Anosognosic graphomimia: an uncommon variety of hypergraphia in right sylvian infarction

Following a massive infarction in the territory of the right sylvian artery, a right-handed patient with a left hemiplegia and anosognosia, developed a peculiar hypergraphia. It consisted of an inappropriate and permanent writing behaviour, disappearing only during sleep. Space-constructional components of writing were severely disturbed. Graphemes were poorly formed. The graphic disturbances were different from hypergraphia previously described in stroke patients who produced linguistically correct but semantically loose writing. They were also different from graphomania reported in a case of fronto-callosal glioma. Our case of anosognosic hypergraphia suggests a sudden loss of inhibition of writing activity of the left hemisphere. The term graphomimia is proposed to distinguish this writing behaviour from the cases of hypergraphia previously reported and from graphomania.     

Psychosocial study of epilepsy in Africa

As documented by many authors, the social position of epileptics in many small scale societies of Africa is marginal at best, and is often characterized by rejection, discrimination, even ostracism. Such negative and noxious attitudes toward persons suffering from epilepsy are rooted in traditional beliefs about causes and nature of convulsive disorders and these have parallels in European history. This article focuses on the psychosociocultural aspects and indigenous concepts of epilepsy, on popular attitudes towards, and social status of, sufferers from epilepsy in a Tanzanian tribal population. The authors present a comparative analysis of focus group discussions conducted with epileptics and with matched controls in two isolated communities. In one community (Mahenge) a clinic for epilepsy has been operating for over 36 years, with a public education component during the last four years, whereas in the other community (Ruaha) epileptics have only been sporadically treated in a small mission dispensary and people have had little opportunity to learn about the nature and modern treatment of convulsive disorders. The responses obtained in focus group discussions reflect the significant change in notions about the illness, in the attitude toward and in the social status of epileptics in Mahenge, while the people of Ruaha still regard epilepsy as a typical "African" affliction fraught with supernatural danger and not effectively treatable by modern medicine.     

Electroencephalogram and clinical focalities in juvenile myoclonic epilepsy

Partial seizures and asymmetric abnormalities seen on electroencephalogram (EEG) are infrequent in juvenile myoclonic epilepsy, but when present, can lead to a misdiagnosis of partial seizures. We report four patients with juvenile myoclonic epilepsy who had generalized spike or polyspike and wave discharges on EEG in addition to clinical and EEG evidence of focality. The clinical course and response to therapy was similar to that in typical juvenile myoclonic epilepsy.     

Algorithm on the clinical evaluation of epilepsy

The aetiology of growth retardation in children with X-linked hypophosphatemic rickets (HPR) has not been totally defined. We evaluated growth hormone (GH)/insulin-like growth factor-I (IGF-I) changes in relation to linear growth and biochemical parameters in seven children with X-linked hypophosphatemic rickets before and after treatment with 1,25-dihydroxyvitaminD3 and phosphate therapy for a year or more. Moreover, we compared patients' growth data and GH/IGF-I changes with those for 20 age-matched children with normal variant short stature (NVSS)[with normal GH secretion and height standard deviation score (HtSDS) before -2]. Before treatment, all children with HPR secreted normal GH in response to clonidine provocation (> 10 microgram/l) and their IGF-I concentration was significantly lower than those with NVSS. The HtSDS and growth velocity (GV) of children with HPR improved significantly after (-3.05, 8.9 cm/year, respectively) v. before (-3.9 and 4.1 cm/year, respectively) therapy. Their serum IGF-I concentration increased significantly from 76.7 ng/ml before to 99.6 ng/ml after treatment. In summary children with HPR had no abnormality of GH secretion but improvement of their linear growth was associated with significant increase of circulating IGF-I concentration after treatment.     

A double-blind controlled clinical trial of oxcarbazepine versus phenytoin in adults with previously untreated epilepsy

In the past 4 years, cytokine flow cytometry has emerged as the premier technique for enumeration of cytokine producing T cells. The multiparameter capability of flow cytometry permits the simultaneous detection of two or more cytokines within a single cell, allowing true Th1 vs. Th2 determination. The high throughput inherent to flow cytometry has enormous advantages when applied to clinical research questions previously not amenable for study using labor intensive techniques such as ELISPOT, limiting dilution and T cell cloning. Furthermore, cytokine flow cytometry allows the study of individual T cells directly ex vivo, minimizing artifacts due to long term culture. As such, cytokine flow yields unique insights into cytokine biology heretofore not possible. We have used cytokine flow cytometry to examine coexpression of cytokines within the memory/effector CD4+, CD27- subset. Doing so, we have found distinct cytokine producing subsets that correlate with the previously described Th1, Th2 and Th0 subsets. The majority of cytokine producing cells were of these first two subsets with fewer cells coexpressing IFN-gamma and IL-4. These results validate the Th1/Th2 hypothesis and demonstrate specific subsets of cytokine producing T cells in fresh ex vivo human T cells.     

