Biomedical subjects

A Spalice

Publications and source records attributed to A Spalice.

23 records · Page 2Linked to original sources

PubMed1992

Pretransplant conditioning with busulfan and cyclophosphamide in acute leukemia patients: neurological and electroencephalographic prospective study.

A neurological and electroencephalographic (EEG) prospective study was performed in 34 leukemic patients receiving busulfan (BU) plus cyclophosphamide (CY) before bone marrow autologous transplant. During BU treatment and briefly thereafter, all patients were given anticonvulsant prophylaxis with phenobarbital. Neurological evaluations were performed daily and EEGs, recorded one week before and soon after the end of the BU regimen, were re-evaluated two months later. Basal EEGs were normal in 23 of the 34 patients, focal abnormalities were detected in 9 cases, and generalized epileptic discharges were present in one (one subject was not studied). EEGs performed at the end of BU administration showed generalized spike/polyspike-and-wave discharges in 21 of the 34 patients, with associated myoclonic epilepsy in 10 subjects appearing on the 3rd or 4th day of treatment. Focal abnormalities were present in 6 patients and constant EEG normality in 7. It is known that myoclonic epilepsy can be induced by drugs. Oral administration of high-dose BU is followed by a high-dose cerebrospinal fluid concentration of the drug. Therefore, myoclonic epilepsy and/or paroxysmal epileptiform EEG discharges may be observed in leukemic patients undergoing high-dose BU therapy.

Acute Disease↗

PubMedDate not supplied

Positron emission tomography in neuronal ceroid lipofuscinosis (Jansky-Bielschowsky disease): a case report.

We report on a 13-year-old girl with late infantile neuronal ceroid lipofuscinosis (NCL) in whom PET scanning with [18F]-2-fluoro-2-deoxy-D-glucose ([18F]/FDG) was performed. Early psychomotor development was normal. At the age of 2 years, neurological signs such as hypotonia and incoordination appeared, followed by visual failure and ataxia. At the age of 4, funduscopic examination showed macular degeneration and papillary atrophy. At the age of 9, myoclonic jerks were observed; subsequently, generalized seizures together with failing vision, mental deterioration, and visual and auditory hallucinations appeared. Brain MRI showed severe cortical and subcortical atrophy. A skin biopsy detected the presence of 'finger-print' inclusions in the cytoplasm of smooth muscle fibers. Late infantile NCL (Jansky-Bielschowsky disease) was diagnosed. FDG/PET revealed a severe reduction of metabolism in all the cortical and subcortical structures. A regional analysis of the distribution of the tracer revealed marked bilateral hypometabolism, particularly in calcarine, lateral, occipital, and temporal cortices and in the thalamus.

Adolescent↗

PubMedDate not supplied

Neuronal migrational disorders in children with epilepsy: MRI, interictal SPECT and EEG comparisons.

Single-photon emission computed tomography (SPECT) is being increasingly used in the investigation of children with epilepsy and may provide insights into congenital malformations. We analyzed the interictal 99Tc-HMPAO-SPECT in a series of seven children with developmental disorders of the neocortex, each of them representing a prototype of cerebral dysgenesis, such as lissencephaly, pachygyria, opercular dysplasia, polymicrogyria, nodular heterotopia and band heterotopia. The patients studied were selected among 22 epileptic children with neuronal migrational disorders (NMDs). Interictal SPECT hypoperfusion was observed in the area homologous to MRI findings in all the examined children. In three patients low perfusion was also present in the opposite hemisphere, probably due to functional involvement or related to an underlying microdysgenesis, not revealed by structural imaging. EEG features were in agreement with low perfusion areas, both anatomically and functionally, in all children. In one patient hypoperfusion area differed from that revealed by MRI and EEG. Ictal SPECT has been considered a useful tool for accurately locating the epileptic focus. Nevertheless, interictal brain perfusion studies, together with proton magnetic resonance spectroscopy, may play an important role in detecting anatomic substrate in developmental disorders of the neocortex.

Cell Movement↗

PubMedDate not supplied

[New drugs in the treatment of childhood epilepsy: vigabatrin (study of 61 subjects)].

This study was conducted to determine the efficacy of vigabatrin in children with epilepsy. Sixty-one children with various types of severe epilepsy were studied. In 12 children vigabatrin was introduced as monotherapy, while in 49 it was added to other antiepileptic drugs. Following the introduction of vigabatrin, 17 patients became seizure free, and 19 responded with a greater than 50% reduction in seizure frequency. The following types of epilepsy responded favorably to treatment (in order of decreasing efficacy): West syndrome, especially if secondary to Tuberous Sclerosis; cryptogenic and symptomatic partial epilepsy; Lennox-Gastaut syndrome, and other symptomatic generalized epilepsy. Optimal responses were found with vigabatrin doses of 30 to 50 mg/kg/day. Forty-one responders continued on vigabatrin, with generally good efficacy. Vigabatrin tolerability was good; in only 6 children was treatment discontinued because of side-effects such as somnolence, irritability, weight gain, and cutaneous rash. This study shows that vigabatrin is effective in the treatment of refractory epilepsy, especially in West syndrome secondary to Tuberous Sclerosis, partial epilepsy, and Lennox-Gastaut syndrome. Further studies are needed to analyze the increased frequency of seizures and the appearance of new seizure types associated with vigabatrin treatment.

4-Aminobutyrate Transaminase↗

PubMedDate not supplied

Clonazepam prophylaxis and busulfan-related myoclonic epilepsy in autografted acute leukemia patients.

A prospective neurological and electroencephalographic (EEG) study was performed in sixteen leukemia patients receiving busulfan (BU) and cyclophosphamide before autologous bone marrow transplantation. All patients were given anticonvulsant prophylaxis with a combination of phenobarbital (PB) and clonazepam (CLZ). Neurological examination and EEG were performed prior to and soon after completion of BU treatment and were repeated two months later. No tonic-clonic and/or myoclonic convulsions were observed. In two patients, comparison of EEG recorded before and upon completion of BU administration revealed modification of features. EEG re-evaluated two months after BU showed normalization in one of the two patients. BU may trigger both generalized and myoclonic seizures together with EEG abnormalities; PB combined with CLZ may be useful prophylactic treatment.

Adolescent↗