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Biomedical subjects

J Viaño

Publications and source records attributed to J Viaño.

34 records · Page 2Linked to original sources
PubMed1996

Hemangiomas of the head, neck, and chest with associated vascular and brain anomalies: a complex neurocutaneous syndrome.

PURPOSE: To describe the vascular and nonvascular intracranial and extracranial anomalies associated with hemangiomas and vascular malformations of the face, neck, and/or chest. METHODS: Seventeen patients had a physical examination and imaging studies consisting of one or more of the following: pneumoencephalography, conventional carotid and vertebral arteriography, CT, MR imaging, and MR angiography. RESULTS: Conventional arteriography revealed persistence of the trigeminal artery in 5 cases, absence of internal or external carotid and/or vertebral arteries in 11 cases, persistence of intervertebral arteries in 1 case, deformities of the aortic arch in 3 cases, and anomalies of the intracranial arteries in 3 cases. MR angiography revealed persistence of the trigeminal artery in 1 case in which conventional arteriography failed to show the malformation, and permitted visualization of narrowing of the intracranial arteries. CT and MR imaging showed a cerebellar anomaly in 8 cases and cerebral cortical dysplasia with cerebral hemispheric hypoplasia in 1 case. Vascular and nonvascular anomalies appeared ipsilateral to the external vascular abnormalities in most cases. CONCLUSION: This study demonstrates the association of cutaneous angiomas with anomalies affecting intracranial and extracranial arteries, the cerebellum, and, less frequently, the cerebral hemispheres and aortic arch. This association constitutes a relatively frequent neurocutaneous disorder, which we call the cutaneous hemangioma-vascular complex syndrome.

Abnormalities, Multiple↗
PubMed1996

[Neuroimaging and epilepsy].

The objective of magnetic resonance neuroimaging is to define the structural changes responsible for epileptogenic lesions and to rule out the presence of dual pathology when partial epileptic seizures are being studied. To this end, the individualization and optimization of protocols and sequences is indispensable. Structural images must be complemented by clinical evidence and function studies (EEG, SPECT, PET) in order to determine whether the structural lesion is responsible for the seizure and might eventually be excised. Spectroscopy provides biochemical information that is somewhat comparable to that obtained by biopsy of the epileptogenic zone. Spectroscopic images in the near future will provide clear "blind" views of these zones. Functional magnetic resonance images will presumably be of great help in the management of these patients.

Arteriovenous Malformations↗
PubMed1996

[Spectroscopic magnetic resonance in hemimegalencephalus].

INTRODUCTION: Hemimegalencephalus (HM) is a disorder of cerebral migration characterized by the overdevelopment of one cerebral hemisphere. It is usually associated with pachygyria, gliosis and neurone loss. We present a study using stereoscopic magnetic resonance (SMR) in a case of HM confirmed by the pathologist. CLINICAL CASE: A girl with right HM had hemigeneralized crisis since birth. A selective right temporoccipital cortectomy was done when she was two and a half years old. The resected piece showed thickening and absence of cortical striation, neurone loss, gliosis, giant neurones and heterotopias. After a symptom-free period the crises reappeared as right fronto-parietal epileptiform anomalies. When she was four years old SMR was done to compare this area with the corresponding area of the radiologically normal left hemisphere. Comparative study showed a marked drop in N-acetyl-aspartate (NAA), glutamate (Glu) and Gaba, and increased choline (Col) and inositol (Ino). We found no difference in the creatinine levels. CONCLUSIONS: The histological findings are in concordance with the levels of metabolites found in the affected hemisphere. The drop in NAA and Glu is related to neurone loss and the increase in glial cells, and the increase in Col and Ino with increase in membranes metabolism, as is observed in the gliosis. SMR is an advance in the identification and grading of changes seen on conventional MR, when establishing the prognosis and choice of treatment in HM.

Brain↗
PubMed1995

Sturge-Weber syndrome without facial nevus.

A patient with Sturge-Weber syndrome without the characteristic facial nevus presented with focal seizures which were difficult to control and borderline mental level. CT disclosed calcification in the right occipital zone. A marked decrease of the regional cerebral blood flow that extended beyond the abnormalities depicted on CT was seen by SPECT. Venous magnetic resonance (MR) angiography revealed reduction of the superficial cortical veins and prominent deep collateral venous system in the same side of the cerebral lesion. Cranial MR imaging with Gd-DTPA demonstrated the pial angioma.

Adult↗
PubMedDate not supplied

[Aggression and mental retardation associated with bilateral cortical and subcortical atrophy].

