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Biomedical subjects

F Mulas

Publications and source records attributed to F Mulas.

At least 55 records · Page 3Linked to original sources

Radiological Diagnosis of Periventricular and Subcortical Leukomalacia.

Nine newborn infants with histories of perinatal asphyxia are presented. The pneumoencephalographic findings which led to the diagnosis are typical and constant. They include marked subcortical atrophy with rounded, dilated, and undisplaced lateral ventricles. Cystography with 3 cc of air demonstrated multiple subcortical and pareventricular cavities, without communication with the ventricular system, but with the typical honeycomb appearance of paraventricular and subcortical leukomalacia described in postmortem findings. The CT findings are typical, and provide the location of the cavities as well as their density.

Birth Weight↗

Occlusion of the cerebral arteries in Recklinghausen's disease.

Two children, aged 18 months and 6 years, who had Recklinghausen's disease, had occlusion of cerebral arteries. One child had no motor deficit but the other had right hemiparesis and partial occlusion of the left posterior cerebral artery, a fact not found in the literature.

Arterial Occlusive Diseases↗

[Prognostic value of neonatal paroxyistic electroencephalogram (author's transl)].

Twenty full-term newborns showing acute neurologic symptoms of different etiologies, with neonatal paroxistic EEG tracings are reported. Clinical and electroencephalographic follow-up at three and twelve months confirmed in every case the presence of severe neurologic sequelae. A review of the literature is made, comparing the results of other investigators with those obtained in the present study specially from an ethiologic, clinical, electroencephalographic and evolutionary points of view.

Brain Diseases↗

Bilateral obstruction of the vertebral arteries in a three-year-old child.

A three-year-old boy developed gait instability, nausea, vomiting, cranial nerve disturbances, hypotonus and dysarthria. Angiography of the four main cranial arteries showed complete obstruction of both vertebral arteries at the level of C1 to C2. Abundant collateral circulation was observed, which by-passed the obstruction to the vertebral arteries before their enterance into the posterior cranial fossa. The left vertebral artery was hypoplastic and both internal carotid arteries showed coiling in their extracranial portions. A high erythrocyte sedimentation rate at the beginning of the disease suggests an inflammatory alteration of both dysplastic vertebral arteries. The child recovered completely one month after the onset of symptoms. All other reported cases of childhood vertebro-basilar obstruction are reviewed and it is emphasized that the site of arterial obstruction has an important bearing on the clinical outcome.

Arterial Occlusive Diseases↗

Cerebro-facio-thoracic dysplasia: report of three cases.

Three children, two girls and one boy, are described as suffering from a new syndrome, 'cerebro-facio-thoracic dysplasia'. The characteristic findings are mental retardation, characteristic facies, narrow forehead, bushy eyebrows with synophrys, hypertelorism, broad nose, wide philtrum, triangular-shaped mouth, short neck, marked maxillary hypoplasia, a low hairline (especially posteriorly in the midline on the neck), brachycephaly, calcified clinoid ligements, and multiple bony abnormalities in the upper thoracic vertebrae and sometimes in the cervical region, together with a variety of deformities of the upper ribs. It seems possible that the condition is hereditary and that it may be inherited as a result of an autosomal recessive trait.

Child↗

Intrathecal chemotherapy-related myeloencephalopathy in a young child with acute lymphoblastic leukemia.

Since the mid-1960s intrathecal chemotherapy (methotrexate [MTX], cytarabine [Ara-C], or both, plus hydrocortisone) has constituted the standard approach to prophylaxis and treatment of central nervous system (CNS) leukemia and lymphoma. Intrathecal chemotherapy-related neurotoxicity has been described in a variable proportion of patients. At least 35 cases of subacute myeloencephalopathy with transient or permanent paraplegia/quadriplegia after intrathecal chemotherapy have been reported. Different factors have been cited: high cumulative MTX dose, meningeal leukemia, cranial irradiation, and preservatives in MTX and Ara-C. A direct toxic effect of the intrathecal chemotherapy seems the most likely mechanism. Early imaging studies are usually normal. We describe a nonfatal case of permanent flaccid quadriplegia after the fourth triple intrathecal chemotherapy in a 6-year-old girl with acute lymphoblastic leukemia and no evidence of meningeal involvement. Six months after intrathecal chemotherapy, CNS magnetic resonance imaging showed severe atrophy of spine, cerebellum, and cerebral hemispheres. The outcome of reported cases is diverse. No treatment has been shown to reverse neurotoxicity. Among the cases reported in the literature, complete recovery of neurologic deficits was observed in 9 patients, partial recovery with variable sequelae in 6, no recovery in 8, and 13 patients died from the initial oncologic disease or neurotoxicity progression.

Antineoplastic Combined Chemotherapy Protocols↗

[Psychogenic paroxysmal disorders in children].

