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R Caraballo

Publications and source records attributed to R Caraballo.

35 records · Page 2Linked to original sources
PubMed1997

[Epilepsies during the first year of life].

OBJECTIVE: An important number of epileptic patients who began with epileptic seizures during the first year of life have not just been well classified. The objective is to identify the different types of epilepsies and epileptic seizures which begin during the first year of life, according to the last classification of epilepsy and epileptic syndromes of 1989. MATERIAL AND METHODS: We have studied 471 patients who consulted to our service in the last 5 years, with epileptic seizures during the first year of life. We excluded neonatal seizures and febrile convulsions. RESULTS: 1. Partial epilepsy: a) idiopathic: 12 (2.5%); b) symptomatic: 130 (28%); c) cryptogenic: 25 (5%). 2. Generalized epilepsy: a) idiopathic: benign myoclonic epilepsy in infancy, 6 (1%); b) cryptogenic-symptomatic: symptomatic West syndrome, 155 (33%); cryptogenic West syndrome, 65 (14%); cryptogenic myoclonic epilepsy, 6 (1%); early infantile epileptic encephalopathy, 8 (1.6%); early myoclonic encephalopathy, 4 (0.8%); other symptomatic generalized epilepsies without specific aetiology, 14 (3%). 3. Epilepsies and syndromes undetermined as to whether they are focal or generalized: a) severe myoclonic epilepsy in infancy, 15 (3%); symptomatic epilepsy with multifocal and independent spikes, 3 (0.6%); other undetermined epilepsies not defined above, 3 (0.6%). 4. Special syndromes: occasional convulsions, 15 (3%). CONCLUSIONS: West syndrome is the commonest type of epilepsy during the first year of life, particularly symptomatic West syndrome. Symptomatic partial epilepsies are in the second place in frequency. We identified 12 patients with idiopathic partial epilepsy, who full-filled clinical and EEG characteristics of a recently described epilepsy: benign infantile familial convulsions. The study showed the predominance of symptomatic epilepsy, generalized (represented by West syndrome) and partials. Finally 6% of the patients have a generalized epilepsy, cryptogenic or symptomatic, not defined in the classification.

Epilepsy↗
PubMed1994

Acquired epileptic aphasia: neuropsychologic follow-up of 12 patients.

A study of specific neuropsychologic, neurolinguistic, and behavioral features of acquired epileptic aphasia or Landau-Kleffner syndrome was conducted in a group of 12 patients followed-up for 2-15 years (mean: 8 yr). Seventy-five percent had exhibited some language disturbance prior to acquired epileptic aphasia. Even when 9 patients had normal electroencephalographic findings in the long-term course of the disease, only 3 achieved normal language. No patient with persisting electroencephalographic abnormalities recovered normal or near normal language. The need to perform detailed neurolinguistic and neuropsychologic evaluations in the work-up and follow-up of children with acquired epileptic aphasia is stressed. An adapted neuropsychologic profile battery proved to be practical and objective for the follow-up of these patients.

Agnosia↗
PubMed1991

Sleep and benign partial epilepsies of childhood: EEG and evoked potentials study.

The electroclinical picture and nosological limits of benign partial epilepsy of childhood with rolandic spikes (BERS) have been better defined by nocturnal sleep records. In all stages of sleep, there is a significant increase in frequency and amplitude of rolandic spikes (RS) without change of their morphology. Another interesting observation is the appearance of independent spike foci in sleep, or brief subclinical spike wave discharges which are limited to the state of drowsiness. More recently, other types of partial epilepsy of childhood with benign evolution have been identified: (a) partial epilepsy with induced spike representing somatosensory evoked potentials; (b) benign psychomotor epilepsy; (c) partial epilepsy with occipital spike waves. In all these forms, the sleep records are essentially similar to those in BERS and have been very helpful in the nosological identification of these forms of epilepsy. For this reason, the sleep records of these special forms are truly informative for the clinician from the diagnostic and prognostic viewpoint. On the other hand, some investigators, have pointed out that, in the initial stage of these benign forms of partial epilepsy, there may be more or less significant intellectual impairment and behavioral disorder, sometimes accompanied by frequent brief absences. From the EEG viewpoint, this condition is characterized by brief discharges of slow spike wave complexes amounting to a pattern of "electrical status epilepticus". This special electroclinical condition mimics the Lennox-Gastaut syndrome but is generally self-limited. Thus, a correct differential diagnosis is very important from the diagnostic viewpoint. There is good evidence that sleep records permit an earlier identification of these conditions and strongly contribute to a correct differential diagnosis. In the benign partial epilepsy the SEPs, during awake and sleep, morphology and latency are normal, while the N60 amplitude is increased. A group of children with benign partial epilepsy shows EEG spikes evoked by tapping, and giant N60 component. This giant component persists during sleep and is not specific for any type of benign partial epilepsy. In conclusion, the results of sleep recordings are conducive to a correct diagnosis and better definition of the nosological delineation of partial epilepsies in childhood; they also provide a better comprehension of their evolution, and thus of their prognosis. The Evoked Potentials seem be a useful tool in the study of benign partial epilepsy.