OBJECTIVE: We wish to show the relationship between the aggressivity and the bilateral frontal lesion. CLINICAL CASE: We describe a 18 years-old patient affected by a severe neurological and psychopathological disorder consisting of mental retardation and behavior alterations, especially expressed by mood changes, irritability and violent reactions with aggressivity. Three dimensional magnetic resonance study disclosed severe bilateral frontal lesion with the presence of very small cortical gyri and enlargement of the frontal zones of the lateral ventricles as well as atrophy of the anterior regions of the corpus callosum. CONCLUSION: The case reported shows the clear relationship between the bilateral frontal lesion and psychopathological disease, especially aggressivity and irritability.

Adolescent↗
PubMedDate not supplied

[Congenital vascular malformations in childhood].

The intracranial congenital vascular pathology, arterial, arteriovenous and venous, is reviewed, including the cavernomas as well. It is commented the possible origin, the clinical, histological and neuroradiological alterations as well as the possible treatment, when necessary, of every entity. The contribution of the magnetic resonance (MR), especially the angiographic MR (AMR) has been conclusive to the diagnosis. There have been considered six main groups which include the main entities: 1. Arterial anomalies; 2. Arteriovenous malformations; 3. Malformations of the Galen vein; 4. Venous malformations, and 5. Intracranial cavernomas.

Brain Neoplasms↗
PubMedDate not supplied

[Occipital leptomeningeal angiomatosis without facial angioma. Could it be considered a variant of Sturge-Weber syndrome?].

INTRODUCTION: The association of cerebral leptomeningeal angioma and facial nevus flameus in the territory of the first branch of the trigeminal nerve ipsilateral to the angioma is known as the Sturge-Weber syndrome. The cases with absence of a facial angioma are usually considered to be variants of the syndrome. OBJECTIVE: To present four cases with occipital leptomeningeal angioma without facial angioma and describe the characteristics which differentiate them from or permit their inclusion within the group of Sturge-Weber syndrome, and also to establish the differences between this and the Gobbi syndrome (occipital cerebral calcifications, epilepsy and coeliac disease. CLINICAL CASES: We selected four cases in whom cranial magnetic resonance was done with intravenous gadolinium and three cases studied to rule out coeliac disease. The cerebral calcifications, unilateral in all four cases, were similar to those observed in the Sturge-Weber syndrome. All cases had leptomeningeal angiomas at the level of the cerebral calcification shown by the uptake of contrast material on magnetic resonance. Three patients had epilepsy but none had facial angiomas, hemiparesis or glaucoma. Coeliac disease was also ruled out, both on laboratory investigations and on intestinal biopsy. CONCLUSIONS: The cases described coincide with the Sturge-Weber syndrome in all having cerebral leptomeningeal angiomas. This differentiated them from the Gobbi syndrome which does not include meningeal angiomata. Another characteristic of the Sturge-Weber syndrome is the occurrence of epilepsy and mental deficiency. Whilst awaiting molecular genetic studies, our cases may be included semantically as a variant of the Sturge-Weber syndrome without the characteristic facial angioma, although they may possibly correspond to genetically different conditions.

Adolescent↗
PubMedDate not supplied

[Carlevoix-Saguenay type recessive spastic ataxia. A report of a Spanish case].

OBJECTIVE: To present the first Spanish case, perhaps the first non-Canadian, with ataxia type Charlevoix-Saguenay. CLINICAL CASE: A patient with important psychomotor delay and non-progressive ataxia has been studied in our Service during the first years of life and his follow-up has been carried out until 30 years of age. He has been studied from the clinical, ophthalmological and neuroradiological with magnetic resonance point of views. RESULTS: Ataxia and psychomotor delay showed a non-progressive evolution. The patient showed myelinated retinal fibers and atrophy of the superior half of the cerebellum. CONCLUSION: Neurological, ophthalmological and magnetic resonance images showing atrophy of the superior part of the cerebellum are the three main signs of the ataxia type Charlevoix-Saguenay. This patient could be the first case reported out Canada.

Adult↗
PubMedDate not supplied

[Hypothalamic hamartomas: control of seizures after partial removal in one case].

OBJECTIVE: To describe a patient with intractable seizures and hypothalamic hamartoma that was only partially resected with complete control of seizures and improvement in behavior after surgery. CLINICAL CASE: He had gelastic seizures from the first months of life associated with hypothalamic hamartoma. We used magnetic resonance spectroscopy to localize and measure the lesion in the temporal lobes and in the hamartoma. The relative intensity of N-acetylaspartate to creatine (NAA/Cr) and NAA/choline (Ch) were not significantly different from normal control subjects for either temporal lobes, whereas the ratio NAA/Ch was decreased and the ratio NAA/Cr was highly increased in the hamartoma. Despite only partial resection of the hamartoma, seizures have been completely controlled and the patient has recovered normal social and work activity and is ending a normal life, that follow three years after surgery. CONCLUSIONS: These findings suggest that gelastic seizures associated with hypothalamic hamartoma are generated within the hamartoma itself, and that it is possible to control epilepsy and to improve intellectual and social problems with only partial resection of the mass.