Neuroradiologic study of partial temporal epilepsy

MR imaging is considered the neuroradiological procedure of choice for the study of intractable partial epileptic patients. The role of CT-scan is limited to the demonstration of calcific components of the lesion and of bony abnormalities of the adjacent skull. In this chapter we describe an MR protocol for the evaluation of temporal lobe epilepsy patient; moreover, here there are described neuroradiological characteristics (MR and CT) of the more frequent temporal lobe lesions.     

A prospective study of the early postsurgical psychiatric associations of epilepsy surgery

OBJECTIVES: To examine prospectively the frequency and nature of psychiatric symptoms seen in patients during the first three months after temporal lobe surgery for chronic intractable epilepsy and in addition to study the relation between presurgical mental state, laterality of surgery, and postsurgical seizure and psychiatric course. METHOD: A consecutive series of 60 patients being assessed for temporal lobe surgery for intractable epilepsy were studied. They were interviewed before surgery and at six weeks and again at three months after operation. RESULTS: At six weeks after surgery half of those with no psychopathology preoperatively had developed symptoms of anxiety or depression and 45% of all patients were noted to have increased emotional lability. By three months after surgery emotional lability and anxiety symptoms had diminished whereas depressive states tended to persist. Patients with a left hemispheric focus were more likely to experience persisting anxiety. CONCLUSION: The early months after surgery for epilepsy are characterised by the relatively common presence of psychiatric symptoms. It is proposed that presurgical and early postsurgical neuropsychiatric involvement in programmes of surgery for epilepsy will help to improve the quality of the treatment package offered to patients.     

Psychopathology of posttraumatic epilepsy in the light of personal clinical observation

The influence of apomorphine, chloral hydrate, haloperidol, morphine, oxotremorine, pargyline, probenecid and promethazine on DOPAC and HVA levels was studied in the substantia nigra (including the ventral tegmental dopaminergic regions) and corpus striatum of the rat brain. The time--effect curves of changes in HVA levels after pretreatment with apomorphine, haloperidol, morphine, oxotremorine or promethazine are presented. The time--effect curves for the substantia nigra showed an initial rapid HVA rise, which was not observed in the corpus striatum. Promethazine treatment caused a small but significant HVA rise in the substantia nigra only. Chloral hydrate, morphine and oxotremorine induced a similar percentage increase in DOPAC and HVA levels in the substantia nigra as well as in the corpus striatum. Haloperidol, however, caused a small percentage change in the metabolite levels in the substantia nigra when compared to the pronounced rise seen in the corpus striatum. The apomorphine-induced HVA decrease observed in both structures provides evidence for the presence of a dopaminergic receptor in the substantia nigra.     

