An unusual case of epileptic transient aphemia. Clinical and neuropsychological findings.

A patient with unusually prolonged epileptic aphemic attacks following a surgical lesion in the left frontal lobe is described. Clinical, electroencephalographic and neuropsychological findings are reported. True aphemia is relatively rare and the described case affords the opportunity of emphasizing that a relatively rare disorder of language as aphemia might also occur with true epileptic attacks.

The importance of the highest normokalemia in the treatment of early hepatic encephalopathy.

An inverse relation is known to link blood potassium with renal synthesis and the release of ammonia. Given the liability of hyperammonemia for precipitating hepatic encephalopathy (HE), 28 patients affected by stage I HE were equally divided into two groups and maintained up to their death at the highest (5.4-5.5 mEq/l) or the lowest (3.5-3.6 mEq/l) normokalemia levels. When compared with the lowest normokalemia group, the highest one showed an early, albeit transient, improvement in the mental state (as assessed by both EEG and psychiatric investigations) and to a lesser extent in hepatic functions (as assessed by the variations in serum bilirubin, GPT, GGT and plasma prothrombin time). In the highest normokalemia group the survival was also prolonged. The cause of this improvement may be related to the induced decrease in blood pH, the consequent depression of renal ammoniagenesis and the rise in the arterial and urine NH+4/NH3 ratios. These factors reduce the entry of ammonia into the cells and enhance the urinary excretion of this metabolite, respectively.

Progression of optic neuritis to multiple sclerosis: a prospective study in an Italian population.

We prospectively studied 40 patients with uncomplicated optic neuritis (ON) to determine the risk of subsequent multiple sclerosis (MS). All patients were followed for at least 12 years. Ten patients (25%) developed MS. Seven of these 10 patients developed MS within 2 years. Both sexes were at high risk if ON occurred between the ages of 21 and 40. There was an overall increased risk of MS with recurrent ON. The course of MS appeared to be fairly benign during the period of observation.

Epidemiologic approach to Huntington's disease in northern Italy (Ferrara area).

As part of a multidisciplinary study program, an epidemiological descriptive survey was carried out in the province of Ferrara, northern Italy. The temporal trend of Huntington chorea (HC) in the last century (1871-1987) was determined in the study area, and the patients and subjects at risk were identified with the aim of creating genetic advisory councils offering preventive interventions to eliminate the disease. The final study population consisted of 10 unrelated families with HC and 47 HC cases who lived in the province of Ferrara in the survey period. The estimate of the prevalence rate varied over the time period considered between 3.09 and 0.36 cases per 100,000 inhabitants. The temporal trend was characterized by increasing and decreasing phases, reflecting the incidence (varying between 0.20 and 0.00 annual cases per 100,000 inhabitants) and mortality (varying between 0.21 and 0.00 annual deaths per 100,000 inhabitants) rates. This trend shows that the frequency of HC in the study area was not stable. It was characterized by a cyclic course with a period of about 50 years. In the recent decades of the study, the incidence and the prevalence rates showed a relative increase. Thus, HC persists in the Ferrara population despite a greater public awareness and the recent lower birth rate. A new peak of prevalence is likely in the near future.

[A rare and little-known method of elaborating the loss of the object in melancholia]. / Sur une rare et peu connue modalité d'élaboration de la perte de l'Objet dans la mélancolie.

The author presents a fully detailed report of the observation of two economically secure young women who were caught shop-lifting in a large department store; one woman was apprehended while suffering from depression, the other was caught just prior to the onset of a depressive episode. It is hoped that the details referred here may contribute to the insufficient data published on this subject up to the present. It is important to note that the thefts committed by these patients show the typical characteristics of "impulsive behavior". Furthermore, the impulse to steal may appear in various psychiatric conditions. Therefore, the author first considered the hypothesis that these thefts may have been computed during an acute dysthymic psychosis. Epilepsy was ruled out in both cases based on anamnestic data, electroencephalographic findings and, even after the administration of a pharmacological trigger, no epileptic symptoms appeared. These negative results prompted the author to propose a psychosemeiogenetic rapport between the depressive conditions and the thefts. Considering basic information regarding the psychodynamic of impulsive behavior in general, and that manifested in the course of melancholia in particular, the author confronts the problem of the psychosemeiogenesis of the impulsive to steal in the depressed patient. In order to understand this problem, the author proposes several interpretative hypotheses (equivalence of suicide or "parasuicide" according to Deshaies; attempt to give a concrete reason for the "feeling of guilt"; conduct to symbolically compensate the loss of the "object"). Following an in-depth, critical discussion, the author concludes that the "parasuicide" hypothesis is most accurate in the case of the first patient, whereas "compensation" more adequately explains the second. In conclusion, the interest that these cases present in the forensic medical sphere is also brought to light.