INTRODUCTION: Paroxystic psychic disorders which imitate organic disorders of the nervous system may have peripheral effects, present as changes in level of consciousness or appear as paroxystic behaviour changes. DEVELOPMENT AND CONCLUSIONS: The types of crises of psychological origin are: tantrums, panic attacks, crises of psychopathic rage, onanism or masturbation, epileptic pseudocrises or pseudoconvulsions and Munchausen's syndrome. In general psychic crises are not frequent in infancy: tantrums are commoner in small children and the other conditions usually occur after puberty or during adolescence. The anamnesis is the most important factor in the correct diagnosis of psychogenic paroxystic disorders. Complementary studies are done in doubtful cases, to rule out different pathological processes which might be causing the paroxystic disorder. Amongst these investigations, we emphasize the importance of the video-EEG for differential diagnosis of paroxystic disorders in children.

Child↗

[Disseminated acute encephalomyelitis in children. A retrospective study of seven patients].

INTRODUCTION AND OBJECTIVE: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system. This study is based on clinical symptoms and diagnostic tests employed. PATIENTS AND METHODS: We describe a seven children series indicating the initial neurologic abnormalities, diagnostic tests, treatments used and clinical-neuroradiological evolution. RESULTS: The mean presentation age was 4.1 years. Initial neurologic symptoms were mainly spastic hemi/paraparesis, cerebellous and pyramidal syndrome, consciousness changes, meningeal signs, seizures and cranial nerve palsies. The cerebrospinal fluid was abnormal in four patients with positive serologic tests in two of them (Coxsackie B). Electrophysiological studies were affected in 50%. MRI findings consisted of multifocal supratentorial white matter lesions. Clinical evolution revealed a progressive improvement with resolution after two months. Follow-up was made between six months and five years. The treatment was based on aciclovir and corticosteroids. CONCLUSIONS: ADEM runs a monophasic course of progressive neurologic abnormalities. Diagnosis is based on suggestive clinical and neuroimaging findings. Generally speaking, MRI showed resolution of multifocal lesions in conjunction with clinical improvement.

Acyclovir↗

[Treatment and prevention of cerebral hemorrhage in newborns].

INTRODUCTION: The risk factors leading to the occurrence of intracranial hemorrhages in premature newborn babies are well known. However, in full-term babies this has not been so systematically studied. Although the incidence is lower it is not rare and they are more varied in the form of presentation than in premature babies. Thus, as well as the classical intraperiventricular hemorrhages, subarachnoid and intraparenchymatous hemorrhages also occur relatively often, particularly the subarachnoid kind. The aetiopathogenesis of the hemorrhages is different, so preventive measures are also different, based particularly on avoidance of perinatal asphyxia and traumatic manoevres during labour. DEVELOPMENT: A study by our Department, in which risk factors associated with 17 newborn babies of over 35 weeks gestation who had a history of intracranial hemorrhage in the early neonatal period showed that over half the cases (58%) had a history of acute foetal distress. A quarter had congenital cardiopathy (detected at birth) and the same number had a respiratory distress syndrome due to various causes, particularly pneumothorax and infections. Regarding treatment of the acute phase, it is essential to maintain cerebral perfusion and monitor both arterial hypertension and hypotension, control the intracranial pressure (avoiding increases) and avoid fluctuations of cerebral blood flow. CONCLUSION: Follow-up by means of serial echographs makes early detection of dilatation and hydrocephaly possible and permits suitable suitable treatment rapidly and with immediate monitoring of its efficacy.

Cerebral Hemorrhage↗

[Periventricular leukomalacia: neurological and radiological sequelae and long term neuropsychological repercussions].

INTRODUCTION: Periventricular leukomalacia is an anatomical diagnosis made by neuroimaging technics, of hypoxic-ischemic origin and a characteristic neurological syndrome consisting in cerebral palsy, visual impairment and/or mental retardation. Is more frequently seen in pre-term infant (5-10%) as a spastic diplegia or tetraplegia. Real prevalence in a-term infant is unknown and is presented as hemiplegia. The correlation between periventricular leukomalacia severity and clinical severity is not absolutely clarified. Is not still known if we can predict mental retardation in an infant with periventricular leukomalacia on magnetic resonance image. DEVELOPMENT: This paper revise the clinical and radiological diagnosis of periventricular leukomalacia and shows the results of a series of 31 patients diagnosed radiologically of periventricular leukomalacia. We correlate the degree of neurological and mental disabilities with the severity of periventricular leukomalacia. CONCLUSION: We reach a good correlation between the severity of periventricular leukomalacia and the severity of neurological disturbances and mental retardation.

Brain↗

[Pharmacological treatment with risperidone in children with behavior disorders].

