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J Narbona

Publications and source records attributed to J Narbona.

At least 19 recordsLinked to original sources
PubMed2001

Transient posterior encephalopathy induced by chemotherapy in children.

The cases of three children, 16, 12, and 12 years of age, who suffered sudden confusional state and cortical blindness lasting 12 to 30 minutes while under treatment with high-dose methotrexate, cyclophosphamide, and dactinomycin for a lower limb osteosarcoma are reported. Transient neuropsychologic deficits arose after the acute phase of treatment: left hemispatial neglect and constructive apraxia (Patient 1); constructive apraxia (Patient 2); and constructive apraxia and alexia without aphasia (Patient 3). The three patients recovered completely from all their deficits within the time frame of 3 hours to 2 weeks. Arterial hypertension and hypomagnesemia were found during the acute phase in all patients. In Patients 2 and 3, magnetic resonance imaging revealed increased parieto-occipital T(2) signal involving gray and white matter. In Patients 1 and 2, HmPAO-SPECT revealed parieto-occipital hypoperfusion that resolved a few days later. The alterations detected by neuroimaging were concurrent with the appearance and disappearance of the clinical symptoms. Such transient acute episodes have been named occipital-parietal encephalopathy. On the basis of our clinical, laboratory, and neuroimaging findings, an explanation for the origin of this syndrome, a migrainelike mechanism, triggered by chemotherapy-induced hypomagnesemia, is proposed.

Acute Disease↗
PubMed1997

[Conners' rating scales in the assessment of attention deficit disorder with hyperactivity (ADHD). A new validation and factor analysis in Spanish children].

The use of Conner's Rating Scales (CRS) is very extended for the assessment of attention deficit disorder with hyperactivity (ADD-H). The main objective of this work is to study the validity of construct of CRS from theoretical basis and data statistical analysis. The study is based on a control group of 633 children and a clinical group of 33 subjects. We used a Spanish version of the Conner's Teacher Rating Scale (CTRS) and Conner's Parents Rating Scale (CPRS) in both groups. From the analysis of scales contents we attribute the items to several behavioral areas and we performed a factorial analysis. Studies of reliability, external validity versus clinical criteria, and analysis of correlations between parents and teachers scales were also performed. Findings from factorial analysis and study of reliability have shown the questions of CTRS to be clustered in three well-defined and reliable factors: hyperactivity, attention deficit, and conduct disorder. The questions dealing with emotional disorders are not sufficiently consistent to constitute a separate factor. CPRS has a lower reliability than CTRS, and its factorial analysis does not allow to consider a factorial distribution. The validity is high in CTRS and no significative in CPRS. Correlation between scales is also low. As a consequence of these results we propose a Spanish experimental revised form of the CTRS, with 20 items. This new form can assess separately: attention deficit; hyperactivity and conduct disorder, according to the current clinical criteria of CIE-10 and DSM-IV.

Attention Deficit Disorder with Hyperactivity↗
PubMed1997

[Botulinum toxin in spastic infantile cerebral palsy: results in 27 cases during one year].

INTRODUCTION AND OBJECTIVES: Positive outcome of patients with spastic cerebral palsy treated with botulinum toxin reported in the last three years has led us to perform this study with the aim to show our experience in the management of spastic cerebral palsy with the toxin, determine its indications, analyze the results and propose new possible indications in the future. MATERIAL AND METHODS: We include 10 hemiplegic and 17 diplegic patients with an average age of 6 years and 7 months, followed up between 5 and 17 months. Clinical improvement was monitored using the PRS and EVFEL scales and articular motion range was measured 6 months before and after the injection while continuing physiotherapy. The injected muscles were adductor, hamstrings, triceps and posterior tibialis, and the doses were 1-2 U/muscle/kg body weight. RESULTS: The values on PRS improved an average of 24%, adductor angle 66% (p < 0.01), knee angle 40% (p = 0.05) and ankle angle 52% (p < 0.01); 96% of patients could get more physiological static or walking patterns because of the decrease of spasticity and those persisted after the effect of the toxin had worn off. It was maximum at 2 months, stabilized 4 to 6 months later and decreased during further 2 months. CONCLUSIONS: This experience leads us to propose higher starting dosage and to take into account the stability of postural pattern of each patient to choice the muscle to be injected. Other therapeutic possibilities are also proposed in children with fixed shortening e.g. combining the toxin with stretching casts.

Adolescent↗
PubMed1996

Chiari type I malformation, glossopharyngeal neuralgia and central sleep apnoea in a child.

