessed by their educational abilities. Four had academic occupations. Eight were married or living unmarried with a partner. Five had healthy children. At follow-up, the EEGs of the 25 normal persons were either normal or slightly abnormal, demonstrated focal findings in 9 (36%), and had unspecific changes in 1. Focal abnormalities were not more common in patients with less good outcomes (37%). In patients with normal neurological outcomes, IS had been classified as cryptogenic only in 9 of 25 (36%) cases. Therefore, some patients with IS apparently have normal intelligence and socioeconomic status as adults, including patients whose spasms were either symptomatic or associated with focal EEG findings.     

Multiple sclerosis and chronic inflammatory diseases A case-control study

Introduction – Disease associations may provide useful etiological leads in relation to diseases of unknown cause. Material and methods – We conducted a hospital-based case-control study of 155 MS patients and 200 controls in Hordaland County, Norway to investigate the possible association between MS and autoimmune diseases. Results – The MS patients had a statistically significant more frequent coexistence of rheumatoid arthritis, psoriasis, and goitre when compared to the controls (OR = 2.96; 95% CI 1.23–7.66). This difference persisted when analysing the definite MS cases separately (OR = 2.90; 95% CI 1.10–7.96). The familial occurrence of chronic inflammatory diseases was not significantly different in cases and controls. A significant increased risk to develop MS occurred in first degree relatives of MS patients (OR = 12.58; 95% CI 1.73–552). Conclusion – Acknowledging the low figures, the uncertain estimates with large confidence intervals, and thus the obvious role of chance in this study, the results might indicate that a generalized, genetically controlled problem of the immune system could result in aggregates of the reported diseases, all of which are partly characterized by abberrations of the immune system.     

Clinical studies on multiple sclerosis

Probable and possible MS cases with a debut during the years 1950–1964 within the city of Gothenburg were identified (312 cases). This corresponds to an incidence of 5.3 per 100,000. The material was stratified according to diagnostic probability into three categories. For the final analysis cases with the lowest diagnostic probability were omitted (about 9 %). The follow-up was completed during 1977, i.e. 13–27 years from onset. A longitudinal analysis of each case was based upon a mixed prospective/retrospective study in which the authors personally examined the majority of the cases during most of the years. The female/male ratio was 1.5–1.6. The mortality rate was higher for males due in part to an earlier progressive development of multiple sclerosis and in part to a higher trend for acquisition of other mortal diseases. Bouts, as the first manifestation of the disease, were more frequent in young ages than in old, and more frequent among females than in males. The average bout frequency decreased significantly with the duration of the disease as well as with the age of onset. The opposite trend was characteristic for development of a progressive course. Among symptoms at onset, those indicating lesions of long sensory and/or motor tracts (particularly the sensory) dominated. Such initial symptoms were seen in 30–40 % of the younger patients and 70 % of the older patients. The rest was divided between cases with optic nerve lesions (20–30 % among younger, 12–15 % among older patients) and brain stem lesions (approx. 25 % among younger, 10–19 % among older patients). The occurrence of various symptoms during the first two decades of the disease was also analyzed and the pattern of symptoms presented graphically. These results will be treated further in subsequent studies.     

Increased apolipoprotein E4 allele frequency is associated with vascular dementia in the Hungarian population

Introduction – The regulatory role of apolipoprotein E in lipid transport and metabolism was utilized to investigate the allelic association between the apolipoprotein E4 (apoE4) allele and vascular dementia (VD) in a selected sample of Hungarian patients with multiple deep subcortical infarcts and leukoaraiosis. Material and methods – Thirty-four Caucasian VD cases and 79 healthy control probands were involved in this study according to the criteria of ICD-10 and NINDS–AIREN International Workshop Diagnostic Criteria. The genomic DNA was isolated from whole blood and the apoE alleles were determined by polymerase chain reaction. Results – The E2, E3 and E4 allele frequencies in the VD group were 5%, 76%, and 19%, respectively; and significant ( P <0.03) differences were found in comparison with the data on the healthy controls (E2, 6%; E3, 87%; E4, 8%). The apoE4 allele frequency was intermediate between HC and Alzheimer's dementia group (28%). Conclusion – These results indicate that the apoE4 allele could be a risk factor not only for certain primary degenerative, but also for vascular dementias.     