Adolescent↗
PubMed1990

Transient focal leukoencephalopathy following intraventricular methotrexate and cytarabine. A complication of the Ommaya reservoir: case report and review of the literature.

A 14-year-old boy, suffering from acute lymphoblastic leukemia with meningeal involvement, was treated with intraventricular methotrexate and cytosine arabinoside, administered via an Ommaya reservoir (OR). Three months later, right occipital headache, vomiting, and lethargy appeared. Cerebrospinal fluid specimens showed increased proteins and a right frontal slow-wave focus was evident on the EEG recording. The computed tomography scan revealed white matter hypodensity within the right frontal and rolandic regions. After injection of medium contrast, an abscesslike hyperdensity appeared, surrounding both a well-placed cannula tip and the right frontal horn of the lateral ventricle. Brain swelling and shift signs were also evident. Nine cases of focal methotrexate leukoencephalopathy have been previously reported, and in six of these there was a misplaced OR cannula tip. The focal methotrexate leukoencephalopathy seems to be related to the neurotoxicity of the drugs administered, and may also exist with a well-placed OR cannula tip. Immediate removal of the catheter may be associated with a benign evolution.

Adolescent↗
PubMedDate not supplied

Intracranial tumors and West syndrome.

Two infants, 6 and 7 months of age, are reported with both West syndrome and cerebral tumors. In both patients, initial neurologic examinations were normal and tumor diagnoses were determined through routine imaging studies. Initial response to adrenocorticotrophic hormone treatment did not differ from that observed in patients with cryptogenic West syndrome. Early treatment of West syndrome and resection of tumor, when possible, may offer successful treatment of patients with both benign brain tumors and West syndrome.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome.

We report the fourth case of partial status epilepticus (SE) in benign epilepsy of childhood with rolandic spikes (BECRS). The child suffered long-lasting attacks involving the mouth and pharynx, clinically manifest as speech arrest, sialorrhea, and drooling. Both clinical and electroencephalogram (EEG) data were compatible with the diagnosis of BECRS. Only during SE was the clinical picture similar to that observed in the operculum or Foix-Chavany-Marie syndrome. SE remission was obtained with the usual antiepileptic drug therapy (diazepam, clobazam, valproate). EEG records showed additional patterns of continuous spike-waves during slow sleep and specific inhibition and blocking of interictal centrotemporal spikes by mouth and/or tongue voluntary movements.

Brain↗
PubMedDate not supplied

[Benign partial convulsions in adolescence].

INTRODUCTION: Loiseau and Orgogozo first described benign partial convulsions of adolescence (BPCA). OBJECTIVE: To analyze the clinical and electroencephalographic characteristics of adolescent patients diagnosed as having BPCA. PATIENTS AND METHODS: We reviewed the clinical histories of 125 patients with onset of partial epilepsy between the ages of 10 and 18 years, followed-up at the J.P. Garrahan Hospital, Buenos Aires, between 1990 and 1997. Fourteen of these patients fulfilled diagnostic criteria for BPCA. We analyzed the following clinical and electroencephalographic parameters: sex, personal and family history, age of onset, semiology, distribution, duration and frequency of crises, neurological examination, neuro-images (brain CT/MR) using conventional techniques, time of follow-up and evolution. RESULTS: Ten patients were boys (71.4%) and four girls (28.6%). The follow-up period was from one to seven years (average 3.1 years). Convulsions had started at between 10 and 16 years old, average 12 years old, with a peak of maximum incidence at between 12 and 13 years old. The crises were of partial simple type in 12 patients (85.7%) and partial complex type in 2 (14.3%); nine cases had secondarily generalized tonic-clonic convulsions (64.2%). Crises were brief in 100%, in 13 cases (93%) they occurred whilst awake and in one case (7%) during sleep. CONCLUSIONS: BPCA occur in a transient condition which affects males more often than females, has a peak onset at between 12 and 13 years of age and is characterized by simple motor partial crises, frequently with secondary generalization, single or inclusters, during waking and is of a benign course. Neurological examination, EEG between crises and neuro-radiological studies were normal.