Occurrence, causes and clinical characteristics of status epilepsy in adults

INTRODUCTION: Status epilepticus, particularly grand mal, is one of the gravest and most dramatic conditions in neurology requiring immediate attention. Status epilepticus can occur in epileptic patients, often with higher mortality rates in symptomatic than idiopathic, but also as an initial symptom of a number of neurological and systemic diseases. No data are available on the exact incidence rates of status epilepticus. According to some assessments, 10% of patients have at least one status epilepticus in their lifetime (3,6). The prognosis mostly depends on the main cause, time in which seizures are stopped and age of patients. Latest data available in literature suggest the mortality rate of 2-8%. MATERIALS AND METHODS: We analyzed frequency of hospital admissions, causes and clinical characteristics of status epilepticus in adults. The study was retrospective, based on case histories of epileptic patients from the Intensive Care Unit of the Neurology Clinic in Novi Sad in 1990, 1993 and 1995. Special emphasis was placed on differences in studied parameters between cases confirmed earlier and those with status epilepticus occurring as an initial symptom of some other illness or condition. RESULTS: Number of hospital admissions rose slightly in the interval observed in comparison with total admissions (0.68% in 1990, 1.24% in 1993, and 1.73% in 1995) (Tabs 1 and 2). During 1993, status epilepticus was more frequent in cases confirmed earlier (69%) compared with the years 1990 (56%) and 1995 (43%) (Graf.1). Epileptic patients were younger on the average than nonepileptic ones (Tab. 3). Status epilepticus occurred more often in male patients (Tab. 4). Irregular treatment was the prevailing cause in epileptic patients (Tab 5). Symptomatic status epilepticus was reported higher in 1990 and 1995, and stroke was definitely the predominant cause (Tab 6). Convulsive grand mal status prevailed in all patients (Graf 2). Focal status was a more frequent finding in nonepileptic patients (Graf 3). Every third in 16 patients died in 1993 and every fifth in 23 in 1995 probably due to the acute destructive brain damage rather than the status itself. No deaths occurred in 1990. DISCUSSION: According to research carried out by other authors, half of grand mal status cases occurred in confirmed epileptics (4). In our study the grand mal status was reported in 70.4% cases of epilepsy. Primary cause was abrupt withdrawal of antiepileptic treatment, infections, alcohol abuse and use of convulsive drugs. This is compatible with our results which confirm that grand mal status either primary or with secondary generalization prevail in both groups of patients (7,8,9). In terms of causes of status epilepticus in nonepileptic patients, literature data mainly suggest cerebral trauma, frontal brain tumors, cerebral arteriosclerosis or other vascular disorders and anaphylaxis (4). Our results point to stroke as the major cause of status epilepticus in nonepileptic patients, similar with data presented by Towne (10). There is no data in literature concerning the relation between sex of patients and occurrence of status. In our study status epilepticus occurred more frequently in male patients. CONCLUSION: The grand mal status was the major clinical type of status in all patients and was primarily caused by discontinued or irregular antiepileptic treatment in patients with confirmed epilepsy, and by stroke in nonepileptic patients.     

Magnetoencephalography in partial epilepsy: clinical yield and localization accuracy

The goals of this study were to determine (1) the yield of magnetoencephalography (MEG) according to epilepsy type, (2) if MEG spike sources colocalize with focal epileptogenic pathology, and (3) if MEG can identify the epileptogenic zone when scalp ictal electroencephalogram (EEG) or magnetic resonance imaging (MRI) fail to localize it. Twenty-two patients with mesial temporal (10 patients), neocortical temporal (3 patients), and extratemporal lobe epilepsy (9 patients) were studied. A 37-channel biomagnetometer was used for simultaneously recording MEG with EEG. During the typical 2-3-hour MEG recording session, interictal epileptiform activity was observed in 16 of 22 patients. MEG localization yield was greater in patients with neocortical epilepsy (92%) than in those with mesial temporal lobe epilepsy (50%). In 5 of 6 patients with focal epileptogenic pathology, MEG spike sources were colocalized with the lesions. In 11 of 12 patients with nonlocalizing (ambiguous abnormalities or normal) MRI, MEG spike sources were localized in the region of the epileptogenic zone as ultimately defined by all clinical and EEG information (including intracranial EEG). In conclusion, MEG can reliably localize sources of spike discharges in patients with temporal and extratemporal lobe epilepsy. MEG sometimes provides noninvasive localization data that are not otherwise available with MRI or conventional scalp ictal EEG.     

A clinical and epidemiological study of gastro-enteritis

Over a 13-year period, 2,903 Asian, Coloured and White children with gastro-enteritis were admitted to the Wilkins Infectious Diseases Hospital, Salisbury. During a pilot study of 250 patients conducted over the first 2 years, a definite seasonal variation in the occurrence of gastro-enteritis, with a peak incidence in winter, was noticed, and this led to a more detailed study of 2,653 patients over the next 11 years. Bacterial pathogens were recovered from 27% of patients, strains of Shigella accounting for 12% Salmonella for 7% and Escherichia coli for 8%. A severe outbreak of E. coli infection occurred between October 1971 and December 1972, and 4 of a total of 9 deaths occurred during this period, all in children suffering from E. coli 0111/B4 infections. The bacterial recovery rate was highest in the summer (40%) and lowest in the winter (12%). Winter diarrhoea broke out over 3-month periods during each year and accounted for 37% of the cases, but none of the children who contracted it died. The clinical picture of winter diarrhoea differed from the others and the most serious illness was that caused by enteropathogenic strains of E. coli. The over-all mortality in the series was 0,27%.     