Does myasthenia gravis provide protection against cancer?

Objectives –  Reports have been made of an altered rate of extrathymic malignancies in patients with myasthenia gravis (MG). This study compared the rate of such malignancies in a group of MG patients with an optimal control group.
Materials and methods –  From the Norwegian Cause of Death Registry, we identified 249 dead MG patients (1951–2001) and a control group of 1,245 individuals (five per patient) dead in the same period, matched for sex and year of birth.
Results –  Patients with MG had a lower occurrence of malignant disease as underlying or contributing cause of death than the controls (8.8% vs 27.2%, P  < 0.001). The main difference was found for colorectal cancer, breast cancer and cancer in the upper digestive tract (esophagus and stomach).
Conclusions –  We report a significantly lower rate of extrathymic malignancies in patients with MG than in controls, and we hypothesize that MG treatment or the immunological mechanisms involved in MG may protect patients with MG from developing an extrathymic malignancy.     

Psychotherapy with the aid of LSD

Psychotherapeutic treatment with hallucinogens, usually called psycholytic treatment, was used at Modum Bads Nervesanatorium (MBN) on inpatients from 1961 to 1976. The hallucinogens used were LSD, psilocybin, and CZ 74. The indications and the treatment procedure at MBN are described. From 1961 to 1976, 379 patients received 2205 psycholytic treatments, mostly with LSD. From 1970 to 1976, only nine patients had their first hospitalization with psycholytic treatment. Sociodemographic data, diagnoses, length of hospitalizations, and number and kind of treatments were registered. Of the treated patients, 40% were women and 60% were men. The diagnostic distribution (ICD 7) was psychoses, 4.5%; obsessive neurosis, 11.1%; other psychoneuroses, 26.3%; sexual deviation, 6.3%; other disorders of character, 46.2%; alcoholism, 5.0%; and drug addiction, 0.5%. In 1961 it was especially obsessive neurosis (35.7%) that was treated, and in the last 7 years it was again the same diagnosis (75.6%). The mean length of hospitalization for the psycholytically treated patients was 132 days, nearly double the average of 68 days for all hospitalized patients at MBN.

□Hallucinogens, Inpatients, LSD, Psycholytic treatment, Psychotherapy.     

Cerebral embolism and epileptic seizures — the role of the embolic source

Objectives – To study the clinical outcome of patients with epileptic seizures due to ischemic stroke (IS) of cardiac or artery-to-artery embolism. Methods – Seizures due to IS of cardiac or artery-to-artery embolism are differentiated by clinical, neuroimaging and cardiovascular test data. Results – From 174 cases with supratentorial IS, 13 patients suffered from epileptic seizures due to cardiac embolism, 11 patients due to artery-to-artery embolism. The patients with cardiac IS showed an equal sex distribution and EEG abnormalities in 6 patients, the initial seizure occurred on average after 222 days (SD, ±69 days). Among the 11 patients with artery-to-artery embolic IS, there were 9 males and 2 females and EEG abnormalities in 10 patients. The initial seizure occurred on average after 447 days (SD, ±177 days). Conclusion – In seizures due to artery-to-artery embolism, there is a male preponderance and a higher incidence of EEG abnormalities, symptomatic seizures appear later compared to IS due to cardiac embolism.     