Adolescent↗
PubMedDate not supplied

[Cerebral magnetic resonance in the study of West syndrome].

INTRODUCTION AND OBJECTIVE: West's syndrome (WS) is an epileptic encephalopathy of the first year of life, associated with different aetiologies. MRI of the brain allows precise determination of the type and extent of the lesions. The aetiology must be recognised in order to establish the prognosis and a suitable therapeutic approach. The objective of this study is to analyse the aetiologies of a population of children with WS and compare the results of cases diagnosed before and after using MRI. PATIENTS AND METHODS: We analyzed the clinical histories of 448 patients fulfilling the diagnostic criteria for WS (infantile spasms and a EEG with a pattern of hypsarrhythmia), 217 in pre-MRI era (group 1) and 231 in the post-MRI era (group 2). The following parameters were analyzed: type of WS, sex, duration of follow-up, age of onset of infantile spasms and particularly the neuroradiological studies. RESULTS: Group 1: symptomatic WS, 157 patients; cryptogenic WS, 60 patients. Group 2: symptomatic WS, 169 patients; cryptogenic WS, 62 patients. The aetiologies of symptomatic WS were: cortical dysplasias, neurocutaneous disorders, cerebral malformation and prenatal clastic lesions, hypoxic ischaemia, post-infection, metabolic, tumors, Down's syndrome, others and unknown cause. CONCLUSIONS: It is known that cerebral MRI gives better definition of these types of cerebral lesions than cerebral CT does. We emphasize the importance of MRI in patients with symptomatic WS for precise determination of the aetiology, and speculate as to whether some of the 21 cases of unknown aetiology of group 1 could have been diagnosed if studied nowadays.

Adolescent↗
PubMedDate not supplied

[Atypical evolutions of benign partial epilepsy of infancy with centro-temporal spikes].

OBJECTIVES: To stress that different atypical evolutions occur in the course of disease in some patients with the so called benign partial epilepsies of childhood (BPEC). To promote interest in finding clinical and/or electroencephalographic clues to recognize which patients might be prone to present these risks. METHODS: Follow up of 26 pediatric patients who started with typical clinical and EEG features of benign childhood epilepsy with centro-temporal spikes (BCECTS) but presented reversible or persistent serious epileptic events including status epilepticus and language, cognitive or behavioral impairments. Repeated neurological examinations, EEG records and neuropsychological evaluations were done in the course of up to 14 years. Brain imaging studies (CAT and/or MRI) were obtained in all patients. RESULTS: Four groups of patients were recognized: 11 children with atypical benign partial epilepsy of childhood (ABPEC) followed during 4 to 13 years. All have finally recovered and attend normal schools, although five have learning difficulties. Three patients met diagnostic criteria for Landau-Kleffner syndrome (LKS). Two of them recovered from aphasia and in one, some language difficulties persist. Seven children showed status epilepticus of BCECTS but all of them are now normal after 3 to 14 years of follow-up. Five children showed mixed features of the three groups mentioned above and three of them fulfilled the criteria for diagnosis of the syndrome of continuous spike-wave during slow sleep (CSWS). CONCLUSIONS: A small proportion of cases starting with BCECTS evolve into either ABPEC, LKS, status of BCECTS or the syndrome of CSWS. Then, BCECTS is not always benign. Clinical and EEG markers should be sought to predict these atypical evolutions.

Anticonvulsants↗
PubMedDate not supplied

[Reflex myoclonic epilepsy in infancy: a new reflex epilepsy syndrome or a variant of benign myoclonic epilepsy in infancy].

