Cognitive functioning in children with multiple sclerosis.

OBJECTIVE: To examine the cognitive functioning of children with multiple sclerosis (MS). METHODS: Six children with a diagnosis of clinically definite MS were evaluated using a neuropsychological test battery. RESULTS: The majority of the children showed deficits in at least two of the administered subtests, with IQ scores within the deficient classification. CONCLUSIONS: Verbal and non-verbal skills were equally impaired, and patients who were older at the moment of the onset of the disease had a better cognitive performance. Cognitive deficits should be regarded as a common occurrence in the course of MS in children.

[Pfeiffer syndrome associated with clover-leaf skull: 1st case described in Venezuela]. / Síndrome de Pfeiffer asociado a cráneo en trébol: primer caso descrito en Venezuela.

In 1964, Pfeiffer described a syndrome consisting of craniosynostosis, broad thumbs, broad great toes, and partial soft tissue syndactyly of the hands and feet. It belongs to acrocephalosyndactyly syndromes. We describe a male baby product of an eighth full-term uncomplicated uncontrolled pregnancy, mother and father normal and unrelated, 32 and 50 years old, respectively. He had all diagnostic and prognostic criteria of Subtype 2 Pfeiffer's Syndrome. The clinical, radiological, tomographic, and genetic aspects are discussed.

[Leigh's encephalopathy (subacute necrotizing encephalopathy). Documentation of its evolution through neuroimaging]. / Encefalopatía de Leigh (encefalopatía necrotizante subaguda). Documentación de su evolución por neuroimagen.

A 30 months-old boy developed bilateral nistagmus, tremor, gait disturbance, hypotonia and disartria. The diagnose of Leigh encephalopathy was suggested on the basis of clinical, neuroimaging and laboratory findings. Computed tomography and magnetic resonance imaging (MRI) at an early stage revealed bilateral and symmetric lesions in the putamen, appearing as hyperintense signal on T2-weighted images. Twelve months later a relatively large hypertense area in the posterior brainstem was observed. At this stage, the patient exhibited marked deterioration, dystonic manifestations, rigidity and respiratory disturbances. He died 6 months later for respiratory arrest during bronconeumonic infection. We believe MRI is a valuable means to allow assessment of the evolution of the disease.

[Intraventricular oligodendroglioma. Description of 2 cases]. / Oligodendroglioma intraventricular. Descripción de dos casos.

Oligodendrogliomas usually arise in the cerebral hemispheres, less frequently they are found in the cerebellar hemispheres and very rarely they adopt an intraventricular location. The authors describe two cases of intraventricular oligodendroglioma. Case No. 1: 18-year old woman with a clinical history of headache, vertigo and dizziness of 6 months duration. Central Nervous System imaging revealed a right lateral ventricle tumor. Case No. 2: 38 year old man with a chief complaint of positional headache and visual impairment. C.N.S. imaging showed a third ventricular lesion. The medical literature was reviewed and theories on the genesis of this peculiar location are offered.

[Gliomatosis cerebri. Ante-mortem diagnosis]. / Gliomatosis cerebri. Determinación ante-mortem.

Gliomatosis Cerebri is an unusual neoplastic entity that affects the central nervous system (C.N.S.); it consists of a marked proliferation of glial cells with variable degrees of differentiation in extensive areas of the brain and/or spinal cord. The process is primarily an infiltrative one rather than destructive. Most of the cases are diagnosed at autopsy. The authors report a new case of Gliomatosis Cerebri in a 46 year old woman, with a long clinical history--15 years--of focal seizures; and during the year prior to her admission: behavioral changes, vertigo, urinary incontinence and frequent falls. C.N.S. imaging revealed extensive involvement of the white matter of both cerebral hemispheres. Stereotactic guided biopsy showed a moderately cellular astrocytic neoplasm. A review of the medical literature led to the conclusion that the present case constitutes one of the very few examples in which the diagnosis of Gliomatosis Cerebri was achieved ante-mortem.

[Extramedullary intracranial solitary plasmocytoma. Report of 2 cases]. / Plasmocitoma solitario intracraneal extramedular. Reporte de dos casos.

Multiple myeloma frequently affects the C.N.S with a high prevalence of spinal cord compression following vertebral neoplastic infiltration. On the other hand, solitary plasmacytomas, tumors that arise in a wide variety of locations due to the universal distribution of plasma cells, are infrequent within the cranial vault and rarely present as extramedullary lesions, arising from the intracranial soft tissues without affecting neighboring bony structures. Only 33 such cases have been reported in the medical literature. The authors report two additional cases of solitary intracranial, extramedullary plasmacytoma. Case 1.--54 year-old woman with a chief complaint of severe headache and behavioral changes of three months duration. C.N.S. imaging revealed a bifrontal mass lesion, arising from the anterior third of the falx cerebri. Case 2.--45 year old-man who complained of headache and blurred vision of a month duration C.N.S. imaging displayed a third ventricle mass lesion. In both cases the neuropathological diagnosis was extramedullary plasmacytoma. Postoperatively the confirmation of solitary intracranial plasmacytoma was achieved only after a thorough work-up to rule out the presence of neoplasm elsewhere in the body. Radiotherapy was given to both patients and follow-up has failed to reveal neoplastic disease 4 and 3 years, respectively, after the diagnosis.