Evaluation of multiple sclerosis diagnostic criteria in Suzhou, China – risk of under-diagnosis in a low prevalence area

Objective –  To evaluate the discharge diagnosis of demyelinating diseases in the central nervous system (CNS) and analyze the predictive value of the new diagnostic criteria in Suzhou, China.
Materials and methods –  We collected clinical information and data of laboratory examinations for all cases with a diagnosis of various demyelinating diseases in the CNS. All data were reviewed individually by four senior neurologists, and a diagnosis was finally given to each patient according to the McDonald criteria and the Poser criteria for multiple sclerosis (MS).
Results –  In the analysis, 176 patients with a diagnosis of demyelinating diseases in the CNS at discharge were included. In 82 patients with a diagnosis of MS at discharge, the MS diagnosis was confirmed for 74 patients according to the McDonald criteria for MS, and the positive predictive value for the discharge diagnosis of MS was 90.2% (74/82). According to the Poser criteria, 61 patients were diagnosed as MS. The consistency of the two diagnostic criteria for MS was 78.4%, based on the results of the evaluation.
Conclusions –  Under-diagnosis of MS could be one of the explanations for the low prevalence of MS in China. Compared to the Poser criteria, the McDonald criteria had a higher sensitivity for the diagnosis of MS.     

Cerebral abscess A long-term follow-up

During the period 1935-1976, 200 cases of brain abscess were treated in the University Clinic of Neurosurgery, Rigshospitalet, Copenhagen. 119 patients survived. Out of 40 deaths during the follow-up period, 37 were unrelated to the previous brain abscess. 12 patients emigrated and were lost to follow-up. History and neurologic examination of the remaining 67 surviving patients with follow-up periods of between 3 and 40 years (mean 18 years) showed no sequelae in 23, unrestricted working capacity in 46, epilepsy in 37, neurologic deficit in 18, and intellectual impairment in 13 patients. The most serious sequelae, incapacitating epilepsy and severe mental reduction, were seen in patients with brain abscess during childhood.     

Clinical predictors in patients with refractory epilepsy exposed to levetiracetam: a single-center study

Objectives –  This aim of the study was to ascertain the importance of clinical parameters on the response to treatment in refractory epilepsy patients on levetiracetam (LEV).
Materials and methods –  We retrospectively evaluated medical records of 132 patients aged 17–78 years with refractory epilepsy (defined as a failure of at least two antiepileptic drugs due to the lack of efficacy) exposed to LEV. We analyzed the response (seizure freedom or continuing LEV) using logistic regression.
Results –  Of 132 patients exposed to LEV, 103 cases continued the drug. Of the discontinuations (29/132), 75% were for lack of efficacy and 25% for tolerability problems. Twenty-three percent of the previously refractory patients achieved seizure freedom for at least 1 year with LEV in combination therapy. The dose of LEV in 80% of seizure-free patients was 1000 mg/day or less. The duration of epilepsy, age and sex were not associated with response to LEV. Seizure freedom was associated with epileptic syndrome or etiology. If no specific syndrome was recognized, there was a significantly greater chance for response compared with temporal lobe epilepsy (OR 20.76; 95% CI 2.12–203.61).
Conclusions –  Our study was based on the careful clinical evaluation of the patients with extensive use of video EEG (50%) and MRI scans (95%). These clinical predictors were evasive in previous studies. This study showed that they are worth pursuing but significantly larger groups of patients need to be investigated to reach significant findings.     

Incidence of newly diagnosed epileptic seizures in a French South Indian Ocean Island, La Réunion (EPIREUN)

