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The concept of idiopathic partial epilepsies in childhood is not yet accepted by all neurologists. We review the diagnostic criteria of these syndromes and stress both wake and sleep EEG characteristics. Different clinical entities have been individualized among other forms: benign partial epilepsy with centro-temporal spikes, benign epilepsy with occipital paroxysms, benign psychomotor epilepsy.
The authors used the definition of the Lennox-Gastaut syndrome (LGS) adopted by the Commission on Classification and Terminology of the International League against Epilepsy. Three hundred and thirty eight patients with childhood LGS have been followed until adulthood; 62.4 p. 100 had an unfavourable outcome. All began their LGS in childhood or infancy (between the ages of 1 and 8.80 p. 100 before the age of 4). In 46.9 p. 100 of cases, complete LGS persisted in the adult. Most cases were apparently primitive. In 15.6 p. 100 of cases, generally symptomatic, the LGS disappeared but an often severe, mostly multifocal epilepsy persisted. 37.6 p. 100 of cases had a more or less favourable outcome. In these cases, the LGS had often begun later (between 7 and 11 years of age) and lasted for less (between 2 and 6 years). Some patients (20.4 p. 100) still had fairly rare partial seizures and neurological or psychiatric symptoms. These cases were mostly symptomatic, 17.4 p. 100 of cases seemed to have been nearly completely cured. These were cases where the LGS had followed another type of epilepsy, mostly of the idiopathic generalized type. Fourty four patients with no prior epilepsy presented with LGS appearing between the ages of 13 and 23. They were divided into 2 groups: in 31 patients, the LGS was associated with focal signs. In all these cases, the evolution was for the worse, whatever the age at onset. In 13 cases, there were no focal signs. In these, there was a different prognosis between those with an onset between the ages of 13 and 15, where the entire syndrome persisted and the evolution is for the worse, and those with an onset after the age of 15, where the outcome was better.
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Thirty patients with the Lennox-Gastaut syndrome presenting one or more status epilepticus (the first before age 15) were studied. Triggering factors, semiology, duration, severity and EEG features were considered. A comparison was made between this group of patients and another group having the Lennox-Gastaut syndrome but without status. No significant difference appears in the long-term prognosis, even when status were prolonged. Only three patients died during a status. For this reason the authors recommend prudence and avoidance of very strong treatment. In their experience, intravenous diazepines, intravenous phenytoin and intramuscular ACTH were the most effective drugs.
In order to confirm, or to deny, the diagnosis of epilepsy a sleep EEG recording as a supplement to the routine EEG investigation has been performed in 229 subjects in 1980 at Saint Paul Center. In 93% of the cases, spontaneous afternoon napping, and in 7% spontaneous nocturnal sleep was studied. Sleep EEG was analysed and correlated to the clinically suspected type of epilepsy. The occurrence of focal or generalized EEG abnormalities, clearly related to the type of epilepsy, confirmed anamnestic and clinical findings in 54% of the cases and gave a more accurate diagnosis in 20% of the cases. Sleep recordings were necessary for the diagnosis of the type of epilepsy in 2% of the cases.
Lombroso and Fejerman (1983) described a syndrome which shares with West syndrome the clinical features of flexion spasm with onset in early infancy. However the syndrome differs from West syndrome in the absence of mental and psycho-motor involvement and having a normal EEG during wakefulness and sleep. We report four cases of these benign spasms of early infancy with polygraphic recording of the spasms providing the following information: the spasms are clinically similar to those often observed in West syndrome; namely they are characterized by a short (2-4 sec.) tonic contraction, there are no significant changes of the EEG concomitant to the spasm, series of spasms can occur not only during the daytime but also during sleep and immediately after awakening. Differential diagnosis is discussed with West syndrome and benign myoclonic epilepsy in infancy and with non-epileptic syndromes. The authors propose to name this syndrome: Benign Non-Epileptic Infantile Spasms.