INTRODUCTION: Behavior disorders are frequently seen in children attending a neuropaediatric clinic. The classical neuroleptic drugs are usually used for treatment. However response tends to be irregular with adverse effects at a cognitive level and extrapyramidal symptoms. PATIENTS AND METHODS: We started to use risperidone in children with serious behavior problems who had not responded to other drugs, and evaluated the clinical course and side effects. RESULTS: A total of 16 patients aged between 7 and 14 years were treated for diagnoses of: hyperactivity attention deficit disorder, mental retardation with non specific behavior disorder, Gilles de la Tourette disorder with hyperactivity attention deficit disorder and generalized disorder of development. The doses of risperidone varied between 0.01 and 0.05 mg/kg/day. In two cases the evolution could not be assessed, was good in 10 and no change was seen in 4. The group of patients with most improvement were those with mental retardation. The commonest adverse effect was weight gain. No patient had extrapyramidal symptoms. CONCLUSION: We consider risperidone to be a safe drug for the pharmacological treatment of children with behavior problems.

Adolescent↗

[Tectal tumours in paediatrics. A review of eight patients].

INTRODUCTION: Tumours of the tectal region form, within the group of gliomas of the brain stem, a sub group with better prognosis which require a different therapeutic strategy. OBJECTIVES: A retrospective review of tectal tumours in children to find the most suitable therapeutic approach and the prognosis to be expected. PATIENTS AND METHODS: We reviewed 8 paediatric patients who, during the past 11 years, had developed tectal tumours diagnosed by means of neuro imaging techniques. We evaluated their clinical features and especially their clinico radiological progress. RESULTS: The average age at the time of diagnosis was 10 years. All cases presented with the clinical features of raised intracranial pressure secondary to obstruction of the aqueduct of Sylvius. MR was the key to visualization of the tectal lesion in all cases but one, which had already been shown on CT. The initial and only treatment given was a ventricular shunt. The average clinico radiological follow up in our series was 4 years, with good progress in all cases so that no other therapeutic measures were required. MR follow up showed that the tumours were stable in all cases but one, which had increased slightly in size but without parallel clinical signs. CONCLUSION: In most cases tectal tumours in children follow a benign course. A ventricular shunt is usually the only treatment necessary. However, close clinical and radiological follow up should be carried out to rule out growth of the tumour which make other treatment also necessary. MR is the method of choice for initial evaluation and follow up of these tumours.

Adolescent↗

[Alternating hemiplegia in young babies].

INTRODUCTION. This paper review the state of the art of alternating hemiplegia of childhood. This entity is a rare neurologic disorder of infancy characterize by transient hemiplegic spells shifting from one side to another, and occasionally affecting to both hemispheres at the same time. Usually start before 18 months, many cases exhibit neonatal symptoms related with the disorder. Early symptomatology include abnormal ocular movements, mainly nystagmus, and tonic or dystonic attacks generally beginning before 6 months of age. These symptoms are frequently misdiagnosis as epilepsy. Typical hemiplegic fits which disappeared when infant felt asleep appeared by 12 months of age. Diagnosis is on clinical basis after excluding any other causes of fluctuating and transitory hemiplegic attacks. Complementary investigations, such as electroencephalograms, TAC, MRI, angiographic MRI, CFS are strictly normal. Cerebral SPECT show controversial abnormalities in some studies. We perform SPECT study in a alert 12 months old girl during an interictal period resulting in a non significant asymmetry, with lesser perfusion in left frontal basal and anterior temporal gyrus. Nevertheless, we observed an striking hyperperfusion in middle occipital area. Treatment is symptomatic with flunarizine. Our patient experience a dramatic decrease in frequency, duration and severity of hemiplegic attacks.

Brain↗

[Anal extrusion: a complication of ventriculo-peritoneal shunt for hydrocephalus (author's transl)].

The authors report a case of rectal perforation eight months after the placement of a ventriculo-peritoneal shunt. There was no peritonitis, but an "E. coli" meningitis was found. The patient died two hours after surgical removal of the shunt. At autopsy, a perforation by the tip of the catheter was found in the anterior face of the rectosigmoid.

Arteriovenous Shunt, Surgical↗

[The effects of psychostimulants on the cognitive results and behaviour of children with the combined subtype of attention deficit with hyperactivity].