Purpose:   To assess the incidence of newly diagnosed epileptic seizures in the population of La Réunion.
Methods:   From July 1, 2004 to June 30, 2005, we conducted a prospective, observational, and multicenter epidemiologic study to identify patients with newly diagnosed epileptic seizures. Febrile and neonatal seizures were excluded.
Results:   Seven hundred sixty-six patients were included. The standardized (2000 U.S. population) incidence rate of all suspected cases of newly diagnosed (provoked and unprovoked) epileptic seizures was 115.4/100.000 person-years [95% confidence interval (CI) 106.7–124.0]. We observed a bimodal distribution: The crude incidence was 99.5/100,000 in the group aged 0–14 years and 330.8/100,000 in those older than 65 years. One hundred thirty-five cases were classified as provoked seizures (17.6%; incidence 17.7/100,000). Alcohol consumption, cranial trauma, and cerebrovascular disease were the most frequent causes (27.4%, 11.1%, and 10.4%, respectively). Six hundred twenty cases were classified as unprovoked seizures (single and recurrent) (80.9%; incidence, 81.2/100,000). Two hundred sixty cases of seizures were due to stable neurologic conditions (incidence, 34.1/100,000) and the most common causes were cerebrovascular disease (46.2%), alcoholism (20.4%), and cranial trauma (5.4%). Evolutive neurologic conditions contributed to 23 cases (incidence, 3.0/100,000). Lastly, unprovoked seizures with unknown etiology were 337 (incidence, 44.2/100,000).
Conclusions:   The global incidence rate of newly diagnosed epileptic seizures in La Réunion was clearly higher than those observed in industrialized countries and similar to those observed in developing countries. The major risk factors were represented by cerebrovascular disease, alcohol consumption, and cranial trauma. Surprisingly, there were few infections.     

Irritable bowel syndrome is more frequent in patients hospitalized for ischaemic colitis: results of a case–control study

Abstract  It has been suspected that there is an epidemiological link between irritable bowel syndrome (IBS) and ischaemic colitis (IC). We performed a retrospective case–control study to compare the frequency of IBS in patients hospitalized for IC compared with that of patients with peptic ulcer bleeding. Cases were patients with a first episode of IC and controls were patients with a first episode of peptic ulcer bleeding, matched to cases for sex and 10-year age-class. Diagnosis of IBS was based on medical information extracted from hospital medical files and a standard self-questionnaire. The association between IBS and IC was tested using Mc Nemar's paired odds ratio (OR); confidence interval at 95% (CI 95%) was calculated; Mantel–Haenzel's Chi² was applied. A total of 113 cases and 113 matched controls were studied. There were 37 males and 76 females and the mean age was 69 ± 15 years in each group. The prevalence of IBS in cases was 16.9% vs 1.8% in controls. The risk of IBS was 11.05 times higher among cases than in controls ( P  < 0.001); CI 95%: (2.45–49.74). A total of 87 pairs with complete data were used for OR calculation. The risk of IBS was 7.5 times higher in cases than in controls ( P  = 0.002); CI 95%: (1.72–32.80). This case–control study shows that IBS is more frequent in IC patients than in controls.     

A prospective study of 133 patients with anorexia nervosa

One hundred and thirty-three patients with anorexia nervosa have been treated in a prospective study from 1958 to 1976. The doctor and the patient alone formed the therapeutic team, the doctor playing the role as an instructor in the pathophysiology of hunger without discussing social or psychological problems. At follow-up 58 % of the patients were grouped as ‘good’, and 28 % as ‘intermediate’, figures which are in accordance with those obtained from other authors. Fourteen per cent of the patients were grouped as ‘poor’. None of the patients died. That therapists with different attitudes to treatment may obtain fairly similar results strengthens our view of a strong tendency to spontaneous recovery in anorexia nervosa. The outlook for patients with a short history of anorexia nervosa seems good.     

The Changing Style of Symptomatology of Hysteria

Abstract: The symptomatology of 412 hysteric women was investigated and analyzed at the Neuropsychiatric Clinic, Tohoku University Hospital, from 1952 till 1973, according to the study stages, 1952–1957 and 1958–1973, and the patients' residence, both rural and urban. Withdrawal with taciturnity was seen in 27% of them and stubbornness with egocentricity in 14% as the frequent personality traits. Physical distress was significantly a frequent problem in the recent rural patients.
Among symptoms insomnia and tinnitus have decreased. Mutism and deviated attitude were seen more frequently in the rural patients. Astasiaabasia and syncope have increased. Generally speaking, hysteric symptomatology seemed to have become of tranquil style recently and the rural patients showed more frequently nonverbal expression in terms of mutism and deviated attitude than the urban patients.     