CASE REPORTS: We report a clinical and EEG study of 8 children with reflex myoclonic epilepsy of infancy to further confirm the existence of this syndrome first described by Ricci et al in 1995. RESULTS: Between February 1990 to July 2002, we identified 64 epileptic patients with myoclonic seizures with an onset in the first six years of life. Eight (12.5%) of these patients had myoclonic seizure stimuli sensible. The seizures were characterized by generalized, myoclonic jerks triggered by tactile stimuli in six patients and acoustic stimuli in two, in one of them myoclonic jerks were triggered by both types of stimuli. The seizures appeared between 5 and 20 months of age. Two of the 8 patients had spontaneous myoclonic attacks during sleep. Interictal EEG was normal during wakefulness and occasional discharges were evident during sleep. In contrast, the ictal EEG during both wakefulness and sleep showed generalized spike wave and polyspike slow wave paroxysms. Neurologic examination, neuroimaging and neurometabolic studies were normal. Myoclonic jerks disappeared in 6 patients after valproic acid administration and in two after clobazan administration. Antiepileptic treatment was discontinued in 6 patients and no seizure recurrence was observed during a median follow up of 6 years. CONCLUSION: Our patients presented electro clinical criteria compatible with the syndrome of reflex myoclonic epilepsy of infancy. This syndrome could be considered to be a new reflex epileptic syndrome or a variant of benign myoclonic epilepsy in infancy.

Anticonvulsants↗
PubMedDate not supplied

[Angelman syndrome: the electroclinical characteristics in 35 patients].

AIMS: The purpose of this study is to report on 35 patients with Angelman syndrome (AS) in whom we evaluated the electroclinical characteristics and the progression of their epilepsy. PATIENTS AND METHODS: The following factors were evaluated: sex, family background, neurological examination, age at onset and semiology of the epileptic seizures, EEG, types of epilepsy according to the international classification and response to therapy. We investigated the karyotype, and conducted FISH and methylation tests for AS. RESULTS: The 35 patients had an average follow up time of 5.6 years. Epilepsy was diagnosed in 25 cases, with an average age of onset of 1.6 years. The epileptic syndromes were: epilepsy with myoclonic seizures in 13, of which seven presented a myoclonic state in their history, focal epilepsy in seven, West's syndrome in three, and Lennox Gastaut syndrome in two. Intercritical EEG showed generalised MSW and SW paroxysms in 13, unilateral spikes in seven, hypsarrhythmia in three, generalised fast rhythm paroxysms and slow SW activity in two. Basal electroencephalographic activity was: slow hypervoltage waves with or without inserted spikes situated at the rear in 19, at the front in six, diffuse in six, and normal in four cases. CONCLUSIONS: 71.4% of patients with AS suffered epileptic seizures; epilepsy with myoclonic seizures was the most frequently observed epileptic syndrome and hypervoltage slow wave activity with or without spikes inserted in the posterior quadrants was a characteristic encephalographic pattern. In patients with mental retardation, with or without epilepsy and these electroencephalographic findings, even in the absence of characteristic clinical signs, methylation and FISH analyses for AS should be performed.

Adolescent↗
PubMedDate not supplied

[Delayed-type subacute measles encephalitis].

INTRODUCTION: We analyze the clinical, neurological, EEG, neuroradiological features and evolution of two patients with subacute measles encephalitis. CASE REPORTS: The patients, aged five years and eleven months respectively showed an acute, progressive neurological compromise and deterioration of consciousness, epilepsia partialis continua and progressive damage on neuroimaging, with a history of measles in the first case and exposure to the virus in the second. The first patient had Hodgkin's disease and the other had a familial C4 deficit disorder. Fundoscopic examination showed lesions on the retina. The EEG showed unilateral slow waves and spikes. Brain CT and MRI revealed progressive cerebral atrophy and a unilateral corticosubcortical lesion. Measles antibodies in CSF were found in the first child and oligoclonal bands in the second. Our first patient died after three months and the second has a severe neurological damage. CONCLUSION: In immunocompromised patients with the exposure to a history of measles, acute neurological compromised and deterioration of consciousness, epilepsia partialis continua and progressive damage on neuroimaging, subacute measles encephalitis should be considered.

Acute Disease↗
PubMedDate not supplied

[Pyridoxine dependence: the importance of the clinical diagnosis and early treatment].

OBJECTIVE: We described the electroclinical features, evolution and family history of two patients with definitive diagnosis of pyridoxine dependency. CASE REPORTS: The first patient is a 15-month-old girl who at 1 month of age started with seizures and irritability. At two months of age, pyridoxine was prescribed with a good control of seizures. At five months of age withdrawal response provoked 7 days after seizures recurrence. Pyridoxine was reintroduced and seizures disappeared. Her sister, at two months of age, started with refractory seizures. This sister also had mental retardation and at four years, she died. Her brother, 16 years old, presents mental retardation, refractory epilepsy and progressive motor and cognitive impairment. At 3 months of age, he started with seizures and at 15 years of age, pyridoxine was prescribed with a significative improvement the number of seizures and a better visual connection. The second patient is a 4-month-old girl who started with clonic seizures at 3 days of age and she had a good response to pyridoxine. Withdrawal response provoked seizure recurrence at 48 hours. Pyridoxine was introduced immediately with total control of seizures. She had two cousins with seizures who died at 3 months and 3 years of age respectively. CONCLUSION: When dealing with an infant with refractory seizures which start in the first two years of life and without etiology, we should consider the diagnosis of pyridoxine dependency. Early diagnosis and treatment with pyridoxine is crucial to avoid high risk morbidity and mortality. All infants in the two first years of life with refractory seizures without etiology must be prescribed oral pyridoxine (50-200 mg per day).