We describe the case of an 8-year-old child who presented with sleep breathing disorders associated with glossopharyngeal neuralgia. Polysomnographic studies showed central sleep apnoea lasting 10 to 18 seconds in an average frequency of 63 times for every hour of sleep. Magnetic resonance imaging (MRI) studies showed a caudal displacement of the cerebellar tonsils down to C3 level, associated with a syringomyelic cavity in the upper cervical cord and an extramedullary cystic collection at C2. Surgical decompression of the cranio-cervical junction completely relieved the apnoea and the glossopharyngeal neuralgia immediately following the procedure. MRI study performed one month later showed the complete disappearance of the syringomyelic cavity. Two years later this child remains asymptomatic. In childhood the association of Chiari type I malformation with sleep apnoea and glossopharyngeal neuralgia has not been previously reported. The successful outcome after surgery suggests a compressive mechanism on the brainstem and on the IX cranial nerve to explain these two clinical features in our patient.

Arnold-Chiari Malformation↗
PubMed1995

Molecular analysis of the Duchenne muscular dystrophy gene in Spanish individuals: deletion detection and familial diagnosis.

Deletion studies were performed in 26 Duchenne muscular dystrophy (DMD) patients through amplification of nine different exons by multiplex polymerase chain reaction (PCR). DNA from paraffin-embedded muscle biopsies was analyzed in 12 of the 26 patients studied. Optimization of this technique is of great utility because it enables analysis of material stored in pathology archives. PCR deletion detection, useful in DMD-affected boys, is problematic in determining the carrier state in female relatives. For this reason, to perform familial linkage diagnosis, we made use of a dinucleotide repeat polymorphism (STRP, or short tandem repeat polymorphism) located in intron 49 of the gene. We designed a new pair of primers that enabled the detection of 22 different alleles in relatives in the 14 DMD families studied. The use of this marker allowed familial diagnosis in 11 of the 14 DMD families and detection of de novo deletions in 3 of the probands.

Alleles↗
PubMed1995

[Sneddon's syndrome vasculopathy with antiphospholipid antibodies in a child].

We studied a ten-year old girl with livedo reticularis and multiple central nervous system ischemic attacks beginning when she was five. Blood tests were positive for cryoglobulins and antiphospholipid antibodies; superior limb angiography showed occlusion and irregularities of the vessel walls and temporal artery biopsy revealed concentric thickening of the vessel wall due to fibrotic subendothelial proliferation. Sneddon's syndrome was diagnosed. Onset of this syndrome during early childhood has not been previously reported.

Adrenal Cortex Hormones↗
PubMed1994

Dystonic storms: a practical management problem.

On rare occasions, torsion dystonia can rapidly worsen and produce life-threatening symptoms. We present reports on two children who had generalized dystonia and who demonstrate the management difficulties of "dystonic storms."

Child↗
PubMed1993

Parkinsonian syndrome in childhood after sodium valproate administration.

Among the side effects attributed to sodium valproate administration, the production of a parkinsonian syndrome is very uncommon, particularly in children. We report a 12-year-old girl with secondary epilepsy; 7 days after the initiation of valproate therapy she developed parkinsonism that disappeared completely when valproate was replaced by carbamazepine. We discuss the possible role of alterations in GABAergic neurotransmission in the extrapyramidal syndrome that developed.

Child↗
PubMed1984

Hemi-dystonia secondary to localised basal ganglia tumour.

An 8-year-old boy with an 18 month history of left limb hemi-dystonia due to a right lenticular nucleus astrocytoma originating in the putamen is reported. Subsequent neuropathological study demonstrated that the tumour was mainly localised to the right lenticular nucleus, with cystic necrosis in the infero-lateral putamen. Solid tumour also infiltrated the right hypothalamus, the anterior commisure and the optic chiasm, and there was perivascular spread into the globus pallidus, internal capsule and roof of the right lateral ventricle. This case, and the few other published reports of symptomatic dystonia due to focal brain lesions verified pathologically, indicate that damage to the lenticular nucleus, and to the putamen in particular, can cause limb dystonia in man.

Astrocytoma↗
PubMed1981

[Intracranial arteriovenous malformations in childhood. A revision of 25 cases (author's transl)].

Clinical features of A-V intracranial malformations during infancy, childhood and adolescence are reviewed on this series of 25 less-than-15-years-old patients. Great cirsoid malformations (2 cases) and aneurysms of the vein of Galen (2 cases) may cause diffuse brain-steal-ischaemia, hydrocephalus and congestive heart failure in early ages, event at birth. The clinical manifestations of brain angiomas rather begin from the fifth year of life. Little sized angiomas cause preferentially intracranial haemorrhagic attacks (17 of 21 cases in this series). Middle sized racemic angiomas cause focal seizures and/or focal neurologic signs. In 5 patients an intracranial bruit of a great diagnostic value was present. Combining brain radioisotopic studies, brain C.A.T. and angiography near 100% of intracranial A-V malformations can be diagnosed early after clinical suspicion.

Adolescent↗