Neuropathological findings in 224 patients with temporal lobe epilepsy

During the period between 1976 and 1990, 247 patients with pharmaco-resistant complex partial seizures and a documented unilateral epileptogenic area in the mediobasal temporal lobe underwent a selective amygdalo-hippocampectomy procedure at our institution. Biopsy specimens from 224 patients (91% of the total) were available for a retrospective histopathological and immunohistochemical review. The tissue specimens of 23 patients without evidence for a macroscopic lesion have been used for neurochemical studies and could not be evaluated histopathologically. The most common temporal lobe pathology were neoplasms in 126 patients, i.e. 56%. Tumor entities observed included 23 astrocytomas (18% of all tumors), 17 gangliogliomas (13%), 15 oligodendrogliomas (12%), 15 cases of glioblastoma multiforme (12%), 13 pilocytic astrocytomas (10%), 12 oligo-astrocytomas (10%), 11 anaplastic astrocytomas (9%) and 20 tumors of various other histologies. In 23 specimens (10%), small foci of oligodendroglia-like clear cells were found. The frequent association of these foci with low-grade gliomas or neural hamartomas raises the possibility that these structures may serve as precursor lesion for neuroepithelial tumors of the temporal lobe. In 98 cases, pathological changes of non-neoplastic origin were encountered. The most common diagnoses in this group included hippocampal gliosis/sclerosis (49 cases, 22%) and vascular malformations (20 cases, 9%). Hamartomas, i.e. focal accumulations of dysplastic neuro-glial cells were diagnosed in 14 patients (6%). In only four cases have we not been able to detect any microscopic pathology. These results indicate that a high proportion of pharmaco-therapy-resistent complex-partial seizures are caused by neoplasms of the temporal lobe, some of which appear to the strikingly overrepresented in this group of patients.     

Evolving pattern of Guillain-Barre syndrome in a community hospital in Israel

Objectives –  To investigate the frequency of axonal Guillain–Barre syndrome (GBS) in our ward over 6 years (1999–2005).
Materials and methods –  Clinical and electrophysiological findings of 40 patients admitted to neurology with abnormalities compatible with acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN) and acute inflammatory demyelinating polyneuropathy (AIDP) were reviewed.
Results –  Electrophysiological findings showed that 25 (63%) patients had AIDP, nine (22%) AMAN and six (15%) AMSAN. There were significant differences in disease severity. Most axonal patients (87%) were hospitalized with moderate or severe symptoms (3–4 Hughes grade score) and progressed to severe grade (4–6) in comparison with AIDP patients (64% admitted with mild forms) (1–2 Hughes grade score) and progressed to severe in 44% of cases. Cranial nerve involvement was more frequent in AIDP (56%) in comparison with the axonal type (13%). Raised cerebrospinal fluid protein at the time of hospitalization was observed in 76% of demyelinating and 33% of axonal patients.
Conclusions –  Axonal GBS occurred more frequently in Israel compared with other Western countries.     

Incidence and prognosis of brain abscess in a defined population: Olmsted County, Minnesota, 1935-1981

The incidence of brain abscess was studied on all cases occurring in residents of Olmsted County, Minn., from 1935 through 1981. Thirty-eight cases (9 cases first diagnosed at autopsy) were identified and followed through the Rochester Olmsted County medical record-linkage system at the Mayo Clinic. The incidence rate was 1.3/100,000 person-years (PY), 1.9 in males and 0.6 in females. Incidence decreased from 2.7 in 1935-44 to 0.9 in 1965-81. Rates were higher in children 5-9 years old (2.4) and after age 60 (2.6 PY). An etiologic agent was identified in 29 cases (76%) with streptococci being the most frequently isolated. Case-fatality ratio was 38% (11/29), stable over time. Concurrent bacterial meningitis was the strongest predictor of death. Neurologic sequelae were observed in 8 (44%) of the 18 surviving patients including epilepsy (5 cases), deafness and motor impairment.     

