Maurizio De Negri

Treatment of electrical status epilepticus by short diazepam (DZP) cycles after DZP rectal bolus test

The effects of rapid rectal diazepam introduction (DZP test) were investigated in 43 patients (age range 5 months-14 years) with electrical status epilepticus (ESE) undergoing EEG monitoring. A remission of the paroxysmal activity was obtained in 58% of cases, a negative response in 42%, particularly in hypsarrhythmic patterns. DZP test responders were aged over 12 months with organized paroxysmal EEG patterns, in particular with ESE during sleep (ESES). The patients who responded to the DZP test underwent short cycles (3-4 weeks) of relatively high dosage DZP (0.5-0.75 mg/kg). The response to treatment was positive in 64%, particularly in ESES conditions. 56% of responders to the DZP test but not to DZP therapy (five out of nine patients) presented a significant mental retardation; maturational factors were also likely to be present.

Treatment of Status Epilepticus in Children

Status epilepticus (SE) is a condition characterised by frequent and prolonged epileptic seizures which frequently develop in the immature brain. Fever, metabolic disorders and subtherapeutic concentrations of antiepileptic drugs are the most common factors precipitating SE in children.Progressive neuronal damage occurs if convulsive SE persists for more than 30 minutes, with neurological, epileptic and cognitive sequelae. Unfortunately, the immature brain is more predisposed to SE and its sequelae than the mature brain.SE may be categorised as convulsive, nonconvulsive or neonatal according to its responsiveness to antiepileptic drugs. Regardless of category, the main objective in the treatment of SE is to abort the seizures and treat the inciting condition.Treatment includes: (i) monitoring of hydration, electrolyte balance, and cardiocirculatory and pulmonary functions; and (ii) rapid intravenous administration of specific antiepileptic drugs.Benzodiazepines (usually diazepam, lorazepam or midazolam) are the most effective agents for the initial treatment of convulsive and nonconvulsive SE. In particular, midazolam infusion is an effective and well tolerated therapeutic approach for the management of childhood SE, including refractory SE. Phenytoin remains an excellent agent because of its long duration of action, but it is not active in nonconvulsive SE. Fosphenytoin, a phenytoin prodrug, represents a significant advance in the treatment of children with convulsive SE. Intravenous phenytoin and intramuscular phenobarbital (phenobarbitone) are generally used in neonatal SE; other agents are rarely used.

SPECT and epilepsy with continuous spike waves during slow-wave sleep

Ten cases of epilepsy with continuous spike waves in slow-wave sleep (CSWS) were evaluated using single photon emission computed tomography (SPECT); in eight patients the EEG paroxysmal abnormalities showed a predominant localization. SPECT carried out using99mTc-HMPAO allows study of cerebral blood flow (CBF); the examination was performed during phases of drowsiness and the results compared to the EEG data. In four cases SPECT revealed areas of low CBF in sites corresponding to those of the prevalent EEG discharges; in two cases the areas of hypoperfusion did not correspond to those indicated by the EEG; lastly, in four cases SPECT results were negative. The areas of hypoperfusion were predominantly located in the frontal, temporal, and parietal regions. Furthermore, the percentage of positive SPECT results was significantly higher (five cases out of six) in the group in which the CSWS phase was prolonged for at least 1 year, compared to the group in which this phase lasted less than 1 year. Thus, in this type of epilepsy, SPECT reveals focal cortical areas of decreased CBF which correlated generally to the predominant sites of EEG abnormalities. A longer duration of the CSWS phase seems to be associated with a more significant cortical disorder, documented by the presence of areas of hypoperfusion.