Life-threatening hyponatremia caused by vinblastine.

Some days after the administration of a third bolus of ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) a patient affected by immunoblastic lymphoma underwent a neurotoxic crisis. The episode lasted 1 week and was followed by a dramatic fall in plasma sodium (104 mEq I-1), associated with a proportionally lesser decrease in plasma chloride and phosphate. Despite the lowest plasma osmolality, detectable levels of circulating ADH were present. After 36 h the hyponatremic episode improved after the infusion of hypertonic sodium chloride. Nevertheless the patient lapsed into a hypotonic coma. The urinary concentrations of the main tubular enzymes (gamma-glutamyltranspeptidase, N-acetyl-glucosaminidase, alpha-glucosidase) proved very high and successively decreased slowly. The most likely cause of such hyponatremic episode is vinblastine. The drug acted through: (a) an already known inappropriate release of ADH, and (b) a hitherto unreported tubular lesion, which impaired the reabsorption of sodium and other coupled solutes.

[Evoked potentials in idiopathic optic neuritis. Possibilities of early diagnosis of demyelinating disease]. / I potenziali evocati nelle neuriti ottiche idiopatiche. Possibilità di diagnosi precoce di malattia demielinizzante.

The AA. examined 36 uncomplicated ON (Optic neuritis) observed from 1-1-1970 to 31-12-1980. An ophthalmological, neurological, and electrophysiological test (VEP, SEP, BAER) is made on this patients from 1-1-1986 to 31-3-1986. The importance of the electrophysiological examination is evaluated to recognize subclinical Multiple Sclerosis.

[Enzyme induction and anticonvulsants]. / Enzyminduktion und Antiepileptika.

Serum gamma-GT-activity was significantly (0.01 greater than P greater than 0.001) elevated in 18 of 30 epileptics receiving carbamazepine (CBZ), i.e. in 60%. The incidence of abnormal gamma-GT-levels in the control group (25 neurotic patients taking minor tranquilizers) was lower (12%). The possible relationships between the enzyme induction or chronic hepatic toxicity and the elevation of serum gamma-GT-activity in epileptics taking CBZ are discussed.

Myasthenia gravis and recurrent retrobulbar optic neuritis: an unusual combination of diseases.

A case is presented that illustrates the unusual concurrence of both myasthenia gravis and recurrent retrobulbar optic neuritis. The association of optic neuritis with myasthenia gravis might support the idea that there is a disorder of immune function in optic neuritis.

[Retrobulbar optic neuritis as an initial symptom of multiple sclerosis. Results of a prospective study]. / La neurite ottica retrobulbare come sintomo d'esordio della sclerosi multipla. Risultati di uno studio prospettico.

40 patients with idiopathic optic neuritis (ON) between 1st January 1967 and 31st December 1977 have been checked again from oculistic and neurological point of view between 1st January and 31st March 1983. 10 of 40 examined patients (25%) are found suffering from multiple sclerosis (MS) at the time of check up. Our research points out that idiopathic ON evolutive risk towards MS is greater in females, in people between 20 and 40 years old, in people with one or more relapses as well as in people with neurological microsymptoms at the time of ON.

[Family with progressive myelopathic muscular atrophy with proximal distribution and onset in adulthood]. / Studio di una famiglia con atrofia muscolare progressiva mielopatica a distribuzione prossimale ed esordio nell'età adulta.

The Authors present two brothers suffering from proximal progressive muscular atrophy arising in adulthood and describe its clinical, bioptic and electromyographic characteristics. Electromyographic and bioptic examinations demonstrate the neurogenic nature of the amyotrophy and localize the causal lesion at the level of the anterior horns of the spinal medulla. With regard to the differential diagnosis, the nosographic position of the disorder in question is described, and the hypothesis advanced that it may represents the late onset variety of Wohlfart-Kugelberg-Welander disease.

[Induction of liver microsomal enzymes in epileptics under treatment with carbamazepine]. / Induzione degli enzimi microsomiali epatici in epilettici in trattamento con carbamazepina.

Serum gamma-GT activity was significantly (0.01 greater than P greater than 0.001) elevated in 18 out of 30 epileptics receiving carbamazepine (CBZ), i.e. in 60%. The incidence of abnormal gamma-GT levels in the control group (25 neurotic patients taking minor tranquilizers) was lower (12%). The possible relationships between the enzyme induction or chronic hepatic toxicity and the elevation of serum gamma-GT activity in epileptics taking CBZ are discussed.