Rolandic epilepsy: an incidence study in Iceland

PURPOSE: We wished to determine incidence, clinical features, and prognosis of benign rolandic seizures (BRS) and benign rolandic epilepsy (BRE) in a total population. METHODS: Cases were ascertained through review of all EEG records, and diagnosis was verified by review of medical records. Follow-up information regarding seizures and treatment was obtained from parents and treating physicians. RESULTS: In the Icelandic population aged 3-15 years, the incidence of BRS is 6.2 and BRE 4.7 in 100,000. Five years after onset 95% were seizure-free. At last follow-up, all were seizure free and had not been treated with antiepileptic drugs (AEDs) for at least 1 year. CONCLUSIONS: Our study demonstrates that BRS is a common entity in children. The prognosis is excellent and treatment is not necessary in all cases. It is important to identify BRE/BRS correctly and distinguish it from other types of epilepsy.     

A test of spatial memory and its clinical utility in the pre-surgical investigation of temporal lobe epilepsy patients

Deficits in memory for figurative detail, spatial composition and the spatial location of objects in a scene have been reported postoperatively in right temporal lobectomy patients. The aim of this study was to examine whether these deficits can be used as a sign of lateralised dysfunction in pre-surgical temporal lobe epilepsy (TLE) patients. Sixty-nine patients with lateralised TLE (27 right, 42 left) were assessed on a battery of neuropsychological tests, including tests of general intellectual functioning and psychomotor speed and standardised memory tests involving the learning and recall of verbal and non-verbal material. A new task, the "Aspects of Spatial Memory Test" (AoSMT), based on the experimental tasks developed by Pigott and Milner [39] was also administered. The RTLE and LTLE groups did not differ in their overall level of intellectual function or on measures of cognitive and motor speed. On the AoSMT the LTLE group recognised significantly more figurative detail changes than the RTLE group. In addition, the RTLE group took significantly longer than the LTLE group to identify changes in orientation, figurative detail and filled/unfilled spaces. Poor scores on the AoSMT were significantly correlated with quantitative MRI measures of right hippocampal pathology. The clinical and theoretical implications of these findings are discussed.     

The lateralizing value of ictal clinical symptoms in uniregional temporal lobe epilepsy

Since 1984, the Cancer and Leukemia Group B (CALGB) has focused its clinical research in stage IV non-small cell lung cancer (NSCLC) on investigations of new agents and combinations. Currently, efforts are aimed at identifying non-cisplatin-based combinations with an increased therapeutic index. In stage III disease multimodality therapies have been pursued. Dillman et al. reported a study comparing standard radiotherapy versus induction chemotherapy followed by radiotherapy in patients with unresectable stage III NSCLC. The chemotherapy-treated patients were found to benefit with a 4-month increase in median survival time compared with patients receiving radiotherapy alone (13.8 vs. 9.7 months) and an increased 3-year survival rate of 23% versus 11%. This was the first randomized cooperative group study demonstrating a survival advantage resulting from the use of induction chemotherapy in locoregionally advanced NSCLC. In a subsequent study, the administration of additional "posterior" chemotherapy was not found to be feasible because of early disease progression and toxicity, while the administration of induction chemotherapy followed by concomitant chemoradiotherapy was feasible; therefore, the latter approach was studied further in a randomized phase III setting. This study compared a standard of two cycles of cisplatin and vinblastine followed by radiotherapy with an experimental arm of cisplatin and vinblastine followed by radiotherapy and concomitant carboplatin. Accrual to this study has been completed and results are expected in the near future. In resectable stage III disease, studies have focused on the optimal sequencing of multimodality therapy. A randomized study comparing standard regional therapy with radiotherapy and surgery versus a previously piloted approach combining chemotherapy, surgery, and radiotherapy was closed prematurely due to poor accrual. The next generation of studies in stage III NSCLC will focus on the integration of new chemotherapy agents into the treatment armamentarium for NSCLC. A randomized phase II study investigating paclitaxel, gemcitabine, and vinorelbine in combination with cisplatin in the induction setting and as concomitant chemoradiotherapy has recently been activated.     