Occlusion of unilateral carotid artery in Down syndrome

The association between moyamoya phenomena and Down syndrome (DS) is reported in the literature. This paper reports a case of DS, which at age 9 presented right hemiparesis, secondary to the occlusion of the left internal carotid artery; cerebral angiography (CAG) showed a collateral circulation that mimicks the moyamoya phenomenon. Clinical recovery was almost complete; a second CAG after 15 months showed a persistent occlusion of the left internal carotid artery and an opacification of the left middle cerebral artery from abnormal vessels; but the collateral circulation is not enhanced. This case proves that in DS cerebrovascular occlusions may present moyamoya-like phenomena. These differ however from the true moyamoya disease in a number of aspects: the arterial occlusion is unilateral, the evolution is favorable and revascularization does not occur through the peculiar abnormal vessels of the moyamoya syndrome.

Electrical status epilepticus in childhood: treatment with short cycles of high dosage benzodiazepine (preliminary note)

A treatment of electrical status epilepticus during childhood with relatively high doses of benzodiazepines (BZ) in short cycles (3 weeks) is proposed for subjects shown to be responsive to BZs (diazepam bolus test: DZP test). This treatment gave positive results in 7 out of 8 patients responsive to the DZP test (87%).

Some critical notes about “the epilepsy-aphasia syndrome” in children

The clinical entity epilepsy-aphasia in children begins from an analytical study of cases published in the literature and from the study of eight cases personally observed. This condition is not an univocal syndrome but it must be differentiated into at least three different conditions even if very often, they are superimposed: 1) a first condition in which the aphasia is critical and transient; 2) a second condition where it acts rather as a serious congenital dysphasia, and 3) a third condition in which it acts as an acquired aphasia, in the strict sense, even though very atypical. The relations between aphasia and epilepsy are discussed especially for what concerns the dynamic of the diffusive forms of epilepsy in the child even in their psychiatric aspects. Both the pharmacological and reeducative problems of therapy are discussed.

Generalized epilepsy in childhood: some notes from a developmental perspective.

Efforts to establish more detailed classification of childhood epilepsy have increased our knowledge in this field, but have also led to a tendency of excessive subdivision, especially in generalized forms. This paper emphasizes the need for the reconsideration of a number of basic factors that favour a more unitary physiopathogenetic and clinical interpretation: 1) the electroclinical pattern is age-related; 2) the electroclinical paroxysmal expressiveness is greatest in childhood; 3) two types of manifestations can be distinguished in the process of spread and generalization (a benzodiazepine-sensitive complex associated with a more regular and organized EEG pattern, and a corticotropin-sensitive complex associated with an irregular, unorganized EEG pattern).

Hyperkinetic behaviour, attention deficit disorder, conduct disorder and instabilitépsychomotrice: identity, analogies, and misunderstandings commentary to Gordon's paper (Brain Dev 1994;15: 169-72)

Abstract ‘Hyperkinetic behaviour’, ‘attention deficit disorder’ and ‘conduct disorder’ with antisocial aspects or delinquency are concepts frequently superimposed. They can be traced back to the classic nosological entity, ‘instabilitepsychomotrice’, still used in French psychiatric nosography. In this paper, the different components of the condition are analyzed (emotional, attentional, posturo-motoic and behavioural) both in normal subjects and in cases of mild encephalopathies, and for different age groups. A criticism is advanced towards the undifferentiated approach to the problem in the most current literature.

Phenomenological Perspectives in Developmental Psychiatry

Phenomenological psychiatry has long been considered an important part of general psychiatry, and yet its contributions to the literature focusing directly on childhood and adolescence have been few, and those few, though on occasion of considerable weight, have been neither systematic nor have they made direct reference to the primary sources of phenomenological research.

Computed EEG topography (CET) and childhood epilepsy: Two years experience

The statistical data obtained with the use of computed EEG topography (CET) in a diagnostic study on 65 epileptic children are reported briefly. The CET technique has proved to be particularly useful in establishing the focal origin of paroxystic activity in a high percentage of cases of generalized epilepsies presumed to be primary (33%) or secondary (88%), and for localizing areas of brain function abnormality not detected by other diagnostic techniques.