ABSTRACT
Three children with circumscribed unilateral pial angiomatosis had both generalised and partial seizures associated with bilateral synchronous spike-wave complexes. Dramatic control of the seizures was obtained by surgical removal restricted to the angiomas and underlying cortex. There was recurrence of seizures in one patient from whom only one of two angiomatous areas was removed but not in the two patients whose excision was total. These cases indicate that secondary bilateral synchrony can occur with lesions of the posterior and external parts of one hemisphere. Surgical removal of a definable lesion, without intracranial recording, can help patients with intractable epilepsy due to unilateral pial angiomatosis, even in the presence of wide diffusion of clinical and electroencephalographic abnormalities.
Subject(s)
Angiomatosis/surgery , Electroencephalography , Epilepsies, Partial/surgery , Meningeal Neoplasms/surgery , Pia Mater/surgery , Adolescent , Angiomatosis/physiopathology , Calcinosis/surgery , Cerebral Cortex/physiopathology , Cerebral Cortex/surgery , Child , Child, Preschool , Epilepsies, Partial/physiopathology , Evoked Potentials , Female , Humans , Meningeal Neoplasms/physiopathology , Postoperative Complications/physiopathologyABSTRACT
A patient with intention and action myoclonus, epilepsy, ataxia, and mental deterioration in association with ragged-red fibers in striated muscle is described. This patient demonstrated a unique form of erratic myoclonus with continuous EEG spike activity during eye closure. Both the myoclonus and the EEG spiking disappeared on opening the eyes. A defect in the activity of complex I in the respiratory chain was demonstrated.
Subject(s)
Epilepsies, Myoclonic/pathology , Mitochondria, Muscle/ultrastructure , Adolescent , Ataxia/physiopathology , Electroencephalography , Epilepsies, Myoclonic/physiopathology , Female , Humans , Intellectual Disability/physiopathology , Muscular Diseases/physiopathology , Sarcolemma/ultrastructure , SyndromeABSTRACT
Seven children with an unusual epileptic syndrome are reported. The main clinical features in each patient included onset between 2 1/2 and six years of age; the occurrence of several types of seizure, especially partial motor fits, atypical absences and myo-atonic seizures; and the persistence of normal neurological and mental function throughout the course. The EEG picture was characterized by a striking contrast between waking records, which usually displayed focal paroxysms, and sleep tracings which showed an almost continuous, diffuse, slow spike-wave activity. Although the electroclinical features suggested the diagnosis of Lennox-Gastaut syndrome or myoclonic epilepsy, the seizures remitted spontaneously in the five oldest patients and may well do so in the two youngest ones. The authors discuss the clinical and electroencephalographic features that permit these cases of atypical benign partial epilepsy to be distinguished from the more severe myo-atonic epileptic syndromes of childhood.
Subject(s)
Electroencephalography , Epilepsies, Partial/diagnosis , Child , Child, Preschool , Diagnosis, Differential , Epilepsies, Myoclonic/diagnosis , Epilepsy, Absence , Female , Humans , Male , SleepABSTRACT
The clinical manifestations and the classification of the epilepsies of infants are briefly reviewed. 207 cases were classified status epilepticus (51 cases), brief partial seizures (57 cases), and brief generalized seizures (99 cases). Lesional factors, especially brain malformations, play a major role in the aetiology, but the importance of genetic factors is suggested by the presence of a positive family history of convulsive disorders in 25% of the cases. The mental and neurological prognosis is grave, as only 22% of the patients developed normally. The prognosis varies with the aetiology of the epilepsies and appears more favorable when familial antecedents of convulsive disorders are present.
Subject(s)
Spasms, Infantile/classification , Electroencephalography , Follow-Up Studies , Humans , Infant , Infant, Newborn , Prognosis , Spasms, Infantile/etiology , Spasms, Infantile/geneticsSubject(s)
Seizures, Febrile/diagnosis , Child, Preschool , Female , Humans , Infant , Male , Prognosis , Seizures, Febrile/etiology , Seizures, Febrile/prevention & controlSubject(s)
Intellectual Disability/etiology , Seizures, Febrile/complications , Seizures/complications , Age Factors , Child , Humans , InfantABSTRACT
Mortality and neurological and mental outcome were studied in infants 28 days to 1 year of age with afebrile seizures not due to an acute postnatal injury. Cases were divided into four seizure types: infantile spasms: status epilepticus; and "others" (patients without spasms or status), generalized and partial. Mortality was studied in 334 cases, mental and neurological prognosis in 313 infants followed 1 year or more. Globally the prognosis was very poor even outside cases of infantile spasms. Mortality was higher and mental and neurological sequelae were more common in symptomatic than in cryptogenic cases. The highest mortality and greatest number of neurological defects were in status epilepticus and in "others" partial groups. Severely retarded subjects were more common in infantile spasms and "others" partial. The proportion of mentally normal patients, however, was no different according to ictal type. Mental and neurological prognosis was less unfavorable when the first seizure occurred at or over 6 months. A family history of epilepsy or febrile convulsions (21% of the cases in the whole series) was more common in the "others" subgroups, especially in the cryptogenic "others" (42%). The less unfavorable outcome obtained in cryptogenic "others" generalized with a positive family history.
Subject(s)
Intellectual Disability/etiology , Nervous System Diseases/etiology , Seizures/mortality , Age Factors , Birth Injuries/complications , Child , Child, Preschool , Follow-Up Studies , Humans , Infant , Infant, Newborn , Infant, Newborn, Diseases/mortality , Prognosis , Seizures/etiology , Seizures/genetics , Spasms, Infantile/mortality , Status Epilepticus/mortalitySubject(s)
Seizures/etiology , Age Factors , Birth Injuries/complications , Birth Weight , Brain/abnormalities , Epilepsy/genetics , Humans , Infant , Intellectual Disability/complications , Prognosis , Seizures/genetics , Seizures/mortality , Sex Factors , Spasms, Infantile/etiology , Status Epilepticus/etiologyABSTRACT
The relationships of certain etiological factors (sex, age, family history of febrile convulsions or epilepsy, term, birth weight, prenatal or perinatal anomalies, temperature, cause of fever) to the duration and localization of the first febrile convulsion (FC) were studied in 402 patients. In patients with prolonged seizures (over 30 min), the mean age was younger, the proportion of girls, common infectious diseases of childhood, and immunization was higher and that of respiratory infections lower than in patients with brief convulsions. In patients with unilateral seizures, the proportion of positive family histories and respiratory infections was lower and that of common infectious diseases of childhood and of immunization was higher than in patients with bilateral convulsions. The association of each of these etiological factors with the duration or lateralization of the first FC was independent of the others.
Subject(s)
Fever/complications , Functional Laterality , Seizures/etiology , Age Factors , Child, Preschool , Communicable Diseases/complications , Female , Humans , Immunization/adverse effects , Infant , Male , Seizures/epidemiology , Seizures/physiopathology , Sex Factors , Time FactorsABSTRACT
Twenty cases of acute neurological complications occuring within 7 days of pertussis immunization are reported. Convulsions were present in every case and status epilepticus was observed in five infants. In only 4 cases were neurological or epileptic sequelae lacking. The clustering of neurological complications in the 24 hours following immunization is not consistent with the hypothesis of a mere temporal coincidence. However, the mechanism and incidence of post-immunization encephalopathies remains obscure and epidemiological studies are in order.