The intravenous administration of the chemotactic and secretagogue peptide N-formyl-L-methionyl-L-leucyl-L-phenylalanine (FMLP; 0.3-30 micrograms kg-1) to the guinea-pig induces bronchoconstriction and dose-dependent leukopenia accompanied by mild thrombocytopenia. No electron microscopic evidence of platelet aggregation in lungs or significant accumulation of 111In-labelled platelets in the thoracic region at the height of bronchoconstriction was noted. Bronchoconstriction and leukopenia induced by FMLP were not affected by prostacyclin, by platelet depletion, by the platelet-activating factor (Paf-acether) antagonist BN 52021 or by the histamine H1-antagonist mepyramine. Bronchoconstriction, but not leukopenia, was inhibited by aspirin, whereas the peptido-leukotriene antagonist compound FPL 55712 and the cyclo-oxygenase lipoxygenase inhibitor indomethacin reduced bronchoconstriction to a limited extent only. The mixed cyclo-oxygenase/lipoxygenase inhibitor compound BW 755C was very effective in blocking bronchoconstriction by the highest dose of FMLP used, but failed to interfere with leukopenia. FMLP-induced dose-dependent contraction of parenchymal lung strips was accompanied by the formation of immuno-reactive thromboxane B2 in amounts markedly less than those formed from exogenous arachidonic acid at concentrations equieffective in inducing contractions. FMLP-induced contractions of the guinea-pig lung strip were not modified by mepyramine nor by FPL 55712. They were reduced by indomethacin and aspirin and an even greater reduction was obtained with aspirin used in combination with FPL 55712. BW 755C suppressed the effects of all the concentrations of FMLP tested, whereas tert-butyloxy-carbonyl-L-methionyl-L-leucyl-L-phenylalanine, a chemical analogue of FMLP, displaced the concentration-response curve to the right, without reducing the maximal contraction obtained. The present results indicate that: (a) bronchoconstriction by FMLP is not due to platelet activation, to cyclo-oxygenase-dependent mechanisms or to peptido-leukotriene formation. The inhibitory effect of aspirin and BW 755C involves a property other than cyclo-oxygenase inhibition, which is not shared by indomethacin. (b) The contractile effects of FMLP on parenchymal lung strips follow an interaction with specific receptor sites, as shown by the effectiveness of tert-butyloxy-carbonyl-L-methionyl-L-leucyl-L-phenylalanine, and involves the combined effects of cyclo-oxygenase and lipoxygenase metabolites.
The external counting method was used to simultaneously measure the kinetics of intravenously injected polymorphonuclear cells labelled with Technetium 99m, in the lungs, liver and blood of rats. When radiolabelled polymorphonuclear leukocytes (PMN) are injected into animals, a large part is taken up by the various organs. This uptake may result from intravascular aggregation, capillary blocking (in which case the transit of the PMN is slowed down), vascular margination or, finally, from tissue infiltration. A rapid increase in PMN was observed initially in the lungs (first seconds), followed by a progressive decrease (t1/2 = 50 min). At the same time, PMN in the liver increased and that in the blood slowly decreased. The kinetics of distribution in the lungs, liver and blood were not modified when the PMN were injected through the aorta, via a carotid catheter, instead of intravenously. Since there was no shunt, vascular resistance was not the cause of the slow transit of PMN through the organs, and we favour the hypothesis that vascular margination of PMN is responsible for their kinetics. When heat damaged PMN were injected, the kinetics of their distribution in the lung, liver and in the blood were clearly altered.
An 11-month-old infant, full-term born after normal pregnancy and delivery had a generalized short tonic-clonic seizure at the 7th hour of life. These seizures were repeated on the 7th, 8th, 9th and 22nd days, and they persisted like bilateral myoclonic fits once a week. The EEG recordings showed asynchronous spikes and spike-waves on the vertex and both frontal areas. The seizure's recording showed a brief burst of bilateral spike-waves. The psychomotor development was retarded but progressive. At 10 months 9 days the patient presented a status without any impairment to all therapeutic trials. Death occurred after 12 days of status. The unexpected severe evolution of this epilepsy with unknown etiology, which did not evoke any metabolic or degenerative diseases, is discussed.
A 15 year-old North-African female showed typical symptoms and evolution of Progressive Myoclonus Epilepsy of the Unverricht type. Pathological examination failed to show either inclusion bodies or any other storage material. The only relevant findings included degenerative changes in the inferior olives and, to a lesser extent, in the cerebellar cortex. The site of lesions was remarkable: in the inferior olives, lesions were bilaterally and symmetrically restricted to the external angles (lateral lamellae); in the cerebellum, loss of Purkinje cells and ascending fibres of the molecular layer was prominent in the lateralmost part of the hemispheres (semilunar lobules). Such a topography implies a system disorder involving the olivo-cerebellar pathway, particularly in that part which projects to the neocerebellum. Twelve other clinico-pathological cases of progressive myoclonus epilepsy of the degenerative group are reviewed. It is suggested that, here again, lesions--although more diffuse--may be related to a primarily olivo-cerebellar involvement.
A prospective study of abdominal and chest films of 212 babies without symptoms of intra-abdominal disease, aged 1 day-12 weeks, showed that the bubbly "fecal pattern" normally seen in older children is rare in young premature babies in the first two weeks of life. When it is seen, it may represent intramural air bubbles and signal the presence of necrotizing enterocolitis.