Female↗
PubMedDate not supplied

[Hemiparetic cerebral palsy and startle epilepsy].

OBJECTIVES: We analyzed electroclinical features and evolution in nine patients with hemiparetic cerebral palsy associated with SE. PATIENTS AND METHODS: Nine patients with mean age 12.3 years and a follow up from 1 to 11 years were studied, analyzing etiology, neurological examination, psychometric evaluation, age at onset and semiology of epileptic seizures, EEGs and neuro-radiological findings, response to treatment and evolution. RESULTS: Etiologies were: porencephaly in 4 cases, hypoxic-ischemic encephalopathy in 2, bacterial meningitis in 1, herpetic encephalitis in 1 and meningo-encephalitis in the last. All cases had mental retardation. Mean age at onset of epileptic seizures was 4 years. Mean age at onset of startle seizures was 6. The startle seizures were characterized by sudden tonic contractions of the paretic hemibody, provoked by auditory stimulus in 6, somatosensory in 2 and both types of stimulus in 1. Falls were observed in 6 patients. Seizures were daily and always when awake. Unprovoked focal seizures with or without secondary generalization were found in 8 cases, and in 6 they presented previously to the startle seizures. Interictal EEGs showed unilateral spikes in 3, bilateral spikes in 3 and generalized polyspike-wave paroxysms in the other three cases. Ictal EEGs were obtained in 8 of the 9 patients and showed diffuse paroxysms of rhythms 6-11 Hz. Cerebral CT scan and/or MRI revealed extensive unilateral encephalomalacia in 5 and porencephaly in 4. The different antiepileptic schedules were unsuccessful in all cases. Surgery was performed in two patients. They are free of seizures after 1 to 4 years of follow up. CONCLUSION: SE should be considered as a distinctive epileptic syndrome or a particular electro-clinical evolution in patients with a large unilateral brain lesion associated with provoked reflex seizures usually refractory to antiepileptic drugs. Epileptic surgery should be considered.

Cerebral Palsy↗
PubMedDate not supplied

[Clinical and electroencephalographic aspects of late infantile neuronal ceroid lipofuscinosis].

AIMS: In this paper we describe the clinical characteristics, and particularly the epileptic seizures and electroencephalographic findings, in 15 patients with a pathology diagnosis of late infantile neuronal ceroid lipofuscinosis (NCL). PATIENTS AND METHODS: Nine female and six male patients were studied and their clinical records covering the period February 1990 to June 2003 were analysed. Neuroimaging, neurometabolic studies, ERG, PE and repeated EEG were carried out in all cases. RESULTS: The mean age on onset of the disease was 3 years (range: 1-5 years). The initial symptom was epilepsy in all cases. Massive myoclonias and myoclonic-atonic seizures were the most frequent kinds of attacks. Focal myoclonias were observed in six patients. Other types of epileptic seizures observed included generalised tonic-clonic, absence, motor focal and complex focal. The epileptic seizures were resistant to therapy. Progressive neurological and visual impairment, pyramidal and cerebellar signs, as well as mental retardation were present in all cases. Intercritical EEG recordings showed diffuse paroxysms with spike and polyspike waves, multifocal spikes and, less often, focal spikes that were predominant in posterior regions. Photostimulation showed high amplitude (300-450) occipital spikes during the application of light stimulation between 1 and 8 Hz. ERG, VEP and SSEP results were pathological. Images showed signs of brain and cerebellar atrophy. Seven of the patients died between 8.5 and 11 years of age. CONCLUSIONS: Late infantile NCL must be considered in the case of a child aged between 1 and 5 years who presents seizures that are predominantly generalised myoclonias and myoclonic-atonic, in association with progressive neurological deterioration including pyramidal, cerebellar and visual signs and an EEG trace showing occipital paroxysms triggered by low frequency photostimulation.

Child↗