A clinical study of dialysis-related osteoarthropathy in long-term hemodialysis patients

Amyloid osteoarthropathy has been seen frequently in long-term hemodialysis (HD) patients, in which the bone X-ray examination reveals characteristic cystic radiolucency (CRL) of the carpal bone, shoulder joint, hip joint and knee joint, and destructive spondylarthropathy (DSA) of cervical vertebrae. To clarify the clinico-pathological significance of CRL and DSA in HD-related amyloidosis, we investigated the grade and frequency of CRL or DSA and these relationship with age, HD duration, primary diseases, osteoarticular symptoms and blood analysis in 817 HD patients (492 male and 325 female, age: 52.6 + 15.5 years, dialysis duration: 6.8 + 5.4 years). The number of cases with osteoarticular symptoms increased with the prolongation of HD duration. CRL and DSA were observed even in patients without osteoarticular symptoms: 26.7% for carpal bone, 26.2% for shoulder joint, 17.3% for hip joint and 22.2% for DSA. The grade and frequency of CRL and DSA also increased in accordance with age and HD duration. In patients with CRL of the carpal bone, shoulder CRL was noticed in 39.7%, hip CRL in 25.8%, and DSA in 14.3% of cases, respectively, and these frequencies increased with the prolongation of HD duration. In the carpal CRL negative group, shoulder CRL was noticed in 14.6%, hip CRL in 7.5%, and DSA in 6.0%, respectively. Although there was no relationship between CRL or DSA and serum beta 2-MG level in any of the cases, the serum beta 2-MG level was lower in patients with HD showing shoulder CRL (+2) and DSA (+) for more than 16 years. No significant relationship was noticed between CRL or DSA and serum C-PTH and aluminum level. These results suggested that aging was related to CRL or DSA formation in dialysis-related amyloidosis. The findings also suggested that systemic bone X-ray examination should be considered in patients with carpal bone CRL, high-age patients and long-term HD patients even without osteoarticular symptoms.     

A prospective clinical study of stereotactic subcaudate tractotomy

This study describes a cohort of 23 patients undergoing stereotactic subcaudate tractotomy. Research Diagnostic Criteria indicated that 70% suffered major depressive disorder; the remainder mostly had a bipolar affective disorder. There were serial assessments pre-operatively and at 2 weeks and 6 months post-operatively using the Hamilton Rating Scale for depression, the Present State Examination (PSE), Newcastle Scale, the Beck Depression Inventory, and the Taylor Manifest Anxiety Scale. Neuropsychological assessment included tests thought to be sensitive to frontal lobe dysfunction, as well as tests of general intelligence, attention, memory, language and visuo-spatial function. Post-operatively, depression rating scale scores decreased significantly but most patients continued to exhibit a number of PSE syndromes. Depression rating scale scores were correlated with 1 year global outcome: there was no significant correlation except for the 6 month assessment when lower Hamilton scores were found to be associated with better global outcome. Correlations between the neuropsychological tests and the Hamilton and Beck depression scales at 2 weeks post-operatively suggested that an improvement in psychiatric condition was associated with greater efficiency on some tests of attention and verbal recall, as well as faster performance on a sorting task. By contrast, the changes at 6 months suggested an association between improvement in psychiatric condition and less efficient performance on certain neuropsychological tests including verbal recognition memory, attention and two tests of frontal lobe dysfunction.     

A clinical and cytogenetic study of Turner syndrome

Forty five case of Turner syndrome diagnosed in the Genetics Clinic, between January 1986 and December 1993, were analyzed. The most commonly observed karyotype was 45, X (44.4%), followed by 45, X/46, XX mosaicism (24.4%). Less frequently demonstrated karyotypes were 45, X/46, X, i (Xq) mosaicism and 46, X, i (Xq) (13.3%). Mosaicism for chromosome was seen in 6.7% of patients. Patients with 45, X karyotype had short stature (85%), dysmorphic facies (60%), delayed appearance of secondary sexual characters (100%) and primary amennorhea (100%). Those with 45, X/46, XX mosaicism were less often dysmorphic and presented with either primary or secondary amenorrhea. Patients with 45, X karyotype were younger at diagnosis and had a significantly shorter mean adult height than those with 45, X/46, XX mosaicism. The phenotype in patients with other karyotypic abnormalities was similar to the 45, X group. Short stature and primary or secondary amenorrhea occurring together in a female strongly suggests the possibility of Turner syndrome, which should be confirmed by chromosomal analysis.     

A clinical study on familial heart diseases

The genetic polymorphisms of the Holy Island population show some differences from a mainland series. The significance of the results is discussed in terms of specialized gene flow and population size. Some interactions between the polymorphic systems are discussed.