This is a case report of an infant born with nasal hypoplasia, stippling of epiphyses, and toe deformities. This embryopathy is due to maternal ingestion of Warfarin during pregnancy. Other defects including ophthalmologic and neurologic abnormalities also occur, but the nasal malformation is the only constant clinical feature.
The results of the surgical treatment of epilepsy were studied in 44 patients 10 or more years after operation. Thirty-seven patients underwent operation only once; these patients were observed 11-26 years postoperatively. Seven patients had a recurrence within 5 years after operation and required a second operation; these patients were observed 11-17 years after the second operation. Recovery persisted for 15-27 years in 32 patients. There was a recurrence in 12 cases 11-19 years after operation. With one exception, these recurrences were satisfactorily treated medically.
The early diagnosis of Lafora's disease is often difficult, from primary generalized epilepsy and other progressive myoclonic epilepsies. The authors emphasize the diagnostic interest of paroxysmal visual manifestations, reported in only 25 per cent of the cases in the literature, and present in 17 of their 31 personal cases. These visual manifestations can be elementary or complex. The elementary ones appear often early, before other symptoms of the disease. Two demonstrative cases are reported. In one of them an occipital seizure was recorded, demonstrating the epileptic critical nature of these elementary visual manifestations. They have a high diagnostic value since they do not occur neither in primary generalized epilepsy (Grand Mal type), nor in the other progressive myoclonic epilepsies. In the 2 reported cases, skin biopsy, as proposed by Carpenter and Karpati, demonstrated the specific storage in the sweat ducts and glands. The interest of this skin biopsy is underlined, its positivity seeming both constant and specific in Lafora's disease.
Among 26 patients suffering from Epilepsia Partialis Continua, 2 major groups were observed. The first, resulting from a fixed lesion of the rolandic area, showed electro-clinical correlation of seizures; the latter disappeared during sleep; clinical and radiological follow-up failed to disclose any worsening of the cerebral lesion. The second group was characterized by progressive mental and motor deterioration, lack of electro-clinical correlation of fits, persistence of the latter during sleep and frank increase of cerebral atrophy observed on serial neuroradiological examinations. This easily recognized group seems to result from a progressive inflammatory disease of unknown cause.
Both our personal experience and the findings of others indicate that the benzodiazepines (a) are the drugs of first choice for control of status epilepticus occurring in patients with primary generalized epilepsy (90%-100% effective) or control of hemiclonic convulsions in children without brain lesions, (b) are effective in approximately 60% of cases of status epilepticus occurring in partial epilepsy, (c) are effective in only 15% to 59% of cases of tonic status or various types of absence status occurring in secondary generalized epilepsy (but no other drug is more effective), (d) are helpful in status epilepticus occurring in nonepileptic patients if there is no overt brain lesion (but give only temporary relief when status is the result of a severe organic brain lesion), and (e) are generally safe drugs.
The authors describe the electroclinical and evolutive aspects of 4 cases (including 2 brothers) of myoclonic epileptic encephalopathy beginning between 2 days and 10 weeks of life. From the onset of myoclonic jerks, polymorphous fits (partial seizures, tonic seizures) and multifocal electrical abnormalities are associated. Repeated spasms and 'suppression-burst' patterns appear later. The neurological status deteriorates progressively, leading within a few months to decerebration posture with opisthotonus. In spite of thorough neuroradiological, biochemical, cytological to metabolic investigations, etiology remains unknown. However, the electroclinical and evolutive patterns are similar to that of metabolic diseases, especially non-ketotic hyperglycemia. The authors discuss the relations between their observations and those in the literature and the nosological problems of this particular epileptic encephalopathy of infancy.
Twenty children (15 males and 5 females) suffering from a particular type of myoclonic epilepsy were submitted to a longitudinal study. All children were neurologically normal. Familial antecedents existed for epilepsy in 25% of the cases (5/20) and for febrile convulsions in 15% (3/20). The first fit appeared with fever at the mean age of 6 months in all cases but one of clonic type. Frequent similar febrile or afebrile clonic seizures recurred in all subjects before the age of 12 months. At this time the EEG was normal in 14 cases and brief discharges of generalized spike-waves during ILS or during sleep were present in 6 cases only. Later, frequent non-febrile clonic unilateral or generalized fits, frequent atypical 'absences' often accompanied by jerks, high photosensitivity and non-epileptic erratic myoclonias appear. Nevertheless, atomic and/or tonic seizures did not appear. The evolution is characterized by the persistence of fits and the appearance of severe language disorder and light cerebellar and pyramidal signs. The authors present their results and discuss the nosological problems of this severe infant myoclonic epilepsy.