INTRODUCTION: Many studies have shown the efficacy of methylphenidate in treating children with attention deficit and hyperactivity to reduce the main symptoms of the disorder and problems of behaviour: behaviour which is disruptive, antisocial, negative and aggressive. OBJECTIVES: 1. Analyze the therapeutic effects of methylphenidate on the cognitive function of children with TDAH, combined subtype, evaluated by means of neuropsychological tests of attention and inhibitory control. 2. Determine the efficacy of methylphenidate in improving the basic symptoms of TDAH C according to the DSM 1V for parents and teachers, and their behaviour, evaluated by their teachers, in the combined subtype. PATIENTS AND METHODS: The sample was made up of 48 children with TDAH C. 24 of these had been referred by paediatricians and received drug treatment with methylphenidate, 0.5 mg/kg once in the morning and once after lunch (TDAH C/CTF). The other group of 24 children with TDAH C were referred from their teachers to a school psychologist so that they followed the usual guidelines for orientation but not a systematically developed treatment (TDAH C/C or control group). RESULTS. In relation to the first objective the results were good for the TDAH C/CTF compared with the TDAH C/C group regarding the proportion of children who improved their results on neuropsychological testing of planning inhibitory control and attention. The differences between the two groups were statistically significant in the latent period of the reflexive impulsive test (MFF), arithmetic, cancellation of rhomboids and cancellation of numbers. Regarding the second objective, the improvement in the TDAH C/CTF group according to the teachers opinions were statistically significant with respect to attention, hyperactivity and impulsiveness, as compared with the control group. However, according to the parents assessment, although improvement was greater in both dimensions, the differences between the two groups did not reach statistical significance. Finally, the teachers observed greater improvement in the medicated group as compared to the control group regarding three variables: learning problems, antisocial conduct and failure to adapt to school. CONCLUSIONS: Overall, our results coincide with those of other excellent reviews, which have shown the effectiveness of methylphenidate in the treatment of cognitive and behaviour disorders in this group.

Attention Deficit Disorder with Hyperactivity↗

[Guillain-Barre's syndrome in childhood (author's transl)].

Seventeen cases of Guillain-Barré's syndrome aged between 14 month ans 7 years are studied from clinical, analytical and evolutives aspects. Neither sex nor season predominance occurs. Sixteen of the patients had a previous viral or bacterial infection. Difficulty in walking was the main symptom at the onset of illness. Arreflexia and motot paralysis were always symmetrical and ascending. Albumino-cytological dissociation in the C.S.F. was found in the whole group. An evolutive study was made of the recovery of movement, C.S.F. and tendon reflexes which occurred in this order except in two cases which showed abnormal evolution. We emphasize the extent and frequency of respiratory difficulties at the onset of illness. Some etiopathogenic aspects, the lack of specifity of the syndrome and the differences of the diagnosis with that of poliomyelitis are commented. We point out the lack of effect of the corticosteroids treatment and the positive effects of phisiotherapy after the acute stage.

Cerebrospinal Fluid Proteins↗

[Intracranial lipomas in paediatrics: a retrospective study of 20 patients].

INTRODUCTION: Intracranial lipomas (ICL) are congenital malformations that are due to an anomalous differentiation of the primitive meninges. AIMS: The purpose of this study is to determine the most frequent symptoms related to ICL in the paediatric age, as well as to evaluate whether they are directly related to their location and to their association with other brain malformations. PATIENTS AND METHODS: A retrospective clinico-radiological study was conducted involving 20 paediatric patients diagnosed as suffering from ICL between 1985 and 2003 at three hospitals in the Valencian Community. RESULTS: 70% of the cases were females and the average age when ICL was diagnosed was 5.3 years (interval from 2 months to 13 years). The most common initial clinical justification for the neuroradiological study was psychomotor retardation in eight patients (40%) and persistent, predominantly migrainous, headaches in six of them (30%). Only one patient was examined because of epilepsy. The prevailing site was the interhemispheric fissure in 10 patients (50%), followed by the quadrigeminal cistern in five (25%) and the mamillary body infundibulum axis in three of them (15%). One case was located in the cerebellum and another was found in the lateral ventricle, with involvement of the choroid fissure. Associated anomalies were observed in eight patients (40%). Alterations were seen in the development of the corpus callosum in six patients (30%), five in an isolated way and in one case there was partial absence of the associated septum pellucidum. Another patient was found to have an isolated partial agenesis of the septum and Goldenhar syndrome was detected in another case. The radiological follow up, which varied according to the initial clinical features, did not show any differences in the morphology or the size of the lipoma. CONCLUSIONS: 1) ICL are more frequently located in the interhemispheric fissure and in the quadrigeminal cistern; 2) In these locations they can be associated with other brain malformations, the most important being defects in the callosa and septa; 3) Most ICL were found incidentally during the course of a neuroimaging examination. Only in the case of three patients (15%) was there any presumed relation between the location of the ICL (pericallosal), the symptomatology (overall psychomotor retardation) and the associated malformation (hypoplasia of the corpus callosum); 4) The incidence of epilepsy was much lower than that claimed in earlier reports, and the predominant associated neurological symptomatology was found to be psychomotor retardation and headache (70%); 5) ICL is a developmental anomaly that does not require neurosurgical treatment and which does not usually change with the passage of time, although it is liable to increase because of adipose hypertrophy.

Adolescent↗