Adult↗
PubMedDate not supplied

[Battered baby syndrome. Report of a case with severe sequelae].

OBJECTIVE: To present a case with shaken-baby syndrome after having seizures and respiratory problems. CLINICAL CASE: A previously normal child of 7 months of age presented an acute picture of status epilepticus with respiratory problems and periods of apnea. He was studied with electroencephalography, computerized tomography, magnetic resonance (MR) imaging and spectroscopic-MR. The child showed the presence of small subdural and epidural hematomas in both frontal regions 24 hours after the onset of the problem. The follow-up with MR studies revealed voluminous subdural bilateral hygroma that increased the size along the following six months, despite treatment with bilateral subdural-peritoneal shunt, and the patient showed infantile spasms. At 8 years of age, the patient shows severe mental retardation with autistic behavior and blindness, though he is able to walk without help and he has not seizures. The subdural hygroma decreased the size, but MR shows severe cortico-subcortical atrophy of both parieto-occipital regions. Spectroscopic MR study discloses severe neuronal lost and gliosis. CONCLUSIONS: The shaken-baby syndrome causes severe encephalopathy and vision problems, blindness in many cases, after showing voluminous subdural and/or epidural hematomas which lead to a severe neuronal lost and gliosis. Shaken-baby syndrome is not always associated with skull fracture nor is necessarily related with battered-child syndrome.

Atrophy↗
PubMedDate not supplied

[Hemangiomas, and cutaneous and intracranial vascular deformations (Pascual-Castroviejo syndrome tipe II ). A case report].

OBJECTIVE: To present a case with several cutaneous hemangiomas associated with intracranial hemangiomas and possibly with an intrahepatic hemangioma. CASE REPORT: A boy, 17 months old, showing several cutaneous hemangiomas and epileptic seizures resisting to antiepileptic medication, who also presented psychomotor retardation. The study by angiographic magnetic resonance (AMR ) revealed the presence of three small intracranial angiomas and possibly a hepatic hemangioma. The seizures were controlled with antiepileptic medication. CONCLUSION: This case shows the association of the cutaneous hemangiomas and the subjacent vascular alterations also hemangiomas in this patient which are included in the most frequent neurocutaneous disease, which is known as Pascual Castroviejo syndrome.

Anticonvulsants↗
PubMedDate not supplied

[Clinical progress of neonatal non-ketotic hyperglycinemia under treatment].

INTRODUCTION: Non-ketotic hyperglycinemia is a congenital error in the breakdown of glycine. The most common type is the classical neonatal form, which begins at the age of a few days with symptoms of lethargy, hypotonia, myoclonia, convulsions, apneas and, frequently, ends in death. Survivors usually develop intractable epilepsy and mental retardation. There is no effective treatment for this condition, but trials have been carried out with a therapy that diminishes the levels of glycine, benzoate (BZ), and another that blocks the excitatory effect in N-methyl-D-aspartate receptors: dextromethorphan (DTM). CASE REPORT: We report on the progress of a classical neonatal case, which began at the age of a few hours with hypotonia and stupor, without myoclonias or seizures, but with a suppression wave trace on the electroencephalogram (EEG). Cerebrospinal fluid (CSF) showed glycine levels of 141 micromol/L (the normal level is 6.66 +/- 2.66 micromol/L), with a CSF/plasma ratio of 0.19 (the normal ratio is < 0.02). Treatment was started on the thirteenth day with BZ and DTM, and alertness and eye fixation improved in just three days; at the same time the EEG readings become normal. The glycine level in plasma returned to normal at two months and that in CSF was considerably reduced, although with CSF/plasma levels that were still high. At present the patient is 4 years old, has never had convulsions, EEG results have always been normal, and continues with BZ, DTM, carnitine and diet. The patient has presented a high degree of hypermotoric behaviour, but is currently more attentive and more sociable, has been walking from the age of 35 months and has a quotient in the different areas of development of 40-50. CONCLUSIONS: The clinical progress made by our patient could be said to be anything but negligible, and we therefore recommend that treatment should be started as early as possible after diagnosis.

Benzoates↗
PubMedDate not supplied

[Neuronal migration disorders: clinical-radiological correlation].

We present the correlation of imaging findings with clinical deficits in 34 children with several anomalies of neuronal migration which were studied retrospectively. The cases studied corresponded to: Schizencephaly 10, lissencephaly 4, heterotopy 9, and hemimegalencephaly 2. The diagnosis of the malformations was performed by computerized tomography (CT) in a few patients and by magnetic resonance (MR) in most cases. Among the clinical alterations, psychomotor delay and seizures of difficult control were the most severe, both appeared at early age. The findings observed in this study suggest the correlations between extension, location and gray matter displasticity and the neurological manifestations.

Adolescent↗