The prevalence of epilepsy and pharmacoresistant epilepsy in adults: a population-based study in a Western European country

Purpose: To determine the prevalence of epilepsy in a defined adult population and identify the frequency and principal features of pharmacoresistant epilepsy.
Methods: From a population over 15 years of age residing in a medium-sized French city, all patients with epilepsy on June 30, 1995 were identified from multiple sources. Pharmacoresistance was defined as failure to control epilepsy by at least two first-line antiepileptic drugs, with a seizure frequency of at least one per month for 18 months. Collected data were examined by experts in epileptology, and responding patients were reexamined using a standardized diagnostic questionnaire. ILAE definitions and classifications were used.
Results: The age-adjusted prevalence of active epilepsy was 5.4 per 1,000 (95% CI: 4.7–6.0) and was higher for males (7.8) than for females (5.2). For epilepsy in remission under treatment, this rate was 0.7 per 1,000 (95% CI: 0.5–0.95). Age-specific prevalence was highest in age groups 25–49 years and declined in the oldest age groups. Localization-related seizures represented 61.1% of cases and generalized seizures 30.9%. The proportion of noncontrolled epilepsy (seizure-frequency at least one per month for 18 months) was 15.6%, corresponding to a prevalence of 0.94 per 1,000. In this group, the mean age at onset was lower (p = 0.0007) and localization-related epilepsy more frequent (p = 0.01).
Conclusion: The findings support previous epidemiological estimates of the prevalence of epilepsy in developed countries. For approximately one patient in eight, epilepsy was not adequately controlled.     

Long-Term Survival of People with Unprovoked Seizures: A Population-Based Study

Summary: Purpose : Few population-based studies of longterm survival in people with seizures or epilepsy have been made.
Methods: Between January 1, 1960 and December 31, 1964, we identified 224 incidence cases of unprovoked seizures in Iceland and determined survivorship status and date of death for the cases as of January 1, 1996. We compared survivorship with that expected based on data from age-/sex-specific life tables from the country for 1961–1990 and calculated the standardized mortality ratio (SMR).
Results: By 30 years after diagnosis, there were 45 deaths among patients with unprovoked seizures as compared with an expected 28 deaths [standardized mortality ratio (SMR) 1.6; 95% confidence interval (CI) 1.2–2.21. Patients with unprovoked seizures of unknown etiology did not have a significant increase in mortality overall (SMR 1.3, 95% CI 0.8–1.9) or in any time interval. For patients with remote symptomatic un provoked seizures, mortality was increased (SMR 2.3, 95% CI 1.4–3.5). This increase was attributable to excess mortality for the first 15 years after diagnosis (SMR 4.1, 95% CI 2.4–6.6), and SMR was not different after that time.
Conclusions: Survivorship was decreased for the population of patients with unprovoked seizures. The increased mortality was primarily due to excess mortality in patients with remote symptomatic seizures, occurring in the first 15 years after diagnosis. Overall mortality for idiopathic unprovoked seizures was not significantly increased.     

Health status and life satisfaction after decompressive craniectomy for malignant middle cerebral artery infarction

Objectives –  To study the long-term outcome in patients with malignant middle cerebral artery (MCA) infarction treated with decompressive craniectomy. The outcome is described in terms of survival, impairment, disabilities and life satisfaction.
Materials and methods –  Patients were examined at a minimum of 1 year (mean 2.9, range 1–6) after the surgery and classified according to the Glasgow Outcome Scale (GOS), the National Institutes of Health Stroke scale (NIHSS), the Barthel Index (BI), the short-form health survey (SF-36) and the life satisfaction checklist (LiSat-11).
Results –  Eighteen patients were included. The long-term survival was 78%. The mean NIHSS score was 13.8 (range 6–20). No patient was left in a vegetative state. The mean BI was 63.9 (5-100). The SF-36 scores showed that the patients' view of their health was significantly lower in most items compared with that of a reference group. According to the LiSat checklist, 83% found their life satisfying/rather satisfying and 17% found their life rather dissatisfying/dissatisfying.
Conclusion –  We conclude that the patients remained in an impaired neurological condition, but had fairly good insight into their limitations. Although their life satisfaction was lower compared with that of the controls, the majority felt that life in general could still be satisfying.